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Clinical data of 18 male patients with agammaglobulinemia
Bruton's disease, in other terms X-linked agammaglobulinemia (XLA), is the first reported primary immunodeficiency in 1952, caused by a single genetic defect. The development of B cell is under control of signals transmitted by the B-cell antigen receptor (BCR) complex. Lyn, Syk, and Bruton's tyrosine kinase (BTK) are cytoplasmic protein tyrosine kinases. XLA is caused by mutations in the Btk gene, and Btk mutations are responsible for 85% of all antibody deficiencies. Btk mutation interrupts the B-cell development at the pre-B-cell stage, resulting in the absence of B lymphocytes and plasma cells in peripheral blood and peripheral lymphoid tissues. Up till now, 380 unique mutations have been identified. Autosomal recessive forms of agammaglobulinemia also result in B-cell defects, but more severe bacterial infections are seen in XLA patients due to absolute block in early B-cell development. All serum immunoglobulin isotypes are decreased, and antibody production especially against vaccine antigens is impaired.
- Absence of B cells
- Bruton's disease
- X-linked agammaglobulinemia
- agammaglobulinemia
- Btk gene mutation
- Bruton's tyrosine kinase defect
1. Introduction
Bruton's disease is an X-linked agammaglobulinemia (XLA OMIM No. 300300) that was first described as primary immunodeficiency in 1952 by Dr. Bruton. It is the best-known antibody deficiency [1–5]. More than half of the patients with Bruton's diseases characterized by recurrent bacterial infections such as otitis, sinusitis, and sinopulmonary infections are developing after 7 to 9 months of age when transplacental maternal immunoglobulin G (IgG) levels decrease below protective levels in infants. In this disorder, genetic abnormalities lead to blockage in the maturation of B cells in the bone marrow and only confined to the B-cell lineage. Streptococcus pneumoniae and Haemophilus influenzae are the most common responsible encapsulated pathogens for recurrence of otitis and sinusitis. Pneumonia, empyema, meningitis, septicemia, and septic arthritis are severe infections, which may be the first warning signs of disease for physician to suspect immunodeficiency. The distinctive clinical features of the disease are early onset bacterial infections, absent mature B cells or remarkably reduced circulating B cells, severe reduction in the basal serum immunoglobulins, inability to produce antibodies against antigens, and occurrence of autoimmune diseases paradoxically. Based on these principal findings, approximately 90% of male patients presumed with XLA are likely to have mutation in Btk [6–9].
2. History
In 1952, an American pediatrician, Dr. Ogden Carr Bruton, described the clinical case of an 8-year-old boy who had recurrent episodes of pneumococcal sepsis [1, 2]. Dr. Bruton vaccinated the patient to prevent infections, but the boy did not produce antibodies to Pneumococcus. Electophoretic analysis revealed a lack of gammaglobulins in the patient's serum. Dr. Bruton treated this patient with monthly injections of exogenous gamma globulin. The patient remained free of sepsis episodes for 14 months during which he received injections. The disorder was observed only in male patients. Based on the observation of five additional male patients similar to Bruton's disease by Janeway and colleagues, the disorder later became known as X-linked agammaglobulinemia [9, 10, 11]. This disease was named Bruton's X-linked agammaglobulinemia after having discovered the first immunodeficient patient [12].
3. Prevalence
The frequency of Bruton's disease has been estimated as 1 per 200,000 live births. Prevalence is approximately 1 per 10,000. The prevalence of XLA varies in different countries obtained from published reports. Based on national registries, the prevalence was ranging between 0.09 and 11.25 per 100,000 population [13–17]. The minimum prevalence has been reported as 0.09 (minimum) from Germany [14], while it has been reported as 11.25 (maximum) in the USA [16]. The prevalence of XLA in Eastern and Central European (ECE) countries (total population, 145,530,870) was found to be 1 per 1,399,000 individuals.
4. Btk genes and function
X-linked agammaglobulinemia is caused by mutations in the gene encoding a cytoplasmic protein tyrosine kinase, called Bruton's tyrosine kinase (Btk), in honor of the discoverer of the disorder, Colonel Ogden Bruton, MD. Btk is signal transduction molecule downstream of pre-B-cell receptor (PreBCR) and the B-cell receptor (BCR). It is a key regulator for B-lymphocyte precursors to differentiate into B cells in bone marrow. Mutation in Btk results in the defective production and function of the enzyme. In a healthy person, the enzyme is activated by the pre-B-cell receptor, and it delivers biochemical signals that prompt the B cells to divide or mature and survive. Therefore, patients with defective Btk have almost complete absence of B cells and plasma cells due to arrest in maturation beyond pre-B cell [5–9].
The gene for this enzyme was identified in 1993 by two independent groups [18, 19]. It is located on the Xq21.3-Xq22, the long arm of the X chromosome [18–24]. Btk belongs to a distinct family of protein kinases. Tec, Itk, Bmx, and Txk are other members of this family. The protein contains five regions, PH, TH, SH3, SH2, and kinase domains, and any of these domains may be affected by mutations causing XLA [23, 24]
Since 1993, the number of genetic studies has increased. XLA is a variable disease in certain patients [25, 26]. The types of mutations causing XLA include missense, nonsense, point, frameshift, slice site, deletion, insertion, and premature stop codon mutations [27]. In general, missense mutations account for 40% of all mutations, whereas nonsense mutations account for 17%, deletions account for 20%, insertions account for 7%, and slice site accounts for 16%. This distribution is similar to those listed in Immunodeficiency mutation database [28].
In a study conducted on 56 patients, mutations affecting the Btk gene were demonstrated in 51 patients. It was shown that 22 mutations were missense [29]. In another study, the number of missense mutations was found to be higher [30]. In the study by Chan et al., 12 patients were evaluated, and 3 deletion mutations, 8 nucleotide substitution mutations, and 1 insertion–deletion mutation were detected [31]. In the study carried out in Central European patients with agammaglobulinemia, the point mutations were observed to be more frequent [32]. The first study in 16 Turkish XLA patients was done by Wang et al. [33], seven novel mutations were identified: 2 missense and 4 deletions resulting in frameshift and premature stop codon, novel mutations were determined in 7 cases; 2 missense, 1 nonsense, and 4 deletion mutations were detected. In the last update, lists of the online BTKbase, 1155 entries have been compiled from 974 unrelated families with 602 unique molecular events [32]. The genetic profile of XLA has been studied in 122 patients from 109 families in Eastern and Central European (ECE) countries in 2009 [17]. BTK sequence analysis revealed 98 different mutations, in which 46 of them were reported for the first time. The mutations included single nucleotide changes in the coding exons (35 missense and 17 nonsense), 23 splicing defects, 13 small deletions, 7 large deletions, and 3 insertions.
We conducted a study in Istanbul University, Cerrahpaşa Medical School, Children's Hospital, to determine the BTK mutation in a total of 19 Turkish boy from 18 unrelated families with recurrent infections, almost no CD19(+) B cells and agammaglobulinemia (Table 1) [34].
|Patient no.
|Age at diagnosis
|IgG (mg/dL)
|IgA (mg/dL)
|IgM (mg/dL)
|CD19+ B cells
|Family history
|P 5a
|P 5b
|3 months
BTK gene mutations were determined within 10 patients. The types of the mutations were 3 missense, 4 frameshift and premature stop codon; 2 splice site; and 1 point. Missense mutations were determined in the three patients (patients 2, 4, and 17). In one patient (patient 17), a novel amino acid substitution was determined within the TH domain in exon 6 (c. 491G > A) (p.G164D), which was not included previously in the ESID database. A novel point mutation within the PH domain in exon 2 (c. 49A > T) (p.K17X) was detected in Patient 7. This mutation as well has not been defined previously in the ESID database. The frameshift and premature stop codon mutations were observed to be frequent, followed by missense mutations (Table 2). Although four of the patients have features relevant with a clinical and immunological diagnosis of XLA, a BTK gene mutation could not be determined. In such cases, other autosomal recessive gene defects (µ heavy chain, surrogate light chain λ5, Igα and Igβ signaling molecules, and B-cell linker adaptor protein (BLNK)) should be investigated [35–40]. These genes map proteins involved in maturation of pro-B cells into pre-B cells. These defects have also been shown to result in agammaglobulinemia and an absence of circulating B cells, which cannot be clinically distinguished from XLA.
|Patient no.
|Amino acid
|Exon 18
|Exon 2
|P 5a
|Exon 13
|P 5b
|Exon 13
|Exon 2
|Exon 9
|Exon 15
|Exon 8
|Exon 12
|Exon 6
In the light of these studies, gene defect has to be defined for the accurate diagnosis of XLA, carrier detection, and prenatal diagnosis.
5. Genetic counseling
XLA is inherited in sex-linked diseases (x-linked). As the defects are connected with the X-chromosome and the inheritance is recessive, only male infants are affected. If a boy inherits a defective gene, since he does not have a healthy gene from his father, the boy may have the disease. Women who carry a mutant allele of the Btk gene on one of their chromosomes are carrier of the disease. Therefore, mothers, sisters, and maternal aunts should be investigated for carrier status because they are obligate carriers. Brothers, uncles, or nephews of the mother must be questioned for this disorder. The family history of XLA is nonexistent in approximately 50% of patients. Some of these patients (15–20%) with XLA may have a de novo mutation in Btk gene, and their mothers are not carriers. If the mutant Btk allele is known previously in the family, carrier testing for at-risk female relatives or prenatal diagnosis is possible [41–43].
6. Immunology of XLA
B cells arise from hematopoietic stem cells in the bone marrow. B cells begin to generate and express B-cell receptors (BCRs) (Fig. 1). The entire developmental process of B cells occurs within the bone marrow. A common lymphoid progenitor (CLP) gives rise to pro-B lymphocytes, which next develop into pre-B lymphocytes and then to B lymphocytes. Stimulated B cells may further differentiate into plasma cells that synthesize and secrete immunoglobulins. Mutation in Bruton tyrosine kinase causes arrest in the development of B lymphocyte at the early stage of large pre-B-cell (CD19+ cytoplasmic μ+) stage in the bone marrow (Fig. 2). This defect is leaky, resulting in a few immature B cell. B-cell developmental defects in bone marrow lead to a marked decrease or absence of fully mature B lymphocytes in peripheral blood, absent or few follicles, and germinal centers in lymphoid organs. Plasmocytes are absent, and reticuloendothelial tissue and lymphoid organs (tonsils, spleen, Peyer plaques, and lymph nodes) are poorly developed. Therefore, secondary lymphoid organs such as lymph nodes and tonsils are reduced in size. The consequence of decreased immunoglobulin-producing B cell is diminished in all serum immunoglobulin isotypes, resulting in inability to produce antibodies against protein and polysaccharide antigens. The percentage of T cell is increased, and T cell functions are intact. These patients have the ability to control viral and fungal infections because of intact cell-mediated immunity. The thymus is in normal size and architecture.
