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   Table of Contents - Current issue
Coverpage
July-December 2022
Volume 7 | Issue 2
Page Nos. 45-83

Online since Tuesday, November 22, 2022

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EDITORIAL  

Infantile wheeze: Phenotypes and trajectories p. 45
A RM Luthful Kabir
DOI:10.4103/pnjb.pnjb_15_22  
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ORIGINAL ARTICLES Top

Correlation of serum phosphate with carotid intimal-medial thickness in children with chronic kidney disease p. 47
Farhana Rahman, Jakia Sultana, Mir Shahadat Hossain, Abdullah-Al Mamun, Tahmina Jesmin, Md Habibur Rahman, Ranjit Ranjon Roy, Golam Muin Uddin, Afroza Begum
DOI:10.4103/pnjb.pnjb_16_22  
Background: Hyperphosphatemia is a complication of advanced chronic kidney disease (CKD). Increased serum phosphate concentration is a significant risk factor for vascular calcification, which is an advanced form of atherosclerosis. Medial deposition of calcium and phosphorus and the resulting increase in vascular stiffness further contribute to the high prevalence of hypertension. Carotid ultrasound imaging is a useful adjunct for assessment of arterial wall thickness in patients with CKD. Objective: To assess the correlation of serum phosphate with carotid intimal-medial thickness in children with CKD. Materials and Methods: This cross sectional study was carried out in the Department of Pediatric Nephrology and Department of Radiology and Imaging, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh, from July 2018 to December 2019. All the CKD patients with stage III-V aged 2–18 years of both sexes attending the Out-patient and Inpatient Department, BSMMU were enrolled in this study. Patients were diagnosed by history, clinical examination and relevant investigations. The glomerular filtration rate (GFR) was calculated from serum creatinine using Revised Schwartz formula. All participants underwent measurement of carotid intimal- medial thickness(CIMT) by using B mode ultrasonography (Philips, Affiniti 30; USA) with 7.5MHz transducer. Results: Total 43 patients were studied (26 male, 17 female). Mean phosphorous level significantly increased with the advancement of disease. As the stage of CKD increases the mean CIMT also increases. There were moderate positive correlation between serum inorganic phosphate and CIMT in stage III CKD (r = 0.500) and stage V(ND) CKD (r = 0.511) and strong positive correlation in stage IV CKD (r = 0.719) and negligible negative correlation in stage V(D) CKD (r = - 0.057). In multiple regression analysis, serum phosphate was significant(β= 0.673, p= <0.0001) and independent risk factor for increased CIMT in addition to other independent risk factors, including serum calcium and serum PTH. Conclusion: Higher serum phosphate level was one of the significant and independent factor associated with increased CIMT in children with advanced stages of CKD.
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Efficacy and short term outcome of intravenous cyclophosphamide therapy in children with lupus nephritis: A tertiary hospital study of Bangladesh Highly accessed article p. 53
Mohammad Imnul Islam, Asif Ali, Mousumi Ahmed
DOI:10.4103/pnjb.pnjb_18_22  
Background: Renal involvement is one of the most common and severe manifestations of paediatric systemic lupus erythematosus patients. Intravenous pulse cyclophosphamide (CYC) therapy is effective for lupus nephritis patients observed in different studies. Objective: To evaluate the efficacy and short term outcome of intravenous CYC therapy in renal involvement among paediatric SLE cases. Materials and Methods: This retrospective study was conducted at the department of Paediatrics, Bangabandhu Sheikh Mujib Medical University from January 2018 to December 2021. Sixty-two pSLE patients with renal involvement who met ACR revised classification criteria and completed their follow-up were enrolled in the study. All patients received intravenous CYC monthly for six months along with initially intravenous pulse methylprednisolone followed by oral prednisolone. During the study period, patients were monitored clinically and laboratory evaluations were done at baseline, 3rd and after 6th months of follow-up. Results: Among the cases, the female:male ratio was 5.9:1 and mean age was found 