Encephalitis in Children: Viruses and Beyond.

Encephalitis is commonly caused by viruses. But beyond viruses there are so many causes of encephalitis. Encephalitis is the inflammation of the brain parenchyma due to any reason. As there are so many causes of encephalitis presentations are also variable. So to diagnose encephalitis a set of clinical, laboratory, electroencephalographic and neuroimaging criteria is used. Any children attend medical facility with sudden onset altered mental status along with any of the following features like fever, seizure, focal neurological signs should be evaluated as encephalitis. Viruses are the common cause of encephalitis. Along with infectious etiologies a vast group of noninfectious like autoimmune causes encephalitis also established. When children presented with above mentioned features along with behavior problem and or movement disorder there is a high suspicion of autoimmune etiology. Any suspected case of encephalitis should initiated treatment with antiviral along with supportive treatment; then step wise evaluation should be done to reach an etiological diagnosis. If infectious etiology could not be established or no significant improvement is found with antiviral therapy; immunomodulating therapy should be considered along. In all cases CSF analysis including biochemistry, cytology, viral PCR along with MRI and EEG should do; further investigations depend upon initial reports and clinical and epidemiological background. Dose and duration of antiviral depends on patient's age and response to treatment and comorbidity. Acyclovir 500mg/m²/BSA per dose 3 times daily for 21 days are adequate for HSV encephalitis. Monitoring of renal function is the essential. Adjuvant treatment with steroid and or manitol for cerebral edema and antiseizure drugs for convulsion is used where necessary. Meticulous fluid and nutritional support as well good general care improve outcome. In spite of adequate treatment of encephalitis mortality and morbidity was found a significant number of cases; among the morbidity behavior problem, seizure focal deficit are common.

Quality of Life Assessment by SF-36 among the Patients with Rheumatoid Arthritis.

Rheumatoid arthritis (RA) patients have substantial functional disability which poses a significant impact on the quality of life (QoL). So, this study aims to assess the QoL of RA patients by the Short Form Health Survey-36 (SF-36) questionnaire. Total sixty-two (62) rheumatoid arthritis patients were included in this cross-sectional observational study and were performed in the Department of Physical Medicine and Rehabilitation, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from August 2018 to September 2019 according to selection criteria. A Bengali validated version of Medical Outcomes Study-36: Item Short Form Health Survey (SF-36) questionnaire was used to assess QoL. Among the sixty-two (62) patients, mean age was 44.18±12.14 years with female preponderance (77.4%). About one-fifth of the patients (19.4%, n=12) were illiterate and the majority (71.0%, n=44) were housewives. Amongst all, 53.2% (n=33) had monthly family income >20,000 taka. Forty percent (n=25) had a disease duration of 1 to 5 years. According to Disease Activity Score Sheet 28 (DAS-28), 36(58.1%) patients had moderate disease activity and 26(41.9%) patients had high disease activity. Among the 8 domains, the mental health domain had the highest score (42.77±13.78) and the role physical domain had the lowest (35.89±15.43). Male, literate patients with higher income had relatively better QoL. Patients with prolonged disease, high disease activity and irregular taking of Disease Modifying Anti-Rheumatic Drugs (DMARDs) had poor quality of life. A significant negative correlation was present between SF-36 scores and the DAS-28 scores (r = -0.803, p<0.001). Rheumatoid arthritis had a relatively poor quality of life regarding both physical and mental health components, but the physical health component was predominantly affected.

Systemic Therapy of Dermtophytosis with Voriconaole: A Clinical Observational Study of 250 Bangladeshi Patients.

Superficial fungal infection or dermatophytosis is one of the most common dermatological problems in a tropical country like Bangladesh. For last 4-5 years this skin problem is on rise with increase in cases of recalcitrant, recurrent and chronic dermatophytosis. In place of an easy task to treat dermatophytosis recently it has evolved into difficult to treat by traditional antifungal agents. Meanwhile, voriconazole, primarily appeared as an agent for deep fungal infections, has come out as a new promising systemic antifungal drug for dermatophytosis also. In Bangladesh recently an endeavor has been made to treat superficial fungal infection with voriconazole. Two hundred and ninety four patients suffering from recalcitrant, recurrent, resistant and chronic dermatophytosis between 12 and 70 years of age and both sexes were selected for the study between August 2018 and November 2018 from different parts of the country. Two categories of therapy were designed: Group A - 200mg 12 hourly for 14 days and Group B - 200mg 12 hourly for 28 days. Patients showing full response in 2 weeks were declared apparently cured and were kept under follow up. Those who did not show significant response (less than 50% improvement) in 2 weeks were considered failed. Unresponsive cases after 28 days were considered failed. Both groups were followed up for 4 weeks. Cases not showing any activity of the disease and negative microscopy on two occasions at 2 weeks interval were considered cured. Two hundred and fifty patients completed the study. Incidence of cure rate was more in Group B (93.04%) which is statistically significant (p<0.005). Relapse rate was observed more in Group A (22.83%). Visual side effects were 4.4% while vertigo and headache were observed in 12.0% and 10.8% respectably. Voriconazole is a promising treatment option for recurrent, recalcitrant and chronic dermatophytosis in dosage of 200mg twice daily for at least 4 weeks with negligible side effects. More diversified studies with longer follow up period is required for further opinion to establish an authenticated dosage schedule for treatment of superficial fungal infection.

