Newborn Screening for Sickle Cell Disease Among Tribal Populations in the States of Gujarat and Madhya Pradesh in India: Evaluation and Outcome Over 6 Years.

Sickle cell disease (SCD) poses considerable public health problems in India. This study was undertaken to understand the clinical course of SCD among children identified during newborn screening programmes in Gujarat and Madhya Pradesh where the frequency of the HbS gene is high. A total of 8,916 newborn babies 8,411 from Gujarat and 505 from Madhya Pradesh were screened over 6 years (2010-2016) using HPLC and the diagnosis was confirmed by molecular analysis in a subset. A total of 128 babies (122 Gujarat, 6 Madhya Pradesh) were identified with sickle cell disease, of whom 87 (69 HbSS, 18 HbS-ß thalassemia) from Gujarat were followed for 0.5-6.6 years. Acute painful events, severe anemia and fever with infections were the major complications and 23 babies required hospitalization. Severe to moderate clinical presentation was found in 13.8% babies with SCD whereas, 86.2% babies had a milder presentation. Presence of ameliorating factors (α-thalassemia and Xmn 1 polymorphism) did not have a discernible effect on the clinical severity. Parents of babies with SCD were educated and counseled for home care. Distribution of mobile phones to 44 families having babies with SCD was beneficial as it allowed regular contact with patients and their families. Genetic counseling to the affected families has increased the awareness and acceptance for prenatal diagnosis and 18 couples opted for prenatal diagnosis in subsequent pregnancies. SCD is not always mild among tribal groups in India. Therefore, facilities for early diagnosis and prophylactic treatment in the tertiary care centers should be made available. The difficulties in regular follow up of the babies in remote rural areas have also been highlighted.

Effect of Combined Therapy Using Diode Laser and Photodynamic Therapy on Levels of IL-17 in Gingival Crevicular Fluid in Patients With Chronic Periodontitis.

Introduction: The aim of this study was to evaluate the efficacy of combination therapy of diode laser and photodynamic therapy (PDT) as an adjunct to scaling and root planing (SRP) on interleukin-17 (IL-17) levels in gingival crevicular fluid (GCF) in patients with chronic periodontitis. Methods: Thirty subjects with chronic periodontitis were included. All teeth received periodontal treatment comprising of SRP. Using a split mouth study design, the test group was additionally treated with a combination therapy of diode laser and PDT. GCF was collected to evaluate IL-17 levels at baseline and 3 months. Results: There was no difference in baseline values for levels of IL-17 in GCF in the test group and the control group. A significant decrease in GCF levels of IL-17 was observed in both treatment groups 3 months after treatment (P < 0.001). However, the treatment groups showed no significant difference (P > 0.05). Conclusion: Based on the results of the present study it was concluded that, GCF levels of IL-17 changed significantly after treatment regardless of treatment modality.

Transfusion in sickle cell disease: experience from a Gujarat centre.

OBJECTIVES: Following impressions that the use of blood transfusion in sickle cell disease may be inappropriately high, transfusion practice at a major blood bank in an area of high prevalence of sickle cell disease was assessed. METHODS: Retrospective review of blood usage in sickle cell disease at a major blood bank in south Gujarat in 2010 was conducted with prospective more detailed data collection over 18 wk period (April 7 through August 15) in 2011. The results were compared with transfusion usage in the Jamaican Sickle Cell Clinic. RESULTS: In 2010, this blood bank processed a total of 19,037 units of which 384 (5.2 %) units were for patients with sickle cell disease. Median transfusion use was 1 unit but 16 patients (4.2 %) of those transfused received 10 units or more and five patients received over 20 units. More detailed prospective analysis revealed that most transfusions occurred between ages 5-15 y, 40 % of subjects had pretransfusion hemoglobin levels below 6 g/dL, symptoms were generally vague such as fever, bone pain, weakness and that 26 % denied any specific symptoms. CONCLUSIONS: Transfusion usage greatly exceeds that in the Jamaican Sickle Cell Clinic. Transfusion therapy carries risks and cost and more detailed investigation and diagnosis of anemic episodes is necessary to define the role of transfusion among other potential therapies. Eventually, guidelines evolved by Indian specialists should determine the indications for transfusion in sickle cell disease.