Nigerian Journal of Paediatrics

Noonan Syndrome in a Nigerian Neonate: A Case Report and Review of Literature

Kasarachi P Akowundu — 2024-10-27

Noonan syndrome (NS) is a genetic condition with a heterogeneous phenotype and multi-systemic involvement. This condition has been linked to mutations in the RAS/MAPK pathway, which is involved in cell differentiation. Noonan syndrome has been associated with multiple anomalies, with complications arising from the cardiovascular system, and this has been recognized as the commonest cause of death. Across the world, there is an upsurge in the number of congenital malformations, especially in mineral and petroleum-producing African countries. The report describes a term male neonate who had features of Noonan syndrome, with the scoring system used in diagnosis and review of existing literature. There is a robust scoring system that has diagnostic value in NS, as used in this report. This report aims to create awareness about the scoring system used in diagnosing NS, especially in resource-limited settings where genetic testing may not be feasible.

Educational Series: Vitamin D-Resistant Rickets, Helicobacter pylori, Hypothyroidism, Tuberculosis, Upper Airway Obstruction, Haemorrhagic Disease of the Newborn

Ebelechuku F Ugochukwu — 2024-10-27

Case Presentation

A boy aged 2-years 8months, presented with progressively worsening, painful abnormal gait and swelling of the wrists, knees and ankles for about a year. He walked at 10 months of age but was noticed to have an abnormal gait. The parents were reassured by neighbours that it was normal and would be self-limiting.

He was adopted a few days after birth, and he is the only child of his parents, who are in their forties. He was formula-fed on demand for six months, and thereafter, home-made maize gruel and soyabean powder was added. He was fed about five times a day. By age one year, he was already on regular home staple foods. He lived with his parents in a three-bedroom apartment on the first floor of a two-storey-building. Both parents were school teachers, and he was usually taken to school with them, and also played with other children. There was no chronic drug history, except for multivitamins from time to time. He has had no cause for hospital visits except for immunization.

Examination findings: A cheerful toddler who weighed 12kg (15^th centile for age) with standing height of 85cm (<3^rd centile for age), occipitofrontal circumference of 51cm (between 75^th and 90^th centiles for age) and mid arm circumference of 15.3cm. There was no pallor, jaundice or oedema. The liver, spleen and kidneys were not palpable. He walked with a waddling gait and his wrists, knees and ankles were swollen.

Prevalence of Constipation Among Adolescent Secondary School Students in Yenagoa, Nigeria

Ihekerenma J Binafeigha — 2024-10-27

Background: Constipation is one of the most common digestive system disorders in children and adolescents.

Objective: To determine the prevalence of constipation among adolescents in secondary schools in Yenagoa, a Nigerian city.

Methods: This was a cross-sectional study of children aged 10-19 attending public and private secondary schools in Yenagoa Local Government Area, Bayelsa State. Eligible adolescents were enrolled using a multistage sampling technique. A validated interviewer-administered questionnaire obtained information on sociodemographic characteristics, dietary patterns, physical activity, and bowel habits. Stool volume and character were assessed using clay models and Bristol stool charts, respectively. Constipation was determined using Rome III criteria. The anthropometric indices of subjects were also measured using standard procedures.

Results: A total of 935 participants were recruited (431 males and 504 females). Their mean age was 14.2±1.9 years. The prevalence of constipation was 18.8%. The risk factors for constipation at the bivariate and multivariate levels of analysis were a positive family history of constipation, attending public school, not taking watermelon, and ≥5 hours of television viewing time/day. Abdominal pain, straining at defecation, and faecal soiling of underpants were also found to be significantly associated with constipation.

Conclusion: Constipation was present in about one-fifth of the participants. The condition was associated with a positive family history of constipation, attending public school, not taking watermelon and ≥5 hours of television viewing time/day.

The State of Human Papillomavirus (HPV) Vaccination Among Secondary School Adolescents in Nnewi, Nigeria Prior to Free Routine HPV Vaccine Rollout

Chinefo G Duru — 2024-10-27

Background: Human papillomavirus (HPV) has been linked to various cancers of the genital, oropharyngeal, and anal regions in both sexes.