Antibodies are produced by plasma cells that are terminally differentiated B cells. When B lymphocytes identify and interact with a specific antigen in the body, it is triggered to mature into a plasma cell that is able to produce specific antibodies. Plasmocytes produce nine antibody isotypes: immunoglobulins G (IgG1, IgG2, IgG3, and IgG4), immunoglobulins M (IgM), immunoglobulins A (IgA1 and IgA2), immunoglobulins D (IGD), and immunoglobulins E (IgE). Antibodies are soluble molecules that bind to antigens to render them harmless by agglutination and neutralization or "tag" the antigens to facilitate destruction and removal by phagocytes and via activating complement components. Antibodies are an important component of humoral immune responses and integral part of body's defense mechanism against bacteria. During the first 6–9 months of life, infants with XLA are protected from infections by transferred maternal IgG antibodies. Reduced maternal antibodies by 6–9 months of age and failures in humoral immunity leave the affected XLA patient with a reduced ability to resist infections and increased susceptibility to encapsulated bacteria and enteroviruses as well. As a consequence, there is a virtual absence of humoral response to recall antigens [9, 12, 44–47].
7. Clinical presentation
Symptoms of patients with XLA most often begin at about 6 to 9 months of age, when the transferred maternal immunoglobulin has been catabolized and the infant becomes dependent on his own immune system. Widespread use of antibiotics may often mask the presentation of disease and despite recurrent sinopulmonary infections diagnosis of XLA may delay until 3–5 years of age or adolescence in some cases [8]. In a small subpopulation of cases with XLA, about 10% to 15%, who are not recognized to have immunodeficiency until 5 years of age, serum immunoglobulin levels may be high [46]. Patients with XLA are clinically characterized by an onset of recurrent bacterial infections due to remarkable decrease in immunoglobulin levels [5–9, 47–50].
The clinical findings leading to the diagnosis of XLA were determined in 82 patients with proven mutations in Bruton's tyrosine kinase. The authors reported that the majority of patients with XLA had a history of recurrent otitis at the time of diagnosis. The physical findings of decreased or absent tonsils and cervical lymph nodes could have alerted physicians to the diagnosis of XLA [51].
Clinical presentations of X-linked agammaglobulinemia have been defined by a multicenter retrospective survey of 96 patients. The onset of infections was in the first 4 months of life (25% of cases), 8 months of life (50% of cases), 12 months of life (75% of cases), and 18 months of life (90% of cases). The most frequent infections involved the upper respiratory tract (75%), the lower respiratory tract (65%), the gastrointestinal tract (35%), the skin (28%), and the central nervous system (16%) [52].
Infection usually develops at the surface of mucous membranes, namely, middle ear, sinuses, and lungs. Pneumonia, otitis media, sinusitis, conjunctivitis, and diarrhea are the hallmark of XLA patients. In some cases, infection can spread through the bloodstream, and septicemia, meningitis, septic arthritis, cellulitis, and osteomyelitis may occur. S. pneumoniae and H. influenzae are the most common responsible encapsulated pathogens of infections. These microorganisms also affect all patients with antibody deficiency, such as common variable immunodeficiency (CVID); however, patients with XLA have susceptibility to enteroviral infection in contrast to other antibody deficiencies.
There are many published data about the clinical presentation of XLA. They are reflecting the results of different population of XLA patients. The frequency of clinical presentations is variable due to different cohorts of XLA patients assessed.
Infection was the most common initial clinical presentation (85%), followed by a positive family history (41%) and neutropenia (11%) in 201 XLA patients included in the United States Registry. The average age of diagnosis was significantly younger in patients with a positive family history (2.59 years) than in patients with a negative family history (5.37 years) (p < 0.001). Seventy percent of patients had at least 1 episode of otitis, 62% at least 1 episode of pneumonia, 60% at least 1 episode of sinusitis, 23% at least 1 episode of chronic/recurrent diarrhea, 21% at least 1 episode of conjunctivitis, 18% at least 1 episode of pyoderma and/or cellulitis, 11% at least 1 episode of meningitis/encephalitis, 10% at least 1 episode of sepsis, 8% at least 1 episode of septic arthritis, 6% at least 1 episode of hepatitis, and 3% at least 1 episode of osteomyelitis. Of 201 patients, 14 (6.9%) were dead at the time they were entered in the registry. Causes of death were disseminated enterovirus infection (n = 6), pulmonary insufficiency (n = 5), adenovirus infection (n = 1), sepsis (n = 1), acquired immunodeficiency disease syndrome (AIDS) (n = 1), myocarditis (n = 1), hepatitis (n = 2), and stem cell transplantation (n = 1) [53].
The median age at the onset of the disease was 8 months, and the median age of diagnosis was 48 months based on the records of 33 Iranian XLA patients. The most frequent infections were seen in the respiratory tract (93.9%), gastrointestinal tract (75.8%), central nervous system (33.3%), and musculoskeletal system (21.2%). Chronic otitis media, chronic sinusitis, chronic diarrhea, and bronchiectasis were developed complications in 75.8% of cases [54].
The mean age of onset was 2.5 years, and the mean age at diagnosis was 7.3 years in six patients with XLA from northern Thailand. Patients had a history of otitis media, pneumonia, arthritis, and sinusitis (5/6); pericarditis (1/6), meningitis (1/6), and pyoderma (1/6) were other experienced infections. Pseudomonas aeruginosa and Staphylococcus aureus were isolated on multiple occasions in five patients. Five cases had developed bronchiectasis and three patients septicemia [55].
Recurrent pulmonary infections and an unusual course of common pulmonary infection should alert physician for underlying immunodeficiency. Lung infections were evaluated in 39 Iranian patients with XLA. The authors reported that 82% (32/39) of patients with XLA experienced at least one episode of pneumonia, and 84% (27/32) of those patients had more than one episode. An average rate of pneumonia episode per patient per year was 1.67 for XLA patients [56].
A patient with XLA presenting with respiratory distress, was reported to have Pneumocystis jirovecii pneumonia. This patient is a reminder that the potential consequences of BTK deficiency in cells other than B-cells should be considered [57].
Mycoplasma infections may involve the respiratory tract, the urogenital tract, and joints, leading to prolonged than severe course or asymptomatic in some cases [58, 59].
Skin infections such as impetigo, abscesses, and furuncles due to group A streptococcus or staphylococcus had been also reported.
Although patients with XLA experience childhood viral infections uneventful, they have susceptibility to certain viruses. Enteroviral infections (echovirus, coxsackievirus, and polio virus) frequently run a severe course and often resist therapy in affected patients. Enterovirus may cause severe and, eventually, fatal progressive encephalitis [5–9, 52, 60, 61].
Clinical manifestations of enteroviral meningoencephalitis demonstrate great variation changing from severe infection to chronic enteroviral infection. Illness may be overt as acute infection with fever, headache, and seizures or tend to progress slowly throughout the years with loss of cognitive skills, ataxia, paresthesia, neurosensory hearing loss, and lethargy [60, 61]. The laboratory assessment of cerebrospinal fluid (CSF) may reveal clear fluid, pleocytosis (<1000/mm3), elevated protein level (0.5–5 g/dL), and in a few cases hypoglycorrhachia or normal CSF results. Polymerase chain reaction (PCR) techniques may detect virus in CSF. Initial magnetic resonance imaging (MRI) or computed tomography (CT) scan reveal no abnormality; however, cortical or subcortical atrophy, ventricular dilatation, periventricular changes, and hydrocephaly may be observed later in life. There are no typical histopathology findings at brain biopsy. Illness is progressive and mortality is very high. As a result of disseminated enteroviral infection, dermatomyositis-like syndrome may develop with erythematous rash and peripheral edema. Hepatitis with increased alanine aminotransferase (ALT) may accompany [60–63].
Gastrointestinal disorders are common problems of XLA patients. Patients may have infections, autoimmunity, or rarely malignancy. Campylobacter jejuni is the most frequent pathogen causing gastrointestinal tract infection. Typically, affected patients suffer from fever, skin rash, and persistent diarrhea [64–66]. Parasitic infection especially Giardia lamblia infection may cause abdominal pain, diarrhea, poor growth, or loss of serum proteins. Giardia can be isolated from stool samples of patients and too hard to eradicate.
Inflammatory disorders in XLA patients unexpectedly occur. A web-based patient survey was conducted in patients with XLA. Based on 128 patient responses, the majority of respondents (69%) reported having at least one inflammatory symptom. Just 28% of them had been diagnosed with an inflammatory condition. Arthritis had been diagnosed only in 7% XLA patients, despite 20% reported painful joints and 11% reported swelling of the joints. Similarly, 21% reported symptoms of chronic diarrhea and 17% reported abdominal pain. However, only 4% had been diagnosed with Crohn's disease. Data from the United States Immune Deficiency Network (USIDNET) Registry on 149 patients with XLA revealed that 12% had pain, swelling, or arthralgia, while 18% had been diagnosed with arthritis [67]. Kawasaki disease is also reported in an XLA patient, providing opportunity to understand the relationship between autoimmunity and XLA [68].
Noninfectious or infectious arthritis may occur in patients with X-linked agammaglobulinemia. Juvenile rheumatoid is relatively common in patients with XLA. The mechanisms are not clear of noninfectious arthritis. H. influenzae, S. pneumoniae, and mycoplasma are pathogens causing septic arthritis. Pain and swelling of joints are presenting symptoms. Erythrocyte sedimentation rate increase, rheumatoid factor (RF), and antinuclear antibody (ANA) tests are negative. Arthritis may be the first presenting symptom of X-linked agammaglobulinemia. That is the reason why physicians must be aware of immunodeficiencies [69, 70].
Growth hormone deficiency associated with XLA patient has been reported [71].
Although the relationship between the Bruton kinase mutation and the development of malignant tumors is unknown, XLA patients seem to be at risk for colorectal cancer and lymphoid malignancies. B-cell precursor acute lymphoblastic leukemia (BCP-ALL) has been reported in a case with XLA. It has been reported that somatic mutation found in MLL2 suggests that the alterations of BTK and MLL2 synergistically function as leukemogenesis [72, 73].
