Van der Knaap disease: a case report

Van der Knaap disease or megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare autosomal recessive degenerative disorder characterized by megalocephaly, cerebral leukoencephalopathy, and motor deterioration. Most cases reported with this disease are from our country India, belong to Agarwal community, who have high rates of consanguinity. We report a 4 and 1/2year old boy, with a history of delayed motor milestones, ataxia, increasing head circumference and abnormal body movements, who is belonging to the Bhat family of Handwara town of Kupwara district of Jammu and Kashmir, India.

Acute Oral Poisoning in Children in Kashmir

Introduction: Acute oral poisoning in children is potentiallyserious but a preventable cause of childhood morbidity andmortality. Most common age group involved is under fiveyears in particular toddlers. Objective of our study was tostudy the clinical profile of acute oral poisoning amonghospitalized children.Material and Methods: This is a retrospective data analysisof admitted/deceased patients spanning over a period of oneyear from March 2018 to Feb 2019.Results: The acute oral poisoning constituted 1.71% of totalpediatric admissions. The organophosphorus poisoning wasthe commonest followed by kerosene and rat killer poisoning.Conclusion: Organophosphorus ingestions are common acuteoral poisoning especially among fruit growing community inKashmir valley.

Role of Serum Prolactin Levels in Diagnosis of Epileptic and Pseudoepileptic Events in Children, A Tertiary Care Experience

Introduction: Transient elevation of serum prolactin levels occurs in immediate postictal period in true epileptic events only. Current study was aimed to investigate the role of postictal serum prolactin levels in differentiation of true seizures from pseudo epileptic events. Material and methods: A total of 110 patients of either sex between ages of 6 months to 12 years out of 676 cases admitted for seizure evaluation were enrolled in our study and divided into four groups. Group 1 included 30 children having frank seizures, group 2 included 30 children with typical febrile seizures, group 3 included 25 children with seizure mimics and group 4 included 25 children without seizures.The serum prolactin levels were quantitatively determined by using CoatA-Count Prolactin Immuno-radiometric assay (IRMA). All data was analyzed by standard statistical methods. Results: There were 64(58.2%) males and 46(41.8%) females with mean age of 60.8 and 61.3 months. The serum prolactin level was highest in group I with mean level of 25.5ng/ml ±10 SD(p value =0.00).The serum prolactin levels were raised in 30.3% (20/66) of patients in group I,II,and III when serum sample was obtained within 20 minutes and in none 0/19 (0.00%) when sample was taken after 20 minutes (p value =0.006).All patients with generalized tonic clonic seizures with abnormal EEG had abnormal prolactin levels while as only 83.3% with complex partial seizures and 20% with simple partial seizures had raised prolactin levels (p value =0.002). Conclusion: There was a significant rise in serum prolactin level in children with epileptic seizures as compared to febrile seizures and seizure like events.

Foreign Body Aspiration Masquerading Respiratory Tract Infection

Foreign body (FB) aspiration is a potentially life threatening event where prompt and precise action can turn tears into smiles. We report a case of an eight-year school going boy, with one-month old history of foreign body aspiration. The boy was treated as a case of respiratory tract infection. It was due to reappearance of symptoms and signs of chest infection supported by chest radiography that prompted for the CT-chest. It was followed by rigid bronchoscopy to confirm the therapeutic diagnosis of FB aspiration. This case report highlights the importance of detailed thoughtful history in pediatrics particularly to FB aspiration.

Clinicobacteriological profile, antibiotic sensitivity patterns and mortality of neonatal sepsis in a tertiary care hospital in Kashmir, Jammu and Kashmir, India

Background: Neonatal sepsis refers to generalized bacterial blood stream infection in first 28 days of life documented by positive blood cultures. It is one of leading causes of neonatal mortality. Objectives was to study clinicobacteriological, antibiotic sensitivity patterns and mortality of neonatal sepsis.Methods: This prospective study was conducted in the Department of Pediatrics of Government Medical College Srinagar in collaboration with Department of Microbiology of same medical college after ethical clearance from ethical committee of Government Medical College Srinagar. One hundred (100) neonates out of 731 neonates admitted between octomber2007 and September 2008 with signs and symptoms of neonatal sepsis were included in our study by random sampling method. After history, examination and laboratory investigation blood culture results were analyzed by standard statistical methods.Results: The blood culture was positive in 40% of neonates. Fifty one (51) neonates were males while as 49 were females. Sixty three (63) neonates had late onset of sepsis while as 37 had early onset sepsis. The positive' blood culture was more common in males, late onset sepsis, babies born in rural areas, home born, vaginal births, preterm and other' low birth weight neonates .The gram negative isolates were most common followed by positive ones .The best sensitivity of gram negative isolates was to ciprofloxacin followed by amikacin and cephalosporins while as gram positive isolates were sensitive to imipenum followed by vancomycin. Pseudomonas was most responsive to pipercillin +tazobactum combination. The neonatal mortality was 35% being higher in early onset sepsis and low birth weights.Conclusions: This study depicts a high rate of neonatal sepsis, mainly caused by gram negative organisms followed by gram positive organisms with rising drug resistance that could bear far reaching implications to the times to come, mandating the implementation of sepsis preventive measures and administration of specific antibiotics.