12.42 ± 2.13 years. After 3 months of CYC therapy, most patients were clinically improved in haematuria, hypertension, oedema, SLEDAI score and biochemically by ESR, C3, C4, S, Creatinine, Anti-ds-DNA titers, 24-hour urine protein. These initial improvements were further enhanced after 6 months of therapy. Short term outcomes demonstrated that 50%, 38.7% and 11.3% of patients had complete, partial and no responses respectively at the last follow-up. Conclusion: Intravenous CYC therapy effectively treated lupus nephritis patients observed in this study. Around 88% of pSLE patients had complete and partial responses according to EULAR criteria. Hypertension, ant-ds-DNA titers, proteinuria and serum complements were identified as a predictor of remission.
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Renal function assessment by estimation of fractional excretion of sodium and magnesium in asphyxiated newborn p. 58
Mohammed Maruf Ul Quader, Mohammed Hanif, Shireen Afroze, Anwar Hossain Khan
DOI:10.4103/pnjb.pnjb_2_22  
Background: Fractional excretion of magnesium (FEMg) is found to be the most sensitive index among fractional excretion of solutes to detect tubular damage in glomerulonephritis, although fractional excretion of sodium (FENa) is used to detect renal tubular dysfunction in perinatal asphyxia till date. Aim and Objective: The aim of this article is to assess the diagnostic performance of FENa and FEMg as renal function test in asphyxiated newborns. Materials and Methods: This cross-sectional study was conducted on 100 asphyxiated newborns in Dhaka Shishu (Children) Hospital, Bangladesh, over 1 year. Serum and urine creatinine, sodium, and magnesium were assessed in each patient; FENa and FEMg were calculated. Serum creatinine was regarded as gold standard for renal function. Results: FENa had 63.64% sensitivity, 100% specificity, 100% positive predictive value, 90.70% negative predictive value, and 92% accuracy. FEMg had 100% sensitivity, 66.67% specificity, 45.83% positive predictive value, 100% negative predictive value, and 74% accuracy. Receiver-operating characteristic curve revealed that area under the curve for FENa was 0.990 and for FEMg was 0.833. So, area under the curve for FENa was more than that of FEMg. Conclusion: FENa is better than FEMg in the assessment of renal function of asphyxiated newborns.
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Concomitant infection of typhoid and rickettsia with dengue fever in acute febrile patient p. 63
Ferdousi Hasnat, Farahnaz Shoma, Syeda Mehnaj, Shamima Farzana, Mohammed Nurullah, Rifat T Anne
DOI:10.4103/pnjb.pnjb_5_22  
Rationale of the study and objectives: Dengue fever is one of the major causes of acute febrile illness. Other causes of febrile illness may be associated with dengue. This cross-sectional observational study was done to find out other coinfections such as typhoid, malaria, and rickettsia with dengue in an acute febrile patient and aimed to reduce the morbidity and mortality of patients. Materials and Methods: This study was conducted at Kurmitola General Hospital from June 2019 to November 2019. A total of 244 patients were included in this study. All patients were admitted to the hospital with fever. All investigations that aimed to diagnose the acute febrile illness were done. Results: Among 244 patients, 62.29% patients were tested positive for dengue, 26.22% for rickettsia, and 11.47% for typhoid. Dengue monoinfection was 44.26%, and coinfection was 18.03%. Malarial case was not found. The mean age of children was 6.4 years. Majority (75%) came from rural areas, and 64.75% of studied children were male. Fever, myalgia, arthralgia, and gastrointestinal symptoms such as abdominal pain, vomiting, loose motion were the main symptoms, and jaundice, hepatomegaly, and splenomegaly were the main signs both in monoinfection and coinfection. Leukopenia, thrombocytopenia, raised serum alanine transaminase, and elevated hematocrit were found in monoinfection as well as in coinfection. Conclusion: Symptoms of monoinfection and coinfection were overlapping, but treatment was rather different and difficult. So for proper treatment, early detection was necessary for reducing death rate and complication in acute febrile illness.
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REVIEW ARTICLE Top

