Síndrome de Goldbloom: síndrome febril prolongado con disproteinemia. Caso clínico / Goldboom's syndrome: prolonged febrile syndrome with dysproteinemia. Case report

El síndrome de Goldbloom es una rara entidad clínica de etiología desconocida que ocurre casi exclusivamente en pediatría. Consiste en un síndrome febril prolongado con hiperostosis perióstica y disproteinemia, que, con frecuencia, simula una patología hematooncológica o linfoproliferativa. El diagnóstico se hace por exclusión de las diferentes causas de dolor de los huesos y se asocia a hipergammaglobulinemia, hipoalbuminemia, eritrosedimentación acelerada e imágenes radiológicas de periostitis. La sintomatología, la radiología y los parámetros de laboratorio remiten en un tiempo variable, que va, habitualmente, de los 3 a los 12 meses. Se presenta a un paciente de 6 años con dolores óseos difusos, hiperostosis perióstica, síndrome febril prolongado de 8 meses de evolución, pérdida de peso y reactantes de fase aguda elevados con disproteinemia (hipergammaglobulinemia e hipoalbuminemia). Debe considerarse el síndrome de Goldbloom en un paciente con las manifestaciones descritas luego de la exclusión de la patología infecciosa, hematooncológica e inflamatoria de otra causa.

Goldbloom syndrome is a rare clinical entity, of unknown etiology that happens almost exclusively in pediatric population. It is a prolonged febrile syndrome with periosteal hyperostosis and dysproteinemia, and often simulates an hematooncology or lymphoproliferative disease. The diagnosis is to rule out the different causes of bone pain associated with hypergammaglobulinemia, hypoalbuminemia, high erythrocyte sedimentation rate and periostitis at the radiographies. Symptomatology, radiology and laboratory parameters refer in a variable time, usually from 3 to 12 months. We report the case of a six-year-old boy with diffuse bone pain, prolonged febrile syndrome (of 8 months of evolution), weight loss and elevated acute phase reactants with dysproteinemia (hypergammaglobulinemia and hypoalbuminemia). Goldbloom syndrome should be considered in patients with prolonged febrile syndrome and cortical hyperostosis after the exclusion of infectious, lymphoproliferative or inflammatory disease.

Factors related to outcomes in lupus-related protein-losing enteropathy

BACKGROUND/AIMS: Protein-losing enteropathy (PLE), characterized by severe hypoalbuminemia and peripheral edema, is a rare manifestation of systemic lupus erythematosus. This present study aimed to identify the distinctive features of lupus-related PLE and evaluate the factors related to the treatment response. METHODS: From March 1998 to March 2014, the clinical data of 14 patients with lupus PLE and seven patients with idiopathic PLE from a tertiary center were reviewed. PLE was defined as a demonstration of protein leakage from the gastrointestinal tract by either technetium 99m-labelled human albumin scanning or fecal alpha1-antitrypsin clearance. A positive steroid response was defined as a return of serum albumin to > or = 3.0 g/dL within 4 weeks after initial steroid monotherapy, and remission as maintenance of serum albumin > or = 3.0 g/dL for at least 3 months. A high serum total cholesterol level was defined as a level of > or = 240 mg/dL. RESULTS: The mean age of the lupus-related PLE patients was 37.0 years, and the mean follow-up duration was 55.8 months. Significantly higher erythrocyte sedimentation rate and serum total cholesterol levels were found for lupus PLE than for idiopathic PLE. Among the 14 patients with lupus PLE, eight experienced a positive steroid response, and the serum total cholesterol level was significantly higher in the positive steroid response group. A positive steroid response was associated with an initial high serum total cholesterol level and achievement of remission within 6 months. CONCLUSIONS: In lupus-related PLE, a high serum total cholesterol level could be a predictive factor for the initial steroid response, indicating a good response to steroid therapy alone.

Niveles de albumina y hemoglobina en lactantes desnutridos severos / Albumin and hemoglobin levels in severely malnourished infants

Introducción: Durante los procesos de desnutrición (DNT), se encuentran déficit de macro y micronutrientes. En Colombia la cifra de DNTsevera en niños de 1 a 2 años corresponde al 2% en el año 2000. Objetivo: Determinar los niveles de albúmina (ALB) y hemoglobina (HB), en niños menores de 24 meses con DNT severa, que ingresaron a un Hospital Universitario de Tercer Nivel de Atención del nororiente colombiano, en un periodo de 6 años. Pacientes y métodos: Estudio retrospectivo y comparativo de 134 niños con DNT severa, se diagnosticaron según signos cl ínicos y bioquímicos característicos de Kwashiorkor (KW) y Marasmo (MAR). La muestra fue dividida en tres grupos: DNT severos tipo KW, MAR y grupo control. Conclusiones: Los niveles de ALB y HB, se encuentran disminuidos en los procesos de DNT severa. El comportamiento de los tres grupos estudiados coincide con lo que reporta la literatura hasta el día de hoy.

Introduction: In the category of malnutrition (MNT), we find two main categories, macro and micronutrients deficiency. In Colombia severe MNT rates in children between the ages of 1 to 2 years, corresponded to 2 % according to 2000's statistics. Objective:To determine the levels of albumin and hemoglobin in children less than 2 years old with severe MNT that were treated at a Colombian Hospital University in a period of 6 years. Patients and Methods: Comparative and retrospective study of 134 children with severe,MNT, were diagnosis according to clinical and biochemical signals of Kwashiorkor (KW) and Marasmus (MAR). The children were divided in 3 groups: severe MNT type KW, MAR, and a control group.Conclusions:The albumin and hemoglobin levels are decreased in severe MNT processes. The results found in the 3 groups studied match with the reports showed by the literature today.