Objective: To assess the vaccination rate and factors influencing the uptake of the HPV vaccine among secondary school students in Nnewi, Nigeria, prior to the introduction of free HPV vaccine in the routine national immunisation program.

Methods: A cross-sectional, descriptive survey was conducted among male and female students aged 10 to 18 years in public and private secondary schools between April and July 2023. A total of 200 participants were recruited using multi-stage sampling. Data on relevant sociodemographic characteristics, awareness about the HPV vaccine, details of HPV vaccination and reasons for not vaccinating were collected using pretested interviewer-administered questionnaires.

Results: Nearly two-thirds (62.0%) of participants were aged 15 to 18 years. Sixty-five per cent had not previously heard about HPV infection or the HPV vaccine. However, only 3 (1.5%) female participants reported receiving the HPV vaccine. Significant associations were found between awareness of HPV infection and HPV vaccination status (p = 0.006). The major reasons for not receiving the HPV vaccine were a lack of information about the vaccine (100%), where to get the vaccine (27.9%]), and the cost of the vaccine (6.0%).

Conclusion: The study revealed a low level of awareness about HPV infection or vaccine and a very low HPV vaccination rate among the participants. Public enlightenment and advocacy about HPV and the HPV vaccine should be prioritised to enhance knowledge and uptake after the free HPV vaccine rollout.

Health Providers’ Readiness for Immunization Services in Selected Primary Health Care Facilities in Kaduna State, Nigeria

Jonathan G Joseph — 2024-10-27

Background: Immunisation is a critical primary health care (PHC) component. However, Nigeria has yet to meet the target of immunisation services. Therefore, the present study examined the interplay between training, motivation and involvement in determining health providers’ readiness for immunisation services.

Method: A descriptive, cross-sectional design was used. Stratified sampling was adopted to select 39 of 43 PHC centres. Using a ballot system, 133 health providers were proportionately drawn to respond to a semi-structured questionnaire. Items were scored under training, motivation, and involvement—the cumulative score of the three variables determined readiness.

Result: The mean age of the respondents was 29.4±6.7 years; 115 (86.5%) were females, and only 32 (24.1%) were Nurses/Nurse-Midwives. The respondents that reported adequate training were 75 (56.4%), adequate motivation 79 (59.4%, and adequate involvement 112 (84.2%). Consequently, 84 (63.2%) respondents were considered ready for immunisation. The Local Government Area of the PHCs was associated with immunisation readiness (p = 0.012). Over a third of the health providers in the study setting were not ready for immunisation services. No significant association was found for immunisation readiness across professional cadres (p = 0.283). Meanwhile, a significant association was found between involvement in providing immunisation and professional cadres (p<0.001).

Conclusion: To ensure optimal readiness for immunisation, improving in-service training and supportive supervision, particularly for nurses/nurse-midwives and demand for enhanced health investments in PHC facilities, should be prioritised.

The Impact of Hydroxyurea Therapy on Clinical and Haematological Parameters in Children with Sickle Cell Anaemia

Nathaniel O Oni — 2024-10-27

Background: Sickle cell anaemia (SCA) is an inherited genetic disorder of the erythrocyte that is frequently encountered worldwide. Hydroxyurea is a myelosuppressive and sickle cell disease-modifying agent.

Objective: To determine the effects of six months of treatment with hydroxyurea on the clinical and haematological parameters of children with SCA.

Methods: A longitudinal study involving 58 subjects was used. Clinical and haematological parameters were recorded at recruitment and intervals within six months of the commencement of hydroxyurea. Pre- and post-HU therapy data were compared.

Results: There was a significant reduction in the frequency of vaso-occlusive crises, acute chest syndrome, hospitalisations, stroke and blood transfusions (p<0.001 respectively). Haemoglobin F value and packed cell volume increased from a median (IQR) of 6.6% (6.0) to 15.9% (7.6) and from 22.0% (3.9) to 27.0% (3.5), respectively (p<0.001 in each case).

Conclusion: This study reports significant improvement in the clinical and haematological profiles of children with SCA following hydroxyurea therapy. Due to its proven clinical and haematological advantages, hydroxyurea should be globally adopted to manage SCA in children.

