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OBJECTIVE: Children with sickle cell disease (SCD) are at risk for neurocognitive deficits that can affect school performance, and psychosocial functioning. The aim of this study was to assess the academic performance of school-aged children with SCD in Jamaica compared to their school peers. METHOD: A cross-sectional survey of academic performance was done in a group of children 11 to 13 years of age, using a standardized state administered examination, the Grade Six Achievement Test (GSAT), covering 5 subjects. Scores were obtained from the Ministry of Education (MOE) for eligible children with SCD, as well as mean scores with standard deviation for unaffected classmates by gender. Socio-demographic and clinical data were obtained from our sickle cell clinic database and an interview administered questionnaire. RESULTS: Sixty-four children satisfied eligibility criteria. Children with SCD had lower percentage scores and significantly lower mean z-scores for 4 of 5 subjects (p < 0.05). Males had significantly lower mean z-scores compared with females. Thirty-seven children (57.8%) were classified as underperformers. Haemoglobin level was a significant predictor of subject score rank. CONCLUSION: Children with SCD in Jamaica perform worse in standardized school examinations than their class peers with boys being particularly vulnerable.
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Desempenho Acadêmico , Anemia Falciforme , Masculino , Feminino , Humanos , Criança , Jamaica/epidemiologia , Estudos Transversais , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , EscolaridadeRESUMO
The association between severe acute malnutrition (SAM) in early childhood and liver fat in adults is unknown. We hypothesized that exposure to SAM, especially severe wasting, is associated with fatty liver later in life. In this observational study, abdominal CT was used to quantify mean liver attenuation (MLA) and liver:spleen attenuation ratio (L/S). Birth weight (BW), serum lipids, insulin resistance (homeostatic model assessment), anthropometry and intrabdominal fat were collected. Mean differences between diagnostic groups were tested and hierarchical regression analysis determined the best predictors of liver fat. We studied 88 adult SAM survivors and 84 community participants (CPs); age 29.0 ± 8.4 years, BMI 23.5 ± 5.0 kg/m2 (mean ± SDs). SAM survivors had less liver fat than CPs (using L/S) (p = 0.025). Severe wasting survivors (SWs) had lower BW (-0.51 kg; p = 0.02), were younger, thinner and had smaller waist circumference than oedematous malnutrition survivors (OMs). In the final regression model adjusting for age, sex, birth weight and SAM phenotype (i.e., oedematous malnutrition or severe wasting), SWs had more liver fat than OMs (using MLA) (B = 2.6 ± 1.3; p = 0.04) but similar liver fat using L/S (p = 0.07) and lower BW infants had less liver fat (MLA) (B = -1.8 ± 0.8; p = 0.03). Greater liver fat in SWs than OMs, despite having less body fat, supports our hypothesis of greater cardiometabolic risk in SWs. Other postnatal factors might influence greater liver fat in survivors of severe wasting, suggesting the need to monitor infants exposed to SAM beyond the acute episode.
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Desnutrição Aguda Grave , Tecido Adiposo , Peso ao Nascer , Pré-Escolar , Edema/complicações , Humanos , Lactente , Fígado , Desnutrição Aguda Grave/complicações , SobreviventesRESUMO
EXpanding Treatment for Existing Neurological Disease (EXTEND) investigated whether hydroxycarbamide lowers transcranial Doppler (TCD) velocities in Jamaican children with sickle cell anaemia (SCA) and elevated TCD velocity with or without previous stroke. Forty-three children (age 2-17 years) with baseline maximum time-averaged mean velocity (TAMV) ≥ 170 cm/s were stratified into three risk categories based on treatment status and stroke history: Group 1 (no history of stroke, on hydroxycarbamide, n = 12); and Groups 2 (no stroke, no hydroxycarbamide, n = 21) and 3 (previous stroke, no hydroxycarbamide, n = 10). Open-label hydroxycarbamide at 20 mg/kg/day was commenced, with escalation to maximum tolerated dose (MTD) based on mild marrow suppression (average dose 25·4 ± 4·5 mg/kg/day). TCD was performed every six months with brain magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) at baseline and after 18-months of hydroxycarbamide. The maximum TAMV decreased significantly compared to baseline (24 ± 30 cm/s, P < 0·0001), with similar declines in all groups. Clinical stroke occurred in five children, one in Group 1, none in Group 2, and four in Group 3, P = 0·0032, comparing group incidence rates. Brain MRI/MRA was stable in children without clinical stroke. EXTEND documents the feasibility and benefits of hydroxycarbamide at MTD to lower TCD velocities and reduce stroke risk in children with SCA and no history of primary stroke in low-resource settings without transfusion management.
