Bangladesh Journal of Child Health

Marfan Syndrome in Two Sibs: A Case Report

2022-06-07T08:43:29+00:00

Marfan syndrome (MFS) is a rare inherited disorder of connective tissue characterized by various phenotypical and genetic manifestations. It involves mutation in FBN1 gene which encodes for microfibrillin glycoprotein fibrillin-1. Predominant involvement occurs in eyes, skeleton and cardiovascular system. Cardiovascular abnormalities such as aortic root dilatation and mitral valve prolapse are the two main life-threatening complications associated with MFS. The diagnosis is based upon clinical findings, some of which are age dependent. If not properly treated, premature death may be caused by the severe cardiovascular and pulmonary complications associated with Marfan syndrome. Therefore, it is important to identify this potentially life-threatening condition in general practice. We are presenting two cases of Marfan syndrome from same family due to its rarity.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 108-110

Newborn with Hereditary Sensory and Autonomic Neuropathy Type- IV: A Case Report

2022-06-07T08:43:49+00:00

Hereditary sensory and autonomic neuropathy type IV (HSAN-IV) is a very rare autosomal recessive disorder characterized by anhidrosis, recurrent fever, insensitivity to pain and temperature, hypotonia, and developmental delay. The most frequent complications of this disease are self-mutilation, febrile seizure, stroke, corneal scarring, multiple fractures, burn, osteomyelitis and joint deformities. There is no definitive treatment for this condition. Early diagnosis can reduce disease complications and number of cases.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 111-115

Idiopathic Neuralgic Amyotrophy: A Rarely Documented Disease in Children

2022-06-07T08:50:25+00:00

Idiopathic Neuralgic amyotrophy (INA) is a neurological disease that occurs in all age groups. It is characterized by sudden onset of pain and subsequent weakness followed by atrophies of arm and shoulder muscles. But in children, it is uncommon and has a diverse clinical spectrum and etiology. Diagnosis is often delayed or even missed. Its treatment protocol and prognosis are still controversial. We herein describe three cases of INA with a thorough review of thepreviously reported cases in children. This will provide an overview of the pediatric phenotype and prognosis.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 116-119

Continuous Renal Replacement Therapy (CRRT) in Critically Ill Children with Multiple Organ Dysfunction Syndromes: A Case Report

2022-06-07T09:09:12+00:00

Acute kidney injury (AKI) is a serious and potentially fatal clinical consequence of disease that increases morbidity and mortality in hospitalized children. In the event of AKI and fluid overload, renal replacement therapy (RRT) is a must-have therapy. In critically sick children with multi organ dysfunction syndrome (MODS) who are hemodynamically unstable, CRRT (continuous renal replacement therapy) is the cornerstone of dialysis. Although several hospitals in the country have begun to utilize CRRT in adult patients in the critical care unit, a lack of expertise and high therapeutic costs have prevented widespread adoption of CRRT. We managed a child with CRRT, who had AKI and MODS as a consequence of dengue shock syndrome and also was hemodynamically unstable in our hospital. The child’s renal function improved as a result of the CRRT therapy, as did his MODS. We have summarized our experience using CRRT in children for the first time in Bangladesh in this article.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 120-123

Generalized Hyperpigmentation - An Unusual Feature in Neurologic Wilson Disease: Report of Two Cases

2022-06-07T09:16:10+00:00

Wilson disease also known as hepatolenticular degenerationis an autosomal recessive disorder of copper metabolism, usually presents either with hepatic or neurological features. But sometimes along with common features, some atypical presentations may also co-exist. We report here two cases of Wilson disease, a 15 years old girl and 14 years old boy presented with some neurological manifestations along with gradual darkening of whole body.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 124-127

Abstract from Current Literatures Vol. 45(2)

2022-06-07T09:38:44+00:00

Abstract not available

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 128-129

Correlations of CT Scan Findings in Different Types of Cerebral Palsy: A Study in a Tertiary Care Centre in Bangladesh

2022-06-06T04:03:07+00:00

Introduction: Cerebral palsy (CP) is one of the most common causes of chronic childhood disability. CT scan is helpful for identification of structural abnormalities, etiology, categorize the types of brain lesions and planning of intervention in the child with CP.

Objective: This study was done to find relationship between types of CP and CT scan findings of brain.

Materials and methods: This cross sectional descriptive study was done among 110 clinically diagnosed CP case from department of Paediatric Neurology & IPNA, BSMMU, during March 2016 to August 2017. Demographic, clinical characteristics & physical examination were done & collected data were recorded. CT scan of brain was done in all cases & all data were analyzed.

