Sickle cell disease associated with thalassemia; description of a rare mutation.

Sickle cell disease (SCD) is a common hemoglobinopathy, secondary to alterations in the ß globin chain, resulting in an abnormal hemoglobin variant named as hemoglobin S. These disorders show a wide phenotypical spectrum, and the prevalence of these disorders has significantly changed over the time because of multiple factors such as migration. We report a case of a 17-year-old black male, born in Gambia, diagnosed with sickle cell disease, who presented an associated mutation only described in a Japanese family (Oshima et al., 1996).

La mayor adhesión a la dieta mediterránea no se asocia a un mejor control metabólico en pacientes con diabetes tipo 1 / Greater adherence to mediterranean diet is not associated with better metabolic control in patients with type 1 diabetes

Objetivos: El objetivo del presente estudio es evaluar el nivel de adhesión al modelo de dieta mediterránea en un grupo de pacientes con diabetes tipo 1 y analizar si un mayor nivel de adherencia a la dieta mediterránea se asocia con un mejor control metabólico y de factores de riesgo vascular. Material y métodos: Estudio observacional descriptivo realizado en 132 pacientes con diabetes tipo 1 de 33,7 ± 11,7 años de edad media. La adhesión a la dieta mediterránea fue evaluada mediante cuestionario validado que proporciona una puntuación entre 0 y 14. Los parámetros clínicos, antropométricos y metabólicos analizados han sido recopilados mediante entrevista personal, estudio del historial clínico y extracción analítica orientada a la evaluación del control metabólico. Resultados: La adhesión media a la dieta mediterránea fue de 8,9 ± 1,9 puntos; el 59% de los pacientes presentó una puntuación igual o inferior a 9 puntos. No se encontraron diferencias significativas en la adhesión a la dieta mediterránea por ninguno de los parámetros clínicos, antropométricos ni analíticos analizados. Los pacientes con mayor nivel de adhesión a la dieta mediterránea no presentaban perfiles lipídicos más favorables, menores niveles de hemoglobina glucosilada ni una menor prevalencia de complicaciones microvasculares. Conclusiones: La mayoría de los pacientes con diabetes tipo 1 evaluados en nuestro estudio muestran un nivel medio-bajo de adhesión al modelo tradicional de dieta mediterránea. La mayor adhesión a la dieta mediterránea no se asoció a un mejor control metabólico ni de otros factores de riesgo vascular(AU)

Objectives: The objective of the present study is to evaluate the level of adherence to the Mediterranean diet model in a group of patients with type diabetes 1 and to determine whether a higher level of adherence to a Mediterranean diet is associated with a better metabolic control or vascular risk factors. Material and methods: A descriptive study was conducted on 132 patients with type diabetes 1 and a mean age 33.7 ± 11.7 years. Adherence to the Mediterranean diet was evaluated by means of a validated questionnaire that provided a score between 0 and 14. The clinical, anthropometric and metabolic parameters analysed were compiled by means of personal interviews; a study of the clinical history and analytical results measured for metabolic control. Results: The mean adherence to the Mediterranean diet scored 8.9 ± 1.9 points, with 59% of the patients having a score of 9 points or lower. Significant differences in adherence to the Mediterranean diet were not found for any of the clinical, anthropometric or analytical parameters analysed. Patients with a higher level of adherence to the Mediterranean diet did not have more favourable lipid profiles, or lower levels of glycated haemoglobin, or less microvascular complications. Conclusions: Most patients with type diabetes 1 evaluated in our study showed a level medium-low level of adherence to the traditional Mediterranean diet model. Higher adherence to the Mediterranean diet was not associated with better metabolic control or other vascular risk factors(AU)

¿Es posible alcanzar en la práctica clínica los objetivos de control metabólico establecidos para pacientes con diabetes mellitus tipo 1 / Can the metabolic control targets established for patients with type 1 diabetets be achieved in clinical in clinical practice?

Objetivo: Evaluar el grado de consecución de indicadores de calidad de atención en pacientes con diabetes mellitus tipo 1 (DM1) atendidos en la Unidad de Gestión Clínica (UGC) de Endocrinología y Nutrición del Hospital Puerta del Mar de Cádiz. Metodología: Explotación de la base de datos incluida en la aplicación informática HP-Doctor utilizada para todos los pacientes atendidos en la UGC (hospitalización, consultas y centros periféricos). Se incluye a todos los pacientes con diagnóstico principal o secundario de DM1. Los indicadores de calidad analizados han sido seleccionados de las principales guías de práctica clínica de atención a pacientes con diabetes. Resultados: Se analiza una amplia cohorte de pacientes con DM1 (489pacientes) en seguimiento por nuestra UGC desde 2005 a 2007. En el período estudiado, el valor medio de glucohemoglobina (HbA1c) mejoró del 7,78 al 7,36%; se incrementó el porcentaje de pacientes con HbA1cmedia menor del 7% desde el 24,6 hasta el 27,1%, y disminuyó del 42,6hasta el 38,7% el porcentaje de pacientes con HbA1c media superior al 8%.En el año 2007, tan sólo un 35,5% de los pacientes mantenían cifras medias de colesterol de las lipoproteínas de baja densidad menores de100 mg/dl. Conclusiones: A pesar de la mejora obtenida en los parámetros de control metabólico, la mayoría de los pacientes con DM1 en seguimiento por nuestra unidad mantienen un inadecuado control glucémico y lipídico (AU)

