Scoping Review of Predisposing Factors Associated with Sensorineural Hearing Loss in Sickle Cell Disease.

PURPOSE: Sickle cell disease (SCD) is a genetically inherited red blood cell disorder that affects people all over the world but is more common among blacks of African ancestry than other races. The condition is linked to sensorineural hearing loss (SNHL). This scoping review aims to evaluate studies that reported SNHL in SCD patients and to identify demographic and contextual risk factors for SNHL in SCD patients. METHODS: We conducted scoping searches for relevant studies in PubMed, Embase, Web of Science, and Google Scholar. All articles were evaluated independently by two authors. The checklist Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) was used. SNHL was detected at hearing levels above 20 decibels. RESULTS: In terms of methodology, the studies reviewed were diverse, with 15 being prospective and four being retrospective. Fourteen of the 19 articles chosen from 18,937 search engine results were case-control studies. Sex, age, foetal haemoglobin (HbF), SCD type, painful vaso-occlusive crisis (PVO), blood parameters, flow-mediated vasodilation (FMV), and hydroxyurea use were all extracted. Few studies investigated SNHL risk factors with noticeable knowledge gaps. Age, PVO, and certain blood parameters appear to predispose to SNHL, whereas decreased FMV, the presence of HbF, and the use of hydroxyurea appear to have an inverse relationship with the development of SNHL in SCD. CONCLUSION: There is a clear gap in the existing literature regarding the knowledge of demographic and contextual risk factors that is required for the prevention and management of SNHL in SCD.

OBJECTIF: La drépanocytose est une maladie héréditaire des globules rouges qui touche des personnes partout dans le monde, mais qui est plus fréquente chez les Noirs d'ascendance africaine que dans les autres races. Cette maladie est liée à la perte auditive neurosensorielle (SNHL). L'objectif de cette revue est d'évaluer les études qui rapportent une perte auditive neurosensorielle chez les patients atteints de DICS et d'identifier les facteurs de risque démographiques et contextuels de cette perte auditive chez les patients atteints de DICS. MÉTHODES: Nous avons effectué des recherches pour trouver des études pertinentes dans PubMed, Embase, Web of Science, et Google Scholar. Tous les articles ont été évalués indépendamment par deux auteurs. La liste de contrôle Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) a été utilisée. Le SNHL a été détecté à des niveaux d'audition supérieurs à 20 décibels. RÉSULTATS: En termes de méthodologie, les études examinées étaient diverses, 15 étant prospectives et quatre rétrospectives. Quatorze des 19 articles choisis parmi les 18 937 résultats du moteur de recherche étaient des études cas-témoins. Le sexe, l'âge, l'hémoglobine fœtale (HbF), le type de DICS, la crise vaso-occlusive douloureuse (PVO), les paramètres sanguins, la vasodilatation médiée par le flux (FMV) et l'utilisation de l'hydroxyurée ont tous été extraits. Peu d'études se sont penchées sur les facteurs de risque du SNHL, avec des lacunes notables dans les connaissances. L'âge, la PVO et certains paramètres sanguins semblent prédisposer au SNHL, tandis qu'une diminution de la FMV, la présence d'HbF et l'utilisation d'hydroxyurée semblent avoir une relation inverse avec le développement du SNHL chez les patients atteints de DICS. CONCLUSION: Il existe une lacune évidente dans la littérature existante en ce qui concerne la connaissance des facteurs de risque démographiques et contextuels qui sont nécessaires pour aider à la prévention et à la gestion du SNHL dans la DICS. Mots Clés: Perte auditive sensoriell; drépanocytose.

Childhood suppurative otitis media in Abakaliki: isolated microbes and in vitro antibiotic sensitivity pattern.

BACKGROUND: Suppurative otitis media (SOM) is the most common pediatric problem seen by otolaryngologists in Nigeria. Devising simple and effective ways of treating pediatric patients with suppurating ears, especially in situations without a specialist care, will help prevent chronicity. Our experience with som at the ebonyi state university teaching hospital (ebsuth), abakaliki, is reviewed in this study. it may serve as a guide in patient care. OBJECTIVE: Determine the bacteriology of SOM in children in Abakaliki and ascertain their sensitivity to common antibiotics. MATERIALS AND METHODS: A 2-year retrospective analysis of ear swabs culture results and case files of children aged 0-18 years with SOM managed in EBSUTH. RESULTS: Sixty-five patients with discharging ears were reviewed. Of these, 73% were ≤ 5 years, of which 41.5% were infants. About 83% had unilateral ear discharge. Acute suppuration was seen in 67%. Overall swab yield was 87.7%; Pseudomonas was (57.4%), Klebsiella (16.4%), and Proteus species (11.5%). Ciprofloxacin showed 60% sensitivity, Gentamicin (58%), and Ceftazidime (35%). Amoxicillin/Clavulanic acid, ceftriaxone, and cefuroxime showed sensitivity of 4.3%-9.7%. CONCLUSIONS: Treatment protocols of SOM in children should emphasize the use of Ciprofloxacin or Gentamicin, especially in situations with limited access to laboratory services or specialist care.

Non-traumatic childhood coma in Ebonyi State University Teaching Hospital, Abakaliki, South Eastern Nigeria.

