HELLP syndrome: the experience at Ile-Ife, Nigeria.

Between 1 January and 31 December, 2006, 34 consecutive cases of severe pre-eclampsia (12), imminent eclampsia (10) and eclampsia (12) who were admitted at the Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife in the south-western part of Nigeria, were investigated for the development of HELLP (haemolysis, elevated liver enzymes and low platelet count) syndrome in a prospective study. The ages of the women ranged from 18 to 38 years, parity 0-5 and the estimated gestational age from 18-41 weeks at presentation. A total of 26 (76.5%) of the patients were unbooked, six (17.6%) of the 34 cases developed HELLP syndrome. Four (33%) of the 12 eclamptics developed HELLP syndrome, while only one (10%) of the cases of imminent eclampsia and 1 (8.3%) of severe pre-eclamptic cases developed the syndrome. Using the Mississippi Triple class system, none of the HELLP syndrome cases belonged to Class I; 4 were categorised in Class II while 2 were in Class III. All of the four eclamptic cases with HELLP syndrome died giving a 100% fatality rate while none of the imminent eclamptic and severe pre-eclamptic patients with the syndrome died. Furthermore, there were six (15.8%) perinatal deaths among the 38 infants delivered by the 34 mothers with severe pre-eclampsia/eclampsia. Our data suggest that the development of HELLP syndrome is more likely in eclamptic patients and when it occurs in them, it is highly fatal. Most of the cases in this study were unbooked. Substandard care may have contributed to the progression of the disease state and consequently, to maternal mortality. It is imperative to draw up an action plan for the identification of the risk factors for the development of pre-eclampsia/eclampsia at peripheral hospitals and maternity centres and for prompt referral of such cases afterwards. Efforts should also be geared towards the minimising of treatment delay in all phases, so as to minimise both perinatal and maternal morbidity and mortality.

Risk factors for osteonecrosis of the femoral head in patients with sickle cell disease.

The mechanisms involved in the pathogenesis of osteonecrosis of the femoral head in sickle cell disease are not fully known. The aim of this study was to identify risk factors for osteonecrosis of the femoral head among sickle cell disease patients. Clinical (frequency of painful crises and hospitalisation) and laboratory parameters (euglobulin clot lysis time, haematocrit, platelet count, and leucocyte count) of 25 consecutive patients with avascular necrosis of the femoral head from sickle cell disease were compared with those of 26 age- and sex-matched sickle cell disease patients without avascular necrosis. The group with avascular necrosis of the femoral head (mean age 23.7+/-4.9 years) had a significantly higher rate of painful crises (p = 0.03) and hospitalisations per year (p = 0.002) than the group without avascular necrosis (mean age 21.6+/-5.2 years). The group with avascular necrosis also had a significantly higher euglobulin clot lysis time than the group without avascular necrosis (p = 0.001). In conclusion, it appears that not all patients with sickle cell disease have impaired fibrinolytic activity. The aetiology of avascular necrosis in sickle cell disease is multifactorial.

Selenium and aspirin in people living with HIV and AIDS in Nigeria.

OBJECTIVES: Aspirin and selenium have been shown in vitro and in vivo to inhibit HIV production through inhibition of the transcription factor, the nuclear factor kappa binding (NF-eB). The aim of this study was to examine the efficacy or otherwise of these drugs in people living with HIV and AIDS (PLWAS) in resource limited countries. PATIENTS AND METHODS: Consenting HAART-naive PLWAS with mean CD4 count of 256.8 +/- 67.6 cells/ul were recruited into the study. Pretherapy blood count, serum biochemistry, chest x-ray, urinary glucose and protein and microscopy and culture of both urine and stool were checked in all cases. Each patient was treated for six months and CD4 counts were repeated at the end of the study. Thirty two patients (23 (72%) females and nine (28%) males), aged 22-52 (median = 36) years were recruited. Twenty-three (72%) were randomised into selenium and aspirin (SAM) and nine (28%) into selenium (SM); multivitamin was added to each arm. RESULTS: Eighteen (56.2%) patients completed the study. Sixteen (88.9%) patients are already on HAART since the termination of the study; one absconded and one died of disease progression. Fourteen (43.8%) of the initial 32 patients dropped out (11 (78.6%) were lost to follow-up, two (14.3%) died and one (7.1%) opted for HAART before completing the study). The post-treatment CD4 count was 293.0 +/-102.2 cells/ml, compared to the pre-therapy mean of 256.8 +/- 67.6 cells/ul, an average rise of 36.2 cells/ul, the difference was not statistically significant (p = 0.059). The post-therapy mean weight was significantly higher than the pretherapy weight, 61.6+/-15.2 kg versus 60.0+/-14.3 kg (p = 0.015). CONCLUSION: The SAM/SM combination regimen improved the quality of life of PLWAS, however, a greater number of patients and a longer period of follow up, are necessary to arrive at a more meaningful conclusion.

