The male patient presented puberty delay and dyslipidemia. [elsevier.es]

Steatosis was present in the majority. Inflammation was present in 8 (42%). Fibrosis was seen in 14 (73%) and was generally mild though 2 had bridging fibrosis. Megamitochondria were described in 7. [adc.bmj.com]

Conversely, HG presents a favorable evolution, with no evidence of significant fibrosis [1, 10, 11]. [karger.com]

Mauriac syndrome is a rare syndrome associated with type 1 diabetes (T1DM) in children presenting with growth retardation, hepatomegaly, and cushingoid features. [scienceopen.com]

• Cushingoid

  Mauriac syndrome is a rare syndrome associated with type 1 diabetes (T1DM) in children presenting with growth retardation, hepatomegaly, and cushingoid features. [scienceopen.com]

  Pierre Mauriac syndrome was described in 1930 and is characterized by retarded growth, a Cushingoid appearance, hepatomegaly, and hypertransaminasemia, mainly in chronically uncontrolled DM1 patients. [revistagastroenterologiamexico.org]

  Introduction: Mauriac Syndrome is characterized by the presence of hepatomegaly, growth retardation, delayed puberty and cushingoid features. [endocrine-abstracts.org]

  Physical examination revealed hepatomegaly and a Cushingoid face. The growth rate of the patient had decreased, and she had not yet experienced menarche. Laboratory findings revealed elevated liver enzyme levels. [koreamed.org]

• Cushingoid Facies

  Physical examination revealed very short stature, overweight, Cushingoid facies, and hepatomegaly. Liver function tests only showed increased transaminases (ALT 158 IU, AST 192 IU), and AF 265 IU. Viral or autoimmune etiology was ruled out. [revistagastroenterologiamexico.org]

  It is associated with poor control of type-1 diabetes mellitus (T1DM) in adolescents, and may present as obesity, hepatomegaly, cushingoid facies and elevated transaminases. [snpedia.com]

  Signs and symptoms[edit] Patients with Mauriac syndrome may present with obesity, hepatomegaly, cushingoid facies, and elevated liver enzymes. [en.wikipedia.org]

  It is associated with poor control of type 1 diabetes mellitus (T1DM) in adolescents, and may present as obesity, hepatomegaly, cushingoid facies and elevated transaminases.[1] It is typically associated with growth failure and delayed pubertal maturation [ncbi.nlm.nih.gov]

• Pediatric Disease

  Succinct, targeted coverage of normal childhood growth and development, as well as the diagnosis, management, and prevention of common pediatric diseases and disorders, make this an ideal medical reference book for students, pediatric residents, nurse [books.google.de]

• Delayed Growth and Development

  Egas Moniz Hospital C.H.L.O., Endocrinology, Lisboa, Portugal Introduction: Mauriac syndrome is characterized by delayed growth and development, obesity, hepatomegaly and elevated transaminases in type 1 diabetes mellitus (T1DM) patients who have poor [endocrine-abstracts.org]

• Vomiting

  She complained of episodes of right upper quadrant abdominal pain associated with nausea and vomiting for the last 2 months with worsening in the last 48 hours. [ejcrim.com]

  Clinical symptoms of one-month duration were characterized by nausea, vomiting, and abdominal pain. Physical examination revealed very short stature, overweight, Cushingoid facies, and hepatomegaly. [revistagastroenterologiamexico.org]

  A 20-year-old male who was on intensive insulin regimen with a history of type 1 diabetes for 11 years was admitted to our hospital with complaints of diarrhea and vomiting for three days. [jcrpe.org]

  Since 1 year prior to admission, abdominal pain, vomiting, and diarrhea were developed twice, but subsided spontaneously without specific medication. [e-cep.org]

  “Glycogenic hepatopathy is a underrecognized complication of Mauriac syndrome that presents with abdominal pain, nausea, vomiting, elevated serum transaminases, elevated plasma lactate levels, and hepatomegaly The pathogenesis stems from an accumulation [gutsandgrowth.wordpress.com]

• Nausea

  She complained of episodes of right upper quadrant abdominal pain associated with nausea and vomiting for the last 2 months with worsening in the last 48 hours. [ejcrim.com]

