The Jamaican Haemophilia Registry: Describing the burden of disease.

INTRODUCTION: Jamaica has an estimated 200 persons with haemophilia (PWH), who face significant constraints in access to specialized haemophilia care, including access to clotting factor concentrates. AIM: The aim of this paper is to establish the current burden of disease in PWH in Jamaica. METHODS: PWH were enrolled through the University Hospital of the West Indies, Jamaica. The impact of haemophilia was assessed using a comprehensive battery of heath outcome measures that included the following: laboratory, clinical information and validated outcome measures of joint structure and function, activity, and health-related quality of life (HRQoL) to provide a health profile of the Jamaican haemophilia population. RESULTS: In all, 45 PWH were registered (mean age: 29, range: 0.17-69 years), including 13 children (<18 years of age) and 32 adults. In this sample, 41 had haemophilia A (30 severe) and 4 had haemophilia B (3 severe); 10 patients with haemophilia A were inhibitor positive. The results indicate that adults with haemophilia in Jamaica have significant joint damage: mean Haemophilia Joint Health Score (HJHS) = 42.1 (SD = 17.3); moderate activity levels - mean Haemophilia Activities List (HAL) score = 64.8 (SD = 17.8); and low HRQoL scores - mean Haemo-QoL-A score = 62.3 (SD = 19.4). Results for children are also reported but should be interpreted with caution due to the small sample size. CONCLUSIONS: There is a very high burden of disease in PWH in Jamaica. The health profiles reported in this paper are an essential first step in advocating for a multidisciplinary Comprehensive Care Program for assessment and care of PWH in Jamaica.

Ischaemic Priapism and Glucose-6-Phosphate Dehydrogenase Deficiency: A Mechanism of Increased Oxidative Stress?

Ischaemic priapism is a devastating urological condition that has the potential to cause permanent erectile dysfunction. The disorder has been associated with numerous medical conditions and the use of pharmacotherapeutic agents. The aetiology is idiopathic in a number of cases. There are two prior case reports of the association of ischaemic priapism and glucose-6-phosphate dehydrogenase (G6PD) deficiency. We report on a third case of priapism associated with G6PD deficiency and review recently described molecular mechanisms of increased oxidative stress in the pathophysiology of ischaemic priapism. The case report of a 32-year old Afro-Caribbean male with his first episode of major ischaemic priapism is described. Screening for common causes of ischaemic priapism, including sickle cell disease was negative. Glucose-6-phosphate dehydrogenase deficiency was discovered on evaluation for priapism. Penile aspiration was performed and erectile function was good post treatment.Glucose-6-phosphate dehydrogenase deficiency is a cause for ischaemic priapism and should be a part of the screening process in idiopathic causes of the disorder. Increased oxidative stress occurs in G6PD deficiency and may lead to priapism.

Blood use in neurosurgical cases at the university hospital of the west indies.

OBJECTIVE: Many neurosurgical cases are done without the need for blood transfusion, yet blood is unnecessarily cross-matched, resulting in wasted resources. This study was undertaken to document and compare the number of units of blood components requested, cross-matched and transfused in neurosurgical cases at the University Hospital of the West Indies (UHWI). METHODS: A prospective, observational study was undertaken over one year. Data collected for each patient included demographic information, relevant perioperative data, and blood banking data including blood components requested, cross-matched and transfused. Data were analysed using SPSS version 16. RESULTS: Data were analysed on 152 patients, 71 females (46.7%) and 81 males (53.3%). The mean age was 48.7 ± 19.6 years and 100 of the procedures were done electively (65.8%). Blood components were ordered in 114 (75%) cases, red cells more commonly in 113 (74.3%) patients, and plasma in 19 (12.5%) patients. Overall, 20 patients (13.2%) were transfused. Most patients (90.9%) needed one to two units of blood. Of the 236 units of blood components that were cross-matched or prepared, only 62 were transfused. The cross-match/preparation to transfusion ratio (CTR/PTR) was 6.00 for red cells and 1.31 for plasma. Preoperative haemoglobin ≤ 10.0 g/dL (p = 0.001), estimated blood loss of ≥ 1 litre (p < 0.001), higher American Society of Anesthesiologists (ASA) physical status score (p < 0.03) and a resident as lead surgeon (p < 0.05), were significant predictors of blood transfusion. CONCLUSION: The transfusion rate was low with a high cross-match to transfusion ratio, suggesting that less cross-matching is needed. A new approach to blood ordering for neurosurgical cases is recommended.

