Yosuke Miyashita

Early Volume Expansion During Diarrhea and Relative Nephroprotection During Subsequent Hemolytic Uremic Syndrome

OBJECTIVES To determine if interventions during the pre-hemolytic uremic syndrome (HUS) diarrhea phase are associated with maintenance of urine output during HUS. DESIGN Prospective observational cohort study. SETTINGS Eleven pediatric hospitals in the United States and Scotland. PARTICIPANTS Children younger than 18 years with diarrhea-associated HUS (hematocrit level <30% with smear evidence of intravascular erythrocyte destruction), thrombocytopenia (platelet count <150 × 10³/mm³), and impaired renal function (serum creatinine concentration > upper limit of reference range for age). INTERVENTIONS Intravenous fluid was given within the first 4 days of the onset of diarrhea. OUTCOME MEASURE Presence or absence of oligoanuria (urine output ≤ 0.5 mL/kg/h for >1 day). RESULTS The overall oligoanuric rate of the 50 participants was 68%, but was 84% among those who received no intravenous fluids in the first 4 days of illness. The relative risk of oligoanuria when fluids were not given in this interval was 1.6 (95% confidence interval, 1.1-2.4; P = .02). Children with oligoanuric HUS were given less total intravenous fluid (r = -0.32; P = .02) and sodium (r = -0.27; P = .05) in the first 4 days of illness than those without oligoanuria. In multivariable analysis, the most significant covariate was volume infused, but volume and sodium strongly covaried. CONCLUSIONS Intravenous volume expansion is an underused intervention that could decrease the frequency of oligoanuric renal failure in patients at risk of HUS.

Isradipine for Treatment of Acute Hypertension in Hospitalized Children and Adolescents

J Clin Hypertens (Greenwich).

Therapy of Acute Hypertension in Hospitalized Children and Adolescents

Acute hypertension (HTN) in hospitalized children and adolescents occurs relatively frequently, and in some cases, if not recognized and treated promptly, it can lead to hypertensive crisis with potentially significant morbidity and mortality. In contrast to adults, where acute HTN is most likely due to uncontrolled primary HTN, children and adolescents with acute HTN are more likely to have secondary HTN. This review will briefly cover evaluation of acute HTN and various age-specific etiologies of secondary HTN and provide more in-depth discussion on treatment targets, potential risks of acute HTN therapy, and available pediatric data on intravenous and oral antihypertensive agents, and it proposes treatment schema including unique therapy of specific secondary HTN scenarios.

Neonatal Polycystic Kidney Disease

This article provides an up-to-date comprehensive review and summary on neonatal polycystic kidney disease (PKD) with emphasis on the differential diagnosis, clinical manifestations, diagnostic techniques, and potential therapeutic approaches for the major causes of neonatal PKD, namely hereditary disease, including autosomal recessive and autosomal dominant PKD and nonhereditary PKD, with particular emphasis on multicystic dysplastic kidney. A brief overview of obstructive cystic dysplasia and simple and complex cysts is also included.

Primary hypertension and special aspects of hypertension in older children and adolescents.

The prevalence of hypertension has increased at an accelerated rate in older children and adolescents. This has raised great concern about premature development of cardiovascular disease, which has major long-term health and financial implications. While obesity and sedentary habits largely explain this phenomenon, there are other social and cultural influences that may unmask genetic susceptibility to hypertension in the pediatric population. While it is essential to exclude numerous causes of secondary hypertension in every child, these disorders are not discussed in this review. Rather, the aim of this review is to familiarize pediatricians with casual and ambulatory blood pressure measurement, epidemiology, pathophysiology, and management of several common conditions that play a role in the development of hypertension in children and adolescents. Besides primary hypertension and obesity-related hypertension, emphasis is given to epidemiology, measurement of blood pressure, including ambulatory blood pressure monitoring, hypertension associated with drug use, teenage pregnancy, and video and computer games. Lastly, because pediatricians are increasingly confronted with special issues concerning the management of the hypertensive athlete, this topic is also addressed.

Atypical Hemolytic Uremic Syndrome and Chronic Ulcerative Colitis Treated with Eculizumab.

Background Hemolytic-uremic syndrome (HUS) presents with hemolytic anemia, thrombocytopenia, and thrombotic microangiopathy of the kidney and usually results from Shiga-toxin induced activation of the alternative complement pathway. Gastroenteritis is a common feature of the Shiga-toxin producing Escherichia coli HUS, referred to as STEC-HUS. An inherited or acquired complement dysregulation may lead to HUS referred to as non-STEC or atypical (a)HUS. Although gastroenteritis is not a common presentation of aHUS, some patients develop ischemic colitis and may be misdiagnosed as acute appendicitis or acute ulcerative colitis (UC). Case Diagnosis –Treatment We present a patient with low circulating complement (C) 3 levels who developed aHUS in the course of chronic active UC. Resolution of renal and gastrointestinal manifestations in response to treatment with eculizumab, a humanized monoclonal antibody against terminal C5 protein suggests the role of alternative complement in the pathogenesis of both, aHUS and UC. Conclusion This case illustrates that dysregulation of the alternative complement pathway may manifest in other organs besides the kidney and that the circulating C3 levels do not correlate with the disease activity or the clinical response to eculizumab.

Diagnosis and management of white-coat hypertension in children and adolescents: A Midwest Pediatric Nephrology Consortium study

Although the definition of white‐coat hypertension (WCH) in children and adolescents is clearly defined, little is known about how this condition is actually approached clinically. To better understand the contemporary approach to the diagnosis and management of WCH in pediatric patients, the authors surveyed the membership of the Midwest Pediatric Nephrology Consortium. Seventy‐four faculty pediatric nephrologists responded to the survey. The survey results demonstrated uniformity in diagnosing WCH, including ambulatory blood pressure monitoring use in 93% of the respondents and a 75% adherence rate according to the 2014 American Heart Association scientific statement on pediatric ambulatory blood pressure monitoring. A total of 85% of respondents would not embark on further diagnostic evaluation once the WCH diagnosis was established, and none would initiate antihypertensive medications. There was a wide variety of practice habits in follow‐up of WCH including frequency of office and out‐of‐office follow‐up blood pressure measurements, the setting and timing of physician follow‐up, and the role of repeat ambulatory blood pressure monitoring. The results of this survey highlight the need for prospective studies aimed at establishing the optimal approach to pediatric patients with WCH.

The Use of Ambulatory Blood Pressure Monitoring As Standard of Care in Pediatrics

Hypertension (HTN) is a significant global health problem, responsible for 7.5 million deaths each year worldwide. The prevalence of HTN is increasing in the pediatric population likely attributed to the increase in childhood obesity. Recent work has also shown that blood pressure (BP) tends to track from childhood to adulthood including BP-related target organ damage. In the last 25–30 years, pediatric use of ambulatory blood pressure monitoring (ABPM) has been expanding mainly in the setting of initial elevated BP measurement evaluation, HTN therapy efficacy follow-up, and renal disease. However, there are many clinical areas where ABPM could potentially be used but is currently underutilized. This review summarizes the current knowledge and the uses of pediatric ABPM and explores clinical areas where it can be very useful both to detect HTN and its longitudinal follow-up. And thus, ABPM could serve as a critical tool to potentially prevent early cardiovascular mortality and morbidity in wide variety of populations. With solid data to support ABPM’s superiority over clinic BP measurements and these clinical areas for its expansion, ABPM should now be part of standard of care in BP evaluation and management in pediatrics.