Cases with vaccine-associated paralytic poliovirus infection as a consequence of attenuated oral polio (Sabin) have been reported in XLA patients [74]. The incubation period of the infection is more than 30 days, and chronic encephalomyelitis develops eventually [75].
8. Physical Examination
Remarkable physical findings of XLA patients are the absence or hypoplasia of tonsils and lymph nodes. Chronic otitis, sinusitis, mastoiditis, or bronchiectasis are consequences of recurrent infections.
9. Diagnosis
Significant progress in the field has been achieved in the light of collaborative studies. Guidelines for screening primary immunodeficiencies have been published by experts, non-immunologists and immunologists [51, 76–78]. The classification of primary immunodeficiency diseases had been updated by the ad hoc Expert Committee of the International Union of Immunological Societies [79]. The hallmark of clinical features and laboratory evidences were provided for each immunodeficiency, which help physicians to recognize and diagnose patients with immunodeficiency timely.
9.1. Clinical clues
X-linked agammaglobulinemia (XLA) should always be considered in children with recurrent, persistent, unusual sinopulmonary infections or a life-threatening severe bacterial infection below 5 years of age. Small tonsils and lymph nodes on physical examination are warning signs of disorder. Patients with strong family history of immunodeficiency relevant with X-linked inheritance should be investigated further.
9.2. Laboratory approach
Based on studies, detailed family and medical history combined with careful physical examination further guide specific blood tests of the immune system, which will be performed step by step to confirm the diagnosis of XLA. Severe defects of immune systems should be ruled out at the first steps of diagnostic protocol for suspected immunodeficiency. Infection with human immunodeficiency virus (HIV) has to be excluded rapidly. Tests have to be performed and interpreted by a specialist immunologist. The use of age-matched reference values for lymphocyte subsets and immunoglobulin levels are highly recommended to avoid misinterpretation.
Baseline blood tests give useful information. The complete blood test (CBC) is a crucial test to reveal anemia, neutropenia, lymphopenia, thrombocytopenia, or eosinophilia, which may give a clue to which type of PID is present. Neutropenia is associated with a small subpopulation of patients with XLA (11%), which can be misdiagnosis as congenital neutropenia [8, 80, 81]. Severe neutropenia is usually in association with pseudomonas or staphylococcal sepsis. A CBC and a manual leukocyte differential can aid in the identification of striking lymphopenia, which is a very important clue for severe combined immunodeficiencies (SCID) accepted as medical emergency.
Screening tests for antibody deficiencies as recommended by experts are presented as follows [8, 50, 76–78, 82]:
Serum total protein level
Serum immunoglobulin assay: IgG, IgA, IgM
Isohemagglutinins (IgM antibodies to A and B blood group antigens)
Specific antibody responses
Tetanus, diphtheria (IgG1)
Pneumococcal and meningococcal polysaccharides (IgG2)
Viral respiratory pathogens (IgG1 and IgG3)
Other vaccines: hepatitis B, influenza, MMR, and polio (killed vaccine)
Lymphocyte subpopulation by flow cytometry (B-cell quantitation)
Lymphocyte proliferation tests
B-cell maturation analysis in bone marrow
Genetic determinations of defect
Serum immunoglobulin concentration should be measured by quantitative techniques, and IgG, IgA, and IgM are routinely measured in serum. Values of determined immunoglobulin levels have to be compared with normal-for-age values. The majority of patients with XLA have less than 200 mg/dL serum IgG level. However, serum IgG concentration is more than 200 mg/dL in 10% of children with XLA. Serum IgM and IgA are classically less than 20 mg/dL. Low serum IgG concentration may also be determined in patients who have protein-losing enteropathy and nephrosis. The concomitant serum protein levels of these patients are low; hence, they produce antibodies normally. Under certain conditions, the determination of IgE and IgD levels may be required.
Isohemagglutinins are natural IgM-class antibodies against A and B blood group antigens. Therefore, they are not found in patients who have type AB blood group. In addition, the measurement of isohemagglutinin titers in the serum is not reliable below 6 months of age. Normal values for anti-A titer is 1:16 or higher and anti-B titer is 1:8 or higher. Isohemagglutinins titer is low in XLA patients due to poor IgM synthesis.
Individuals with XLA fail to make antibodies against vaccine antigens or pathogens such as tetanus, H. influenzae, or S. pneumoniae. Since children are vaccinated with diphtheria–tetanus—acellular pertussis (DTaP), conjugated H. influenzae type b, and conjugated pneumococcal vaccine (PVC), the measurement of antibodies against these antigens is informative. The measurement of specific antibodies against diphtheria and tetanus before and 2 weeks after DTaP or booster DT immunization reveals a patient's ability to produce IgG antibodies against protein antigens. Pneumococcal polysaccharide vaccine or conjugated pneumococcal vaccine (PVC) is used to evaluate a patient's ability to respond to polysaccharide antigen (IIb C). The measurement of pneumococcal antibody titers before and 4 to 8 weeks after vaccination should be done in patients more than 2 years of age (Ib A). The normal response to each pneumococcal serotype is defined as a titer equal to or greater than 1.3 mg/mL antibody (IIb C) [83]. The ability of antibody production against antigens and the response to vaccination are severely impaired in XLA patients.
Patients who have agammaglobulinema need lymphocyte phenotyping. The number of B lymphocytes in the peripheral blood can be enumerated by flow cytometry using dye-conjugated monoclonal antibodies (CD 19 and CD 20), which are specific B-cell markers. B cells constitute 4–10% of the peripheral lymphocyte. The reduced number of CD19+ B cells in the peripheral blood would be indicative of defective B-cell differentiation and would suggest XLA if not combined with depleted T-cell numbers. B cell is markedly reduced below <1% in patients with XLA [76–79, 83].
Mutations in BTK gene can be scanned by sequence analysis, which can detect approximately 90% of mutations in BTK.
10. BTK protein testing
BTK mutations occur in the absence of the BTK protein in monocytes. The detection of BTK protein in monocytes by immunofluorescence or western blot [84, 85] can confirm the diagnosis of XLA.
Female carriers may be determined by mutation analysis or Btk protein expression on blood cells by flow cytometry. They have normal immune functions, but they have a 50% chance of transmitting the disease to each of her sons.
The following diagnostic criteria for X-linked agammaglobulinemia were published by Conley et al. in 1999 [86]:
Definitive diagnosis—males with less than 2% CD19+ B cells and at least one of the following:
Mutation in BTK
Absent Btk mRNA on northern blot analysis of neutrophils or monocytes
Absent Btk protein in monocytes or platelets
Maternal cousins, uncles, or nephews with less than 2% CD19+ B cells
Probable diagnosis—males with less than 2% CD19+ B cells and the following:
Onset of recurrent bacterial infections in the first 5 years of life
Serum IgG, IgM, and IgA more than 2 SD below normal for age
Absent isohemagglutinins and/or poor response to vaccines
Exclusion of other causes of hypogammaglobulinemia
Possible diagnosis—males with less than 2% CD19+ B cells in whom other causes of hypogammaglobulinemia have been excluded and who has at least one of the following:
Onset of recurrent bacterial infections in the first 5 years of life
Serum IgG, IgM, and IgA more than 2 SD below normal for age
Absent isohemagglutinins
Chest X-ray or CT scan of patients with XLA reveals bronchiectasis most commonly distributed in the middle or lower lobes, atelectasis, and bronchial wall thickening. CT scan of sinuses may suggest the presence of chronic sinusitis.
Prenatal diagnosis—this may be achieved by using mutation analysis in amniotic fluid cells and Btk protein expression on cord blood cells by flow cytometry.
11. Newborn screening
Kappa-deleting recombination excision circles (KRECs) are chosen as markers for B lymphopenia at birth, indicative of X-linked agammaglobulinemia. The measurement of KRECs in newborn would help the early diagnosis of XLA patients [87].
12. Differential diagnosis
Differential diagnosis should be done by other disorder with hypogammaglobulinemia such as CVID, X-linked hyper IgM syndrome, and X-linked lymphoproliferative disease.
It had been known for several years that there were girls who had an immunodeficiency that looked just like XLA, and immunologists had suggested that there were forms of agammaglobulinemia with autosomal recessive inheritance (ARA). Since 1996, several genes (μ heavy chain deficiency, λ5 deficiency, Igα deficiency, Igβ deficiency, BLNK deficiency, PI3 kinase deficiency, and E47 transcription deficiency) that can cause ARA have been identified. All of these genes code for proteins that work with BTK to support the maturation of pro-B cells into pre-B cells. Patients with mutations in any of these genes have clinical and laboratory findings that are very similar to those seen in patients with mutations in Btk (Table 3) [35–39].
|Genetic defect
|Serum Ig
|Associated features
|Btk deficiency
|Mutation in Btk, a cytoplasmic tyrosine kinase activated by cross-linking the BCR
|All isotypes decreased
|Severe bacterial infections: normal numbers of pro-B cells
|μ heavy chain deficiency
|Mutation in μ heavy chain: essential component of the pre-BCR
|All isotypes decreased
|Severe bacterial infections: normal numbers of pro-B cells
|λ5 deficiency
|Mutation in 15: part of the surrogate light chain in the pre-BCR
|All isotypes decreased
|Severe bacterial infections: normal numbers of pro-B cells
|Igα deficiency
|Mutation in Igα (CD79α) : part of pre-BCR and BCR
|All isotypes decreased
|Severe bacterial infections: normal numbers of pro-B cells
|Igβ deficiency
|Mutation in Igβ (CD79β) : part of pre-BCR and BCR
|All isotypes decreased
|Severe bacterial infections: normal numbers of pro-B cells
|BLNK deficiency
|Mutation in BLNK: a scaffold protein that binds to Btk
|All isotypes decreased
|Severe bacterial infections: normal numbers of pro-B cells
|PI3 kinase deficiency
|Mutation in PIK3R1; a kinase involved in signal transduction in multiple cell types
|All isotypes decreased
|Severe bacterial infections: decreased or absent pro-B cells
|E47 transcription deficiency
|Mutation in TCF3:a transcription factor required for control of B-cell development
|All isotypes decreased
|Recurrent bacterial infections
|Myelodysplasia with hypogammaglobulinemia
|May have monosomy 7, trisomy 8,or dyskeratosis congenita
|One or more isotypes may be decreased
|Infections: decreased numbers of pro-B cells
|Thymoma with immune deficiency
|One or more isotypes may be decreased
|Bacterial and opportunistic infections: autoimmunity: decreased numbers of pro-B cells
13. Treatment
There is no curative treatment for XLA. Therapeutic measures consist of intravenous immunoglobulins (400–600 mg/kg monthly in order to maintain the IgG levels at 500–800 mg/dL), specific treatment of bacterial infections with antibiotics, and bronchodilators. The mainstay of treatment consists of immunoglobulin replacement therapy and prolonged antibiotic treatment of suspected bacterial infections. Immunoglobulin replacement therapy is essential for the XLA patients who are unable to produce sufficient antibodies against antigens. IgG is purified from thousand of human plasma and contains a wide range of antibodies against so many infections. Thus, it is life saving for XLA patients, and they have to continue to receive to survive. The aim of immunoglobulin treatment given by intravenous (IVIG) or subcutaneous (SCIG) infusions is to avoid acute infections, to decrease the number of bacterial infections, to improve quality of life, and to increase life expectancy of patients [8, 9, 47, 88–92].
