Mauriac Syndrome in a 3-Year-Old Boy

Mahesh, Shefali; Karp, Robert J.; Castells, Salvador; Quintos, Jose Bernardo

doi:10.4158/ep.13.1.63

Cited by 29 publications

(30 citation statements)

References 12 publications

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“…6 Although being an adolescent greater risk, MS can occur in any age if good metabolic control is not achieved. 2 In poorly controlled DM1 patients, the hyperglycemic periods followed by occasional hyperinsulinization and the high cortisol levels as hypoglycemia contra-regulatory hormone lead to hepatic glycogen storage. In hyperglycemic situations, glucose goes freely into the hepatocyte and is also stored as glycogen.…”

Section: Discussionmentioning

confidence: 99%

“…1 Cushingoid features may also be present. 2 There may be different forms and etiologies involved in Mauriac syndrome. However, there are common features noted in these patients and with adequate insulin treatment there is reversal of growth failure and hepatomegaly if present.…”

Section: Introductionmentioning

confidence: 99%

“…4 Although its real incidence is not well-known, due to the reduced number of reported cases in the literature, equal incidence is reported in males and females, with most of the cases occurring in adolescents and young adults. 2,5 The authors present a retrospective analysis of patients presenting diagnostic criteria of MS from the type 1 diabetic population followed currently by pediatric endocrinology in Braga's Hospital.…”

Section: Introductionmentioning

confidence: 99%

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Mauriac syndrome still exists

Dias

Martins

Carvalho

et al. 2013

Endocrinología y Nutrición

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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...

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Mauriac syndrome still exists

Dias

Martins

Carvalho

et al. 2013

Endocrinología y Nutrición

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“…Mauriac syndrome is a complication in diabetes mellitus type 1, which includes short stature, glycogen laden enlarged liver, limited joint mobility, tight waxy skin, growth maturation delay, moon facies, protuberant abdomen, and proximal muscle wasting and also it is frequently associated with retinopathy and nephropathy [1,2]. We hereby report a case of mauriac syndrome as very few cases have been reported previously in India [3].…”

Section: Introductionmentioning

confidence: 92%

Mauriac Syndrome: A Rare Complication of Poorly Controlled Diabetes Mellitus

Tiwari

2013

Int J Clin Pediatr

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“…A syndrome of uncontrolled type 1 diabetes, growth restriction and hepatomegaly which used to be commonly referred to as the Mauriac syndrome during the pre long acting insulin era is being increasingly reported of late albeit sporadically [1][2][3][4]. Although they are growth restricted, they look cushingoid due to excess deposition of fat and glycogen giving them a plump look ( Figure 1) in contrast to children with type-1 diabetes who otherwise look thin and wasted.…”

Section: Introductionmentioning

confidence: 99%

Mauriac Syndrome in the Age of Newer Insulin Analogues

Ch¹,

rasekaran²,

Baineni³

et al. 2015

J Pediatr Care

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The authors describe a five year-old plump looking female child with poorly controlled type 1 diabetes mellitus, hepatomegaly and growth retardation. Contrary to the usual phenotype of thin emaciated appearance in type 1 diabetes mellitus, the presence of above features should make one suspect Mauriac syndrome. Since the basis of this syndrome is poor metabolic control, early recognition is important. The authors emphasize the importance of optimal glycaemic control in preventing complications associated with Mauriac syndrome.

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Mauriac Syndrome in a 3-Year-Old Boy

Cited by 29 publications

References 12 publications

Mauriac syndrome still exists

Mauriac syndrome still exists

Mauriac Syndrome: A Rare Complication of Poorly Controlled Diabetes Mellitus

Mauriac Syndrome in the Age of Newer Insulin Analogues

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