Mucocele of Concha Bullosa: A Case Report

MUCOELE in middle turbinate is rare occurrence. Most common site is frontal sinus. Next comes the ethmoid sinus. It occurs due to obstruction of drainage pathway of sinuses causing retention of secretion lead to its formation. It commonly present with very common complaints similar to other diseases of nose such as nasal obstruction and headache. Pre-endoscopic era it was very difficult to examine it properly, CT is the investigation of choice. It was successfully treated endoscopically.

Firearm Injuries of Head and Neck: Our Experience

Background: Injuries inflicted on head & neck  are of different types. It could be either blunt injury, sharp penetrating injury or gunshot injury. Multiple cranial nerve injury, airway injury, injury to bone and soft tissues are common outcome of such injury Methods: This study was a retrospective undertaken in the Department of ENT, JNMC, AMU over a period of five years from January 2000 to June 2005. In our present study we have exclusively dealt with firearm injuries inflicted on head & neck area and those patients who survived and were treated in our medical college. We studied ten cases of firearm injury. We had discarded all those cases of head & neck firearm assault who were brought dead. Results:  The study was conducted in the Department of ENT, JNMC over a period of five years. The age of presentation ranged from 15yrs-50 yrs. The male & female ratio was of 4:1. Most of the patients belonged either to areas east of Aligarh or Aligarh itself. Conclusion: The firearm injuries are now more common because of the development of sophisticated ammunition, intolerance and hatred. Timely intervention if given saves lifes of many patients. Team management and sound facilities are significant in the management of firearm injuries

​Concurrent Hyperfractionated Chemoradiation Versus Conventional Fractionated Chemoradiation With Low-Dose Weekly Paclitaxel In Locally Advanced Non–Small-Cell Lung Cancer In A Tertiary Cancer Center In Kashmir: A Prospective Study

Introduction: Lung cancer is the most common cancer worldwide and has a poor prognosis but integration of chemoradiationhas led to an increase in overall survival time and percentage of cured patients with acceptable toxicity.Purpose: The purpose of this study was to compare the efficacy of hyperfractionated (HFX) radiotherapy with conventionalradiotherapy and weekly concurrent paclitaxel in stage IIB/III non-small-cell lung cancer (NSCLC).Materials and Methods: A total of 60 patients were enrolled, of which 30 patients were given twice daily radiotherapy(1.2 Gy each) to a total of 72 Gy over 5–6 weeks and 30 patients were given single daily fraction (2 Gy) to a total of 66 Gy forthe same duration to achieve a comparable biological effective dose. Both groups received weekly 50 mg/m2 paclitaxel.Results: An overall response of 83.3% versus 56.6% with a partial response of 70% versus 53.3% and complete response(CR) of 13.3% versus 3% was seen in HFX radiotherapy versus conventional radiotherapy which was statistically significant(P = 0.04). 10 of 25 patients and 11 of 17 patients who achieved response in study and control groups, respectively, progressed.The median survival of patients in HFX radiotherapy arm was 18 months, compared to 9 months in conventional radiotherapyarm. The median time to local recurrence was 19 versus 11 months with local recurrence-free survival of 72% versus 66% at1 year follow-up. The 1 and 2 year survival rates were 76% and 40% in study arm and 50% and 26% in control arm (P - 0.005).Esophagitis (70% vs. 63.3%), skin reaction (70% vs. 63.3%), and radiation-induced pneumonitis (50% vs. 43.3%) were thecommon toxicities with no statistical significance between the two groups. Overall, there was mild chemotherapy-related toxicity.Conclusions: The combination of HFX radiation with weekly paclitaxel is effective treatment with a moderate degree of toxicityin stage IIB/III NSCLC. An average response to treatment and the use of lesser drugs have made us to consider this therapyin locally advanced NSCLC.

Otogenic Intracranial Complications - A Case Series

Background: Intracranial complications of otogenic origin is now a rarity. Advent of antibiotics are both blessings if used intelligently and curse if used indiscriminately especially by quacks. Antibiotic resistance and cross resistance have given adverse results. Methods: A total of twenty cases were studied in the Department of ENT at Jawaharlal Nehru Medical College, AMU, Aligarh, Uttar Pradesh Results: Maximum number of cases belonged to 10 to 20 years age group, i.e eight cases (40%) followed by six cases (30%) in the age group greater than 30 years, four cases (20%) in 20 to 30 years age group, and 2 cases (10%) below 10 years. Males were affected more than females in the ratio of 4:1. The commonest presenting symptom was otorrhea, headache, fever and hearing loss. About half of the patients had vomiting, some had vertigo, otalgia and one patient had facial weakness. Conclusions: Almost all the intracranial complications encountered were intensively and meticulously treated by medical and surgical exercise which gave excellent results with collective team approach.