Avascular necrosis of the femoral head in sickle cell disease in Nigeria: a retrospective study.

BACKGROUND: Avascular necrosis (AVN) especially of the femoral head, has long been recognised as a manifestation of sickle cell disease (SCD). Despite this knowledge the actual prevalence of this complication among sickle cell patients in Nigeria is not known. OBJECTIVE: To determine the prevalence and patterns of presentation of avascular necrosis of the femoral head in sickle cell disease. PATIENTS AND METHODS: A retrospective study carried out at the Orthopaedic and Haematology clinics of Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria. Four hundred and sixteen patients with sickle cell disease seen over a 15-year period. RESULTS: Of the 416 patients with sickle cell disease 340 (81.7%) had haemoglobin SS genotype while 76(18.3%) had SC genotype. Sixty-six (15.9%) patients 35 males (53%) and 31 females (47%) had clinical and radiologic features of avascular necrosis (AVN) of the femoral head. Fifty-three of them (80.3%) had Hb SS while 13 had Hb SC. The peak age incidence in both Hb SS and Hb SC was 21-30 years. Forty patients (60.6%) presented with stage IV disease (Ficat and Arlet Staging) while 21.2% had stage III and 18.2% had stage II. No patient was diagnosed in stage 1 or 0. CONCLUSION: The prevalence of AVN of femoral head in Hb SC and HbSS is similar i.e. the difference is not statistically significant (P=0.48). Most of our patients present late with advanced diseases. Considering the paucity of facilities available for total hip replacement in Nigeria and the young age group affected we recommend regular screening of patients with sickle cell disorder for AVN and regular community educational programmes for early diagnosis.

Multifocal osteonecrosis in HIV/AIDS: a case report.

Many patients with Human Immunodeficiency Virus (HIV) Infection now live long due to the availability of antiretroviral drugs. This has resulted in many hitherto unknown complications being reported. We report a 56-year-old trader who presented with a seven-month history of pain in both shoulders and hips. Laboratory investigations and x-rays confirmed oesteonecrosis of both humeral heads and femoral heads in HIV/AIDS. She is presently on antiretroviral drugs and conservative management of her multiple joint disorders.

Clinical and prognostic features of Nigerians with chronic myeloid leukemia.

AIMS AND OBJECTIVES: Chronic myeloid leukemia (CML). PATIENTS AND METHODS: CML patients confirmed at the OAUTHC, Ile-Ife between June 1986 and December 1999 were studied prospectively until death or the last visit to the clinic. Stages of the disease at diagnosis, presenting clinical features and laboratory parameters, as well as the drug history were noted for each patient. Survival of each patient was computed from the date of diagnosis until the date of death. Kaplan-Meier statistical technique was used for analysis of survival. Factors were compared with log rank test. Student's t-test was used for comparing means of presenting clinical and laboratory parameters between groups. P-value of <0.05 was considered significant. SPSS for windows Release 10 (Real Stats, Real Easy, 1989-1999) was used for all calculations. RESULTS AND CONCLUSION: There were 42 patients (27 males and 15 females, ratio of 1.8:1) with ages ranging from 12-74 (median=38.0) years. Philadelphia chromosome was positive in the five patients (12.0%) that underwent cytogenetic analysis. Thirty-three of the patients (78.6%) presented in treatable chronic phase and the other 9 (21.4%) were seen in advanced stages (7 in accelerated & 2 in blastic phase). The median survival of patients that presented in chronic phase was 31.7 months compared to 0.16 months in patients presenting in advanced stages, the difference was statistically significant (log rank=7.8, p-value=0.005). Significant positive correlation was obtained between spleen size and total white cell count at diagnosis (r=0.36, p=0.02). Univariate regression analysis showed negative relationship between survival and ages of patients at diagnosis, haematocrit value, spleen and liver sizes, and blast count. The relationships were statistically significant for only liver and spleen (b=-0.63, p=0.004 and b=-0.51, p=0.026, respectively). Regression of both the liver and spleen with survival, still confirms the significant negative relationship between hepatomegaly and survival.

