Paediatric Nephrology Journal of Bangladesh

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Background: Most patients with idiopathic steroid-sensitive nephrotic syndrome have frequent relapses. So, it is very important to find out such children who are prone to develop frequent relapses. The objective of this study is to find out the predictors of relapse. Materials and Methods: This prospective longitudinal study was carried out in the Department of Paediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh, from September 2015 to August 2016. A total of 110 patients of idiopathic nephrotic syndrome (INS) with the initial attack, aged 1–18 years, were enrolled in this study. All patients were treated with prednisolone 60 mg/m²/day, single morning dose for 6 weeks, followed by 40 mg/m² every alternate day for another 6 weeks. Among 110 patients, seven patients were lost during follow-up, and six patients were excluded from this study due to unresponsive to oral prednisolone 60 mg/m²/day within 4 weeks. The remaining 97 were analyzed and followed up. Results: Among them, 59 (60.9%) were males, 38 (39.1%) were females, with a male:female ratio of 1.5:1. Twenty-nine (29.9%) children had no relapse, 24 (24.7%) had infrequent relapse, and 44 (45.3%) had frequent relapse. Children responding between 2 and 4 weeks after the start of treatment had a more chance of relapse (P = 0.002) than those who responded less than 1 week. Children with INS who had respiratory tract infection at onset had 4.4 times more chance of relapse. Children with low serum albumin (<15 g/L) at onset had 2.1 times more chance of relapse. Conclusions: There was significant correlation of INS associated with male sex, delayed response to steroid treatment, respiratory tract infection at onset, and severe hypoalbuminemia with more relapse.

Background: Patients with nephrotic syndrome (NS) have altered serum immunoglobulin E (IgE) levels during relapse and remission, while presentation may be typical or atypical. Objective: To evaluate serum IgE level in childhood NS with the atypical and typical presentation. Materials and Methods: A total of 66 children with NS aged between 1 and 12 years were enrolled in this cross-sectional study. Among them, 33 children were with atypical presentations, and an equal number of children were with the typical presentations. Serum IgE level was measured in all cases. Result: The mean age of patients was 5.9 ± 3.1 years with a male-to-female ratio of 1.4:1. The mean serum IgE level was 1063.2 ± 1042.6 IU/mL in NS atypical presentation and 1099.8 ± 842.1 IU/mL in NS typical presentation, both of which were higher than the normal range for age but statistically not significant (P = 0.877). Conclusion: Elevated level of serum IgE is a feature of childhood NS with atypical and typical presentations.

Adrenoleukodystrophy (ALD) is the most common peroxisomal disorder associated with mutations in the ABCD1 gene. Seizures, progressive neurologic deficits, hyperactivity, or learning problems are the most common symptoms in children with X-linked adrenoleukodystrophy (X-ALD). Visual impairment usually appears later in the course of the disease. We present a 5-year-old boy who came to us with visual loss only. His ophthalmologic evaluation revealed no abnormalities and brain MRI findings suggested X-ALD. This case report aimed to show that visual loss may be the initial presentation in ALD. Therefore, a high level of suspicion should be kept in patients with visual impairment as X-ALD affects the cortical regions connected to vision.

Background: Nephrotic syndrome (NS) is the most common glomerular disease in childhood. With corticosteroids, 80%–85% of children achieve remission, although 10%–15% remain refractory or develop steroid resistance in NS. Steroid-resistant nephrotic syndrome (SRNS) management is difficult due to its varied etiology, frequent lack of remission induced by immunosuppressive treatment, and consequences including drug toxicity, infections, thrombosis, end-stage kidney disease, and recurrence following renal transplantation. Renal histology and genetic mutation play an important role in the management of SRNS. Calcineurin inhibitor (CNI) is the first-line drug of SRNS. In CNI-resistant and multidrug-resistant cases, rituximab and newer drugs are a good option. There is a 35%–50% chance of developing chronic kidney disease (CKD) in SRNS, and supportive measures are required in that case. Few studies have described clinical characteristics, renal biopsy patterns, and treatment outcomes in Bangladeshi children with SRNS. This study evaluated the clinical characteristics at diagnosis, histological pattern, immunosuppressive therapy, and patient outcomes after immunosuppressive medication. Materials and Methods: All the records of children (1–18 years old) with idiopathic NS who had been followed up in the Pediatric Nephrology Department, Bangabandhu Sheikh Mujib Medical University (BSMMU), from January 2019 to December 2021 were reviewed retrospectively. Results: Among 606 NS cases during the study period, 90 cases (14.85%) were SRNS. Males comprised 70% of the population, whereas females made up 30%. The mean age at the time of diagnosis was 76.21 ± 51.55 months. Hematuria was found in 35.5% of cases, hypertension in 40.8%, and impaired renal function in 34.2% at the time of presentation. Children had primary resistance in 65.7% of cases. Biopsy report was found in 65 cases. The most common histopathological pattern were minimal change disease (MCD) (43.08%) and focal segmental glomerulosclerosis (FSGS) (38.46%). Complete remission was achieved in 66% of cases, and 14% developed CKD. Renal impairment and mesangioproliferative glomerulonephritis (MesPGN) increased the risk for CKD. Patients who received CNI or CNI and mycophenolate mofetil had a higher rate of complete remission. Conclusion: Hypertension and hematuria were prevalent in SRNS children. Most lesions were MCD. CNI or combination therapy remissioned more children. Renal impairment at presentation and non-MCD lesion increased CKD risk (FSGS and MesPGN). Long-term follow-up is needed to confirm poorer renal survival.

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.

Intolerance to lactose-containing foods is not so uncommon. Clinical symptoms of lactose intolerance in children include abdominal pain, flatulence, and diarrhea after ingesting milk or milk-containing products. These symptoms have been attributed to lactose malabsorption, which results from low levels of small intestinal lactase. Reduced lactase level may be due to mucosal injury or, much more commonly, reduced genetic expression of the enzyme lactase-phlorizin hydrolase. However, lactose intolerance cannot be cured. Still, it can be adequately managed by using lactose-reduced milk and milk products and over-the-counter supplements of lactase, lactose-depleted products, and other essential milk nutrients such as calcium. This article will review the clinical manifestations, diagnosis, and management of lactose intolerance in children.

An 11-month-old Bangladeshi female baby, the first issue of her non-consanguineous parents, presented with dysmorphic facies, prognathism, lack of subcutaneous fat in all body parts, and apparent muscular hypertrophy, excessive body hair (sparing the face, axilla, and pubic area), enlarge hand-feet, clitoromegaly and hepatomegaly, diagnosed as Berardinelli-Seip congenital lipoatrophy.

Online since 24^th March 2021