Renal impairment as a complication of uterine fibroids: a retrospective hospital-based study.

Leiomyomas can cause obstructive renal impairment and renal failure. This was a retrospective study of women with renal impairment seen at the University of the West Indies Hospital, Jamaica, between 2000 and 2004, looking at aetiology and severity (group 1). We also evaluated patients, in the same hospital, with fibroids who had ultrasonography during a later period (2006-2011), comparing those who had hydronephrosis and those without (group 2). In group 1, 274 women were coded as renal impairment. Case notes for 160 patients (59%) were analysed. Uterine fibroids accounted for 13/160 (8.1%) of cases. Comparing cases with and without fibroids, none of those with fibroids were over 50 years old compared with 59.3% of the others, OR 0.02 (CI 0.00-0.35) p = 0.0001. Hospital data for renal failure showed that most mean values were significantly better for those with fibroids. Urea, 8.59 mmol/l (SD 9.89) vs 17.00 mmol/l (SD 13.41) p = 0.003; Creatinine 300.15 µmol/l (SD490.92) vs 424.05 µmol/l (SD553.29) p = 0.022 and Creatinine clearance 73.21 ml/min (SD 38.92) vs 44.25 ml/min (SD 49.71) p = 0.017. However, mean potassium values were similar, 4.52 mmol/l (SD 0.61) vs 4.85 mmol/l (SD1.03) p = 0.2. In group 2, there were 216 patients and we found 31 (14.35%) patients at ultrasonography with hydronephrosis from fibroids. These patients had significantly larger uteri than those without hydronephrosis but renal function was similar, with only urea values significantly worse. Leiomyomas can cause renal impairment, however the prognosis appears good.

Bone mineral density in Jamaican black women after hysterectomy and bilateral oophorectomy: an observational study.

OBJECTIVE: To see if black Jamaican postmenopausal women who had hysterectomy were at increased risk of osteoporosis. To assess the risk of osteoporosis in hysterectomized Jamaican postmenopausal patients. METHOD: We reviewed 809 women (403 hysterectomized and 406 controls) for cardiovascular disease risk. We did a demographic history and examination looking at blood pressure, waist hip ratio and body mass index and investigations done included fasting blood glucose and total and high density lipoprotein (HDL) cholesterol. We also measured bone density at the heel in all women using the Achilles ultrasound bone densitometer looking at T-score and Z-score. RESULTS: There was a significant association of hysterectomy status and bone mineral density (BMD) status with a smaller than expected proportion of women with osteoporosis in the hysterectomy group (χ2 = 18.4; p = 0.001). The mean T-score was significantly higher in the hysterectomized women, adjusting for age, waist circumference and sociodemographic factors. The relationship between the various predictors and BMD was explored by stepwise regression modelling. The factors that were significantly related to low BMD were hysterectomy status, age, waist circumference and being employed. CONCLUSION: Hysterectomy was not found to be a significant risk factor for osteoporosis. The osteoporosis risk among menopausal women in Jamaica appears to be due to other risk factors which probably existed prior to the operation.

Ovarian lymphoma.

Ovarian lymphomas are rare. We reviewed three cases seen at our hospital to determine any similarities and outcome. The women presented with abdominal discomfort and swelling and were staged as stage I, II and IV. All three cases were B-cell lymphomas confirmed with positive staining with CD45 (LCA), CD20 (L26), CD 45 (4KB) CD45RA (MB1) and MB2 on immunohistochemistry. The two patients with more advanced disease died soon after treatment but the one with stage I (primary ovarian lymphoma) was still alive after 2 years. Ovarian lymphoma when detected late carries a poor prognosis.

Febrile neutropaenia in cancer patients.

