Asociación entre el fenotipo hipertrigliceridemia-obesidad abdominal, la resistencia a la insulina y las variables cardiometabólicas en el síndrome de ovario poliquístico / Association among the hypertriglyceridemic waist phenotype, insulin resistance and the cardiometabolic variables in the polycystic ovary syndrome

Introducción: El síndrome de ovario poliquístico se asocia con frecuencia a alteraciones cardiometabólicas; y su asociación con el fenotipo hipertrigliceridemia-obesidad abdominal ha sido poco estudiada en Cuba. Objetivo: Identificar la frecuencia del fenotipo hipertrigliceridemia-obesidad abdominal en mujeres de edad mediana con síndrome de ovario poliquístico y su asociación con la resistencia a la insulina, trastornos del metabolismo de la glucosa y ateroesclerosis subclínica. Método: Estudio descriptivo, transversal, en 30 mujeres. Se tomaron variables clínicas: edad, peso, talla, índice de masa corporal, circunferencia de cintura y cadera, índice cintura/cadera, tensión arterial, además de concentraciones de glucosa, insulina, colesterol total, triglicéridos, HDL-c y LDL-c, e índice HOMA-IR. La aterosclerosis subclínica se evaluó por doppler carotideo y ecocardiograma (hipertrofia ventricular izquierda y grasa epicárdica). El fenotipo hipertrigliceridemia-obesidad abdominal se definió como triglicéridos elevados (≥ 1,7 mmol/L) y circunferencia de la cintura ≥ 80 cm. Resultados: La frecuencia del fenotipo hipertrigliceridemia-obesidad abdominal fue 43,3 por ciento (13/30). Los valores medios de circunferencia abdominal, tensión arterial, así como de glucemia (p < 0,003), insulinemia (p = 0,028), triglicéridos (p < 0,0001), e índice HOMA-IR (p = 0,012) fueron más elevados en el grupo de mujeres con esa condición. A pesar de no haber diferencias significativas la frecuencia de mujeres con incremento del grosor íntima-media carotídeo y de grasa epicárdica fue superior en aquellas con el fenotipo. Conclusiones: La presencia del fenotipo hipertrigliceridemia-obesidad abdominal es frecuente en mujeres con síndrome de ovario poliquístico, y se asocia con alteraciones del metabolismo de la glucosa y la resistencia a la insulina. Este pudiera ser utilizado en la práctica clínica como un marcador de riesgo para alteraciones cardiometabólicas(AU)

Introduction: The polycystic ovary syndrome is frequently associated to cardiometabolic alterations; and its relation with the hypertriglyceridemic waist phenotype has been poorly studied in Cuba. Objective: Identify the frequency of the hypertriglyceridemic waist phenotype in middle age women with polycystic ovary syndrome and its association with insulin resistance, disorders in the glucose metabolism and subclinical atherosclerosis. Methods: Descriptive, cross-sectional study in 30 women. As clinical variables there were used: age, weight, size, body mass index, waist-hip circumference, waist/hip index, blood pressure; glucose, insulin, total cholesterol, triglycerides, HDL-c and LDL-c concentrations, and HOMA-IR index. Subclinical atherosclerosis was assessed by a carotid doppler and an echocardiogram (left ventricular hypertrophy and epicardial fat). The hypertriglyceridemic waist phenotype was defined as high triglycerides levels (≥ 1.7 mmol/L) and CC ≥ 80 cm. Results: The frequency of the hypertriglyceridemic waist phenotype was 43.3 percent (13/30). The mean values of abdominal circumference, blood pressure, as well as glycemia (p < 0.003), insulinaemia (p = 0.028), triglycerides (p < 0.0001), and HOMA-IR index (p = 0.012) were higher in the group of women with that condition. Although there were not significant differences, the frequency of women with increase of the carotid intima-media thickness and epicardical fat was higher in those with the phenotype. Conclusions: The presence of the hypertriglyceridemic waist phenotype is frequent in women with the polycystic ovary syndrome, and it is associated with alterations of the glucose metabolism and insulin resistance. This can be used in the clinical practice as a marker of risk for cardiometabolic alterations(AU)

Caracterización de hipertrigliceridemia y antropometría en pacientes adultos en la comunidad rural / Characterization of hypertriglyceridemia and anthropometry in adults patients in a Rural community