BACKGROUND: Coma is a medical emergency, and optimal management, especially in a resource-poor setting, would depend on the knowledge of its etiology and predictors of outcome. This communication reviews the etiology and outcome of non-traumatic childhood coma in Ebonyi State University Teaching Hospital (EBSUTH), Abakaliki. OBJECTIVE: To determine the incidence, etiology and outcome of non-traumatic coma in children seen at the EBSUTH, Abakaliki. MATERIALS AND METHODS: This is a retrospective analysis of records of all children admitted to the children emergency ward of EBSUTH in coma of a non-traumatic origin between 1 st of January and 31 st of December, 2007. RESULTS: Forty children presented with coma out of 673 children admitted during the study period, giving an incidence rate of 5.9%. The majority of the children (62.5%) were aged between 1 and 5 years of age, and 79.5% of them were deeply comatose on admission. Most of the cases (85%) of non-traumatic coma were due to infective causes, mainly cerebral malaria (47.5%), pyogenic meningitis (17.5%) and septicemia (10%). Twenty-four (60%) children recovered while 13 (32.5%) died. CONCLUSION: Infections were the predominant causes of non-traumatic coma in EBSUTH. In view of the high mortality among this group of patients, efforts at the control of malaria and other infections would significantly reduce the incidence of non-traumatic coma in this study site.

Non-Traumatic Childhood Coma in Ebonyi State University Teaching Hospital; Abakaliki; South Eastern Nigeria

Background: Coma is a medical emergency; and optimal management; especially in a resource-poor setting; would depend on the knowledge of its etiology and predictors of outcome. This communication reviews the etiology and outcome of non-traumatic childhood coma in Ebonyi State University Teaching Hospital (EBSUTH); Abakaliki. Objective: To determine the incidence; etiology and outcome of non-traumatic coma in children seen at the EBSUTH; Abakaliki. Materials and Methods: This is a retrospective analysis of records of all children admitted to the children emergency ward of EBSUTH in coma of a non-traumatic origin between 1 st of January and 31 st of December; 2007. Results: Forty children presented with coma out of 673 children admitted during the study period; giving an incidence rate of 5.9. The majority of the children (62.5) were aged between 1 and 5 years of age; and 79.5of them were deeply comatose on admission. Most of the cases (85) of non-traumatic coma were due to infective causes; mainly cerebral malaria (47.5); pyogenic meningitis (17.5) and septicemia (10). Twenty-four (60) children recovered while 13 (32.5) died. Conclusion : Infections were the predominant causes of non-traumatic coma in EBSUTH. In view of the high mortality among this group of patients; efforts at the control of malaria and other infections would significantly reduce the incidence of non-traumatic coma in this study site

Occurrence of anaplastic oligodendroglioma in a patient with Williams syndrome: a case report with analysis of mutational profile of tumor.

Williams syndrome is a rare congenital developmental disorder characterized by a constellation of distinctive facial dysmorphisms, mental retardation, cardiovascular anomalies, infantile hypercalcemia, delayed developmental milestones, dental and musculoskeletal anomalies and distinctive personality traits. A majority of patients with Williams syndrome exhibit a hemizygous micro-deletion of chromosome 7q11.23, which is the locus of some 20-30 genes including the ELN gene that encodes the structural protein elastin. Chromosome 7q contains putative tumor suppressor genes and is one of the chromosomes that are frequently involved in chromosomal aberrations in human malignancies. A paucity of tumors (three) has been reported in the literature to occur in patients with Williams syndrome. We report a case of anaplastic oligodendroglioma that occurred in a 31-year-old man with Williams syndrome. Mutational profiling by loss of heterozygosity analysis using a panel of polymorphic micro-satellite markers indicated combined deletion of chromosome 1p and 19q. We draw attention to this apparently rare or possibly under-reported occurrence of tumors in patients with Williams syndrome and suggest that Central Nervous System [CNS] tumors be considered as differential diagnoses in such patients when they present with unanticipated neurologic symptoms that are not attributable to those commonly associated with Williams syndrome.

Childhood Malignancy in Abakaliki: A Growing Concern

Aim: To determine (a) the prevalence of malignancies in children in Abakaliki as well as the outcome of therapy. (b) Compare findings with data from other parts of Nigeria. Patients and Methods: A 4-year descriptive retrospective review of childhood malignancies was carried out based on data collected from case notes of patients attending the Paediatrics clinics of Ebonyi State University Teaching Hospital (EBSUTH) and Federal Medical Centre (FMC) Abakaliki. Results: A total of 38;802 cases were reviewed out of which 53 had various types of malignancies; giving a prevalence rate of 0.14; with a male to female ratio of 2.1:1. The mean age of patients was 8.14 years with a standard deviation of 3.77 years. This constituted 1.7 of the 3088 Ward admissions. Burkitts lymphoma was the commonest malignancy (60.5) followed in order of frequency by hepatocellular carcinoma (9.2 ) and retinoblastoma (7). Leukemia; rhabdomyosarcoma and Wilm's tumour had a prevalence of 4.7each. A review of the Burkitts lymphoma cases showed a male dominance with a male to female ratio of 3:1; though this is not statistically significant. There was a preponderance of abdominal tumours. Most of the patients had chemotherapy but outcome was poor. Conclusions: Prevalence of childhood malignancies in Abakaliki is high with a high frequency of Burkitt's lymphoma. Outcome of therapy was poor. Late presentation; absence of proper diagnostic machinery; inadequacy of chemotherapy; poverty and ignorance were some of the factors responsible for the poor outcome of the various malignancies