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Anemia Falciforme/tratamento farmacológico , Antidrepanocíticos/uso terapêutico , Circulação Cerebrovascular , Transtornos Cerebrovasculares/etiologia , Hidroxiureia/uso terapêutico , Ultrassonografia Doppler Transcraniana , Adolescente , Anemia Falciforme/fisiopatologia , Velocidade do Fluxo Sanguíneo , Transtornos Cerebrovasculares/fisiopatologia , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Jamaica , Imageamento por Ressonância Magnética , Masculino , Neuroimagem , Estudos Prospectivos , Recidiva , Método Simples-Cego , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/prevenção & controleRESUMO
BACKGROUND: Nonobese nonalcoholic fatty liver disease is reported in several populations. However, because persons of African origin display unique fat accumulation, insulin resistance, and lipid profiles, we investigated fatty liver in nonobese persons of African origin. METHOD: We recruited 78 urban Jamaican volunteers. CT was used to estimate liver and abdominal fat and dual-energy X-ray absorptiometry to measure body composition. Fasting blood was collected for lipids, alanine aminotransferase (ALT), adiponectin, and fetuin-A. Homeostatic model assessment of insulin resistance (HOMA-IR), whole-body insulin sensitivity index (WBISI), insulinogenic index (IGI), and oral disposition index (oDI) were calculated after a 75-g oral glucose tolerance test. RESULTS: Fifty-two percent of participants were male; mean (±SD) age was 28.5 ± 7.8 years, and body mass index was 22.4 ± 3.0 kg/m2. Mean liver attenuation (MLA) and liver/spleen (LS) ratio, both inversely correlated to liver fat, were 62.8 ± 4.3 HU and 1.2 ± 0.1, respectively; 3.8% of participants had liver fat >30% (LS ratio < 1). In age, sex, and BMI-adjusted correlations, MLA was negatively associated with weight (r = -0.30; P = 0.009) and height (r = -0.28; P = 0.017) and was associated with fasting glucose (r = 0.23; P = 0.05), fasting insulin (r = 0.42; P ≤ 0.001) and HOMA-IR (r = 0.35; P = 0.004). Serum lipids, ALT, adiponectin, fetuin-A, WBISI, IGI, and oDI were not associated with liver fat. CONCLUSIONS: In nonobese Afro-Caribbean participants, greater liver fat was associated with weight and height and lower fasting insulin and hyperinsulinemia appears to be influential in the reduction of NAFLD. These findings may be influenced by ethnicity, body size, and method of estimating liver fat.
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This study investigated the association of nutritional and haematological variables with maximum time-averaged mean velocity (TAMV) measured by transcranial Doppler (TCD) velocity and the agreement of classification between two protocols. TCD categories included: normal (<170 cm/s), conditional (170-199 cm/s) and abnormal (≥200 cm/s) based on TAMV in distal internal carotid artery (dICA), middle cerebral artery (MCA), internal carotid bifurcation, anterior and posterior cerebral arteries. Of 358 children with sickle cell anaemia (SCA) examined, the mean age (±standard deviation) was 7·4 ± 2·7 years; 13·1% and 6·7% had conditional and abnormal velocities, respectively. Children with abnormal TCD velocities had higher prevalence of prior stroke (P = 0·006). Increased TAMV was associated with younger age (P = 0·001), lower weight (P = 0·001), height (P = 0·007) and oxygen saturation (P = 0·005). There was no association of TAMV with height-age or body mass index (BMI) z-scores. Adjusting for gender, BMI z-score, age, previous stroke and oxygen saturation, mean corpuscular volume (P = 0·005) and reticulocyte count (P = 0·013) were positively associated with TAMV, while haemoglobin concentration (P = 0·009) was negatively associated. There was good agreement [99%; weighted Kappa 0·98 (95% confidence interval 0·89-1), P = 0·0001] in TCD classification using data from five vessels versus two vessels (dICA and MCA). Haematological variables, rather than nutritional status, may be useful markers that identify high-risk children with SCA.