BACKGROUNDS: Febrile neutropaenia is a common complication of chemotherapy in cancer patients. Empirical antibiotic regimes are based on the epidemiological characteristics of bacterial isolates globally and locally. METHOD: This study retrospectively reviewed all cases of febrile neutropaenia in patients with confirmed cancer admitted at the University Hospital of the West Indies in the four-year period between, January 1, 2003 and December 31, 2006 and who received chemotherapy. Cases were identified from blood culture records and hospital charts which were reviewed to determine the aetiological agents causing bacteraemia, their antimicrobial susceptibilities and clinicalfeatures. These cases were compared with non-neutropaenic cancer patients admitted with fever. RESULTS: A total of 197 febrile episodes in cancer patients were reviewed. Thirty-seven per cent had febrile neutropaenia while 62% were non-neutropaenic. Acute myeloid leukaemia was the most common haematological malignancy and the most common solid tumour was breast cancer. Twenty-six per cent of patients had a positive blood culture. In febrile neutropaenic patients, Escherichia coli was the most common organism isolated followed by coagulase-negative staphylococci while in non-neutropaenic patients, coagulase-negative staphylococci was most common. Acinetobacter infections was prominent in non-neutropaenic patients but absent in neutropaenic patients. More than one organism was cultured in 9 neutropaenic and 18 non-neutropaenic patients. Mortality was 10.8% in neutropaenic and 24.4% in non-neutropaenic patients. CONCLUSION: Gram-negative organisms are the predominant isolates in febrile neutropaenic episodes in this cohort of patients. Non-neutropaenic patients had an increased mortality with an increase in Acinetobacter infections and multiple isolates.

Cardiovascular disease risk factors in menopausal Jamaican black women after hysterectomy and bilateral oophorectomy: an observational study.

OBJECTIVE: To determine differences in prevalence of cardiovascular risks and diseases in black Jamaican postmenopausal women who had hysterectomy (hysgroup) compared with those without (control). METHOD: Eight hundred and nine (809) women (hysterectomized (HYSGRP) = 403; non-hysterectomized (controls) = 406) were enrolled. Sociodemographic information and lifestyle history, measured blood pressure, waist hip ratio, body mass index, fasting blood glucose, total and HDL cholesterol were obtained. RESULTS: Of the 809 women, complete cardiovascular risk data were available in 341 controls and 328 in the HYSGRP group. There was no difference in mean age, blood pressure and body mass indices between the subjects excluded and the subjects in the data analytical sample. A significantly lower proportion of women in the control group exercised, attained post-secondary education and were of higher parity. Systolic (mean diference with 95% CI; 6 (3, 9) mmHg and diastolic (3 (1, 5) mmHg) blood pressure were lower in the HYSGRP compared with controls but total cholesterol (0.2 (0.07 to 0.4) mmol/L was greater HDL cholesterol was not different between both groups 1.3 mmol/L (SD 0.3) vs 1.3 mmol/L [SD 0.4] (p = 0.8435). There was no difference in the prevalence of diabetes, hypertension and high waist-hip ratio in hysterectomized women compared with controls adjusting for hormone replacement therapy usage, cigarette smoking, exercise and educational status. Within the HYSGRP there was also no diference in cardiovascular disease or risk in women who had bilateral oophorectomy compared with women who had at least an ovary preserved at time of operation. CONCLUSION: Hysterectomy was not associated with an increased risk of cardiovascular disease. This must be taken cautiously since data did not allow for analysis on duration of menopause.

Venous thromboembolism as a complication of uterine fibroids: a retrospective descriptive study.

This study describes cases of fibroids with venous thromboembolism (VTE) managed at UHWI between the years 1999-2004. We examined records to find patients with fibroids and VTE diagnosed before or after surgery. We found 438 women with VTE and 72 of these (16.4%) with fibroids. A total of 1,979 patients had fibroids during the period; cases were therefore 3.63% of fibroids. Cases were younger, median age 44 years than the total cohort: the median age was 57 and the mean age was 55.43 (SD 19.87) p = 0.000 about 9.5 years older than cases. Age over 50 years was less common in cases 27% vs 52% p = 0.0001 as were other risk factors for VTE such as cancer 4.9 vs 14% p = 0.03; diabetes 8.6 vs 25.2% p = 0.004 and cardiac disease 8.6% vs 26.6% p = 0.002 . However, thrombocytosis was more frequent, 23% vs 9% p = 0.008. Other risk factors were not significantly different. A total of 21 cases (29.1%) had surgery for fibroids; 15 (71.4%) hysterectomy, and six (28.5%) myomectomy. Most cases 67/72 (93%) had VTE without surgery. Of the five cases with VTE after surgery for fibroids, none had prophylactic heparin. Of the cases, 15 died--23% of women with VTE at PM and 0.8% of those with fibroids.

