Childhood haemoglobinopathies in Benghazi

ABSTRACT

To determine the epidemiological pattern of haemoglobinopathies in Benghazi through the Children's Hospital in Benghazi as well as geographical distribution of cases. The information was initially collected retrospectively through the haematology clinic records, and then refined from the patients during their visit in the last 5 months. A total of 78 files of haemoglobinopathies, represented [18.1%] the total cases in the clinic. Sickle cell anaemia [74%], sickle cell trait [17%] haemoglobin C+S [6%], haemoglobin C trait [3%]. Consanguinity in 61% of the families. Their age at evaluation ranged from 6 months to 26 years with a mean of 10.84+6.75. mean of haemoglobin 7.5+2. Male to female ratio 11.4. For each patient the number of admissions ranged from 1-10 times with a mean of 2.6, and the frequency of blood transfusions ranged from 0-10 times. cholelithiasis [4.8%], stroke [hemiplegia] 6[9.5%], hepatitis B and C [9.5%], HIV [3.1%], hypersplenism [6.3%], acute chest syndrome [6.3%]. One patient died from overwhelming sepsis [post splenectomy]. Almost all the cases were originally from the southern areas, dark skinned, poor school performance. Symptomatic cases were registered, with a high non-compliance, and higher percentage of consanguinity. A national programme for the control of the disease by reducing carrier marriage is needed