Outcome of pregnancy in a population of Nigerian women with sickle cell trait.

Although variable clinicopathological entities have been documented in sickle cell trait in pregnancy, such information is absent in this environment. This study therefore was aimed at examining the outcome of pregnancy in a population of Nigerian women with sickle cell trait. A prospective analytical study was carried at Ile-Ife, Nigeria comparing morbidities and mortalities between 210 pregnant women with sickle cell trait and 210 women with HbAA. Data were processed using SPSS 11.0 and PEPI packages, and the p value was set at =0.05. There were no significant differences between mothers with sickle cell trait and HbAA in terms of sociodemographic characteristics, the course of labour, deliveries and morbidity patterns. However, mothers with sickle cell trait had significantly fewer attacks of malaria in pregnancy (25.7% compared with 34.8%) and faster recovery of their newborn from birth asphyxia at 1 min (0.9% compared with 4.9%). Sickle cell trait may confer greater resistance to malaria in pregnancy and carries no extra risk to the outcome of pregnancy.

Peripheral blood and bone marrow changes in patients with acquired immunodeficiency syndrome.

There is dearth of information on the haematologic complications of HIV/AIDS in this country. The aim of this work was to evaluate the importance of peripheral blood and bone marrow changes in a population of adult Nigerians managed for symptomatic HIV infection at the OAUTHC, Ile-Ife, between 1995 and 2001. The study was prospective. Peripheral blood cells counts (haemetocrit value, total and differential leucocyte counts, and platelet counts) and bone marrow cytology of serologically confirmed HIV/AIDS patients seen within the study period were studied. The associated opportunistic disorder (s) was noted for each patient. Individuals with conditions that are ordinarily associated with haematologic disorders (e.g. cancer and inherited haemoglobinopathies) and patients diagnosed in pregnancy were not included. Significant levels of differences in mean values of blood cells within groups were determined by student's t-test. Seventy-two patients were recorded, out of which 49 (68%) were evaluable. There were 32 (65%) males and 17 (35%) females, all aged between 21 and 51 (median = 36) years. None of the patients had conventional antiretroviral therapy. Lymphopenia (lymphocytes < 2 x 10(9)/l) was seen in 64.4% of the patients, 50% and over 40% of the patients had moderate-severe anaemia and neutropenia, respectively. Blood cells values were not significantly different between patients with mild disease and those with moderate-severe diseases. The most characteristic marrow abnormality was the abundance of naked nuclei of megakarycytes in 20 (60.1%) of the patients. Dysplatic changes were evident in 15 (45.5%) of the bone marrow specimens studied. Such changes are characterised by dysgranulopoiesis, Pelger-Huet anomaly in some of the mature granulocytes, vacuolation of some erythroid and myeloid cells, unilobular micromegakaryocytes and megaloblastic erythroid precursors (15.1% of the marrow).

Burkitt's lymphoma of the head and neck region in a Nigerian tertiary hospital.

