Factors Associated With Hypertension and Cardiovascular Parameters in Children With Infrequently Relapsing Nephrotic Syndrome

Objective: To assess the prevalence of hypertension in children with infrequently relapsing nephrotic syndrome (IRNS) and its association with dyslipidemia, and end organ damage including left ventricular hypertrophy (LVH), at relapse and after steroid induced remission. Methods: Prospective observational study conducted in 83 children aged 1-12 years with IRNS, presenting in relapse. Blood pressure, fundus examination, blood and urine investigations were done at relapse and then at 4 weeks of therapy. Echocardiography at 4 weeks was performed for assessment of LVH and relative wall thickness (RWT) for concentric geo-metry (CG). Results: 27 patients (32.5%) developed hypertension, out of which 21 patients (25.3%) had stage I hypertension. Hypertension in first episode (63.0%, P<0.01) and in previous relapses (87.5%, P<0.001) was significantly associated with hypertension in the current episode. 12 patients had a positive family history of hypertension, of which 8 (66.7%) were classified under the hypertensive group (P=0.016). Concentric geometry (CG) was found in 28% of hypertensive and 5.5% of non-hypertensive children (P=0.011). On regres-sion analysis, a lower Up:Uc at the time of relapse was found to have a protective role for development of hypertension. Conclusion: One third children with IRNS had hypertension at relapse and a high proportion of hypertensive patients had CG pattern on echocardiography.

Vitamin D-dependent rickets (VDDR) type 1: case series of two siblings with a CYP27B1 mutation and review of the literature / Raquitismo dependente de vitamina D: tipo 1: série de casos de dois irmãos com mutação CYP27B1 e revisão da literatura

Abstract Two siblings presented with clinical and biochemical features of rickets, initially suspected as hypophosphatemic rickets. There was no improvement initially, hence the siblings were reinvestigated and later diagnosed as having vitamin D-dependent rickets (VDDR) type 1 due to a rare mutation in the CYP27B1 gene encoding the 1α-hydroxylase enzyme. Both siblings improved with calcitriol supplementation. The initial presentation of VDDR is often confusing and algorithmic evaluation helps in diagnosis. We also present a brief review of the literature, including genetics.

Resumo Dois irmãos apresentaram características clínicas e bioquímicas do raquitismo, com suspeita clínica inicial de raquitismo hipofosfatêmico. Não houve melhora no início, portanto os irmãos foram reavaliados e, posteriormente, diagnosticados com raquitismo dependente de vitamina D (VDDR) tipo 1 devido a uma rara mutação no gene CYP27B1, que codifica a enzima 1a-hidroxilase. Ambos os irmãos melhoraram com a suplementação de calcitriol. A apresentação inicial do VDDR geralmente é confusa e a avaliação algorítmica ajuda no diagnóstico. Também apresentamos uma breve revisão da literatura, incluindo genética.

American Academy of Pediatrics Clinical Practice Guidelines for Screening and Management of High Blood Pressure in Children and Adolescents: What is New?

Childhood hypertension has become a significant health concern. There have been a slew of important new findings in this field over thelast decade. This has led to an update by the American Academy of Pediatrics of the original recommendation of United States FourthWorking Group on blood pressure. We herein describe the important changes in the guideline, which include an updated normativedata, change in blood pressure classification, strong endorsement of ambulatory blood pressure measurement and the reduction in theblood pressure target for both chronic kidney disease and non-chronic kidney disease hypertensive children.