El informe sobre las enfermedades no transmisibles de la Organización Mundial de la Salud (OMS) del 2010 reporta que 80% de las defunciones se deben a enfermeda-des no transmisibles, y dentro de estas están las enfermedades metabólicas que afectan más a poblaciones de ingresos medios y bajos, diferente a lo que se creía anteriormente, lo cual podría reducirse, si la población evidencia que sus hábitos influyen en su salud. Objetivo: Caracterizar los fac-tores de riesgo asociados a niveles de hiper-trigliceridemia en pacientes de 28-40 años que asisten al centro de salud del municipio de Ceguaca, Santa Bárbara, Honduras de septiembre del 2016 a febrero del 2017. Pa-cientes y métodos: Estudio cuantitativo, descriptivo, no experimental, trasversal. Muestra no probabilística por conveniencia de 50 pacientes que cumplían con los crite-rios de inclusión, uno de los criterios fue firmar el consentimiento informado. La tabu-lación de datos se realizó en SPSS 23, para los gráficos se utilizó el Software Microsoft Excel 2016. Se realizaron medidas de frecuencia y porcentaje. Resultado: La mayoría eran mujeres con 84% (42), y varo-nes 16% (8), edades: 37-40 años 40% (20), 28-32 años 34% (17) y de 33-36 años 26% (13). Nivel de triglicéridos: hombres> 150 mg/dl 100% (8), mujeres:> 150 mg/dl 64.3% (27), indice de masa corporal (IMC) entre 30-39.9: 50% (25), perímetro abdomi-nal: en hombres> 102 cm 50% (4), mujeres> 88 cm 86% (36), actividad física: 68% tenían un estilo de vida sedentario y también un 68% presentan antecedentes familiares de dislipidemias. Conclusión: La mayoría de adultos jóvenes presentó hipertrigliceride-mia; como factores determinantes la calidad de la dieta, la falta de actividad física y el factor hereditario...(AU)

La hipertrigliceridemia como marcador temprano de resistencia a la insulina en obesidad infanto-juvenil / Hypertriglyceridemia as early marker of insulin resistance in children-juvenile obesity

Introducción: la obesidad en infantes y adolescentes constituye un problema de salud pública en aumento. La resistencia a la insulina es una reducción de respuesta fisiológica de los tejidos diana a la acción de la insulina. Objetivo: determinar el valor de la hipertrigliceridemia e hiperglucemia como marcadores tempranos de resistencia a la insulina en niños y adolescentes obesos. Métodos: el estudio fue de enfoque cuantitativo, diseño no experimental, corte transversal, de tipo observacional y correlacional. La población la constituyeron niños y adolescentes con sobrepeso u obesidad, atendidos en la consulta externa del Hospital Dr. Francisco Icaza Bustamante. Resultados: se demostró la utilidad de parámetros bioquímicos como predictores precoces de insulinorresistencia. El 37,4 por ciento presentó hipertrigliceridemia, 19,8 por ciento hiperglucemia y el 51,6 por ciento insulinorresistencia. El promedio más elevado de triglicéridos fue encontrado en el grupo etario de 6 a 10 años, y en el sexo masculino. El análisis estadístico demostró asociación significativa entre hipertrigliceridemia e insulinorresistencia (p< 0,05). Conclusiones: la hipertrigliceridemia tiene valor predictivo de resistencia a la insulina en obesidad infanto-juvenil(AU)

Introduction: obesity in infants and adolescents is a growing public health problem. Insulin resistance is a reduction in the physiological response of target tissues to the action of insulin. Objective: to determine the value of hypertriglyceridemia and hyperglycemia as early markers of insulin resistance in obese children and adolescents. Methods: the study was of a quantitative approach, non-experimental design, cross sectional, observational and correlational type. The population was constituted by children and adolescents who were overweight or obese, and that were attended in the outpatient clinic of Dr. Francisco Icaza Bustamante Hospital. Results: the usefulness of biochemical parameters as early predictors of insulin resistance was demonstrated. 37.4 percent presented hypertriglyceridemia, 19.8 percent hyperglycemia and 51.6 percent insulin resistance. The highest average of triglycerides was found in the age group of 6 to 10 years, and in the male sex. Statistical analysis showed a significant association among hypertriglyceridemia and insulin resistance (p< 0.05). Conclusions: hypertriglyceridemia has a predictive value of insulin resistance in children-juvenile obesity(AU)

Sobrepeso y dislipidemias en adolescentes / Overweight and dyslipidemias in teenagers

INTRODUCCIÓN: actualmente hay consenso en que el proceso aterosclerótico se inicia en la infancia, y la dislipidemia es uno de los principales factores de riesgo aterogénicos que deben ser estudiados, así como la valoración nutricional para una adecuada prevención. OBJETIVO: identificar algunas señales de aterosclerosis tempranas como el sobrepeso y las dislipidemias en adolescentes. MÉTODOS: se realizó un estudio descriptivo transversal de 372 adolescentes de la Secundaria Básica "Protesta de Baraguá". Se hicieron mediciones de peso, talla, índice de masa corporal y circunferencia de la cintura. Se tomó muestra de sangre venosa con ayuno de 12 horas. Se midió el colesterol total, el colesterol unido a lipoproteína baja y alta densidad, y triglicéridos. En el análisis estadístico se realizaron prueba de comprobación de media entre variables de valoración nutricional y lipídicas entre sexos (prueba t), y se trabajó con una probabilidad de error menor de 0,05. RESULTADOS: la media del peso, talla y circunferencia de la cintura fueron mayores en el sexo masculino (p< 0,05). El exceso de peso fue 23,7 %, y a partir del percentil 90-97 hubo un 21,8 % de exceso de grasa abdominal. La media del colesterol total, lipoproteínas de alta y baja densidad y triglicéridos, fue similar en los diferentes sexos, sin relación significativa (p> 0,05). El 18,5 % presentó colesterol total limítrofe alto, el 26,6 % tenía triglicéridos limítrofe alto y 7,5 % alto, con predominio del sexo femenino. CONCLUSIONES: alrededor de la cuarta parte de los adolescentes tenían exceso de peso e incremento de la grasa abdominal, casi la cuarta parte tenía el colesterol total limítrofe y alto, y la alteración lipídica observada con mayor frecuencia fue la hipertrigliceridemia.

