Research Article

Epidemiological, Clinical and Ethnic Aspects of Sickle-cell Anemia in Pediatric Outpatient Children at the El Idrissi Hospital (Kenitra, Morocco)

Khalid Hafiani1, Youssef El Guamri2,*, Halima Bazoui3, Omar Amahmid2, Youssef El Madhi4, Mustapha Hafid5, Samira Marhabene5, Khadija El Kharrim1, Driss Belghyti1
1Laboratory of Biotechnology, Environment and Quality (LABEQ), Department of Biology, Faculty of Sciences, University Ibn Tofail, Kenitra, Morocco
2Regional Centre of the Trades of Education and Training, CRMEF of Marrakesh, Morocco
3Regional Direction of Health, Kenitra, Morocco
4Regional Centre of the Trades of Education and Training, CRMEF of Rabat, Morocco
5Department of Physics, Faculty of Sciences, University Ibn Tofail, Kenitra, Morocco

*Corresponding author:

Y El Guamri, Department of Biology, Faculty of Sciences, University Ibn Tofail, Kenitra, Morocco, Email: elguamri3000@yahoo.fr

Keywords:

Children, Hemoglobin, Heterozygous, Homozygous, Kenitra, Major sickle cell syndromes, Morocco, Sickle cell disease

Objective: Sickle cell syndromes pose real public health problems in many African countries also described in the populations of the Mediterranean basin. In Morocco, their frequency and their distribution are poorly known. The objectives of this study are; first, to highlight the epidemiological characteristics second, plot the current mapping of the disease in children under 15 years old.

Methods: A retrospective study was carried out on 86 children from 01 January to 31 December 2015 at the pediatric department at El Idrissi Regional Hospital Center in Kenitra.

Results: The mean age of patients was 7.8 ± 3.1 years.  For gender, the proportion of males was slightly higher (54.6%). The SS form represented the majority of cases reaching 79.1%.  Clinical anemia was the most recorded symptom with 31.4% of cases while splenomegaly was identified in 7% of patients, and the average hemoglobin level ranged from 6.83g/dL to 9.54g/dL.

Conclusion: Sickle cell anemia remains a reality in Morocco and not perfectly understood yet by health professionals. A screening policy and a sustainable management program can reduce morbidity and mortality caused by hemoglobinopathies in the studied area. An action plan should be designed and implemented at national level to improve the quality of management of main sickle cell syndromes.

Sickle cell anemia is a codominant autosomal genotypic disease due to the presence of abnormal hemoglobin called hemoglobin S. Sickle Cell Disease (SCD) is recognized as a public health priority with a significant prevalence of major forms SS. On the basis of data from the United Nations, it has been estimated that 307,630 babies were born with sickle cell anemia in 2010, and large regional disparities in SCD occurrence were reproted, with fewer birth cases in India and much more in Africa [1].

The Major Sickle Cell Syndromes (MSCS), associated to SCD, include the SS homozygous form, the composite heterozygous form SC and the Sβ- thalassemia form [2]. In Morocco, the epidemiology of hemoglobinopathies remains unknown and WHO estimates the rate of carriers in Morocco at 6.5%; with a total number of approximately 30 000 cases of major forms [3].

The North-West of Morocco is a zone of prediction of hemoglobinopathies and the region of Gharb Chrarda Bni Hssen (GCBH) seems to be most affected especially in the province of Kenitra considered as a focus of the major form sickle cell disease [4].

The aim of this study was to report the current mapping of sickle cell syndromes and to specify their epidemiological characteristics in order to adopt appropriate therapeutic approaches.

This retrospective study was carried out on a cohort of 86 hospitalized patients in the pediatric department at El Idrissi Regional Hospital Center in Kenitra between January and December 2015. Several epidemiological parameters and variables concerning the children with hemoglobin SS, SC, or Sβ-thalassemia were specifically investigated (i.e. age, sex, socio-geographic status, ethnicity, hemoglobin electrophoresis, data on hemograms and their means). The data were recorded in appropriate individual survey forms. Data management and analysis was done using the Epi-Info 2011 software (version 7.0.3.8).

During the study period, 86 children with MSCS were admitted and identified in the Pediatric Department of El Idrissi Regional Hospital Center. The recorded data regarding MSCS patients are summarized in table 1.


