Membranoproliferative glomerulonephritis in a child with congenital portosystemic shunt.

Congenital portosystemic shunts (CPSS) are rare congenital vascular anomalies characterized by abnormal connections between the portal vein and systemic circulation, bypassing the liver. They can lead to complications such as recurrent encephalopathy, liver nodules, portopulmonary hypertension, and neurocognitive issues due to hyperammonemia and rarely kidney involvement. Hepatic hemodynamic changes can lead to liver nodules and hepatocellular carcinoma, particularly in extrahepatic shunts. We describe here an 11-year-old girl with type 1 intrahepatic portosystemic shunt with focal nodular hyperplasia in the liver, presenting with nephrotic syndrome that was diagnosed as membranoproliferative glomerulonephritis on kidney biopsy and that responded partially to therapy with immunosuppressants.

Clinical and antimicrobial susceptibility profile of typhoid fever in children in the era of antibiotic resistance.

Enteric fever remains a significant public health problem in low- and middle-income countries with further challenges from emerging antimicrobial resistance. Our prospective study evaluated the current clinical and antimicrobial susceptibility profile of enteric fever in 88 children and compared it to previously established literature. Enteric fever usually presents with nonspecific signs and symptoms, with predominant respiratory complaints. A paradigm shift in the antimicrobial sensitivity pattern has been noted, with increasing resistance for first-line antibiotics and older antibiotics such as ampicillin, cotrimoxazole, and chloramphenicol showing good sensitivity. Thus, the introduction of the latter merits consideration.

Quality of life and disease perceptions in caregivers of children with Congenital Adrenal Hyperplasia.

Background: Congenital adrenal hyperplasia (CAH) is a chronic disorder requiring lifelong therapy. False perceptions, poor knowledge, and social isolation adversely affect the psychosocial health and quality of life of patients and caregivers. The study was undertaken to ascertain the quality of life of caregivers of children with CAH. Method: A hospital-based cross-sectional study enrolled caregivers of children diagnosed with CAH where newborn screening was unavailable. Quality of life (QoL) was measured using the WHO-QoL-BREF questionnaire to compare physical, psychological health, social relationships, and environment. Results: Eighteen children with CAH (9 males), 14 salt-wasting, and 4 simple virilizing CAH with median (IQR) age of 4.3 (2.6, 9.8) years and a follow-up period of 3.8 (1.3, 8.4) years were enrolled. Out of 18 caregivers, 6 were fathers and 12 mothers with a mean (SD) age of 38 (8) years. The mean (SD) of the total score was 69.2 (10.6); physical, psychological, environmental, and social domain were 69.8 (15.1), 57 (12.6), 64 (14.5), and 65 (9.9), respectively. Physical, psychological, and environmental domain scores were significantly higher in upper-middle than lower-middle socioeconomic strata (P < 0.05) and similar between salt-wasting and simple virilizing phenotypes. There was no significant correlation between QoL scores and duration of disease (r = 0.257, P = 0.44). Parents of 7 (38%) patients discussed disease with extended families or neighbors, but none of the school teachers were informed. Death (56%), social acceptance (27%), and infertility (18%) were the major concerns acknowledged. Conclusion: The study reflects the suboptimal quality of life in caregivers of children with CAH. There was no correlation of the duration of disease with the QoL scores. Psychological and social relationship scores were poorest, indicating the need to creating more awareness to remove social taboos associated with this disease.

Daily v. weekly oral vitamin D3 therapy for nutritional rickets in Indian children: a randomised controlled open-label trial.

The aim of the study was to compare the efficacy of daily v. weekly oral vitamin D3 therapy in radiological healing of nutritional rickets. Children 6 months to 12 years (n 132) diagnosed with nutritional rickets were randomised into three groups (n 44): group A - 2000 IU daily vitamin D3 for 12 weeks, B - 60 000 IU weekly for 3 weeks, C - 60 000 IU weekly for 6 weeks. Serum calcium, phosphorus, 25-hydroxyvitamin D (25(OH)D), parathyroid hormone and X-ray score were estimated at baseline and 12 weeks (endline). The proportion of children who achieved complete radiological healing at endline was compared between three groups by χ2 and delta change in laboratory parameters by ANOVA (parametric data) or Kruskal Wallis test (non-parametric data), respectively. Baseline 25(OH)D ≤ 20 ng/ml was seen in 119 (90·2 %), hyperparathyroidism in 90 (68·8 %) and hypocalcaemia in 96 (72·7 %). A total of 120/132 children completed the study. Complete radiological healing seen in 30 (75 %) in group A, 23 (60·5 %) in group B and 26 (61·9 %) in group C; P = 0·15, with comparable endline X-ray scores; P = 0·31. The median (interquartile range (IQR)) delta X-ray score (baseline-endline) was 7 (4,9), 5 (2·25, 6) and 6 (4,7) in groups A, B and C, respectively; P = 0·019. Median (IQR) 25(OH)D endline levels in groups A, B and C were 50·0 (26·5, 66·5), 42·1 (28·4, 54·4) and 53·5 (33·7, 71·2) ng/ml, respectively; P = 0·045. Radiological scores were comparable at endline among daily and weekly vitamin D groups with greater change from baseline in daily supplemented group.

