Screening for protein energy wasting in children with chronic kidney disease using dual energy x-ray absorptiometry as an additional tool.

BACKGROUND: The current diagnosis of protein energy wasting (PEW) is based on scoring systems that lack precision in measuring muscle deficits. We undertook this cross-sectional study to determine the prevalence of PEW in children with chronic kidney disease (CKD) using a scoring system that included dual energy x-ray absorptiometry (DEXA) for measuring lean body mass (LBM) and to determine the prevalence of selected markers in PEW. METHODS: Thirty CKD and 20 healthy children (1-18 years) were evaluated for (1) reduced dietary protein intake (DPI); (2) BMI < fifth centile for height age (BMI/HA); (3) serum albumin < 3.8 g/dl, cholesterol < 100 mg/dl, or CRP > 3 mg/L; (4) LBM < fifth centile for height age [LBMr] on DEXA. PEW was scored as minimal-one parameter positive in 2/4 categories; standard-one parameter positive in 3/4 categories; or modified-standard plus height < 2 SD. RESULTS: Twenty children with CKD (66.7%) had PEW, (5/9) 55% in CKD 3, and (15/21) 71% in advanced CKD; minimal 12, standard 1, and modified 7. LBMr was seen in 20 (100%), reduced DPI in 16 (80%), and BMI/HA in 6 (30%) children with PEW. LBMr had 100% sensitivity and BMI/HA 100% specificity. LBMr was seen in 8 who had no other criteria for PEW. None of the parameters were positive in controls (p < 0.01). CONCLUSIONS: PEW prevalence in CKD was high. Both prevalence and severity were higher in advanced CKD. LBMr was a highly sensitive marker to detect PEW. LBMr seen in some children with CKD who were negative for other markers could represent subclinical PEW.

Prevalence and subtypes of hypertension in normal-weight and obese Indian adolescents: a cross-sectional study.

This cross-sectional study examined the prevalence, stages, subtypes of hypertension, and the associated risk factors in adolescent school children in Western India. We screened 2,644 adolescents, from 10 different private and government schools in urban and rural areas for hypertension, as defined by the 2017 Clinical Practice Guidelines. The association of stages and subtypes with age, gender, body mass index, type of school, and place of residence was analysed. 197 children (7.5%) had hypertension; 170 (6.4%) had stage I, 27 (1%) had stage II and 76 (2.9%) had elevated blood pressure (EBP). The risk of EBP was higher in children > 15 years of age (p = 0.006). Compared with normal-weight children, obese, and overweight children had a higher risk of hypertension [odds ratio (OR) 9 (5.84, 13.88) and 3.77 (2.59, 5.48) respectively], whereas underweight children had a lower risk [OR 0.39 (0.16, 0.98)]. Normal-weight hypertension was seen in 5.2% and was higher in children from government schools (9.4%). Systolic-diastolic hypertension (SDH) was the most common subtype, seen in 136 (5.1%). SDH was more common in girls, in rural children, and in those with stage II hypertension. Isolated diastolic hypertension, seen in 51 (1.9%), was more common in boys, in urban children, and in those with EBP.

Clinico-pathological Profile and Outcome of C-3 Glomerulopathy in Indian Children.

INTRODUCTION: There is paucity of data of C3 glomerulopathy in Indian children. METHODS: First Indian pediatric case series where consecutive renal biopsies done over a period of ten years were reviewed to identify those patients who had isolated or predominant C3 deposits on immunofluorescent microscopy, fulfilling the criteria for C-3 glomerulopathy. The clinical, biochemical, serological, histopathological profile, eGFR and the need for renal replacement therapy was analyzed. RESULTS: Eighteen patients, comprising 5.3% (18/298) of all renal biopsies, had C3 glomerulopathy, four with Dense Deposit Disease (DDD) and fourteen with C3 Glomerulonephritis (C3GN) with a median follow-up of 38.2 months. Median age of presentation was 7.45±3.03 years (2.5yrs- 13.5yrs) with nine boys and nine girls. Presentation was nephrotic syndrome in seven (39%), acute nephritic syndrome in three (16.7%), hematuria in five (27.7%) and acute kidney injury in three (16.7%). Median eGFR was 69 ml/min/1.73m2 (8.2-107 ml/min/1.73m2). Hematuria was seen in 16 (88%), proteinuria in 18 (100%) and low C3 in 16 (88%) at the time of presentation. Mesangioproliferative glomerulonephritis was the predominant pattern in DDD while C3GN showed a mix of mesangioproliferative, membranoproliferative, endocapillary and crescentic GN (p = 0.43).Complete or partial remission was seen in seven patients who received long term alternate day steroids alone or with added mycophenolate mofetil. The cumulative patient survival was 70.8%. Kaplan Meir analyses for renal survival without progression to ESRD was 60.2% at one year and 48.1% at five and ten years. CONCLUSION: Interstitial fibrosis and tubular atrophy on renal biopsy was an independent predictor of adverse renal outcome in the cohort (p = 0.013, HR8.1;95% CI -1.6-42).

