Introduction: Disorder of sex development (DSD) is defined as a condition where the development of gonadal, chromosomal or anatomic sex is atypical. Objective: To study the clinical and laboratory profile of patients with disorders of sex development (DSD) and classify them. Methods: A retrospective study was conducted in BSMMU from 1st May 2016 to 31st December 2021 including all patients diagnosed with DSD. Data were obtained by reviewing the medical records of the patients. Results: A total 67 patients were diagnosed as DSD. Among them 30 patients (44.8%) had 46,XX DSD, 22 patients (32.8%) had 46,XY DSD, 13 patients (19.4%) had sex chromosome DSD and 2 patients (2.9%) had ovotesticular DSD. Majority (60%) of the patients with 46,XX DSD were diagnosed as congenital adrenal hyperplasia (CAH) followed by adrenal tumor (16.7%) and patients with partial trisomy of chromosome 9q (16.7%). Among 22 patients with 46,XY DSD, eight patients (36.3%) had androgen biosynthetic defect, 6 cases (27.2%) were under virilized CAH and 2 patients (9%) had androgen insensitivity syndrome. Majority of sex chromosome DSD were turner syndrome (69.2%). Only 2 patient had mixed gonadal dysgenesis. Thirteen percent of the children with 46, XX DSD were reared as male and 27% of the 46, XY DSD cases were reared as female. Conclusion: Congenital adrenal hyperplasia is the most common cause of DSD. Majority of 46 XY, DSD is due to androgen bio synthetic defect while CAH is the most common cause of 46XX, DSD. Rare chromosomal disorders are also found among DSD cases. Bangladesh J Child Health 2023; Vol 47 (2) : 103-108
Beta thalassemia results from absent or reduced production of beta globin gene leading to excess of alpha chain which causes ineffective erythropoiesis and marked anemia. b-thalassemia major patients usually present early within 2 years of life mostly in 2nd six months of first year of life. Only curative treatment till date is bone marrow transplantation. However, it is not feasible for all because of rarity of matched donar and obviously for its high cost and unavailability of services particularly in a developing country like Bangladesh. Hence regular blood transfusion remain the ultimate choice of their survival, growth and development with the cost of iron overload in the resource constraints country like ours. Recently HbF inducing drugs are showing promising result in the management of thalassemia children by inducing fetal hemoglobin. However, data are scarse from Bangladesh.Here we are reporting a case of betathalassemia who was diagnosed at 18 months of age and treated with only blood transfusion upto 2 years then hydroxyurea was given for 12 months with no response and then she was kept on regular blood transfusion with iron chelation up to 6 years with regression splenic size to normal and to maintain normal growth and development. After then she was given hydronix plus thalidomide in combination and responded well and remained transfusion free for last 3 years. Bangladesh J Child Health 2023; Vol 47 (2) : 117-120
Background: Mitochondrial diseases are rare group of heterogeneous, genetically determined disorders that affect multiple organs with variable severity due to the dysfunction of mitochondria. They usually present with acute or chronic features with intermittent decompensation. The common features are neuro-regression, hypotonia, failure to thrive, stroke, seizures, myopathy, cardiomyopathy, deafness, blindness, movement disorder, lactic acidosis etc. The aim of this study was to describe the clinical, neuroimaging and genetic analysis of mitochondrial diseases. Methodology: This retrospective study was done among sixteen admitted patients in the Department of Pediatric Neurology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from January 2016 to December 2023. Results: Total sixteen patients were included in the study. The age of the patients ranged from 3 months-8 years; 11 patients were male. Developmental delay and regression, seizure, hypotonia, dystonia, ataxia were the key clinical manifestations. About 12.5% had persistent hypoglycemia. 6.13% presented with periodic paroxysms of dyskinesia and weakness. In MRI of brain, predominant feature was bilateral basal ganglia involvement. Genetic analysis showed variable presentation. Here, 12.5% was diagnosed with Leigh syndrome, 12.5% had primary carnitine deficiency, 12.5% had SURF-1 mutation causing mitochondrial complex IV deficiency, 6.13% had mitochondrial short chain enoyl co-A hydratase deficiency,12.5% had mitochondrial DNA depletion syndrome and 6.13% had progressive external ophthalmoplegia with mitochondrial DNA deletions. Conclusion: Multisystem heterogenous involvement gives a clue to the suspicion of mitochondrial disease in pediatric population. Bangladesh J Child Health 2023; Vol 47 (2) : 65-71
