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This essay describes the rich tradition of research in the English-speaking Caribbean and the possibilities for meaningful collaboration between Caribbean researchers and scientists from developed countries. Significant contributions include work related to the human T-lymphotropic virus type I (HTLV-I), Jamaican vomiting sickness, veno-occlusive disease of the liver, J-type diabetes, and the role of skin sepsis and streptococcal infection in the etiology of glomerulonephritis. In the fields of malnutrition, human metabolism, child development, and sickle cell anemia, the Caribbean has been at the forefront of medical research internationally. Many characteristics of the Caribbean population, including the disease profile, offer advantages and unique opportunities for significant research, despite difficulties related to the "brain drain" and weaknesses of the infrastructure.
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PMID:Is serious research possible in the Caribbean? 184 51

A retrospective review of children having sickle cell anemia and abdominal painful crisis (APC) was performed in order to delineate the pattern of their presenting clinical characteristics. Of 43 children accounting for 106 cases of APC that occurred during a 4-year period, 94 cases involved a child who had had a previous episode of APC. Symptoms included vomiting (10) and concomitant bone or joint pain (42). Physical findings showed 98 children with normoactive bowel sounds, 96 with a nonrigid abdomen to palpation, two with involuntary guarding, and two with rebound tenderness. Certain clinical characteristics are useful in distinguishing children with sickle cell anemia and APC. As compared with traditional findings that suggest a "surgical" abdomen, most of the children who have sickle cell APC do not experience vomiting, and present with normoactive bowel sounds, nonrigid abdomen, and absence of involuntary guarding or rebound tenderness. Children having presumed APC who receive analgesic medication in an outpatient setting with symptomatic improvement should be hospitalized for observation to monitor for recurrence/progression of symptoms indicative of an abdominal disease process requiring surgery.
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PMID:Clinical features of abdominal painful crisis in sickle cell anemia. 231 97

Between January 1976 and December 1978, the Microbiology Department of University College Hospital (UCH) Ibadan, isolated Salmonella typhi from the blood cultures of 93 children aged 0-14 years, who were admitted to the paediatric wards. Clinical case notes were retrieved and reviewed in 64 (68.8%) of them. Fifteen (23%) of the 64 children were less than one year of age while 22 (34%) were under the age of five years. The commonest presenting symptoms were fever, anorexia, diarrhoea and vomiting. A febrile convulsion was the presenting symptom in 13 (20%) of the patients, all of whom were under the age of five years. Hepatomegaly was almost twice as frequently observed as splenomegaly. Intestinal perforation was present in five of the patients. There was a high proportion of SS children who presented with fever, pallor, jaundice, generalized aches and pains and other clinical features of sickle cell disease and it is possible that such children are specially susceptible to typhoid fever. A clinical diagnosis of typhoid fever on admission was made in only 14 of the 64 children. Reasons are given for the low index of suspicion and it is suggested that any child with unremitting fever after adequate anti-malarial chemotherapy should be treated for enteric fever.
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PMID:Problems in the clinical diagnosis of typhoid fever in children in the tropics. 618 69

After the development of monophasic combined oral contraceptives (COCs), containing a fixed dose of estrogen and progestogen, biphasic and triphasic COCs were introduced in the 1980s; in these the dose of ethinyl estradiol and progestogen changes during the pill cycle. In the so-called every day pills, the 21 pills of active steroid combination are followed by 7 inactive pills containing starch, iron, or bran. Method failures of OCs are among the lowest ranging from 0.2-1/100 woman-years. User failures can be as high as 6.2/100 women-years. The individual difference in peak plasma levels of estrogens in women taking identical OCs can be 10-fold. Conditions that affect the bioavailability of contraceptive steroids are: 1) drug interaction (vitamin C, drugs that induce liver enzymes, and antibiotics); 2) vomiting; 3) vegetarianism; 4) missing pills; and 5) malabsorption. Metabolic effects of COCs pertain to carbohydrate metabolism, lipid metabolism, hemostasis, and vitamins. Prescribing of COCs involves counseling clients about contraindications to COCs, starting routines, and the pill-free interval, as well as follow-up and monitoring, the problem of missing pills, and selection criteria for OC use. Medical conditions in which COC use requires special consideration are sickle cell disease, trophoblastic disease, HIV disease, gallstones, epilepsy, valvular heart disease, oligomenorrhea/amenorrhea, inflammatory bowel disease, and surgery. Side effects of COCs may include depression, nausea, vomiting, headaches, urinary tract infection, and lower genital tract infections. 6 months after stopping the OC 1% of users become amenorrheic. Many of the common causes of amenorrhea, such as weight loss amenorrhea and polycystic ovarian disease, may be treated with the COC until the couple desires to have a baby. The new progestogens desogestrel, norgestimate, and gestodene are highly selective compared to first and second generation progestogens.
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PMID:Combined oral contraceptives: acceptability and effective use. 832 4