 

Provinces

 

Kenitra

Sidi Slimane

Sidi Kacem

Overall

Localities

     Name

n(%)

Name

n(%)

Name

n(%)

     n(%)

 

My Bousselham

4(4.65)

Sidi. Yahia

9(10.46)

Sidi kacem

6(6.98)

-

 

Sidi Med Lahmer

7(8.14)

Sidi Slimane

5(5.82)

-

-

-

 

Sidi Med Benmansour

23(26.74)

-

-

-

-

-

 

Benmansour

10(11.63)

-

-

-

-

-

 

M’nasra

8(9.30)

-

-

-

-

-

 

Kenitra

3(3.64)

-

-

-

-

-

 

Mograne

8(9.30)

-

-

-

-

-

 

Sidi Allal Tazi

1(1.16)

-

-

-

-

-

 

Souk El Labraa

2(2.32)

-

-

-

-

-

Total cases

-           

66(76.74)

-

14(16.28)

-

6(6.98)

86(100.00)

Population (0-15 years)

315246

 

96671

 

158770

 

570687

Prevalence per 100 000 inhabitants

20.9

 

14.5

 

3.8

 

15.07

Ethnic group

Gharbaoui

 

Hasnaoui

 

Charadi

 

-

Age Group

             

       [1 – 6]

23(74.19)

 

4(12.90)

 

4(12.90)

 

31(36.05)

       [7 – 15]

43(78.18)

 

10(18.18)

 

2(3.64)

 

55(63.95)

Gender

             

      Male

38(80.85)

 

8(17.02)

 

1(2.13)

 

47(54.65)

      Female

28(71.79)

 

6(15.38)

 

5(12.82)

 

39(44.35)

Genotype

             

SS

50(73.53)

 

13(19.12

 

5(7.35

 

68(79.1)

SC

2(66.67)

 

0(0.00)

 

1(33.33)

 

3(15.12)

Sβ-thalassemia

14(93.33)

 

1(6.67)

 

0(0.00)

 

15(5.8)

      Total

66(76.74)

 

14(16.28)

 

6(6.98)

 

86(100.00)

 Table 1: Prevalence and distribution of MSCS patients by geographic status, ethnic group, age and gender.

The M’nasra area comprising three localities (Benmansour, Sidi Mohamed Ben Mansour and M’nasra) accounted for the highest rate reaching 47.67% of the total recorded cases in the study region, and corresponded to 62.12% of the identified cases in the province of Kenitra. In Kenitra, which represents the ethnic Gharbaoui group, 66 cases of MSCS were detected, while Sidi Slimane province, which represents the ethnic Hasnaoui, accounted 14 cases, and only 6 cases of the Charadi ethnic group were recorded in the province of Sidi Kacem.

                    According to the found results, the prevalence by province varied from 3.8/100,000 inhabitants in Sidi Kacem to 20.9/100,000 in the province of Kenitra (Table 1).

With regards to age factor, the investigated children were between 1 and 15 years old with a mean age of 7.8 ± 3.1 years. By gender, males were slightly predominant accounting for 54.6% with a sex ratio of 1.2:1 (Table 1).

With respect to the phenotypic level, 68 cases (79.1%) of homozygous sickle cell disease SS were detected, versus 13 cases (15.1%) of SC cases while only 5 cases (5.8%) of Sβ- thalassemia were observed (Table 2). The severity of the disease is assessed through the number of Vaso-Occlusive Crises (VOC) which represented 11.63% in the study population and splenomegaly was recorded in 7% of cases versus 18.6% for conjunctival pallor. The highest proportions were recorded for clinical anemia and bacterial infections (including pulmonary, bone and digestive infections) reaching 31.4%.

For the hemoglobin level, an average value of 7.2±2.52 g/dL was found. It varied according to hemoglobin type reaching 6.83g/dL for SS form, against 9g/dL and 9.54g/dL for Sβ-thalassemia and SC forms, respectively (Table 2)

Type of SCD

SS

SC

Sβ-thalassemia

Total

Clinical symptoms n(%)

 

 

 

 

Vaso-occlusive crises

5(5.81)

4(4.65)

1(1.16)

10(11.63)

Clinical anemia

23(26.74)

2(2.33)

2(2.33)

27(31.40)

Conjunctival pallor

15(17.44)

1(1.16)

0(0.00)

16(18.60)

Splenomegaly

3(3.5)

1(1.16)

2(2.33)

6(7)

Bacterial infections

22(25.58)

5(5.81)

0(0.00)

27(31.40)

Total

68(79.1)

13(15.1)

5(5.8)

86(100.00)

Hb (g/dL)

6.83 ± 1.06

9.54 ± 0.48

9.00 ± 0.80

-

 Table 2: Clinical symptoms and Hb concentration according to types of Sickle Cell Disease (SCD) in MSCS patients.

