Abstract Secondary hypertension (HTN) should be a primary suspicion in people of young age. Secondary HTN must be appropriately diagnosed and treated. Renovascular HTN accounts for approximately 5%–10% of pediatric HTN. It can occur as an isolated entity or as a hypoplasia of the renal artery combined with stenosis. In particular, HTN remains refractory despite anti-hypertensive therapy. Here, we have discussed four cases of pediatric renal artery stenosis (RAS) presenting with HTN. Angiogram of both renal arteries in all the cases was performed, and stents were placed successfully in three cases, and the blood pressure (BP) was normalized. Another patient had undergone total unilateral nephrectomy, which resulted in normalization of BP with only one anti-hypertensive agent. The aim of this case series is to describe diagnosis and management of secondary HTN due to RAS in young patients.

Rickets is a major public health concern globally. It results from impaired mineralization of the growing bone at its growth plate associated with abnormal calcium and phosphate metabolism. Among different classifications, nutritional deficiency is the commonest variety, and the genetic form of rickets has also been identified frequently in this genomic era. Treatment and management of rickets should be targeted as per type along with the pathogenesis of development of this condition. Management differs for each form of rickets and therapy requires distinct treatment, monitoring and follow-up schedule according to the pattern of response to therapy. The objective of this review is to summarize the treatment and management of different types of rickets in the light of recent guidelines and relevant literature.

Renal size is an important parameter used for the clinical evaluation of renal growth and renal diseases in children. Renal length is the most useful parameter for measuring renal size. Therefore, establishing the normal value of renal length in children is valuable, as it can serve as a baseline for the diagnosis of renal diseases and possible interventions. The objective of our study was to assess renal length in Bangladeshi children by ultrasonography. This cross-sectional study was carried out in the Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh, from January 2019 to June 2020. In total, 369 apparently healthy children aged 1 month to 18 years were included in this study. Renal length was measured by ultrasonography by same experienced radiologist. The mean renal length ± standard deviation was calculated for each age group. The correlation of renal length with age and anthropometric parameters was tested using Pearson's correlation test. No statistical difference was found in renal length between the two sexes. The left kidney was significantly longer than the right kidney (P <0.001). There were strong positive correlations of renal length with age and anthropometric parameters. The best positive correlation was found between renal length and height (r = 0.95 and 0.94 for the right and left kidneys, respectively). Linear regression equations were obtained to predict the renal length from age and anthropometric parameters. Renal length has a strong positive correlation with body length/height.

Abstract Background: Nephrotic syndrome (NS) is a common disorder in the pediatric age group. The majority of them developed relapse due to infection. About 50%–70% relapses occur due to respiratory tract infection. Therefore, pneumococcal and influenza vaccination may prevent the number of relapses. Materials and Methods: This prospective, case–control study was carried out in the department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, from November 2016 to February 2018. A total of 120 patients aged 2–18 years old with an initial episode of idiopathic NS (INS) were included in this study. They were divided in two groups according to age and administration of the Haemophilus influenzae vaccine: Group A and Group B. Group A included 60 children of younger age group (2–8 years). All of them received Haemophilus influenzae vaccine through the Expanded Program on Immunization (EPI) schedule. They were further subdivided into two subgroups: Group A1 and Group A2. Group A1 included 30 children who received pneumococcal and influenza vaccines. Group A2 also included 30 children who refused vaccination. Similarly, Group B included 60 children, and they belonged to the older age group (>8–18 years). They did not receive Haemophilus influenzae vaccine through the EPI schedule. They were further subdivided into two subgroups: Group B1 and Group B2. Group B1 included 30 children who received pneumococcal and influenza vaccine. Group B2 also included 30 children who refused vaccination. All of them were followed up for 1 year to compare the clinical effects. Results: A total of 120 patients were studied. Among them, the number of patients without relapses was high in the younger age vaccinated group than non-vaccinated group (36.7% vs. 10%; P = 0.003). In addition to this, older age vaccinated group developed less relapses compared to the non-vaccinated group ( P < 0.05). This indicates that vaccination has a beneficial role in preventing relapse in children with INS. Conclusion: Vaccination against pneumococcus and influenza lowers relapses in children with INS.

Abstract Beyond its classical role in calcium metabolism and bone health, vitamin D serves numerous non-classical functions crucial for overall well-being. It modulates immune function, influencing both innate and adaptive immunity, thereby aiding in the prevention of infections and autoimmune diseases. Vitamin D also plays a role in regulating cellular growth and differentiation, impacting processes such as cell proliferation and apoptosis, which are essential for maintaining tissue homeostasis and preventing cancer development. Additionally, emerging evidence suggests its involvement in cardiovascular health, insulin secretion, and neurological function. These non-classical functions highlight the importance of adequate vitamin D levels for overall health beyond bone strength.