A Retrospective Study of the Prevalence and Pattern of Intraventricular Haemorrhage Among Preterm Neonates in Makurdi, Nigeria

Aondoaseer A Michael — 2024-10-27

Background: Globally, an estimated 2.5 million newborns died in the first month of life in 2018, with preterm birth complications as the leading cause of death. Preterm neonates who survive are at greater risk of a range of short-term and long-term morbidities. Intraventricular haemorrhage (ICH) is an important cause of brain damage in premature newborns.

Objective: To determine the prevalence, risk factors, pattern and outcomes of intraventricular haemorrhage amongst the preterm neonates in a tertiary facility.

Methods: A six-year (Jan 2018-Dec 2023) retrospective study was carried out at the Special Care Baby Unit (SCBU) of the Benue State University Teaching Hospital, Makurdi. The hospital records of all preterm neonates admitted into the unit during the period under review were retrieved and assessed using the study proforma.

Results: A total of 121 preterm neonates were admitted with a male-to-female ratio of 1.1:1. The majority, (93; 76.9%) of the neonates had the first brain scan within the first seven days of life, while 10.7% had the scan between 8 and 14 days. Intraventricular haemorrhage was observed in 39 (32.2%), while 82 (67.8%) had normal brain scans. The severity pattern revealed Grade I, Grade II, and Grade III haemorrhages in 16.5%, 10.7%, and 5%, respectively. There were seven deaths accounting for 17.9% mortality.

Conclusion: Intraventricular haemorrhage in preterm neonates is a silent cause of mortality among preterm neonates. Proactive measures are needed to prevent the condition and manage affected babies.

Clinical and Laboratory Profile, Parental Perception and Management of Micropenis in Childhood: Experience at a Referral Centre in Abidjan, Cote d'Ivoire

Marie E Dainguy — 2024-10-27

Background: Micropenis is a congenital condition that is part of the group of disorders of sex differentiation (DSD). It is a condition that has not received much attention among children in our environment.

Objective: To describe the epidemiological, clinical profile, and therapeutic profile of children with micropenis and the management outcome.

Methods: This was a descriptive, retrospective study of children with micropenis at the Paediatric unit of the Military Hospital, Abidjan, over six years (May 2016 to June 2022).

Results: A total of 70 children with micropenis were studied. The mean age was 9.3±3.9 years (1 month to 15 years). About one-third (30%) of the children were obese. The mean penile length was 2.55 ±0.65 cm (1 cm to 4 cm). In more than three-quarters of the cases, the mothers discovered a small-sized penis and anxiety in 91.4% of the parents. In the majority of cases (62.9%), the aetiology was unknown, while primary and central hypogonadism were observed in 24.3% and 11.4%, respectively. Affected children received testosterone treatment with favourable outcomes in all cases. The mean increase in penile length was 2.68 cm ± 0.97 (minimum of +1 cm and maximum of 6 cm).

Conclusion: It is essential to raise awareness about micropenis among the population and health personnel to reduce undue anxiety in parents and children.

The State of School Health Services of the School Health Programme in Nigeria: A Position Paper by the Paediatric Association of Nigeria (PAN) Sub-Committee on School Health Programme

Maduka D Ughasoro — 2024-10-27

This position paper summarises the current understanding of the School Health Services (SHS) component of the School Health Programme (SHP) in Nigeria in light of the results of the Nationwide Situation Analysis (SITAN) Survey conducted, existing literature exploration, and findings from the interactive sessions with relevant stakeholders on SHP. This article on SHS complements and is intended to integrate with parallel position papers on other components of the School Health Programme: Healthful school environment, School feeding services, School-based health education, and School home and community relationships in Nigeria. Challenges and solutions related to pre-entry medical screening, routine health screening/examinations, immunisation, school health records, sick bay, first aid and referral services were all reviewed. Gaps in our understanding of SHS in Nigeria and avenues for further research were also examined. Recommendations include a mandatory review of the National School Health Policy of 2006, training and retraining of staff and students on attitudinal change to become advocates for health in both the schools and communities, linkage of schools with healthcare providers, especially paediatricians and nurses, in a hub and spoke model; and development of precise performance -monitoring indicators for SHS. Gaps in our understanding of SHS in Nigeria and avenues for further research work were also highlighted.