INTRODUCTION: there is a current consensus that the atherosclerotic process begins at childhood and that dyslipidemia is one of the atherogenic risk factors to be studied together with the nutritional assessment for the adequate prevention of atherosclerosis. OBJECTIVE: to identify some early signs of atherosclerosis such as overweight and dyslipidemias in teenagers. METHODS: a cross-sectional descriptive study of 372 teenagers from "Protesta de Baragua" junior high school. Weight, height, body mass index and waist circumference were measured. Blood samples were taken after 12 hour fasting. Total cholesterol, low and high density lipoprotein cholesterol and triglyceride were all measured. The statistical analysis included the t test among nutritional assessment and lipid variables between sexes and the error probability was lower than 0.05. RESULTS: weight, height and waist circumference means were higher in males (p< 0.05). Overweight was 23.7 % and from the 90-97th percentile, the excessive abdominal fat was 21.8 %. The mean of total cholesterol, high and low density lipoproteins and triglycerides was similar in both sexes, with no significant relation (p> 0.05). In this group, 18.5 % of adolescents presented with borderline high total cholesterol, 26.6 % showed borderline high triglyceride rate and 7.5 % had high cholesterol, being females predominant. CONCLUSIONS: one fourth of adolescents approximately exhibited overweight and increased abdominal fat; almost 25 % had reached borderline and high total cholesterol whereas the most observed lipid disorder was hypertrigliceridemia.

Fenótipo cintura hipertrigliceridêmica: associação com alterações metabólicas em adolescentes / Hypertriglyceridemic waist phenotype: association with metabolic abnormalities in adolescents

OBJETIVO: O presente estudo objetivou identificar a prevalência do fenótipo cintura hipertrigliceridêmica (CHT) e avaliar sua associação com alterações metabólicas em adolescentes de baixa condição econômica. MÉTODO: Estudo transversal com amostra probabilística de 1.076 adolescentes entre 11 e 17 anos, de ambos os sexos, estudantes de escolas públicas. Os participantes foram submetidos à avaliação antropométrica (peso, altura e circunferência da cintura) e à dosagem dos níveis de colesterol total, LDL-C, HDL-C, colesterol não HDL, triglicérides (TG) e glicemia de jejum. Foram obtidas informações referentes às condições econômicas das famílias dos participantes.O fenótipo CHT foi definido pela presença simultânea da circunferência da cintura aumentada (> percentil 90 por idade e sexo) e dos níveis séricos de triglicérides elevados (> 100 mg/dL). A análise de regressão logística foi utilizada para avaliação das associações de interesse. RESULTADOS: A prevalência do fenótipo CHT foi de 7,2% entre os adolescentes, sendo mais elevada na presença de obesidade (63,4%), do colesterol não HDL (16,6%) e do LDL-C (13,7%) altos. A análise bivariada indicou que, das variáveis metabólicas, apenas a glicemia não se associou ao fenótipo CHT. A análise multivariada, ajustada por sexo e idade, indicou que o fenótipo CHT se associou positivamente com o colesterol não HDL alto (odds ratio, 7,0; IC 95% 3,9-12,6) e com o HDL-C baixo (odds ratio, 2,7; IC 95%, 1,5-4,8). CONCLUSÕES: Este estudo mostrou que o fenótipo CHT se associou com um perfil lipídico aterogênico e sugere esse fenótipo como uma ferramenta de screening que pode ser utilizada para identificar adolescentes com alterações metabólicas.