Background: Steroid-sensitive nephrotic syndrome (SSNS) is frequently associated with alteration of calcium and vitamin D metabolism, including hypocalcemia, reduced serum vitamin D metabolites, and elevated levels of parathyroid hormone (PTH). These alterations occur usually due to intestinal malabsorption of calcium as well as excessive urinary losses of various vitamin D metabolites and their binding proteins which, in turn, lead to a decrease in bone mineral density. So, early identification and management of the abnormal levels of calcium, vitamin D, and PTH can ameliorate growth retardation. Objective: Our aim is to compare the level of calcium, vitamin D, and PTH in different subgroups of SSNS. Materials and Methods: This cross-sectional study was carried out in the Department of Paediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh, from January 2018 to June 2019. A total of 45 patients with previously diagnosed SSNS, aged 2–18 years of both sexes, were included in this study. The children were divided into three groups of 15 each. Group I comprised of 15 patients who presented with infrequent relapse nephrotic syndrome(IRNS), Group II comprised 15 patients who hadfrequent relapse nephrotic syndrome or steroid dependent nephrotic syndrome (FRNS/SDNS), and Group III comprised of 15 patients who were in remission for last 3 months without any medication. Then serum calcium, vitamin D, and PTH levels were compared among the study population. Results: A total of 45 patients were studied. Serum calcium was significantly lower in groups I and II compared to group III ( P < 0.05). Mean serum PTH levels were 34.02 ± 15.33, 50.52 ± 19.22, and 40.33 ± 14.58 pg/mL in groups I, II, and III, respectively. Among the study group, vitamin D levels were deficient in 26 (58%), insufficient in 14 (31%), and sufficient in 5 (11%) children. Among the subgroups, mean serum vitamin D levels were 8.98 ± 1.96 ng/mL in patients with IRNS, 4.27 ± 1.37 ng/mL in FRNS/SDNS group, and 18.49 ± 7.34 ng/mL in patients who were in remission for more than 3 months. Serum vitamin D levels were low in groups I and II compared to group III, with statistically significance ( P < 0.001). Conclusion: This study finding concluded that mean serum vitamin D levels were low in all the subgroups of SSNS children which were statistically significant. Specifically, all children with frequent relapse and steroid dependent nephrotic syndrome were vitamin D deficient.

Rickets is an ancient disease and for centuries different conceptions were adopted regarding its type, causes, and treatment options. The discovery of vitamin D transformed the landscape of rickets and was followed by the discovery of several new therapies that improved treatment out-comes. In parallel, the development of rickets detection technology and new workup for vitamin D improved rickets management. Remarkably a century later, vitamin D remains the cornerstone of rickets treatment. In this review, we aim to highlight the lessons learned from the limitations of vitamin D knowledge over the past century. Finally, progress continues in the field of bu-rosumab, a human monoclonal antibody to FGF23, which is approved for the treatment of X-linked hypophosphatemia among children 1 year and older, perhaps the ultimate frontier in rickets management.

Abstract Background: Acute kidney injury (AKI) has been associated with high morbidity and mortality in children, including neonates. Early diagnosis would be of great value for treatment and prevention. Objective: The goal of this study was to assess the role of cystatin C to detect AKI early in children who were at risk. Materials and Methods: This prospective analytical study was conducted at Department of Paediatric Nephrology and performed on critically and non-critically ill patients admitted in the Department of General Paediatrics and Allied, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. The study period ranged from May 2018 to July 2019. Patients aged 4 days–17 years were included, who were at risk for AKI. The risk was considered when patients presented with symptoms of hypovolemia, shock, sepsis, and used nephrotoxic agents. Anthropometry was measured in all patients with the standard method. Then, blood samples were collected to assess creatinine at 0, 48, and 72 h for the contrast group and on the fifth day instead of 72 hr for the non-contrast group, to assess cystatin C at 0 and 48 h. For diagnosis of AKI, Kidney Disease Improving Global Outcomes criteria were used for all patients. Results: A total of 52 patients at risk of AKI were enrolled in this study, among which 42 were in the older age group and 10 were neonates. Twenty patients (38%) developed AKI. The mean age of the older age group was 11.3 ± 3.7 years with male preponderance, and mean age in neonates was 10.8 ± 5.4 days with female preponderance. The major risk factor for AKI was nephrotoxic drugs, followed by hypovolemia in the older age group. In neonates, the primary risk factor for AKI was hypovolemia, followed by sepsis and asphyxia. Cystatin C level increased in serum 1–2 days before creatinine in patients with AKI. Only 6 (30%) patients with AKI were detected by creatinine levels within 48 h and all AKI patients by cystatin C within 48 h. The ability of serum cystatin C to predict AKI at 48 h was analyzed, which revealed the area under the curve (AUC) was 0.93 with 95% confidence interval (CI) = 0.864–0.995. Using a cut-off value of 1.35 mg/L for cystatin C, the sensitivity and specificity were 95% and 84% (95% CI = 0.779–0.997 and 0.737–0.873), respectively. The positive predictive value and negative predictive value, accuracy, positive likelihood ratio, and negative likelihood ratio were 79%, 96%, 88.7%, 6.08, and 0.06, respectively. The association between age, gender, height, weight, body mass index, and risk factors and cystatin C was explored, revealing no effects on cystatin C. Conclusion: Serum cystatin C is an effective and early marker for detection of AKI in children at risk.