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Anemia Falciforme , Artérias Cerebrais , Circulação Cerebrovascular , Hemoglobinas/metabolismo , Estado Nutricional , Ultrassonografia Doppler Transcraniana , Fatores Etários , Anemia Falciforme/sangue , Anemia Falciforme/diagnóstico por imagem , Anemia Falciforme/fisiopatologia , Biomarcadores/sangue , Velocidade do Fluxo Sanguíneo , Peso Corporal , Artérias Cerebrais/diagnóstico por imagem , Artérias Cerebrais/fisiopatologia , Criança , Pré-Escolar , Feminino , Humanos , Jamaica , Masculino , Fatores SexuaisRESUMO
BACKGROUND: Cerebral vasculopathy in sickle cell anemia (SCA) begins in childhood and features intracranial arterial stenosis with high risk of ischemic stroke. Stroke risk can be reduced by transcranial doppler (TCD) screening and chronic transfusion therapy; however, this approach is impractical in many developing countries. Accumulating evidence supports the use of hydroxyurea for the prevention and treatment of cerebrovascular disease in children with SCA. Recently we reported that hydroxyurea significantly reduced the conversion from conditional TCD velocities to abnormal velocities; whether hydroxyurea can be used for children with newly diagnosed severe cerebrovascular disease in place of starting transfusion therapy remains unknown. OBJECTIVE: The primary objective of the EXpanding Treatment for Existing Neurological Disease (EXTEND) trial is to investigate the effect of open label hydroxyurea on the maximum time-averaged mean velocity (TAMV) after 18 months of treatment compared to the pre-treatment value. Secondary objectives include the effects of hydroxyurea on serial TCD velocities, the incidence of neurological and non-neurological events, quality of life (QOL), body composition and metabolism, toxicity and treatment response, changes to brain magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA), genetic and serologic markers of disease severity, and cognitive and pulmonary function. METHODS: This prospective Phase II trial will enroll children with SCA in Jamaica, between the ages of 2 and 17 years, with either conditional (170-199 cm/sec) or abnormal (≥ 200 cm/sec) TCD velocities. Oral hydroxyurea will be administered daily and escalated to the maximum tolerated dose (MTD). Participants will be seen in the Sickle Cell Unit (SCU) in Kingston, Jamaica monthly until achieving MTD, and then every 3 months. TCD will be performed every 6 months. RESULTS: Currently, 43 participants have been enrolled out of a projected 50. There was one withdrawal due to immigration, with no permanent screen failures. Of the 43 enrolled, 37 participants have initiated study treatment. CONCLUSIONS: This trial investigates the effects of hydroxyurea treatment at MTD in children with conditional or abnormal TCD velocities before transfusion therapy and may represent an important advance towards establishing a suitable non-transfusion protocol for stroke prevention in children with SCA. The trial outcomes will have profound significance in developing countries where the disease burden is highest. CLINICALTRIAL: ClinicalTrials.gov NCT02556099; https://clinicaltrials.gov/ct2/show/NCT02556099 (Archived by WebCite at http://www.webcitation.org/6k1yMAa9G).
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Summary We report a case with interesting imaging findings as well as an unfortunate but not unexpected clinical outcome. Our patient, an 8-year-old Jamaican boy of Afro-Caribbean descent with homozygous sickle cell disease, presented with left-sided upper limb weakness. He had a history of recurrent cerebrovascular accidents and transient ischaemic attacks beginning at 4â years of age. MRI revealed old bilateral infarctions and the ivy sign on fluid-attenuated inversion recovery sequences. MR angiography demonstrated numerous collaterals, most apparently arising from the left internal carotid, consistent with moyamoya syndrome. The patient had a full recovery and remained well for almost 2â years when he suffered another stroke.
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Anemia Falciforme/complicações , Doença de Moyamoya/complicações , Acidente Vascular Cerebral/etiologia , Anemia Falciforme/diagnóstico , Angiografia Cerebral , Criança , Diagnóstico Diferencial , Humanos , Angiografia por Ressonância Magnética , Masculino , Doença de Moyamoya/diagnóstico , Recidiva , Acidente Vascular Cerebral/diagnóstico , Síndrome , Tomografia Computadorizada por Raios XRESUMO
We present a case of a 44-year-old man who visited his general practitioner for recurrent neck swelling, which was found to be a neck abscess. It was aspirated, later recurred, and then surgically excised. Histology was consistent with an infected branchial cyst. Eight months after discharge, the patient presented with a history of progressive neck pain and stiffness and eventually bilateral upper limb weakness. MRI demonstrated a prevertebral abscess complicated by cervical osteomyelitis and atlanto-axial instability. The abscess was drained and appropriate antibiotic treatment was administered. The patient responded well with full recovery of his upper limb strength and resolution of the abscess. However, he had mild persistent neck stiffness.
