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OBJECTIVE: To describe the unusual clustering of systemic lupus erythematosus (SLE) in a family from the Cayman Islands. METHOD: An observational retrospective study of SLE was done following an index case of mixed connective tissue disease in a 51-year old West Indian woman of African descent. Her two daughters of the same father, who is of Cayman Islands origin, were also diagnosed with SLE. A family tree was subsequently drawn up to 1890 to identify other cases in the same family. RESULTS: There were 13 cases identified and all occurred between the 6th and the 8th generation. A family tree linked all cases to a man from the Cayman Islands who died in 1890. The nine cases with full medical records showed eight females and one male (8:1). The mean age at diagnosis was 29 years; polyarthritis occured in all nine patients (100%), kidney involvement in 6/9 (66.6%), skin rash in 6/9 (66.6%), pleuritis and pericarditis in 6/9 (66.6%) and anaemia in 6/9 (66.6%). The autoantibodies were mainly ANA in all patients (100%) and anti-dsDNA in 8/9 (88.8%). CONCLUSION: The unusual extensive familial clustering in this study represents the first to be described in a West Indian population where SLE is most prevalent and may suggest a genetic predisposition.

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The 2009 Pandemic Novel Influenza A [HIN1] resulted in mild disease mostly but severe cases and death were associated with pneumonia, respiratory failure and multi-organ failure. We present a case of severe disease with acute heart failure and arrhythmia due to fulminant myocarditis in a 50- year old obese man with diabetes mellitus.

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Yellow nail syndrome is a very rare clinical entity usually diagnosed from a combination of yellow dystrophic nails, lymphoedema and respiratory diseases. The aetiology is not known though dysfunctional hypoplastic lymphatics is speculated. Most cases occur sporadically but few cases may be associated with systemic diseases or may be inherited. This report documents another case in a 56-year old Caribbean female who presented with a six-year history of recurrent respiratory symptoms and later yellow dystrophic nails and lymphoedema. She responded well to vitamin E and oral fluconazole. We also did a short literature review of yellow nail syndrome.

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The 2009 Pandemic Novel Influenza A [H1N1] resulted in mild disease mostly but severe cases and death were associated with pneumonia, respiratory failure and multi-organ failure. We present a case of severe disease with acute heart failure and arrhythmia due to fulminant myocarditis in a 50-year old obese man with diabetes mellitus.

La nueva gripe A [H1N1] pandémica resultó ser una enfermedad leve en su mayor parte, pero se produjeron casos graves y muertes asociadas con neumonía, insuficiencia respiratoria y fallo multiorgánico. Presentamos un caso de enfermedad severa con insuficiencia cardíaca aguda y arritmia debido a miocarditis fulminante en un hombre obeso de 50 años de edad con diabetes mellitus.

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Yellow nail syndrome is a very rare clinical entity usually diagnosed from a combination of yellow dystrophic nails, lymphoedema and respiratory diseases. The aetiology is not known though dysfunctional hypoplastic lymphatics is speculated. Most cases occur sporadically but few cases may be associated with systemic diseases or may be inherited. This report documents another case in a 56-year old Caribbean female who presented with a six-year history of recurrent respiratory symptoms and later yellow dystrophic nails and lymphoedema. She responded well to vitamin E and oral fluconazole. We also did a short literature review of yellow nail syndrome.

El síndrome de las uñas amarillas es una entidad clínica muy rara, la cual usualmente se diagnostica a partir de una combinación de uñas amarillas distróficas, linfedemas, y enfermedades respiratorias. Se desconoce la etiología, aunque se especula que se debe a vasos linfáticos hipoplásticos disfuncionales. La mayoría de los casos ocurre esporádicamente pero pocos casos pueden asociarse con las enfermedades sistémicas o pueden ser heredados. Este informe documenta el caso de una mujer caribeña de 56 años, que se presentó con antecedentes de síntomas respiratorios recurrentes y más tarde con uñas amarillas distróficas y linfedemas. Durante el tratamiento, respondió bien a la vitamina E y al fluconazol oral. El trabajo también realiza una breve revisión de la literatura del síndrome de las uñas amarillas.