Efeito da ingestão protéica nos marcadores nutricionais e complicações clínicas em pacientes politraumatizados / Efecto de la ingesta de proteínas en los marcadores nutricionales y las complicaciones clínicas en los pacientes politraumatizados / Effect of protein intake on nutritional markers and clinical complications in polytrauma patients

Objetivo: Avaliar o efeito da ingestão protéica nos níveis de albumina sérica, na prevalência de complicações clínicas relacionadas à dieta, no estado nutricional e na mortalidade de pacientes politraumatizados. Método: Pacientes politraumatizados atendidos na Unidade de Terapia Intensiva entre janiero e setembro de 2006 foram divididos em 2 grupos, sendo o grupo 1 composto por aqueles que ingeriram diariamente quantidade de proteínas=1g/kg e o grupo 2 por aqueles que ingeriram diariamente quantidade de proteínas>1g/Kg, com suplementação protéica. Foram coletados semanalmente albumina sérica, leucócitos e bastonetes, dados clínicos das fichas médicas, e escore da avaliação subjetiva global, e compararam-se os dados iniciais e finais. Resultados: Pacientes do grupo 2 demonstraram tendência em apresentar níveis mais elevados de albumina sérica do que os indivíduos do grupo 1. As complicações clínicas relacionadas à dieta apresentadas pelos pacientes dos grupos 1 e 2, respectivamente, foram diarréia (41,37% vs. 30%; p>0,05), hiperglicemia (68,96% vs. 55%;p>0,05) e estase gástrica (58,6% vs.25%; p=0,04), sendo somente a última com diferença estatística significativa entre os grupos. No grupo 1,8% dos pacientes apresentaram desnutrição grave, e a prevalência de infecção foi maior que no grupo 2 (18,7% vs. 11,1%, respectivamente; p=0,02). Conclusões: Este estudo sugere que a ingestão protéica acima de 1g/kg/dia, associada à quantidades adequadas de energia, diminui a suscetibilidade às infecções, e pode estar relacionada com melhora do estado nutricional e dos níveis séricos de albumina.

Objetivo: Evaluar el efecto de la ingesta de proteínas de suero de los niveles de albúmina, la prevalencia de complicaciones clínicas relacionadas con la dieta, el estado nutricional y la mortalidad de los pacientes politraumatizados. Método: Los pacientes politraumatizados atendidos en la Unidad de Cuidados Intensivos entre septiembre de 2006 y Janiero se dividieron en dos grupos, un grupo que está compuesto por aquellos que consumían la cantidad diaria de proteínas y el grupo 2 = 1g/kg por los que tomaron la cantidad diaria de proteínas > 1g/kg con suplementos de proteína. Semanalmente, se recolectaron suero leucocitos albúmina, y las barras, los datos clínicos de los registros médicos, y la puntuación subjetiva de evaluación global, y compararon los datos iniciales y finales. Resultados: Los pacientes en el grupo 2 tienden a presentar niveles más altos de albúmina de suero de los pacientes en el grupo 1. La dieta de complicaciones relacionadas con la clínica que presentan los pacientes en los grupos 1 y 2, respectivamente, fueron: diarrea (41,37% frente al 30%, p> 0,05), hiperglucemia (68,96% frente al 55%, p> 0 , 05) y la estasis gástrica (58,6% vs.25%, p = 0,04), y sólo este último con una diferencia estadísticamente significativa entre los grupos. En el grupo de 1,8% de los pacientes padecían de desnutrición severa, y la prevalencia de infección fue superior en el grupo 2 (18,7% vs 11,1%, respectivamente, p = 0,02). Conclusiones: Este estudio sugiere que la ingesta de proteínas por encima de 1g/kg/day, junto con cantidades adecuadas de energía, disminuye la susceptibilidad a las infecciones, y pueden estar relacionados con la mejora del estado nutricional y la albúmina sérica.

Objective: To evaluate the effect of protein intake on serum albumin levels, the prevalence of clinical complications related to diet, nutritional status and mortality of polytrauma patients. Method: polytrauma patients treated at the Intensive Care Unit between September 2006 and janiero were divided into two groups, one group being composed of those who ate the daily amount of protein and group 2 = 1g/kg by those who took daily amount of protein > 1g/kg with protein supplementation. Were collected weekly serum albumin, leukocytes and rods, clinical data from medical records, and subjective global assessment score, and compared the initial and final data. Results: Patients in group 2 tended to present higher levels of serum albumin than patients in group 1. The diet-related clinical complications presented by patients in groups 1 and 2, respectively, were diarrhea (41.37% vs. 30%, p> 0.05), hyperglycemia (68.96% vs. 55%, p> 0 , 05) and gastric stasis (58.6% vs.25%, p = 0.04), and only the latter with a statistically significant difference between groups. In group 1.8% of patients had severe malnutrition, and prevalence of infection was higher than in group 2 (18.7% vs. 11.1%, respectively, p = 0.02). Conclusions: This study suggests that protein intake above 1g/kg/day, coupled with adequate amounts of energy, decreases the susceptibility to infections, and may be related with improvement of nutritional status and serum albumin.