Efficacy and safety of prolonged daily hemodialysis in critically ill children weighing less than 10 kg.

INTRODUCTION: Extracorporeal renal replacement therapy may be needed in those infants in whom peritoneal dialysis is not feasible or is ineffective. Prolonged daily hemodialysis (PDHD) remains the only available extracorporeal dialytic modality in children weighing less than 10 kg, in setups where continuous renal replacement therapy is unavailable and sustained low-efficiency dialysis is not feasible due to lack of dedicated HD machines capable of delivering very low dialysate flows. The use of PDHD in critically ill children weighing less than 10 kg has not received much attention. METHODS: Retrospective analysis of the efficacy and safety of PDHD in critically ill children weighing less than 10 kg. FINDINGS: Four critically ill children received a total of 49 sessions of PDHD for acute kidney injury (AKI) associated with sepsis and multiorgan dysfunction syndrome (MODS). PDHD was delivered for a duration of 6 to 8 hours daily with low ultrafiltration rates, low blood flow rates but with dialysate rates that were four times the blood flow rates, due to limitations of minimal dialysate flow rate on standard machines. The mean duration of the sessions was 6.59 ± 1.61 hours. Mean hourly ultrafiltrate (UF) rate during the sessions was 9.28 ± 2.57/kg/hour with mean patient fluid removal rate of 5.4 ± 2.56 mL/kg/hour. Predialysis serum creatinine decreased to 30% of the starting value by the fourth hemodialysis (HD) session. Thirty-three (67.4%) sessions were heparin free. Intradialytic hypotension occurred in 10 (20.4%) sessions. Premature termination of the session occurred in six (12.4%), due to hypotension in two and filter clotting in four. Hypokalemia was seen in 12 (24.4%) and hypophosphatemia in 6 (12.24%). All four patients survived. On follow-up, three are dialysis free and one is on maintenance hemodialysis. CONCLUSION: PDHD is effective and safe in critically ill small children.

Diabetic Ketoacidosis with Extreme Hypernatremia in a 4-Year-Old Girl.

A 4-year-old girl admitted with altered mental status, new-onset diabetes mellitus, and diabetic ketoacidosis (DKA) had a rapid rise in serum sodium from 158 mEq/L (corrected sodium 165 mEq/L) at the admission to 204 mEq/L within 18 hours of admission despite standard fluid and insulin therapy recommended for the treatment of DKA. During her illness, she developed arterial and deep vein thrombosis (DVT), bloodstream infection with Candida species, and extensive skin blistering and denudation. The child needed mechanical ventilation, insulin infusion, careful fluid titration to bring down the sodium gradually, and low-molecular weight heparin for her DVT. She had a prolonged Intensive Care Unit and hospital stay but recovered completely without any neurological sequelae.

Acute Renal Failure in the Neonate.

Acute renal failure (ARF) is a common disorder in high-risk neonates. ARF may be oliguric or nonoliguric, the latter having a better prognosis. Risk factors for ARF include prematurity, respiratory and vascular disorders, heart failure, congenital uropathies, and the use of nephrotoxic drugs. Chemical analysis of urine and ultrasounds help differentiate the nature and the type of ARF: prerenal, intrinsic, or postrenal. Conservative management of prerenal forms of ARF consists in carefully restoring cardiac output and controlling fluid and electrolyte balances. Early relief of obstruction is mandatory in severe postrenal forms of ARF. Renal replacement therapy is often necessary when ARF is secondary to intrinsic renal damage: peritoneal dialysis is the treatment of choice. Hemodialysis and continuous venovenous hemofiltration may be used in specific cases. Overall prognosis of ARF depends on the nature and severity of the renal injury that has led to renal failure.

Joubert syndrome with nephronophthisis in neurofibromatosis type 1.

Joubert syndrome (JS) is a rare developmental disorder of the central nervous system, characterised by brainstem and cerebellar malformations, hypotonia, episodic hyperapnea and apnea and mental retardation. It may be associated other systemic abnormalities like ocular (e.g., retinal dysplasia, etc.), oculomotor, musculoskeletal and renal (e.g., cystic dysplasia, nephronophthisis), with renal failure. We describe a case of JS with nephronophthisis in neurofibromatosis Type 1 leading to end-stage renal disease, a association that has never been described earlier in the medical literature to the best of our knowledge.

Moyamoya disease with renal artery and external iliac artery stenosis.

Moyamoya disease is a rare, progressive occlusive disease of the cerebral vasculature, mainly involving internal carotid and proximal cerebral arteries with development of fine collateral vascular network in brain. Coexistence of renal vascular lesion with cerebral vascular lesion has rarely described and association with external iliac and femoral vascular stenosis is not known to the best of our knowledge. This is the first case of renovascular hypertension with Moyamoya disease being reported in India with involvement of other extra cranial vessels.