Introduction: Worldwide, sickle cell disease (SCD) is the most common hemoglobinopathy among which SS pattern is more common. Although hydroxyurea (HU) is approved by the Food and Drug Administration for the treatment of recurrent moderate-to-severe painful crises in pediatric sickle cell anemia, there is a fear of toxicities. Objectives: The objectives of the study were to evaluate the short-term safety and beneficial effects of low-dose HU therapy in SCD (SS pattern) children. Materials and Methods: This prospective cohort study enrolled 40 cases of severe SCD and started HU in a fixed dose of 10 mg/kg/day. During follow-up, cases were evaluated for compliance of HU, its toxic effects and adverse events from their histories, clinical examinations, and laboratory parameters. Furthermore, beneficial effects of HU therapy were evaluated by assessing blood transfusion rate, frequency of painful events, strokes, acute chest syndrome, avascular necrosis of femur, and estimation of hemoglobin F (HbF) level after 2 years of therapy. HU was discontinued temporarily if any toxicity or minor adverse drug events occurred during therapy and was restarted at the same dose after normalization of deranged laboratory parameters. Results: The clinical adverse drug events seen were nausea (8.33%), diarrhea (2.78%), and hematuria (2.78%). The most common hematological toxicity was anemia and thrombocytopenia. Renal and hepatic toxicities were transient in nature. The mean acute painful events and blood transfusion rate reduced significantly on HU therapy. It increased Hb and HbF level significantly in SCD children. Conclusion: HU is a safe drug without significant toxicity or adverse events in a dose of 10 mg/kg/day for short duration and it is beneficial in SCD (SS pattern) children in reducing acute painful events and decrease blood transfusion rate.

Background: Snake bite is generally considered to be a rural problem and has been linked with environmental and occupational condition is a neglected public health problem. This study was conducted to ascertain clinical profile, complication and outcome of snake bite in pediatric population. Methods:Retrospective observational study was done in paediatrics wards and Paediatric Intensive care unit of tertiary care institute. A medical record files of 80 patients of snakebite, between the periods of January 2011 to February 2016 were recovered from Medical Record and Statistics Section of Institute. Files were analyzed for demographic data; variables included site of bite, time of bite, type of primary treatment and treatment provider, and type of snake poison, common symptoms suggestive of hematotoxicity, myotoxicity and neurotoxicity.Results: Out of total 80 patients, 58.75% were male and most of snake bites were seen in age group of 6-12 years. Clinically 57.5% patients were presented with signs and symptoms of envenomation and most of them were bitten during the months of June to September. All bites were nonprovocative and in 57.5% lower limb was the commonest site of bite. A 77.5% patient were primarily treated by medical personnel, 56.25% patients were received treatment within 1 hour of bite and hospitalized within 6 hours of bite. Coagulation failure was commonest complication (58.33%) followed by hypotension (25%) in vasculotoxic snake bite and respiratory paralysis (68.18%) in neuroparalytic bites. Renal replacement therapy was required in 5%, blood/blood product transfusion in 11% patients and case fatality rate was 11.25%.Conclusions: The most vulnerable to snake bites are boys aged more than 5 years. There is an urgent need to spread awareness among the community for avoidance of traditional treatment and any delay in medical intervention in snakebite incidents.

Objective: Recognizing the clinical presentation of Guillain-Barre syndrome in children. Design: Retrospective observational study. Setting: Pediatric wards and Paediatric Intensive Care Unit. Method: A medical record files of 52 patients of GBS, between the periods of March 2011 to February 2016 were recovered from Medical Record and Statistics Section of institute. Files were analyzed for demographic data, clinical features, co-morbid conditions, investigations, electrophysiological data, mode and result of treatment and data was analyzed. Result: In our series males are outnumber to female and most of the patients in age group 6-12 years, 48% were belongs to lower socioeconomic status. In 53.8% patients antecedents were present at the beginning of clinical picture and upper respiratory tract infection (28.84%) & diarrohea (19.23%) was noted with predominant occurrence in monsoon season (52%). Bilateral lower limb weakness was noted in all patients and in 61.9% patients were common complaint pain in lower limb. Cranial nerve involvement was evident in 28.85% and bulbar involvement was commonest followed by facial nerve. A 23.07% patients were presented with respiratory involvement, 9 of them (15.38%) were needed assisted ventilation. Dysautonomic sign/symptoms were present in 26.92% with 73.33% had demyelinating pattern of neuropathy and 9.6% mortality. Conclusion: The clinical characteristics of GBS in children are not consistent and differ from geographical area and racial diversity.

The objective of present study is to recognizing risk factors those are associated with poor outcome in children with severe malaria. A Prospective observational study was set in Pediatric Intensive Care Unit of tertiary care hospital. A 170 cases of severe malaria between the age group of 6 months to 12 years of either sex with confirmed slide positivity for malaria parasite who satisfied WHO 2000 criteria for severe malaria were enrolled, after approval from institutional ethical committee and parent's informed consent. Detail clinical evaluation was done including assessment of various risk factors like respiratory distress syndrome, multiorgan dysfunction, hypoglycemia, jaundice, renal failure, impaired conscious, cerebral malaria, spontaneous bleeding, hyperparasitemia, shock and severe anaemia. Case fatality ratio was 19.4% and maximum in the age group between 6 months to 2 years. A MODS was most significant variable associated with poor outcome followed by shock, coma and bleeding tendency. MODS, shock, cerebral malaria and bleeding tendency are significant variable predicting poor outcome with severe malaria.

Objective: The objective of this study is to evaluate the presence and severity of cardiac involvement in asphyxiated neonates and to established which clinical and laboratory parameters (electrocardiogram [ECG], echocardiography [ECHO], and cardiac enzyme) are reliable predictors of myocardial ischemia. Design: Nested case–control study. Setting: Neonatology unit of tertiary care institute. Materials and Methods: The study group consist of 90 term neonates who had APGAR score <7 at 5 min as cases and 90 term neonate who had APGAR score >9 at 5 min as controls. On the 2nd day of life (between 24 and 36 h), all neonate underwentECG, ECHO, and enzyme estimation (CK-MB). Result: The cases were divided into three groups according to SARNAT scale andGroup I consist 25, Group II - 54, and Group - III, 11 neonates. The mean gestational age was 36.97±0.35 and 37.12±0.46 week for cases and controls respectively while mean birth weight was 2.79±0.36 kg and 2.68±0.76 kg for cases and controls, respectively. ECG changes were present in 70 (77.7%) of cases. ECG changes of Grade 3 and 4 were present in only Group III neonates and all neonates with Grade 4 changes were died in 1st week of life. Fractional shortening (FS) decreased progressively from Group I to Group III (p<0.001) and showed a significant difference between cases and controls as well as in different groups except in controls versus Group I. Peak aortic velocity behaved similarly. Concerning mean acceleration, the significant difference wasobserved between control and cases (all 3 groups). CK-MB activity increased from Group I to Group III and statistically significant difference was seen in controls and different Groups as well as in between different groups except in Controls versus Group I. Conclusion: Severe ECG changes (Grades 3 and 4), CK-MB elevation and reduced FS can be considered as reliable marker of myocardial ischemia in perinatal asphyxia.