OBJECTIVE: This study aimed to identify the prevalence of hypertriglyceridemic waist (HTW) phenotype, and to evaluate its association with metabolic abnormalities in adolescents of low socioeconomic status. METHOD: This was a cross-sectional study with a random sample of 1,076 adolescents between 11 and 17 years, of both genders, from public schools. The participants underwent anthropometric measurements (weight, height, and waist circumference), and levels of total cholesterol, low-density-lipoprotein cholesterol (LDL-C), high-density-lipoprotein cholesterol (HDL-C), non-HDL cholesterol triglyceride (TG), and fasting glucose were measured. Information regarding the socioeconomic status of the participants' families was obtained. The HTW phenotype was defined by the simultaneous presence of increased waist circumference (> 90th percentile for age and gender) and serum triglyceride levels (> 100 mg/dL). A logistic regression analysis was used to evaluate the associations of interest. RESULTS: The prevalence of HTW phenotype was 7.2% among the adolescents, being higher in the presence of obesity (63.4%) and high levels of non-HDL cholesterol (16.6%) and LDL-C (13.7%). The bivariate analysis indicated that, of the metabolic variables, only blood glucose was not associated with the HTW phenotype. Multivariate analysis adjusted for age and gender indicated that the HTW phenotype was positively associated with high non-HDL cholesterol (odds ratio: 7.0; 95% CI: 3.9-12.6) and low HDL-C levels (odds ratio: 2.7; 95% CI: 1.5-4.8). CONCLUSIONS: This study demonstrated that the HTW phenotype was associated with an atherogenic lipid profile, and this phenotype is suggested as a screening tool to identify adolescents with metabolic alterations.

Association of Serum 25-Hydroxyvitamin D Levels with Markers for Metabolic Syndrome in the Elderly: A Repeated Measure Analysis

The purpose of current study was to investigate associations of serum 25-hydroxyvitamin D (OHVD) levels with markers for metabolic syndrome in elderly Koreans. We conducted a panel study on 301 individuals over 60 yr old in Seoul, Korea, and repeatedly measured serum OHVD, glucose, insulin, and lipid levels. Mixed effect model and generalized estimating equations were used to investigate relationships between serum OHVD levels with marker levels for metabolic syndrome and each of its categories. Of all subjects, 76.6% were vitamin D deficient (< 50 nM) and 16.9% were insufficient (< 75 nM). Inverse association was demonstrated between serum OHVD levels and insulin (P = 0.004), triglyceride (P = 0.023) and blood pressure (systolic blood pressure: P = 0.002; diastolic blood pressure: P < 0.001). Vitamin D deficiency was found to increase risk of 'hypertriglyceridemia' category of metabolic syndrome (odds ratio: 1.73, 95% confidence interval: 1.13-2.66). In conclusion, we found from our repeated measure analysis that decreasing serum OHVD levels are associated with increasing insulin resistance, increasing serum triglyceride levels and increasing blood pressure in elderly Koreans, and confirmed on the risk of 'hypertriglyceridemia' in vitamin D deficient subjects.

Hipertrigliceridemias de origen genético / Hypertriglyceridemias of genetic origen

Hypertriglyceridemia (HTG) is defined as plasma triglycerides (TG) > 150 mg/dL, and it is a frequent disease in the general population. When plasma TG reach concentrations > 500 mg/dL (severe HTG), there is usually a genetic defect involved. This defect can involve a single gene or be of polygenic inheritance. In polygenic HTG, the phenotypic expression of the disease is usually associated to the presence of certain diseases such as diabetes, obesity or insulin resistance. The most common known genes associated with monogenic hypertriglyceridemia are LPL and APOC2, but in recent years a few cases caused by mutant APOA5, GPIHBP1 and LMF1, have been identified. Furthermore, genome wide association studies (GWA) have brought up new genes that are related to discrete changes in triglyceride plasma levels of the general population. Among them, it is worth mentioning GCKR, TRIB1, MLXIPL, GALNT2, APOB, APOC2, APOA5, APOE, LPL, ANGPTL3 and NCAN. It is remarkable that most severe hypertriglyceridemias are of polygenic origin, and they could involve a major susceptibility gene. Only in a few cases of severe or very severe HTG (TG > 2.000 mg/dL) the genetic cause is known.

Síndrome de Berardinelli-Seip: descrição genética e metabólica de cinco pacientes / Genetic and metabolic description of five patients with Berardinelli-Seip syndrome

OBJETIVO: Descrever o perfil genético e metabólico de portadores da síndrome de Berardinelli-Seip (BSCL) acompanhados no Instituto da Criança do HC-FMUSP. SUJEITOS E MÉTODOS: Pacientes com as características clínicas da BSCL (n = 5), todas do sexo feminino, foram avaliadas com dosagens de glicose e insulina, lípides, leptina, enzimas hepáticas, análise de DNA, ultrassonografia abdominal. RESULTADOS: A deficiência de leptina e a hipertrigliceridemia foram constatadas nas cinco pacientes. Três evoluíram para diabetes melito (DM). Quatro tiveram mutação no gene AGPAT2 e uma no gene CAV1. CONCLUSÃO: As alterações metabólicas mais precoces foram a hipertrigliceridemia e a resistência insulínica, culminando no surgimento do DM à época da puberdade, sendo as mutações no gene AGPAT2 as mais frequentes em nossa casuística.

