Dear editor,
Studies report clinical heterogeneity in sickle cell anaemia (SCA) cases in India [1, 2]. Although sickle hemoglobin has been reported from Rajasthan, clinical information is limited [3, 4]. We report case series analysis of eight cases of SCA among tribal school children from Udaipur, Rajasthan. Fresh 20 µL capillary blood was drawn from tip of left little finger. Sickle haemoglobin was detected by solubility test using sodium dithionite. Solubility test-positive cases were further subjected to haemoglobin electrophoresis for diagnosing SCA. Subsequently, diagnosed SCA cases were assessed clinically by local medical officer well versed in tribal dialect. Medical history taking and clinical examination was done as per standard medical procedure.
Total 182 children were screened, in whom 35 were solubility positive. Out of these 35, eight children were diagnosed with SCA cases. Among 8 SCA cases, 5 were boys. Detailed data on lifetime history of 12 clinical events, past 15 days’ history of 6 clinical events, and 16 current findings on general and systemic clinical examination are provided in ‘Supplementary file’. Only Case 5 had lifetime history of being hospitalized. Case 1 and Case 3 had reported the history of leg ulcers. Case 4 had a positive past 15 days history of severe cough/chest pain and joint pain/problem with walking and joint movement. Case 5 reported a past 15 days’ history of abdominal pain/distension. Table 1 describes the selected clinical findings from the collected data. None of the studied cases was severely anaemic, and haemoglobin levels ranged from 8.6 to 11.8 gm/dl.
Table 1.
Findings on clinical examination and medical history taking among sickle cell anaemia cases, Rajasthan, India
| Findings on clinical examination and medical history | Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | Case 6 | Case 7 | Case 8 | |
|---|---|---|---|---|---|---|---|---|---|
| Current clinical findings on medical examination | Breath rate/minute | 16 | 16 | 16 | 20 | 20 | 16 | 15 | 16 |
| Pulse rate/minute | 74 | 72 | 72 | 90 | 84 | 72 | 85 | 92 | |
| Haemoglobin gm/dl | 8.6 | 11.3 | 11.8 | 10.2 | 10.1 | 9.6 | 10.8 | 11 | |
| Past 15 days history of clinical events | Abdominal pain, distension | No | No | No | No | Yes | No | No | No |
| Severe cough, chest pain | No | No | No | Yes | No | No | No | No | |
| Joint pain and problems with walking and joint movement | No | No | No | Yes | No | No | No | No | |
| Ever history of clinical events | Ever history of hospitalization | No | No | No | No | Yes | No | No | No |
| Ever history of leg ulcer | Yes | No | Yes | No | No | No | No | No | |
The majority of cases were clinically asymptomatic with respect to ever history of 11 clinical events studied. Two cases, Case 1 and Case 2, had a history of leg ulcers. Past 15 days’ history concerning 6 clinical events was negative in 7 out of eight studied cases. Only Case 4 had affirmative past 15 days’ history with respect to severe cough/chest pain and joint pain. On clinical examination, none of these studied cases had shown positive findings of 13 clinical signs and symptoms. The majority of these cases had never been admitted to hospital. On studying the past 15 days’ history of clinical events and findings of clinical examination, we find these cases to be relatively mild clinically.
SCA patients of the non-tribal groups of Nagpur exhibit a severe clinical phenotype. Study reported ever history of hospitalization and blood transfusion among 90% and 76% of patients, respectively [1]. Whereas SCA cases from western India showed milder clinical presentation [2]. Available studies on SCA in tribes of Rajasthan have reported range of clinical complaints. Study in Sirohi, reported clinical complaints like pallor, recurrent fever, weakness, musculoskeletal pain, and splenomegaly [3]. Another study in Sirohi and Udaipur reported pain, pallor, weakness, fever, splenomegaly, and hepatomegaly [4]. However, in this study, most of the diagnosed cases of SCA did not report any clinical complaints in the past or present. Our findings are in contrast to the earlier published hospital setting studies in tribes of Rajasthan [3, 4]. This variation may be due to the bias in selection of participants. Other possible reasons can be foetal haemoglobin concentration, and environmental factors [2, 5]. However, findings of the study should not be generalized because of less sample size.
Supplementary Information
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Footnotes
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References
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