Uncharted surgical therapy for acquired perforating dermatoses.

Acquired perforating dermatoses represent an uncommon group of chronic papulonodular dermatoses of unknown aetiology, characterized clinically by intense itching; and histopathologically by transepidermal elimination of dermal components. Definitive targeted pharmacological approaches are lacking, and the disease is usually refractory to therapy. We report here a case of rapid remission of acquired perforating dermatosis following simple thymectomy. If a role for the thymus in the pathogenesis of acquired perforating dermatoses was to be established, this could pave the way for a yet uncharted surgical therapy for these debilitating conditions.

Syrian national growth references for children and adolescents aged 2-20 years.

BACKGROUND: During the past three decades, growth charts have become one of the principal tools for monitoring anthropometric development in individuals and populations as well. Growth references by the CDC and other countries have been widely used in our hospitals and healthcare units for clinical assessment of children's development. The apparent overestimation and underestimation of many children's anthropometrics indicated the need to construct our own references. The objective of this study is to establish the national growth references for the Syrian population 2-20-year-old. METHODS: A multicenter cross-sectional sample of 13,548 subjects, aged 2-20 years, were recruited from various kindergartens, schools, and universities across the Syrian Arab Republic between February and May-2019. Response variables (stature, weight, and BMI) were fitted against age using P-splines and three empirical distributions: Box-Cox T, Box-Cox Power Exponential, and Box-Cox Cole and Green. Residuals diagnostic Q-tests and worm plots were used to check the validity of fitted models. RESULTS: Box-Cox T provided the best fit for stature-for-age, whereas Box-Cox Power Exponential provided the best fit for weight-for-age and BMI-for-age. Residuals diagnostics revealed adequate models fitting. BMI cutoffs revealed an increased prevalence of obesity (4.5% and 3.66%) and overweight (20.1% and 19.54%), for boys and girls respectively, in our population. CONCLUSIONS: Growth charts are available for use now in our hospitals and healthcare units. For 0-2-year-old children, we recommend using the World Health Organization's standards.

Congenital erythropoietic porphyria (Gunther disease): a case report.

Congenital erythropoietic porphyria (CEP or Gunther disease) is a very rare subtype of porphyria with a prevalence of <0.9 per 1 000 000. A 13-year-old female patient came to our hospital complaining of a severe cutaneous ulceration and scarring. The symptoms began in her first year of life as urine discoloration and skin blistering in sun-exposed areas. The family had been trapped in a high-risk conflict zone in Syria for many years, which precipitated the aggravation of symptoms. Based on clinical examination and laboratory tests, we diagnosed the patient with CEP and treated her with vitamin D supplementation alongside chronic blood transfusions, strict photoprotection and psychotherapy. After 7 months, there were no longer active ulcers or novel complications. Psychotherapy and patient education were important for her psychological development at this age. This treatment limited the deterioration of the symptoms and made the patient more committed to the periodic examinations.