Cerebral internal venous thrombosis are rare and diagnosis is difficult. We report three cases in male adults. Clinical data were headaches, vomiting, dizziness and coma, in relation with an intracranial hypertension, or in a case, cardiocirculatory arrest. Cerebral internal veinous thrombosis was diagnosed by a CT scan and cerebral angiography twenty four hours after the admission in neurosurgical intensive care. CT scan showed hemorrhagic and ischemic lesions of thalami in two cases, diffuse cerebral edema in two patients, early or delayed hydrocephaly in two cases. No patient survived despite intensive treatment including heparinotherapy, ventricular CSF drainage, osmotherapy, dehydration, barbiturate, other antiepileptic drugs and mechanical ventilation. In two cases, general or local illness was found, sickle cell disease or radiotherapy for pineal tumor, and in case 3 clinical signs evoked autoimmune disease, not demonstrated by biological samples.
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PMID:[Cerebral deep vein thrombosis: three cases]. 1048 48

Blackwater fever is characterized by severe intravascular hemolysis with renal failure caused by recurrent use of quinine for prophylaxis. Once described in European patients, sporadic cases have been reported more and more often in autochthonous Africans and Asians. Newer antimalarials including aminoalchohol mefloquine, and halofantrine have also been implicated in Blackwater fever. In this report we describe two cases of blackwater fever involving patients with sickle cell anemia (HbSS). Symptoms including fever, acute hemolytic anemia, emesis, back pain, and hemoglobinuria were characteristic of blackwater fever. Both patients died. Although the underlying mechanism of blackwater fever remains unclear, a likely explanation is an immunoallergic reaction to quinine. Association with glucose-6-phosphate dehydrogenase deficiency has often been reported. Our cases suggest that blackwater fever may also be correlated with hemoglobinopathy such as HbSS.
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PMID:[Blackwater fever in adults with sickle cell anemia. Two fatal cases]. 1110 Apr 42

Neuroimaging and management advances require review of indications for excluding cerebral venous sinus (sinovenous) thrombosis (CSVT) in children. Our goals were to examine (i) clinical presentations of CSVT, (ii) prothrombotic risk factors and other predisposing events, (iii) clinical and radiological features of brain lesions in CSVT compared with arterial stroke, and (iv) predictors of outcome. We studied 42 children with CSVT from five European paediatric neurology stroke registries. Patients aged from 3 weeks to 13 (median 5.75) years (27 boys; 64%) presented with lethargy, anorexia, headache, vomiting, seizures, focal signs or coma and with CSVT on neuroimaging. Seventeen had prior chronic conditions; of the 25 previously well patients, 23 had recent infections, eight became dehydrated and six had both. Two children had a history compatible with prior CSVT. Anaemia and/or microcytosis (21 probable iron deficiency, five haemolytic, including two with sickle cell disease and one with beta-thalassaemia) was as common (62%) as prothrombotic disorder (13/21 screened). High factor VIII and homozygosity for the thermolabile methylene tetrahydrofolate reductase polymorphism were the commonest prothrombotic disorders. The superficial venous system was involved in 32 patients, the deep in six, and both in four. Data on the 13 children with bland infarction and the 12 with haemorrhage in the context of CSVT were compared with those from 88 children with ischaemic (AIS) and 24 with haemorrhagic (AHS) arterial stroke. In multiple logistic regression, iron deficiency, parietal infarction and lack of caudate involvement independently predicted CSVT rather than arterial disease. Five patients died, three acutely, one after recurrence and one after 6 months being quadriparetic and blind. Follow-up ranged from 0.5 to 10 (median 1) years. Twenty-six patients (62%) had sequelae: pseudotumour cerebri in 12 and cognitive and/or behavioural disabilities in 14, associated with epilepsy in three, hemiparesis in two and visual problems in two. Eighteen patients, including six with haemorrhage, were anticoagulated. Older age [odds ratio (OR) 1.54, 95% confidence limits (CI) 1.12, 2.13, P = 0.008], lack of parenchymal abnormality (OR 0.17, 95% CI 0.02, 1.56, P = 0.1), anticoagulation (OR 24.2, 95% CI 1.96, 299) and lateral and/or sigmoid sinus involvement (OR 16.2, 95% CI 1.62, 161, P = 0.02) were independent predictors of good cognitive outcome, although the last predicted pseudotumour cerebri. Death was associated with coma at presentation. Of 19 patients with follow-up magnetic resonance (MR) venography, three had persistent occlusion, associated with anaemia and longer prodrome. A low threshold for CT or MR venography in children with acute neurological symptoms is essential. Nutritional deficiencies may be modifiable risk factors. A paediatric anticoagulation trial may be required, after the natural history has been further established from registries of cases with and without treatment.
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PMID:Cerebral venous sinus thrombosis in children: risk factors, presentation, diagnosis and outcome. 1569 61