Nuclear Imaging in Pediatric Kidney Diseases

Renal scintigraphy is a useful tool in diagnosis and management of various nephro-urological conditions. Tc-99m dimercaptosuccinicacid renal scintigraphy (Tc-99m-DMSA), Tc-99m mercaptoacetyltriglycine (Tc-99m-MAG3) or Tc-99m diethylenetriaminepentaaceticacid (Tc-99m-DTPA) dynamic renal scintigraphy, and Radionuclide micturating cystography are the common scans used in children withkidney diseases. These studies are minimally invasive, easily available, and offer both anatomic details and functional informationrequired for thorough evaluation. At the same time, it is essential to have appropriate knowledge to interpret these studies and be awareof their limitations and pitfalls. The advent of Positron emission tomography-computed tomography/magnetic resonance imaging (PET-CT/MRI) has broadened the scope of nuclear medicine. This article focuses on the technique, interpretation, indication and recentpractice guidelines of renal scintigraphy in children with kidney diseases.

The 2015 Academic College of Emergency Experts in India’s INDO-US Joint Working Group White Paper on Establishing an Academic Department and Training Pediatric Emergency Medicine Specialists in India.

PRASHANT MAHAJAN, PRERNA BATRA1, BINITA R SHAH2, ABHIJEET SAHA3, SAGAR GALWANKAR4, PRAVEEN AGGRAWAL5, AMEER HASSOUN2, BIPIN BATRA6, SANJEEV BHOI5, OM PRAKASH KALRA7 AND DHEERAJ SHAH1 From Department of Pediatrics and Emergency Medicine, Wayne State School of Medicine, Michigan, 2Department of Emergency Medicine, SUNY Downstate Medical Center, New York, 4University of Florida, Department of Emergency Medicine, Jacksonville, Florida, USA; Departments of 1Pediatrics and 7Medicine, University College of Medical Sciences and Guru Tegh Bahadur Hospital, Delhi, 3Department of Pediatrics, Post Graduate Institute of Medical Education and Research and Ram Manohar Lohia Hospital, 5Department of Emergency Medicine, All India Institute of Medical Sciences, and 6National Board of Examinations, New Delhi, India. Correspondence to: Dr Prerna Batra, Department of Pediatrics, University College of Medical Sciences and Guru Tegh Bahadur Hospital, Dilshad Garden, Delhi 110 095, India. drprernabatra@yahoo.com W H I T E P A P E R The concept of pediatric emergency medicine (PEM) is virtually nonexistent in India. Suboptimally organized prehospital services substantially hinder the evaluation, management, and subsequent transport of the acutely ill and/or injured child to an appropriate facility. Furthermore, the management of the ill child at the hospital level is often provided by overburdened providers who, by virtue of their training, lack experience in the skills required to effectively manage pediatric emergencies. Finally, the care of the traumatized child often requires the involvement of providers trained in different specialities, which further impedes timely access to appropriate care. The recent recognition of Doctor of Medicine (MD) in Emergency Medicine (EM) as an approved discipline of study as per the Indian Medical Council Act provides an unprecedented opportunity to introduce PEM as a formal academic program in India. PEM has to be developed as a 3- year superspeciality course (in PEM) after completion of MD/Diplomate of National Board (DNB) Pediatrics or MD/DNB in EM. The National Board of Examinations (NBE) that accredits and administers postgraduate and postdoctoral programs in India also needs to develop an academic program – DNB in PEM. The goals of such a program would be to impart theoretical knowledge, training in the appropriate skills and procedures, development of communication and counseling techniques, and research. In this paper, the Joint Working Group of the Academic College of Emergency Experts in India (JWG-ACEE-India) gives its recommendations for starting 3-year DM/DNB in PEM, including the curriculum, infrastructure, staffing, and training in India. This is an attempt to provide an uniform framework and a set of guiding principles to start PEM as a structured superspeciality to enhance emergency care for Indian children.

OEIS complex with major cardiac malformation: a case report.

OEIS Complex is a rare congenital multisystem defect that consists of omphalocele, exstrophy, imperforate anus and spinal defects. We report a case of such complex with additional major cardiac and other multisystem anomalies which are rarely described in literature. The authors give a review of literature on this infrequent complex along with a discussion on its pathogenesis, differential diagnosis and prenatal diagnosis.