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]
Case presentation We hereby report a case of 11-year-old male who presented with hepatic glycogenosis mimicking Mauriac’s syndrome. [ijponline.biomedcentral.com]
Entire Body System
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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]
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Enuresis
Five years prior to admission, she was diagnosed as diabetes mellitus at hospital due to polyuria, enuresis and polydipsia. [e-cep.org]
Skin
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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]
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
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]
[…] 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]
The mainstay of treatment for hepatic glycogenosis is strict control of glucose levels, with an excellent prognosis with improved glycaemic control. [ejcrim.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]
[…] 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]
Etiology
39 Prader-Willi syndrome 40 Etiologic classification of DM (c’d) III. [slideplayer.com]
Viral or autoimmune etiology was ruled out. A noncontrast abdominal computed tomography (CT) scan revealed hepatomegaly with a shiny aspect (fig. 1). [revistagastroenterologiamexico.org]
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]
We aimed to emphasize that the etiology of elevated liver enzymes should clearly be explained in type 1 diabetes mellitus patients. [jcrpe.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]
Pathophysiology
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]
[…] 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]