Adherence to Iron Chelation Therapy and Its Determinants.

Background: Thalassemia is a chronic disease requiring lifelong treatment. The adherence to regular iron chelation therapy is important to ensure complication-free survival and good quality of life. The study aim to assess the adherence to iron chelation therapy (ICT) in patients with transfusion-dependent thalassemia (TDT), evaluate various causes of non-adherence and study the impact of non-adherence on the prevalence of complications secondary to iron overload. Materials and Methods: Patients with TDT on ICT for > 6 months were enrolled in the study. Hospital records were reviewed for demographic details, iron overload status, treatment details, and the prevalence of complications. A study questionnaire was used to collect information on adherence to ICT, knowledge of patients, and the possible reasons for non-adherence.   Results:  A total of 215 patients with a mean age of 15.07+7.68 years and an M: F ratio of 2.2:1 were included in the study. Non-adherence to ICT was found in 10.7% of patients. Serum ferritin levels were significantly higher in the non-adherent group (3129.8+1573.2 µg/l) than the adherent population (2013.1+1277.1 µg/l). Cardiac as well as severe liver iron overload was higher in the non-adherent patients. No correlation was found between disease knowledge and adherence to ICT. Difficulties in drug administration and many medicines to be taken daily were statistically significant reasons for non-adherence. There was no difference in the co-morbidities arising due to the iron overload in the two groups. Conclusion: Nearly 11% of patients with TDT were non-adherent to ICT. Non-adherence results in higher iron overload.

An ICET-A survey on occult and emerging endocrine complications in patients with ß-thalassemia major: Conclusions and recommendations.

In adult thalassemia major (TM) patients, a number of occult and emerging endocrine complications, such as: central hypothyroidism (CH), thyroid cancer, latent hypocortisolism, and growth hormone deficiency (GHD) have emerged and been reported. As the early detection of these complications is essential for appropriate treatment and follow-up, the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) promoted a survey on these complications in adult TM patients, among physicians (pediatricians, hematologists and endocrinologists) caring for TM patients in different countries. The data reported by 15 countries are presented.The commonest endocrine complications registered in 3.114 TM adults are CH and GHD (4.6 % and 3.0 %, respectively), followed by latent hypocortisolism (1.2%). In 13 patients (0.41%) a cytological papillary or follicular thyroid carcinoma was diagnosed in 11 and 2 patients, respectively, and a lobectomy or thyroidectomy was carried out. Of 202 TM patients below the age of 18 years, the  reported endocrine complications were: GHD in 4.5%, latent hypocortisolism in 4.4% and central hypothyrodisim in 0.5%. Transition phase was an area of interest for many clinicians, especially as patients with complex chronic health conditions are responding to new treatments extending their lifespan beyond imagination.. In conclusion, our survey provides a better understanding of  physicians' current clinical practices and beliefs in the detection, prevention and treatment of some endocrine complications prevailing in adult TM patients. Regular surveillance, early diagnosis, treatment and follow-up in a multi-disciplinary specialized setting are recommended.

Chronic/recurrent cough in rural children in Ludhiana, Punjab.

OBJECTIVE: To determine the prevalence, age distribution and common causes of chronic/recurrent cough in rural children. DESIGN: Prospective study. SETTING: Pediatric population in five villages of Dehlon Block of Ludhiana, Punjab. METHODS: 2275 children in the age group of 1 to 15 years were screened by house to house survey for chronic/recurrent cough using defined criteria. A detailed work up of selected cases was carried out. Underlying etiology was determined using clinical and laboratory parameters. Five hundred children in the study population formed the control group. Variables associated with chronic/recurrent cough were analyzed in cases and controls. RESULTS: Twenty four children were diagnosed with chronic/recurrent cough showing a prevalence rate of 1.06 percent. The most common cause was bronchial asthma (66.7 percent) followed by postnasal drip syndrome (25 percent). Family history of allergy/asthma was noted in 11 (45.8 percent) children as compared to 52 (10.4 percent) in the control group (p < 0.01). Family history of smoking was recorded in 16.7 percent of cases in contrast to 6.4 percent in controls (p = 0.05). There was no significant association with overcrowding, pets and kind of cooking fuel used. CONCLUSIONS: The most common cause of chronic/recurrent cough was bronchial asthma. There was a significant association with family history of allergy/asthma and smoking.