IVIG infusions have to be done at hospital or home by professionally educated staff if possible. The common recommended dose of IVIG treatment for antibody replacement is between 0.3 and 0.6 g/kg, administered every 2 to 4 weeks via the intravenous route. The first IVIG infusion must be given slowly starting with a rate of 0.5 to 1.0 mg/kg per minute. Patient should be monitored closely for any adverse reactions during infusion. If the patient tolerates well, the infusion rate may be increased to 1.5 to 2.5 mg/kg per minute after 15 to 30 minutes. The maximal infusion rate is 4 mg/kg per minute, and infusion of an IVIG product should last 2 to 4 hours. The aim of IVIG therapy in patients with PID is to maintain serum IgG levels between 350 and 500 mg/ dL. Since there is a large variation in individual IgG elimination rates, the periodic measurement of serum IgG concentration is critical to monitor the adequacy of replacement during therapy. Retrospective studies in patients with XLA revealed that the severity and the number of infections especially pulmonary diseases are decreased depending on IVIG dose [88]. Serious bacterial illnesses and enteroviral meningoencephalitis were prevented when maintained IgG levels were above 800 mg/dL [89–95].
A 5-year multicenter prospective study on 201 patients with CVID and 101 patients with XLA was conducted to identify the effects of long-term immunoglobulin treatment and the IgG trough level to be maintained over time required to minimize infection risk. Overall, 24% of patients with XLA remained infection free during the study. In addition, in XLA, the comorbidity risk factor identified for pneumonia was the presence of bronchiectasis [96].
Infusion-related adverse effects and transmission of blood-borne viruses are adverse effects of immunoglobulin replacement therapy [97]. Reduced adverse reactions are reported with improved and new IVIG products. The subcutaneous IG (SCIG) therapy was reported to be effective, safe, and well tolerated in children and adults. High treatment satisfaction (TS) scores and health-related quality of life (HRQOL) were advantages of SCIG. Subcutaneous infusions are recommended to patients who are small children, reactive to IVIG, or have problem with vascular access. SCIG is given as a parent-managed or a self-managed treatment. Norway, Sweden, United Kingdom, and Belgium are the countries in which SCIG is often applied to children [98, 89]. Clinical records of 1151 XLA patients identified from ESID were included in ESID registry. According to ESID registry, 305 XLA patients were treated with IVIG (73%) and 114 patients were treated with SCIG (27%) [98, 99].
Bacterial infections treated with a high dose of selected antibiotics or antibiotics sensitive to yielded pathogens for prolonged periods.
Six young patients with XLA treated with cord blood or bone marrow transplants were reported. No one benefited from transplantation, and expected increase in serum IgM or blood B-cell number was not observed [100].
14. Vaccination
Live viral vaccines, such as oral polio, rotavirus, yellow fever, live attenuated influenza, and live bacterial (e.g., typhoid [Salmonella typhi, Ty21a]) vaccines should not be applied to patients with X-linked agammaglobulinemia. Therefore, inactivated polio vaccine (Salk) should be given to patients with XLA and their family contacts. These patients may develop vaccine-acquired diseases such as central nervous system infection due to oral poliovirus vaccine.
The effectiveness of the measles and varicella vaccines are uncertain because most patients receive IVIG and do not have capacity to produce antibody responses [101, 102].
However, bacille Calmette–Guérin (BCG) vaccine applied to 50 patients with X-linked agammaglobulinemia did not reveal any systemic infection [102].
15. Complications
The most common secondary complications of XLA are chronic sinusitis, chronic lung disease, malabsorption, and enteroviral infection. The delay in diagnosis of XLA remains a significant problem, as a consequence of recurrent pneumonias, bronchiectasis, pulmonary hypertension, and finally cor pulmonale may develop. Many patients have been diagnosed after chronic sequel had already been existed. The aggressive use of antibiotics can decrease the incidence of chronic sinusitis and lung disease. Hearing loss is a consequence of chronic otitis media. Early diagnosis and treatment of bowel infections may decrease the risk of inflammatory bowel disease. Renal AA amyloidosis had been reported in 38-year-old patient with Bruton's disease [103].
16. Mortality
Chronic lung diseases and infections, especially disseminated viral infections, and meningoencephalitis due to enterovirus are major causes of mortality. Treatment with newly developed and improved immunoglobulin products and antibiotics and improvement of care of immunodeficient patients would reduce the mortality rates and increase the survival of XLA patients. Registered 41 XLA patients were followed up 20 years until 2010. Among 41 patients, 26.8% died during the follow-up period. All of the complications existed before the initiation of treatment was reduced after immunoglobulin replacement therapy, except sinusitis and conjunctivitis. The associations between some immunological and clinical characteristics such as lymphocyte subsets, consanguinity marriage, and mortality were documented [104].
17. Prognosis
In the case of the early diagnosis of XLA and an appropriate therapy before the appearance of sequel, prognosis is well. They are encouraged a full active lifestyle and children can attend to all regular school. Attention should be paid to pulmonary infections and complications in the long-term follow-up of patients. XLA is a chronic disease; patients need immunoglobulin replacement therapy to avoid infections and care of a multidisciplinary team of specialists for the rest of their lives [98, 99].


text:
Posted by EyeFootball 20 hrs ago
Posted by astamfordbridgetoofar 21 hrs ago
Chelsea enquire about Barcelona star Samuel Umtiti According to Express, Chelsea have met with Barcelona star Samuel Umtiti's agent as they plan to bring the player to Stamford Bridge. The 26-ye... [Read More]
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Welcome to the second Chelsea FC News Now update today and in this update we feature a look at Marina Granovskaia in addition to news from the Premier League and Chelsea Transfer News. Check out the ... [Read More]
Posted by DailyMail 23 hrs ago
Hudson-Odoi was one of the first Premier League figures to be diagnosed with the deadly disease, along with Arsenal manager Mikel Arteta, and their cases led to the shutdown of the top flight. [Read More]
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Welcome to today's five minute Chelsea News and Chelsea Tranfer News this lunchtime with plenty to feature including Jadon Sancho's £400k per week potential wages, Chelsea's squad value decrease to th... [Read More]
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The latter stages of this season's Champions League and Europa League are reportedly set to be moved to July and August as the coronavirus pandemic continues to wreak havoc. [Read More]
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subset: commoncrawl


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Swimming: The Panthers swim team competed at South Albany on Jan. 28 against the Dallas Dragons and South Albany Red Hawks.
The girls team tallied up 74 points for first place, and the boys team took last place, with 46 points.
The Panthers girls team got things started with a first-place finish for the 200-yard medley relay, with senior Dalia Robolledo-Hernandez, senior Olivia Cooper, senior Lynze Bradley and freshman Taylor Hagedorn swimming to a 2:21.37.
In th 200-yard freestyle, Bradley picked up gold with a time of 2:25.51. Robolledo-Hernandez swam to first place in the 100-yard freestyle, clocking in at 1:13.72.
Hagedorn clocked a 5:34.53 in the 500-yard freestyle event for a first-place finish. She also took first in the 200 Individual Medley relay, finishing at 2:20.88.
For the 100-yard breaststroke, Cooper clinched first place with a time of 1:23.51.
Sisters Olivia Cooper and Sarah Cooper, and Hagedorn and Bradley competed together in the 200-yard freestyle, finishing in first place in 2:01.75.
In the final relay of the day, the 400-yard medley relay, Bradley, Sarah Cooper, Robolledo-Hernandez and Madeline Altenburg made a strong showing to nab first place in 4:47.92.
On the boys side, senior Sean Stephenson took first place in the 100 freestyle, clocking in at 2:12.70.
For the 400 medley relay, Clayton Amerman, Nathanial Thompson, Moises Falcon and Stephenson swam competed to win first place in a time of 4:16.01.
The team traveled to Silverton on Tuesday after press time.
Swimming: After competing on Jan. 28 against South Albany and Central, the Dallas Dragons took last place for the girls side, with 15 points, and the boys took second with 47 points.
Wrestling: The Dragons competed in the Reser's Tournament of Champions on Friday and Saturday, batlting against some of the toughest teams in the state. Senior Ashton Brecht, 285, came away with a second-place finish after two grueling days of wrestling. Senior Cooper Hise nabbed a fifth-place finish in the 195-pound bracket, and junior Cael Morrison picked up fourth place for 126.
The Dragons compete at Silverton on Saturday at 10 a.m.
For the girls team, freshman Loony Stork took first place in the 100-yard backstroke, clocking in at 1:08.05.
On the boys team, Jakob Lloyd, a junior, swam to a first-place finish in the 100 freestyle with a time of 58.48.
Sophomore David Beasley clinched gold in the 100 backstroke, with a time of 1:13.93.
In the 200 IM relay, senior Gabe Applegate finished first, clocking in at 2:46.74.
The team traveled to Silverton after press time on Tuesday.
Falls City
Boys Basketball: After a 77-62 loss against Livingstone Adventist Academy (6-8, 3-6 Casco League) on Thursday, the Mountaineers (14-6, 7-3 Casco) picked up an easy 51-34 win at Triangle Lake (2-16, 1-9 Mountain West League).
The team traveled to Willamette Valley Christian (5-14, 4-7 Casco) on Tuesday after press time.
Girls Basketball: On Thursday, the Mountaineers (10-10, 4-6 Casco) suffered a 38-19 loss against Livingstone Adventist Academy (9-5, 5-4 Casco), but ended the week with an exciting, 23-22 win at Triangle Lake (10-8, 8-2 MWL). The girls were on the road to Willamette Valley Christian (12-7, 7-4 Casco) on Tuesday after press time.
Men's Basketball: The Wolves edged out an explosive 111-78 victory at Concordia on Jan. 28, followed by 98-75 road win at Montana State University Billings.