OBJECTIVE: To report the genetic and metabolic profile of patients with Berardinelli-Seip syndrome (BSCL) followed at Instituto da Criança, HC-FMUSP. SUBJECTS AND METHODS: Patients with clinical features of BSCL (n = 5), all female, were evaluated through serum levels of glucose, insulin, lipids, leptin, and liver enzymes. Abdominal sonography and DNA analysis were also performed. RESULTS: Leptin deficiency and hypertriglyceridemia were found in all the patients. Three progressed to diabetes mellitus. Four patients have mutations in AGPAT2 gene and one have a mutation in CAV1 gene. CONCLUSION: The earliest metabolic abnormalities were hypertriglyceridemia and insulin resistance, culminating in the onset of diabetes at the time of puberty. Mutations in the AGPAT2 gene were the most frequent in our patients.

Fármacos hipolipemiantes / Hypolipidemic drugs

A importância do tratamento das dislipidemias na prevenção da doença aterosclerótica é sobejamente reconhecida. Neste artigo são abordados aspectos farmacológicos e terapêuticos de medicamentos, em uso habitual, com ação predominante na hipercolesterolemia e na hipertrigliceridemia. Ao final, são apresentados os medicamentos em desenvolvimento, incluindo aqueles capazes de elevar a fração HDL-colesterol. Para a escolha do hipolipemiante, devem ser considerados: o diagnóstico preciso da dislipidemia (primária e secundária), possível interação com outros fármacos, idade, fases pré e pós menopausal, co-morbidades, presença de alteração hepática ou renal, etilismo e disponibilidade em farmácias institucionais. Quanto à associação de hipolipemiantes com diferentes mecanismos de ação, além de adequar as doses, exige-se maior atenção aos efeitos adversos(clínicos e/ou laboratoriais).

Prevalencia de hipertensión arterial en adultos de El Progreso / Prevalence of hypertension in adults in El Progreso, Yoro, Honduras

ANTECEDENTES. La hipertensión arterial(HTA) representa un creciente problema de salud a nivel mundial. En los países en desarrollo el mejoramiento de las condiciones socioeconómicas, el cambio en los estilos de vida, la creciente migración hacia las zonas urbanas y el envejecimiento de la población, han condicionado un incremento, en la prevalencia de las enfermedades no transmisibles. En la ciudad de El Progreso, Honduras se desconoce cual es la prevalencia de la hipertensión y otros factores de riesgo cardiovascular. METODO. Se realizó un estudio descriptivo transversal para determinar la prevalencia de hipertensión arterial y otros factores de riesgo cardiovascular en la cuidad de El Progreso, Honduras. Se obtuvo una muestra de 246 personas, mayores de 18 años, de ambos sexos y con participación voluntaria, excluyéndose a las mujeres embarazadas. Se utilizó la definición de JNC 7 para categorizar las cifras de presión arterial obtenidas. RESULTADOS. Se detectaron valores normales de presión arterial en 24.8%, prehipertensión arterial en 42.3%, hipertensión arterial estadio 1 en 19.1% y estadio 2 en 13.8% de los individuos. Sin embargo, al tomar en cuenta las personas con antecedente de hipertensión pero que en el momento de la medición de la presión arterial obtuvieron cifras normales, se encontró frecuencia de hipertensión arterial de 45.52%. Del total de personas hipertensas, el 31.2% desconocía su enfermedad. Se observó que los diabéticos tiene el doble riesgo de tener HTA que las personas no diabéticas. CONCLUSIONES. La hipertensión arterial se presenta con una frecuencia en la población adulta de 18 años en el El Progreso. La prevalencia de prehipertensión en los adultos jóvenes entre los 18 y 57 años, presentan una elevada frecuencia de hipertensión arterial. Entre los factores de riesgo para hipertensión estudiados, la diabetes mellitus fue el más significativo, seguido por obesidad y obesidad central...

Comportamento alimentar e síndrome metabólica: aspectos psicológicos / Eating behavior and metabolic syndrome: psychological aspects

Hábitos alimentares têm grande importância no desenvolvimento e na morbidade de doenças associadas que constituem a síndrome metabólica e que sãoinfluenciadas por fatores psicológicos. A ingestão alimentar, um comportamento complexo que envolve aspectos biopsicossociais, é uma das bases do desenvolvimento das representações psíquicas, elementos constituintes do mundosimbólico, do pensamento, que permitirá a percepção da realidade e o alívio das tensões psíquicas. A compulsão alimentar, quadro derivado de falhas nodesenvolvimento do mundo representativo, é comumente associada à obesidade e às dislipidemias. Além da compulsão alimentar, os estudos epidemiológicos apontam que a depressão se encontra freqüentemente associada à síndrome metabólica, em especial à obesidade, ao diabetes e à hipertensão. Os dinamismos psíquicos envolvidos na depressão e na compulsão alimentar são importantes fatores que explicam as limitações dos resultados no tratamento da síndrome metabólica.

