Abstract
Background: Sickle cell anaemia is a very common disease condition in Nigeria. Its co-existence with type 1 diabetes mellitus is however rare, with only a few cases having been reported in literature and only two children have been reported from Nigeria. The genetic basis for this has not been fully reviewed. This case represents one of the few documented of both type 1 diabetes mellitus (T1DM) and homozygous sickle cell disease coexisting in an individual. More research is necessary to identify the factors that influence this co-morbidity and its effects on disease progression and patient management.
Case report: Q. O, a ten year old girl presented in October 2011 with 9 year history of recurrent bone pains, yellowness of the eyes and poor growth. She also had a short history of polyphagia, polydipsia and polyuria. Haemoglobin electrophoresis showed SS while a random plasma glucose done at least twice was greater than 200mg/dl. There was no ketosis nor did she have any other adverse complications. She is currently being managed as a case of sickle cell haemoglobinopathy with T1DM. Her management has been hampered by severe financial constraints.
Conclusion: This report seeks to increase the awareness of this rare co-existence in this environment, as well as to highlight the antecedent challenges in management.
Keywords: Diabetes, sickle cell anaemia, polymorphism, hyperglycemia, genetic testing, co-morbidity.
Résumé
Introduction: La cellule faible anémiée est une maladie très commune au Nigéria. Sa coexistence avec le type 1 de diabète est cependant rare, avec seulement un peu de cas ayant été reporté dans la littérature et seulement deux enfants ayant été reportés du Nigéria. La base génétique pour ceci n’a pas été complètement revue. Ce cas représente undes peu documentés cas du type 1 diabète (T1DM) et homozygote cellule faible anémiée coexistant dans un individu. Plus de recherche est nécessaire pour identifier les facteurs qui influencent cette co-morbidité et ces effets sur la progression de la maladie et le traitement du patient.
Rapport de cas : Q. O., une jeune fille de dix ans présentée, en Octobre 2011, avec 9 ans d’histoire de douleur d’os récurrent, jaunissement des yeux et pauvre croissance. Elle avait aussi une courte histoire de polyphagie, polydipsie et polyurie. L’électrophorèse d’hémoglobine montrait SS tandis que le glucose du plasma fait au moins deux fois au hasard était plus grand que 200 mg/dl. Il n’y avait pas de cétoses ni plus avait elle tout autre adverse complications. Elle est présentement traitée comme un cas de cellule faible hémoglobinopathie avec T1DM. Son traitement a été entravé par des sévères contraintes financières.
Conclusion : Ce rapport cherche à augmenter l’information de cette rare coexistence dans cet environnement, aussi bien que de pointer les défis antécédents pendant traitement.
Mots clés: Diabète, cellule faible anémiée, polymorphisme, hyperglycémie, test génétique, co-morbidité.
Correspondence: Dr. Olumide Olatokunbo Jarrett, Department of Paediatrics, University College Hospital/College of Medicine, University of Ibadan, Ibadan, Nigeria. E-mail: tokunbojarret@yahoo.com
References
Honig GR. HemogIobin disorders. In Behrman RE, Kliegman RM, Jenson HB. Nelson Textbook of Pediatrics. 16th ed. Philadelphia. WB Saunders; 2000;1478-1483.
World Health Organization. Sickle cell anaemia. WHO Fifty-Ninth World Assembly A59/9, 2006.
Fleming AF. The presentation, management and prevention of crisis in sickle cell disease in Africa. Blood Rev 1989; 3:18-28.
Norris AW and Wolfsdorf JI. Diabetes Mellitus. In Brook CD, Clayton PE, Brown RS. Clinical Paediatric Endocrinology 5th ed. Blackwell publishers. 2005. 447-473.
Sperling MA, Weinzimer SA and Tamborlane WV. Diabetes Mellitus. In Sperling MA. Pediatric Endocrinology 3rd ed. Sanders publishers. 2008; 374 – 421.
Raine J and Donaldson M. Diabetes mellitus. In Raine J, Donaldson M, Gregory J, Savage M, Hintz R. Practical Endocrinology and diabetes in children. 2nd ed. Blackwell publishers. 2006; 1 – 10.
Morrison JC, Schneider JM, Kraaus AP and Kitabchi AE. The prevalence of diabetes mellitus in sickle cell haemoglobinopathies. J Clin Endo Met 1979; 48: 192-195.
Kar BC. Sickle cell disease in India. J Asso Phy Ind 1991; 39: 954-960.
Kar BC, Clinical profile of sickle cell trait. J Asso Phy Ind 2002; 50: 1368-1371.
Reid HL, Photiades DP, Oli JM and Kaine W. Concurrent sickle cell disease and diabetes mellitus. Trop Geogr Med 1988; 40: 201-204.
Miodovnik M, Hurd WW, Lobel JS and Siddiqi TA. Pregnancy associated with both insulin dependent diabetes mellitus and sickle cell disease – a report of two cases. J Reprod Med 1987; 32: 317-319.
Mohapatra MK. Type 1 Diabetes Mellitus in Homozygous Sickle Cell Anaemia. J Asso Physicians India 2005; 53: 895-896.
Adekile AD and Jegende AO. Juvenile onset diabetes mellitus in a sickle cell anaemia patient. East Afr Med J 1990; 67:591-593.
Reid HL, Ene MD, Photiades DP, Famodu AA. Insulin dependent diabetes mellitus in homozygous sickle cell anaemia. Trop Geogr Med 1990; 42: 172-173.
Goldberg MF. Retinal detachment associated with proliferative retinopathies (sickle cell disease, retrolental fibroplasia and diabetes mellitus). IsrJMedSci.1972; 8: 1447-1457.
Shimizu K, Kobayashi Y and Muraoka K. Mid-peripheral fundus involvement in diabetic retinopathy. Ophthalmology 1981; 88:601-612.
Goldberg MF. Natural history of untreated proliferative sickle retinopathy. Arch Ophthalmol 1971; 85: 428-437.
Abraham EC and Elseweidy MM. Non-enzymatic glycosylation influences HbS polymerization. Hemoglobin. 1986; 10:173-183.
Wautier J-L, Paton RC, Wautier M-P, et al. Increased adhesion of erythrocytes to endothelial cells in diabetes mellitus and its relation to vascular complications. N Engl J Med. 1981; 305:237-243.
Hebbel RP, Boogaerts MAB, Eaton JW and Steinberg MH. Erythrocyte adherence to endothelium in sickle cell anaemia: a possible determinant of disease severity. N Engl J Med. 1980; 302: 992-995.
Koduri PR, Patel AR, and Bernstein HA. Concurrent sickle cell haemoglobin C disease and diabetes mellitus: no added risk of proliferative retinopathy? J Natl Med Assoc. 1994; 86: 682–685.