Venous thromboembolism in Jamaican women: experience in a university hospital in Kingston.

OBJECTIVE: To review cases of venous thromboembolism (VTE) at UHWI from 1999-2004, to identify methods of diagnosis, risk factors and to evaluate differences between survivors and fatalities. METHODS: Patients coded with the diagnosis of thromboembolism at the University Hospital of the West Indies (UHWI) from 1999-2004 were identified. The medical records were reviewed to determine the prevalence of thromboembolism and possible variables associated with this diagnosis. In addition, variables associated with fatality were examined by evaluating cases diagnosed at autopsy RESULTS: There were 959 patients coded for thromboembolism between 1999-2004 at UHWI. Of these, 657 (68.5%) were females and 302 were males (31.5%). During that period, 65,657 women and 40,826 men were admitted to hospital with prevalence rates for thromboembolism of 1% in women and 0.7% in men. Of the 657 females, 520 case notes were located (case identification 80%). Of this, 435 were analysed as confirmed thromboembolism. The median age was 51 years with a range of 2-95 years. Common associations were obesity, 53.5%; age over 50 years, 52.5%; hypertension, 44.7%; immobilisation, 36.3%; cardiac disease, 26%; diabetes, 19.4%; fibroids, 16.3%; surgery, 15.8% and cancer 14%. Recurrent venous thromboembolism occurred in 12.8% and 15.8 % of women (66) died, diagnosed with PE at post-mortem. Using logistic regression analysis, leading risk factors in fatalities compared to survivors were hypertension and increased age. Obesity and surgery were significantly more likely in survivors. CONCLUSION: Venous thromboembolism was common in this cohort of women and avoidance of risk factors and institution of prophylaxis in high risk women is important to decrease morbidity and mortality.

Extravasation injuries.

OBJECTIVES: To evaluate the time and type of treatment following extravasation from intravenous infusion and the sequelae of the injuries. METHOD: The charts of 12 patients who were referred to the Plastic and Orthopaedic Services at the University Hospital of the West Indies were reviewed. The study period was between May 2003 and January 2007. Data were collected on age, gender, site of extravasation, extravasated agent, treatment of the extravasation, necrosis interval, duration of hospital stay for treatment of injury and whether the intravenous line was resited and at what site in relation to the injury. RESULTS: The age of patients ranged from three days to 67 years. The female-to-male ratio was 2:1. In five patients, the intravenous infusion was discontinued immediately after the swelling was noticed. In two patients, the intravenous infusion was stopped after seven hours and in five patients it was discontinued within 12 to 22 hours. The necrosis interval ranged from 12 hours to three weeks. Immediate treatment following extravasation and discontinuation of the infusion included limb elevation in three patients and application of cold compresses in one patient. Eleven patients developed skin necrosis of varying severities. There was no skin necrosis in one patient. Ten patients spent an average of 31 extra days in hospital for treatment of the extravasation injury. Two patients were treated in an out-patient clinic. CONCLUSIONS: Extravenous leaks can cause severe tissue injuries. Morbidity is increased by delay in recognition and treatment of the extravasation. A protocol for the treatment of extravasation is recommended.

White blood cell counts in healthy Jamaican adults.

The investigation of presumed neutropenia places a burden on the health services, especially those of developing countries, including Jamaica. This may be because the normal ranges used in the laboratory are based on the values generated from the Caucasian population. Previous studies looking at African and Afro-Caribbean groups have found lower counts for these populations compared with Caucasians. To address this issue, 195 healthy adults donating blood at the National Public Health Laboratory and the University Hospital of the West Indies blood banks in Kingston, Jamaica, were screened for complete blood count (CBC) differentials between June 2001 and June 2006. The geometric means for the neutrophil counts were found to be 2.4 x 10(9)/L for men and 2.7 x 10(9)/L for women, with 95% confidence intervals of 2.2-2.8 x 10(9)/L and 2.5-3.1 x 10(9)/L respectively. Values for the Jamaican population were similar to those of other Afro-Caribbean groups. Based on this distribution, 14% of healthy Jamaicans would fall below the normal ranges derived from Caucasians and therefore presumed to have neutropenia. We recommend that the lower reference ranges obtained for Afro-Caribbean adults be adopted for that population.