Cystinosis and kidney: What do we know about it? p. 67
Md Abdul Qader, Syed Saimul Huque, Mohammed Hanif
DOI:10.4103/pnjb.pnjb_6_22  
Cystinosis is the most common cause of renal Fanconi syndrome caused by defective CTNS gene resulting accumulation of cystine in the lysosomes of multiple organs of the body. Although Cystinosis can affect multiple organs, but kidney is the primary organ affected. After kidney, it affects eyes and can cause retinopathy and blindness. Patients can have hypothyroidism, diabetes mellitus and gonadal dysfunction in males secondary to cystine crystal deposition in thyroid, pancreas, and gonads respectively. The most common form is the infantile nephropathic cystinosis may presents with features of Fanconi syndrome. Other rare forms include juvenile form which have mild Fanconi syndrome and adult-onset ocular form of cystinosis. Diagnosis of cystinosis can be confirmed by a slit lamp examination to find corneal cystine crystal deposition, genetic tests for CTNS gene and leukocyte cystine level both for diagnosis and monitoring of the therapy. Children with cystinosis need specific therapy to delay the progression of the disease with the advancement of clinical research, newer therapies are under clinical trial and hematopoietic stem cell transplantation shows promising results including improvement of kidney structure and Fanconi syndrome.
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CASE REPORTS Top

Acute kidney injury following rhabdomyolysis due to multiple wasp stings p. 73
Tahmina Jesmin, Rina Biswas, Abdullah-Al Mamun, Mst Shanjida Sharmim, Syed Saimul Huque, Afroza Begum, Ranjit Ranjan Roy
DOI:10.4103/pnjb.pnjb_17_22  
Wasp stings are a common form of envenomation in tropical countries. Multiple wasp stings may cause fatal complications such as anaphylactic reactions, intravascular hemolysis, rhabdomyolysis, acute kidney injury (AKI), or even death also. Here we report a 9-year-old boy who was admitted to a tertiary care hospital in Bangladesh with complaints of multiple wasp stings all over the body 15 days back. He had oliguria and generalized edema for 7 days. The urine test was negative for red blood cells and his renal function was gradually deteriorating. His serum creatinine phosphokinase was gradually rising. Intermittent hemodialysis through a central venous catheter was started immediately. Renal biopsy revealed myoglobin cast nephropathy. On the basis of history, clinical manifestations, and laboratory investigation, he was diagnosed as AKI following rhabdomyolysis due to multiple wasp stings. Clinical and biochemical picture started to improve including urine output was normalized on seventh day of post-admission. Timely initiation of dialysis and supporting therapy can improve renal survival in case of AKI due to wasp stings.
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Joubert syndrome presented as chronic kidney disease: A rare event p. 78
Abdullah Al Mamun, Samina Masud Santa, Tahmina Jesmin, Mst Shanjida Sharmim, Syed Saimul Huque, Ranjit R Roy, Afroza Begum
DOI:10.4103/pnjb.pnjb_19_22  
Joubert syndrome (JS) is a rare disorder affecting the multisystem of the body. It involves defects in the cerebellar vermis, abnormal eye movement, renal aplasia/hypoplasia, and polydactyle. Here is a report of a 9-year-old boy suffering from JS presented as chronic kidney disease.
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A case report of methylmalonic acidemia associated with CYBT gene mutation p. 81
Rumana Islam, Mizanur Rahman, Kanij Fatema
DOI:10.4103/pnjb.pnjb_12_22  
Mutation in mitochondrial cytochrome b (CYTB) gene is associated with severely impaired complex III activity and oxidative phosphorylation and commonly presents with mitochondrial myopathy, exercise intolerance, and often multisystem involvement. We report a rare case of Methylmalonic acidemia who was finally found to have a pathogenic novel missense mutation (m.14766C>T, p.Thr7IIe) in the CYTB gene.
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