Cow’s milk allergy is a common problem in childhood that needs frequent visits to a pediatrician. The objective of this review is to provide the general pediatrician with an overview of cow’s milk allergy in children discussing the etiology, patient evaluation, and management. This review provides an approach to a child with cow’s milk allergy based on the best available evidence from electronic literature searches. Cow’s milk allergy may be IgE-mediated, non-IgE-mediated, and mixed. IgE-mediated reactions occur immediately after ingestion of milk. Skin manifestations like erythema, hives, pruritus, angioedema, and flaring of eczematous lesions are common manifestations of IgEmediated food allergy. Non-IgE-mediated cow’s milk allergies are proctocolitis, enterocolitis, enteropathy, and contact dermatitis. Cow’s milk-induced allergic proctocolitis is the common cause of rectal bleeding from 1 day to 6 months of age. The key to the diagnosis of food allergy depends on obtaining a good history and physical examination. Quantitative measurements of serum cow’s milk specific IgE and skin prick test are useful tests. Strict avoidance of the offending food is the mainstay of management. Bangladesh J Child Health 2023; Vol 47 (2) : 59-64
Now-a-Days, attitudes of paediatric urologists have been changed from surgical to observational regarding definite management of congenital ureteropelvic junction obstruction (UPJO), because many cases of UPJO goes away on its own before baby is born. In the last decade, this type of management trend has become progressively increased despite the lack of precise predictors of outcome. But these patients should be brought into regular follow-up by physical examinations, ultrasonography and nuclear imaging to observe resolution events. In some clinical scenario surgical intervention is inevitable such as solitary kidney or child with infection. Now-a-days, it is a good practice by the modern pediatric urologists frequently consults with the guardians about advantage and disadvantage of observational and surgical management of antenatally detected hydronephrosis due to UPJO of their babies and their future outcome. As a result guardians feel comfortable in decision making. Bangladesh J Child Health 2022; Vol 47 (2) : 54-58
Background: To assess neonatal mortality, many validated scores have been developed. The modified sick neonatal score (MSNS) is an easy, less complex suitable for both term and preterm neonates. This study was conducted to predict mortality upon admission to a NICU using the MSNS score. Methods: An observational study was conducted in the Department of Neonatology, BSMMU, Dhaka, over a period of one year. Parameters for MSNS score were collected from data sheet. Outcome expressed as survived or expired. All data were analyzed using SPSS 20. To determine the cutoff value for predicting mortality, a receiver operating curve was generated. The cutoff score’s sensitivity, specificity, positive predictive value, and negative predictive value were calculated. Results: Among the enrolled 114 neonates, mean gestational age and mean birth weight were 34.22±3.10 weeks and 1967.87±752.33g respectively. About 75.4% neonates were <2500 grams and 70.2% of babies were preterm. During the study period, two third (69.3%) baby survived. The mean MSNS among survived neonate was 11.77± 2.29 and in expired neonates was 9.66±2.32 which was statistically significant (p-<0.01). The area under the ROC curve was 0.740 (95% CI: 0.645- 0.835). The optimal cutoff value obtained to predict mortality was 11.50. With this cutoff score the sensitivity and specificity were 80% and 58%. Positive predictive value and negative predictive value were 46% and 87% respectively. Conclusions: MSNS tools can be used to predict early mortality with early referrals and prompt treatment to reduce neonatal mortality. Bangladesh J Child Health 2023; Vol 47 (2) : 72-77
