Background: Acute lymphoblastic leukemia (ALL) has 5-year survival of more than 90% in many advanced cancer research institutes. However, advanced cancer care is not available to majority of poor in developing countries. The experience of treating such patients in a resource-scarce setting is described herewith. Of the 75 individuals studied, 11% of the children were stunted, >21% were underweight, and 16% of the under-five children had acute malnutrition. Massive hepatosplenomegaly and lymphadenopathy were present in 75% and 77% children, respectively. About 71% patients achieved complete remission (CR). A total of 30 (40%) children lived for >5 years after diagnosis and 21 (28%) of them had event-free 5 years. Weight for height for under-five children ( P = 0.029) and total count ( P = 0.019) were found to be significantly associated with deaths during induction. Weight for age ( P = 0.024), weight for height of under-five children ( P = 0.009), and lymphadenopathy ( P = 0.049) had a strong association with 5-year event-free survival. Using multivariate model, only weight for height among under five remained significantly associated with induction deaths ( P = 0.021) and absence of lymphadenopathy with event-free 5-year survival ( P = 0.042). Context: ALL has 5-year survival of >90% in many advanced cancer research institutes. However, advanced care is not available to majority of poor in the periphery of developing countries. Data available on the survival and the factors affecting the outcome among patients treated in poor resource settings are limited. Aims: This study aims to find the 5-year survival rate and the factors for risk-directed therapy in the region. Settings and Design: Cross-sectional analytical study at a tertiary center of public health in central Kerala. Subjects and Methods: Retrospective analysis of case sheets of 75 children who were treated at the institute from March 2006 to March 2011. Statistical Analysis Used: Univariate and Multivariate analysis using IBM SPSS Statistics for Windows, Version 20.0. Results: : Of the 75 individuals studied, 11% of the children were stunted, >21% were underweight, and 16% of the under-five children had acute malnutrition. Massive hepatosplenomegaly and lymphadenopathy were present in 75% and 77% children, respectively. About 71% patients achieved CR. A total of 30 (40%) children lived for >5 years after diagnosis and 21 (28%) of them had event-free 5 years. Weight for height for under-five children ( P = 0.029) and total count ( P = 0.019) were found to be significantly associated with deaths during induction. Weight for age ( P = 0.024), weight for height of under-five children ( P = 0.009), and ly...
Stiff-person syndrome (SPS) is an autoimmune neuronitis with progressive myoclonus and stiffness. It is a rare but treatable disorder with few case reports in children. SPS is due to autoantibodies against the enzyme glutamic acid decarboxylase which is present in neuronal and nonneuronal tissues. This is the case report of an 8-year-old boy with clinical and investigational features suggestive of SPS with associated myoglobin-induced renal failure, who completely recovered with treatment.
The aim of this study was to determine the clinical profile and etiology of diabetes mellitus (DM) with onset at < 6 months of age. All children aged < 6 months diagnosed with DM at a tertiary referral center between 2005 and 2008 were included in the study. Three cases of DM with onset at < 6 months of age were identified. All patients were female and of the same ethnic origin, with nonconsanguineous parents. Intrauterine growth retardation was noted in all three patients, and diabetic ketoacidosis and hypertriglyceridemia in two of the three. Blood samples from all three patients and their parents were analyzed for mutations in the KCNJ11 gene (inwardly-rectifying potassium channel, subfamily J, member 11 gene; OMIM 600937). A heterozygous de novo mutation in the KCNJ11 gene was detected in one patient, which confirmed the diagnosis of permanent neonatal DM. Neither C-peptide secretion nor circulating islet cell antibodies were detected in any patient during diagnosis, but C-peptide elevation was detected in the patient with permanent neonatal DM after treatment with sulfonylurea. One infant had clinical and immunological evidence of congenital cytomegalovirus infection while the diabetes in another case was postulated to be syndromic. DM within the first 6 months of life is a rare condition with various etiologies. The high prevalence of Kir6.2 mutations in neonatal diabetes means that all children < 6 months of age diagnosed with diabetes should be tested for Kir6.2 mutations at diagnosis.
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