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Query: UMLS:C0011854 (type 1 diabetes)
 20,749 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Over the last 50 years the prognosis for growth and pubertal development in children with type 1 diabetes mellitus (T1DM) has improved considerably. The early reports of Mauriac's syndrome were related not only to relative deficiency of insulin but also reduced caloric intake. Improved insulin delivery and liberalisation of caloric intake has resulted in improved growth, but subtle abnormalities persist. The frequently reported increased height at diagnosis may relate to prior hyperinsulinaemia and genetic background with respect to lDDM2 the insulin gene VNTR. Subsequent growth faltering is thought to be related to impairment of the GH/IGF-1 axis but children with T1DM are also more at risk of hypothyroidism and coeliac disease. At puberty, persisting abnormalities of the GH/IGF-1 axis and our inability to reverse these totally, even with intensified insulin therapy, contribute to the blunted pubertal growth in the girls but abnormal sex steroid concentrations may also be important. Intensification of insulin therapy may result in leptin resistance and excessive gains in fat mass, particularly in girls. Although it is likely that most children with T1DM will have normal final heights, this excessive weight gain in girls may lead to problems with compliance. Furthermore, hyperinsulinaemia in these subjects may also lead to ovarian hyperandrogenism, increased early risk of microvascular complications and long-term risk of cardiovascular disease.
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PMID:Growth and body composition in type 1 diabetes mellitus. 1237 17

A young woman with type I diabetes mellitus, was hospitalized for a voluminous hepatomegaly associated with hepatocellular glycogen overloading suggesting Mauriac's syndrome. Two factors are involved in the physiopathology of this syndrome, hyperglycemia, and hyperinsulinemia which activates glycogenesis and inhibits glycogenolysis. The prognosis is normally favourable if diabetes is controlled.
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PMID:[Voluminous hepatomegaly in a young diabetic patient]. 1473 52

Patients with type 1 diabetes and poor metabolic control can develop hepatomegaly due to intrahepatic glycogen deposition. If these patients also have elevated liver enzymes, dyslipidemia, cushingoid features and delayed growth or sexual maturation, Mauriac syndrome can be diagnosed. This disorder is common and reversible with optimization of insulin therapy. We report three adolescents with type 1 diabetes and a long-standing history of poor glycemic control, who developed hepatomegaly, elevated liver enzymes and dyslipidemia with preserved liver function. One of these patients also had delayed growth and another had hypogonadotropic hypogonadism. Liver ultrasound showed changes suggestive of glycogenosis. In all three patients, optimization of insulin therapy achieved good glycemic control and reversed the manifestations within 2 weeks. The etiology of Mauriac syndrome is controversial since both prolonged hyperglycemia and hyperinsulinization produce glycogen accumulation in the liver. Hypercortisolism (due to ketosis or hypoglycemia) contributes to glycogen storage and also causes growth and sexual maturation delay.
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PMID:[Hepatomegaly due to glycogen storage disease and type 1 diabetes mellitus]. 1769 62

Growth failure in Type 1 Diabetes Mellitus (T1DM) can occur for several reasons. Mauriac syndrome is a rare cause of severe growth failure in T1DM. There may be different forms and etiologies involved in Mauriac syndrome. However, there are common features noted in these patients. We have compiled a review of cases reported in English in the last 30 years. With adequate insulin treatment there is reversal of growth failure and hepatomegaly if present. However, overly aggressive insulin delivery could result in rapid deterioration of diabetic retinopathy and nephropathy. Close monitoring of growth and pubertal maturation in children with T1DM is essential.
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PMID:Mauriac syndrome: growth failure and type 1 diabetes mellitus. 1880 15

A 17-year-old male with type 1 diabetes mellitus (T1DM) presented to clinic with elevated transaminases and a positive antinuclear antibody (ANA) screen. Due to concern for autoimmune hepatitis, a liver biopsy was performed which revealed Mauriac syndrome. This case report is the second known description of a child with Mauriac syndrome presenting with positive autoimmune markers.
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PMID:Mauriac syndrome in a child with a positive antinuclear antibody screen. 1999 17