Deferasirox is a once-daily, oral iron chelator developed for treating transfusional iron overload. Preclinical studies indicated that the kidney was a potential target organ of toxicity. As patients with sickle cell disease often have abnormal baseline renal function, the primary objective of this randomised, open-label, phase II trial was to evaluate the safety and tolerability of deferasirox in comparison with deferoxamine in this population. Assessment of efficacy, as measured by change in liver iron concentration (LIC) using biosusceptometry, was a secondary objective. A total of 195 adult and paediatric patients received deferasirox (n = 132) or deferoxamine (n = 63). Adverse events most commonly associated with deferasirox were mild, including transient nausea, vomiting, diarrhoea, abdominal pain and skin rash. Abnormal laboratory studies with deferasirox were occasionally associated with mild non-progressive increases in serum creatinine and reversible elevations in liver function tests. Discontinuation rates from deferasirox (11.4%) and deferoxamine (11.1%) were similar. Over 1 year, similar dose-dependent LIC reductions were observed with deferasirox and deferoxamine. Once-daily oral deferasirox has acceptable tolerability and appears to have similar efficacy to deferoxamine in reducing iron burden in transfused patients with sickle cell disease.
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PMID:A randomised comparison of deferasirox versus deferoxamine for the treatment of transfusional iron overload in sickle cell disease. 1723 48

A common side effect of opioids is nausea and vomiting; however, the incidence in hospitalized patients receiving opioids for acute pain is unknown. We performed a retrospective study in adult patients with sickle cell disease admitted for an acute pain crisis during a six-month period to evaluate the incidence of nausea and vomiting and characterize the prescribing of antiemetics. Eligibility included normal hepatic and renal function. Thirty-four subjects with a total of 97 admissions were evaluated. As expected, opioids were prescribed during all admissions. Fifty percent of the subjects experienced nausea or vomiting during the study period and these same patients accounted for the majority of the admissions, 17 subjects with 71 admissions (Group I). Nausea was reported in 18 (25%) of these admissions and vomiting was reported in 24 (34%) of these admissions. The most common antiemetics prescribed were: prochlorperazine, metoclopramide, and promethazine and antiemetics were ordered during 22 (23%) separate admissions for all subjects included in the study. The clinical benefit of these medications is limited due to uneven documentation. In conclusion, many of our patients experienced nausea or vomiting with antiemetics infrequently prescribed on an as needed basis. This suggests a need for better approaches to manage nausea and vomiting in patients receiving opioids.
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PMID:Evaluation of opioid induced nausea and vomiting in sickle cell disease. 1772 90

Bordetella holmesii is a rare cause of bacteremia. It occurs mainly in hyposplenic patients, such as those affected by sickle cell anemia. The most frequent clinical signs are not very specific: fever, cephalalgia, cough, dyspnea, vomiting, etc. B. holmesii is frequently isolated from blood cultures. We describe the case of a 26-year-old sickle cell patient, presenting with dry cough and fever caused by a B. holmesii blood stream infection, identified by 16S rRNA gene sequencing. The outcome was favorable with amoxicillin. It is useful to know about B. holmesii, especially for physicians managing sickle cell or hyposplenic patients, because of its variable susceptibility to beta-lactams.
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PMID:[Bordetella holmesii bacteremia in a 26-year-old patient with sickle cell disease]. 1958 32


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