Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0015695 (fatty liver)
 13,941 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Severe liver disease complicates pregnancy in only 0.1% of the cases. Viral hepatitis is the most common cause (40%). (Liver cirrhosis usually results in amenorrhea). Liver disease unique to pregnancy comprises "intrahepatic cholestasis of pregnancy" (Increased fetal risk), "acute fatty liver of pregnancy" (AFLP) and "HELLP-syndrome", both with high maternal and fetal risk when untreated. AFLP and HELLP-syndrome are diseases of the third trimester and show similar clinical signs of jaundice, coagulopathy and elevated liver enzymes. The immediate termination of pregnancy preferably by Caesarean section has been shown to improve both, maternal and fetal outcome. Imaging methods like ultrasound are invaluable in the differential diagnosis and detection of complications like subcapsular hematoma in the liver patients with HELLP-syndrome. Fulminant hepatic failure requires intensive care, liver transplantation is an additional therapeutic option. Recurrent AFLP has been reported recently.
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PMID:[Acute hepatopathies in pregnancy: diagnosis and therapy]. 152 90

Extreme forms of insulin resistance are a rare cause of type 2 diabetes. However, individuals with severe insulin resistance pose unique diagnostic and therapeutic challenges, and have often acted as 'experiments of nature' providing important novel information regarding endocrine physiology and mechanistic insights relevant to the study of more common disorders. Progress in understanding the molecular pathogenesis of such syndromes is also beginning to yield novel therapeutic options. Severe insulin resistance typically presents in 1 of 3 ways: (1) disordered glucose metabolism including both diabetes and/or paradoxical hypoglycaemia; (2) acanthosis nigricans, a velvety hyperpigmentation of axilliary and flexural skin often associated with skin tags; or (3) hyperandrogenism in girls (hirsutism, oligo-/amenorrhoea and polycystic ovaries). Lipodystrophy is a major cause of severe insulin resistance and needs to be looked for very carefully, particularly in the patients with significant dyslipidaemia and fatty liver. Specific treatments are now available for some forms of severe insulin resistance; for example, leptin replacement in patients with generalized lipodystrophy. In the absence of a specific diagnosis and therapy, metformin is a useful insulin sensitizer and should be used in conjunction with aggressive diet and exercise interventions.
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PMID:A clinical approach to severe insulin resistance. 1798 32

HELLP syndrome complicates PE in 5 to 20 % of cases. The clinical manifestations (i.e. epigastric pain, elevated liver enzymes, thrombocytopenia and hemolysis) are secondary to the fibrin deposit within the peri-portal sinusoids. The clinical presentation of HELLP syndrome can be misleading. It is therefore necessary to suspect this complication whenever a PE patient develops gastro-intestinal pain. The interruption of pregnancy is the only effective treatment against HELLP syndrome. If it can be safely performed passed the 34(th) week of amenorrhea, a protective attitude should be adopted prior to reaching this date. This consists of the administration of corticosteroid therapy for fetal pulmonary maturation, intensive clinical, biological and sonographic monitoring of the mother's parameters. The administration of corticosteroids or performing a plasmapharesis is not recommended for the treatment of established HELLP syndrome because neither improves the maternal or neonatal outcome. The differential diagnosis may also include acute fatty liver of pregnancy. An early liver impairment, polyuria-polydipsia syndrome and a rise in INR support this diagnosis.
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PMID:[Liver and preeclampsia]. 2034 56

Polycystic ovary syndrome is now a well-recognized condition affecting 6%-25% of reproductive-aged women, depending on the definition. Over the past 3 decades, research has launched it from relative medical obscurity to a condition increasingly recognized as common in internal medicine practices. It affects multiple systems, and requires a comprehensive perspective on health care for effective treatment. Metabolic derangements and associated complications include insulin resistance and diabetes, hyperlipidemia, hypertension, fatty liver, metabolic syndrome, and sleep apnea. Reproductive complications include oligo-/amenorrhea, sub-fertility, endometrial hyperplasia, and cancer. Associated psychosocial concerns include depression and disordered eating. Additionally, cosmetic issues include hirsutism, androgenic alopecia, and acne. This review organizes this multi-system approach around the mnemonic "MY PCOS" and discusses evaluation and treatment options for the reproductive, cosmetic, and metabolic complications of this condition.
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PMID:Polycystic ovary syndrome: update on diagnosis and treatment. 2485 38

Leptin has key roles in the regulation of energy balance, body weight, metabolism, and endocrine function. Leptin levels are undetectable or very low in patients with lipodystrophy, hypothalamic amenorrhea, and congenital leptin deficiency (CLD) due to mutations in the leptin gene. For these patients, leptin replacement therapy with metreleptin (a recombinant leptin analog) has improved or normalized most of their phenotypes, including normalization of endocrine axes, decrease in insulin resistance, and improvement of lipid profile and hepatic steatosis. Remarkable weight loss has been observed in patients with CLD. Due to its effects, leptin therapy has also been evaluated in conditions where leptin levels are normal or high, such as common obesity, diabetes (types 1 and 2), and Rabson-Mendenhall syndrome. A better understanding of the physiological roles of leptin may lead to the development of leptin-based therapies for other prevalent disorders such as obesity-associated nonalcoholic fatty liver disease, depression and dementia.
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PMID:Leptin treatment: facts and expectations. 2515 86

Recombinant methionyl human leptin or metreleptin is a synthetic leptin analog that has been trialed in patients with leptin-deficient conditions, such as leptin deficiency due to mutations in the leptin gene, hypothalamic amenorrhea, and lipodystrophy syndromes. These syndromes are characterized by partial or complete absence of adipose tissue and hormones derived from adipose tissue, most importantly leptin. Patients deficient in leptin exhibit a number of severe metabolic abnormalities such as hyperglycemia, hypertriglyceridemia, and hepatic steatosis, which can progress to diabetes mellitus, acute pancreatitis, and hepatic cirrhosis, respectively. For the management of these abnormalities, multiple therapies are usually required, and advanced stages may be progressively difficult to treat. Following many successful trials, the US Food and Drug Administration approved metreleptin for the treatment of non-HIV-related forms of generalized lipodystrophy. Leptin replacement therapy with metreleptin has, in many cases, reversed these metabolic complications, with improvements in glucose-insulin-lipid homeostasis, and regression of fatty liver disease. Besides being effective, a daily subcutaneous administration of metreleptin is generally safe, but the causal association between metreleptin and immune complications (such as lymphoma) is still unclear. Moreover, further investigation is needed to elucidate mechanisms by which metreleptin leads to the development of anti-leptin antibodies. Herein, we review clinical aspects of generalized lipodystrophy and the pharmacological profile of metreleptin. Further, we examine studies that assessed the safety and efficacy of metreleptin, and outline some clinical perspectives on the drug.
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PMID:New advances in the treatment of generalized lipodystrophy: role of metreleptin. 2639 24