Exploring Factors Associated with Quality of Life in Caregivers of Children and Adolescents with Sickle Cell Disease and HIV: A Comparative Analysis.

Introduction: Paediatric HIV and sickle cell disease (SCD) are two stigmatising and potentially fatal illnesses that place a significant burden on families. HIV patients benefit from a longstanding free-service national programme in Cameroon, and this could considerably alleviate burden of care on HIV caregivers, possibly leading to better quality of life (QoL) in HIV caregivers compared to SCD caregivers. Our study aimed to compare the QoL between caregivers of children and adolescents with SCD and HIV and explore factors associated with this QoL in Cameroon. Methods and Materials: We conducted a hospital-based cross-sectional analytic study at Douala Laquintinie Hospital from February to May 2023. A questionnaire was administered to caregivers of paediatric patients (≤18 years) with SCD and HIV. The Pediatrics Quality of Life-Family Impact Module (PedsQL FIM), the 7-item Generalized Anxiety Disorder (GAD-7), and the 9-item Patient Health Question (PHQ-9) tools were used as measures of quality of life, anxiety, and depression, respectively. Multivariable linear regression was used to determine factors associated with quality of life. A significance level was set at p < 0.05. Results: We included 199 caregivers: SCD = 104 and HIV = 95. The mean age of caregivers in our sample was 40.47 ± 10.18 years. Caregivers of paediatric patients with HIV had a better mean quality of life than SCD (93.01 ± 7.35SD versus 64.86 ± 9.20SD, p < 0.001). PHQ-9 score (B = -1.52, 95% CI = [-2.08; -0.96], p=<0.001), GAD-7 score (B = -1.46, 95% CI = [-2.09; -0.83], p=<0.001), spending less than 75 000 FCFA on medications monthly (B = 12.13, 95% CI = [5.73; 18.94], p=<0.001), and being a SCD caregiver (B = -11.62, 95% CI = [-18.46; -4.78], p=0.001) were factors independently associated with quality of life on multivariable analysis. Conclusion: Quality of life is lower in caregivers of children and adolescents with SCD than with HIV. Preventing depression and anxiety as well as advocating for the subsidization of medications through a national SCD program may improve quality of life in SCD caregivers.

Pubertal patterns in children with sickle cell anemia: A case-control study in Cameroon.

BACKGROUND: Puberty may be impaired in children with sickle cell anemia (SCA). Therefore, we aimed to explore the clinical and hormonal features of puberty in Cameroonian children. METHODS: In a case-control study, we included 64 children aged 8-18 years with SCA matched to healthy controls. We assessed height, weight, body mass index, body composition, and Tanner stages. Hormonal measurements included anti-mullerian hormone, follicle-stimulating hormone, luteinizing hormone, and sex hormones (estrogens/testosterone). We used the Mann-Whitney Wilcoxon test to compare the median values between cases and controls. We looked for associations between the severity criteria of SCA and delayed puberty through multivariate analysis. RESULTS: Delayed puberty was reported in 27.3% of girls and 10% of boys with SCA. The median age of menarche was delayed by 2 years compared to controls. SCA patients had a low lean body mass compared to controls (p = 0.03). Anti-mullerian hormone levels were significantly higher in boys with SCA than those of controls (45.9 ng/mL vs. 17.65 ng/mL; p = 0.018). A history of severe infection, acute chest syndrome, and low hemoglobin level was associated with delayed sexual maturation in children with SCA. CONCLUSION: Our study revealed delayed puberty in children with SCA. Moreover, puberty is affected by the severity of the disease. This highlights the importance of regular monitoring of puberty during the follow-up of these children.

Clinical, biochemical, and biomolecular aspects of congenital adrenal hyperplasia in a group of Cameroonian children and adolescents.

OBJECTIVES: Congenital adrenal hyperplasia (CAH) remains one of the most challenging endocrine disorders to diagnose, manage, and treat, especially in Africa where there is lack of neonatal screening program, and limited access to care. Data on biomolecular anomaly are sparse, therefore type of mutations are unknown, increasing management challenges and genetic counseling. The present study aims to describe clinical, biomolecular aspects of a group of Cameroonian patients. METHODS: We did an observational retrospective study at the pediatric endocrinology unit of the Mother and Child Centre of the Chantal Biya Foundation in Yaounde from May 2013 to December 2019, including all patients diagnosed with CAH. RESULTS: We consecutively included 31 patients aged less than 21 years, diagnosed CAH. Median age at diagnosis was 1.71 years (IQR 0.08-2.57 years). Abnormal genitalia was the main complain in 48.4%(n=15). The most prevalent genetic anomaly found in our study population (n=24) was on CYP11, found in 16 patients (66.6%) followed by CYP21A2 mutation found in 8 patients. Homozygous mutation of p.Q356X was found in half of patients with 11 hydroxylase deficiency. This mutation was mostly found in people from semi-Bantu tribes, declared non consanguineous. CONCLUSIONS: 11 hydroxylase deficiency is the most prevalent form of CAH found in this group of Cameroonian children.

[Administration of analgesics at home for the treatment of vaso-occlusive crises before admission to hospital]. / Traitement antalgique des crises vaso-occlusives à domicile avant l'arrivée en consultation.

INTRODUCTION: vaso-occlusive crises (VOCs) are the primary reason for admission and hospitalization in children with sickle cell disease and most often they require home care before arriving to consultation. The purpose of this study was to describe home-based therapy for children with sickle cell disease admitted with VOCs. METHODS: we conducted a descriptive and analytical cross-sectional study, in the pediatric emergencies at the Mother and Child Centre of the Chantal Biya Foundation (CBF) over a 4-month period from February to May 2018. Consecutive sampling was used. All patients with sickle cell disease presenting with VOCs were included in the study. RESULTS: one hundred and fifty-two patients were enrolled. The most represented age group was 5- 10 years. Eighty-two patients (54%) presented more than 24 hours after the onset of the crises and 70 (46%) in the first 24 hours; 80% (n=122) of our sample had received analgesic treatment at home. Escalation of therapy was found in 31.2% of cases. The use of prescription medications (75.4%, n=92) was dominant as the 1st recourse. The dosage for analgesics was not correct in 67% of cases (overdose in 70% of cases). The correct dosage of home-based analgesics was administered in only 33% of patients. CONCLUSION: this study shows that home-based management of VOCs is inadequate. Measures must be taken to ensure that all patients with sickle cell disease can effectively manage minor to moderate VOCs at home.