Results: The mean age of the studied children was 2.6±2.2 years (1 to 14 years). Among them quadriplegic CP were the most common (39.1%), followed by 28.2% cases of hemiplegic CP, 16.4% were of mixed type, 7.3% were diplegic. Most common finding on CT scan of brain was cerebral atrophy (62.7%), followed by encephalomalacia (15.5%), calcification (13.6%), and brain malformations (11.8%). Abnormal CT scan findings were found in 88.2% of studied children. Children with hemiplegic CP had brain atrophy in 87% of cases, calcification was found mostly in quadriplegic CP. Brain malformation was found mostly in mixed type of CP.

Conclusion: Most common CT scan finding was brain atrophy and commonly found in hemiplegic CP. Encephalomalacia and calcifications were commonly found in quadriplegic CP. Brain malformation were commonly found in mixed type of CP.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 67-73

Hemoglobin A1c Value of Pregnant Diabetic Women with Their Neonatal Outcome

2022-06-06T04:25:52+00:00

Background: HbA1c is now accepted as a standard measure for diagnosing type 2 diabetes mellitus. Maternal high HbA1c levels have been associated with increased risk of adverse neonatal outcomes.

Objective: To find out the relationship between maternal HbA1c and neonatal outcomes.

Materials & Methods: This prospective observational study was conducted from February 2018 to January 2019 in the Department of Neonatology and Department of Gynecology & Obstetrics, BSMMU, Dhaka. Diabetic mothers with known HbA1c Value and their infants who were born in this study period in BSMMU were enrolled. Neonates were divided into two groups. One group that had maternal HbA1c <6% and the other group >6%. Neonatal weights, routine capillary blood glucose were recorded.

Results: Among the total 94 mothers, HbA1c level were <6% in 56 mothers and ³6% in 38 mothers. The mean weight, BMI and previous history of gestational diabetes of mothers were significantly higher in HbA1c ³6% group. Birth weight and large for gestational age infants was high in ³6% HbA1c group. Neonatal hypoglycemia developed more in high HbA1c group. Total visible birth defect was 4 and all were in high HbA1c group (p=0.022).

Conclusion: Elevated HbA1c level during pregnancy is a predictor for large for gestational age infants as well as neonatal hypoglycemia.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 74-78

Dyslipidaemia Among Bangladeshi Children and Adolescents with Type-1 Diabetes: An Observational Study

2022-06-06T04:40:15+00:00

Introduction: Dyslipidemia increases the frequency and severity of micro and macrovascular complications of type 1 diabetes (T1D).

Materials & Methods: This cross-sectional study was aimed to evaluate the frequency of dyslipidaemia and its association with other risk factors in children and adolescents with type 1 diabetes. A total of 397 Type 1 diabetes (T1DM) patients aged 10-18 years who attended, Paediatric diabetes clinic in BIRDEM over one year period were included in this study.

Results: The overall frequency of dyslipidaemia was 63.5% and median duration of diabetes was 3.0[2.0- 5.0 years]. The High LDL was most cmmmon dyslipidaemia (81%) in our study population. FBS was significantly higher 12.8[10.0-15.5] vs 10.8[7.7- 12.6] (p<0.0001)) and higher median HbA1c 9.9 [8.5-11.9] vs 9.0[7.9-10.5] (p<0.0001)) was found in dyslipidaemic patients. Median systolic blood pressure was higher (110 vs100) in dyslipidaemic patients ( p =.042).

Conclusion: The frequency of dyslipidemia was high among the children and adolescents with T1DM which was associated with poor glycaemic control. Our results provide strong support for prior literature in emphasizing the importance of good glycemic control, to reduce the risk of dyslipidaemia.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 79-82

Efficacy and Safety of Piperacillin-Tazobactam and Cefepime as Empirical Therapy for Febrile Neutropenic Children with Acute Lymphoblastic Leukaemia (ALL)

2022-06-06T04:47:00+00:00

Background: Infection is a major clinical challenge in ALL treatment. Prompt administration of empirical antibiotic in febrile neutropenic patients has reduced the mortality. Both Cefepime or Piperacillin-Tazobactam has been used as empirical treatment.

Objective: To compare the efficacy and safety between Piperacillin-Tazobactam with Cefepime in febrile neutropenic children with ALL.

