Presentation
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. [kclpure.kcl.ac.uk]
The male patient presented puberty delay and dyslipidemia. [elsevier.es]
Conversely, HG presents a favorable evolution, with no evidence of significant fibrosis [ 1, 10, 11 ]. [karger.com]
It classically presents with growth retardation, hepatomegaly and cushingoid features. [ntvg.nl]
Entire Body System
- 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]
- Buffalo Hump
They present with dwarfism, hepatomegaly, and a cushingoid appearance, including truncal obesity and “buffalo hump.” Hepatomegaly is caused by fatty deposition in the liver. Hepatic function is usually normal. [accessanesthesiology.mhmedical.com]
Gastrointestinal
- Hematemesis
The patient had no history of jaundice or scleral icterus, and he denied right upper quadrant pain, pruritis, weight loss, ascites, hematemesis, or rectal bleeding. [hindawi.com]
- Rectal Bleeding
The patient had no history of jaundice or scleral icterus, and he denied right upper quadrant pain, pruritis, weight loss, ascites, hematemesis, or rectal bleeding. [hindawi.com]
Liver, Gall & Pancreas
- Scleral Icterus
The patient had no history of jaundice or scleral icterus, and he denied right upper quadrant pain, pruritis, weight loss, ascites, hematemesis, or rectal bleeding. [hindawi.com]
Jaw & Teeth
- Tooth Discoloration
discoloration, gray-blue sclerae, high-arched palate, and skin abnormalities. [accessanesthesiology.mhmedical.com]
Eyes
- Blue Sclera
sclerae, high-arched palate, and skin abnormalities. [accessanesthesiology.mhmedical.com]
Musculoskeletal
- Normal Stature
[…] described in MS not being reported here might have two explanations: the first is that all the patients were 13–17 years old by the time of diagnosis, meaning that they had already achieved a normal stature before the detioration of metabolic control [elsevier.es]
Urogenital
- Secondary Amenorrhea
At presentation, the 5 girls presented cushingoid features, puberty dysfunction (primary amenorrhea in 2 patients and secondary amenorrhea in 3) and mixed dyslipidemia (high total cholesterol, low HDL[high density lipoprotein] cholesterol and high triglycerides [elsevier.es]
- Primary Amenorrhea
At presentation, the 5 girls presented cushingoid features, puberty dysfunction (primary amenorrhea in 2 patients and secondary amenorrhea in 3) and mixed dyslipidemia (high total cholesterol, low HDL[high density lipoprotein] cholesterol and high triglycerides [elsevier.es]
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]
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]
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]
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]
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]
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]
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]
Etiology
Viral or autoimmune etiology was ruled out. A noncontrast abdominal computed tomography (CT) scan revealed hepatomegaly with a shiny aspect (fig. 1). [revistagastroenterologiamexico.org]
39 Prader-Willi syndrome 40 Etiologic classification of DM (c’d) III. [slideplayer.com]
We aimed to emphasize that the etiology of elevated liver enzymes should clearly be explained in type 1 diabetes mellitus patients. [jcrpe.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]
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]
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]
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]
[…] 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]
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]
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]
Conclusions: Increased awareness of GH in clinicians will prevent delay in diagnosis and management. Our case highlights the importance of growth monitoring, focused examination in diabetes follow up. [endocrine-abstracts.org]
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]
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]