Nephrogenic Diabetes Insipidus Secondary to Obstructive Uropathy – An Unusual Presentation- A Case Report

Diabetes insipidus is a clinical condition, characterized by polyuria and polydipsia, that results from either insufficient production or end organ resistance to antidiuretic hormone (ADH). Diabetes insipidus is of two types: central diabetes insipidus (CDI) and nephrogenic diabetes insipidus (NDI).Children with diabetes insipidus may present with polyuria, polydipsia, frequent dehydration, constipation, and failure to thrive. Acquired NDI may results from various drugs and conditions but NDI due to obstructive uropathy is very rare. There are very few case reports of NDI due to obstructive uropathy. We report a 2-year-old child who presented with diabetes insipidus as a consequence of obstructive uropathy.Keywords: Nephrogenic diabetes insipidus; Obstructive Uropathy; Child.

Efficacy of Cyclophosphamide versus Cyclosporine in Frequent Relapse Nephrotic Syndrome – A Hospital Based Study

Introduction: The clinical outcome of patients with Frequent Relapse Nephrotic Syndrome (FRNS) or Steroid Dependent Nephrotic Syndrome (SDNS) treated with cyclophosphamide or cyclosporine (CsA) is yet to be established. This study was carried out to compare the efficacy of CsA with cyclophosphamide in patients with FRNS or SDNS.Materials and Methods: A total of 54 FRNS or SDNS children were randomly enrolled in this prospective study from August 2013 to July 2014. All the study subjects were treated with prednisolone 60 mg/m2 /day until the patients were in remission for three consecutive days. The patients were then randomly divided into two groups (Group-A & Group-B). Group-A was treated with cyclophosphamide at a dose of 2.5 mg/kg/day for 60 days, along with tapering dose prednisolone for 8 weeks. The Group-B study population was treated with cyclosporine at a dose of 3 mg/kg/day for 6 months or longer along with tapering dose of alternate day prednisolone for the initial 8 weeks. Four patients in Group-B and one patient in Group-A did not continue the treatment. So, we followed-up 49 children during this period.Results: The efficacy of both drugs was good after 6 months of treatment. Remission was observed in 80% of the cases in Group-A and 79% of the cases in Group-B. Even after 6 months of treatment 6.7% and 10.5% of the patients with SDNS in Group-A and Group-B needed to continue corticosteroid therapy, respectively. The side effects of immunosuppressive therapy were more frequently observed in Group-B patients. On the other hand, the mean serum creatinine level after 6 months therapy was 0.55±0.21mg/dl in Group-A and 0.84±0.43 mg/dl in Group-B. The difference between the two groups was statistically significant (p<0.05).Conclusions: This study showed that both drugs were effective in FRNS and SDNS.Keywords: Child; Nephrotic syndrome; Cyclophosphamide; Cyclosporine

Mycophenolate Mofetil versus Cyclosporine in Children with Frequent Relapse Nephrotic Syndrome

Introduction: Children with frequently relapsing nephrotic syndrome (FRNS) frequently develop adverse effects of prednisolone and attempts to induce long term remission in such patients have varying degree of success. Materials & Methods: We conducted a randomized clinical trial in a tertiary care level hospital to compare the efficacy and safety of a 1 year treatment with mycophenolate mofetil (MMF) and Cyclosporine (CsA) in 60 pediatric patients with FR-SSNS during one year period. We assessed the frequency of relapse as the primary end point and evaluated clinical and laboratory profile after 3 and 6 months of treatment. Results: Mean number of relapse was 1.50 ± 1.44 in MMF group and 0.72 ± 1.30 in CsA group at 6 months (p=0.045). Diarrhoea was statistically significant in MMF group. Hypertrichosis & hypertension were statistically significant in CsA group. There was no significant difference between groups with Hb levels, lipid profile, eGFR. Conclusion: The result of the study showed MMF is inferior to CsA in preventing relapse in patient with FRNS but less nephrotoxic

Echocardiographic Assessment of Systolic Myocardial Dysfunction in Children with Chronic Kidney Disease

