Stefan G. Kiessling

Rabson-Mendenhall syndrome: medullary sponge kidney, a new component

Rabson-Mendenhall syndrome is a rare genetic disorder characterized by severe insulin resistance, extreme hyperinsulinemia, postprandial hyperglycemia, growth retardation, and dysmorphisms. Enlargement of the kidneys and nephrocalcinosis have been described previously. We report a 10-year-old boy who presented with gross hematuria, unilateral hydronephrosis, and the initial diagnosis of bilateral extensive medullary nephrocalcinosis. Medullary sponge kidney (MSK) was included in the differential diagnosis given the ultrasound findings. Further evaluation by intravenous pyelogram confirmed the suspected bilateral MSK. Given the patient’s history of hydronephrosis due to an obstructing renal stone and MSK, urine calcium excretion was assessed and found to be markedly increased at 9.5xa0mg/kg per day. To our knowledge, this is the first report of Rabson-Mendenhall syndrome and an association with MSK. We recommend evaluation for nephrocalcinosis, MSK, and hypercalciuria in all children diagnosed with Rabson-Mendenhall syndrome.

Pediatric nephrology in the ICU

Disorders of Salt and Water Balance.- Disorders of the Acid-Base Status.- Dyskalemias.- Disorders of Calcium and Phosphate Regulation.- Abnormalities in Magnesium Metabolism.- Acute Kidney Injury: General Aspects.- Pharmacotherapy in the Critically Ill Child with Acute Kidney Injury.- Renal Replacement Therapy in the ICU.- Nutrition for the Critically Ill Pediatric Patient with Renal Dysfunction.- Tools for the Diagnosis of Renal Disease.- Urosepsis.- Hypertension in the Pediatric Intensive Care Unit.- Acute Glomerulonephritis.- Acute Interstitial Nephritis.- The Tumor Lysis Syndrome: An Oncologic and Metabolic Emergency.- Hemolytic Uremic Syndrome.- Vasculitis.- Renal Issues in Organ Transplant Recipients in the PICU.- Acute Kidney Injury Following Cardiopulmonary Bypass.- Contrast-Induced Nephropathy.- Intoxications.

An Unusual Case of Severe Therapy-Resistant Hypertension in a Newborn

Hypertension can occur in up to 2% of neonates, and the spectrum of potential causes is broad. Prompt and thorough evaluation with a main focus on kidney disease is key for appropriate therapy. Here we describe a 2-day-old neonate with feeding intolerance and elevated blood pressure readings. Within 24 hours after birth, the infants blood pressure increased significantly, with sustained mean arterial pressure >85. Renal Doppler ultrasound showed decreased venous blood flow in the right kidney with an abnormal Doppler wave form suggestive of unilateral renal venous thrombosis. Despite aggressive antihypertensive therapy including hydralazine and enalaprilat, hypertension remained sustained. On day-of-life 4, the infant developed clinical signs of hypertensive encephalopathy and significant cardiac dysfunction. A renal angiography showed complete, likely thrombotic occlusion of the right renal artery. Renal MAG3 imaging showed minimal function of the affected kidney, and a nephrectomy secondary to medically uncontrollable hypertension and worsening cardiac dysfunction was performed. The child is developing normally in all aspects on follow-up evaluations at 6 months and 1 year of age. Reevaluation of the working diagnosis in neonates with hypertension can be necessary to optimize the outcome. The overall prognosis can be excellent even in newborns with profound cardiac and neurologic involvement.

A guideline for the inpatient care of children with pyelonephritis.

Background and Objectives :Febrile urinary tract infections and pyelonephritis are common in children and frequently lead to hospitalization for management, especially in the child who appears toxic. The American Academy of Pediatrics (AAP) practice parameter on the diagnosis, treatment and evaluation of the initial urinary tract infection in febrile infants and young children provides experience and evidence-based guidelines for the practitioner caring for children between the ages of 2 months to 2 years. No established guideline exists for older children and the AAP guideline does not specifically focus on inpatient care. Methods : We conducted a comprehensive review of recently published literature and practice guidelines to develop a consensus on the inpatient diagnosis and management of children with pyelonephritis. Results : Eight recommendations are proposed for the diagnosis and management, including revised guidelines for the imaging studies postpyelonephritis on the basis of current best evidence. Conclusion : Proper diagnosis of pyelonephritis, timely initiation of appropriate therapy and identification of children at risk for renal injury will help to reduce immediate as well as long-term complications due to chronic kidney disease.