Background: Although, the primary organ of involvement are lungs in Coronavirus disease 2019 (COVID-19), however liver, and gastrointestinal (GI) involvement is being increasingly reported in the emerging data from various centers across the world. Objective: To observe the gastrointestinal symptoms and liver dysfunctions in children with Covid 19 infection. Methods: This retrospective observational study was conducted in children with Covid 19 RT PCR positive, aged between 0 and 15 years admitted from March 2020 to March 2022. Data of 103 consecutive children were collected retrospectively. We analyzed the clinical and laboratory data of 101 children after exclusion 2 children as associated acute HAV infection and liver cirrhosis using Statistical Package for Social Science (SPSS) for Windows version 29. Results: A total of 101 children with Covid 19 were analyzed in this study. Of these 101 children, 61 were male. The mean age was 5.4 years (SD 4.9). 44 children had gastrointestinal presentation in the form of vomiting, diarrhea, abdominal pain. Most common gastrointestinal presentation were nausea and vomiting (34.7%), then diarrhea (21.8%), abdominal pain (15.8%), hepatosplenomegaly (3%), ascites and jaundice 1% in each respectively. Total 17(16.8%) patients presented with gastrointestinal manifestations without any respiratory symptoms or any other coinfections including 10 acute gastroenteritis, 4 acute gastritis, 2 dysenteries (bloody stool) with stool culture negative and 1 intussusception. Liver dysfunctions were found in 16(15.8%) patients in the form of raised ALT (16) or AST (4) or low albumin (10). All 10 children with low albumin had MIS-C. None of them developed liver failure. There was statistically no significant difference between covid 19 children with GI symptoms and without GI symptoms clinically and biochemically except longer hospital stays more frequent in GI symptoms group (p=0.03). Conclusion: Gastrointestinal symptoms may be a presenting clinical feature even without any respiratory symptoms in Covid 19 children. Liver dysfunction is uncommon in children with mild Covid 19 infections. If liver dysfunction is found, we think that it may progress to more severe infection like MIS-C or associated with co infection. Bangladesh J Child Health 2023; Vol 47 (2) : 84-88
Background: Pancreatitis is an inflammatory condition of the pancreas, which might extend to local and distant extra-pancreatic tissues. It can be acute, acute recurrent or may be chronic. The diagnosis of different types of pancreatitis is based on a combination of clinical signs and symptoms, laboratory investigations and imaging techniques. Objective: To describe and find out the association of the laboratory and imaging profiles of different variety of pancreatitis in children. Methods: It was a an observational study conducted at the department of Paediatric Gastroenterology and Nutrition of Bangabandhu Sheikh Mujib Medical University from July 2018 through June 2020. A total of 48 cases were included in this study. The diagnosis of acute, acute recurrent and chronic pancreatitis was based on diagnostic criteria made by INSPPIRE group. Clinical characteristics, hematological, biochemical and imaging profile of the different variety of pancreatitis and their hospital outcome were observed. Result: Among 48 cases, acute recurrent pancreatitis were 56.3%. Mean age of the patients at presentation was 10.3 years. Laboratory tests showed serum calcium and C-reactive protein was significantly altered among acute, acute recurrent and chronic pancreatitis with characteristic imaging findings. Among imaging modalities, abdominal ultrasonogram showed swollen pancreas (58.3%) was significantly common in acute recurrent pancreatitis than chronic pancreatitis, whereas shrunken pancreas (28%) was significantly common findings in chronic pancreatitis. In addition to ultrasonogram, MRCP aided diagnosis of chronic pancreatitis. Complications developed in 25% of acute pancreatitis cases, which included hypocalcemia (58.3%), ascites (86.7%), pleural effusion (66.7%), pseudocyst (33.3%) and pancreatic necrosis (25%). Hospital stay was significantly prolonged in acute pancreatitis cases, when compared to other types of pancreatitis . Conclusion: Low calcium level and high CRP level were found in acute pancreatitis than in acute recurrent and chronic pancreatitis. Swollen pancreas with beaded and tortuous pancreatic ducts were found in case of chronic pancreatitis. Bangladesh J Child Health 2023; Vol 47 (2) : 95-102