  Clinical symptoms of one-month duration were characterized by nausea, vomiting, and abdominal pain. Physical examination revealed very short stature, overweight, Cushingoid facies, and hepatomegaly. [revistagastroenterologiamexico.org]

  “Glycogenic hepatopathy is a underrecognized complication of Mauriac syndrome that presents with abdominal pain, nausea, vomiting, elevated serum transaminases, elevated plasma lactate levels, and hepatomegaly The pathogenesis stems from an accumulation [gutsandgrowth.wordpress.com]

  Results: Symptoms included abdominal pain, anorexia, nausea and vomiting. All had tender hepatomegaly; two had splenomegaly. Liver biopsy was performed on two patients. [typeset.io]

• Chronic Diarrhea

  After 15 years of diabetes, the patient was referred for gastroenterology clinic due to chronic diarrhea and exuberant hepatomegaly. Laboratory showed persistent elevation of aminotransferases and triglycerides. [scielo.mec.pt]

  At this point, we established the diagnosis of diabetic glycogenosis in the clinical context of MS and the diagnosis of chronic diarrhea as a consequence of diabetic neuropathy. [karger.com]

• Hepatomegaly

  Growth rate improved significantly and hepatomegaly regressed. Puberty progressed rapidly in two older patients with poor final height. [scienceopen.com]

  Mauriac's syndrome should be considered in subjects with brittle type 1 diabetes and hepatomegaly. [ncbi.nlm.nih.gov]

  An abdominal CT scan done at 14 years of age demonstrated hepatomegaly with a 20 cm liver span. [ijcasereportsandimages.com]

  The clinical features consists of growth retardation, hepatomegaly, and cushingoid features. [journals.lww.com]

• Osteoporosis

  Updated clinical guidelines for diabetes, lipid disorders, obesity management, osteoporosis, and more, as well as essential treatment updates for the medical management of acromegaly, Cushing's Disease, hypercalcemia, and diabetes mellitus. [books.google.de]

  Development of osteoporosis (slowing the development of bone tissue). Diseases of the eye, including retinal diseases, and subsequently the development of cataracts. [acikgunluk.net]

  The clinical picture Develops obesity (see) with characteristic redistribution of fat (fatty deposits on a trunk at thin extremities), osteoporosis (see), lag in growth, a delay of sexual development. [bigmed.info]

  Osteoporosis Osteoporosis is a bone condition defined by low bone mass and decreased bone quality resulting in increased fragility and risk of bone fractures. [empoweryourhealth.org]

  View Article PubMed Google Scholar Szulc P, Munoz F, Claustrat B, Garnero P, Marchand F, Duboeuf F, Delmas PD: Bioavailable estradiol may be an important determinant of osteoporosis in men: the MINOS study. [rbej.biomedcentral.com]

• Osteopenia

  However significant osteopenia was appreciated on X-ray of long bones [Figure 1] c. [jomrjournal.org]

  Received Date: Oct 09, 2017 / Accepted Date: Oct 27, 2017 / Published Date: Oct 31, 2017 Mauriac syndrome is rarely noted complication of type-1 diabetic children characterised by dwarfism, osteopenia and glycogen laden enlarged liver [ 1 ]. [omicsonline.org]

• Suggestibility

  […] puberty, cushingoid features and hepatomegaly, usually associated with poor control of Type 1 Diabetes mellitus (T1DM) in children and adolescents.[1-4] The pathophysiology of MS is not entirely understood, but some mechanisms for glycogenosis have been suggested [ir.unilag.edu.ng]

  Her liver tests revealed AST - 3915 U/L and ALT - 1037 U/L, which markedly decreased three days later (AST - 166 U/L, ALT - 359 U/L), suggesting an acute insult to the liver. [ijcasereportsandimages.com]

  This suggests that high blood sugar is not the only factor necessary to cause the syndrome. [en.wikipedia.org]

  Liver imaging suggested diffuse fat infiltration. The clinical suspicion of hepatic glycogenosis was confirmed by liver biopsy. [ejcrim.com]

• Enlargement of the Liver

  History of poorly controlled DM, acute liver injury (marked elevation in aminotransferases and characteristic histologic changes on liver biopsy are diagnostic of glycogen hepatopathy (GH) The other main cause of liver enlargement and deranged liver tests [qingningbian.blogspot.com]