Background: Spontaneous bacterial peritonitis is a serious complication of childhood nephrotic syndrome (NS). A precise knowledge of organism causing peritonitis is important primarily to treat infection and decrease morbidity, prevent antibiotic resistance and finally to reduce mortality. Objectives were to observe the clinical and bacteriological profile and antibiotic sensitivity pattern of peritonitis in childhood NS. Methods: This cross-sectional observational study was conducted in pediatric nephrology department of Bangabandhu Sheikh Mujib medical university (BSMMU). The 44 diagnosed patient of spontaneous bacterial peritonitis among childhood idiopathic NS were enrolled as cases. The peritoneal fluid was obtained following standard procedure and examined for gross appearance, cytogical and biochemical analysis, microscopic examination with gram stain and peritoneal fluid culture along with antibiotic sensitivity. Results: All patients had complaints of abdominal pain followed by fever, vomiting, lethargy and anorexia. All cases had edema and ascites followed by abdominal tenderness, abdominal wall rigidity, rebound tenderness and cellulitis in areas other than abdominal wall. Peritoneal fluid showed neutrophilic pleocytosis and exudative fluid. We observed 27.27% gram + cocci, 6.82% gram-bacilli in gram stain examination. Here, only 6.9% cases showed positive growth including 2.3% E. coli, 2.3% Pseudomonas, 2.3% Acinetobacter and remaining 93.1% cases showed no growth. We also described the antibiotic sensitivity pattern in this study. Conclusions: In our study, patients predominantly had abdominal pain, fever, edema, ascites and abdominal tenderness. We found only 6.9% cases showed positive growth which included E. coli, Pseudomonas and Acinetobacter and demonstrated the antibiotic sensitivity and resistance pattern for these organisms.

BackgroundOcular disorders can arise in the advanced stages of chronic kidney disease (CKD) for various reasons, including uraemia, biochemical abnormalities, hypertension and inadequate haemodialysis treatment.MethodsWe conducted a cross-sectional study at the Pediatric Nephrology Department, both inpatient and outpatient, of from January 2020 to July 2021. The study aimed to identify and compare ophthalmological changes among children at different stages of CKD to assess potential visual threats. A total of 92 children with advanced-stage CKD, aged 5–18 years, meeting the inclusion and exclusion criteria, were included in the study. Comprehensive assessments, including medical history, physical examinations, relevant tests and detailed ophthalmological examinations, were conducted.ResultsThe mean age of the participants was 12.1±3.68 years. Most of the children were boys (66%). Twenty-nine patients exhibited impaired visual acuity, children with (6/60–6/24) scores in Snellen’s chart Lid oedema and conjunctival pallor were observed in 20.7% and 60.9% of cases, respectively, which were found to statistically significant with advancing CKD stages (p<0.001). Dry eyes were found in 9.8% of CKD stage V patients receiving dialysis (VD) (p=0.003). One patient had a posterior subcapsular cataract, and 7.6% had conjunctival congestion. Patients with conjunctival congestion and hypertensive retinopathy had significantly higher levels of serum phosphate and calcium phosphate product (p<0.001). Hypertensive retinopathy was present in 16.3% of cases, with a significantly higher proportion in the haemodialysis group (93%). Haemodialysis patients exhibited higher blood pressure, lower haemoglobin levels, and a more severe reduction in estimated glomerular filtration rate (p<0.001).ConclusionThis study highlights the significant ocular complications associated with CKD, underscoring the need for regular ophthalmological screenings.

