Cerebral hemodynamics in children with sickle cell disease in India: An observational cohort study.

India has the second highest number of cases of sickle cell disease (SCD) and affects the most socioeconomically disadvantaged communities living in a horizontal belt from Gujarat to Odisha state. Despite high prevalence, information about cerebral hemodynamics among children with SCD in India remains scarcely described. We performed transcranial Doppler (TCD) to assess cerebral hemodynamics among Indian children with SCD and evaluated their association with clinical and hematological parameters. Children aged 3-18years, diagnosed with SCD living in Raipur in Chhattisgarh and Ahmedabad in Gujarat state were recruited. TCD was performed to obtain flow velocities from middle cerebral (MCA), intracranial internal carotid (ICA) and basilar artery. Associations were evaluated between timed-average-mean-maximum velocities (TAMMV) and various clinical and hematological parameters. Our prospective study included 62 consecutive children with known SCD. Mean ± SD age of the study population was 9.8 ± 3.9 years and 31 (50%) were male. Mean ± SD hemoglobin was 8.64 ± 1.34 Gm/dL while the mean HbSS ± SD was 70.25 ± 15.27%. While 6 (9.6%) children had suffered from stroke during previous 2 years, 7 (11%) demonstrated abnormal TAMMV. Higher HbSS level along with history of iron chelation therapy, blood transfusion and/or stroke showed a trend towards having higher TAMMV. Stroke and cerebral hemodynamic alterations are common among Indian children with SCD. Larger studies with detailed neuroimaging and genetic evaluations are needed for better understanding, characterization, risk stratification as well as optimization of the timing of blood transfusion to reduce physical disabilities among Indian children with SCD.

The Bone Pain Crisis of Sickle Cell Disease and Malaria: Observations from Gujarat, India.

BACKGROUND: Sickle cell disease is a common problem across central India, but its clinical features may differ from that in African populations. There is a need to define the features of sickle cell disease in India, and the current study addresses some features of the bone pain crisis. OBJECTIVES: The objective of the study was to describe the epidemiology of the bone pain crisis of sickle cell disease in Gujarat and explore the relationship with infection by Plasmodium vivax. MATERIALS AND METHODS: This was a prospective review of all admissions in patients with sickle cell disease to a private pediatric institution in Bardoli, Gujarat, in the year 2015. Hemoglobin electrophoresis of all patients was consistent with homozygous sickle cell disease, but family studies indicated that at least seven cases had the severe sickle cell-beta + thalassemia presumed to be the common IVS1-5G>C mutation. Clinical, hematological, and parasitological features were recorded. RESULTS: There were 914 admissions among 654 patients who had between one and seven admissions. The bone pain crisis accounted for 763 (83%) of admissions and increased between July and October coinciding with the monsoon period. Blood smears were examined for malarial parasites in 811 admissions and were positive for P. vivax in 73% patients. There was no evidence that P. vivax infections varied with the cause of admission or increased during the monsoon period. CONCLUSIONS: There was a high prevalence of P. vivax infection in hospital admissions of sickle cell patients, but the data did not support an etiological role in the bone pain crisis. A trial of malarial prophylaxis might determine its effect on the clinical features and outcome of sickle cell disease.

Screening for the sickle cell gene in Gujarat, India: a village-based model.

The sickle cell gene in India reaches its highest prevalence among the tribal people, many of whom are marginalized in the Indian society, living in remote rural areas which are often in the hilly regions of the Deccan plateau. Delivery of all services including health care presents special challenges which are addressed in this study by an outreach program and a mobile clinical unit. Another concern among the tribal people, a suspicion of centrally provided services conceived as being imposed from the outside, has been addressed by the concept of the Sickle Cell Swa (self) Suraksha (protection) Abhiyan (movement), which seeks to educate tribal communities in sickle cell (SS) disease so that the request for screening emanates from the community itself. This program has now screened 7,307 subjects in nine villages, finding the sickle cell trait in 23.7 % (range 18.5-30.9 %) and probable SS disease in 112 subjects. The organization of the program is described along with the delivery of results on a laminated card displaying the hemoglobin genotype, advice related to the genotype, blood group information (specifically requested by the villagers), contacts within the village sickle cell committee, and clinical contacts for medical advice. In addition, a local villager has been given basic health care training to regularly visit and monitor cases of SS disease and refer those with significant complications to the hospital coordinating the screening program. It is too early to determine the success of this program, but it represents a village-based model of detection of the sickle cell gene and care for cases with the disease which is accepted by the affected communities and may have broader implications for sickle cell disease in India.

Results of posterior dislocation of elbow associated with bony and soft tissue injury.

UNLABELLED: Elbow trauma is challenging to manage by virtue of its complex articular structure and capsuloligamentous and musculotendinous arrangements. We included 17 patients with elbow dislocation and associated injuries in this study. The study protocol included early elbow reduction and planned fixation of the medial or lateral condyle, coronoid and radial head. The sample was 73% male and 27% female with mean duration follow-up of 8 months, and mean age of 37 years. The mean Mayo Elbow Performance Score was 96 points at conclusion of follow-up, indicating an excellent result in 14 patients. Whenever the radial head was excised, we performed a strong transosseous ligamentous repair of the medial and lateral collateral ligaments. Fixation of the coronoid is essential for elbow stability. A small avulsed fragment can be fixed using an ACL jig. We found this technique very useful. Early planned intervention, stable fixation, and repair provide sufficient stability and enhance functional outcomes. KEY WORDS: elbow dislocation, ligament instability, coronoid fracture.

Evaluation of the utility of the Ponseti method of correction of clubfoot deformity in a developing nation.

Clubfoot is the commonest congenital deformity in babies. More than 100,000 babies are born worldwide each year with congenital clubfoot. Around 80% of the cases occur in developing nations. We treated 154 feet [mean Pirani score (total) 5.57] in 96 children (78 males, 18 females) by the Ponseti method from January 2003 to December 2005. A prospective follow-up for a mean duration of 19.5 months (range 6-32 months) was undertaken. After six months of treatment the Pirani score was reduced to zero for all patients. The results show that corrective surgery, sometimes multiple, can be avoided in most cases which are usually associated with the development of a stiff, painful foot. Low socio-economic status and illiteracy prevailing in developing nations increases the prevalence of neglected clubfoot that is still harder to correct. Integration into various programs and proper use of available resources can decrease neglected clubfoot and improve chances of successful and timely correction of deformity. Bracing constitutes an important part of treatment and proper motivation and education of the parents mitigates the chances of losing correction. The Ponseti method of correcting clubfoot is especially important in developing countries, where operative facilities are not available in the remote areas and well-trained physicians and personnel can manage the cases effectively with cast treatment only.