Clinical and haematological profile of children with sickle cell anaemia admitted to a rural medical college of Chhattisgarh, India

Abstract

Background: Sickle cell disease (SCD) is the most common single gene disorder resulting in hemolytic anemia. Aim of the study was to describe the clinico-haematological profile of children with sickle cell anaemia admitted to Paediatric ward/PICU with any acute clinical event and to find out the association between high HbF level, frequency of crises episodes and requirement of blood transfusion in sickle cell anemia. Methods: Hospital based descriptive study. Retrospective data analysis was done from medical records of patients between 0-15 years age group admitted to hospital from March 2014 to August 2017. Results: Total 68 clinical events were recorded in 60 patients during the study period. More than half of the children were in 0-5 years age group. Mean age of diagnosis was 2.79 years. Severe anemia requiring blood transfusion was the most common cause of hospitalization followed by painful crises. Mean Hb level in the children was 6.65(±2.38). More than one third of children had associated nutritional anemia. Children with high HbF level were found to have less number of painful crises episodes. Conclusions: Severe anemia followed by painful crises were the commonest presentations requiring admission in our hospital. Chronic anemia with microcytosis and high HbF level is the hematological profile of the present study group. Children with high HbF level suffered from less number of painful crises episodes when compared to children with low HbF. But the requirement of blood transfusion was similar in both groups.

Prevalence, Pattern of Disease and Outcome of Children with Sickle Cell Disease Admitted in a Private Health Facility in Southern Nigeria

“…In the present study, about half (50.8%) of the children with SCD admitted were of age group  5 years which was consistent with reports by George & Opara [15] Faruk et al [19] and Nayak et al [21] in a previous study in Port Harcourt, Zaria in Nigeria and India respectively. Ikefuna and Emodi [16] in Enugu, Nigeria reported age group 1-5 years as the commonest age group admitted whereas Jain et al [3] in India reported 1-< 3 years as the commonest age group implicated.…”

“…Males predominated in the present study as similarly documented in other studies in Nigeria [10,[15][16][17]19,20], Malawi, [14] India [3,21] Iraq [18] and Barbados [22]. Although SCD is an autosomal recessive disorder and as such not related to gender, Gladwin et al [23] and Ilesanmi [24] in their studies showed that nitric oxide was found more in females than males during both steady state and crisis which thus indicates that males could be more prone to crisis and attendant complications.…”

“…It is worthy of note that it is a regular practice to commence all children diagnosed with SCD in the present study centre on prophylactic penicillin. Acute painful crisis/VOC which was 2 nd commonest in the present study was reported as the commonest morbidity in other parts of Nigeria [10,15,19,20], Saudi Arabia [1], Iraq [18] and Barbados [22] whereas severe anaemia was the commonest in Malawi [14] and India [21] while malaria was the commonest in Ibadan [17] and a previous study in Port Harcourt [15] Nigeria. Malaria being the 3 rd commonest cause of hospitalization in the present study, a private health facility could be attributed to the possibility of less exposure to the mosquitoes, better housing and better compliance to malaria chemoprophylaxis.…”

“…This was consistent with other studies [14,17] which observed priapism as the least common morbidity. In contrast, priapism was not documented in some other studies [3,10,15,18,21]. This is not surprising as priapism is an uncommon event, mainly seen in much older children above 15 years [9].…”

Significance of genetic modifiers of hemoglobinopathies leading towards precision medicine

“…Mpalampa et al 16 considering the mean HbF cut-off as 10%, in 216 sickle cell anemia patients observed a strong negative correlation of HbF levels with the total number of transfusions (r = − 0.181, P: 0.004), hospitalisations rate (r = − 0.173, P: 0.006), and significant positive correlation with the age at diagnosis (r = 0.151, P: 0.013) 16 . In the Indian context, Nayak et al 17 studied 60 sickle cell anemia patients and observed fewer episodes of painful crises in children with high baseline HbF level as compared to children with low HbF level 17 . Correspondingly in our study as well, the mean age of diagnosis among 100 SCA patients was found to be 6.3 ± 5.2 years which is very much delayed as compared to the patient cohort studied by Mpalampa et al 16 .…”