Kidney and urinary tract diseases in the newborn

Kidney and urinary tract diseases in the newborn / , Kidney and urinary tract diseases in the newborn / , کتابخانه دیجیتال جندی شاپور اهواز

Management of pediatric hypertension

Hypertension is one of the major contributors to cardiovascular, renal and CNS morbidity and mortality. Although it is more prevalent in adults, hypertension and its sequelae are being seen with increasing frequency in pediatrics recently. The majority of children have hypertension secondary to renovascular and renal parenchymal disease, and it appears that the increased incidence is primarily related to the epidemic of obesity, which is a known risk factor for the development of the condition. After the diagnosis of hypertension is established, a thorough evaluation for secondary causes should be conducted according to existing guidelines. Nonpharmacologic interventions should be discussed in any child, even though the diagnosis of hypertension may not be confi rmed, but if risk factors are identifi ed. Those interventions should be even more encouraged if the diagnosis of hypertension or prehypertension is confi rmed. Nonpharmacologic intervention focuses on salt restriction, diet modifi cation, exercise and physical activity, as well as improved sleep habits. Initiation of medical therapy is guided by the degree of blood pressure elevation, presence of symptoms and existing risk factors. A wide variety of oral medications and intravenous therapies are available for use in children with mild, moderate or severe blood pressure elevations. This article reviews the diagnosis and evaluation of hypertension in children, and provides an update on the pharmacologic and nonpharmacologic treatment options for hypertension, including hypertensive urgency and emergency.

Vitamin D in incident nephrotic syndrome: a Midwest Pediatric Nephrology Consortium study

BackgroundCross-sectional studies of children with prevalent nephrotic syndrome (NS) have shown 25-vitamin D (25(OH)D) deficiency rates of 20–100xa0%. Information on 25(OH)D status in incident patients or following remission is limited. This study aimed to assess 25(OH)D status of incident idiopathic NS children at presentation and longitudinally with short-term observation.MethodsMulticenter longitudinal study of children (2–18xa0years old) from 14 centers across the Midwest Pediatric Nephrology Consortium with incident idiopathic NS. 25(OH)D levels were assessed at diagnosis and 3xa0months later.ResultsSixty-one children, median age 5 (3, 11) years, completed baseline visit and 51 completed second visit labs. All 61 (100xa0%) had 25(OH)Du2009<u200920xa0ng/ml at diagnosis. Twenty-seven (53xa0%) had 25(OH)Du2009<u200920xa0ng/ml at follow-up. Fourteen (28xa0%) children were steroid resistant. Univariate analysis showed that children prescribed vitamin D supplements were less likely to have 25(OH)D deficiency at follow-up (OR 0.2, 95xa0% CI 0.04, 0.6). Steroid response, age, and season did not predict 25(OH)D deficiency. Multivariable linear regression modeling showed higher 25(OH)D levels at follow-up by 13.2xa0ng/ml (SE 4.6, pu2009<u20090.01) in children supplemented with vitamin D.ConclusionsIn this incident idiopathic NS cohort, all children at diagnosis had 25(OH)D deficiency and the majority continued to have a deficiency at 2–4xa0months. Supplemental vitamin D decreased the odds of 25(OH)D deficiency at follow-up, supporting a role for supplementation in incident NS.

