ABSTRACT
Background: Haemophilia A and B are the most common severe bleeding disorders and are inherited as X linked recessive pattern. The main clinical manifestations include bleeding into musculoskeletal sites or soft tissues mainly causing joint impairment and thus resulting in various morbidities. Prophylaxis therapy and inhibitor management have contributed a lot to the management of haemophilia cases. However in resource poor setting countries, like India, availability of factors, prophylactic therapy is farfetched leading to joint abnormalities, decreased physical activity and thus leading to different nutritional states. Studies from developed countries reveal obesity and overweight instances in children with Hemophilia. However not many studies have been undertaken to evaluate the nutritional status of such children in India.Methods: This study was conducted in the Comprehensive hemophilia Care Centre, Victoria Hospital, attached to Bangalore Medical College. A total of 50 children were included in the study. Children aged between 4 and 18 years attending the hemophilia Clinic were included in the study. Observations and review of relevant documents were done.Results: Among 50 children of haemophilia, 18(36%) children were aged less than 10 years and 32(64%) children were aged more than 10 years. The mean age of onset of disease in haemophilia A was 27.5 months (SD of 24.84; range 6-120) and in haemophilia B was 8 months (SD of 2.72; range 3-12). The mean BMI among children aged more than 10 years was more (21.35; SD= 4.02) compared to the children less than 10 years (16.87; SD= 3.41).Conclusions: The prevalence of overweight and obesity among children with Haemophilia is more in adolescent age group compared to children in the first decade.
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Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation and the presence of circulating autoantibodies directed against self-antigens. The reported prevalence of SLE in children and adolescents (1-6/100,000) is lower than that in adults (20-70/100,000). The study among pediatric population' were few and hence this study from south India was undertaken. Objective of this study was to study the clinical and immunological profile of children with systemic lupus erythematosus (SLE). To study the Distribution of Renal Lesions according to ISN/RPS Classification of Lupus Nephritis'Methods: Retrospective hospital based observational study in tertiary level centre in Bengaluru (BMCRI). Medical records of children with SLE admitted in Pediatric department from the period of 2010-2019 through the hospital information system were analyzed. Clinico pathological features and immunological profile were compared with other studies.Results: Among 25 patients studied male to female ratio was1:2.5. The mean patient's age at the time of presentation was13.2 year, the youngest child being 7 year. The mean duration of disease before diagnosis was 1year, most common systems involved were haematological (92%), followed by kidney (88%), GIT (72%), mucocutaneous (68%) cases. 19 (91%) cases were ANA positive and two ANA negatives. Anti-ds DNA was positive in 18(85%) patients, 5 were anti smith antibody positive. Diffuse proliferative glomerulonephritis (ISN/RPS class IV) was the most commonly seen histological pattern, seen in 9(56%) patients, 4(25%) patient had focal and segmental proliferative glomerulonephritis (ISN/RPS class III) and 2(12%) had membranous glomerulonephritis (grade ISN/RPS class V).Conclusions: SLE can present with diverse, unpredictable clinical manifestations, the primary diagnosis can often be missed if the index of suspicion is not high, since childhood SLE does not present with classical manifestations.
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Background: Perinatal asphyxia refers to a condition during first and second stage of labour in which impaired gas exchange leads to foetal hypoxemia. Perinatal asphyxia causes cardiac dysfunction in 24 to 60 percent of the cases. The reduced cardiovascular reserve is associated with hypoxic brain damage and has high impact on neonatal mortality and adverse neurological outcomes. It has been challenging to diagnose myocardial dysfunction in resource constraint setting. Aim and objective of this study was to Determine N-Terminal Pro BNP concentrations in perinatal asphyxia and correlate with modified Sarnat stages of hypoxic ischemic encephalopathy.Methods: Among 120 Neonates admitted in neonatal intensive care unit with diagnosis of perinatal asphyxia were considered for the study. 2mL of venous blood drawn within 48hours of life was analyzed for quantitative N-Terminal Pro BNP and was correlated with modified Sarnat stages of hypoxic ischemic encephalopathy.Results: A Total of 120 cases of perinatal asphyxia were considered for the study, among which 44 cases had HIE stage 1, 48 had HIE stage 2 and rest 28 had HIE stage 3. The mean and standard deviation of N-Terminal Pro BNP concentrations in stage 1 was 1,502.86'3,581.170 pg/mL, stage 2 was 4,916.31'8,001.674 pg/mL and stage 3 was 8,912.41'13,927.152 pg/mL with significant p value of 0.003.Conclusions: Early N-Terminal Pro BNP concentrations may provide a useful marker for the anticipated severity of myocardial dysfunction.
