Clinical practice recommendations for native vitamin D therapy in children with chronic kidney disease Stages 2-5 and on dialysis

Vitamin D deficiency is widely prevalent and often severe in children and adults with chronic kidney disease (CKD). Although native vitamin D {25-hydroxyvitamin D [25(OH)D]} is thought to have pleiotropic effects on many organ systems, its skeletal effects have been most widely studied. The 25(OH)D deficiency is causally linked with rickets and fractures in healthy children and those with CKD, contributing to the CKD–mineral and bone disorder (MBD) complex. There are few studies to provide evidence for vitamin D therapy or guidelines for its use in CKD. A core working group (WG) of the European Society for Paediatric Nephrology (ESPN) CKD–MBD and Dialysis WGs have developed recommendations for the evaluation, treatment and prevention of vitamin D deficiency in children with CKD. We present clinical practice recommendations for the use of ergocalciferol (vitamin D2) and cholecalciferol (vitamin D3) in children with CKD Stages 2–5 and on dialysis. A parallel document addresses treatment recommendations for active vitamin D analogue therapy. The WG has performed an extensive literature review to include meta-analyses and randomized controlled trials in healthy children as well as children and adults with CKD, and prospective observational studies in children with CKD. The Grading of Recommendation, Assessment, Development and Evaluation (GRADE) system has been used to develop and grade the recommendations. In the absence of applicable study data, the opinion of experts from the ESPN CKD–MBD and Dialysis WGs is provided, but clearly GRADE-ed as such and must be carefully considered by the treating physician, and adapted to individual patient needs as appropriate

Renal Features of Bardet Biedl Syndrome: A Single Center Experience

WOS: 000382082600288

An infant with hyponatremia, hyperkalemia, and metabolic acidosis associated with urinary tract infection: Questions

PubMedID: 31053899[No abstract available

Clinical and genetic features of congenital nephrogenic diabetes insipidus: A Single Center Experience

WOS: 000382082600267

Symptomatic kidney involvement in a child with tuberous sclerosis

PubMedID: 15074379Tuberous sclerosis complex is an autosomal dominant disorder of cellular proliferation and differentiation with variable penetrance and a high spontaneous mutation rate that affects multiple organs, including the kidney. Kidney involvement is commonly asymptomatic and bilateral, and rare in childhood, especially under 10 years. Herein, we report a case of unilateral renal angiomyolipoma in a nine-year-old girl with tuberous sclerosis who had symptoms of pain and macroscopic hematuria

Hypodipsia-hypernatremia syndrome associated with holoprosencephaly in a child: A case report

PubMedID: 12405444We report a child with diabetes insipidus and hypodipsia associated with holoprosencephaly. A two-year-old girl with the history of several admittances to hospital during and after the newborn period with hypernatremic dehydration, acute renal failure and convulsions is presented. The patient had hypodipsia, hypernatremia, microcephaly, failure to thrive, and unilateral cleft lip and palate. Magnetic resonance imaging revealed lobar type holoprosencephaly. Increased plasma osmolality and decreased urinary osmolality were detected. Her urine ADH level was 10 ng/day. Plasma osmolality levels returned to normal after hydration and administration of a vasopressin analogue. These findings suggest that in children with hypernatremia-hypodipsia syndrome, the possibility of cerebral malformations should always be kept in mind

The effects of gemfibrozil on hyperlipidemia in children with persistent nephrotic syndrome

PubMedID: 11858378Persistent nephrotic syndrome is frequently accompanied by severe hyperlipidemia, and this may pose a substantial risk for cardiovascular disease. Lipid-lowering drugs are prescribed by many nephrologists for adult patients but rarely for nephrotic children. The present investigation was designed to evaluate the safety and efficacy of gemfibrozil in nephrotic children. Eight girls and four boys aged from 5 to 17 years were enrolled in this study. They were all steroid and immunosuppressive resistant patients with nephrotic range proteinuria. Placebo was administered to five patients and gemfibrozil was administered to seven patients for four months. Blood samples were taken for the determination of cholesterol, triglyceride, low-density lipoprotein (LDL), high-density lipoprotein (HDL), BUN, serum creatinine (Scr), ALT, AST, CPK, apolipoprotein A (apo A), apoliporotein B (apo B), and serum albumin levels during the initial and subsequent examinations. At the end of the fourth month, gemfibrozil reduced total cholesterol by 34%, LDL by 30%, apo B by 21% and triglycerides by 53% (p < 0.05). HDL cholesterol and apo A levels were not significantly altered. Renal function and urine protein excretion were not affected by gemfibrozil. In this study gemfibrozil therapy had no side effects and had favorable effects on the lipoprotein profile of nephrotic patients

Early onset of diabetic nephropathy in a child with type 1 diabetes mellitus

PubMedID: 12718374An 11-year-old prepubertal girl with a history of metabolically poorly controlled type 1 diabetes for six years was diagnosed with diabetic nephropathy based on persistent overt proteinuria, hypertension, and renal biopsy findings typical of diabetic nephropathy. This case illustrates that diabetic nephropathy can develop even before puberty when metabolic control of the disease is not sufficient

Should we aim a lower blood pressure load (15%) to prevent left ventricular hypertrophy in children on chronic peritoneal dialysis?

WOS: 000257594600333

Clinical manifestations and outcomes of 420 children with Henoch Schönlein Purpura from a single referral center from Turkey: A three-year experience

PubMedID: 31662011Objectives: Henoch Schönlein Purpura (HSP) is the most common systemic vasculitis in childhood. We aimed to evaluate the clinical features, seasonal variation, treatment outcomes and the possible predicting factors related to outcome among a large cohort of pediatric HSP patients. Methods: We conducted a medical record review study between July 2016 and January 2019 and evaluated the clinical manifestations and potential risk factors for severe gastrointestinal (GI) involvement, biopsy-proven nephritis and relapses. Results: The study included 420 HSP patients, of which the mean age at diagnosis was 7.68 ± 3.15 years. Clinical manifestations were arthralgia and/or arthritis (n = 244, 58.1%), abdominal pain (n = 235, 56%), subcutaneous edema (n = 163, 38.8%), and renal involvement (n = 125, 29.8%). Disease recurred for at least once, in 69 (16.4%) patients and colchicine treatment yielded a favorable response in 11 of 12 relapsing patients, who did not respond to ibuprofen or steroids. Frequencies of renal involvement and biopsy-proven nephritis were higher in patients with severe GI involvement. Besides, patients with biopsy-proven nephritis had higher rates of abdominal pain, intussusception, severe GI involvement, and systemic steroid administration. Conclusion: We speculate that renal involvement, biopsy-proven nephritis and severe GI involvement can be related to each other. Colchicine may be effective in patients with relapsing disease. © 2019, © 2019 Japan College of Rheumatology