Osteomalacic Myopathy in Children and Adolescents with Vitamin-D Deficiency.

BACKGROUND: Osteomalacic myopathy secondary to vitamin-D deficiency is an under-recognized cause of muscle weakness in children and adolescents. AIM: To describe a cohort of children and adolescents with osteomalacic myopathy. SETTINGS AND DESIGN: Pediatric neurology unit of a tertiary care hospital. METHODS AND MATERIAL: Charts of children and adolescents with osteomalacic myopathy were retrospectively reviewed for demographics, clinical presentation, laboratory investigations, and treatment response. Diagnosis of vitamin-D deficiency was made on the basis of a combination of clinical, biochemical, and radiographic findings. Response to treatment with vitamin-D confirmed vitamin-D deficiency as the cause of myopathic symptoms. RESULTS: Twenty-six children-15 girls and 11 boys aged between 20 months and 19 years-with osteomalacic myopathy were identified. Fifteen (58%) children were between 10 years and 19 years of age. Twenty-one (81%) children presented with myopathic symptoms of progressive walking difficulty, with eventual loss of ambulation in six. Four children came to attention through hypocalcemic seizures. One nonambulatory child with cerebral palsy presented with loss of previously attained ability to roll over and sit. All children had proximal muscle weakness on examination. Fifteen (58%) children had clinical signs of rickets. All the children who underwent biochemical (n = 24) and radiographic (n = 16) investigations had results consistent with vitamin-D deficiency. Only in one child, the diagnosis of osteomalacic myopathy was made on the basis of clinical findings. Response to vitamin D was uniformly good. CONCLUSIONS: Vitamin-D deficiency should be considered in the differential diagnosis of proximal myopathy in children and adolescents.

Predictors of Successful Kasai Portoenterostomy and Survival with Native Liver at 2 Years in Infants with Biliary Atresia.

OBJECTIVES: Kasai portoenterostomy (KPE) is the primary treatment for biliary atresia (BA) with subsequent liver transplantation in failed cases. The aim of this work was to study the outcome of KPE in children with BA and identify the factors predicting a successful KPE. METHODS: Children diagnosed with BA and undergoing KPE between January 2010 and January 2018 were included in the study. A successful KPE was defined as decrease in bilirubin to less than 2 mg/dL at 6 months after KPE. Factors affecting the outcome (successful KPE and survival with native liver [SNL] at 2 years) were evaluated by logistic regression. RESULTS: A total of 79 children with post-KPE BA were included. Successful KPE was achieved in 29 (36.7%) of 79 children undergoing KPE. The data for survival with native liver at 2 years were available for 61 children as 9 were lost to follow up before 2 years and another 9 were aged less than 2 years at the time of analysis. Twenty-seven (44.3%) of these 61 survived with their native liver at 2 years. On logistic regression analysis, lower age at KPE, use of postoperative steroids and absence of cholangitis were significant predictors of a successful KPE. A successful KPE at 6 months was the lone independent predictor of SNL at 2 years in these children. CONCLUSION: Early age at KPE, use of postoperative steroid and prevention of cholangitis can result in successful KPE. Those with successful KPE are likely to survive with their native liver at 2 years.

Outcome of Children with Severe Acute Malnutrition and Diarrhea: a Cohort Study.

PURPOSE: Severe acute malnutrition (SAM) is an important public health problem which contributes to significant number of under five deaths. Protocol based management significantly decreases risk of deaths in children with medical complications. METHODS: Outcome of children aged 2 months-5 years admitted and fulfilling definition of SAM having diarrhea (group A) was compared to children with SAM having medical complications other than diarrhea (group B). Both groups were managed according to standard recommended protocols and monitored and followed up for 12 weeks after discharge. RESULTS: The average weight gain, defaulter rate, primary failure, secondary relapse rate and readmission rate were similar in both groups. Length of stay in group A was three days longer (p-value=0.039). Discharge rate was comparable with overall 68% of children successfully discharged and 50% of children reaching weight/height >-2 standard deviation at follow-up of 12 weeks. CONCLUSION: The current management protocol is equally effective for managing children with SAM having diarrhea. Good adherence to management protocol of dehydration and timely modification of therapeutic feeds in children with persistent diarrhea results in satisfactory weight gain.

Osteopetrosis: presenting as maxillary osteomyelitis.

