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Pancreatitis as a manifestation of mitochondrial disorder

We appreciated the article by Debray et al. [2006] about a patient with Kearns–Sayre syndrome and recurrent pancreatitis, which was attributed to the mitochondrial disorder (MD). We want to add some further information and experience with patients who have MD and pancreatitis. Debray et al. [2006] regard pancreatitis as a rare manifestation of MD. We agree when looking on the published cases. A MEDLINE research on the topic ‘‘mitochondrial disorder, pancreatitis’’ detects 15 references, of which only 10 are of clinical relevance. However, when looking at our own experience the prevalence of pancreatitis is much higher. Pancreatitis not only occurs in patients with MD and diabetes, but also as a single-organ manifestation of a MD or, more frequently, as part of a multi-organ MD. In most patients with pancreatitis it usually recurs more or less frequently. Pancreatitis in MD patients often takes a mild course with only abdominal pain and elevation of the pancreatic enzymes a-amylase and lipase but hardly necrosis or shock. In a 38-year-old male with multi-system MD, the homoplasmic A!G transition at np8381 manifested as disabling myopathy, stroke-like episodes, epilepsy, left ventricular noncompaction, anarthria, and pancreatitis [Finsterer et al., 2004]. Pancreatitis had occurred several times in this patient so far and was associated with diarrhea but did not result in the development of diabetes so far [Finsterer et al., 2004]. In a 51-year-oldMDpatientwith juvenile onset seizures, short stature, impaired hearing, left ventricular noncompaction, and myopathy, pancreatitis was associated with recurrent emesis and diarrhea and resulted in diabetes [Finsterer et al., 2006]. In a patient with multi-system MD, including migraine, epilepsy, polydipsia, atrial fibrillation, arterial hypertension, diarrhea, and hepatic steatosis, pancreatitis had occurred 15 times so far [unpublished]. All these patients responded well to pancreatine. Other MD patients with pancreatitis reported were a 10-year-old girl with MERRF syndrome due to the A8344G mtDNA mutation, presenting with easy fatigability, tremor, and astatic seizures [Toyono et al., 2001], a newborn male with mitochondrial depletion syndrome [Tsao et al., 2000], a 7-month-old boy carrying the A3243G mtDNA mutation who presented with failure to thrive, stroke-like episodes, microcephaly, developmental delay, seizures, and lactic acidosis in addition to pancreatitis with hyperglycemia [Kishnani et al., 1996], anda 10-year-oldMD boy with myopathy due to complex-IV-deficiency [Kato et al., 1990]. Chronic pancreatitis with pancreatic calcifications was also reported in the index patient of a pedigree with maternally inherited diabetes and deafness (MIDD) syndrome due to the A3243G mtDNA mutation [Schleiffer et al., 2000]. In a family with MELAS syndrome due to the A3243G mutation pancreatitis resulted in beta-cell damage and impaired glucose tolerance, respectively, hyperglycemia with high levels of islet cell antibodies [Oexle et al., 1996]. Acute hemorrhagic pancreatitis has been observed in a 17-year-old male with Alpers syndrome, which is a developmental mtDNA depletion syndrome due to POLG mutations [Montine et al., 1995; Nguyen et al., 2006]. A recent report also showed that mtDNA mutations, which go along with diabetes, are associated with poorly developed islet structure, reduced size of the exocrine pancreas, and subclinical chronic pancreatitis [Clark et al., 1995]. Pancreatitis may be also observed in HIV-positive patients in whom anti-retroviral therapy had induced lactacidosis [Sheng et al., 2004].