Frequency

Table 1.Recapitulative results.

During the study period, 150 cases of sickle cell syndrome were identified among 2570 patients, representing a frequency of 5.81%. Among these, 99.33% (n = 149) were homozygous SS and 0.67% (n = 1) were SC.

Age and Sex

Male patients were predominant at 60% (n = 90), while female patients accounted for 40% (n = 60), resulting in a male-to-female sex ratio of 1.5. The majority of patients fell within the age group of 24 to 60 months, representing 34% (n = 51). The mean age was 63.15 months, with extremes ranging from 2 months to 192 months.

Geographic Origin and Occupation

Most patients resided in the Urban Commune of Agadez, accounting for 80.67% (n = 121) of cases. The majority of fathers of sickle cell children were unschooled, representing 53.34% (n = 80), with most of them being merchants (53.34%, n = 80). Mothers were unschooled in 82.67% (n = 124) of cases, with the majority being homemakers (82.67%, n = 124).

Personal History

First and second consanguinity was observed in 40% (n = 60) of cases. More than half of the patients had received one to five blood transfusions, accounting for 61.33% (n = 92) of cases. Additionally, 49.33% (n = 74) of patients had up-to-date EPI vaccination status

The majority of sickle cell syndromes were diagnosed before 24 months of age in 67.33% (n = 101) of cases, with a mean age at diagnosis of 18.33 months. Hand-foot syndrome (Figure 1) was the most common presenting feature, observed in 32.67% (n = 49) of cases.

Figure 1. Hand-foot syndrome.

Followed by pale skin and mucous membranes in 30.67% (n = 46) (Figure 2).

Figure 2. Pale skin and mucous membranes.

Vaso-occlusive crises in 27.33% (n = 41), fever in 23.33% (n = 35), and jaundice in 7.33% (n = 11) of cases (Figure 3).

Figure 3. Jaundice.

Acute complications were dominated by acute drop in hemoglobin levels in 95.33% (n = 143) of cases, followed by infections in 79.33% (n = 119) and bone vaso-occlusive crises in 64.67% (n = 97). No chronic complications were recorded.

The majority of sickle cell syndromes, 99.33% (n = 149), were homozygous SS, while 0.67% (n = 1) were SC. Most patients had hemoglobin levels between 6 and 9 g/dL in 39.33% (n = 59) of cases. The anemia was normochromic normocytic in 36% (n = 54) of cases. Severe anemia (hemoglobin ≤ 6 g/dL) was observed in 45.33% (n = 68) of patients. Blood group O Rh-positive was the most common, accounting for 36% (n = 54) of cases.

All the children’s parents received therapeutic education, representing 100% of cases. Patients who were regularly followed up accounted for 90% (n = 135) of cases. Children under 5 years of age were systematically placed on penicillin V.

During the study period, 150 cases of sickle cell syndrome were identified among 2570 patients, representing a frequency of 5.81%. Djibrilla-Almoustapha A et al. in Niger (Diffa in 2024 and Dosso in 2025) reported frequencies of 4.88% and 1.08%, respectively [7][8]. Among the cases, 99.33% were homozygous SS. Cissouma A et al. in Mali reported a frequency of 57% for the SS form [9]. The majority of patients fell within the age group of 24 to 60 months, accounting for 34% (n = 51). The mean age was 63.15 months, with extremes ranging from 2 months to 192 months. Ngolet LO et al. in Brazzaville, Mali, reported a mean age of 26.04 years [10]. Male patients were predominant at 60%, with a sex ratio of 1.5. Sonia Douamba et al. in Burkina Faso in 2017 reported 51.9% in favor of females [11]. Djibrilla-Almoustapha A et al. in Niger in 2021 found a male predominance with a sex ratio of 1.3 [12]. This difference is likely due to chance, as sickle cell disease is an autosomal recessive disorder. The majority of fathers of sickle cell children were unschooled (53.34%), and merchants accounted for 53.34% of cases. Mothers were unschooled in 82.67% of cases, with the majority being homemakers (82.67%). Elhadji-Chefou M et al. in Niger (Maradi) in 2024 reported similar findings [13]. These factors may impact the quality of care. Consanguinity was observed in 40% of cases. Diallo D in Mali in 2016 reported 32.8% of cases [14]. However, this result is lower than that of Nehoulne G in Chad in 2003, who reported 52% of cases [15].

The majority of sickle cell syndromes were diagnosed before 24 months of age in 67.33% of cases, with a mean age at diagnosis of 18.33 months. In Niger in 2024, Abba Kaka HY et al. (2025) and Inoussa BD et al. (2022) reported similar findings [16][17]. Hand-foot syndrome was the most common presenting feature, observed in 32.67% of cases. Djibrilla-Almoustapha A et al. in Niger (Diffa) in 2024 reported similar results [7]. Acute complications were dominated by acute drops in hemoglobin levels (95.33%), followed by infections (79.33%). Diarra Y et al. in Burkina Faso reported a predominance of infectious complications [18].

Most patients had hemoglobin levels between 6 and 9 g/dL (39.33%). The anemia was normochromic normocytic in 36% of cases. Severe anemia was observed in 45.33% (n = 68) of patients. Djibrilla-Almoustapha A et al. in Niger (Diffa and Dosso) reported similar findings [7][8].

All patients received therapeutic education (100%). This is an important step in the management of sickle cell disease, aimed at preventing significant complications. Patients who were regularly followed up accounted for 90% of cases. Diarra Y in Burkina Faso reported 80% of cases with regular follow-up [18].

Of the patients, 49.33% (n = 74) had up-to-date EPI vaccination status. Sonia Douamba et al. in Burkina Faso reported 81.2% [11]. This low vaccination coverage rate may be linked to purchasing power on one hand and/or irregular follow-up on the other, both associated with low educational levels.