Primary antiphospholipid antibody syndrome presenting with adrenal insufficiency in a child: case report and review of literature.

A 7-year-old boy presented with adrenal insufficiency. He subsequently developed venous thrombosis in the limbs and was diagnosed with primary antiphospholipid syndrome (PAPS) based on clinical and laboratory parameters. Both adrenals were normal on imaging. He required thrombolysis and anticoagulation. The progressive course of PAPS was controlled with methylprednisolone. There are few reports of PAPS in pediatric patients, and associated adrenal involvement is rare. The unusual presentation, course and management of the patient and of four other reported children with adrenal insufficiency heralding manifestation of PAPS are discussed.

Delayed puberty: experience of a tertiary care centre in India.

BACKGROUND: Pubertal delay can be a manifestation of a wide variety of diseases, the proportions of which may vary between developing and industrialised countries. OBJECTIVE: A retrospective study was undertaken to investigate the aetiology of delayed puberty in northern India. SUBJECTS AND METHODS: Follow-up records of patients with delayed puberty presenting to the endocrine clinic between 2003 and 2007 were analysed. RESULTS: Forty-two patients (19 boys, 23 girls, age range 14-27 y) of 46 who initially presented had complete evaluation. The main causes of pubertal delay were chronic systemic illnesses (16), e.g. malnutrition, anaemia and chronic infections, hormone deficiencies (11), hypergonadotrophic hypogonadism (7) and constitutional delay (6). While the majority of girls (11/23) were found to have underlying systemic disorders, endocrinopathies (6/19) were the major causes of pubertal delay in boys. CONCLUSION: Chronic systemic illnesses are the major cause of pubertal delay in developing countries. Social awareness and education leading to early detection and treatment can prevent pubertal delay in a large proportion of cases.