Hypertriglyceridemia in pediatrics

A 12-year-old boy was referred for evaluation of pallor since infancy. When 2 years, he was diagnosed as thalassemia major because of pallor, hemolytic red blood cell morphology and markedly raised fetal hemoglobin [58% as assessed with Betke's method of hemoglobin estimation]. He was transfused twice per year till he was 4 years of age and then only recently at 12 years. The boy also had recurrent diarrhea with malabsorptive stools and episodic itching for the last 6 years. He frequently had nocturnal enuresis and was slow to learn. He never had periorbital swelling, dysuria or burning micturation. He was born prematurely [28 weeks] to consanguine parents and there was mild delay in the development. On examination the boy was pale with anthropometric measurement [height, weight, and occipito-frontal circumference] below 3rd centile but frontal bossing, maxillary prominence or depressed bridge of the nose were not very significant. The liver and spleen were only 1 cm palpable below costal margins. Being in doubt, about the diagnosis of the thalassemia major, the hemoglobin electrophoresis was asked from Aga Khan Laboratories, Karachi and it was normal [Hemoglobin A1 ' 98.1% and Hemoglobin A2 ' 1.9%]. Incidentally we noticed that blood turned diffusely milky on standing. Suspecting hyperlipidemia, we re-examined the boy. The xanthomas, corneal arcus and tonsillar hypertrophy were absent. The parents were non-diabetic, normotensive and non-smoker. Laboratory investigations showed reticulocytosis with decreased hemoglobin. The urine report, renal parameters and liver function tests showed nothing abnormal. The total lipids were 3015 mg/dl [N=500-1000mg/dl] and triglycerides were 2227 mg/dl [N=46-236mg/dl]. The cholesterol and low-density lipoproteins [LDL] were 132 mg/dl [N=160-200mg/dl] and 80 mg/dl [N=100-130mg/dl], respectively. The high-density lipoproteins [HDL] were 12 mg/dl [N=35]. The low HDL to this extent has only been reported with LCAT deficiency and Tangier's disease. The absence of large yellowish tonsils in this child ruled out the Tangier's disease. Keeping in view the clinical picture, red blood cell morphology of hemolytic anemia with markedly increased serum triglycerides and markedly decreased high-density lipoproteins, we made the diagnosis of lecithin cholesterol acyltransferase deficiency in this boy. Since the enzyme level are not possible in our circumstances so it is more of a diagnosis of exclusion. The child was advised low fat diet and unsaturated oils

Association between isolated hypercholesterolemia, isolated hypertriglyceridemia and coronary artery disease in south Indian type 2 diabetic patients.

Very high prevalence rates of coronary artery disease have been reported among Indians. The aim of this study was to determine the relative importance of isolated hypercholesterolemia, isolated hypertriglyceridemia, isolated high low-density lipoprotein and isolated low high-density lipoprotein in coronary artery disease among South Indian type 2 diabetic subjects. The study group comprised of 17,885 type 2 diabetic patients attending our institute. A history of documented myocardial infarction was considered as the diagnostic criteria for coronary artery disease. Isolated hypercholesterolemia was defined as serum cholesterol over 200 mg/dL with normal serum triglyceride levels (< or = 200 mg/dL); isolated hypertriglyceridemia was defined as serum triglyceride level over 200 mg/dL with normal serum cholesterol levels (< or = 200 mg/dL). Isolated low high-density lipoprotein was defined as one below 35 mg/dL with normal serum triglyceride levels. Isolated high low-density lipoprotein cholesterol was defined as one over 150 mg/dL with normal serum triglyceride levels. Normolipidemia was defined as serum cholesterol and serum triglyceride both upto 200 mg/dL, high-density lipoprotein 35 mg/dL or above and low-density lipoprotein upto 150 mg/dL. The prevalence of coronary artery disease was significantly high among patients with isolated hypercholesterolemia (4.1%; p < 0.001), isolated high low-density lipoprotein (4.5%; p < 0.001) and isolated low high-density lipoprotein (3.9%; p = 0.005) compared to normolipidemic individuals (2.8%), but not in those with isolated hypertriglyceridemia (3.4%). The odds ratios for coronary artery disease increased with each quartiles of isolated cholesterol, isolated low-density lipoprotein cholesterol and total cholesterol to high-density lipoprotein ratio and reached statistical significance in the last quartile (p < 0.05). There was no significant increase in the odds ratios for coronary artery disease in relation to quartiles of isolated triglycerides. For isolated low high-density lipoprotein, when the last quartile was taken as the reference, the odds ratio for coronary artery disease in the first quartile reached statistical significance (p = 0.03). Multivariate regression analysis revealed age (odds ratio 1.06; p < 0.001), male sex (odds ratio 1.7; p < 0.001), hypercholesterolemia (odds ratio 1.26; p = 0.07) and high low-density lipoprotein levels (odds ratio 1.22; p = 0.043) to be strongly associated with coronary artery disease. Among South Indian type 2 diabetic subjects, serum isolated hypercholesterolemia and high low-density lipoprotein cholesterol but not isolated hypertriglyceridemia appear to be associated with coronary artery disease.