Background: Anaemia is a common childhood health problem. Among the causes of anaemia besides the nutritional anaemia congenital hemoglobin disorder such as thalassemia and haemoglobinopathies has a great role which can be combated by increasing awareness in the population. Objective: To detect clinical features, common causes, risk factors of childhood anaemia and to make awareness among the population to prevent the deadly effects of anaemia in children as well as in the society. Methodology: A cross-sectional study was carried out at outpatient department of Delta Medical College and Hospital over a period of 3 years. Children aged between 6 months to eighteen years who visited outdoor for acute illness and were clinically pale and CBC supporting the condition were included. Children with malignancy and haemorrhagic diseases were excluded from the study. Total 200 cases were collected. Results: Among 200 cases 62.5% were male. Most vulnerable age group was 6 months to 2 years (63%). Most common cause of anaemia was iron deficiency anaemia (64.5%). Congenital haemoglobin disorder was the second most common cause (28%). Among haemoglobinopathies Hb E trait contributes (82.35%). Most common presentation of anaemic children was frequent attack of RTI. Low protein diet was the most common risk factor for iron deficiency anaemia. Under five years, males were more affected but thereafter females were more affected than male children. Among 200 cases only 32(15%) cases were malnourished. Most of the cases were term baby. Among inherited cases only one care giver had knowledge about thalassemia. Most cases came from lower middle class family (65%). Most common morphologic variant of anaemia was microcytic hypochromic anaemia (99%). RBC count was normal in iron deficiency anaemia. Conclusion: Besides iron deficiency anaemia, haemoglobinopathies and thalassemia have a great contribution in childhood anaemia. Family education, increased awareness, screening out of thalassemia and haemoglobinopathies carrier can combat this heath problem in children and can reduce social burden. Bangladesh J Child Health 2023; Vol 47 (2) : 89-94
Neuronal ceroid lipofuscinoses (NCLs) are a group of lysosomal storage disorders characterized by progressive, neurodegenerative course. NCLs are classified into four subtypes according to age of onset. Among them, late infantile variety is the second most common condition. Patients typically manifest with seizures, cognitive, motor deterioration and vision loss. Here, we reports two cases of NCLs, because of rarity of this disease in such a young age. As the disease advanced, patients experienced repeated episodes of seizures, ataxia, gradual deterioration of vision and loss of ambulation and speech. The electroencephalogram showed focal epileptiform discharges over left central and parietal region. Magnetic resonance imaging of Brain revealed extensive cerebral and cerebellar atrophy. A skin biopsy extracted from the armpit area displayed periodic acid-schiff (PAS) stained eosinophilic intracytoplasmic inclusions (curvilinear like). These findings are compatible with neuronal ceroid lipofuscinoses. Genetic testing for NCL is the gold standard investigation but patient can also be diagnosed by clinical correlation and positive histopathology findings in low income countries. Bangladesh J Child Health 2023; Vol 47 (2) : 113-116
Hand-foot-and-mouth disease (HFMD) is a common self-limiting viral infection in children caused by Coxsackievirus A16 and human enterovirus 71, presenting with fever, erythematous papulovesicular eruptions/ blisters on the palms, soles, knees, buttocks, and oral mucosa. Although rare, complications may arise in nails e. g. onychomadesis (nail separation) and Beau’s lines (transverse lines on the nails). These typically appear within four to eight weeks of HFMD and persist for around 5-6 weeks. No active treatment is required and these nail changes resolve spontaneously within few weeks as the nail bed remain intact. We present here a boy of 4 years who presented with such nail changes after around 5 weeks of HFM disease and diagnosed as onychomadesis and Beau’s lines after ruling out other possible causes of nail changes. BANGLADESH J CHILD HEALTH 2023; VOL 47 (1) : 47-50
Background: Kawasaki Disease (KD) is recognized as the leading cause of acquired heart disease in children in developed countries. This is a pioneer study from Bangabandhu Sheikh Mujib Medical University (BSMMU), documenting the common epidemiological, clinical, laboratory and echocardiographic findings, including management and immediate outcome of KD children. Method: This retrospective study includes all patients with a diagnosis of KD, who attended the pediatric rheumatology division at BSMMU from January 2015 to December 2019. Data were collected from medical records at diagnosis and immediately after management. Relevant statistical tests were done and a p value < 0.05 was considered as statistically significant. Result: A total of 129 cases were diagnosed with KD, among them complete KD cases were 62% and incomplete KD was 38%. The mean age at onset was significantly higher in incomplete KD than complete KD. The majority of the cases (67%) were between 1 to 5 years of age and male (72%) children were predominant. Fever was the