The Wolves head to Seattle Pacific on Thursday; tipoff is 7:30 p.m.
Women's Basketball: For its fifth loss in a row, the Wolves fell 68-59 at Simon Fraser on Thursday, followed by a 61-57 loss at Western Washington University.
The team hosted Concordia on Tuesday after press time.
Men's and women's track and field: Not only did junior Derek Holdsworth take gold in the men's 800-meter race at the University of Washington indoor meet on Saturday, but he also set a new record for WOU, as well as for the Great Northwest Athletic Conference in a field of 40 runners. Additionally, he was the only Division II runner to finish in the top 10. He finished the race in 1:47.74.
Senior Justin Crosswhite nabbed a new personal record in the mile, placing 25th overall in a time of 4:07.30, and moving to a third-place spot in the WOU record books, behind David Ribich and Dustin Nading.
Junior Moana Gianotti was the second competitor at the meet to place in the top-10 in shot put for DII. She reached a throw of 44-09.00 to grab 10th place.
The Wolves travel to the University of Portland Feb. 8 and 9.
Women's Softball: Over the weekend, the Wolves competed in the Desert Stinger classic in Las Vegas. The team started things off on Jan. 31 with a 11-1 loss against San Francisco State University, followed by a 5-1 victory that afternoon against Sonoma State. On Feb. 1, the Wolves clinched another win, this time against Colorado Christian, 10-7. That afternoon, they fell 14-0 to Oklahoma Christian.
For the team's final game on Feb. 2, the Wolves lost 7-2 to California State, San Bernardino.
On Saturday, the Wolves host Eastern Oregon University for a doubleheader, starting at 11 a.m.


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ReThink Media is a unique, nonprofit organization that strengthens movements. We help build the collaborative media and communications capacity of state and national advocacy groups; increase the voice share of their spokespeople, experts, and allies; and provide in-depth media, messaging, and opinion research to inform movement strategy. Working with our funding partners, we focus on long-term engagement with our advocacy partners and with the issues we take on—all driven by a belief that the stronger the groups are across an issue sector and the tighter their collaboration, the more likely they are to win real and lasting policy victories. At present, ReThink works in three distinct, yet interconnected, issue areas: the protection of human and civil rights; strengthening our democracy; and a more constructive US foreign and national security policy.
Currently accepting applications for:
Program Director, Rights & Inclusion
The Opportunity
2020 will be a pivotal year for Muslim, Arab, Sikh and South Asian Americans and indeed, for all Americans who believe in a pluralistic, just, and inclusive society where every person is treated with dignity and respect.
While some politicians have exploited prejudice and fear for their own self-interest, impacted communities and their allies have pushed back, built power for their organizations, and struggled against policies grounded in bigotry. Overturning the Muslim Ban will most likely be decided by the end of 2020 and the context of the political debate will define much of the struggle to advance the rights of individuals to be free from discrimination, anti-Muslim prejudice, racial and religious profiling, and unwarranted surveillance and suspicion.
ReThink Media seeks a passionate and high-performing advocate to lead our Rights and Inclusion program – supervising a team of communications professionals, working alongside our outstanding Media and Opinion Analysis team, and drawing upon the media skills and extensive contacts of our full staff. Our Rights and Inclusion team works with dozens of Muslim, Arab, Sikh, and South Asian American communities across the country, with U.S.-based Muslim and non-Muslim allies promoting cultural equity and inclusion in the arts, and with allies supporting these goals, including interfaith, business, civic, and veteran's groups.
This is also a unique moment where we can either hire a seasoned senior leader who is ready to start in the Program Director role immediately or an Associate Director who can be mentored for 6-9 months with the expectation that they will grow into the Director role in that time.
The Program Director, in coordination with the Director of Strategy and the Executive Director, will serve as the organizational lead for all of our work within this sector. In this senior level position, the Program Director will oversee initiatives to develop shared media and messaging strategies, provide media and spokesperson training, build organizations' media outreach and rapid response capacity, provide groups with media and opinion analysis, and support multi-organizational communications initiatives and campaigns.
The ideal candidate will be a highly ambitious and motivated communications strategist with a minimum of six or more years of experience working in the field of advocacy, political or campaign communications and will have a sophisticated understanding of the workings of the non-profit research and advocacy community. They will have direct experience and/or demonstrated commitment to the issues of the collaborative and an understanding of the organizations working in this field. They will also have a proven track record of project management and a demonstrated pattern of innovation and creativity. Strong management skills are critical. Experience working as a Capitol Hill or NGO communications staffer, supervising or managing a communications team or campaign, and/or a proven track record in developing media outreach strategies, and successfully pitching and placing content and/or spokespeople in the media is preferred.
Principal Duties
Specific goals will be set quarterly, corresponding to the following duties:
- Develop annual work plans to achieve organizational objectives and grant deliverables. Meet regularly with the Director of Strategy and the Deputy Director of Strategy to discuss work, adapt and reevaluate plans, respond to breaking events, etc.
- Serve on ReThink's senior management team. Participate in setting organizational policies, establishing strategic priorities, advancing organizational development plans, etc.
- Effectively supervise and lead a dedicated team of communications professionals. Facilitate ongoing check-ins and planning meetings, conduct annual performance reviews, manage the hiring process for any new hires, ensure adherence to organizational policies and values, and develop written professional development plans for each team member.
- Assist in the development of annual grant proposals and prepare regular progress reports and briefings of key stakeholders and funders of the collaborative.
- Serve as a primary point of contact with organizations served by the collaborative, helping to set priorities and identify media strategies that will have the greatest positive impact for organizations and campaigns.
- Act as the final editor for the quality of all content produced by the Rights and Inclusion team including daily news clips, messaging and talking points, shared press lists, training materials and presentations, media and rapid response resources and engagement on listservs, and any other guides or publications.
- Develop expertise regarding the issues and organizations served by the collaborative, the issues they work on, their unique media profiles, key spokespeople, areas of expertise, and their access to outside validators.
- Keep abreast of the evolving media landscape and identify media opportunities for organizations. Be able to quickly identify the most appropriate organizations or experts to connect to a reporter, provide spokespeople, write an op-ed, etc.
- Coordinate opportunities for multi-organizational media engagement and support ongoing campaigns and policy initiatives.
- Manage the delivery of high quality media skills training to improve the core skills of key leaders from the field.
- Facilitate the use by our allied organizations of a range of media tools, including a media database, distribution system, and news clipping service.
- Participate in longer-term research initiatives such as message testing and media and public opinion audits; coordinate between ReThink's research team or outside consultants and member organizations to ensure that research is aligned with the field's needs.
- Facilitate rapid response media engagement, using coverage monitoring to identify and coordinate joint responses to breaking news developments.
NOTE: We are open to the possibility of hiring an Associate Director who will be coached and mentored in all of the above with the expectation that they will assume primary responsibility for the work after six to nine months.
Essential Skills and Qualifications
- A strong commitment to advancing a social justice agenda.
- Ambition to develop and execute innovative campaigns, capitalize on media opportunities, and drive policy objectives via the media.
- The ability to be substantially self-directed and self-reliant and to drive work forward following consultations on broad strategic objectives.
- Strong writing and presentation skills.
- Demonstrated experience in communications strategy and campaign management.
- Strong facilitation and project management skills.
- Detail-orientation and a proven track record working in a very fast-paced, multi-faceted work environment, ideally involving media and communications and/or coalition relations.
- Strong supervisory skills with experience effectively managing and mentoring others.
- Knowledge of print, broadcast, online and social media.
- Demonstrated knowledge and background in the major issues facing Muslims, Arabs, Sikhs, and South Asians in the United States.
- Ability to communicate effectively, both verbally and in writing, with varying levels of organizational staff, clients, contractors, and foundations.
- Bachelor's degree in Public Relations, Communications, Journalism or other applicable field, or demonstrated equivalent experience.
Location, Benefits, & Salary
This is a full-time position based in either our Washington, DC or SF Bay Area office, with a modest preference for the former. Roughly 45% of ReThink's staff is in California, 45% in DC, and 10% in other states. Applicants must be comfortable with the requirements of remote management. The position will require moderate travel to conferences and training workshops, as well as staff and planning meetings. Candidates should have at least six years of full-time experience in advocacy communications.
The salary range for this position is $80,000–95,000. Benefits and related issues will be shared with qualified candidates prior to interviews.
Application Instructions
This position is open until filled and candidates are applying on a rolling basis. Our number one priority is finding the ideal candidate, but we are moving quickly to fill this position, so we encourage you to apply as promptly as possible. All applications will be acknowledged, but please, no phone calls. To apply, send an email to [email protected], and include the words "Program Director-R&I" in the subject line of your email. Include your resume and cover letter as PDF attachments and make sure to have your name in the filename of each attachment (e.g., cover letter-Jones.pdf).
Join our team, check out our fellowship and internship program
ReThink is committed to a just and equitable society wherein all people are treated equally, opportunity is justly distributed, and all voices are heard. We affirm and seek diversity in gender, race, color, ethnicity & national origin, age, sex (including pregnancy, gender identity and sexual orientation), class, religion, ability, life experiences, and background as both a positive contribution to our organizational environment and a critical asset for informing and enabling our work. With this in mind, we are committed to maintaining a work environment that recognizes, understands, respects, and encourages the unique contributions of each member and we approach every hiring decision with a commitment to workplace diversity.
ReThink Media is an equal opportunity employer grounded in progressive political values. We embrace applicants of any race, religion, sex, national origin, ethnicity, age, political affiliation, sexual orientation, gender identity, color, marital status, medical condition (cancer-related) or conditions Acquired Immune Deficiency Syndrome (AIDS) and AIDS-related conditions (ARC). Reasonable accommodation will be made so that qualified disabled applicants may participate in the application process. Please advise in writing of special needs at the time of application.


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Think about the first time you really fell in love with sports, it was probably when you were a child. Parents and children spend an awful lot of time and money on athletics – from clothes and supplies to lessons and transport. Even children as young as a few weeks old are participating in athletics like "Mommy and Me" yoga.
In 2017, more parents spent money on sports and athletics than ever before. You can bet that brands see the promise here and will spend a great deal of money on marketing and development in the next few years.
Read for yourself this CNBC Report on the rising costs for kids to play sports!
tags: little league youth sports


text:
Hard Times: Shell Down De Street
in collaboration with Levi Naidu-Mitchell
Degree Show One, Central Saint Martins, London UK
In our streets, our collective rhythmic gravitation towards a celebrative revolt of the body may free us from any expectation of work. [...] By the pelvic flare of our indeterminable bodies, we refuse the right to work. By the explosive scream that casts our bodies towards freedom, We clamour for the right to wuk.
