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Mauriac's Syndrome

Diabetes Dwarfism Obesity Syndrome


Presentation

  • A 22 year follow-up of a female with Mauriac syndrome is presented. Despite incomplete growth she had normal, though delayed, sexual development with two successful pregnancies. There is no clinical evidence of degenerative complications.[ncbi.nlm.nih.gov]
  • Five patients presented diagnosis of short stature, but growth's deceleration was found in all of them. All the patients presented dyslipidemia.[dmsjournal.biomedcentral.com]
  • Under- or malnutrition may not only affect present and future growth, but also a child's ability to develop skills.[books.google.de]
  • Presented in part at the 40th Annual Meeting of the American Diabetes Association, Washington. DC, June 1980, and at The Macula Society, Scottsdale, Arizona, February 1981. Reprints requests: Louis A.[journals.lww.com]
Short Stature
  • 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]
  • DIABETIC RETINOPATHY IN MAURIAC'S SYNDROME: PDF Only Abstract We report four children with marked short stature, hepatomegaly, and delayed adolescence.[journals.lww.com]
  • Five patients presented diagnosis of short stature, but growth's deceleration was found in all of them. All the patients presented dyslipidemia.[dmsjournal.biomedcentral.com]
  • 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.[scienceopen.com]
  • His anthropometry is suggestive of short stature, height/110.5 cm ( (Figure 2) . CVS, RESP and CNS examination was normal. On admission random blood sugar was not controlled, ketones were negative.[omicsonline.org]
Developmental Delay
  • Due to severe neglect and developmental delay, care was taken over by dad and step mum after social services involvement at 1 year of age. Secondary to difficult childhood he had severe attachment disorder needing mental health input for few years.[endocrine-abstracts.org]
  • […] motor and social function & generalized epilepsy so called DEND syndrome Developmental delay, Epilepsy and Neonatal Diabetes Patients have all the clinical features of insulin dependency do not have detectable C peptide.[slideplayer.com]
Pathologist
  • Liver Pathology will be essential reading for every pathologist who evaluates liver pathology specimens. In addition it will be a valuable resource for pathology residents and fellows.[books.google.de]
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]
  • 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]
  • 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]
  • 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]
Macula
  • DC, June 1980, and at The Macula Society, Scottsdale, Arizona, February 1981. Reprints requests: Louis A. Lobes, MD, Eye & Ear Hospital. 230 Lothrop Street, Pittsburgh, PA 15213. The Ophthalmic Communications Society, Inc.[journals.lww.com]
Stroke
  • Take advantage of a wealth of images that capture the clinical manifestations and findings associated with Kawasaki disease, lupus, lymphoma, stroke, and many other disorders seen in children.[books.google.de]
  • MELAS syndrome: mitochondrial myopathy Encephalopathy lactic acidosis stroke-like syndrome Mitochondrial diabetes is commonly associated with sensorineural deafness and short stature.[slideplayer.com]

Workup

Dyslipidemia
  • 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]
  • All the patients presented dyslipidemia. Just 3 patients were adherent to diabetes management and only one of them has never been hospitalized for diabetic ketoacidosis.[dmsjournal.biomedcentral.com]
  • The male patient presented puberty delay and dyslipidemia.[elsevier.es]
  • 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]
  • J Obes Metab Res 2014;1:247-9 Introduction Mauriac syndrome (MS) is a rare complication of type 1 diabetes mellitus (T1DM), characterized by hepatomegaly (hepatic glycogenosis), puberty and growth delay, dyslipidemia, transaminase elevation and reduction[jomrjournal.org]

Treatment

  • 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]
  • 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]
  • Make the best clinical decisions with an enhanced emphasis on evidence-based practice and expert opinions on treatment strategies. Zero in on the most relevant and useful references with the aid of a more focused, concise bibliography.[books.google.de]
  • It is a rare syndrome firstly described by Mauriac in 1930 and has become even less common after the emergence of advances on diabetes treatment, but still exists.[dmsjournal.biomedcentral.com]

Prognosis

  • […] 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]
  • 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]
  • […] with good glycemic control, [3] whereas NASH has been reported to progress to fibrosis in up to 50% and cirrhosis in up to 15% of adult patients. [4] It is important to recognize and distinguish GH from NASH as it impacts the management and long-term prognosis[ijcasereportsandimages.com]
  • The long-term prognosis for these children depends on the degree of damage done by the illness or impairment and the amount of time it takes to recognize and diagnose MBPS.[kidshealth.org]

Etiology

  • 39 Prader-Willi syndrome 40 Etiologic classification of DM (c’d) III.[slideplayer.com]
  • Its etiology has no genetic base, but rather, is reliant on a sequence of events, one following the other. Robin sequence is the preferred term rather than Pierre Robin syndrome, as most cases are not syndromic 4 .[radiopaedia.org]
  • Management is etiological. Vitamin deficiencies are ubiquitous in T1DM and if left untreated, can lead to neurological, haematological and skeletal dysfunction.[jpma.org.pk]
  • The etiology and a pathogeny M. of page develops at the wrong treatment of a diabetes mellitus insulin (see) or absence and non-compliance with the mode of clinical nutrition.[bigmed.info]
  • […] characterized by hepatomegaly (hepatic glycogenosis), puberty and growth delay, dyslipidemia, transaminase elevation and reduction of IGF1 (insulin-like growth factor 1). 1 Cushingoid features may also be present. 2 There may be different forms and etiologies[elsevier.es]

Pathophysiology

  • […] release 51 Metabolic events during the fed and fasted states (Adipose tissue) High-Ins (fed) & Low Ins (fasted)state Glucose uptake Absent Glucose uptake Lipid synthesis Lipolysis and fatty acid release Triglyceride uptake Absent triglyceride uptake 52 Pathophysiology[slideplayer.com]
  • 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]

Prevention

  • 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]
  • Special attention and a multidisciplinary team are necessary to monitor these patients in order to help treatment compliance and prevent complications in the future.[dmsjournal.biomedcentral.com]
  • 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]
  • 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]

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