Bangladesh Journal of Child Health

Sweet Syndrome: A Rare Skin Disorder in Children

2022-11-20T06:54:27+00:00

Sweet syndrome (SS) is a rare dermatosis of unknown etiology. It is characterized by fever, neutrophilia, raised and painful plaques on the skin of the face, neck, limbs, and histologically by dermal infiltration of neutrophils. Here, we present a ten-year-old boy who presented with fever and multiple skin lesions for 15 days. On examination, he was febrile and presented with multiple nodular, tender, erythematous rash on face and limbs. Laboratory findings revealed raised inflammatory markers with neutrophilic leukocytosis, skin biopsy showed hyperkeratosis and neutrophilic infiltration of the dermis. Typical history, laboratory investigations including skin biopsy findings were suggestive of diagnosis of SS. Prolonged fever and characteristics skin lesions in any child should be suspected for this rare syndrome. It should be kept in mind as a differential diagnosis in the day-today clinical practice for effective management of this rare disease.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 172-174

Alternating Hemiplegia of Childhood with ATP1A3 Gene Mutation- A Case Report

2022-11-20T08:14:34+00:00

Alternating hemiplegia of childhood (AHC) is a rare neurological disorder manifested by transient repeated episodes of alternating hemiplegia associated with other neurological events. Though the exact etiology was largely unknown, recently the pathogenic mutation in the ATP1A3 gene encoding for the alpha3 catalytic subunit of Na+/ K+ATPase was found to be the primary cause of AHC. We present the case of 8.5 year old girl with AHC who had a heterozygous de-novo p.Leu839Pro (c.2516T>C) pathogenic mutation of ATP1A3 gene on chromosome 19q13.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 175-177

Edward’s Syndrome-A Case Report

2022-11-20T08:25:09+00:00

Edward’s syndrome is commonly known as the trisomy 18 syndrome. It is a devastating genetic disorder that can be characterized by multiple congenital anomalies. About 50% babies with this syndrome do not survive beyond one week of age and approximately 95% does not survive past the first year of life. The feature of Edward’s Syndrome involves in craniofacial, skeletal, cardiovascular, central nervous system and genitourinary malformations. The mortality rate among infants with trisomy 18 is high as a result of cardiac and renal malformations, feeding difficulty, sepsis and central nervous system defects. Here we report a neonate with Edward’s syndrome.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 178-181

Unnoticed Foreign Body (Metallic Hair Clip) Ingestion in An 18 Month Old Child: A Case Report

2022-11-20T08:32:27+00:00

Accidental foreign body ingestion is common in the pediatric age group particularly between 6 months and 5 years. Most ingested foreign bodies pass easily through the esophagus, into the stomach, and are expelled from the body without complications. However, endoscopic removal will require only 10% - 20% of cases to avoid complications. We present the endoscopic removal of a metallic hair clip in the duodenum of an 18 month old child that was unnoticed initially and diagnosed incidentally.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 182-185

Neurodevelopmental Outcome in A Child With West Syndrome On Multidisciplinary Intervention – A Case Report

2022-11-20T08:40:04+00:00

West syndrome is a triad of infantile spasms with hypsarrhythmia and developmental regression. It results in a poor neurodevelopmental outcome due to delayed diagnosis and treatment which is further compounded by a delay in referral to multidisciplinary interventions. Our case was a male child with west syndrome who came to us at 3 years of age with neurodevelopmental concerns of global developmental delay with features of autism spectrum disorder. Based on thorough developmental evaluation he was started on an individualised, short term, goal oriented multidisciplinary therapy with physiotherapy, occupational therapy and parental counselling for his neurodevelopmental concerns. As a result improvement was noted in his motor as well as socioemotional skills. Early intervention with multidisciplinary therapy with adherence by motivated parents is essential for a better neurodevelopmental outcome in a case of west syndrome.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 186-190

Abstract from Current Literatures Vol. 45(3)

2022-11-20T08:59:01+00:00

Abstract not available

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 191-192

Notes & News Vol. 45(3)

2022-11-20T09:03:47+00:00

Abstract not available

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 193-194

COVID-19 in Children: Bangladesh Experience

2022-11-20T03:50:30+00:00

Abstract not available

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 131-133

Infectious Complication During Induction Chemotherapy in Children with Acute Myeloid Leukemia- A Single Center Study

2022-11-20T04:00:58+00:00

Background: Acute Myeloid Leukemia (AML) contributes to about 20% of leukemia. Most of the AML patients suffer from infection.

