{'fraction_non_alphanumeric': 0.025056947608200455, 'fraction_numerical': 0.0193621867881549, 'mean_word_length': 4.996587030716723, 'pattern_counts': {'":': 0, '<': 0, '<?xml version=': 0, '>': 0, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 0, 'zh_pornsignals.json': 0}}
G591(P) The Treatment of Camurati-Englemann Disease with Losartan

Objective Camurati-Engelmann disease (CED) is a rare, autosomal dominant bone dysplasia characterised by hyperostosis and sclerosis of the diaphyses of the long bones and skull. It typically presents in childhood with bone pain, myopathy and progressive immobility. It is caused by a number of mutations that increase activity of transforming growth factor β1 (TGF-β1). Evidence for treatment is based on a number of case reports, most of which describe the response to glucocorticoids. Losartan is known to reduce expression of TGF-β1 and there are reports of two children with CED who showed significant improvement in pain and mobility in response to this treatment. Here we report a child with CED treated with losartan. Methods A 10 year old child with a clinical and radiological diagnosis of CED (Figures 1 and 2) was commenced on losartan at a dose of 0.6 mg/kg daily. We assessed the response to treatment using the 6 min walk test, Functional Mobility Scale, Child Health Assessment Questionnaire (CHAQ), formal assessment of gait, biochemical markers of inflammation and bone turnover and radiological appearance including radiographs and densitometry. We observed for side effects, including specific monitoring for hypotension and electrolyte abnormalities.Abstract G591(P) Figure 1Abstract G591(P) Figure 2 Results Four weeks into treatment we observed improvement in his 6 min walking test from 357m to 366 m, and in the global evaluation portion of his CHAQ score from 1.5 to 0.9 on a scale of 0–3. Conclusions The early response to treatment with losartan in our patient has led to modest improvement in pain and mobility; further outcomes will be evaluated in due course.