Current Pediatric Research

Background: The most prevalent hemolytic anemia-causing single-gene disorder is Sickle Cell Disease (SCD). In a paediatric population, it is one of the often occurring reasons of repeated hospitalization, morbidity, and mortality. Limited information is available on the acute morbidity pattern and organ dysfunction in younger Indian children with SCD.

Methods: This prospective observational study was carried out in the tertiary care centre between January 2018 and June 2019. All of the patients ranged in age from six months to twelve years. In the study, cases of SCD that had previously been identified and had an SS pattern detected by High- Performance Liquid Chromatography (HPLC) or Hb electrophoresis were assessed for acute morbidity and multiple organ involvement.

Results: 178 children with SCD who had been identified by HPLC were included in the study. 108 of the children, or 60.68% of the total population, were between the ages of 5 and 10. Pain was the most prevalent presenting symptom, occurring in 123 (69.10%) instances. Severe pallor in 108 instances (60.67%) and splenomegaly in 48 cases (26.97%) were the other common presenting symptoms. Hospitalisation was brought on by a Vaso-Occlusive Crisis (VOC) in 114 (64.04%) of the patients, severe anaemia in 104 (58.42%), and acute febrile illness in 80 (44.94%). Out of the 178 patients admitted to the hospital for acute morbid events, it was found that 64 (35.95%) had multiple organ dysfunction. Hepatobiliary involvement was observed in 27 (42.18%) cases, splenic involvement in 12 (18.75%) cases, neurological involvement in 16 (25%) cases, Acute Chest Syndrome (ACS) involvement in 7 (10.93%) cases, and cardiac involvement in 2 (3.12%) instances, among other organs. In six (3.37%) cases, mortality was noted. One case of acute chest syndrome, two cases of stroke and seizure, two cases of sequestration crisis, one case of cardiac dysfunction and severe anaemia, and two cases each of stroke and seizure all resulted in death. 172 patients, or 96.63% of the total, were discharged.

Conclusion: The most frequent symptom in children under 18 is Vaso-Occlusive Crisis (VOC). A statistically significant relationship between organ failure and age in years was discovered (p=0.0181). The correlation between blood transfusions and hepatobiliary involvement was established, and the anticipated P-value was shown to be statistically extremely significant (p=0.0001).

Author(s): Lakshmikant A Rohadkar, Dipak Madavi, Bhagyashree Tirpude, Soumya Bilawad, Vaibhav Adgokar, Shamama Subuhi, Milind M Suryawanshi

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