DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20172609

A study of clinical and hematological profile of children with sickle cell disease in a tertiary care hospital, Valsad, India

Kinjal G. Patel, Chintu Chaudhari, Diwakar Sharma

Abstract


Background: Sickle cell disease is commonly seen in rural population of south part of Gujarat in India. It is one of the common causes of recurrent hospitalization, morbidity and mortality in pediatric population. This study was therefore undertaken to evaluate the clinical profile of sickle cell disease in a tertiary care hospital.

Methods: This was the prospective observational study done from November 2015 to October 2016. All the hospitalized diagnosed case of sickle cell disease and trait in age group of 6 months to 14 years were taken in this study. Sickle cell disease with some genetic or metabolic disease and sickle-beta-thalassemia patients were not included in this study.

Results: Total 61 patients were admitted over a one year of study period, out of which 47 were sickle cell disease and 14 sickle cell trait patients. Morbidity events were commonly observed in 5-12 years of age groups (68.85%). Seasonal variation also observed, 47.54% of total cases are seen in winter season. Pain (60.65%) was the most common presenting symptom. Severe pallor (39.34%) and splenomegaly (24.59%) was the most common sign in both groups. Vaso-occlusive crisis (59.01%) was the most common morbidity event observed, of which abdominal pain was the most common site of pain involvement. On laboratory analysis, there was statistically significant difference observed in disease and trait. In patients with sickle cell disease acute painful crisis (59.57%) was the common morbidity event observed while in sickle cell trait patients acute febrile illness (71.42%) observed.

Conclusions: Vaso-occlusive crisis is the commonest manifestation in pediatric age group. Comprehensive medical care and management is required to decrease the morbidity and mortality. 


Keywords


Acute painful crisis, Morbidity events, Sickle cell disease, Vaso-occlusive crisis

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References


Weatherall DJ, Clegg JB. Inherited hemoglobin disorders: an increasing global health problem. Bull World Health Organization. 2001;79:704.

WHO Genes and Human disease- World Health Organization. Available at www.who.int/genomics/public/genetic disease/en/index2htm. Accessed 28 May 2017.

DeBaun M.R., Frei-Jones M, Vichinsky E. Sickle cell disease. In: Kliegman R, Stanton B, Geme J, Schor N. Nelson Textbook of Pediatrics, 19th ed. Saunders:Elsevier publications;2011:1663-70.

Leikin SL, Gallagher D, Kinney TR, Sloane D, Klug P, Rida W. Mortality in children and adolescent with sickle cell disease. Cooperative study of sickle cell disease. Pediatrics. 1989;84:500-8.

Jain D, Bagul AS, Shah M, Sarathi V. Morbidity pattern in hospitalized under five children with sickle cell disease. Indian J Med Res. 2013;138:317-21.

Kamble M, Chaturvedi P. Epidemiology of sickle cell disease in a rural hospital of central India. Indian Pediatr. 2000;37:391-6.

Swarnkar K, Kale A, Lakhkar B. Clinico-epidemiological and hematological profile of sickle cell anemia with special reference to penicillin prophylaxis in a rural hospital of Central India. The Internet J Epidem. 2010;9:2.

Serjeant GR, Charles DE, Ceulaer, Lethbridge, Joanne Morris, Singhal A et al. Painful crisis of homozygous sickle cell disease: clinical features. British J Haematol.1994;87:586-91.

Redwood AM, Williams EM, Desal P, Serjeant GR. Climate and painful crisis of sickle cell disease in Jamaica. British Med J. 1976;1:66-8.

Jones S, Duncan ER, Thomas N, Walters J, Dick MC, Height SE, et al. Windy weather and low humidity are associated with an increased number of hospital admissions for acute pain and sickle cell disease in an urban environment with a maritime temperate climate. Br J Haematol. 2005;131:530-3.

Panigrahi S, Patra P, Khodiar P. The Screening and Morbidity Pattern of Sickle Cell Anemia in Chhattisgarh. Indian J Hematol Blood Transfus. March. 2015;31(1):104-9.

Mandot S, Khurana LV, Sonesh JK. Sickle cell anemia in Garasia Tribals of Rajasthan. Indian Pediatrcs. 2009;46(3):239-40.

Subramaniam S, Chao JH. Managing Acute Complications of Sickle Cell Disease in Pediatric Patients. Pediatr Emerg Med Pract. 2016;13(11):1-28.

Rao SS, Goyal JP, Raghunath SV, Shah VB. Hematological profile of sickle cell disease from South Gujarat, India. Hematology Reports. 2012;4(2):e8.

Kaur M, Das GP, Verma IC. Sickle cell trait and disease among tribal communities in Orissa, Madhya Pradesh and Kerala. Indian J Med Res. 1997;55:104-9.

Goswmai S, Das K. Socio-economic and demographic determinants of childhood anemia. J Pediatr. 2015;91(5):471-7.

Akar NA, Adekile A. Ten year review of hospital admissions among children with sickle cell disease in Kuwait. Med Princ Pract. 2008;17:404-8.

Patel AB, Athvale AM. Sickle cell disease in central India. Indian J Pediatr. 2004;71:789-93.

Shinde S, Bakshi AP, Shrikhande AV. Infections in sickle cell disease. Int Archiv Integrated Medicine. 2015; 2(11):26-34.

Aidoo M, Terlouw DJ, Kolczak MS, McElroy PD, Ter Kuile FO, Kariuki S, et al. Protective effects of the sickle cell gene against malaria morbidity and mortality. Lancet. 2002;359:1311-2.