ABSTRACT
Background: Thalassemia is a common genetic hematological disorder worldwide. It is also common in North India includingJammu region. These patients need lifelong repeated blood transfusions and iron chelation therapy for their survival. Chelationtherapy is known to be associated with various complications including sensorineural hearing loss (SNHL). Till now, no dataare available regarding SNHL in pediatric thalassemia major patients in Jammu region. Hence, we planned a study to assessthe prevalence of hearing loss in children with thalassemia major in the age group of 10–20 years.Methods: All the children with beta-thalassemia major in the age group of 10–20 years registered with Thalassemia Day CareCenter, Department of Pediatrics, SMGS Hospital, Government Medical College, Jammu, were enrolled in this cross-sectionalstudy. Hearing was assessed by pure tone audiometry. Clinical and demographic data of these patients were recorded onpretested pro forma and analyzed.Results: A total number of 34 children with thalassemia in the age group of 10–20 years were enrolled in this study whichcomprised 18 males and 16 females. Out of these 34 patients, 5 (14.7%) were found to have SNHL and 1 (2.9%) had conductivehearing loss. Four of the five patients in SNHL group had low- as well as high-frequency mild hearing loss (25–40 db) whileone patient had high-frequency mild hearing loss at 4000 HZ. Four out of these five patients had unilateral hearing loss on theleft side while one had bilateral SNHL. Two out of five patients in the SNHL group were taking chelation therapy in the form ofcombination of deferiprone and deferasirox at the dose of 75–100 mg/kg/day and 30–40 mg/kg/day, respectively, for more than5 years. The other three patients were taking only deferasirox at the dose of 30–40 mg/kg/day for more than 5 years.Conclusions: Regular blood transfusions and chelation therapy are essential for long-term survival of thalassemia majorpatients but are also associated with complications like SNHL.
ABSTRACT
Background: Cerebral palsy (CP) is one of the leading causes of childhood disability worldwide with the greatest burdenfound in developing countries. Motor impairments are the hallmarks of CP, but in many individuals, other impairments suchas vision, hearing, speech, cognition, behavior, and epilepsy may at times produce even greater activity limitation in daily life.Early diagnosis and comprehensive management with a multidisciplinary approach are required for satisfactory managementof a child with CP. In low- and middle-income countries, there are gaps in knowledge especially in spheres of epidemiologicalresearch, intervention, and service utilization.Materials and Methods: This cross-sectional observational study was conducted among the children coming to the inpatientand outpatient Department of Paediatrics SMGS Hospital, Government Medical College Jammu.Aims and Objectives: The aim of the study was to study the clinical pattern and etiological factors of CP and to determine the prevalenceof associated disabilities or handicaps in CP. Our study population included 100 cases of diagnosed CP up to 18 years of age.Results: A total of 100 children of CP were evaluated of which 59% were boys and 41% were girls. CP patients belonged tovarious age groups as, <2 years (46%), 2–4 years (30%), 4–6 years (12%), 6–12 years (10%), and 12–18 years (2%). CP patientscoming to our hospital belonged to various districts such as Jammu (27%), Rajouri (20%), Doda (17%), Reasi (11%), Udhampur(8%), Kathua (7%), Poonch (5%), and Samba (5%). The most common etiological factors were birth asphyxia (48%). The spastictype was the most common (65%), followed by ataxic (15%), dyskinetic (10%), and mixed (10%). Among the spastic quadriplegic,subtype was seen in 69%, diplegia in 23%, and hemiplegia in 8%. Speech delay was the most common associated problem(80%), followed by seizures (56%), feeding difficulty (46%), and contractures and deformities (20%). Formal vision assessmenthad been done in only 48% of the patients, among them, 30% had normal vision, 9% had refractive error, 7% were havingstrabismus, and 2% were blind. Spectacles were being used by only three patients. A mere 30% of the patients had undergonehearing assessment; among them, 6% were having moderate to profound hearing loss. Hearing aid was being used by twopatients. Physiotherapy services were being availed by 47% of patients. Early intervention and appropriate rehabilitation servicesshould be provided to such children to limit the disability. Medical college hospitals of India, where a number of these childrenreport with their various problems, can play an important role as nodal centers for evaluation and registration of such patients.