Major sickle cell syndromes (MSCS) are considered as the most frequent hemoglobinopathies in Morocco. The diagnosis of sickle cell forms in the study population was carried in the age range of 1 to 15 years. The age of 1-6 years corresponding to onset stage of clinical signs accounted only for 36%, while the most cases were recorded in the age group 7-15 years with a calculated proportion of 64%. The result revealed that the disease diagnosis was made within a late stage having repercussions on the clinical state of the patients in particular growth disorder and bones abnormalities.

The mean age of the study population was 7.8 years, and 63.95% of cases were aged 7-15 years. In fact, with improved comprehensive medical care, it has been suggested that 93% of children born with sickle cell anemia will live to 18 years old [5].

With regard to the prevalence of SCD in the studied region it ranged from 3.8/100,000 inhabitants to 20.9/100,000 inhabitants depending to provinces. However, the SCD prevalence may be underestimated as it was calculated based on the medical records and hospital admission.  Furthermore, children with milder variants of sickle cell disease might not have presented clinically at early age, also leading to an underestimate of prevalence.                    These findings corroborate those of previous studies carried out in Kenitra, Casablanca, Rabat and Oujda [6-9]. The current study showed a slight difference of MSCS distribution by gender with males accounting for 54.65% of cases, and a male to female ratio of 1.2:1. These results are similar to those reported by Hafiani, Bouzaid and Mahmouh quoting a preponderance of MSCS in boys with M/F sex ratios of  1.2, 1.2 and 1.6, respectively [6,8,9].  In contrast, Dokekias and Ouakasse found a female predominance with respective sex ratios of 0.8 and 0.7 [10,11]. However, sickle cell anemia has an autosomal and not a sex-related transmission. Accordingly, the gender factor should not affect the disease transmission, and any recorded differences between the quoted series may be due to recruitment or management bias with low number of sample population, and equal distribution of MSCS cases by gender were reported [12].

Three ethnic groups were defined in the studied region (i.e Gharbaoui, Hasnaoui and Chardi).  The study carried out revealed a predominance of MSCS cases in the Gharbaoui ethnic group with a proportion of 76.74% against 16.28% for the ethnic group Hasnaoui while only 6.98% of cases belonged to Charadi ethnic group. These results are in accordance with those of other studies carried out in Morocco [13]. This predominance of the Gharbaoui ethnic group in our series may be due cultural and environmental factors. In fact, cultural habits may make more common consanguineous marriages and environmental factors such as infections, nutrition, and socioeconomic status may also influence the course of the disease. Previous studies reported a high rate of consanguineous marriages in the studied region and confirmed the significant association between consanguinity and socioeconomic, cultural levels of the spouses [14].  

On the basis of the geographic distribution of sickle-cell anemia, M’nasra zone may be considered as a particular geographical focus. This matter may be a result of two major risk factors, the coexistence of β-thalassemia and β-S genes in high prevalences in the same locality in addition to the high frequency of consanguineous marriages engendering the emergence of composite homozygous and heterozygous forms, with severe pathological consequences.

The main clinical signs found in the studied series (underweight, conjunctival pallor, splenomegaly, Murphy positive sign, systolic murmur, jaundice...) are those classically described in the literature. Conjunctival pallor is the clinical translation of anemia, ranging from moderate coloration to complete discoloration of conjunctivae depending on the severity of anemia. This Chronic anemia is responsible for the systolic murmur and stunting. The cholelithaisis with jaundice and hepatomegaly is due to hemolysis .

The splenic sequestration crises are very specific to children under 6 years of age. It is a syndrome that affects the hemoglobin level making it lower than usual values [15]. The mean Hb level was 7.2 g/dL, i.e 6.83 g/dL in the SS and 9.54 g/dL in the SC. These results are in line with those of Tiendrebeogo [16] who found an average hemoglobin level of 8.03g/dL (9.54g /L in SC patients and 6.83g/dL in SS cases).                                                      

In areas where MSCS are prevalent persons at risk have to determine their hemoglobin type. In France, a growing number of children diagnosed for SCD was recorded since the 1980s.

To face this situation a progressive introduction of neonatal screening for sickle-cell anemia was launched by the French association for screening and prevention of disabilities in children (AFDPHE) in 1995 and was generalized in 2000 [17]. As the sickle-cell anemia is a public health priority in Morocco a similar plan should be elaborated and implemented in the country.

The conducted study proved that sickle cell syndromes represent a relatively common pathology in Morocco. Since the study is non-exhaustive and instantaneous, the actual situation on the ground may be underestimated. Therefore, an action plan must be implemented at national level to improve the quality of management of sickle cell syndromes.

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Citation: J Precision Med Public Health 1: 006.

Published: 04 September 2017

Copyright:

© 2017 Hafiani et al.. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.