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Articulação Atlantoaxial , Branquioma/diagnóstico , Neoplasias de Cabeça e Pescoço/diagnóstico , Luxações Articulares/diagnóstico , Osteomielite/diagnóstico , Abscesso Retrofaríngeo/diagnóstico , Infecções Estafilocócicas/diagnóstico , Adulto , Branquioma/complicações , Neoplasias de Cabeça e Pescoço/complicações , Humanos , Luxações Articulares/etiologia , Masculino , Osteomielite/etiologia , Recidiva , Abscesso Retrofaríngeo/etiologia , Infecções Estafilocócicas/etiologiaRESUMO
We present a case of a postmenopausal female who visited her doctor for a slowly growing mass in the vulval region. The mass did not show typical clinical features of a vulval neoplasm. Magnetic resonance imaging (MRI) revealed a mass arising from the undersurface of the symphysis pubis. A literature review revealed characteristics of this lesion compatible with a subpubic cartilaginous cyst-a rare benign degenerative condition of the symphysis pubis. As a result the mass was managed non-operatively and the patient remains well 2 years later. This article highlights the role of MRI in the diagnosis of this unusual vulval mass.
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BACKGROUND: Fatty infiltration of the liver is associated with an increased morbidity and mortality in children with severe protein-energy malnutrition (PEM), but its pathogenesis remains unclear. Although impaired synthesis of VLDL apolipoprotein B-100 (VLDL-apo B-100) is generally accepted as the pathogenetic mechanism, the rate of it synthesis has not been measured in children with PEM. OBJECTIVE: The objective of the study was to ascertain the relation between the degree of hepatic steatosis and the rate of VLDL-apo B-100 synthesis in children with PEM. DESIGN: The fractional and absolute rates of VLDL-apo B-100 synthesis were measured with a prime-constant intravenous infusion of [2H3]leucine in 13 severely malnourished children (8 boys and 5 girls) aged 7-18 mo. Hepatic fat content was estimated by computerized tomography scanning by using the ratio of liver to spleen (L:S) attenuation. The ratio is inversely related to hepatic fat content such that the lower the L:S, the greater the amount of fat in the liver. RESULTS: There were significant inverse relations between L:S attenuation and VLDL-apo B-100 concentration (P < 0.02), the absolute rate of VLDL-apo B-100 synthesis (P < 0.02), and plasma triacylglycerol (P < 0.02) and serum cholesterol (P < 0.05) concentrations. CONCLUSIONS: These results suggest that children with PEM synthesize VLDL-apo B-100 at a faster rate as the degree of hepatic fat infiltration increases. Thus, fatty infiltration of the liver in PEM is not due to a reduction in the synthesis of VLDL-apo B-100.
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Apolipoproteínas B/biossíntese , Fígado Gorduroso/metabolismo , Desnutrição Proteico-Calórica/metabolismo , Apolipoproteína B-100 , Feminino , Hospitalização , Humanos , Lactente , Masculino , Desnutrição Proteico-Calórica/terapia , Tomografia Computadorizada por Raios XRESUMO
Impalpable breast lesions that are detected during screening mammography for breast cancer must be accurately identified and adequately sampled in biopsy specimens. Open wire-localized breast biopsy using hookwires remains the main method of sampling these lesions in centres without expensive stereotactic facilities. However, the hookwires can shift or become dislodged in the biopsy specimen. We have successfully modified a simple technique for the localization of impalpable lesions in these biopsies. The insertion of a small hypodermic needle into the fixed specimen with the assistance of the compression paddle and crosshairs on the mammography machine resulted in the precise localization of clusters of microcalcifications in 15 of 16 (94%) cases. In contrast, calcifications were identified in sections taken in the plane of the hookwire in only three cases (19%). The rate of detection of malignancy was 50% and the majority of malignant lesions were represented by ductal carcinoma in situ.