Objective: To evaluate the quality of healthcare in patients with type 1 diabetes attended in the Endocrinology and Clinical Nutrition Unit of the Hospital Puerta del Mar in Cadiz (Spain).Methodology: The database included in the computer application HP-Doctor used forall patients attended in our unit(admissions, consultations and peripheral centers) was analyzed. All patients with a principal or secondary diagnosis of type 1diabetes were included. The quality indicators analyzed were selected from the main clinical practice guidelines for these patients. Results: A large cohort of patients with type 1 diabetes (n = 489) followed-up from2005 to 2007 was analyzed. During the study period, the mean glycated hemoglobin level (HbA1c) decreased from7.78% to 7.36%, the percentage of patients with HbA1c lower than 7% increased from24.6% to 27.1% and the percentage patients with a mean HbA1c of more than8% decreased from 42.6% to 38.7%. In2007, only 35.5% of patients maintained low-density lipoprotein concentrations ofless than 100 mg/dl. Conclusions: Despite the improvement obtained in metabolic control parameters, most of the patients with type 1 diabetes studied showed inadequate glycemic and lipid control (AU)

Can the metabolic control targets established for patients with type 1 diabetes be achieved in clinical practice?

OBJECTIVE: To evaluate the quality of healthcare in patients with type 1 diabetes attended in the Endocrinology and Clinical Nutrition Unit of the Hospital Puerta del Mar in Cadiz (Spain). METHODOLOGY: : The database included in the computer application HP-Doctor used for all patients attended in our unit (admissions, consultations and peripheral centers) was analyzed. All patients with a principal or secondary diagnosis of type 1 diabetes were included. The quality indicators analyzed were selected from the main clinical practice guidelines for these patients. RESULTS: A large cohort of patients with type 1 diabetes (n=489) followed-up from 2005 to 2007 was analyzed. During the study period, the mean glycated hemoglobin level (HbA(1c)) decreased from 7.78% to 7.36%, the percentage of patients with HbA(1c) lower than 7% increased from 24.6% to 27.1% and the percentage patients with a mean HbA(1c) of more than 8% decreased from 42.6% to 38.7%. In 2007, only 35.5% of patients maintained low-density lipoprotein concentrations of less than 100 mg/dl. CONCLUSIONS: Despite the improvement obtained in metabolic control parameters, most of the patients with type 1 diabetes studied showed inadequate glycemic and lipid control.

Hospital discharge records under-report the prevalence of diabetes in inpatients.

The objective of our study was to estimate the hospital inpatient prevalence of diabetes mellitus in a Spanish tertiary care teaching hospital. We analyzed a cohort of 1036 patients consecutively admitted over a 7-day period to our hospital. We classified this total of hospitalized patients based on information obtained from individual analysis of medical history and values of plasma glucose after fasting, into groups with the following conditions: recognized diabetes, unrecognized diabetes, other hyperglycaemic situations, impaired fasting glucose (IFG) or non diabetes. One hundred and seventy-eight patients were estimated to have diabetes (total prevalence: 17.2%), including 158 patients with recognized diabetes and 20 patients with diabetes unrecognized before admission. Additionally, 25 patients were considered to have other hyperglycaemic situations and 20 patients were estimated to have IFG. The mean age of the diabetic patients was 65+/-13.7 years (50.5% men), and 94.4% had type 2 diabetes. Diabetes disproportionately affects the elderly inpatient, with a prevalence of 30.9% in people older than 64 years. Of the total number of patients with diabetes, only 144 (diabetes prevalence: 13.8%) were registered in hospital discharge records as having diabetes. We conclude that the extent of hospital diabetes prevalence considerably exceeds levels reported in the literature, suggesting that true diabetes prevalence in hospitals could be significantly under-reported, resulting in a serious underestimate of required expenditures.

Prevalence of latent autoimmune diabetes of adults (LADA) in Southern Spain.

OBJECTIVE: To study the prevalence of diabetes mellitus and islet autoantibodies in an adult population from Southern Spain. RESEARCH AND METHODS: A cross-sectional study in Southern Spain of 1226 people, age 18-65 years. Clinical data were obtained and a blood sample taken to measure autoantibodies (glutamic acid decarboxylase antibodies (GADAb), tyrosine phosphatase antibodies (IA2Ab), and insulin antibodies (IAA)). An oral glucose tolerance test (OGTT) was also given to 982 of the subjects. RESULTS: The overall prevalence of diabetes mellitus according to the WHO 1979 criteria was 10.9% and according to the ADA 1997 criteria it was 14.7% (8.8% were unaware of their diabetes). The prevalence of impaired fasting glucose (IFG) was 12.4% and of impaired glucose tolerance (IGT) 11.5%. The prevalence of GADAb+ in the general population was 0.9% and in the diabetic population 3.7%. There were no significant differences between groups in the prevalence of IA2Ab or IAA (both were 0.8% in the general population). Of the three autoantibodies studied, only GADAb were significantly different in the diabetic population (P=0.0006). CONCLUSIONS: The prevalence of Type 2 diabetes and LADA are high in the south of Spain.