scholarly journals Hemoglobin Patterns in Sickle Cell Hemoglobinopathies- A Large Prospective Study in North Maharashtra

2017 ◽  
Vol 4 (1) ◽  
pp. 84 ◽  
Author(s):  
Manjusha Punjaji Tambse ◽  
Maya Suresh Vasaikar ◽  
Sunil Santaram Chavan

Introduction: Sickle cell haemoglobinopathy is an inherited hemoglobinopathy resulting from a mutation occurring in betaglobin gene, on chromosome 11. The gene is prevalent in some tribes of North Maharashtra. The main aim of the study is to determine haemoglobin patterns in cases with sickle cell hemoglobinoathies in North Maharashtra using HPLC testing system. Material and Methods: This is a prospective study done over a period of 6 years. 10081 patients having positive solubility test or negative solubility test but having clinical suspicion of Sickle cell hemoglobinopathies were studied in detail and all samples were subjected for HPLC testing. Results: Prevalence of sickle cell hemoglobinopathy in this study was 70.36%. Most common pattern of haemoglobin observed was SA (89.72%). A slight female preponderance (54%) was noted. Predominant age group was paediatric (39.96%), followed by12-20yrs (33.97%). Oldest case for HbSS was 55yrs male. Predominant category affected was ST (82.05%). Conclusion: A very high prevalence of Sickle cell hemoglobinopathy was noted in this study. This is because the study was done in areas where Pawara and Bhill community resides who have a high frequency of HbS gene. Solubility test was found to be cost effective and easy screening test (Sensitivity being70.36%). HPLC found to be Rapid and accurate test for diagnosis of hemoglobinopathy and had helped in diagnosis of some rare heterozygous disorders like SA-HBQ India, SA-Hereditary persistence of foetal haemoglobin, HBD-SA. This is one of the largest and first of its kind prospective study which will help in prevention and cost effective management in targeted population.

Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 84
Author(s):  
Jeanne Sigalla ◽  
Nathalie Duparc Alegria ◽  
Enora Le Roux ◽  
Artemis Toumazi ◽  
Anne-Françoise Thiollier ◽  
...  

The majority of hospitalizations of patients with sickle cell disease (SCD) are related to painful vaso-occlusive crises (VOCs). Although the pain of VOC is classically nociceptive, neuropathic pain (NP) has also been demonstrated in SCD patients. The aim of our study is to specify the prevalence of NP during VOCs in SCD children using a dedicated scale and to measure its characteristics. We performed a prospective study that included SCD children hospitalized for an acute VOC. The presence of NP was sought with the DN4 scale on the second and fourth days of hospitalization. A total of 54 SCD children were included in the study. Overall, 41% of the patients (n = 22) experienced neuropathic pain during the VOC, mostly at an early stage (Day 2). The median age, the sex ratio, the location of the pain, and the morphine consumption were similar for patients with and without NP. Our study shows that neuropathic pain is very common during VOCs in SCD children. The absence of identified risk factors should prompt us to be vigilant regardless of the patient’s age, sex, and clinical presentation.


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Piyawat Komolmit ◽  
Vinita Oranrap ◽  
Sirinporn Suksawatamnuay ◽  
Kessarin Thanapirom ◽  
Supachaya Sriphoosanaphan ◽  
...  

1992 ◽  
Vol 71 (12) ◽  
pp. 643-651 ◽  
Author(s):  
L.E. Leguire ◽  
Robyn D. Fillman ◽  
Deborah R. Fishman ◽  
Don L. Bremer ◽  
Gary L. Rogers

In a prospective study of 505 hearing impaired and deaf students conducted at Children's Hospital Eye Clinic, 48.7% were found to have significant ocular abnormalities. The prevalence of myopia, astigmatism, and pathological intraocular changes was found to be significantly increased over the general population. In addition, the prevalence of ocular abnormalities generally increased with the severity of the hearing loss. Of importance was the high prevalence of rubella-consistent abnormalities in the hearing impaired and deaf student populations. Rubella syndrome may be responsible for a major portion of high refractive errors and ocular pathological changes. It is recommended that ENT specialists and pediatricians be aware of the nearly 50% chance of ocular abnormality in hearing impaired and deaf students, and that ophthalmologic examination may be valuable for identification or confirmation of the etiology of hearing impairment.


2016 ◽  
Vol 55 (4) ◽  
pp. 390-395 ◽  
Author(s):  
Salam Itani ◽  
Asma Arabi ◽  
Dana Harb ◽  
Diana Hamzeh ◽  
Abdul-Ghani Kibbi

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