Hemoglobinopathies are genetic disorders of haemoglobin, with autosomal recessive inheritance affecting the protein fraction of haemoglobin. There are two types of hemoglobinopathies: hemoglobinoses and thalassemias. Sickle cell hemoglobinosis or sickle cell disease is the most widespread. According to the World Health Organization (WHO), 5% of the world’s population is a carrier of a sickle cell mutation and 240,000 newborns with sickle cell disease are born each year worldwide. Similarly, nearly 7% of pregnant women are thought to be carriers of hemoglobinosis or thalassemia [1] .

In Côte d’Ivoire, data on the prevalence of sickle cell disease are outdated [2] . Since 2022, the country has had a national strategic plan to control sickle cell disease, focusing on early screening, particularly neonatal screening, early treatment using innovative new therapeutic protocols, and capacity-building for medical staff. However, diagnostic facilities are still inadequate, especially in rural areas, and neonatal screening is in the pilot phase in a few level III referral facilities. This screening allows appropriate management of newborns born to mothers suffering from hemoglobinopathies. In fact, the association of sickle cell disease and pregnancy is a haunting for the medical team because it is often punctuated by complications in the mother and the fetus [3] [4] . By initiating this study, we have set ourselves the general objective of contributing to improving the quality of care for newborns of hemoglobinopathic mothers. Specifically, the aim was to describe the characteristics of mothers and their newborns, to describe the morbidity, to determine the neonatal mortality rates and identify causes of death, describe the profile of newborns born to mothers with sickle cell disease.

We carried out a cross-sectional study with analytical purposes that took place over a period of 2 years (November 2017-November 2019) in the neonatal block of the Cocody University Hospital in Abidjan. All medical records of newborns whose mothers were carriers of a hemoglobin abnormality were included in the study. Unusable files (incomplete, incorrectly filled in or not containing hemoglobin electrophoresis) and not found were not retained.

Data collection and analysis: Data were retrospectively collected from hospitalization registers, medical records and a pre-established survey form for each file. The variables studied were as follows.

For mothers: age, occupation, gynaecological-obstetric history (spontaneous abortion, stillbirth, pregnancy, pregnancy, parity, prenatal consultation and check-up, complications during pregnancy), type of pregnancy, hemoglobin electrophoresis profile.

For newborns: sex, mode of delivery, age at admission, place of origin, clinical characteristics (gestational age, anthropometric measurements, Apgar score at the 5th minute, reason for admission and physical signs, diagnosis retained), and biological characteristics (CBC, CRP, Blood glucose, GE), evolution (death or recovery). Gestational age was estimated from the date of the last menstrual period and/or from ultrasound if performed before 12 weeks and/or from the Finnstrom morphological score [5] . Since the term is defined by gestational age, any birth between 28 and 36 weeks and six days was considered preterm and after 42 weeks as post-term [6] . Birth weight was analyzed from the Luchencho and Leroy curves to establish trophicity. Hypotrophy is defined as a weight below the tenth percentile, which is the second standard deviation [6] .

The statistical analysis was done with SPSS software version 20.0. The comparison of the qualitative variables was carried out by the KHI 2 test and the Fischer test in case of insufficient numbers. A p value strictly less than 0.05 and an odds ratio (OR) whose 95% confidence interval (CI) did not exceed 1 were considered statistically significant.

Our findings are limited by the retrospective nature of our study. The files were not sufficiently detailed, particularly concerning follow-up of sickle cell disease during pregnancy, and several of them could not be examined because they were missing from our archives. On the other hand, in the context of our practice, bacterial infection of the newborn is rarely documented, given the high cost of bacteriological investigations. The majority of women were unemployed (42.6%) or worked in the informal sector (29.3%) and the prenatal work-up, which remains the responsibility of the patients, was incomplete in 64.4% of cases. The diagnosis of neonatal bacterial infection is most often based on anamnestic data, the newborn clinical condition, biological results and the positive outcome of antibiotic treatment.

The electrophoretic profile was comparable to that found by some African authors [7] [8] . Among the major forms of hemoglobinosis, the double heterozygous SC form was the most common (47.22%), followed by the homozygous SS form (19.4%) and the forms associated with thalassemia (33.4%).

Pregnancy in a mother who is a carrier of major forms of hemoglobinosis in general and sickle cell disease in particular is at high risk for the mother, the fetus and the newborn. Pregnancy worsens the medical complications of the disease, and the disease complicates pregnancy, leading to high maternal and perinatal mortality [9] . The usual complications in the mother and the fetus are vaso-oclusive crises, anaemia, infection, placental vascular complications (pre-eclampsia, retroplacental hematoma, growth retardation in utero and fetal death in utero), prematurity [10] . Several studies in the literature show that these complications can also occur in patients with heterozygous forms of AS or AC during pregnancy [11] [12] . In our series, mothers had a history of stillbirth in 3% of cases. Eclampsia (14.4%), severe anaemia (6.3%), HRP (5.4%), vaso-oclusive crisis (4.8%) were the main complications found during pregnancy. Nearly a quarter (22%) of newborns born to mothers who were carriers of major forms were preterm infants. Low birth weight and hypotrophy were found in 47% and 53% of cases respectively. Newborns of mothers with major hemoglobinopathy were twice as likely to have low birth weight in our study (p = 0.046, OR = 2.05 CI [1.01 - 4.1]). These neonatal adverses have also been found by other authors [13] - [15] .

In addition, mothers with major hemoglobinopathic diseases were more likely to have newborns with neonatal bacterial infection (p = 0.009, OR = 1.82 CI [0.89 - 3.7]). This high rate of neonatal infection seems to be overstated without bacteriological evidence. However, the frequent occurrence of infectious diseases in pregnant sickle-cell patients, especially in the third trimester of pregnancy, could explain this fact. Moreover, neonatal infection is the leading cause of hospitalization in our unit [7] .

The mortality rate of 14.8% was comparable to those found in other studies [15] . The main etiologies of death in the two groups of newborns were: anoxoischemic encephalopathy (35%), prematurity (24%), neonatal bacterial infection (16%) and anemia (8%). Our results were consistent with those in the literature [16] .

Newborns should be tested for hemoglobinopathies as soon as possible. Systematic screening has proved highly effective in reducing infant mortality, as well as severe infectious, anemic and neurovascular complications in early childhood [17] [18] .

For early care of children born to mothers with hemoglobinopathies, we need to extend neonatal screening to the various peripheral health facilities, and build the capacity of health-care personnel.

Pregnancy with a hemoglobinopathic mother is at high risk for both the fetus and the newborn. Low birth weight, hypotrophy, prematurity, infection, were more common in newborns born to mothers with a major form of sickle cell disease. Mortality was high for both groups and the leading causes of death were preterm birth and IAE. In view of this high morbidity and mortality, a well-codified care of newborns of hemoglobinopathic mothers is essential from birth, starting with the generalization of the systematic newborn screening program for hemoglobinopathies in the various health centers. A study is needed to better understand the prevalence of these newborns and identify possible risk factors for morbidity and mortality.