Background and Aim: Chronic Kidney Disease (CKD) is associated with significantly increased morbidity and mortality.  Cardiovascular causes contribute to the large proportion of morbidity and mortality in this group. The aim of the current study was to assess systolic myocardial dysfunction of the heart in children with chronic kidney disease (stage V). Methods: This cross-sectional study was carried out in a tertiary center from January 2018 to December 2018. Children aged 1 to 18 years having chronic kidney disease (stage V) were included in the study. All the patients underwent color Doppler echocardiography for the evaluation of cardiac abnormalities. Age and sex-matched healthy children with no clinical evidence of renal and cardiovascular disease were taken as a comparison group. Results: Out of Thirty children with Chronic kidney disease (stage V), 21(70%) were male and 9 (30%) female. Echocardiographic findings revealed a statistically significant difference in case of CKD patients group and a comparison group in relation with left atrium (LA), left ventricular internal diameter in diastole (LVIDd), left ventricular internal diameter in systole (LVIDs), interventricular septum in diastole (IVSd), interventricular septum in systole (IVSs), left ventricular posterior wall thickness in diastole (LVPWd) and left ventricular posterior wall thickness in systole (LVPWs) (p<0.001) but no difference in relation to FS and EF (p-0.934 & p-0.754 respectively). Conclusion: It can be concluded from the present study that children with chronic kidney disease are prone to develop left ventricular structural abnormality but systolic functional changes are less frequent

Predictors of Relapse in Idiopathic Nephrotic Syndrome in Children Presenting to a Tertiary Center

Background and Aim: Idiopathic nephrotic syndrome (INS) is a disease of relapse and remission in children in which about 20-30% of the patients may experience a single episode in their lives while the rest suffer from relapse. Some factors contribute to relapse. The objective of this study was to explore the predictors of relapse in INS.Methods: A prospective longitudinal study was conducted in the Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University (BSMMU) from September 2015 to August 2016. One hundred and ten patients with the first episode of INS were uninterruptedly enrolled in this study. After screening and subsequent confirmatory diagnosis, the patients were treated with a single morning dose of oral prednisolone 60 mg/m2/day for 6 weeks followed by oral prednisolone 40 mg/m2 every alternate day for the subsequent 6 weeks. The total number of relapses within six months after the initial episode was collected in a data collection sheet. Every relapse was correlated with age of onset, sex, initial serum albumin level, fasting serum cholesterol level, and time to response in the initial attack. The association between relapse and its potential risk factors was investigated using logistic regression analysis. Results: The study found that INS was significantly associated with sex and age (p< 0.05). Male children were 3.04 times (95% CI= 1.09- 8.45, P=0.03) more likely to experience relapse. Furthermore, children with respiratory tract infection  had a higher chance of relapse (OR= 4.43, 95% CI= 1.26-15.53, P=0.02). Lower cholesterol levels (≤500 mg/dl) were found to be a protective factor (OR= 0.13, 95% CI= 0.04-0.54, P=0.005) in this study. The chance of relapse was higher in children who responded after 2-4 weeks of starting treatment compared to those who responded in less than one week (p<0.005).Conclusion: The study found that the first episode INS had a higher risk of relapse in boys with respiratory tract infection and in patients who needed more than two weeks of prednisolone treatment to achieve remission.Keywords: Children; Frequently Relapsing; Predictors; Idiopathic Nephrotic Syndrome

Spectrum of Hospital Acquired Acute Kidney Injury in Critically ill Children in a Tertiary Level Hospitall