Ionized Blood Magnesium in Sick Children: An Overlooked Electrolyte.

INTRODUCTION: Magnesium is a less frequently monitored electrolyte in critically ill patients. Hypomagnesemia is associated with increased need for mechanical ventilation, mortality and prolonged ICU stay. The present study was undertaken to identify the proportion of children with abnormal magnesium levels and correlate it with disease outcome. METHODS: This observational study included children aged 1 month to 12 years hospitalized at the emergency room. Heparinized blood was collected for determination of ionized magnesium, ionized calcium, sodium, potassium and lactate using Stat Profile Prime Plus (Nova Biomedical, Waltham, MA, USA). Clinical outcomes for duration of hospitalization, and death or discharge were recorded. RESULTS: A total of 154 (102 males) children with median (IQR) age of 11 (4, 49.75) months were enrolled. Sixty one (39.6%) had ionized magnesium levels below 0.42 mmol/l, 63 (40.9%) had normal levels and 30 (19.4%) had hypermagnesemia (>0.59 mmol/l). Hypomagnesemia was associated with hypocalcemia (p < 0.001), hyponatremia (p < 0.001) and hypokalemia (p < 0.02). A higher proportion of children with hypermagnesemia required ventilation than hypomagnesemia (26% vs. 9%) and succumbed (35% vs. 20%), respectively; p > 0.05. Ninety-three (60.3%) had hypocalcemia and 10 (6.5%) children had hypercalcemia. There was good correlation between ionized calcium and magnesium values (r = 0.72, p < 0.001). CONCLUSION: Both hypomagnesemia and hypermagnesemia were seen in critically ill children. Patients with hypomagnesemia had significantly higher proportion of other electrolyte abnormalities.

Point of Care Testing of Serum Electrolytes and Lactate in Sick Children.

OBJECTIVE: To evaluate the electrolyte and lactate abnormalities in hospitalized children using a point of care testing (POCT) device and assess the agreement on the electrolyte abnormalities between POCT and central laboratory analyzer with venous blood. METHODS: This observational study recruited hospitalized children aged 1 month to 12 years within two hours of admission. A paired venous sample and heparinized blood sample were drawn and analyzed by the central laboratory and POCT device (Stat Profile Prime Plus-Nova Biomedical, Waltham, MA, USA) for sodium and potassium. Lactate was measured on the POCT device only. The clinical and outcome parameters of children with electrolyte abnormalities or elevated lactate (>2mmol/L), and the agreement between POCT values and central laboratory values were assessed. RESULTS: A total of 158 children with median (IQR) age 11 (6-10) months and PRISM score 5 (2-9) were enrolled. The proportion of children with abnormal sodium and potassium levels, and acidosis on POCT were 87 (55.1%), 47 (29.7%) and 73 (46.2%), respectively. The interclass coefficient between POCT and laboratory values of sodium and potassium values was 0.74 and 0.71 respectively; P<0.001. Children with hyperlactatemia (81, 51.3%) had higher odds of shock (OR 4.58, 95% CI: 1.6-12.9), mechanical ventilation (OR 2.7, 95% CI 1.1-6.6, P=0.02) and death (OR 3.1, 95% CI 1.3-7.5 P=0.01) compared to those with normal lactate. CONCLUSION: POCT can be used as an adjunct for rapid assessment of biochemical parameters in sick children. Lactate measured by POCT was a good prognostic indicator.

Sleep Problems and Their Correlates in Children with Autism Spectrum Disorder: An Indian Study.

Sleep problems were studied in both typically developing (TD) children and those with autism spectrum disorder (ASD) using the Sleep Disturbance Scale for Children. Factors associated with these problems were also studied in children with ASD. Seventy-three children with ASD and their age and sex matched TD controls in age group of 3-12 years were enrolled in the study. Higher sleep problems were found in children with ASD than TD children. Most common sleep problem reported in children with ASD was Sleep Wake Transition Disorders, followed by Disorder of Initiation and Maintenance; while in TD controls, it was Sleep Breathing Disorders. Apart from severity of Autism; hyperactivity, sensory issues and poor motor skills were significantly associated with sleep problems, which may be important targets for intervention in children with sleep problems.