BACKGROUND: Burkitt's lymphoma is endemic in Nigeria; it forms about 39% of all childhood cancers. In recent times more of these cases are being seen presenting first to the Ear Nose and Throat clinic. OBJECTIVE: This study is designed to look at the pattern of presentation of head and Neck Burkitt's lymphoma at a Nigerian Tertiary hospital and to evaluate current treatment modality. DESIGN: It is a retrospective study of all confirmed Burkitt's lymphoma of the head and neck region seen at the Obafemi Awolowo University Teaching Hospital Ile Ife (OAUTHC) between 1986 and 2002. PATIENTS AND METHODS: The medical records of all the patients with the histopathologically confirmed Burkitt's lymphoma over a 17-year period (1986-2002) were evaluated. The proportion of the tumor affecting the Head and neck region were noted. The data extracted were entered into a questionnaire and analysis of data was done using the SPSS 10.0 software. RESULTS: A total of 196 cases of Burkitt's lymphoma were seen over the period out of which 140 (71.4%) were in the head and neck region. There was a male preponderance with the incidence of 72% and 28% in females. The peak age incidence was found to be within the first decade of life. The most common sites that were affected are; the jaw (65.9%), nasal and paranasal sinuses (12.2). Majority of the patients presented with advanced disease. Combination Chemotherapy comprising Cyclophosphamide, Oncovin, Methotrexate and Prednisolone (COMP) was the mainstay of management. The treatment outcome was only favorable in 36.6%. Default rate was (11.7%) while the mortality rate was (12.6%). Relapse\recurrence was found in (5.1%) of cases. Frank drug resistance was found in (2.6%). Blindness was found to be a major morbidity associated with this disease. Septicemia and severe anemia were found to be the major causes of mortality. Some complications of treatment were noted. DISCUSSION: The importance of the findings in this work was discussed in line with the existing literature. CONCLUSION: Head and neck remain the mostly affected parts in Burkitt's lymphoma in this environment. Presentation with advanced disease is the bane. This partly explains high morbidity and mortality in affected children.

Haematological parameters in prospective nigerian blood donors rejected on account of anaemia and/or microfilaria infestation.

BACKGROUND: Worldwide, prospective blood donors are screened for blood transfusion-transmissible diseases. In addition, predonation fitness requires adequate haematocrit and, in the tropics, negative screening for microfilaria that may precipitate allergy. The high prevalence of anaemia and microfilaria, though treatable, has contributed to the dearth of eligible blood donors. This study aims to characterize anaemia in prospective blood donors rejected for anaemia and find haematological effects of microfilarial infestation in prospective blood donors. METHODS: This prospective study was carried out from 1st of August to November 30th, 2002 at the blood transfusion unit of the Haematology Department of Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife. All consenting prospective blood donors that were rejected for anaemia and or microfilaria infestation during the study period were studied for their age, sex and haematological profile. A randomly selected population of successful donors was similarly studied. RESULTS: Sixty rejected prospective blood donors (5.2% of the total screened)) were studied. Forty-five (75%) of them were rejected for anaemia alone, 10 (16.7%) for microfilaria alone and 5 (8.3%) for both anaemia and microfilaria. The mean ages of those rejected were 33.3(+/- 9.9) years for anaemia alone, 29.9(+/- 8.5) years for microfilaria alone and 35.4(+/- 8.3) years for those with anaemia and microfilaria combined. The mean age of the successful group was 28.9(+/- 8.5) years. Of the 60 rejected subjects, 53 were males while 7 were females. Blood film of the anaemia group revealed features suggestive of iron deficiency anaemia (hypochromic microcytic cells) in 60% of them. The white cell count (WCC) was significantly increased in the microfilarial group compared to others and it revealed lymphocytosis and eosinophilia. CONCLUSION: The importance of these findings have been discussed in line with the existing literature. The need for intensive health education to encourage voluntary donation and promote the interest of females in blood donation is emphasized.

Pulmonary alveolar microlithiasis--a case report.

Pulmonary alveolar microlithiasis (PAM) is a rare disease of unknown aetiology. It is characterized by the presence of calcific concretions (calcospherites) inside the alveoli. We present a case of a 22 year old sickle cell anaemia patient with an incidental finding of PAM. This report is to highlight the fact that though rare, PAM is not entirely unheard of in this environment and should be kept in mind as a cause of diffuse opacities of the lungs.

Fibrinolytic activity in Nigerian diabetics.