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The aim of the study was to assess, by echocardiography, the cardiac abnormalities in a group of patients with chronic renal failure and to determine the cardiovascular predictors of mortality. The study comprised forty-five patients from the Renal Unit, University Hospital of the West Indies, Kingston, Jamaica, and was undertaken between October 1, 1998 and July 31, 2000. All echocardiography was done by a single operator. The parameters assessed were systolic dysfunction, diastolic dysfunction, ejection fraction, regional wall motion abnormalities and valvular disease. Left ventricular cavity size, septal and posterior wall thickness were measured and left ventricular mass calculated. Demographic data were obtained directly from each patient by interview. The patients were mainly of African/mixed-African origin. Their mean age was 43.2 +/- 16.0 years. The average body mass index was 23.7 +/- 6.9. Twenty-eight (60.9%) patients were male and seventeen (39.1%) female. Hypertension, chronic glomerulonephritis and diabetes mellitus were the leading causes of chronic renal failure. Blood pressure was controlled at a mean value of 145/90 mm Hg pre-dialysis and 140/90 mm Hg postdialysis. The mean duration of renal failure was 2.8 years. Echocardiographic M-mode and two dimensional apical, four chamber view measurements indicated that mean left ventricular internal diameter (LVID) diastole was 55.7 +/- 7.9 mm (normal 38-56 mm) and LVID systole was 38.9 +/- 9.8 mm (normal 24-45 mm); the mean thickness of the chamber walls was 10.3 +/- 2.8 mm and 10.6 +/- 2.4 mm for the interventricular septum (normal 6-11 mm) and left ventricular posterior wall (normal 6-11 mm) respectively. Diastolic dysfunction was seen in 15 (34%) patients and systolic dysfunction in 12 (23%) patients who had ejection fractions less than 50%. The mean left ventricular ejection fraction was 56.3% +/- 16% (normal 65-85%), mean stroke volume was 82.9 +/- 27.2 mls (normal 51-96 ml). After 21 months enrolment in the study, Kaplan Meier analysis revealed a two-year mortality of 28.3%. Cox regression analysis indicated that a history of smoking current or past, low haemoglobin level, high aorta flow velocities, severity of mitral regurgitation and a negative association with serum creatinine were independent predictors of mortality. The correction of anaemia and control of other factors that impact negatively on cardiac function in dialysis patients is vital to enhance survival.

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Tuberous sclerosis complex manifests predominantly as a neurocutaneous disorder Lung involvement was considered rare. Lymphangioleiomyomatosis which occurs mainly in women of childbearing age is the major pulmonary disorder seen in tuberous sclerosis. Multifocal micronodular pneumocyte hyperplasia has also been described in tuberous sclerosis. The case of a 51-year old female diagnosed with tuberous sclerosis is described after she presented with progressive shortness of breath and was found to have interstitial lung disease. Tuberous sclerosis should be considered as a differential in patients with interstitial lung disease especially in association with cutaneous lesions.

Assuntos

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The aim of the study was to assess, by echocardiography, the cardiac abnormalities in a group of patients with chronic renal failure and to determine the cardiovascular predictors of mortality. The study comprised forty-five patients from the Renal Unit, University Hospital of the West Indies, Kingston, Jamaica, and was undertaken between October 1, 1998 and July 31, 2000. All echocardiography was done by a single operator. The parameters assessed were systolic dysfunction, diastolic dysfunction, ejection fraction, regional wall motion abnormalities and valvular disease. Left ventricular cavity size, septal and posterior wall thickness were measured and left ventricular mass calculated. Demographic data were obtained directly from each patient by interview. The patients were mainly of African/mixed-African origin. Their mean age was 43.2 +/- 16.0 years. The average body mass index was 23.7 +/- 6.9. Twenty-eight (60.9%) patients were male and seventeen (39.1%) female. Hypertension, chronic glomerulonephritis and diabetes mellitus were the leading causes of chronic renal failure. Blood pressure was controlled at a mean value of 145/90 mm Hg pre-dialysis and 140/90 mm Hg postdialysis. The mean duration of renal failure was 2.8 years. Echocardiographic M-mode and two dimensional apical, four chamber view measurements indicated that mean left ventricular internal diameter (LVID) diastole was 55.7 +/- 7.9 mm (normal 38-56 mm) and LVID systole was 38.9 +/- 9.8 mm (normal 24-45 mm); the mean thickness of the chamber walls was 10.3 +/- 2.8 mm and 10.6 +/- 2.4 mm for the interventricular septum (normal 6-11 mm) and left ventricular posterior wall (normal 6-11 mm) respectively. Diastolic dysfunction was seen in 15 (34%) patients and systolic dysfunction in 12 (23%) patients who had ejection fractions less than 50%. The mean left ventricular ejection fraction was 56.3% +/- 16% (normal 65-85%), mean stroke volume was 82.9 +/- 27.2 mls (normal 51-96 ml). After 21 months enrolment in the study, Kaplan Meier analysis revealed a two-year mortality of 28.3%. Cox regression analysis indicated that a history of smoking current or past, low haemoglobin level, high aorta flow velocities, severity of mitral regurgitation and a negative association with serum creatinine were independent predictors of mortality. The correction of anaemia and control of other factors that impact negatively on cardiac function in dialysis patients is vital to enhance survival.

Assuntos

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Tuberous sclerosis complex manifests predominantly as a neurocutaneous disorder Lung involvement was considered rare. Lymphangioleiomyomatosis which occurs mainly in women of childbearing age is the major pulmonary disorder seen in tuberous sclerosis. Multifocal micronodular pneumocyte hyperplasia has also been described in tuberous sclerosis. The case of a 51-year old female diagnosed with tuberous sclerosis is described after she presented with progressive shortness of breath and was found to have interstitial lung disease. Tuberous sclerosis should be considered as a differential in patients with interstitial lung disease especially in association with cutaneous lesions.