Hard Times, a collaboration with Levi Naidu-Mitchell, involved a series of Wukshops (workshops), performative interventions, lectures and discussions leading up to the mobilisation of a small-scale Mas band. A brief interview, facilitated by University of the Arts London, covering some of the intentions of this work, may be seen here.


text:
Teen gets frisky during quad bike ride into woods. Aroused black sluts fuck each other with dildo in fantastic orgy. Hardcore orgy in an orgy with horny students casting Daisy, Gail and others. Jennifer Black gets a guests in her asshole after bike riding. The video has been added to your member zone favourites. Fuck and Creampie Sister bike gym. Two hot tempered whores ride quad bike and meaty cock.
Alexa. Age: 21.
The video has been added to your member zone favourites.
Addyson. Age: 25.
Sexy biker girl Mia Stone masturbates outdoors over her black bike
Cumshots at Fantasy Fucking Orgy. Exotic black haired chick Lee Lexxus prepares her anus for an orgy. Brazzers House Orgy Finale.


text:
What(!) a Homo(?)February 17, 2007 at 9:08 am | Posted in basketball, whitney | 2 Comments
I guess one of us should write something on Amaechi being gay all of a sudden, and I figure that someone might as well be me. I am, admittedly, no basketball expert (though I used to know a hell of a lot about the Jazz. Is Hornasec still around?) but I think I'm probably the resident gay expert on the site since I once had a gay roommate who introduced me to all his gaywad friends and I recently went to dinner with a table of lesbians – one being a gypsy.
Okay. So he's gay. And if we take a look at the various statistics on homosexuality, at least every other professional team has a gay teammate. Talk of "we can't trust him if he didn't tell us!" strikes me as the most ridiculous argument you could come up with. Never having been on a professional sports team I might not completely understand, but there are a lot of things I don't tell my film crew about my personal life and we survive. They can still count on me for a decent wardrobe/script supervision/craft services. So and so once had a kid and he won't pay child support despite the fact that he earns millions. Would not knowing this information affect whether or not you can pull off a terrific double play? I submit that it will not. (A revolutionary statement, I'm sure)
No, I don't want dudes in my locker room. I don't want people who might (implausible) be sexually attracted to me taking showers with me. Okay. I guess I understand that argument….if I didn't see you every night giving interviews in a towel, naked people roaming two lockers away. Some of those reporters are women. Explain yourself.
And I love how Tim Hardaway got himself into trouble by being a total homophobe, as though he has a right to be so. All over talk radio they're comparing his statements to other statesments on mortality. As though "I hate gays" is just like saying "I hate child molestors" or "I hate terrorists." My dad made a good point about these hate mongerers being so twisted that in their minds these homos are just as bad morally as a child molestor. So maybe the right approach is a kind of education. But doesn't it seem really obvious that being gay involves the individual consent of two adults whereas child molestation…doesn't? Tim Hardaway is the biggest idiot since that woman manager that hated black people.
This is a pretty worn out subject and I have no new insights, but since I don't spend much time listening to talk radio, the news is very new to me. And distressing. This Don't Ask Don't Tell stuff is getting fairly ridiculous when every single person that comes on the radio – be he Michael Jordan, his personal assistant, or his waterboy – is asked what they think of Amaechi as though it is any of their business. Some might argue that Amaechi is making it our business by using it as such a publicity stunt. But isn't publicity exactly what professional sports needs on this issue when no one has come out before retirement?
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this article, while still showing tim h to be a homophobe, shows how he was probably set up by espn to create an even larger controversy in the hope of selling more books: http://dwil.wordpress.com/2007/02/19/dan-lebastard-does-tim-the-hard-a-way/
Comment by brian— February 22, 2007 #
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What's up, guys? Jeff Cavaliere, ATHLEANX.com. We're going to break this sucker out one
more time today to help you see how you can take one exercise and allow it to hit two
completely different sides of your body. A push with your chest, or a pull with your
back. It's all about how you do it. So when you're training at home I think
one of the biggest benefits of an exercise that you can come up with is versatility. The more versatile a home exercise is, without
using equipment, the more beneficial it becomes, and the more important it becomes to your
training regimen when you're trying to train with nothing around you. At ATHLEANX we have mastered how to train
with your own body, and how to build muscle while doing it. A lot of you don't know how to build muscle
by training with your own body. Here's how you work your chest and your
back. If you're at home, you're going to do
the exercise I'm showing you here. This is called a floor pullover. Now we know that the dumbbell pullover is
one of the best exercise you can do for building your back, and I've also shown you in the
past videos how tweaking the contraction, and the inhiation of the contraction on a
dumbbell pullover can actually shift a lot of the focus right here to your chest. Your upper chest. Now I want to show you how you can do it in
the home version of this. The No Equipment version of it. When you're in here, and you're pulling
down it's all about where you initiate that contraction. We know that you can either initiate the contraction
with your lats by pulling with your elbows. So when I'm out here, in this position,
if I pull down and lead with my elbows – so you watch the exercise here again as I'm
doing it. If I were to lead with my elbows and pull
down we're going to maximize the contraction of the lat, and the contribution of the lat
to the pullover. It's the same way if you were doing a dumbbell
version of this. You'd have the dumbbell over your head,
you lead with your elbows, with your lats all in one piece. Now, the tweak I want to make here – if
you want to shift the focus and get some work in on your upper chest – is instead of pulling
down and leading with the elbows, you actually try to pull in, and squeeze. The inhiation of this contraction is what's
going to pull the arm down, and back, or your body in this case, back up. So here's how it works. Here's why I'm breaking out the marker. As you see here, we have discussed in previous
videos how the upper chest fibers – the ones that go from your clavicle – they all
come in this way. They all come up like this. Up to your upper arm here. So they're all angled that way. When our upper arm is up, over our heads,
now you can see that they're angled up this way. If we were to contract them this way they're
going to pull down that way. So it's pulling my arm down here to initiate
a pullover, but we're using the upper chest to do that. So when you're in this position what you
want to do is – okay, I'm on the floor now, out in that fully extended position. When I'm on the floor, when I pull, I stick
my chest out, and then I contract there. I'm contracting this way. I'm pulling my hands, even though they're
fixed on the floor, in that way, to pull me down. Then come up, and over the top. Here, into the floor, pull my body up, and
down. Here, into the floor, pull my body up, and
over. You can see that we can shift the focus. So what I like to do with this exercise is
do it both ways. Either do a set to focus more on the contraction
on the top of the chest, or do a set to focus more on the contraction here in the lats. Or alternate your reps. A set for the top, a set leading with the
elbows. A set here for the top, a set – I mean,
a rep – from the top, and a rep from the elbows through your back. Guys, you can build muscle, and you can make
this exercise very, very difficult depending upon how far you allow your body to stretch
out. It's more that you have to overcome to pull
yourself back. By overloading, using your own body – forget
it. Your body is just like dumbbells, and weights,
and everything else. If you can provide an overload, you can build
muscle. That's how we do it here, and that's how
you have to start doing it. especially if you're going to try to train
and build your chest at home, with nothing at all. Our ATHLEAN0 program is a completely bodyweight
only program. No equipment at all. We can allow you to build some serious muscle
in just six weeks by following that program. You can get that over at ATHLEANX.com. In the meantime, if you've found this video
helpful make sure you leave your comments and thumbs up below. Let me know whatever else you want to see
on this channel. This is a Bodyweight Wednesday workout video. Sometimes it will be Washboard Wednesday. If you guys want more Bodyweight Wednesday
I'll make sure I do that, too. Just let me know in the comments below and
I'll do my best. Guys, I'll see you soon.


text:
Aduro U-Grip Plus Universal Bicycle Mount
Seriously woot, quit lying about your original prices. This thing is $15 every day on amazon.
The crossed out price isn't Woot's original price.
It's the MSRP.
I bought two of these from Woot a week or so ago. They seem decent quality, but the part that goes onto your bike frame is exceptionally large. It was too large for either of my bike handlebars, and only is barely small enough for the main pipe that goes down from the handlebars and connects to the frame. This is on a 24" Mongoose and a 26" Magna. Neither of these are small little kid bikes (one mid sized and one adult). Never had a problem with other brand cell phone holders. It barely works, but is not tight on either bike and a small amount of pressure from one finger can move the mount around which makes me worry about stability and the phone falling out on a bump.
Although neither are considered kids bikes, they were designed for the price tag. I'm guessing that the bars on your bike are 25.4mm diameter versus the beefier 31.8mm that the less price-conscious bikes tend to use.
If you have some rubber strips, it can take up the space under the clamps to make a tighter fit.
I also ordered one a week ago and it fits my Cannondale just fine. It comes with not one but two inserts that you can use to make the clamp smaller. You really tried both and it still was too large? I'm wondering just how tiny your bike's handlebars are. I found the thing almost too small.
Anyway, I did a 20-miler today on bumpy roads with this thing on and it held my iPhone very well; better than the previous, far more expensive phone holder I'd been using. I'm quite satisfied.
@Narfcake, You seem to be can expert on handlebar sizes. What size are mine?
[unfiltered comment]Mustache rides?[/unfiltered comment]
Yup. You guys are right. The wife had put them on the kids bikes. After the suggestions I went and tried again, and they are OK as long as I use both of the rubber pieces. Not everyone wants to pay >$500 for a bike for a junior high aged kid. We're all on a deal a day website for a reason, right? These are just normal bikes. I'm sure the info will help someone else out there. Yes, they will work fine on the regular bikes. Thanks!
I don't either, and it's for myself! It's probably why I enjoy KevCentral over other YouTube channels too.
(At least not all at once. The pay-as-you-go upgrade method has worked out well for my 5½ bikes, though.)


text:
Laural Roaldson: Wilderness Calling
By James Florence, Diamond Certified Resource Reporter
WALNUT CREEK — While it took some time for Laural Roaldson to realize her true calling, it was nevertheless something she'd known all along in her heart. "I grew up in the woods of Virginia, and I've always felt at home amidst nature," she says. "Early in adulthood, I got a job in the corporate sector, but over time, I felt a growing sense of disconnectedness. Eventually, I left to pursue a career in landscaping." Years later, Laural launched a second foray into the corporate world, only to find the same results as before. "I felt so unfulfilled; it was like the universe was telling me, 'No, you don't belong here.' I knew I had to get back to what made me happy, so I decided to return to the landscaping industry for good."