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Background: Severe acute malnutrition is the important health issue children affected in India. It is often associated with Iron, Vitamin B12 and Folic acid and other micronutrient deficiencies. These reports of declining trend of Iron and folic acid deficiency with implementation of national anemia control programme. Estimation of Serum Folate and Vitamin B12 levels in SAM children aged 6-60 months with anemia was the objective of the study.Methods: A hospital based observational study on 80 children admitted to nutritional rehabilitation center were suffering from severe acute malnutrition (SAM) in the age group of 6-60 months enrolled in the study during the period November 2018 to May 2019 .Data was collected using a predesigned and pretested Proforma, containing details of feeding, socio-economic and development history. Blood samples were sent for measurement of plasma vitamin B12 and serum folate levels. Statistical analysis was done using SPSS version 20.0.Results: Out of 80 children, majority were between the age group of 24-60 months and majority were boys (53.8%). Mean Vitamin B12 and folic acid levels being 384.61 and 8.95 respectively. 45% and 3.8% had vitamin B12 and folic acid deficiencies respectively. Majority of neurological changes and developmental delays were noted in B12 deficient group. Of the 36, B12 deficient children, majority had moderate anaemia (40.2%), normal total count (41.7%), 60% had neutropenia, 44.4% had lymphocytosis, majority had normocytic (30.9%) anemia.Conclusions: There was a moderate to high prevalence of vitamin B12 deficiency among malnourished children. Folate deficiency was found only in few. Efforts should be directed to prevent its deficiency in pregnant and breastfeeding women and their infants with special attention on malnourished children.B12 and folate deficiency shall be considered in all cases with SAM irrespective of blood indices. Treatment will have impact on prognosis of child.
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Hypoplastic internal carotid artery has been reported in about 100 patients, most being incidentally diagnosed. Association with other anomalies rarely described. Authors report 6-year-old male presenting failure to gain weight and precordial bulge from past 6 months, past history was significant-diagnosed to have right dysplastic kidney at 8 months age. Detailed investigations revealed left hypoplastic internal carotid artery, vertebral segment anamoly, right dysplastic kidney due to right renal artery stenosis. Child was treated medically and was clinically better at latest follow up. All cases with dysplastic kidney need to be searched for vertebral and carotid anomalies, left ventricular dysfunction.
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Background: SAM children have increased requirements for phosphorus during recovery. If requirements are not met, they may develop refeeding hypophosphatemia leading to increased morbidity and mortality. However, no much studies known about the effect of current therapeutic diets (F-75 and F-100) on serum phosphate in SAM children.Methods: Prospective observational study, in which measuring serum phosphate at admission, at end of stabilization phase and at discharge in SAM children between 6-59 months.Results: Among 35 children enrolled, mean serum phosphate was 4.3 '0.6 mg/dl at admission, 4.1' 0.8mg/dl at end of stabilization phase and 4.4'0.7mg/dl at discharge. 17% of children had hypophosphatemia at admission, 31% at end of stabilization phase and 17% at discharge. mean weight gain in hypophosphatemia and normophosphatemia groups are 1.3'1.46mg/kg/day and 2.51'2.63mg/kg/day (p=0.1) respectively. Mean duration of stay in hypophosphatemia and normophosphatemia groups are 11.6'1.26 and 10.26'1.54 days respectively (p=0.016).Conclusions: Hypophosphatemia was common among children with SAM at admission and increased at end of stabilization phase. Serum phosphate remains subnormal in about 1/5th of the children at discharge. This could be problematic for further recovery as phosphorus is needed for catch-up growth and bioavailability of phosphorus is low in local diets. Hence, authors suggest phosphorus supplementation in SAM children.