Permanent neonatal diabetes mellitus: epidemiology, mode of presentation, pathogenesis and growth.

Permanent neonatal diabetes mellitus (PNIDDM) is a rare form of IDDM with unclear etiology and pathogenesis. We determined the incidence and prevalence rates and studied the clinical and biochemical features of PNIDDM in the Sultanate of Oman. The mean incidence rate during the study period from January 1989 to December 1994 was 1.788 +/- 0.82 per 100,000 live births per year. At the end of December 1994 the prevalence rate was 2.4 per 100,000 children below the age of 5 years. They constituted 41.6% of all cases of IDDM in this age group. Diarrhoea, fever, lethargy, poor feeding and failure to thrive were the most common presenting symptoms. Dehydration and tachypnoea were the most common signs. All patients who developed IDDM during the neonatal period had intrauterine growth retardation and 4.5 presented with diabetic ketoacidosis (plasma glucose 37 +/- 9 mmol/L, pH 7.12 +/- 0.1). Hypertriglyceridemia was a constant feature (19.4 +/- 4.8 mmol/L). They were products of consanguineous marriage with significantly high prevalence of IDDM and NIDDM in their family members. None of the infants had clinical or immunological evidence of congenital viral infection. Three of the five children had HLA-DR2, the diabetes resistance alleles. C-peptide secretion was absent during and after metabolic control of hyperglycemia in all the studied infants and none had circulating islet cell antibody at presentation or during the first year after diagnosis. Despite marked growth retardation at birth, there was a significant improvement of growth after initiating insulin therapy. Four of the 5 patients had normal developmental milestones, one had mild developmental delay following a severe and prolonged attack of hypoglycemia. None of the patients had exocrine pancreatic deficiency. In summary, the very high rate of parental consanguinity, occurrence in both sexes and in two siblings in the same family, absence of islet cell antibodies and the presence of HLA-DR2 loci in 3/5 of patients suggest that PNIDDM is a different disease process to standard IDDM in childhood and an autosomal recessive mode of transmission.

Prevalencia de factores de riesgo de enfermedad coronaria en trabadores del Hospital General de México / Prevalence of risk factors of coronary disease in personnel of the Generla Hospital of Mexico

Objetivo. Conocer la prevalencia de algunos factores de riesgo coronario en trabajadores de una institución de salud. Material y métodos. De 1993 a 1995 se evaluaron 2228 trabajadores del Hospital General de México, 1531 mujeres (68.7 por ciento) y 697 hombres (31.2 por ciento) con edades entre 16 a 65 años, y se les clasificó por áreas de trabajo: en Intendencia hubo 477 participaciones (21.4 por ciento); en Administración, 697 (31.2 por ciento); en Personal Médico, 495 (22.2 por ciento), y en Enfermería, 559 (25.0 por ciento). Se les practicó historia clínica, mediciones antropométricas y determinación de glucosa, colesterol total, LDL, HDL y triglicéridos. Resultados. Trescientos sesenta y siete sujetos (14.9 por ciento) tenían colesterol por arriba de 240 mg/dl, con valores altos en las mujeres del área administrativa (17.1 por ciento) y en los hombres del departamento de enfermería (26 por ciento) que presentó la mayor tendencia (26 por ciento). Se encontraron niveles de triglicéridos por arriba de los 200 mg/dl en 471 personas (19.1 por ciento); obesidad, en 329 (13.5 por ciento); hipertensión arterial, en 549 sujetos (22.2 por ciento), y tabaquismo positivo, en 32 por ciento de los trabajadores. La prevalencia de diabetes mellitus fue de 6.24 por ciento. Conclusiones. Se confirma la elevada prevalencia de factores de riesgo de enfermedad cardiovascular entre los trabajadores del Hospital General de México, factores que en muchos casos son modificables, lo que confiere la posibilidad de realizar acciones preventivas

Objective. To evaluate the prevalence of risk factors of coronary heart disease in the personnel of the General Hospital in Mexico City. Material and methods. We studied 2 228 workers, 1 531 female (68.7%) and 697 male (31 .2%) whose ages ranged from 16 to 65 years old in the period of 1993 to 1995. They were divided in work areas: Intendancy 477 (21.4%), Administrative, 697 (31.2%), Physicians, 495 (22.2%) and Nurses, 559 (25.0%). We collected clinical histories, anthropom etric measures, and laboratory determinations of glucose, total cholesterol, LDL, HDL and triglicerydes. Results. We found that 367 (14.9%) had total cholesterol above 240 mg/dl, with high values in females of the administrative area (17.1%) and males in the nursing department (26%), which was the highest tendency. Trigliceryde levels above 200 mg/dl were found in 208 males (24.6%) and 263 females (16.2%), with high prevalence in the nursing and administrative departments, in males (39.1 and 34.1% respectively). Obesity was present in 236 females (14.5%) and 97 males (11.5%). High blood pressure in 549 individuals (22.2%), 297 females (18.3%) and 252 males (29.8%) without significance regarding to work area. Smoking habits were positive in 32% of the total with highest prevalence in males from 30 to 45 years and in females from 30 to 50 years. We found an incidence of 6.24% of diabetes in all the subjects studied, 2.27% ignored the diagnosis at the moment they were studied. Conclusions. In t his study we confirmed the high prevalence of risk factors of coronary heart disease in personnel of the General Hospital in Mexico City. In most cases, these risk factors that can be modified and, therefore, prevented.

