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.

Anti-sickling Efficacy of Methanolic Extract of Medicinal Plants from Gwagwalada, Nigeria.

BACKGROUND: Medicinal plants are used in many rural communities in Abuja for the management of Sickle Cell Disease (SCD) without any scientific evidence to validate the effectiveness of such phytomedicines. The aim of this study is to investigate the anti-sickling efficacy of some medicinal plants used by rural communities in Gwagwalada, Abuja, for management of SCD. METHODS: Phytochemical screening and mineral analysis were carried out. Anti-sickling activity of the extracts was evaluated by monitoring (within 150 minutes) the percentage reduction in sickle red cells, after incubating different doses of the methanolic extracts with sickled red blood cells induced with 2% sodium metabisulphite. Parahydoxybenzoic acid (PHBA) and normal saline was used as positive and negative control respectively. RESULTS: Phytochemical screening of the methanolic extracts revealed the presence of alkaloids, tannins, phenols and terpenoids. Significant anti-sickling activity was observed in two, out of the four plant extracts tested. Pavetta crassipes and Ziziphus mauritiana at the high dose of 0.5mg/ml caused a significant reduction in percentage of sickle red cells from 44.0% to 15.3% (PC) and 44.9% to 20.5% (ZM) respectively at 150 minutes. This is comparable to the control sample (PHBA) where sickle red cells reduced from 43.9% to 14.6 % at 150 minutes. CONCLUSION: The results of this study showed that methanolic extract of P. crassipes and Z. mauritiana possess anti-sickling activity. This provides a scientific basis for their use as antisickling phytomedicine and makes further research into their mechanism of action necessary.

Bone malignancies in orthopaedic hospital Igbobi Lagos, Nigeria.

AIMS AND OBJECTIVES: To document the pattern of bone malignancies in a highly populated orthopaedic hospital in Lagos Nigeria; PATIENTS AND METHODS: A total of 21 cases of primary malignant bone tumours were studied. This comprised 12 cases of Osteosarcoma, 7 cases of Malignant Fibrous Histiocytoma (MFH) and 2 cases of Chondrosarcoma. Males (13) were affected more than females (8) giving a male to female ratio of 1.6 to 1. The age range was 7 to 45 years with a median age of 24 years. The diameter of the swelling ranged from 6 to 20 cm with a median of 12 cm. All patients had ablative surgery except for those with affectation of the ilium. Data was analysed using the Statistical Package for Social Sciences (SPSS 16). Enneking's classification was used to grade the tumour. The duration of symptoms of all the patients before presentation ranged from 3 weeks to 4 years with a mean of 7 months. The commonest site affected was around the knee (76.2%); distal femur had 42.9% and proximal tibia 33.3%. RESULTS: Osteosarcoma was the most common malignant bone tumour in this series and accounted for 57.1%. The peak incidence was found in the 2nd decade of life. The youngest patient was 7 years old and the oldest 43 years. The tumour was found primarily around the knee. 7 cases were in the distal part of the femur, 4 in the proximal part of the tibia and 1 case was found in the distal radius. Out of the 12 patients with osteosarcoma, 8 had paraosteal type (5 high grade, 3 intermediate grade), the remaining 4 had periosteal (all high grade) Malignant Fibrous Histiocytoma was found in 7 patients and accounted for 33.3%. The peak incidence was found in 3rd and 4th decades. 4 out of the 7 patients were high grade pleomorphic osteosarcoma, 2 were myxoid high grade dedifferentiated and one was low grade giant cell tumour type. Chondrosarcoma was found in 2 patients, accounting for 9.5%. both cases were in the ilium CONCLUSION: Primary malignant bone tumours occurred in children and young adult in this study. It is commoner among males and most of the patients presented late to the hospital. Osteosarcoma is the commonest followed by Malignant Fibrous Histiocytoma, both occurred commonly around the knee and chondrosarcoma on the ilium.

Current sickle cell disease management practices in Nigeria.

BACKGROUND: Although Nigeria has the highest burden of sickle cell disease (SCD) worldwide, there is still variable and poor utilisation of standard-of-care practices for SCD patients in the country. METHODS: This was a questionnaire survey of doctors in some dedicated SCD clinics in Nigeria in order to document the facilities available and common management practices. RESULTS: There were responses from 18 clinics based in 11 institutions. The number of patients being followed in each centre ranged from 15 to approximately 11 000. All clinics provided malaria prophylaxis and folic acid routinely to their patients. Only eight clinics prescribe penicillin prophylaxis. Eight prescribe hydroxyurea to patients who can afford it when indicated. All of the centres except three have electronic cell counters, but all had access to haemoglobin electrophoresis. Three had high-performance liquid chromatography machines installed but none was being routinely used. One institution had a functioning molecular biology laboratory. There is no official newborn screening programme in the country. All had access to microbiology and chemistry laboratories. Nine institutions had CT, six had MRI and three had transcranial Doppler facilities. CONCLUSION: The care available for SCD in Nigeria is still suboptimal and there is an urgent need for concerted effort to tackle the problem, but to make a significant impact on the burden of the disease would require more focus at the primary care level. Some steps to achieving this are outlined.