Objective: To evaluate the rate of infection, type and site of infection, organisms responsible for infection and to assess antibiotic sensitivity pattern and infection related mortality in AML.

Material & Methods: Newly diagnosed AML patients aged between 1-18 years, admitted to receive induction chemotherapy were enrolled. They received induction of chemotherapy according to Modified MRC’ 12 in our department. Patient was on followup throughout the induction period and all the infectious complication occurred during this period were analyzed.

Result: A total of 38 episodes of infection developed in 34 patients in both cycles of induction (1.12 episodes/ patient). Among the total 38 episodes of infection, fever was the commonest clinical presentation. In both chemotherapy cycles, 7(18.4%) episodes of infection were culture positive. A total of 9 organisms were isolated of which most of them was gram-negative. Fever without focus was found in 15(39.5%) episodes. Profound neutropenia was present in 15(39.47%) episodes and profound neutropenic episodes were found to be culture positive in 5(71.4%) cases. About 6(17.5%) patients died during 1st cycle of induction.

Conclusion: Among children with AML, profound neutropenic cases had high susceptibility to culture positive infection. Further prospective study is needed to identify means to prevent infectious complication of AML patients receiving induction chemotherapy particularly in those with profound neutropenia.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 134-140

Sensitivity and Specificity of Toxic Granules in WBC and Immature to Total Neutrophil Ratio in Diagnosis of Neonatal Sepsis

2022-11-20T05:27:55+00:00

Background: Septicemia is the commonest cause of neonatal mortality and severe morbidity. Positive bacterial blood culture result confirms clinically suspected sepsis but it is too late to start antibiotic. We need a easy, reliable and cost effective test to diagnose sepsis in time.

Objective: To see the relation of toxic granule and immature neutrophil with neonatal sepsis.

Material & Methods: This hospital based, cross-sectional study was conducted on 152 neonate with suspected sepsis admitted in Dhaka Shishu Hospital during January 2013 to April 2014. After enrollment all studied neonate were investigated with complete blood cell count, CRP and Blood culture before starting treatment with antibiotic. Raised immature to total neutrophil ratio >0.2 and toxic granules in neutrophil were correlated with positive blood culture test using x2 test.

Result: Out of 152 studied neonate 61.89% were male, 41.94% premature, 48.85% low weight. Blood culture result was found positive in 7.24% neonate. Leukopenia (WBC<5000/mm3), Neutropenia (Neutrophil <1750/mm3), Immature to total neutrophil ratio (I:T) >0.2 and toxic granules were present significantly in sepsis. The sensitivity and specificity of toxic granules to diagnose neonatal sepsis was correspondingly 91.67% and 75% & that of I: T>0.2 were 90.91% and 66.67%.

Conclusion: Toxic granules and Immature to total neutrophil ratio (I: T) >0.2 can be considered as useful tools in the diagnosis of neonatal sepsis to start antibiotic earlier, especially where there is limited modern investigation facilities.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 141-146

Analysis of Pediatric Rasmussen Encephalitis: Experience from A Tertiary Care Hospital in Bangladesh

2022-11-20T05:32:48+00:00

Background: Rasmussens encephalitis (RE) is rare progressive cerebral inflammatory disease predominantly affecting pediatric population. The key clinical features are seizure and hemiparesis. Seizure is mostly focal with or without epileptia partialis continua (EPC). Diagnosis is established by EEG showing epileptic discharges with focal slowing, focal cortical atrophy in neuroimaging and T cell dominated encephalitis in biopsy or autopsy. This study was done to describe the clinical, laboratory features and treatment of RE in Bangladesh perspective.

Materials & Methods: This study was done in Department of Pediatric Neurology, IPNA, BSMMU, Dhaka, Bangladesh. Four admitted children who were diagnosed as RE from January 2016 to July 2019 were included in this study. Retrospective detail data of the all four patients were collected and analyzed.

