Volume 23, Issue 2, May 2016, Pages 268–273
Amr Loutfi1, Samira Jache2, Mohamed El Hioui3, Mohamed Khattab4, and Ahmed Omar Touhami Ahami5
1 Équipe de Neurosciences Comportementale & Santé Nutritionnelle, Laboratoire de Biologie & Santé, Département de Biologie, Faculté des Sciences, Université Ibn Tofail, BP 133 Kénitra 14 000, Morocco
2 Équipe de Neurosciences Comportementale & Santé Nutritionnelle, Laboratoire de Biologie & Santé, Département de Biologie, Faculté des Sciences, Université Ibn Tofail, BP 133 Kénitra 14 000, Morocco
3 Équipe de Neurosciences Comportementale & Santé Nutritionnelle, Laboratoire de Biologie & Santé, Département de Biologie, Faculté des Sciences, Université Ibn Tofail, BP 133 Kénitra 14 000, Morocco
4 Service d’hématologie et d’oncologie pédiatrique SHOP Hôpital d’enfant de Rabat, Morocco
5 Head Professor, Clinical and Cognitive Neurosciences Unit, Biology and Health Laboratory, Department of Biology, Faculty of Sciences, Ibn Tofail University, Kenitra, Morocco
Original language: French
Copyright © 2016 ISSR Journals. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Beta thalassemia major (also known as Cooley's anemia) is an inherited red blood cell disorder that results in the complete absence or decreased synthesis of the beta globin chains of hemoglobin. Beta thalassemia is among the most common genetic diseases in Morocco and characterized by mild to severe anemia, delay of growth and puberty, enlargement of the spleen, cardiac disease and dysmorphic bone changes. Untreated newborns have a higher risk of death within the first two years of life. Aim: The aim of this study is to evaluate the hematological and nutritional profile in young beta-thalassemia major patient in the Rabat commune. Material and Methods: This retrospective study assessed 104 young beta-thalassemia major patient followed at the division of pediatric hematology-oncology, Rabat children’s hospital, Morocco between October 2014 and 30 June 2015. Patients received regular transfusions every three weeks to maintain hemoglobin levels greater than 10 g/dl. To reduce iron overload, chelation therapy was considered to maintain the serum ferritin levels between 800-1000 ng/mL. Epidemiological, clinical and biological data were collected from medical records and transfusion files of patients. Furthermore, evaluation of the nutritional status and anthropometric measures such as weight-for-age and height-for-ageof subjects were investigated. Results: Data from medical records showed that the average age in our cohort is 11.42 years (the youngest age is 6 and the oldest age is 16). Low hemoglobin count (9.70 g/dl ±1.20) indicateda severe anemia which result in an important microcytosis and hypochromia. Indeed, 39.5% of patient developed leukocytosis, 12.5% neutrophilia, 23% eosinophilia, 9.6% basophilie, 85% monocytopenia, and 28% lymphocytosis. The percentage of subjects presenting stunting and wasting were 39.4%and 19.2%, respectively in patients. Conclusion: This study substantiates that beta thalassemia major young patients require a monitoring from birth, life-long transfusion support, reduction of overloaded iron, and regular nutritional assessment to prevent or reduce the risk of hematological and anthropometric complications.
Author Keywords: Beta thalassemia major, Anemia, Iron overload, Morocco.