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Biópsia por Agulha/instrumentação , Neoplasias da Mama/patologia , Mama/patologia , Calcinose/patologia , Feminino , Humanos , MamografiaRESUMO
Impalpable breast lesions (AU)
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Humanos , Neoplasias da Mama/diagnóstico , Neoplasias da Mama , Jamaica , Biópsia/métodos , Calcinose/diagnóstico , Região do Caribe , Mamografia , Instrumentos CirúrgicosRESUMO
AIM: We sought to determine the relationship between age, and other clinical characteristics such as parity, oestrogen use, dietary factors and menstrual history on breast density in Jamaican women. METHODS AND MATERIALS: A retrospective study was done of 891 patients who attended the breast imaging unit. The clinical characteristics were extracted from the patient records. Mammograms were assessed independently by two radiologists who were blinded to the patient clinical characteristics. Breast densities were assigned using the American College of Radiology (ACR) classification. RESULTS: The concordance between the ACR classification of breast density between the two independent radiologists was 92% with k = 0.76 (SE = 0.02, P < 0.001). Women with low breast density were heavier (81.3 +/- 15.5 kg vs 68.4 +/- 14.3 kg, P < 0.0001, mean +/- standard deviation (SD)) and more obese (body mass index (BMI), 30.3 +/- 5.8 kg m(-2) vs 26.0 +/- 5.2 kg m(-2), P < 0.0001). Mammographic breast density decreased with age. The age adjusted odds ratios (ORs) for predictors significantly related to high breast density were parity, OR = 0.79 (95%CIratio0.71, 0.88), weight, OR = 0.92 (95% CIratio0.91, 0.95), BMI, OR = 0.83 (95% CIratio0.78, 0.89), menopause, OR = 0.51 (95% CIratio0.36, 0.74) and a history of previous breast surgery, OR 1.6 (95% CIratio1.1, 2.3). CONCLUSION: The rate decline of breast density with age in our population was influenced by parity and body composition.
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Envelhecimento/fisiologia , Negro ou Afro-Americano , Mama/fisiologia , Mamografia , Adulto , Idoso , Envelhecimento/etnologia , Antropometria , Índice de Massa Corporal , Terapia de Reposição de Estrogênios , Feminino , Humanos , Jamaica/etnologia , Pessoa de Meia-Idade , Obesidade/fisiopatologia , Razão de Chances , Paridade , Estudos RetrospectivosRESUMO
One per cent of all brain tumours and twenty per cent of meningiomas eventually develop an extracranial extension. The least common site is the neck. We report a case of malignant meningioma with extension into the neck of a 39-year-old male.
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Adulto , Humanos , Masculino , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Neoplasias Meníngeas/tratamento farmacológico , Meningioma/tratamento farmacológico , Prognóstico , Encéfalo/patologia , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Neoplasias de Cabeça e Pescoço/terapia , Jamaica , Meningioma/terapiaRESUMO
One per cent of all brain tumours and twenty per cent of meningiomas eventually develop an extracranial extension. The least common site is the neck. We report a case of malignant meningioma with extension into the neck of a 39-year-old male.(Au)
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Adulto , Relatos de Casos , Humanos , Masculino , Meningioma/tratamento farmacológico , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Neoplasias Meníngeas/tratamento farmacológico , Jamaica , Cérebro/patologia , Neoplasias de Cabeça e Pescoço/terapia , Imageamento por Ressonância Magnética , Meningioma/terapia , Prognóstico , Tomografia Computadorizada por Raios XRESUMO
Aneurysms of the brain were probably first recognized at necropsy by Morgagni in the 18th century. Autopsy data are at variance with the incidental findings in infancy and later life. Factors such as growth of aneurysm, size at equilibrium, clinical profile and radiological methods of detection play a role in determining the probability of an aneurysm being present and detectable in an individual at a given time. Most aneurysms do not rupture. Most intracranial aneurysms are found on the Circle of Willis (90 percent), the anterior communicating artery being most often quoted as the predominant site (30 to 35 percent). We have reviewed the cases presenting to the University Hospital of the West Indies between 1954 and 1997 in order to determine the distribution of intracranial aneurysms at that institution and potential management implications.(AU)
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Lactente , Humanos , Relatos de Casos , Aneurisma Intracraniano , JamaicaRESUMO
Inflammatory pseudotumour of the orbit is an unusual condition of unknown aetiology which rarely extends beyond the orbit. To our knowledge 19 cases with intracranial extension have been reported, one of which involved the pituitary fossa or sphenoid sinus. Most required cytotoxic agents, surgery or radiotherapy in addition to corticosteroids. We present a case of orbit pseudotumour with extension into the pituitary fossa, sphenoid sinus and cavernous sinuses with vascular compression. Marked clinical improvement occurred on steroid therapy alone.(AU)