most common presenting feature (100%), followed by bilateral conjunctivitis, mucosal involvement, rash, changes in extremities, cervical lymphadenopathy and others. Coronary artery dilatation was present in 30.5% cases with no significant differences between complete and incomplete KD cases. Aspirin was given to all the cases and IVIG was given to only 46.6% children. Rests of the children were treated with methyl prednisolone. All the cases improved after treatment and there was no immediate mortality. Conclusion: It seems that KD is not an uncommon disease and incomplete KD cases are relatively more prevalent. About 30% KD cases had coronary artery abnormalities. Immediate outcome of management is excellent but needs long term follow-up. BANGLADESH J CHILD HEALTH 2023; VOL 47 (1) : 9-16
Background: The prevalence of asthma in Bangladesh is about 7%. The spirometric values in healthy children were obtained in a single urban school more than a decade ago. No rural data is available till date. The objective of this study is to determine the spirometric values of healthy school going children in three rural schools of Bangladesh. Materials and Methods: This cross-sectional study was carried out from July 2019 to June 2020 among 300 healthy children among both boys and girls aged 11 to 15 years in three rural schools. Among 350 approached students 332 students responded to the study, 18 students did not meet inclusion criteria and 14 students could not follow spirometry procedure. Informed written consent was taken from guardians. History regarding socio-demographic profile, passive smoking, concurrent or past respiratory illness and medication were taken and weight, height and BMI were measured of the children. Spirometry was done with spirometer and reports were generated by software installed in a laptop. Results: Boys showed higher FVC, FEV1 and PEFR but lower FEV1/FVC ratio than those of girls. Regression equations for lung function values were determined for boys and girls considering height as independent variable. Conclusions: Spirometric indices were higher in boys than those of girls and they tended to increase with height, except FEV1/FVC ratio that was higher in girls with a negative correlation with height. BANGLADESH J CHILD HEALTH 2023; VOL 47 (1) : 17-23
Background: Immunoglobulin M nephropathy (IgMN) is a new clinicoimmunopathologic entity which present mainly as idiopathic nephrotic syndrome in both children and adults. The clinical manifestation of IgMN are highly variable presenting with isolated haematuria or asymptomatic proteinuria. About 6 to 23 percent of IgMN patient developed end stage kidney disease. Usually treated with immunosuppressive agents (cyclosporine/tacrolimus) along with corticosteroid has response rates up to 50%. In our institute last few years we have observed a surprisingly rise of IgM nephropathy in children. Objective: To see the response of IgMN children treated with tacrolimus or mycophenolate mofetil(MMF) and long term follow up to observe their outcome. Methodology : This was a prospective observational study done in the Department of Paediatric Nephrology, National Institute of Kidney Diseases & Urology, Dhaka, starting from January/2014 to December/2017. The study population consists of 86 IgMN children, age ranges from 2 to 12 years. The study was approved by institutional ethical review committee and informed written consent was taken from every parents. Twenty seven patients(Group-I) were selected purposively and treated with MMF and 59 patients (Group- II) treated with tacrolimus for 2 years. Patients were followed up three monthly onward in a prescribed form including adverse effect of drugs. Clinical outcome data like remission, active disease, active disease with renal impairment and dependency on renal replacement therapy(RRT) were documented for statistical analysis. Results: Of 86 IgMN children most of them were older than >8-12 years in both groups and the highest percentage were clinically diagnosed as frequently relapsing nephrotic syndrome. In Group I 43.48% children remain on remission, 13.04% have active disease, 30.43% are active disease with impaired renal function and 4.35 % went into RRT. In Group II 55.56% children remain on remission, 12.96 % have active disease, 16.67% are active disease with impaired renal function and 5.56% dependent on RRT. Conclusion: From above observations, the overall prognosis of IgMN children is not good. The clinical course and disease outcome did not differ significantly between two groups treated with mycophenolate mofetil and tacrolimus. BANGLADESH J CHILD HEALTH 2023; VOL 47 (1) : 34-38