Sickle cell disease: new opportunities and challenges in Africa.

Sickle cell disease (SCD) is one of the most common genetic causes of illness and death in the world. This is a review of SCD in Africa, which bears the highest burden of disease. The first section provides an introduction to the molecular basis of SCD and the pathophysiological mechanism of selected clinical events. The second section discusses the epidemiology of the disease (prevalence, morbidity, and mortality), at global level and within Africa. The third section discusses the laboratory diagnosis and management of SCD, emphasizing strategies that been have proven to be effective in areas with limited resources. Throughout the review, specific activities that require evidence to guide healthcare in Africa, as well as strategic areas for further research, will be highlighted.

Surgical consideration for benign bone tumors.

BACKGROUND: The surgical management of symptomatic benign bone tumor has been described in various manners in medical literature. However, there are few published reports on the presentation and surgical management of benign bone tumors in black African patients. OBJECTIVES: To determine the pattern of presentation of benign bone tumors and evaluate the common indications for surgery in a Nigerian Orthopedic Center. MATERIALS AND METHODS: This is a prospective study of 67 patients, surgically treated for benign bone tumors, over a three-year period, at the National Orthopedic Hospital, Lagos, Nigeria. RESULTS: The common histological types include, osteochondroma, giant cell tumor, and the simple bone cyst. These tumors have varying anatomic locations, but are more commonly located around the knee joint. In this series, most of the patients have presented with an active or aggressive stage of the disease. The most common indication for surgery is painful swelling; other indications include a pathological fracture, restricted range of movement, and peripheral nerve compression. The surgical procedures performed are simple excision, curettage, and stabilization; and 1-stage and 2-stage wide resection with reconstruction. Patients with significant bone defects have autologous bone grafting or methylmethacrylate cement application. Further stabilization is achieved with intramedullary or compression plate and screw fixation. Amputation has only been necessary in one patient with a huge aneurysmal bone cyst. At the average follow-up period of 28.6 months, five patients showed recurrence. All were with a histological diagnosis of giant cell tumor. CONCLUSIONS: The mode of presentation of benign bone tumors in this group of black African patients is heterogenous, demanding various surgical options. Limb sparing is a largely feasible option, but the recurrence rate is particularly higher for giant cell tumors. Increase in the number of patients presenting with giant cell tumors raises the possibility of an increase in the incidence of this condition in the black African population. Larger multicenter studies in the black African population may shed more light on the actual incidence of giant cell tumors and other bone tumors in this group of patients.

Challenges in the diagnosis and management of musculoskeletal tumours in Nigeria.

The management of musculoskeletal tumours is important because of the high mortality rates associated with the available treatment modalities. A 5-year prospective study of bone and soft-tissue tumours is presented, along with the difficulties encountered in diagnosis and treatment. There were 71 patients (male:female ratio of 1.7:1, age range 5-85 years, mean age 32 years) with an average duration of 24.7 weeks (range 1 day to 34 years) before presentation. No patient had computed tomography (CT) scanning or magnetic resonance imaging (MRI) due to financial constraints, 95% had biopsies and X-rays, 15% could afford chemotherapy/ror chemotherapy is a sad consequence. 50% agreed to amputation. In the soft-tissue sarcoma group, only one of three patients could pay for limb-sparing surgery. In the benign group, 65% had limb-sparing surgeries and 15% had amputation. Fifty percent (50%) of patients were lost to follow up within 3 months and 39% of the malignant group died within the same period. Musculoskeletal tumours are a reality in our environment and a significant portion of our population have financial limitations. Ignorance and cultural beliefs promote late presentation to our hospitals, which are poorly equipped to give optimal care, despite the presence of trained personnel.

Pathological study of bone tumours at the National Orthopaedic Hospital, Lagos, Nigeria.

BACKGROUND: Although primary bone tumours are relatively uncommon, they constitute the most important tumours in patients under 20 years. OBJECTIVE: To update the literature on the relative frequency and clinico-pathologic characteristics of bone tumours in this environment. MATERIALS AND METHODS: The clinical and histopathological records of patients presenting with diagnosis of bone tumours between 1999 and 2004 and managed at the National Orthopaedic Hospital, Igbobi, (NOHI) Lagos, Nigeria were review and where necessary, new ones were prepared from the paraffin blocks and stained with routine haematoxylin and eosin stain. The clinical data such as the age, sex, presenting signs and symptoms, site distribution of lesions, radiological finding as well as the record of other investigation and management were extracted from the clinical case notes of patients. RESULTS: Seventy-seven cases were recorded; 61 (79.2%) benign and 16 (15.6%) malignant. The male:female ratio for all tumours was 2:1. The commonest benign bone tumours were osteochondroma and giant cell tumour accounting for 52 (67%) of all cases with > 60% in males. The most common primary malignant bone tumour was osteosarcoma, all in males. The peak incidence was in the second and third decades and commonest sites were the distal femur and proximal tibia. Four (5.2%) cases of metastatic bone tumours located commonly in the proximal femur and humerus were also recorded. CONCLUSION: Osteochondroma and giant cell tumours are the commonest benign tumours while osteosarcoma is the most common primary bone tumour all occurring in the first two decades of life. The age and sex distribution and morphology are similar to those already established in the African and international literature.

