<p>Sickle cell disease (SCD) is the most common genetic disorder worldwide, caused by abnormal hemoglobin (HbS) in red blood cells (RBC), leading to severe life-threatening complications. This study aimed to describe the epidemiological and clinical characteristics of SCD among Moroccan children. A prospective descriptive study was conducted at the Provincial Hospital Center of Larache from March 2023 to March 2024. Medical data were collected from medical records and interviews with the children’s parents or legal guardians. The study included 194 Moroccan children (97 SCD patients and 97 healthy controls). Among SCD patients, the mean age was 7.59 ± 3.39 years, with a female predominance (58.76%). Most patients (77.3%) resided in rural areas, and 54.6% reported parental consanguinity. Cluster analysis identified three clinical profiles : mild chronic anemia, recurrent vaso-occlusive crises (VOC) with chronic pain, and acute severe anemia with infections. Jaundice and fever were more frequent in the acute anemia group (<i>p</i> &lt; 0.001 and <i>p</i> = 0.02), while musculoskeletal pain predominated in the VOC cluster (59.5%, <i>p</i> = 0.013). Hematological parameters revealed a significant decrease in RBC count (<i>p</i> = 0.003) and mean corpuscular hemoglobin (<i>p</i> = 0.027) in SCD patients. Higher fetal hemoglobin levels were protective against acute complications (OR = 0.58, <i>p</i> = 0.044) and reduced transfusion needs (<i>p</i> = 0.011). Our findings highlight the persistent burden of SCD in the studied region of Morocco, requiring effective nationwide management strategies focused on awareness campaigns, therapeutic education, genetic counseling, and screening programs to improve patient outcomes.</p>

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Epidemiological and clinical profile of sickle cell disease in a series of Moroccan children

  • Kenza Arbai,
  • Fatima Zahra Alaoui Ismaili,
  • Zeineb Zian,
  • Mouade El Bali,
  • Chourouk Mansour,
  • Amina Lhoussni,
  • Seddik Belahsen,
  • Naima Ghailani Nourouti,
  • Amina Barakat,
  • Mohcine Bennani Mechita

摘要

Sickle cell disease (SCD) is the most common genetic disorder worldwide, caused by abnormal hemoglobin (HbS) in red blood cells (RBC), leading to severe life-threatening complications. This study aimed to describe the epidemiological and clinical characteristics of SCD among Moroccan children. A prospective descriptive study was conducted at the Provincial Hospital Center of Larache from March 2023 to March 2024. Medical data were collected from medical records and interviews with the children’s parents or legal guardians. The study included 194 Moroccan children (97 SCD patients and 97 healthy controls). Among SCD patients, the mean age was 7.59 ± 3.39 years, with a female predominance (58.76%). Most patients (77.3%) resided in rural areas, and 54.6% reported parental consanguinity. Cluster analysis identified three clinical profiles : mild chronic anemia, recurrent vaso-occlusive crises (VOC) with chronic pain, and acute severe anemia with infections. Jaundice and fever were more frequent in the acute anemia group (p < 0.001 and p = 0.02), while musculoskeletal pain predominated in the VOC cluster (59.5%, p = 0.013). Hematological parameters revealed a significant decrease in RBC count (p = 0.003) and mean corpuscular hemoglobin (p = 0.027) in SCD patients. Higher fetal hemoglobin levels were protective against acute complications (OR = 0.58, p = 0.044) and reduced transfusion needs (p = 0.011). Our findings highlight the persistent burden of SCD in the studied region of Morocco, requiring effective nationwide management strategies focused on awareness campaigns, therapeutic education, genetic counseling, and screening programs to improve patient outcomes.