Today, as owner of Laural Landscapes, Inc., Laural says her favorite part of her job is the spiritual satisfaction she receives from cultivating the land. "When I'm focused on working with plants and figuring out what the land wants, I feel content…at peace. There's a connection I experience with the Earth that grounds me and reminds me of who I am."
Laural came to the Bay Area in 1998 and resides today in Concord. Outside of work, she engages in a variety of active and artistic pastimes. "I love performance art like live music and theater, but I like going to the movies, too," she says. "I also enjoy music. I like to sing, and I've written a few songs." Additionally, Laural enjoys activities like camping, swimming and spending time with her daughter, Amy, who is currently a business major at Cal Poly in San Luis Obispo.
In regard to her professional career, Laural believes landscape design should be a client-centered mode of expression. "When I design a landscape, I want to make sure it reflects the customer's tastes and personality," she explains. "In our increasingly fast-paced, detached culture, nature is an avenue for people to reconnect with themselves, which is why it's crucial for your landscape to be representative of your self—a place where you can feel comfortable, relaxed and at home."
When asked the first thing she'd do if she were to retire tomorrow, Laural says she'd take her wilderness calling to the next level. "I would buy a piece of land that was rich in flora and fauna and turn it into a wildlife sanctuary. Ideally, it would be a place where certain endangered species could be introduced and allowed to flourish."
Ask Me Anything!
Q: If you could travel anywhere in the world, where would you go?
A: I'd like to go to South or Central America, just to see a rainforest in person.
Q: What's your favorite kind of tree?
A: That's a tough one, but I'd have to say the valley oak, just because of how big, old and enchanting they are.
Q: What's your favorite thing to do on a rainy day?
A: Watch a movie…or the rain itself.
Q: What's your favorite local restaurant?
A: Swagat Indian Cuisine in Concord.
Q: Do you collect anything?
A: Plants!


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Bridgemen - 1980
Suncoast Sound - 1988
Freelancers 1983
Sky Ryders 1987
Knights of Geneseo - 1983
- 1
Bridgemen - 1980
Suncoast Sound - 1988
Freelancers 1983
Sky Ryders 1987
Knights of Geneseo - 1983
I would have to say:
1. Boston's Witch burning, and also the acting in Wicked Game
2. BD's Off-the-Line beginning
3. Mandarins' awesome take on Sound of Silence
4. Cavaliers running "joke"
I just wanted to add my voice to the chorus of fans who absolutely loved this show! It was, hands down, one of the best live drum corps experiences I've ever had - and, given that I've been involved with this activity for over 30 years, says something.
Looking back, there are only a handful of shows that stirred my emotions like Wicked Games. The commitment to character, talent of all sections, and stunning production values all combined to put 12 minutes of pure magic on the field. Then, if that wasn't enough, that absolutely brilliant "burn at the stakes" effect at the end was, by far, one of the all-time best pieces of visual mastery that will be talked about for years to come.
Thank you, BAC, for Wicked Games...a show I will talk about and savor for many, many years.
OK - I truly think that it's important to separate the two issues - that way both the current financial urgency can be addressed, and the long-term solvency of the corps as a whole can be dealt with afterwards.
First - I have known Ibe for about 10 years and I have never questioned his dedication to the kids, or the organization. While there have been previous organizations who have made a similar plea (only to have it turn out to be b.s.), Ibe is not the sort of person who would put out a distress call unless it was truly an emergency.
Also, the paramount issue at hand is making sure those kids are taken care of...and, right now, letting them complete their season is at the top of the list. Why? Because they have put everything they've got into the corps...and to have it cut short with nowhere to go at this late juncture just isn't fair to them.
As for the other issue - this can, and should be, addressed after the season. The organization will need to review what happened, determine how or if it could have been prevented, and determine whether they can recover...or cease operations.
That's my $.02
Shadow has loads of potential, as well as an extremely well organized and established booster group - something that many new corps lack their first year. Add to that an infrastructure that already understands touring to a certain degree, and you have an administrative base that's light year's ahead of most first-year corps.
Also, they were one of MACBDA's more successful groups, so they also have a strong performance/competitive history.
I'm really looking forward to seeing what this group brings, this year.
Blue Devils 2016 "As Dreams Are Made On"
OH MY...as someone who studied Shakespeare in college, this has me giddy with anticipation and excitement!!!
While BD has never been at the top of favorites list, this show has the potential for converting me.
Yes, indeed, there will be a second percussion judge - which was voted on by the instructors and passed on to the Directors, who approved it.
The reason was centered on safety. With only one field judge, there were several instances where that judge caused, or nearly caused, collisions with performers - mainly because they had to cover so much downstairs and were practically running all over the field - between battery and pit.
However, the second judge will be primarily upstairs and will observe the front whenever the field judge is focusing on the battery...thus making it so the on-field judge doesn't have to run through sections, but rather can maneuver more cautiously.
Initially this will only be at regionals and championships, but eventually should go full-season.
I live in Columbus and know the Director, Marshall Cheathum. He's been leading the Saints drum line since their inception 11 years ago and is committed to making the drum & bugle corps a locally focused group. If the corps is anywhere near as successful as the drum line has been - both competitively, as well as with developing their members; most of whom have no other outlet - then this corps will be fantastic.
Best of luck, Saints!
The 2013 Racine Scouts are having auditions for color guard.
WHEN: Saturday, May 4
REGISTRATION: 9:00am
CAMP: 9:30am-7:30pm
WHERE: Church of the Nativity
3658 E Plankinton Ave
Cudahy, Wisconsin 53110
RSVP, or MORE INFO: Matt Mackowiak
Music City is a no show...anyone know what's up?
Strange...because DCI schedule has them going to Indy, along with several shows en-route.
Another lightning warning. Stands about to be cleared until further notice.
Show has been delayed until 7:05.
Hey there...
I'm interested in fielding a corps and have some questions before I declare my intention.
1. What's the difference between the divisions?
2. Would I have to start in D-3 and work up to D-1, or could I start in D-1?
3. Is there any cost to register?
That should be a good start...and I'm looking forward to participating in the circuit...
YAY!!! The Khaki/Tan was/is my favorite!
And how'd you get in for $14? It cost us $15 at Crosswoods (about 5 miles and 10 minutes away)?
(That guy probably looked at you and said "What a dork.")
Actually, that guy was me .
Regarding the theater experience...
Overall - sound wasn't too bad...of course, Blue Stars sounded the loudest of all the groups because of their staging and extremely mic'd pit.
OK - some observations:
Overall - some interesting productions. Yes, some had more dirt than others, and the fact that they were all either 100% completed, or #### close. Great job, everyone.
Blue Stars: Move the ramp/platform thingy further back, or situate it so that you can use more of the field.
SCV: Way too esoteric and bland for me. Some intricate drill, but the concept of escaping from Hell does not come across at all.
Phantom: I really wanted to like this show, but I just couldn't. This show is seriously lacking impact moments, which makes the show flat. I'm really hoping they add and tweak A LOT, or this will not be a good year for Phantom.
Bluecoats: Love the first 1-2 minutes of the show. Unfortunately, the show is 11 minutes long. Percussion is the strongest section and they play the heck out their book. However, the concept of the show is lost behind the overall muddled musical selections.
Crown: Far and away my favorite show. I NEED to see this show live because, if I loved it in the theater, I can't imagine how WOWED I'll be when I see it in-person. Contrary to a lot of other observations, this show has a lot of demand, but it's executed well. This show is why I love Crown - it's an audience accessible show with a great concept that they sell 100%.
Blue Devils: OK, I've made smarta** comments about BD's new uniforms, as well as their shows for the past several years. I won't here, but I need to ask fans of this year's BD production; what am I missing? My thoughts when seeing/hearing this show is that it's not a top-5 show in any way. There is no demand, it's lacking in even medium-velocity drill, and I don't understand any of what they're trying to convey. So, I ask again; what am I missing? because I just don't see how this show is even breaking a 70.
Cadets: Love the concept, am BLOWN AWAY at their drill (they are seriously bookin'), and really love how the entire corps is conveying the product. The two separate colored uniforms work incredibly well with the drill design, and they look really good, too. When they clean this up, they'll be contending for the title.
Cavaliers: These are the Cavies I've come to know and love. The drill is tight, the guard is performing at a higher level than this part of the season usually affords, and the concept/design works! Could easily win the title.
My Favorite Corps / How I Saw the Placement:
8. SCV / Blue Stars
7. Blue Devils / SCV
6. Phantom / Blue Devils
5. Bluecoats / Phantom
4. Blue Stars / Bluecoats
3. Cadets / Cadets
2. Cavaliers / Crown
1. Crown / Cavaliers
I'm really looking forward to seeing how everyone improves and transforms throughout the season.
Not one worth sharing at least.
Hmmm...this is a thread on 2011 uniforms, which is a topic geared towards offering opinions about said uniforms. I stated my opinions, but obviously you disagree. That's fine.
However, you also think dissenting opinions aren't worth expressing. Sorry if you're offended, but get over it. Until this site becomes a one-opinion only forum, my opinions are worth just as much as yours.
OK...so, for what my 2 cents are worth (yes, I know, 2 cents...)...
Cascades - Pretty nice, especially the lighter shades of green. I always thought the dark/forest green got lost on the field, so this is a nice change.
Colts - hmmmmm. I think it's better than what they've been wearing since '07, but that's not saying too much. While the bolty thingy is intresting, I don't know how well it's going to read on the field.
Cadets - nice concept, but need to see it on the field. However, they are a nice variation on the traditional Cadets look.
Phantom - I really love this new uniform. A nice change of style without completely going off the deep end. However, I'm still one of those rare creatures who thought the Khaki uniforms were the best...and I still do.
Trooper - I like the way the uniform looks, but not on the Troopers. I'm a fan of the original...it concretely defined the corps and gave them their identity.
Academy - uh, excuse me, but your corps is based in Arizona, the summer around the rest of the United States is generally hot to scorching...AND YOU HAVE BLACK-FREAKING UNIFORMS? I love the way they look, BUT IT'S A BLACK-FREAKING UNIFORM. 'nuff said.
Blue Devils - two words: EPIC FAIL! I know some of the drum corps universe thinks this is the quintessential, state-of-the-art piece of brilliance that makes BD the gold-standard of the activity (you also probably thought Constantly Risking Absurdity was the all-time best show)...and, that's the great thing about a subjective activity - their will never be 100% agreement, or vice-versa. However, this uniform is bordering on vomit-inducing. I don't care that it supposedly fits their show. In my opinion, it ranks as one of the worst ever...not that I have an opinion.