Results: The age ranging was 3-12 years, all were male. Cognitive decline, speech disorder, hemiparesis were observed in all the patients. Among them 1 had prodrome of fever. All the patients had seizure, mostly focal onset. Among them 2 had EPC. Treatment was given mainly with immune modulatory drugs (Intravenous methyl prednisolone, IV immunoglobulin, oral steroids) and antiepileptic drugs. On follow up all the patients had consequences in the form of hemiparesis, epilepsy, dysphasia, cognitive disorder etc.

Conclusion: Cognitive decline, speech disorder, focal seizure and hemiparesis were the common presentation. EPC was observed in 50% of cases and consequences on follow up in all cases.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 147-154

Frequency and Risk Factors for Hyperglycemia in Children with Acute Lymphoblastic Leukemia During Induction Chemotherapy

2022-11-20T05:35:56+00:00

Background: Hyperglycemia is a common side effect and it has long been recognized as a consequence of corticosteroids and L-asparaginase, chemotherapeutic agents key to ALL treatment. This study was done to determine the frequency and identify the risk factors for hyperglycemia in paediatric ALL during induction chemotherapy.

Material & Methods: This prospective observational study was done among 87 newly diagnosed ALL cases of 1-18 years of age. After initial work up patient got induction chemotherapy according to UK ALL 2003 (modified) protocol in BSMMU. Anthropometric measurements and CBC, RBS/ 2 hours after glucose/ FBS and corresponding urine sugar was assessed after 2nd, 4th, 6th and 9th dose of Lasparaginase and after completion of induction. Data were analyzed by SPSS 17.0.

Results: Eight patients (9.75%) of the study cohort developed hyperglycemia. Among ³10 years of age 36.8% had hyperglycemia compared to 1.6% of younger children (p<0.001). No gender difference was found. Obese and overweight (p <0.001 in both cases), positive family history of diabetes mellitus using 4 drugs during induction remission phase of chemotherapy were found significant risk factors for hyperglycemia.

Conclusion: In this study frequency of hyperglycemia was 9.75% and frequency of transient hyperglycemia (TH) was 7.3%. Older age, obese or overweight children, positive family history of diabetes mellitus, patient getting regimen B according to UK-ALL 2003 (modified) protocol had shown increase the risk of hyperglycemia.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 155-161

Role of Phenobarbitone Maintenance Therapy in Asphyxiated Neonate with Encephalopathy to Control Seizure- A Randomized Clinical Trial

2022-11-20T06:32:41+00:00

Background: Seizures after perinatal asphyxia may worsen brain injury. Phenobarbital (PHB) is commonly prescribed anticonvulsant worldwide to control seizure in asphyxiated neonates. The evidence of the best use of maintenance drugs is limited.

Objective: To assess the effectiveness of phenobarbitone maintenance therapy in asphyxiated neonates with seizure.

Material & Methods: A total of 79 asphyxiated neonates (HIE-II/ III), ³35 weeks were enrolled from January 2019 -January 2020 in this randomized clinical trial where cases were categorized into three groups. Group A received phenobarbitone 4mg/ kg/day twice daily for 6 weeks and Group B received 2mg/kg/day once daily for 2 weeks while Group C didn’t receive any anti-seizure medication after acute management. Clinical and electrophysiological study was done at discharge, one and half month of age. Data were analyzed by SPSS version 20.

Result: Mean age in days was 0.96 ± 1.77 in Group-A, 0.66 ± 1.20 in Group-B and 0.55 ± 1.30 in Group-C. Both Group A and C had seizure in 33.34% and 12.5% in group B at 1½ month. During discharge most of the cases had normal EEG, EEG abnormalities were found in 25% cases in group B, 16.67% in group C and 8.33% in group A. At 1 ½ month, EEG abnormality was found more among group C (33.34%) than group A (25%) and B (25%) which was statistically insignificant.

Conclusion: This study concluded that early discontinuation of phenobarbitone after acute management may not increase the risk of clinical and electrographic seizure in future.

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 162-167

Prematurity Management- Needs Priority Attention

2022-11-20T06:42:53+00:00

Abstract not available

BANGLADESH J CHILD HEALTH 2021; VOL 45 (3) : 168-171