Influence of BMI in nephrolithiasis in an Appalachian pediatric population: A single-center experience

INTRODUCTIONnThe prevalence of pediatric nephrolithiasis has increased significantly in the past 20 years. Metabolic abnormalities predisposing adults to nephrolithiasis in obese patients include increased urinary sodium and uric acid excretion as well as low urine pH; however, limited data are available in the pediatric population.nnnOBJECTIVEnThe aim was to investigate whether obese pediatric patients presenting with nephrolithiasis have a unique metabolic profile similar to reported findings in obese adults with nephrolithiasis.nnnSTUDY DESIGNnA retrospective chart review was performed in children aged 1-18 years seen at Kentucky Childrens Hospital between 2010 and 2016. Inclusion criteria included all patients with documented stones confirmed by ultrasonography or computed tomography.nnnRESULTSnA total of 111 patient charts were reviewed in the study with a mean age of 11.8xa0±xa04.2 years. Seventy patients (63%) had a normal BMI and 41 patients (37%) were considered overweight/obese. There was no statistically significant relationship between BMI and stone recurrence. Obese patients had significantly decreased levels of urinary citrate, oxalate, magnesium, and potassium with significant elevations of urinary urea nitrogen, ammonia, and low urine pH compared with normal weight patients (Summary Figure).nnnDISCUSSIONnSeveral groups have reported on metabolic findings within obese and non-obese pediatric patients. A Turkish study reported increased oxalate excretion and hypocitraturia in obese patients while a Korean study also reported increased rates of hypocitraturia in recurrent stone formers. Similar to these studies, we did find significant differences in citrate within our study population; however, we found significantly lower levels of urinary oxalate in obese patients. The majority of these studies do not report an association with BMI and urine pH although this has been reported in the adult population and our findings support an inverse relationship between body mass index (BMI) and pH. Our group found a higher level of calcium phosphate stones, supporting of Eisners findings that high BMI is associated with increased supersaturation of calcium phosphate. Limitations of our study include being a single center and retrospective in nature.nnnCONCLUSIONnOur study demonstrates differences in types of stones and urinary metabolites in an obese pediatric population suggestive of different metabolic profiles contributing to stone disease. We report similar association between BMI and urine pH, urinary potassium, and citrate. This study confirmed our primary hypothesis that obese pediatric patients would have a different urinary mineral profile as evidenced by lower levels of citrate and potassium and low urine pH; however, obese patients did not exhibit significantly elevated urinary sodium and uric acid when normalized to weight, as described in the adult population. Our study did not confirm our secondary hypothesis that stone composition would be associated with BMI status or stone recurrence.

Congenital Nephrotic Syndrome

Congenital nephrotic syndrome (CNS) is a group of rare conditions that present with high-grade urine protein (albumin) losses, hypoalbuminemia, and edema within the first 3 months of life. It is important to differentiate CNS from other presentations of nephrotic syndrome, mainly the infantile form (4–12 months of age) and childhood nephrotic syndrome (onset after the 1st year of life). Unless treatment is initiated, CNS is universally fatal.

Adults who had kidney disease in childhood

Abstract A young adult male who recently turned 21 years of age has been followed by a group of pediatric kidney specialists since birth as he was born with a single dysplastic kidney. He progressed towards end stage renal disease and started hemodialysis in a pediatric inpatient center at the age of 19 years. Owing to being significantly overweight, he is not a suitable renal transplant candidate. He lives at home with his mother, who is his primary caregiver and his brother. He dropped out of school before the age of 18 years and has currently no intention to finish high school. He has short stature as he refused daily growth hormone injections in the past. During healthcare visits he has always been passive and his mother is making decisions for him. During the last several visits, he and the mother have requested initiation of transition into adult care as he feels it is now time. Both patient and mother also start to ask questions about the adult implications of his chronic kidney disease (CKD). CKD in children is somewhat complex and the implications for adults living with advanced kidney disease since childhood are significant. Optimal care of the adult patient who has been affected by advanced kidney disease since childhood requires a solid understanding of three main domains with significant overlap: (i) the underlying type of kidney disease and the multiple effects that CKD has on the body and mind of a child, (ii) the process of care transitioning from the pediatric to the adult environment, and (iii) the physical and psychosocial effects of the disease on the adult patient.