Discontinuation of RAAS Inhibition in Children with Advanced CKD

Background and objectives Although renin-angiotensin-aldosterone system inhibition (RAASi) is a cornerstone in the treatment of children with CKD, it is sometimes discontinued when kidney function declines. We studied the reasons of RAASi discontinuation and associations between RAASi discontinuation and important risk markers of CKD progression and on eGFR decline in the Cardiovascular Comorbidity in Children with CKD study. Design, setting, participants,& measurements In this study, 69 children with CKD(67% male, mean age 13.7 years, mean eGFR 27 ml/min per 1.73m(2)) who discontinued RAASi during prospective follow-up were included. Initial change in BP, albuminuria, and potassium after discontinuation were assessed (median time 6 months). Rate of eGFR decline (eGFR slope) during a median of 1.9 years before and 1.2 years after discontinuation were estimated using linear mixed effects modeling. Results Physician-reported reasons for RAASi discontinuation were increase in serum creatinine, hyperkalemia, and symptomatic hypotension. After discontinuation of RAASi, BP and albuminuria increased, whereas potassium decreased. eGFR declined more rapidly after discontinuation of RAASi (23.9 ml/min per 1.73m2 per year; 95% confidence interval, 25.1 to 22.6) compared with the slope during RAASi treatment (21.5 ml/min per 1.73 m(2) per year; 95% confidence interval, 22.4 to 20.6; P=0.005). In contrast, no change in eGFR slope was observed in a matched control cohort of patients in whom RAASi was continued. Conclusions Discontinuation of RAASi in children with CKD is associated with an acceleration of kidney function decline, even in advanced CKD

Surgical Complications After Pediatric Renal Transplant.

Renal transplant is the most appropriate treatment for both adult and pediatric patients with end-stage renal failure. Here, we analyzed surgical complications after pediatric renal transplant at our center

Outcome of the Double-J Stent Placement in Pediatric Kidney Transplant: A Single Center Experience.

Double J stent placement at kidney transplant may reduce stenosis or leakage complication rates. However, placement may also increase risk for early urinary tract infection (ie, < 3 mo after transplant). In children, the usefulness of double J stent placement is not well defined

Posterior Reversible Encephalopathy Syndrome in Henoch-Schonlein Purpura and Hemolytic Uremic Syndrome.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome, composed of symptoms such as headache, seizures, visual disturbances, lethargy, confusion, stupor, focal neurologic findings and radiological findings of bilateral gray and white matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres. PRES is associated with significant morbidity and mortality if it is not expeditiously recognized. Magnetic resonance image (MRI) represents the most sensitive imaging technique for recognizing PRES. PRES has been seen in various clinical settings including renal disorders such as acute glomerulonephritis, lupus nephritis, nephrotic syndrome, and drug usage such as calcineurin inhibitors. We aimed to present two study cases for such clinical setting. In this report, we present two patients with PRES in whom the primary diagnosis was hemolytic uremic syndrome (HUS) and Henoch-Schonlein purpura (HSP). Both of them were treated with anticonvulsant and proper antihypertensive drugs. A repeated MRI scan of the head, an ophthalmologic assessment, and a follow-up electroencephalogram produced normal results with no sequelae. Early recognition of PRES as a complication during different diseases and therapies in childhood may facilitate the appropriate treatment, so that intensive treatment should be performed as soon as possible to avoid neurological sequelae

The effect of antidiuretic hormone on urine and serum electrolyte levels in children with primary monosymptomatic nocturnal enuresis

Background/aim: The data concerning the effects of desmopressin on water/electrolyte disturbances of children with primary monosymptomatic nocturnal enuresis (PMNE) are limited. In the present study we aimed to evaluate the effect and tolerability of desmopressin on blood and urine electrolytes and osmolality in PMNE. Materials and methods: Thirty-five children with PMNE between the ages of 5 and 15 participated in the study. Patients collected urine during the daytime and acknowledged the night time fluid restriction before starting to use the desmopressin tablets. The medication was taken orally at least 1 h before bedtime. Blood and urine samples were collected before the introduction of the treatment (day 0) and on the third and seventh days of the administration of desmopressin to determine osmolality and electrolyte levels. Results: Thirty-five patients participated in the study. Twenty-one patients (60\%) were male and 14 (40\%) were female. The mean age was 9.6 +/- 2.7 years. There were no significant changes in serum osmolality, urine osmolality, and serum sodium concentration. Mean urine calcium/creatinine ratio was 0.03 +/- 0.01 mg/mg at the beginning, 0.06 +/- 0.02 mg/mg on the third day, and 0.04 +/- 0.01 mg/mg on the seventh day of the study. No significant changes were seen in urine calcium/creatinine ratio before and after treatment. Conclusion: Desmopressin appeared to be a well-tolerated drug and provided a safe and effective treatment for children who were following fluid intake restriction for PMNE