Materials and methods: This randomized study was conducted from August 2015 to August 2016 in BSMMU. Sixty one episodes of febrile neutropenia in children with ALL, aged 0 to18 years were included in this study. Patients were randomized into two groups. One group received Piperacilln/Tazobactam and another group received Cefepime and data of 60 febrile neutropenic episodes were analyzed.

Results: Febrile neutropenic episodes in the Piperacillin/Tazobactam group were 28 and in Cefepime group was 32 episodes and 34(57.63%) were male and 25 (42.37%) female. Median age was 5 years and 38(62.3%) neutropenic episodes were in induction phase. Majority had fever without focus 21(35%). Microorganisms isolated in 13 (21.66%) patients and majority 6 (46.15%) had blood infection. Most of the isolated organisms were Gram negative 11(84.61%). Overall treatment success without modification in the Piperacillin/Tazobactam group was 17(60.7%) and in Cefepime group 18 (56.3%) and that comparison was not statistically significant (p= 0.732). Significant difference was also not found comparing the mean duration of fever, neutropenia and hospital stay.

Conclusion: Both Piperacillin/Tazobactam and Cefepime were found effective and safe as an empirical therapy for febrile neutropenic children with ALL.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 83-88

Effect of Single Dose Fenofibrate as an Adjunct to Phototherapy on Unconjugated Neonatal Hyperbilirubinemia: A RCT

2022-06-06T10:48:12+00:00

Background: Fenofibrate is used as an adjunct therapy to reduce bilirubin level and duration of phototherapy. This study was done to determine the efficacy of fenofibrate in the treatment of unconjugated neonatal hyperbilirubinemia.

Materials & Method: Total 60 neonates were enrolled and devided into two groups. In group A, a single dose of oral fenofibrate (10mg/kg) was given along with phototherapy, and Group B only phototherapy. Blood samples were collected every 24 hours for total and indirect bilirubin. SGPT was done before and after 24 hours of giving fenofibrate.

Result: Baseline serum bilirubin was 16.86± 0.89 in group A and 17.31± 1.19 in group B. After 24 hours of starting phototherapy, the bilirubin level came down to a lower value of 14.83 ± 0.95 mg/dl in group A and 15.73 ± 1.32 mg/dl in group B. After 48 hours it was 12.85 ± 1.06 mg/dl and 14.20 ± 1.24 mg/dl in group A and group B respectively. (P-value 0.004 and 0.001). The mean time needed for phototherapy was 51.20 ± 8.29 hours in group A and 70.40± 6.08 hours in group B (P-value 0.001). The duration was shorter in the fenofibrate group in comparison to the control group. No side effects of fenofibrate were observed after a single dose administration.

Conclusion: Administration of a single dose of fenofibrate as an adjunct along with phototherapy to neonates showed a significant reduction of serum bilirubin and duration of phototherapy.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 89-93

Frequency and Risk Factors of Seizures in Children During Induction Remission Chemotherapy for Acute Lymphoblastic Leukemia (ALL)

2022-06-06T10:49:32+00:00

Background: Seizure is one of the most common CNS complications among patients receiving chemotherapy for acute lymphoblastic leukemia (ALL). Seizures are seen in 8-13% of patients with ALL. Most seizure occurs during the induction and consolidation phases of treatment. However, not much is known about the risk factors of these seizure.

Materials & Method: This prospective observational study was conducted in the department of Pediatric Hematology and Oncology, BSMMU. One hundred and five newly diagnosed admitted children with ALL aged >1 year to <18 years, from May 2017 to March 2018 for induction remission chemotherapy and who fulfilled the criteria of case definition were enrolled into this study.

Results: This study showed that seizure frequency was14.3% in children with ALL getting induction remission chemotherapy. Female patients developed seizures more than males which was statistically significant by univariate analysis (p = 0.029) but was not found to be a risk factor by multivariate analysis (p = 0.059). All the seizures developed in B-lineage ALL but there was no statistically significant association between lineage of ALL with development of seizures.

Conclusions: In this study slightly higher frequency of seizure in ALL patients on induction remission chemotherapy was found than previous studies (14.3% vs. 13%). Females developed seizures more than males.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 94-98

Notes & News Vol. 45(2)

2022-06-07T09:38:20+00:00

Abstract not available

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 130-131

Breastfeeding in COVID-19 Infection

2022-06-06T10:52:10+00:00

Abstract not available

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 99-107

COVID-19 Related Multi System Inflammatory Syndrome in Children:Spectrum of Kawasaki Disease or Different Entity? A Challenging Mystery for Paediatricians

2022-06-06T03:54:36+00:00

Abstract not available

BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 63-66