The drum corps universe grew a person smaller yesterday as Rich Skare, alum of SCV, BD, Renegades & Frontier, passed away.
Rich didn't revolutionize the activity, didn't set new standards for a particular section, or wasn't even a legend (for most of y'all here, at-least).
Rich was, however, someone I believe was somewhat of a poster child for the drum corps life: he played his instrument good n loud, had a great time doing it, insisted that everyone else do the same, and had the kind of infectious attitude that just made you want to be around him. He gave drum corps everything he had...and didn't regret it for a second.
I marched with Rich in the SCV Tournament of Roses Parade corps in 2004. I consider myself fortunate that we were able to meet and begin a friendship (yeah, it was occasional chatting on Facebook, but we always had a blast).
So, I just want to say to Rich...you left us before we wanted you to. You're going to be missed, but not forgotten. Please say hi to G.R. for all of us Vanguard alumni...and, most importantly, rest in peace.
May You Never Walk Alone...May Your Sound Always Be Heard.
I also saw the brothers at OSU...I had no idea they were going to be there. For some reason, I decided that I was going to have my morning coffee while walking around campus. When I arrived at Ohio Stadium, I was pleasantly surprised to find the equipment truck parked just outside. Guess I picked the right day to walk around campus...
Great rehearsal, and amazing run through.
I'm proud to be an alum, and really can't wait 'til they march onto the field at Lucas Oil Stadium on Saturday night...go get 'em, brothers! May You Never Walk Alone!!!
Hey Genius, this isn't a thread about the BK penalty, it is a discussion about penalties in general. Check your stuff before you make a post that is irrelevant, inaccurate, and completely unnecessary.... and oh by the way got PWNED by someone who took the time to read the thread title.
Hey, dude...why the rude comment? Alright, so this isn't about the BK non-penalty, but that was the catalyst for this thread in the first place, so back off the kid. So sorry you didn't approve of his post...I never realized that you alone are the judge of topic post relevancy.
To address a few of the issues listed above -
At the 2-minute mark of the corps entering the field, the announcer is supposed to announce, "Ladies and Gentlemen, please welcome to the field, from (insert hometown here), (insert corps name here).
Then, at the 30-second mark (I believe), the announcer asks if the corps is ready - at which point, the d.m. salutes and the announcer says, "presenting their show, 'blah, blah, blah,' Drum Corps International is proud to present," etc.
Now, because pre-shows seem to have different timing so that they can have maximum impact, a staff member usually prompts the announcer so that everything times to the corps' actual beginning, which opens up the problem of actual timed judging. I wish everything would be consistent...but, because the pre-show has now taken on a much more integrated part of the overall design, this concept has pretty much gone the way of the 2-valve bugle.
As for penalties assessed because a corps is late - shows are scheduled to the minute so that they can have ample time for the entire competition, but also not start so early that fans can't be there in-time for the start. In addition, noise ordinances are much more strict these days, with many cities having curfews that begin as early as 10pm. Because of this, if a corps is late, it really messes with the overall timing and puts the show sponsors in jeopardy of violating city law and receiving their own penalty/fine.
Yes, it sucks that a corps receives a penalty for being late, but it is a rule that was voted on by the Board of Directors. Regardless of the reason for being late, the penalty needs to be enforced across the board.
Interesting. I haven't seen any shows this year yet thus why I read reviews like no tomorrow. But it is interesting that most of the reviews see something completely opposite then you. Others have said that the Cavaliers theme is not clear and it's not cohesive.
Thanks for the review. Shows everyone that everyone's perspective is different.
There are some clarity issues in this show...that I will agree with other posters/reviewers. However, when I saw/heard the show, the clarity issues - in my opinion - were minor. In this day of themed shows, I look at shows from a theater director's perspective; does the product have an understandable beginning/middle/end, do the performers deliver the story line successfully, and does the show produce an emotional response. For me, these answers were all yes, to varying degrees.
I will agree with other folks in that this is a show that you either love, or hate. There is no like about it.
But, as you said, everyone interprets art in different ways...and, I love that folks have been respectfully giving their comments.
I was probably a bit harsh and too "smartassy" with that comment...I'm sure that y'all will make considerable and fantastic progress as the season continues. Oh, and just for the record, my spouse - who has zero drum corps background, but who probably gives the best commentary on the corps - said that you guys have a show that he'd love to see later on this summer.
All the best as you guys continue...and, I'm definitely going to avoid stadium nachos from now on...
Alright, I just got home from the Emerald City Artistry in Motion show in Dublin (suburb of Columbus), and I'm quite jazzed about the shows I saw tonight (for the most part)...and, with that, here is my take...
VENUE - This stadium is a great drum corps stadium. The stands rise nearly 50 rows high, and are on a nice/steep pitch. I was on side 1, at about the 30-35 yard line in row NN (basically - row 40) and had a fantastic view of the drills...I loved it, and a great value at $19.
Cincinnati Tradition (all-age): I'm pretty much guessing this was their Opening Night show...and it was pretty obvious. Show was a Latin-style show that featured, "One More Time Chuck Corea," that was practically the same arrangement, note for note, played by BD in 1982 (complete with pelvic thrust and audible grunt). Musically this show was very hard to digest (and the nachos I had a few minutes earlier didn't help matters). Horns hacked/fracked and were trying to find notes the entire time (soloists somehow thought they were in BD and tried to play that way with, let's just say less than successful results).
Guard was small, and it was clear that they only had work for about 2/3 of the show. They spent the entire closer on the front sideline with handheld fans, which they used the entire time (and, with the really hot temperature at showtime, may have helped to keep them cooler).
Overall - this was a tough first show for them. And, if somehow this wasn't their first show, well then all I can say is, "OY!"
Legends (Open Class): The title of their show (Ruins), their choice of music (Vesuvius, Eruption [from ELP's Tarkus], Selections from Pines of Rome, Unstoppable, and Prelude-Main Title from Ben Hur, and what I heard/saw on the field...None of It Made Sense.
I couldn't make out the music they were supposed to be playing, their performance didn't evoke any of what it was supposed to, and the overall concept was completely lost on me.
That said, the kids are doing everything they can with what they've been given. Execution wasn't bad, but it's obvious they can't convey the emotion of the show when I'm sure they don't know what that emotion is supposed to be.
This has been a running theme with this corps. I've always found that they have talented kids, but they aren't being given a product where they can show off and maximize to their fullest. I've always found that the design team creates overly esoteric shows that sound great to them, but don't translate to the field.
Overall - Kids did everything they could, but the show design and concept just prevented them from showing off their talent.
Teal Sound: Let me state right up front, I really like this show. Well created/designed, it has a clear theme throughout, and it's something that I want to see again as the season continues. For their first statement in WC, they're making it a strong one!
Some folks have commented on DCP that their guitars were too loud and distracting. Tonight, they were at all. Balance wasn't a major issue, and their electronics weren't a negative.
The big thing holding them back right now is execution. Individual performer problems abound (especially in the visual department), their hornline was quiet reserved (which in this show should be the opposite), and they suffer from some stamina issues. But, nothing is too severe that they can't fix throughout the season.
Overall - this is a show/corps that has a lot of untapped potential. If they get it clean and fix the individuals, they can slip into semis.
Madison Scouts: I'm a Scout alum, I'm biased, and I admit that. I marched in the corps the last time they played Rhapsody in Blue (1985), and I was looking forward to seeing/hearing this year's rendition.
Simply - THIS SHOW ROCKED!!! It's good ol' fashioned drum corps...no cutesy title, no having to explain what's supposed to be happening...none of that. They enter the stadium, the play full arrangements of Slaughter on 10th Avenue and Rhapsody in Blue (not momentary excerpts), they're powerful, they have the swagger/aura back, and I LOVE IT!
Dirt? Of course. But everything about this year's corps feels like a blast from the past - retro style uniforms, familiar music, all sections strong...or, to sum it up: MADISON!!!
Overall - they're back in the top-12 this year. This show is really good...but, they do need to work on the little things - especially in the visual department - if they're going to get back to the upper-echelons of the drum corps world.
Blue Stars: The Houdini show is cute. However, it's one of the shows where there is so much happening from start to finish that you miss a lot. And, from what I can interpret, there is going to be more coming to help tell their story. The problem with that, however, is that there's just going to be more things to miss seeing from a fan's perspective.
However, there are some absolutely beautiful moments - the ballad is really nice, and the guard spinning silks with a key on them adds a nice touch.
Overall - this show is nice, but I just don't see it going any further - competitively, or otherwise.
Cavaliers: This is another show that has a lot of stuff going on. BUT, they do a much better job telling the story, "Mad World." This is another show that I really like because it has a clear theme, a clear beginning/middle/end, and is wonderfully performed. The all three sections mix/mingle throughout the show (the entire hornline joining the guard on rifles was a great moment) and compliment each other's strengths.
Overall - The Cavies are, once again, a solid corps with a great show. I don't agree with their score/placement tonight, but I have no doubt that they'll be solidly in contention for a medal.
Carolina Crown: To quote Dom DeLuise in the movie, History of the World Part 1, this show is "Nice. Not wonderful, not incredible, Nice."
There is nothing bad about this show - I like the music, the performance, and the performers...but, there really isn't any true WOW moments at any point. They had an OK run, but I disagree with their placement tonight...I don't think their performance tonight warranted First Place.
Overall - It could easily win the whole thing this year, but I personally don't believe this show will medal in Indy.
Bluecoats: There is something about this show that clicks for me in a way no other drum corps show in this era of electronics/synths/etc. has. I can't really describe. I personally thought they had a Gold Medal performance tonight. The show flowed well and was engaging from start to finish, and for me is one of the best Bluecoats corps in a long time.
I can see why they've been scoring as high as they have, and why the reviews on here have been raving...they deserve it, and tonight's performance just added to that list of positive reviews.
Overall - I can see Bluecoats as a dark horse for the title because this show is the real deal.
FINAL THOUGHTS: I'm glad I was able to attend because there's some #### good stuff happening this year. Every show has a lot more choreography with ALL the elements, not just guard...the entertainment factor is considerably higher than in previous years, and the quality of performers overall is fantastic.


text:
Due Friday 2/14/20
Rachel's Open Mind Assignment- follow directions
Eleven CER #2- use CER Cheat Sheet- pay attention to your REASONING
It is important that students attend school tomorrow- we need to finish our Eleven and Point of View Test. Students will NOT do their best if they try to take the test after a week off of school. Please be here tomorrow!
Mid-Winter Break is next week. There is NO SCHOOL Monday 2/17 through Friday 2/21. School resumes on Monday 2/24.