Neurologic involvement in atypical hemolytic uremic syndrome and successful treatment with eculizumab

İstanbul Bilim Üniversitesi, Tıp Fakültesi.Background Atypical hemolytic uremic syndrome (aHUS) is associated with defective regulation of the complement pathway. Neurological involvement is the most common extrarenal complication and represents a major cause of mortality and morbidity. Case-diagnosis/treatment Two girls aged 11 and 6 years, respectively, developed aHUS and were treated immediately with plasma exchange (PE) and fresh frozen plasma infusion (PI). Although initial improvement in renal function was seen in both cases, the first patient showed progressing thrombotic microangiopathy (TMA) despite daily PE, and neurological manifestations (seizures, vision loss, loss of balance, and confusion) developed after 1 month. The second patient developed cerebral TMA (seizures, vision loss, and nystagmus) 6 days after initial presentation and remained unresponsive to PE/PI. Neurological symptoms were similar in both patients, even though they had different complement protein mutations. Treatment with eculizumab achieved complete control of neurological symptoms within 24 h and gradually normalized hematological and renal parameters in both children. Conclusions Based on our two cases, we conclude that eculizumab is a rapid-acting, effective, and life-saving treatment for pediatric patients with aHUS and severe neurological involvement, which works by inhibiting complement-mediated TMA in the kidney and other organs, such as the brain

Neurologic involvement in atypical hemolytic uremic syndrome and successful treatment with eculizumab

WOS: 000316571400019PubMed ID: 23389237Atypical hemolytic uremic syndrome (aHUS) is associated with defective regulation of the complement pathway. Neurological involvement is the most common extrarenal complication and represents a major cause of mortality and morbidity. Two girls aged 11 and 6 years, respectively, developed aHUS and were treated immediately with plasma exchange (PE) and fresh frozen plasma infusion (PI). Although initial improvement in renal function was seen in both cases, the first patient showed progressing thrombotic microangiopathy (TMA) despite daily PE, and neurological manifestations (seizures, vision loss, loss of balance, and confusion) developed after 1 month. The second patient developed cerebral TMA (seizures, vision loss, and nystagmus) 6 days after initial presentation and remained unresponsive to PE/PI. Neurological symptoms were similar in both patients, even though they had different complement protein mutations. Treatment with eculizumab achieved complete control of neurological symptoms within 24 h and gradually normalized hematological and renal parameters in both children. Based on our two cases, we conclude that eculizumab is a rapid-acting, effective, and life-saving treatment for pediatric patients with aHUS and severe neurological involvement, which works by inhibiting complement-mediated TMA in the kidney and other organs, such as the brain.Alexion PharmaceuticalsAssistance with writing this manuscript was provided by Bioscript Stirling Ltd, UK, and funded by Alexion Pharmaceuticals. We give special thanks to Dr. Reyhan Diz Kucukkaya in the Department of Internal Medicine, Division of Hematology at Istanbul Bilim University, Istanbul, Turkey

IgA Nephropathy and Psoriasis in a Child

Ig A nephropathy is the most common primary glomerulonephritis worldwide. Painless macroscopic hematuria is frequent in children and often coincides with infections of the upper respiratory tract and/ or digestive system. The distinctive carbohydrate side chains of IgA1 molecules play an important role in the pathogenesis of IgA nephropathy. Definitive diagnosis of IgA nephropathy requires evaluation of a renal biopsy specimen. Psoriasis is also a common childhood chronic skin disease with changes in epidermal differentation and proliferation. There are also a few literature reports on the co-existence of IgA nephropathy and psoriasis mainly in adult patients. Herein, we report a child with psoriasis who was diagnosed as Ig A nephropathy after periods of painless hematuria

Heavy Metal Exposure in Alport Syndrome in an Adolescent: A Case Report: Heavy Metal Exposure in Alport Syndrome

Alport syndrome is an inherited glomerular disease characterized by hematuria, proteinuria,hypertension, progressive kidney failure, hearing loss, and ocular pathologies. It is causedby a mutation in COL4A3, COL4A4, or COL4A5 genes. A lamellar or uniformly thinnedglomerular basement membrane is a pathognomonic histologic appearance for Alportsyndrome. Light microscopy shows nonspecific findings, including mesangial matrixexpansion and hypercellularity. Renal tubules are other main components of the kidney and themajor sites in response to injuries. They are vulnerable to various conditions, such as hypoxia,proteinuria, and nephrotoxic substances, including heavy metals, like lead and mercury.We demonstrated that a patient with asymptomatic Alport syndrome may have acceleratedworsening of kidney functions due to occupational exposure to lead and mercury. Regardingthe initial diagnosis with current clinical and laboratory findings in patients, it is noteworthythat there is always the possibility of another pathology, and additional investigations maybe needed. Besides, when considering public health issues and the financial burden due tooccupational diseases, we desired to draw attention to the importance and need to create saferwork environments and make frequent inspections