TEES - A New Horizon

Background: Transcanal endoscopic ear surgery has many advantages over conventional microscopic ear surgery and is used as adjunct to microscope or is used independently. Precise large visual field and angular vision has edge over the clumsy, expensive, peeping tubular vision of microscopes. Moreover, it is a better teaching methodology for the undergraduates and postgraduates. Our study comprised of twenty-five patients who had safe ear and underwent transcanal endoscopic ear surgery (TEES). Methods: This prospective study was carried out between October 2014 to December 2015 at Hayaat hospital, Allahabad, Uttar Pradesh, India and Hind Institute of Medical Sciences, Safedabad, Barabanki. Twenty-five patients of age group more than fifteen years were selected. All the patients selected had dry and safe type of chronic suppurative otitis media (CSOM). Results: Of the twenty five people ten were females and fifteen males in the ratio of 2:3. All the patients selected had dry ear and safe type of chronic suppurative otitis media (CSOM). All had conductive hearing loss of variable decibels but not greater than fifty-five decibels. Conclusions: Further research is needed in the field of operative TEES. We all need to discuss and more research work is needed to prove its genuinity and practicality.

Oral approaches for benign lesions- a novel approach.

Introduction -Cosmetically acceptable surgical results are the demand of this modern era. Aesthetic facial look has lots of impact on financial, social and personnel life. Material and Methods- A total of twenty cases were studied in the Department of ENT at Hind Institute of Medical Sciences (HIMS), Barabanki, Uttar Pradesh. FNAC, USG and CECT were done and only benign lesions were selected which could be approached intraorally. Results-There were 20 patients aged between fifteen years to sixty years. There were ten cases of submandibular swellings, four cases of sebaceous cyst, three cases of lipoma and two cases of parapharyngeal space swelling and one case of plunging ranula. The mean operation time of the IOA group was significantly longer than that of the TCA group, but decreased gradually with surgical experience. The mean hospital stay of the IOA group was significantly shorter than that of the TCA group. Conclusions-The innovative surgical approaches were studied and meticulous, cosmetically acceptable results which were highly appreciated by the patients and their attendants were achieved. The stay in the hospital and complication rates were also quite low in intraoral approach than conventional external approach. Hence, intraoral approaches should be used for benign lesions which are amenable to surgery.

Urinary Tract Infection in Children.

We selected the pediatric urinary tract infection related research and review articles in English language using keywords or phrases such as Infant; children; culture; Escherichia coli; fever, UTI. Aims: The purpose of this review is to provide summary of the latest research in particular to the practical aspects of management of UTI in children. Background: Urinary tract infection (UTI) is an important medical entity commonly diagnosed during early childhood. Prevalence and incidence of UTI varies with age and gender. UTI can be missed just on history and clinical examination. Screening of UTI in high risk children is important and should be well balanced against cost and risk of missing UTI. Despite latest evidence from research there are still controversies in managing UTI in children. Conclusion: Despite major advances in management of UTI in neonates and children, uniform guidelines for the imaging and management of recurrent UTI are lacking, prompting a multinational large research project to fill in the knowledge gap.

Anemia in pregnant women in a rural block of Kashmir valley: Its prevalence and socio-demographic associates.

Background: Anaemia is a major public health problem in developing countries like India and is especially seen among women of childbearing age, during pregnancy and lactation. Nearly two-thirds of pregnant and one-half of non-pregnant women in developing countries have anaemia, which increases the risk of maternal and fetal mortality and morbidity. Aims & Objective: The study was designed to assess the prevalence of anaemia in pregnant women and to report the socio-demographic factors associated with anaemia during pregnancy. Material and Methods: This is a Cross sectional study. The study was conducted in Block Hajan, the rural field practice area under the department of community medicine, Sher-i-Kashmir Institute of Medical Sciences (SKIMS) Soura.482 pregnant women were examined and screened for anaemia. Haemoglobin level, age, education, income, type of family and parity were among the various variables studied. Statistical analysis was done by using Chi-square test using SPSS statistical package version (Chicago 11, IL). Results: A high prevalence (91%) of anaemia (Hb<11g/dl) was observed. The prevalence of anaemia was significantly higher in those aged ≥ 35 years. A highly significant association was found with woman’s age, parity, educational and socio-economic status. However, family type and birth interval were not significantly associated with anaemia. Conclusion: There is a high prevalence of anaemia among pregnant women and there is need for correcting this to improve the overall health of pregnant women.

Spectrum of Congenital Heart Diseases in Kashmir, India.

A retrospective analysis of case-records data of 53,653 patients (0-18 years) over a two and half year period was conducted to ascertain the spectrum of congenital heart diseases. Two hundred and twenty one patients were found having congenital heart diseases; a prevalence of 4.1/1000. Ventricular septal defect (VSD) was the most frequent lesion seen in 69 (31.2%), followed by patent ductus arteriosus (PDA) in 36 (16.3%) children. Tetralogy of Fallot (TOF) was the most frequent cyanotic heart disease seen in 17 (7.8%) patients.