Osteopetrosis is a rare congenital disease presenting with recurrent fractures, hematopoietic insufficiency and hepatosplenomegaly. Though osteomyelitis is a known complication in osteopetrosis, osteopetrosis presenting as osteomyelitis is rare. Management consists of multidisciplinary approach for complications and bone marrow transplant for the infantile form of disease.

Prevalence of abnormal lactose breath hydrogen tests in children with functional abdominal pain.

OBJECTIVES: The study was undertaken to determine the prevalence of abnormal lactose breath hydrogen test in children with non-organic chronic abdominal pain. METHODS: Children with chronic abdominal pain were examined and investigated for organic causes. All children without a known organic cause underwent lactose and glucose breath hydrogen test. After a standard dose of 2 g/kg of lactose to a maximum of 50 g, hydrogen in breath was measured at 15 min intervals for 3 h. A rise of 20 ppm above baseline was considered suggestive of lactose malabsorption. RESULTS: Of 108 children screened, organic causes were found in 46 children. Sixty-two patients without any organic cause underwent hydrogen breath test. Lactose hydrogen breath test (HBT) was positive in 36 of 62 (58%), while 11 (17%) had positive HBT with glucose suggestive of small intestinal bacterial overgrowth (SIBO). Twenty out of 34 (59%) improved on lactose free diet while 8 out of 11 (72%) children of SIBO improved on antibiotics. CONCLUSIONS: Lactose malabsorption was seen in 58% of children with non-organic chronic abdominal pain.

Spatial distribution of ectoparasites on the gills of the mullet, Liza macrolepis: the effects of pollution.

Spatial distribution of ectoparasites on the gills of the mullet Liza macrolepis collected from two sites, the unpolluted reference site (Site I) and the heavily polluted North western arm of Visakhapatnam harbour (Site II), was analysed. Of the various ectoparasite species found on the gills of the mullet, only Ancyrocephalus vanbenedeni (Monogenea) and Dermergasilus varicoleus (Copepoda), occurred commonly at both the sites with more or less high prevalence and mean intensity. A. vanbenedeni at site I preferred gill arch 1 and posterior most and central sections, whereas at site II, it showed preference for gill arch 3, and posterior and proximal sections of the gill. D. varicoleus is highly specific in site selection. It preferred gill arch 1 and the anterior and distal parts at both the sites. Ergasilus rostralis favoured gill arch 3 and anterior and proximal sectors, a safe place providing absolute protection from the environmental pollution. It is concluded that the distribution of various ectoparasites on the gills of L. macrolepis is determined by the force of ventilation water current, the behavioral response of the parasite to the stress induced by pollution and the surface area of gill arches.

Enteric Fever.

Enteric fever is an important public-health problem in India. The clinical presentation of typhoid fever is very variable, ranging from fever with little other morbidities to marked toxemia and associated multisystem complications. Fever is present in majority of patients (>90 %) irrespective of their age group. Mortality is higher in younger children. Blood culture remains gold standard for diagnosis. Widal test has low sensitivity and specificity but may be used in second week to support the diagnosis. Emerging resistance to several antibiotics should be kept in mind when selecting antibiotics or revising the treatment. The key preventive strategies are safe water, safe food, personal hygiene, and appropriate sanitation. Vaccination is an additional effective tool for prevention.

Safety and Efficacy of Low-osmolarity ORS vs. Modified Rehydration Solution for Malnourished Children for Treatment of Children with Severe Acute Malnutrition and Diarrhea: A Randomized Controlled Trial.

World Health Organization-recommended rehydration solution for malnourished children (ReSoMal) for rehydrating severe acute malnourished children is not available in India. In present study, 110 consecutive children aged 6-59 months with severely acute malnourishment and acute diarrhea were randomized to low-osmolarity oral rehydration solution (ORS) (osmolarity: 245, sodium: 75) with added potassium (20 mmol/l) or modified ReSoMal (osmolarity: 300, sodium: 45). In all, 15.4% of modified ReSoMal group developed hyponatremia as compared with 1.9% in low-osmolarity ORS, but none developed severe hyponatremia or hypernatremia. Both groups had equal number of successful rehydration (52 each). Both types of ORS were effective in correcting hypokalemia and dehydration, but rehydration was achieved in shorter duration with modified ReSoMal.