Age and Tumor Location Predict Survival in Nonmetastatic Osteosarcoma in Upper Egypt.

AIM: The aims of this study were to assess survival outcome of pediatric patients with localized osteosarcoma of the extremities in Upper Egypt, identify factors of prognostic significance for survival, and to determine factors predictive of surgical methods used in these patients, as well as developing a clinical model for risk prediction. PATIENTS AND METHODS: A retrospective analysis of data assembled from medical records of 30 pediatric patients with a histologically verified nonmetastatic osteosarcoma of the extremities treated at South Egypt Cancer Institute with a unified chemotherapy protocol between January 2001 and December 2015 was carried out. Prognostic factors were determined using univariable and multivariable methods. A model for surgical outcomes in these patients based on the baseline clinical factors, and the parameters predictive of their tumor response to chemotherapy, was developed. RESULTS: With a median follow-up of 63 months for the study population, the estimates for event-free survival and overall survival (OS) at 3 and 5 years were 69.5% and 79% and 65.2% and 65.3%, respectively. Age 16 years or above was independently associated with both worse metastasis-free survival (hazard ratio [HR]=6.05, 95% confidence interval [CI]: 1.43-25.6, P=0.015) and OS (HR=7.9, 95% CI: 1.71-36.2, P=0.008). In the multivariable analysis, a proximal location within the limb gained a statistical significance to be independently associated with worse OS (HR=2.4, 95% CI: 1.13-22.1, P=0.003). Poor response to chemotherapy was marginally associated with worse metastasis-free survival (HR=4.9, 95% CI: 1.02-23.8, P=0.047) only in the univariable analysis. The patients found to be more likely to undergo an amputation surgery (odds ratio=14.1, 95% CI: 1.34-149.4, P=0.028) were those in whom a tumor was poorly responding to chemotherapy. CONCLUSION: In Upper Egypt, despite the reasonable survival outcomes in nonmetastatic osteosarcoma, a relatively high limb amputation rate has been encountered. The development of a clinical prediction model for future planning of possible outcome improvement in these patients, however, is still feasible.

Hepatitis B Antibody Level Falls after Initial Phage of Chemotherapy in Childhood Haematological Malignancy.

Hepatitis B virus (HBV) infection is a worldwide one of the major public health problem. Vaccination against HBV is highly effective at a very low cost. During treatment and for a variable period after completion of chemotherapy and radiotherapy children became immunosuppress. The main laboratory marker used to assess hepatitis B immunity is IgG antibody to the surface antigen (HBsAg). Surgical procedures and multiple blood transfusions increase the risk of hepatitis B virus infection in these immune suppressed patients. In Bangladesh, data are unavailable regarding prevalence of HBsAg in children with cancer and their protective immune status against hepatitis B virus while on treatment or after completion of chemotherapy. The results of this study may aid physicians in the development of evidence-based guidelines for revaccination of children with cancer during and after treatment with chemotherapy. This observational study was conducted from November 2013 to February 2015 in the department of Paediatric Haematology and Oncology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. Children were recruited from both the inpatient and outpatient units of the department of Paediatric Hematology and Oncology at BSMMU. Blood specimens were collected from the children who were receiving inpatient and outpatient care from the department of Pediatric Hematology and Oncology, 2.0ml of venous blood was drawn and serum was separated and stored at -20°C until testing then HBV antibody titer was assayed at the department of Virology laboratory of BSMMU. A structured questionnaire was used for data collection. Among 28 children, 19(67.9%) were male and 9(32.1%) female, male female ratio 2.1:1. Among the 28 children, ALL was in 24(85.7%), NHL 3(10.7%), and APML 1(3.7%). Among 28 children, anti-HBs titer was more than 10mIU/ml in all patients. But six month after initiation of chemotherapy, 8(28.57%) patients had anti-HBs titer less than 10mIU/ml. Hepatitis B antibody titre level significantly reduced after 6 months chemotherapy in children with hematological malignancies.