  After identifying the mutation, MacDonald and his colleagues sequenced the DNA of 250 patients with Type 1 diabetes who did not have enlarged livers. None had the mutation possessed by the patient with the enlarged liver. [archive.jsonline.com]

  Liver function tests, renal function tests, and urine routine examination were normal. Ultrasound abdomen showed liver enlargement 15.5 cms with increased echotexture, with normal spleen and portal vein and no free-fluid. [journals.lww.com]

  Enlargement of the liver, accompanied by roundabout venous circulation. Development of osteoporosis (slowing the development of bone tissue). Diseases of the eye, including retinal diseases, and subsequently the development of cataracts. [acikgunluk.net]

  The hallmark of the syndrome is extreme liver enlargement from massive deposition of glycogen. [diabetes.diabetesjournals.org]

• Hyperglycemia

  Continuous glucose monitoring showed hyperglycemia after meals and frequent hypoglycemia before meals. To control hyperglycemia, we increased insulin dosage by using an insulin pump. [koreamed.org]

  Ketosis activates cortisol synthesis promoting the release of fatty acids and hyperglycemia [ 7 ]. Growth failure, delayed puberty and hepatomegaly in Mauriac syndrome improve with glycemic control [ 8 ]. [omicsonline.org]

  The case demonstrates how the interaction between the gene mutation and hyperglycemia is the root cause of Mauriac Syndrome. Read the full study here. Post navigation [pediatrics.wisc.edu]

  This patient’s case proves that, although neither hyperglycemia alone nor a mutated enzyme alone are sufficient to cause Mauriac syndrome, a mutant enzyme of glycogen metabolism combined with chronic hyperglycemia can cause the syndrome. [diabetes.diabetesjournals.org]

• Dyslipidemia

  The male patient presented puberty delay and dyslipidemia. [elsevier.es]

  Mauriac's syndrome is a rare disease, which includes short stature, growth maturation delay, dyslipidemia, moon facies, protuberant abdomen, hepatomegaly with transaminase elevation. [ncbi.nlm.nih.gov]

  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. [comum.rcaap.pt]

• Hypercholesterolemia

  The laboratory data included high glycated hemoglobin (maximum of 14.8%), hypertriglyceridemia (1,066 mg/dL), hypercholesterolemia (224 mg/dL), and hypercortisolemia (25.5 μg/dL). [karger.com]

  […] nonalcoholic fatty liver disease, non-alcoholic steatohepatitis Abstract Mauriac syndrome is typically diagnosed in young patients with poorly controlled type 1 diabetes mellitus and is characterized by growth retardation, delayed puberty, cushingoid features, hypercholesterolemia [rjdnmd.org]

  Discussion: Our case demonstrated hepatomegaly, liver dysfunction, growth faltering and hypercholesterolemia secondary to poorly controlled diabetes. [endocrine-abstracts.org]

  Mauriac syndrome (MS) is a glycogenic hepatopathy, initially described in 1930, characterized by growth failure, delayed puberty, cushingoid appearance, hepatomegaly with abnormal liver enzymes, and hypercholesterolemia. [scielo.mec.pt]

• Liver Enzymes Abnormal

  As in children, liver enzyme abnormalities are unreliable in predicting the presence or the extent of glycogenosis. [typeset.io]

To control hyperglycaemia, initially we titrated daily insulin dosage, and then intravenous insulin treatment was practiced with the consequent normalization of liver enzymes. [ncbi.nlm.nih.gov]

In this treatment of both syndromes is aimed at compensated treatment of diabetes mellitus. Treatment of the Mauriac Syndrome Direct treatment of the syndrome as such does not exist. [acikgunluk.net]

He was irregular with the treatment, with 4 hospitalisations for DKA till date. [journals.lww.com]

The forecast and Prevention the Forecast at timely begun treatment can be favorable. The duration of a disease of a diabetes mellitus is more and the more its manifestations are expressed, the worse than M. the page will respond to treatment. [bigmed.info]

Mauriac's syndrome at the child Treatment of a syndrome of Mauriac: Main objective of treatment — permanent compensation of a diabetes mellitus. Treatment difficult includes selection of an adequate dose of insulin. [en.medicalmed.de]

