Hearing Loss in Thalassemic Children on Chelation Therapy

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.

Echocardiographic Findings In Thalassemia Major Patients: An Experience From Jammu

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.

Scrub Typhus in Children: Jammu Experience.

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.