Pediatric Osteosarcoma of Extremities: A 15-year Experience From a Tertiary Care Cancer Center in Upper Egypt.

AIM: To assess the outcome and determine predictors of survival in pediatric patients with osteosarcoma of the extremities treated with a unified chemotherapy protocol at a single institution over a 15-year period. MATERIALS AND METHODS: We performed a retrospective analysis of medical records of 48 pediatric patients with histologically verified osteosarcoma of the extremities diagnosed at South Egypt Cancer Institute and received treatment between January 2001 and December 2015. RESULTS: With a median follow-up of 61 months for the entire cohort, estimates of overall survival (OS) for 3- and 5-year were 50.9% and 42.1%, respectively. While the estimates of OS for 3- and 5-year in the nonmetastatic group were 79% and 65.2%, respectively. In the multivariable analysis, both metastatic disease at diagnosis and poor response to chemotherapy retained their statistical significance as independent predictors for event-free survival. Whereas for OS, a metastatic disease at diagnosis remained as the lone predictor of a dismal outcome, while a poor response to chemotherapy became marginally associated with an inferior outcome. CONCLUSIONS: In Upper Egypt, whereas slightly less than two thirds of children with localized osteosarcoma of extremities survives their disease, metastasis at presentation remains the key predictor of dismal survival outcomes.

Skin Biopsy and Histopathological Diagnosis of Dermatological Diseases.

Microscopic examination of skin and mucous membrane is an established investigation in diagnosis and follow up of many dermatological conditions frequently encountered during practice. Histopathological examination is performed with biopsied specimen from skin and or mucous membrane where required. However neither do all skin biopsies produce neither a conclusive diagnosis nor the dermatologists routinely perform this procedure to every patient they consult. This cross sectional descriptive study conducted by reviewing the records of all biopsied cases studied for histopathology in Mymensingh Medical College, Mymensingh and Shaheed Mansur Ali Medical College, Dhaka, Bangladesh from January 2012 to December 2016. The aim of this study was to investigate the favourable clinical diagnoses set by dermatologists when performing skin biopsy, the diagnoses reached by the dermatopathologists after microscopic examination, and the relationship between them and finally to comment on the instances that skin biopsy fails to fulfill the diagnostic task. Fourteen hundred and thirty six (1436) cases of skin biopsy were reviewed and descriptive statistics were performed. Maximum cases 39.1% (562) were in 2nd decade with almost equal sex distribution. The most frequently proposed clinical diagnoses included papulosquamous dermatoses 28.6% (410), whereas histological confirmation was in 13.1% (188). Histological diagnosis was more as nonspecific dermatitis 31.6% (454). After microscopic examination, a specific histological diagnosis was found in 83.5% (1199) of the cases and a consensus between clinical and histological diagnoses was observed in 69.2% (994). Neither histological diagnosis nor any feature consistent with clinical suspicion was observed in 10.1% (145) cases. Histopathological examination of skin biopsy is still a valuable diagnostic tool in many skin diseases and dermatoses with diagnostic dilemma. There are cases in which diagnostic inefficiency are being faced even after skin biopsy examination.

A Rare Case of Metachromatic Leukodystrophy Confirmed by Arylsulfatase A.

Metachromatic leukodystrophy (MLD) is the rare neurometabolic disease caused by the deficiency of a lysosomal enzyme arylsulfatase A (ARSA) activity. The absence or deficiency of arylsulfatase a leads to accumulation of cerebroside sulfate within the myelinseath of the central nervous system (CNS) and the peripheral nervous system (PNS). This in turn causes the CNS and PNS to progressively deteriorate leading to both features of upper and lower motor neuron dysfunctions. Metachromatic leukodystrophy gets its name from the way cells with an accumulation of salfatides appear when viewed under a microscope. The salfatides form granules that are described as metachromatic which means they pick up colour differently than surrounding cellular material when stained for examination. The clinical features of brain dysfunction like gait disturbance, speech, hearing and visual problems appear gradually, become progressive and fatal over time. Our patient a 5 years and 6 months old developmentally normal boy presenting walking difficulty since his 2 years and 6 months which was gradually increasing. During this period he also developed speech difficulty, seizure followed by unconsciousness and severe respiratory distress for ten days. His investigations were suggestive of metachromatic leukodystrophy. There is no specific treatment to cure the disease. So proper counseling was done regarding the bad prognosis of the disease with symptomatic treatment.