Fibrinolytic activity, using euglobulin lysis time (ELT), was assessed in 46 Nigerians with type 2 diabetes mellitus to study the effect of the disease on fibrinolytic component of haemostasis. There were 20 females and 26 males. Fifty age matched non-diabetics and apparently healthy Nigerians were similarly studied as controls; there were 24 females and 26 males. In the patients, the mean (SD) age of the females was 56.7 (12.0) years and mean (SD) ELT was 276.4 (62.2) min; the mean age of the males was 55.7 (8.5) years and mean ELT was 303.5 (51.5) min. The mean age for female controls was 54.3 (12.6) years and their mean ELT was 198.3 (37.5) min; the mean age of the male controls was 53.4 (11.0) years and mean ELT was 181.6 (39.4) min. There was reduced fibrinolytic activity in diabetic Nigerians as revealed by significantly prolonged ELT in diabetic patients compared with healthy controls. There was good correlation between the blood glucose level and ELT. The observed changes in fibrinolytic activity in this study were not affected by duration of illness. The prolonged ELT in the diabetic population is an additional risk factor for thromboembolic disorders. Fibrinolytic agents may therefore be useful in the management of diabetes mellitus.

The search for a predictor of CD4 cell count continues: total lymphocyte count is not a substitute for CD4 cell count in the management of HIV-infected individuals in a resource-limited setting.

Total lymphocyte count (TLC) has been recommended as a substitute for CD4 cell count for the management of HIV-infected individuals living in resource-limited settings. To confirm this, 151 TLCs and CD4 cell counts were obtained from 109 patients who had not yet started treatment and analyzed. CD4 cell counts of <200 cells/mm3 were found in 42 cases (37.8%) with TLCs of > or =1200 cells/mm3. Thus, 1 in 3 individuals would have been deprived of needed treatment. Therefore, in this setting, TLC is not a reliable predictor of CD4 cell count in HIV-infected individuals.

Determinants of survival in Nigerians with Burkitt's lymphoma.

There is paucity of information on the factors influencing survival of patients with Burkitt's lymphoma (BL) in Nigeria. This work was undertaken to examine the roles of presenting clinical and laboratory features in the survival of a number of Nigerian patients with BL. Confirmed cases of BL diagnosed in the hospital between January 1986 and December 1999 were studied. Diagnosis of the tumour was based on fine needle aspiration cytology of easily accessible mass in a large majority of patients. Median survival was calculated for all the dead patients using Kaplan-Meier technique. SPSS 10 statistical software and EPI 6.04b were used for all statistical calculations. A total of 213 patients were recruited over the 13-year period; with ages ranging from 3-45 years (median = 9) at diagnosis. The male-female ratio was 1.8:1. At the time of analysis in December 2001, 166 (77.9%) of patients had defaulted, 44 were known to have died and 3 were still alive. Only 81 (38%) of the patients had adequate chemotherapy (4-6 cycles). Fifty one (23.9%) patients had an initial complete remission. The mean survival of the 44 patients that died was 10.5 weeks (95% Cl = 3.9-17.1). It is concluded that survival in Burkitt's lymphoma was significantly better in patients who had adequate therapy, mean 40.8 +/- 12.2 (SE) weeks compared to those who had less than adequate cycles of chemotherapy, mean 3.7 +/- 1.0 (SE) weeks (p = 0.0000). Inadequate therapy and high default rate were partly responsible for the poor survival results obtained in this study.

Priapism in southwestern Nigeria.

