Endocrinopathies in adolescents with thalassaemia major receiving oral iron chelation therapy.

BACKGROUND: Endocrinopathies are common in patients with thalassaemia major (TM) despite parenteral iron chelation therapy with deferoxamine. There are only a few studies on the efficacy of oral deferiprone in preventing endocrine dysfunction. AIM: To determine the growth and endocrine complications in children with TM receiving oral iron chelation with deferiprone. METHODS: All adolescents with TM receiving regular blood transfusion and deferiprone were evaluated prospectively for growth and pubertal status over a 1-year period. Tests for endocrine function included oral glucose tolerance test, calcium, phosphate, alkaline phosphatase, parathyroid hormone and thyroid profile and, in those with delayed/arrested puberty, sex steroids and gonadotropins. Clonidine-stimulated growth hormone (GH) was measured in patients with height ≤-3 SD. RESULTS: 89 patients [51 males, 38 females, mean (SD) age 13·6 (2·5) years] were evaluated. Mean (SD) pre-transfusion haemoglobin was 9·2 (1·1) g/dl and the mean (SD) age of starting deferiprone was 5·1 (2·4) years. Mean (SD) ferritin was 9159 (3312) pmol/L (normal <2247). 49 (55%) subjects were of short stature and 25 (27%) had a height Z-score ≤ -3. GH testing was performed in 19 patients, of whom 17 had peak GH values <10 µg/L. Delayed puberty and/or hypogonadism was present in 54·1% patients at or beyond the age of normal puberty. Impaired glucose tolerance/diabetes mellitus, hypoparathyroidism and primary hypothyroidism (subclinical) were present in 13·0%, 10·1% and 8·9%, respectively. Overall, 44 (49·4%) adolescents had at least one endocrinopathy. CONCLUSION: Adolescents with TM on oral iron chelation therapy with deferiprone experienced a high prevalence of growth faltering and endocrinopathies which was comparable to that previously reported with deferoxamine. A combination of deferoxamine and deferiprone may be necessary to prevent growth and endocrine problems.

Plasmodium vivax malaria admissions and risk of mortality in a tertiary-care children's hospital in North India.

BACKGROUND: In 2010, there was an increase in the severity of malaria admissions to Kalawati Saran Children's Hospital, New Delhi and this report describes the morbidity and mortality profile. METHOD: A retrospective chart review of patients admitted with parasitologically confirmed malaria between January and December 2010. RESULTS: There were 156 cases: P. vivax 105 (67.3%), P. falciparum 39 (25%) and mixed infections 12 (7.7%). Thrombocytopenia (platelet count <150×10(9)/L) was present in 90 (85.7%) patients with P. vivax mono-infection. There were 91 (58.3%) patients with severe malaria: P. vivax mono-infection 46 (50.5%), P. falciparum mono-infection 35 (38.5%) and mixed 10 (11%). Severe anaemia and severe thrombocytopenia (platelet count <20×10(9)/L) were detected significantly more often in P. falciparum and P. vivax mono-infection, respectively. Complications including cerebral malaria, acute renal failure, shock, acute respiratory distress syndrome (ARDS) and multiple-organ dysfunction syndrome (MODS) were similar in both groups. The mortality rate of around 20% was similar in severe P. vivax and P. falciparum mono-infection. Risk of mortality in vivax malaria was highest in patients with ARDS followed by MODS and shock. CONCLUSION: Increased morbidity owing to P. vivax malaria was observed and risk of mortality was highest in patients with ARDS and MODS.