Una nueva prueba funcional para el diagnóstico etiológico de las hipertrigliceridemias primarias, mediante la administración de ácidos grasos n-3 / An news test functional for the diagnostic etiologic the primary hipertriaglyceridemias, intervening the administration of fatty acids n-3

Las hipertrigliceridemias humanas se producen por aumento de síntesis de triglicéridos (TG) y/o por déficit de degradación. La sobreproducción hepática de lipoproteínas de muy baja densidad (VLDL) es difícilmente demostrable. Para tipificarla utilizamos ácidos n-3 (AG n-3), que inhiben la producción hepática de TG-VLDL. Se ha diseñado una prueba funcional que consiste en administrar 3,6 g/día de AG n-3 durante 5 días, midiendo lipoproteínas basales y post prueba. Se estudiaron 22 pacientes con hipertrigliceridemias primarias, con peso normal, cuya adherencia a la prueba se comprobó por el aumento de AG n-3 plasmáticos de 2,8+0,5 a 7,2+0,7% (X+ES) (p<0,001). Se agruparon los pacientes según su fenotipo de dislipoproteinemia clasificada por OMS. Diez pacientes, tipo IV, mostraron las siguientes variaciones (pre a post, mg/dl) (X+ES) TG: 454+50 a 237+22 (p<0,001): colesterol de LDL (C-LDL): 112+8 a 144+7 (p<0,01). Colesterol de VLDL (C-VLDL): 62+18 a 30+8 (p<0,02). Ocho pacientes tipo IIb mostraron TG:344+72 a 233+55 (p<0.001). C-LDL:197+9 a 196+19 (NS) y C-VLDL: 43+11 a 33+8 (NS). Un paciente, tipo V con lipoproteínas lipasa inactiva y lipasa hepática de actividad normal no varió los TG, otro del mismo tipo disminuyó los TG de 1.644 a 700. Dos pacientes de tipo III con colesterol en lipoproteínas intermedias de 50 a 67 mg/dl respectivamente, no modificaron la trigliceridemia. Se distinguen los pacientes de tipo IV, que respondieron a la prueba disminuyendo la síntesis de TG de los tipos V y III que no respondieron. Los pacientes IIb no disminuyeron el C-LDL, lo que sugiere que este aumento no se debe a sobreproducción de la precursora VLDL.

Efecto de las lipoproteínas ricas en triglicéridos sobre la relación entre C-LDL y Apo B determinada por EID en suero total: I. indicadores de riesgo para la enfermedad coronaria. Valores de Apo B/C-HDL / Effect the triglyceride rich lipoproteins upon the relation between C-LDL and Apo B determined by EID in total serum: I.rick indications of coronary disease. Values of Apo B/C-HDL

En este trabajo se determinaron los valores promedio, desviación estándar y percentilos para el cociente Apo B/C-HDL y, por otra parte, se analizó el efecto de las lipoproteínas ricas en triglicéridos sobre la relación entre C-LDL y Apo B, determinada por electroinmunodifusión en suero total. Se estudiaron 74 individuos de 20 y más años, aparentemente sanos. Para Apo B/C-HDL se obtuvo un valor medio de 2,16+0,78. El percentilo 50 fue 2,00, el percentilo 75 fue 2,60 y el percentilo 95 fue 3,60. Los individuos con Apo B/C-HDL>3,60 estarían en riesgo respecto de la aterosclerosis coronaria. La relación entre C-LDL y Apo B, es importante para la detección de sujetos con hiperapo B, la cual está fuertemente relacionada con la aterosclerosis coronaria. Utilizando los triglicéridos como estimadores de masa de las lipoproteínas ricas en triglicéridos, se halló que mientras TG<160 mg/dl la correlación entre Apo B y C-LDL fue r= +0,65,P <0,001. Un 86% de los pacientes con C-LDL entre 80 y 165 mg/dl tenían Apo B entre 55 y 120 mg/dl, mientras que un 12% tenían Apo B por encima de 120 mg/dl. En estos pacientes se puede suponer la presencia de hiperapo B en estas condiciones experimentales. Cuando TG>160 mg/dl, la correlación entre Apo B y C-LDL disminuye a r=+0,39,P<0,1. Un 21% de los pacientes con C-LDL entre 80 y 165 mg/dl tenían Apo B entre 55 y 120 mg/dl, pero un 79% tenían Apo B por encima de 120 mg/dl. En estas condiciones experimentales no se podrían discriminar los pacientes con hiperapo B.