Background: Prednisolone is the 1st choice of drug in Idiopathic Nephrotic Syndrome (INS). Deflazacort (DFZ) is a new step in this regard. Aim of this study was to compare the efficacy of prednisolone and DFZ in children with INS. Materials and methods: 76 children of 2-16 years with INS were enrolled in a Randomized Controlled Trial (RCT). Patients were randomized to either group-A (DFZ) or group-B (Prednisolone) and 38 children were allocated in each groups. After giving treatment with both drugs, these patients were followed up at 3 months interval for 2 times to compare the clinical effects. Due to lost follow up finally 65 patients were analyzed. Data was documented on pre-structured data sheet and analyzed by SPSS version 22.0. Chi square test for categorical data and unpaired ttest for continuous data were done. A probability (p) value < 0.05 was considered statistically significant. Results: Mean time to get remission was 5.32±1.28 days in DFZ group and 8.00±2.55 days in prednisolone group. It was statistical significant (p= <0.001). Mean duration of remission was 171.29±19.27 days and 146.66±54.61 days in Group A and Group B respectively and was statistically significant (p= 0.020). Total number of relapse by treatment with DFZ is less in comparison of prednisolone. Conclusion: DFZ was more effective as shorter time was required to induce remission and achieved remission was maintained for longer durationin INS. Number of relapse by using DFZ was less than prednisolone. Number with no relapse were more in DFZ than prednisolone on follow up time. Chatt Maa Shi Hosp Med Coll J; Vol.22 (2); July 2023; Page 18-21

Abstract Viral infection is considered an important trigger factor for developing or flare-up of lupus manifestations. Dengue is a viral infectious disease, which may trigger the antiphospholipid antibody-mediated progression to thrombosis in lupus patients by dysfunctional immune response. Here, we report a case of a 10-year-old boy with lupus nephritis who developed dengue, followed by deep vein thrombosis, and found an abnormal ratio of lupus anticoagulant (LA) screening assay with low phospholipid content (LA1) and LA confirmation assay with high phospholipid content (LA2); LA1/LA2 ratio of 1.3, indicating the presence of LA and diagnosed as antiphospholipid syndrome (APS). He was promptly treated with an infusion of unfractionated heparin, followed by warfarin, and was also given aspirin as prophylaxis for antiphospholipid antibody syndrome and the patient responded well to the treatment. The co-occurrence of dengue and lupus can lead to the triggering of autoimmunity and may cause secondary APS, and thromboembolic events. Therefore, early diagnosis, screening for thromboembolism, and prompt management are essential to improve the patient’s prognosis.

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.

Abstract Background: The clinical spectrum of coronavirus disease 2019 (COVID-19) ranges from asymptomatic course to severe illness. Children have accounted 1%–5% of diagnosed cases so far. This study aimed to observe the clinical features and outcomes of renal disease in children with COVID-19. Materials and Methods: This retrospective study was conducted in the Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh, from November 2021 to April 2022. The data were collected from 24 COVID-19-positive children under 18 years old with preexisting renal disease admitted in BSMMU from March 2020 to September 2021. Results: The mean age of the participants was 8.7 (±4.4) years. Among them, preexisting renal diseases were mainly nephrotic syndrome (50%), chronic kidney diseases (29%), and lupus nephritis (8%). Among the participants, 58% of patients became reverse transcription- polymerase chain reaction negative within 14 days, and the mean duration of hospital stay was 14.5 (±5.9) days. Most frequently presented clinical features were fever (75%), cough (71%), respiratory distress (54%), proteinuria (71%), hematuria (21%), diarrhea and acute kidney injury (17%), rapidly progressive glomerulonephritis (4%), heart failure (33%), and decreased saturation of peripheral oxygen ≤90% in 58% of cases. Mean white blood cell count was 8773.9 (±5178.1)/mm 3 , raised serum creatinine 62.5%. Pneumonia in chest X-ray was present in 50% of cases. Children getting more immunosuppressive drugs, especially those who got rituximab, had milder symptoms. Overall case-fatality rate was 25% with the highest rate in chronic kidney disease (CKD) patients. Conclusion: Children with preexisting renal disease got affected slightly at a higher percentage than the normal child, and those who got immunosuppressive therapy, especially rituximab, had milder symptoms. Children with CKD had fatal outcomes.

Patients with systemic lupus erythematosus (SLE) frequently have elevated serum levels of liver enzymes. Hepatic abnormalities are suspected to be caused by hepatotoxic drugs, viral hepatitis, autoimmune hepatitis, fatty liver, and disease-related hepatitis. In the presenting cases, a drug-induced liver injury was diagnosed, which quickly returned to normal after the suspected causative medication was discontinued. Elevated liver enzymes are a major concern in SLE patients and should be thoroughly investigated.