Glycogenic hepatopathy (GH) is a rare cause of serum transaminase elevations in type 1 diabetes mellitus (DM). We describe a 29-year-old woman with a history of poorly controlled type 1 DM who presented with hepatomegaly and severe transaminase flares. Liver histology confirmed GH, with glycogen accumulation due to severe fluctuations in both glucose and insulin. GH can be regarded as an adult variant of Mauriac's syndrome. Despite severe laboratory abnormalities, it does not cause liver cirrhosis. Treatment consists of improving glycaemic control.
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PMID:Glycogenic hepatopathy: a rare cause of elevated serum transaminases in diabetes mellitus. 2000 16

Mauriac syndrome is a rare condition that affects people with uncontrolled diabetes. The case history of a 17-year-old female patient diagnosed with Mauriac syndrome and concurrently suffering from type 1 diabetes mellitus (T1DM), stunted growth, hepatomegaly, and severe periodontal disease is described. Non-surgical periodontal therapy was the treatment provided. Severe periodontal destruction was seen in conjunction with the unique features of this syndrome, such as hepatomegaly, dwarfism, moon-shaped face, cushingoid fat deposition, delayed puberty, and brittle diabetes. Treatment resulted in decreased plaque and gingival scores. A bi-directional relationship has been established between diabetes mellitus and periodontal disease and there is an increasing emphasis on perio-systemic interrelationships. Given the effect that such systemic disorders have on periodontal health, periodontists should be involved in the early diagnosis and treatment of such patients.
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PMID:Periodontal findings in a patient with Mauriac syndrome: a case report. 2241 89

The treatment of children with type 1 diabetes mellitus has improved dramatically over the last few decades. The maintenance of acceptable metabolic control, nevertheless, remains challenging because the success of treatment is so dependent on patient compliance. Children with type 1 diabetes and poor metabolic control are at risk of developing Mauriac syndrome, a condition characterised by hepatomegaly, growth retardation and cushingoid features. A similar complication may occur in type-1 or type-2 adult diabetics; namely, glycogenic hepatopathy. We describe two children, a 12-year-old girl and a 16-year-old boy, who presented with classic symptoms of Mauriac syndrome. After metabolic control was achieved, reduction of hepatomegaly and the disappearance of cushingoid features were observed, proving the reversibility of the syndrome. Awareness that this syndrome still exists despite improved insulin therapy is crucial for earlier recognition and treatment.
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PMID:[Mauriac syndrome--a rare complication of type 1 diabetes mellitus]. 2280 90

Mauriac syndrome is a rare syndrome associated with type 1 diabetes (T1DM) in children presenting with growth retardation, hepatomegaly, and cushingoid features. Recently, there has been re-emergence of this syndrome, especially with the use of premix insulin. A 15-year old type 1 diabetic boy, who was on premix insulin with erratic blood glucose, was referred to us for evaluation of short stature. He had significant short stature, hepatomegaly, and cushingoid features. His growth hormone (GH) stimulation was normal, and so was the overnight dexamethasone suppression test, based on which the diagnosis of Mauriac syndrome was reported. He was made to switch over to basal bolus regime, and was advised to follow-up for 6 months. He had reduction in hepatomegaly and a height gain of 3 cms.
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PMID:Re-emergence of a rare syndrome: A case of mauriac syndrome. 2425 Nov 87

Mauriac syndrome is associated with poor metabolic control of type 1 diabetes mellitus, hepatomegaly and elevated transaminases characterized by growth failure and delayed puberty, which may be reversible with good glycemic control. There are few references published in recent years following the introduction of glycemic self-monitoring, new insulins and intensified treatment. We describe a series of five patients evaluated in our service with Mauriac syndrome characteristics, conducting a literature review of cases in Argentina.
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PMID:[Mauriac syndrome in Argentina in the XXI century: series of 5 cases]. 2458 99


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