Introduction: Although hospital acquired acute kidney injury (hAKI) is common and significantly increases the risk of hospital mortality, little is known about its frequency in developing countries where ICU facilities are limited. The purpose of this study was to investigate the frequency, cause, and outcome of hAKI in critically ill children in a tertiary level hospital.Materials and Methods: In this prospective cross-sectional study, a total 36 critically ill patients with hAKI were analyzed. hAKI was diagnosed according to the AKIN criteria. The clinical data of the patients admitted to the Pediatrics and Allied Departments in this hospital from November 2014 to October 2015 were collected.Results: A total of 3950 patients were admitted during the study period and 1103 (27.9%) were critically ill patients. Among the critically ill children, 36 (3.3%) were diagnosed with hAKI. Among different age groups, the highest incidence (5.05%) of hAKI was seen in children aged above 10 years. Sepsis was the major cause of hAKI accounting for 44.1% followed by antibiotics (27.1%), hypovolemia (13.6%), nephrotoxic agents (10.2%), and contrast agents (5.0%). Renal replacement therapy was required only in 8.3% of the cases.Conclusions: In comparison to other studies, this study showed a low incidence of hAKI where ICU facilities are limited. Among the hospital admitted critically ill patients, a significant number of patients may develop AKI mostly due to sepsis and use of antibiotics. Keywords: Hospital acquired AKI; Sepsis; Critically ill children; Incidenc

Are Boys at Risk of Low Immune Response to Recombinant Hepatitis B Virus Vaccine in Steroid Sensitive Nephrotic Syndrome?

Introduction: This study was conducted to evaluate the immune response following vaccination with different doses and to compare the antigen specific antibody response to Hepatitis B Vaccine (HBVac) in Steroid Sensitive Nephrotic Syndrome (SSNS) patients between boys and girls.Materials and Methods: This prospective study was conducted in 30 SSNS children at Bangladesh Institute of Child Health, Dhaka, Bangladesh from July 2012 to June 2013.Thirty patients who had all features of Minimal Change Nephrotic Syndrome (MCNS) according to International Study for Kidney Diseases for Children (ISKDC) and received oral prednisolone every alternate day and were HBsAg negative were enrolled in the study. The patients were randomly assigned to one of the two treatment group to receive either 0.5 ml (10μg) or double dose 1ml (20μg) of HB vaccine in a 0-1-2month schedule. After excluding hepatitis B virus infection, the vaccine was administered at a standard dose in group-A (0.5 ml or 10 microgram) and at a double dose (1 ml or 20 microgram) in group-B. After one month of the last dose, the seroprotection rate was measured and compared among sexes.Results: The mean age of the participants in group-A was 5.81 ± 1.73 years with a boy to girl ratio of 9:6 and the mean age of the subjects in group-B was 5.65 ± 1.68 years with a boy to girl ratio of 8:7. The mean vaccine titer was 25.60 ±19.97 mIU/ml in group-A and 617.47 ±292.11 mIU/mlin group-B, with a significant difference (p<0.05) between the two groups. Irrespective of the dose, the mean vaccine titer was higher in girls (37.33 ± 19.45 mIU/ml) compared to boys (16.22 ± 14.81mIU/ml) and the difference was statistically significant in group-A. It was also observed that the mean vaccine titer was significantly higher in girls (743.00±252.34mIU/ml) compared to boys (394.88±246.63 mIU/ml) in group-B (p<0.05,t-test).Conclusions: The results of our study showed a reduced response to HB Vaccine boys with SSNS in comparison to girls. As the study size was small, single center study and time limited follow-up, we cannot draw any valid conclusions.Keywords: Immune deficiency; Sex differences; Steroid Sensitive Nephrotic Syndrome; Seroconversion

Mutation of NPHS1, NPHS2, WT1, LAMB2, COL4A5 and other genes in children with idiopathic steroid resistant nephrotic syndrome