OBJECTIVES: To determine the aetiology, pattern of presentation, treatment regimen and outcome of management of priapism in our environment and to compare our findings with previous studies in this country and elsewhere. DESIGN: A 10-year retrospective study from January 1991 to December 2000. SETTING: Obafemi Awolowo University Teaching Hospital Complex, Ile-Ife, Nigeria. PATIENTS AND METHODS: Hospital records of 16 patients managed for priapism over 10 years (January 1991 to December 2000) were analysed. Information extracted included the age, occupation, duration of symptoms, precipitating factors, past medical history, haemoglobin genotype, drug and social history, physical findings, treatment regimen, outcome of treatment, complications and duration of follow up. Eighteen patients were treated for priapism during the period but only sixteen case files available for analysis were reviewed in this study. RESULTS: The mean age of the 16 patients under review was 20.4 years (range: 2.5-38 years). Thirteen patients (81%) were single and 10 (62.5%) were students. All the patients presented late with pain and woody hard penis with mean duration of eight days (range; 7 hrs-30 days). Eleven patients (68.7%) had previous episodes of priapism. Fourteen patients (87.5%) had sickle cell disease (SCD) and two (12.5%) were psychiatric patients on oral chlorpromazine. Associated medical conditions include urinary tract infection, malaria, acute urinary retention, bone pain crises and acute psychosis. All the patients received initial conservative management. Six patients had needle aspiration with irrigation plus injection of 2 ml of adrenaline solution (1 ml 1/1000 adrenaline in 200 ml saline) in both corpora cavernosa. One (16.7%) out of the six patients achieved full detumescence with normal erection. The remaining five patients later had cavernotomy with full detumescence and normal erection in three (60%) and weak erection in two (40%). Eight patients had Cavernosa-glandular shunt, full detumescence and normal erection was achieved in five patients (62.5%) while three (37.5%) became impotent. Two of the three patients with impotence presented with the longest duration of symptoms (14 and 30 days respectively), while the third patient reported earlier after five days, but he had suffered more than six (>6) previous attacks of priapism. Duration of hospital stay was 3-10 days and the average duration of follow up was 80.7 weeks. CONCLUSION: Sickle cell disease account for 87.5% of priapism in our community. Late presentation and previous episodes of priapism, which are common features in most of these patients, are associated with poor prognosis with higher risk of impotence. Conservative management and aspiration with intracavernous adrenaline therapy appears ineffective in late case. However, good results obtained with surgery indicate that late presentation should not be a deterrent to surgical intervention. Surgeries in form of cavernotomy or cavernosa-glandular shunt, when carefully done, are effective and safe.

Haematological profile and malarial parasitaemia in Nigerian children requiring emergency blood transfusion.

BACKGROUND: This study was carried out to determine the haematological profile and malarial parasitaemia of children requiring emergency blood transfusion. METHODS: This prospective study was carried out from 1st August to 30th of November, 1999 at the Children Emergency Ward (CHEW) of the Obafemi Awolowo University Teaching Hospital (OAUTH) Ile-Ife, Nigeria. All children requiring emergency blood transfusion seen at the CHEW were included in the study. Blood samples were taken on admission for haematocrit, white cell count and differentials, thin and thick blood films. Other data recorded include age, sex, clinical impression on admission. RESULTS: Four hundred children were admitted into the CHEW during the study period and 173(43%) required emergency blood transfusion. One hundred and forty-five (84%) of the children who required emergency transfusion were below the age of five years. There was no sex predilection. Although, clinical impression of malaria was made either singly or in combination with other diseases in almost all the patients, malaria parasitaemia was confirmed in 86 (50%) of the patients. The mean haematocrit was 0.14 L/L. In 60% of the children, there were mixed microcytic hypochromic and macrocytic red bloodcells. CONCLUSION: We conclude that severe anaemia requiring emergency blood transfusion is a major cause of morbidity and mortality in under five children. We also confirm that malaria and nutritional deficiencies are the major cause of severe anaemia in Nigerian children. We therefore recommend urgent need for prevention and prompt treatment of malaria in children under the age of five years. We also recommend the need for improvement in the nutritional intake of children under the age of five.

Acceptability of prenatal diagnosis of sickle cell anaemia (SCA) by female patients and parents of SCA patients in Nigeria.

The acceptability of prenatal diagnosis (PND) of sickle cell anaemia (SCA) as a means of controlling sickle cell disorder in Nigeria was examined using a structured questionnaire. The respondents were comprised of 92 adult female patients with SCA, aged 15-20 (23 +/- 6) years; 53 HbAS mothers, aged 20-61 (37 +/- 11) years and 48 HbAS fathers, aged 33-65 (46 +/- 8) years. More than 85% of the respondents would like PND to be offered in Nigeria and 92% of the HbAS mothers as well as 86% of the fathers would like to have the investigation or allow their wives to go through the procedure, respectively. Only 35% of the patients as compared to 63% of the mothers and 51% of the fathers would opt for termination of an affected pregnancy. Fear of the complications of abortion and religious convictions were the two most frequently cited reasons for opposing pregnancy termination. The high percentage of parents who would opt for termination of an affected pregnancy was associated with the societal emphasis on perfection and on previous experience in management of SCA patients. It is interesting that most of the respondents still favoured effective genetic counselling as the best means of controlling SCA.