Mauriac syndrome still exists

Abstract

Background/objective: Mauriac syndrome (MS) is a rare complication of type 1 diabetes mellitus (DM1). It is related to low insulin concentrations and is less common since longer-acting insulins became available. It is characterized by hepatomegaly, growth and puberty delay, and the presence of elevated transaminases and serum lipids. The aim of this study was to describe the patients from a pediatric diabetic population that fulfill the criteria of MS. Materials and methods:A retrospective analysis of the pediatric diabetic population with diagnostic criteria of MS currently followed at Hospital de Braga, was performed. Results: From a population of 91 patients with DM1 18 years, 6 patients with the criteria for MS were identified: 5 girls, and 1 boy. The age at presentation was 13---17 years, with a minimum interval between DM1 diagnosis and MS criteria of 4 years. All the patients were prescribed intensive insulin therapy (median daily insulin dose: 0.88 U/kg). All had a previous history of poor glycemic control before the diagnosis of MS with glycated hemoglobin (HbA1c) between 8.8 and 12.9%. Increase of hepatic enzymes was present in all the patients; 4 of them had associated hepatomegaly. All the girls presented puberty delay and cushingoid features. None of the patients presented short stature and 5 of them presented mixed dyslipidemia. Conclusions: Although MS is an ancient entity described in DM1, it still exists, particularly in adolescent females. Being aware of MS is of extreme importance since most of the clinical features are reversible with better glycemic control. © 2012 SEEN. Published by Elsevier España, S.L. All rights reserved. PALABRAS CLAVESíndrome de Mauriac; Diabetes mellitus; Adolescencia El síndrome de Mauriac todavía existeResumen Introducción/objetivo: El síndrome de Mauriac (SM) es una complicación rara de la diabetes mellitus de tipo 1 (DM1), relacionada con bajas concentraciones de insulina, y es menos común desde que están disponibles insulinas de larga duración de acción. Se caracteriza por la hepatomegalia, el retraso del crecimiento y de la pubertad y la elevación de las transaminasas y de los lípidos séricos. El objetivo de este estudio fue la descripción de los pacientes con criterios de SM en una población pediátrica con DM1. * Corresponding author. E-mail address: joanamprdias@gmail.com (J. Dias).2173-5093/$ -see front matter © 2012 SEEN. Published by Elsevier España, S.L. All rights reserved. J. Dias et al.Material y métodos: Análisis retrospectivo de una población pediátrica diabética atendida en el Hospital de Braga con criterios diagnósticos de SM. Resultados: De una población de 91 pacientes con DM1 menores de 18 años fueron identificados 6 pacientes con criterios de SM: 5 mujeres y un varón. La edad de presentación fue de 13 a 17 años con un intervalo mínimo entre el diagnóstico de DM1 y SM de 4 años. Todos los pacientes tenían prescrita una terapia insulínica intensiva (dosis media diaria: 0,88 U/kg). Todos tenían una historia previa de mal control glucémico a...

Mauriac Syndrome: A Rare Complication of Type 1 Diabetes

“…bir yaşta ortaya çıkabilmektedir. 3,4 Diyabet kontrolünün daha iyi sağlanmasıyla bu sendromun insidansı hızla azalmıştır. Günümüzde çok nadir görülen bir sendromdur.…”

“…Kortizolün yükselmesi hepatik glikojen depolamasına yol açmaktadır. 4,6 Hiperglisemik durumlarda, glukoz hepatosit içine serbestçe girmekte ve glikojen olarak depolanmaktadır. 3 Hepatomegali nedeninin karaciğerde glikojen birikimine bağlı olduğu düşünülmektedir.…”

“…Düşük IGF-1 düzeyleri boy kısalığını açıklayabilir. 3,4,7 Bununla birlikte dokularda yetersiz glukoz kullanımı, IGF-1 ve büyüme hormonuna dirençli reseptörlerin varlığı, hiperkortizoleminin de büyüme ve puberte gecikmesine katkıda bulunabileceği düşünülmektedir. 7 Olgumuzda da büyüme-gelişme geriliği ve puberte gecikmesi mevcuttu.…”

“…Multidisipliner yaklaşım tedavinin temelidir. 1,4,6 Bu hastalarda eklem kontraktürleri, osteopeni, hiperlipidemi görülebilmekte, ayrıca retinopati ve albuminüri gibi diyabetik mikrovasküler komplikasyonlar da eşlik edebilmektedir. 6,8,9 Olgumuzda da retinopati, hiperlipidemi mevcuttu.…”

Mauriac syndrome — is already a history?

“…Portugese authors presented an analysis of 91 patients with type 1 diabetes mellitus in which 6 patients were diagnosed with Mauriac syndrome [13].…”