Management of Giant Cell Tumour : a Nigerian Experience

Giant cell tumours (GCT) are the commonest bone tumours worldwide. It is rarely malignant but when it does it progresses to fibrosarcoma with high mortality. Otherwise it causes poor cosmesis; disability and pathological fractures. A total of 19 cases of histologically established Giant cell tumour of the bone were reviewed prospectively in a 5 year study. 14 cases were benign; 4 malignant and one was a malignant transformation. Lesions around the knee accounted for 42.2of the cases; but the radius was the commonest single bone affected with 26.3. Eleven patients had curettage; five of them had autogenous bone grafting while the remaining six had bone grafting and plate augmentation. One patient had fore-quarter amputation while seven had tumour resection. There was no recurrence recorded among those that had currretage and autogenous bone grafting. 33of those that had curettage and bone cementing as well as 16.6of those that had resection presented with recurrence. One patient died within 3 months of surgery due to metastasis to the lungs; liver and spleen. Mean follow up was 9.2 months (range of 2 to 60 months). With early presentation; curretage and bone grafting is often effective; late presentation however has an increased risk of recurrence due to soft tissue involvement; dearth of investigative tools and financial constraints

Efficacy and safety of enoxaparin, a low molecular weight heparin in the prevention of deep vein thrombosis in Nigerian patients after orthopaedic surgery.

The objective was to determine the efficacy and safety of Enoxaparin as an antithrombotic agent in orthopaedic patients at risk for thromboembolism. 49 patients who had lower limb orthopaedic surgery were studied. They received subcutaneous Enoxaparin 40mg 12 hours before surgery and subsequently, daily for one week. Blood specimens were drawn at 2 and 12 hours after the first injection, and 24 hours after the fourth injection for anti Factor Xa assay. Specimens were also taken preoperatively, 1st, 5th and 7th post operative days (POD) for determination of Packed Cell Volume (PCV), Haemoglobin level, White Blood Cell (WBC) and Platelet Counts. The mean pre-treatment, 2, 12 and 24 hours anti Factor Xa clotting times were 14.5 +/- 0.8, 36.2 +/- 5.6, 30.6 +/- 9.8 and 25.8 +/- 9.3 seconds respectively. The changes were significant, P = 8.2 x 10(-12). The 2 and 24 hours clotting times corresponded to plasma heparin concentration level of 0.12 - 0.22U/ml read off from prepared Enoxaparin standardisation curve. Significant changes were observed in haemoglobin level, PCV, WBC and Platelet Counts when preoperative, 1st, 5th and 7th POD mean values were compared by Analysis of Variance--P < 0.01 in all cases. The study showed that Enoxaparin 40 mg daily caused hypocoagulation within prophylactic range of 0.12 - 0.22U/ml of heparin in the plasma. Changes in blood counts were within the limits expected post surgery.

Cost effectiveness of autologous blood transfusion--a developing country hospital's perspective.

An autologous blood donation program was set up at National Orthopaedic Hospital, Igbobi Lagos in 1992 in response to the rising sero prevalence of HIV observed in our "relative replacement" donors. A retrospective batch analysis of patients who received autologous transfusion and those who received homologous blood in our hospital in 1997 was carried out. Based on hospital charges, the mean charge (from the day of operation and excluding the cost of surgery) was dollars 116 (+/- dollars 7), median dollars 102 for those who donated and used their own blood compared to the mean charge of dollars 259.7 (+/- 116.3), median dollars 224, for homologous blood recipients (P=008). This was found to be due to a significant difference in the means of length of hospital stay of 21 days for autologous blood recipients, 34 for homologous blood recipients (P=0.009). The rate of infection was 85.7% for homologous blood recipients and 14.3% for autologous blood recipients. There was no significant difference in the means hospital charges, length of hospital stay and rate of infection in the entire population of patients who received blood transfusion when analysed by ward and consultant. We conclude that homologous blood transfusion in this hospital is significantly more expensive than autologous transfusion mainly due to greater infective morbidity in homologous blood recipients.

Tuberculosis masquerading as 'constant malaria'.

Four hundred adults aged 20-60 years, (200 females and 200 males) were studied. All the subjects were residing in the urban areas of Lagos, Nigeria. Thirteen percent claimed they were having "constant malaria" (> 8 times per year), 5% (20) claimed to have cough mostly during the cold period, 2.5% (10) produced mucoid sputum, 2.5% unproductive cough, 13% were AFB smear positive, 1.5% had positive chest X-ray for pulmonary Tuberculosis (PTB), 1.5% were HIV positive and 50% were mantoux positive (> 10 mm induration). All who complained of "constant malaria" were AFB positive. Malaria parasite density was lower in those who complained of "constant malaria" than those who did not complain (P = 0.003). The complaint of frequent malaria attack decreased after Antituberculosis therapy for 6 months. This study revealed that in a malaria and tuberculosis endemic region, early stage of tuberculosis can masquerade as "constant malaria". Therefore any such complaint should be fully investigated.