ABSTRACT
Current Study was done to assess the echocardigraphic changes in Thalassemia Major patients. All Thalassemic Major patients above 10 years of age registered with Thalassemia day care centre at SMGS hospital, Government Medical College Jammu with normal electrocardiogram and no clinical features of heart failure were included in this retrospective study. Their medical records were analyzed in terms of age, gender, average hemoglobin levels, serum ferritin levels and echocardiographic findings. 95 patients who were more than 10 years of age and had normal electrocardiogram and no clinical features of heart failure were included in this study. Out of these 95 patients 55 showed abnormal findings on echocardiography. All patients except one showed normal systolic function. Echocardiography in 54 patients showed only diastolic dysfunction and one had tricuspid regurgitation (TR) in addition . 55 Thalassemia major patients with abnormal echocardiographic findings comprised of 30 males and 25 females. All the patients in the age group of more than 20 years and 12 patients in the age group of 10 years to 20 years showed abnormal echocardiographic findings. Average hemoglobin and serum ferritin in these children were around 5-7gms /dl and more than 3000 ng/ml respectively. Echocardiographic findings suggestive of left ventricular diastolic dysfunction were the commonest findings indicating early cardiac dysfunction in patients with Thalassemia major in 2nd and 3rd decade of their life.
ABSTRACT
Snake bite is a common neglected emergency in children. Due to lack of adequate epidemiological data its incidence is underestimated. This study analyses the clinical profile of neurotoxic snake bites in children. Retrospective analysis of 37 children with features of neurotoxicity with or without history of snake bite was done. 37 cases of neurotoxic envenomation had a median age of 8 years. 30 were males. Majority of bites occurred during night hours and in upper limbs. Among the 17 who were bitten during night 12 were sleeping on floor. The most common systemic presentation was ptosis (94.59%) followed by difficulty in breathing (78.37%), pain abdomen (64.86%) blurring of vision and diplopia (54.04%), decreasing level of consciousness (45.94%) and vomiting (29.72%). 13 patients (35.13%) with clinical features of neurotoxic envenoming but without any history of snake bite were brought with history of early morning sudden onset of abdominal pain. All patients were given Anti Snake Venom (ASV) with medial dose of 600 units. Twenty patients received ventilator support. Ten (27%) patients died all of whom received ASV after a delay of at least 5 hours.
ABSTRACT
We report a 12 year old girl with snakebite, who developed hemothorax 5 days after admission. One liter of blood was aspirated. The bite was presumed to be that of saw scaled viper (Echis carinatus) that resulted in DIC and direct endothelial injury leading to bleed. Selective bleed into the pleural cavity is a rarity.
ABSTRACT
The present retrospective study describes clinical profile of the children hospitalized in SMGS hospital with Scrub Typhus infection in Jammu during period Oct 08 to Sept 09. 21 hospitalized children with clinical features suggestive of Rickettsial disease (fever, generalized rash) who tested ELISA positive for IgM against Scrub Typhus were included in the present study between the age of 5 years and 18 years. 52.4% children belonged to Sunderbani-Naushera - Rajouri belt and majority of them reported during the months of August to November. All children presented with fever and maculopapular rash. 61.9% had lymphadenopathy, 57.12% had edema and 46% patients had conjunctival congestion. 76.13%, 23.8%, 19.04%, 9.2% had hepatosplenomegaly , pain abdomen ,altered sensorium and gastrointestinal bleed respectively. All of the 21 children were treated with chloramphenicol. None of them died. Rickettsial infection is quite common in Jammu region and thus, high degree of suspicion, knowledge of geographical distribution and clinical features of Rickettsial disease helps in its early diagnosis and treatment.
ABSTRACT
The present study was conducted in all neonates < 28days of life who were hospitalized in the neonatal division, Department of Pediatrics, SMGS hospital, Govt. Medical Collage Jammu, over a period of 6 months from Dec. 2000 to May 2001 to assess the prevalence of neonatal seizures. The study revealed a seizure prevalence in hospitalized neonates of 19.2% with male sex preponderance. 71.4% neonates had early onset serizures.
ABSTRACT
We report a 11 year old boy and his father both Noonan’s. Noonan syndrome occurs in 1 out of 2000 live births. Short stature, webbing of neck, pectus carinatum or pectus excavatum, hypertelorism cubitus valgus, epicanthus, downward slanted palpebral fissures, ptosis, microganthia and ear abnormalities are the common features of Noonan syndrome.