El complejo de esclerosis tuberosa se manifiesta predominantemente como un desorden neurocutáneo. El compromiso pulmonar era considerado raro. La linfangioleiomiomatosis que se presenta principalmente en mujeres en estado de gestación, es el principal trastorno observado en la esclerosis tuberosa. La hiperplasia micronodular pneumocítica ha sido también descrita en la esclerosis tuberosa. Se describe el caso de una mujer de 51 años a quien se le diagnosticó esclerosis tuberosa, luego de que se presentara con disnea y se hallara que padecía la enfermedad intersticial del pulmón. La esclerosis tuberosa deber ser considerada como un diagnóstico diferencial en pacientes con la enfermedad intersticial del pulmón, especialmente cuando se encuentra asociada con lesiones cutáneas.

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The colon responds monomorphically to a variety of insults thus making it difficult to differentiate invasive amoebic colitis and inflammatory bowel disease (IBD). The authors present a case with chronic dysentery, haematochezia, anaemia and hypoproteinaemia. The endoscopic findings were suggestive of IBD. The stool examination was negative for trophozoites or cysts of parasites. The recto-colonic biopsy specimens showed mucosal inflammation with exudates containing amoebic trophozoites. The patient was successfully treated with metronidazole and iodoquinol. He recovered within two weeks and repeat colonoscopy four weeks after the treatment showed a normal rectum and colon. Clinicians should have a high level of suspicion for amoebic colitis in cases of colitis especially in regions where amoebiasis is still present. Efforts should be made to find the amoebic trophozoites in multiple stool and colonic biopsy specimens.

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The colon responds monomorphically to a variety of insults thus making it difficult to differentiate invasive amoebic colitis and inflammatory bowel disease (IBD). The authors present a case with chronic dysentery, haematochezia, anaemia and hypoproteinaemia. The endoscopic findings were suggestive of IBD. The stool examination was negative for trophozoites or cysts of parasites. The recto-colonic biopsy specimens showed mucosal inflammation with exudates containing amoebic trophozoites. The patient was successfully treated with metronidazole and iodoquinol. He recovered within two weeks and repeat colonoscopy four weeks after the treatment showed a normal rectum and colon. Clinicians should have a high level of suspicion for amoebic colitis in cases of colitis especially in regions where amoebiasis is still present. Efforts should be made to find the amoebic trophozoites in multiple stool and colonic biopsy specimens

El colon responde de manera monomórfica a una variedad de insultos, lo cual hace difícil distinguir entre la colitis amebiana invasiva y la enfermedad intestinal inflamatoria (EII). Los autores presentan un caso con disentería crónica, hematoquexia, anemia e hipoproteinemia. Los resultados endoscópicos apuntaban a una EII. El análisis de las heces fecales arrojó resultados negativos en cuanto a presencia de trofozoitos o quistes de parásitos. Esto condujo a un diagnóstico erróneo y el paciente fue tratado por una EII. Sin embargo, los especímenes de la biopsia rectocolónica mostraron una inflamación mucosal con exudados en los que se hallaban presentes trofozoitos amebianos. El paciente tuvo un tratamiento exitoso con metronidazol y iodoquinol. Se recuperó en dos semanas, y se le repitió la colonoscopia cuatro semanas después de que el tratamiento mostró un recto y colon normales. Los clínicos debían mostrar un alto nivel de sospecha ante la colitis amebiana, especialmente en aquellas regiones donde la amebiasis todavía está presente. Deben hacerse esfuerzos por encontrar trofozoitos amebianos en múltiples especímenes de heces fecales y biopsia colónica.

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This paper reports the case of a 21-year-old Afro-Caribbean pregnant woman with hyperthyroidism and hypokalaemic quadriparesis and reviews the literature on the topic. Thyrotoxic periodic paralysis is a very rare condition in the Caribbean. This case reminds West Indian physicians to consider this rare condition in any patient that presents with paralysis.

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This paper reports the case of a 21-year-old Afro-Caribbean pregnant woman with hyperthyroidism and hypokalaemic quadriparesis and reviews the literature on the topic. Thyrotoxic periodic paralysis is a very rare condition in the Caribbean. This case reminds West Indian physicians to consider this rare condition in any patient that presents with paralysis

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A sonographic study of 49 randomly selected healthy adult Jamaicans was conducted to establish a guide for renal dimensions in the population. The mean length of the right kidney was 9.7 +/- 0.7 cm and the left 10 +/- 0.7 cm. The left kidney was longer than the right in the overall group and in males. There was no difference in width between right or left kidneys in the group as a whole or within either gender. There was a significant association between the weight of males and width of their kidneys; however, this association was not seen in females. The lone association between weight of participants and renal length occurred in females and only with respect to the left kidney. Lengths and widths of kidneys were not associated with height in either gender. Renal surface area (RSA) was similar between the genders and also between right and left kidneys. Similarly, there was no significant association between renal length and body surface area (BSA) overall or within the genders. Renal index (RI) which is more reliable at assessing renal parenchymal mass than renal length alone was 20.92 and 22.86 for the right and left kidneys, respectively, in males. Similarly, RI for the right and left kidneys in females was 23.76 and 25.54, respectively.

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