The combination of zidovudine and interferon alpha-2B in the treatment of adult T-cell leukemia/lymphoma.

Adult T-cell leukemia/lymphoma (ATL) is frequently a very aggressive malignancy with a poor survival despite aggressive multiagent chemotherapy. The combination of the antiretroviral drug zidovudine (AZT) and interferon alpha (IFNalpha) has been reported to induce remissions in patients with ATL. The purpose of this study was to evaluate the clinical response and toxicity following administration of a combination of IFNalpha-2b and AZT in patients with human T-cell lymphotropic virus type I (HTLV-I)-associated ATL. Eighteen patients with ATL (chronic. crisis, acute or lymphoma type) were treated with the combination of AZT (50 - 200 mg orally 5 times a day) and IFNalpha-2b (2.5 - 10 million units subcutaneously daily). Three patients had objective responses lasting more than one month. One patient had a clinical complete remission, lasting 21.6 months and two patients had partial remissions lasting 3.7 and 26.5 months. Six patients were not considered evaluable for response due to short and/or interrupted periods of treatment. Seventeen patients have died with a median survival time after initiation of therapy of 6 months. Neutropenia and thrombocytopenia were the dose limiting toxicities. In conclusion, the response rate in this study was lower than noted in the two previous published series. This may be due to the amount and type of prior treatment our patients had received.

Zidovudine and interferon therapy for adult T-cell leukaemia/lymphoma. Results of a preliminary study at UHWI-Mona.

Seven patients with adult T-cell leukaemia/lymphoma (ATL) were treated with a combination of zidovudine (AZT) and interferon after failed chemotherapy. One patient showed a major response for nine months. The remainder showed progressive disease further complicated by drug toxicity. The poor responses could be explained by patient selection, since most patients had advanced disease refractory to chemotherapy. A larger more protracted study is required for further evaluation of this treatment option.

Lupus anticoagulant in Jamaican primiparae and the clinical significance in asymptomatic patients.

Sera from one thousand pregnant primiparous women from the antenatal clinic of the University Hospital of the West Indies (UHWI) were screened for lupus anticoagulant. Twenty-three women (2.3%) were seropositive. A review was performed after delivery and eight hundred and sixty-eight (87%) of the case notes were found: 20/23 (87%) of those with positive tests and 848/977 (87%) of those with negative tests. The outcome variables examined were pre-eclampsia, postpartum haemorrhage, type of delivery, birth weight of the baby, foetal outcome, foetal distress, still birth, neonatal death, Apgar score at birth and need for admission to the special care nursery. No differences were found between the two groups for any of these variables.

Successful pregnancies in patients with antiphospholipid syndrome treated with low-dose aspirin.

Four patients with bad obstetrical histories and with positive lupus anticoagulant tests were treated with low-dose aspirin, prednisone or heparin. They had pre-term deliveries of live babies who all survived.

Cerebral sinovenous thrombosis in a patient with paroxysmal nocturnal haemoglobinuria.

Paroxysmal nocturnal haemoglobinuria may be complicated by life-threatening venous thrombosis. A patient who presented with the classical clinical picture of cerebral sinus thrombosis is described. Management of the condition is discussed.

Malignant lymphoma of gastric mucosa-associated lymphoid tissue in Jamaica.

The recently appreciated concept of Mucosa-Associated Lymphoid Tissue (MALT) and the characteristics of the lymphomas arising therein are discussed with reference to the clinical, histological and immunohistochemical features of the first four cases of gastric MALT lymphomas diagnosed at the University Hospital of the West Indies. These tumours are low-grade B-cell lymphomas, which may undergo high-grade transformation. They are aetiologically associated with Helicobacter pylori (H pylori) infection in the stomach and may be cured in the early stages with antibiotics. Our cases were diagnosed from gastrectomy specimens removed for suspected carcinoma. All were high-grade, and associated with H pylori; 3 cases presented as advanced disease. Documentation of the features of these lymphomas will increase awareness and earlier recognition.