Amr Loutfi1, Samira Jache2, Mohamed El Hioui3, Mohamed Khattab4, and Ahmed Omar Touhami Ahami5
1 Équipe de Neurosciences Comportementale & Santé Nutritionnelle, Laboratoire de Biologie & Santé, Département de Biologie, Faculté des Sciences, Université Ibn Tofail, BP 133 Kénitra 14 000, Morocco
2 Équipe de Neurosciences Comportementale & Santé Nutritionnelle, Laboratoire de Biologie & Santé, Département de Biologie, Faculté des Sciences, Université Ibn Tofail, BP 133 Kénitra 14 000, Morocco
3 Équipe de Neurosciences Comportementale & Santé Nutritionnelle, Laboratoire de Biologie & Santé, Département de Biologie, Faculté des Sciences, Université Ibn Tofail, BP 133 Kénitra 14 000, Morocco
4 Service d’hématologie et d’oncologie pédiatrique SHOP Hôpital d’enfant de Rabat, Morocco
5 Head Professor, Clinical and Cognitive Neurosciences Unit, Biology and Health Laboratory, Department of Biology, Faculty of Sciences, Ibn Tofail University, Kenitra, Morocco
Original language: French
Copyright © 2016 ISSR Journals. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Beta thalassemia major (also known as Cooley's anemia) is an inherited red blood cell disorder that results in the complete absence or decreased synthesis of the beta globin chains of hemoglobin. Beta thalassemia is among the most common genetic diseases in Morocco and characterized by mild to severe anemia, delay of growth and puberty, enlargement of the spleen, cardiac disease and dysmorphic bone changes. Untreated newborns have a higher risk of death within the first two years of life. Aim: The aim of this study is to evaluate the hematological and nutritional profile in young beta-thalassemia major patient in the Rabat commune. Material and Methods: This retrospective study assessed 104 young beta-thalassemia major patient followed at the division of pediatric hematology-oncology, Rabat children’s hospital, Morocco between October 2014 and 30 June 2015. Patients received regular transfusions every three weeks to maintain hemoglobin levels greater than 10 g/dl. To reduce iron overload, chelation therapy was considered to maintain the serum ferritin levels between 800-1000 ng/mL. Epidemiological, clinical and biological data were collected from medical records and transfusion files of patients. Furthermore, evaluation of the nutritional status and anthropometric measures such as weight-for-age and height-for-ageof subjects were investigated. Results: Data from medical records showed that the average age in our cohort is 11.42 years (the youngest age is 6 and the oldest age is 16). Low hemoglobin count (9.70 g/dl ±1.20) indicateda severe anemia which result in an important microcytosis and hypochromia. Indeed, 39.5% of patient developed leukocytosis, 12.5% neutrophilia, 23% eosinophilia, 9.6% basophilie, 85% monocytopenia, and 28% lymphocytosis. The percentage of subjects presenting stunting and wasting were 39.4%and 19.2%, respectively in patients. Conclusion: This study substantiates that beta thalassemia major young patients require a monitoring from birth, life-long transfusion support, reduction of overloaded iron, and regular nutritional assessment to prevent or reduce the risk of hematological and anthropometric complications.
Author Keywords: Beta thalassemia major, Anemia, Iron overload, Morocco.
Abstract: (french)
La bêta-thalassémie majeure, ou anémie de Cooley, fait partie des hémoglobinopathies les plus fréquentes au Maroc. L’évolution spontanée en l’absence de traitement est le décès entre deux et cinq ans dans un tableau d’anémie extrême, de défaillance cardiaque, de retard massif de croissance et de dysmorphie majeure. Objectif : l’objectif de cette étude était d’évaluer le profil hématologique et nutritionnel des enfants malades de bêta-thalassémie majeure. Sujets et méthodes : Cette étude rétrospective (1 octobre 2014–30 juin 2015) portant sur 104 patients bêta-thalassémiques suivis au service d’hématologie et d’oncologie pédiatrique SHOP Hôpital d’enfant de Rabat Maroc. Les données épidémiologiques, cliniques et biologiques ont été récoltées à partir des dossiers médicaux et transfusionnels des malades. Le protocole transfusionnel adopté visait à maintenir un taux d’hémoglobine > 10 g/dl par des transfusions régulières toutes les trois semaines. Un traitement chélateur en fer dans le but de maintenir une ferritinémie< 1500 ng/mL était introduit lorsque la ferritinémie dépassait 800 à 1000 ng/mL.Ainsi que l’état nutritionnel de l’enfant évalué par poids par âge et taille par âge. Résultats. – l’âge moyen est de 11.42 ans ± 3.71 et des extrêmes à 6 et 16 ans. La répercussion de l’anémie sur le plan biologique, est intense, au-dessous de 9.70±1.20 d’Hb, avec une microcytose et une hypochromie très importante. En ce qui concerne la lignée leucocytaire, 39.5% des malades soufrent de leucocytose, 12.5 % de neutophilie, 23% d'éosinophilie, 9.60% de basophilie, 85 % de monocytopénie et 28% de lymphocytose. La répercussion de bêta-thalassémie s’était traduite par un retard statural chez 39.4% par contre l’insuffisance pondérale a été notée chez 19.2%. Conclusion : Les résultats de notre étude suggèrent que, la bêta-thalassémie majeure est un problème de santé le plus fréquente chez les enfants de moins marocain dans notre région.
Author Keywords: Bêta-thalassémie , Hématologie , Anémie , Maroc.
How to Cite this Article
Amr Loutfi, Samira Jache, Mohamed El Hioui, Mohamed Khattab, and Ahmed Omar Touhami Ahami, “Profil hématologique et nutritionnel chez les malades béta thalassémies majeur (BTM) au service d’hématologie et d’oncologie pédiatrique SHOP Hôpital d’enfant de Rabat, Maroc,” International Journal of Innovation and Scientific Research, vol. 23, no. 2, pp. 268–273, May 2016.