Background: Many children with idiopathic steroid resistant nephrotic syndrome have been reported worldwide due to mutation of NPHS1, NPHS2, WT1 and LAMB2 genes. This study aimed to determine the frequency of mutation of NPHS1, NPHS2, WT1, LAMB2, COL4A5 and other genes and their association with renal histopathological patterns of idiopathic steroid resistant nephrotic syndrome patients. Methods: This cross-sectional study was conducted on 25 patients with idiopathic steroid resistant nephrotic aged 1-17 years in the Department of Paediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Bangladesh, from July 2017 to June 2018. Next Generation Sequencing and mutation analysis were performed after DNA extraction from patients\u27 venous blood lymphocytes. Histopathological study of renal tissue was done among 17 patients. Results:  A little more than half (56%) of the patients were male. The mean age at the initial attack of nephrotic syndrome was 94.2 months. They mostly had minimal change disease (41%) and IgA nephropathy (12%). One subject had the NPHS2 gene mutation, histopathologically diffuse mesangial proliferative glomerulonephritis, and clinically stage-4 chronic kidney disease. Another subject had the COL4A5 gene mutation and focal segmental glomerulosclerosis.  Both were male and had no familial renal disease, consanguinity, or hematuria. Conclusion: Children with idiopathic steroid resistant nephrotic syndrome showed NPHS2 and COL4A5 gene mutations. Histopathologically, they showed diffuse mesangial proliferative glomerulonephritis and focal segmental glomerulosclerosis

Clinicopathological Spectrum and Treatment Outcome of Clinically Suspected Rapidly Progressive Glomerulonephritis: An Analysis of 35 Cases in a Tertiary Care Center, Bangladesh

Background and Aim: Rapidly progressive glomerulonephritis (RPGN) is characterized by a rapid decline in the renal function and urinary abnormalities. There is limited information on epidemiological factors and clinical and histopathological patterns of RPGN from developing countries. Therefore, the objective of this study was to identify the etiology, clinical features, histopathological patterns, and treatment outcomes of patients with clinically suspected RPGN. Methods: This retrospective study was conducted in the Pediatric Nephrology Department of Bangabandhu Sheikh Mujib Medical University from January 2014 to January 2019. Patients with clinically suspected RPGN that underwent renal biopsy were enrolled in this study. Results: Thirty-five patients were recruited in this study. Macroscopic hematuria, edema, hypertension, uremia, and oliguria were common clinical presentations. Diffuse proliferative GN (28.5%) and crescentic GN (22.8%) were the most common histological diagnoses in this study. Immune mediated GN (62%) followed by idiopathic GN (25%) were found to be the most frequent cause of crescentic GN. Renal replacement therapy was required in 45% of the cases and 11.4% of the patients developed end-stage renal disease.Conclusion: Renal histology is an integral part of the investigation of patients with suspected RPGN for both diagnostic and prognostic purposes. Diffuse proliferative GN was the most common histopathological diagnosis in patients with clinical RPGN in our population. Preservation of renal function depends on early intervention and detection of RPGN in pediatric patients.Keywords: Nephritis; Glomerulonephritis; ESRD; End-Stage Kidney Disease; Child.

Mutation of NPHS1, NPHS2, WT1, LAMB2, COL4A5 and other genes in children with idiopathic steroid resistant nephrotic syndrome

Background: Many children with idiopathic steroid resistant nephrotic syndrome have been reported worldwide due to mutation of NPHS1, NPHS2, WT1 and LAMB2 genes. This study aimed to determine the frequency of mutation of NPHS1, NPHS2, WT1, LAMB2, COL4A5 and other genes and their association with renal histopathological patterns of idiopathic steroid resistant nephrotic syndrome patients. Methods: This cross-sectional study was conducted on 25 patients with idiopathic steroid resistant nephrotic aged 1-17 years in the Department of Paediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Bangladesh, from July 2017 to June 2018. Next Generation Sequencing and mutation analysis were performed after DNA extraction from patients' venous blood lymphocytes. Histopathological study of renal tissue was done among 17 patients. Results:  A little more than half (56%) of the patients were male. The mean age at the initial attack of nephrotic syndrome was 94.2 months. They mostly had minimal change disease (41%) and IgA nephropathy (12%). One subject had the NPHS2 gene mutation, histopathologically diffuse mesangial proliferative glomerulonephritis, and clinically stage-4 chronic kidney disease. Another subject had the COL4A5 gene mutation and focal segmental glomerulosclerosis.  Both were male and had no familial renal disease, consanguinity, or hematuria. Conclusion: Children with idiopathic steroid resistant nephrotic syndrome showed NPHS2 and COL4A5 gene mutations. Histopathologically, they showed diffuse mesangial proliferative glomerulonephritis and focal segmental glomerulosclerosis