Goldenhar syndrome - a case report.

Goldenhar syndrome is a developmental abnormality of 1st & 2nd branchial arch involving the craniofacial microsomia with ocular & vertebral abnormality. Though most of the cases are sporadic, some familial association is also found in autosomal dominant or recessive manner. Teratogenic effect of some toxic substances may lead to the condition. Ocular abnormalities are epibulbar dermoid, lipodermoid & coloboma. Otic defects are preauricular tags, microtia, anotia & conductive hearing loss. Cardio-pulmonary & genitourinary abnormalities are common associations. Here we have described the case of a 10 years old girl had ocular, auricular & vertebral changes consistent with Goldenhar syndrome, she was managed with multidisciplinary approach and she was symptomatically improved but corrective surgery was planned as schedule of respective department.

Neurologic manifestations, diagnosis and management of Wilson's disease in children - an update.

Wilson's disease (WD) is a genetic disorder of copper (Cu) metabolism. It is a progressive hepatolenticular degenerative disease due to toxic accumulation of copper in the various tissues particularly in the liver, brain and eyes. The neurologic manifestations of Wilson's disease are extremely varied like simple behavioral change such as irritability, depression, deterioration of school performance to severe form of neurologic presentations like dystonia, dysarthria, tremor and gait disturbance. Early diagnosis is possible by history of progressive neurologic dysfunction, clinical examination of Kayser Flescher rings (K-F rings) in eyes, along with some important investigations like low serum ceruloplasmin, high 24 hours urinary excretion of copper, presence of basal ganglia lesion in neuro imaging of the brain. Though hepatic copper estimation done by liver biopsy is the gold standard, is not available in Bangladesh. Most of the neurodegenerative diseases have no specific treatment and worse outcome. But it has a specific treatment with life long medication that reduces copper absorption or removes the excess copper from the body. Children on therapy must be monitored regularly for response, side effects and compliance. The aim of this article is to gather update information of neurologic manifestations of Wilson's disease and proper management as well to prevent the major neurological complications and better out come.

An unusual cause for a painful Birmingham hip resurfacing.

The painful total hip arthroplasty requires careful evaluation and investigation. This is usually focused on the prosthesis and adjacent anatomical structures. We present a case report of a 64-year-old man who had a Birmingham hip resurfacing procedure for primary osteoarthritis. His hip pain worsened following the procedure and was under systematic investigation for this. Subsequent investigation for vascular disease revealed a total infrarenal aortic occlusion. An aortobifemoral bypass improved the hip pain and function dramatically, and the patient now has an excellent quality of life.

Congenital hypertrichosis (Were Wolf Syndrome): a case report.

Hypertrichosis is abnormal increase in body hair, when it becomes extensive known as Were Wolf Syndrome. Any part of body can be affected and body hairs are longer and darker. Hairs may be of any type like lanugo, vellous or terminal. It may be present since birth or may occur later in life. A 8 years old boy was admitted in our hospital with excess body hair, he was diagnosed as a case of Were Wolf syndrome after excluding possible acquired causes of hypertrichosis. He had history of delayed developmental milestone and has been suffering from epilepsy. He was treated with developmental stimulation and anti epileptic drug. Then he was discharged after proper counseling.

Structural lesion in the brain is a risk factor of childhood intractable epilepsy.

Most of the intractable epilepsy cases are of paediatric age group. Eighty per cent of intractable epilepsy is symptomatic and have got structural lesion. These lesions are easily detectable by doing neuro-imaging. One hundred and four children with epilepsy were enrolled in two groups; in group A 52 intractable epilepsy patients and in group B 52 well controlled epilepsy patients from the child neurology unit of Bangabandhu Sheikh Mujib Medical University. Mean age of intractable group and well-controlled group was 4.45±3.66 years and 6.45±5.24 years respectively. Male were more in both groups. Abnormal neuro-imaging was significantly higher in intractable group; 78.8% children of the intractable epilepsy group had one or more structural lesions. Cortical atrophy, ventricular dilatation and encephalomalacia were the most frequent detectable lesions. Bilateral lesions and presence of multiple abnormalities were more in association with intractability. Structural lesions were detected more in cases with focal neurological deficit and structural abnormality having statistically significant difference.

Appendico-colic fistula complicating appendicitis in cystic fibrosis.

The case of a boy with cystic fibrosis who presented with an unusual complication of appendicitis is reported. Delayed presentation, with complications of appendicitis such as perforation and abscess formation, is not uncommon in cystic fibrosis; however, this case represents the first report of an isolated appendico-colic fistula following appendicitis in association with cystic fibrosis.