The mainstay of treatment for hepatic glycogenosis is strict control of glucose levels, with an excellent prognosis with improved glycaemic control. [ejcrim.com]

[…] serum transaminases, elevated plasma lactate levels, and hepatomegaly The pathogenesis stems from an accumulation of glycogen in the liver…The diagnosis…is made …when all other causes of liver disease have been excluded…When glucose control is achieved, prognosis [gutsandgrowth.wordpress.com]

With the comprehensive treatment of the already developed syndrome with modern drugs, the prognosis is quite favorable, but provided all the recommendations of the doctor are fulfilled. [acikgunluk.net]

HG is a condition with good prognosis and fast resolution after adequate glycemic control (although it has potential for relapse), with no case of evolution to end-stage liver disease described. [scielo.mec.pt]

Decreased insulin-like growth factor-1 and growth hormone levels, along with poor bioactivity of these hormones, hormone receptors dysfunction, insulin deficiency and/or autoimmune factors. [7] The prognosis is good with improved glycemic control, and [jomrjournal.org]

Viral or autoimmune etiology was ruled out. A noncontrast abdominal computed tomography (CT) scan revealed hepatomegaly with a shiny aspect (fig. 1). [revistagastroenterologiamexico.org]

We aimed to emphasize that the etiology of elevated liver enzymes should clearly be explained in type 1 diabetes mellitus patients. [jcrpe.org]

39 Prader-Willi syndrome 40 Etiologic classification of DM (c’d) III. [slideplayer.com]

In fact diagnosis of Celiac Disease is regularly performed by means of the screening in type 1 Diabetes Mellitus patients.There may be different forms and etiologies involved in Mauriac syndrome. [ijirms.in]

Craig ME, Hattersley A, Donaghue KC; Definition, epidemiology and classification of diabetes in children and adolescents, ISPAD Clinical Practice Consensus guidelines 2009 compendium; Pediatric Diabetes 2009: 10 (suppl.12); 3-12. [ Links ] 2. [scielo.pt]

Pubertal delay.Introduction Mauriac syndrome (MS) is characterised by growth failure, delayed puberty, cushingoid features and hepatomegaly, usually associated with poor control of Type 1 Diabetes mellitus (T1DM) in children and adolescents.[1-4] The pathophysiology [ir.unilag.edu.ng]

Pathophysiology Mauriac Syndrome is primarily of historic interest. The hepatomegaly is thought to be due to the deposition of glycogen in the liver, and similar subcutaneous deposition gives rise to the round, moon-like facies. [accessanesthesiology.mhmedical.com]

Genetic, pathophysiological, and clinical aspects of nephrocalcinosis. Am J Physiol Renal Physiol 2016;311:F1243-F1252. Oeschgef VV, Prieto M. [mmsjournals.org]

Wide fluctuations in plasma glucose levels, with periods of hyperglycemia and hyperinsulinism, appear to be essential to the pathophysiology of HG [2, 5]. High glucose levels promote the flow of glucose into hepatocytes. [karger.com]

Isolated patients with this syndrome are still being reported.3,7–10 The pathophysiology of this entity has not been elucidated. [ncbi.nlm.nih.gov]

Providing practical, data-driven resources based upon the totality of the evidence, this important text helps the reader understand the basics of pediatric obesity and T2DM and implement strategies to prevent and treat obesity and diabetes in children [books.google.de]

To prevent lipodystrophy, a number of preventive procedures are carried out, for this purpose massage, physiotherapeutic procedures are prescribed and a scheme for injections with insulin is made. [acikgunluk.net]

In addition, we prescribed uncooked cornstarch to prevent hypoglycemia. After strict blood glucose control, the patient's liver functions and size normalized. The patient subsequently underwent menarche. [koreamed.org]

This prevents the tongue from descending which in turn prevents fusion of palatal shelves resulting in glossoptosis (retraction of tongue), high or U-shaped palate and airway obstruction. [radiopaedia.org]

Prevention consists in timely transition to treatment by insulin with the prolonged action that promotes achievement of the fullest and permanent compensation of diabetes. [bigmed.info]