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 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The course of preeclamptic/eclamptic patients may be complicated by HELLP syndrome, a syndrome of intravascular hemolysis (H), elevated liver enzymes (EL) and low platelet count (LP). These patients typically present at early third trimester with epigastric or right upper quadrant pain, nausea and vomiting. They may present without the clinical signs of preeclampsia (hypertension and proteinuria or edema), thus an initial wrong nonobstetric diagnosis is not uncommon. The most frequent maternal complication is intravascular coagulopathy (30%). Placental abruption and acute renal failure are also common. Ten cases of maternal deaths were reported among 295 cases reviewed in the English language literature, while the perinatal mortality rate was 226/1000. The grave prognosis for mother and fetus warrants physician awareness in order to accomplish early diagnosis and proper management. This paper is a review of the literature in English.
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PMID:HELLP syndrome--a syndrome of hemolysis, elevated liver enzymes and low platelet count--complicating preeclampsia-eclampsia. 168 23

A 15-yr-old girl presented with complaints of right upper quadrant pain and jaundice. Elevation of serum alkaline phosphatase, signs of protal hypertension, and computed tomographic scan findings suggested a diagnosis of primary sclerosing cholangitis. However, cultures of the bile and of the common bile duct specimen obtained during a surgical procedure grew Cryptococcus neoformans. Treatment with amphotericin B was begun. An episode of upper gastrointestinal bleeding, however, led to the hepatorenal syndrome, and the patient died before antifungal therapy was completed. At autopsy, active sclerosing cholangitis associated with cryptococci involved the common bile duct. We suggest that opportunistic infection of the biliary tree should be considered in pediatric patients with presumed primary sclerosing cholangitis.
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PMID:Cholangitis associated with Cryptococcus neoformans. 397 25

Five cases of pregnant woman with symptoms of right upper quadrant pain, hemoconcentration, liver dysfunction, and thrombocytopenia are presented as representative of impending gestosis. Plasma volume expansion achieved by either bed rest or intravenous albumin administration appeared to be effective therapy. It is presumed that impending gestosis represents an early form of severe toxemia (edema/proteinuria/hypertension [EPH[ gestosis).
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PMID:Impending gestosis. 731 42

An atypical case of HELLP syndrome is reported. The case is unique in that the patient lacked the usual symptoms and signs of hypertension, abdominal right upper quadrant pain, and tenderness. Early detection and immediate delivery resulted in a successful outcome.
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PMID:An atypical case of hemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome. 790 61

Seventy-three highly selected patients (35 type II, 38 type III) with intractable biliary-type pain were studied with biliary manometry after a baseline endoscopic retrograde cholangiopancreatography was normal or showed only duct dilatation. No differences between the two groups were noted in regard to baseline sphincter hypertension (60% versus 55%), improvement after endoscopic sphincterotomy at mean follow-up of 3 years, or post-procedure pancreatitis rates (15% versus 16%). Although not statistically significant, a tendency for patients with bile ducts > or = 12 mm to have sustained clinical improvement after sphincterotomy was noted in comparison with patients having ducts < 12 mm; an inverse correlation between improvement in symptoms and presence of an intact gallbladder at baseline was also seen. The authors suggest that the current classification, which divides patients with recurrent right upper quadrant pain into types I, II, and III, is inadequate to define either incidence of sphincter of Oddi dysfunction or subsequent response to endoscopic sphincterotomy.
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PMID:Long-term outcome after endoscopic sphincterotomy in patients with biliary colic and suspected sphincter of Oddi dysfunction. 801 15

The HELLP syndrome is a severe and life-threatening complication of preeclampsia with typical laboratory findings. The frequency of the disease is 1 to 150-300 live births in perinatal centers. The median gestational age at presentation is 34 weeks, however, the disease may also develop during the early postpartum period. Right upper quadrant pain is the most striking clinical symptom; in up to 15% of cases neither hypertension nor proteinuria is present on admission. For the detection of hemolysis determination of haptoglobin levels is the most suitable method. Coagulation disorders are more pronounced in patients with the HELLP syndrome as compared to those with preeclampsia without the HELLP constellation, however the full-blown syndrome of disseminated intravascular coagulation (DIC) is not a leading symptom but the consequence of delayed diagnosis and/or therapy of the primary disease. The course of the HELLP syndrome is unpredictable. On the one hand, complete reversal of symptoms under conservative treatment have been reported in individual cases, on the other hand, rapid, therapy-resistant deterioration of the disease had been observed in the majority of patients accompanied by severe complications (e.g. liver rupture). As a consequence the mother and the newborn need intensive care, and these women should be delivered in an obstetric intensive care unit. The maternal mortality reported from the international literature is 3.3%, and the perinatal mortality 22.6%.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[HELLP syndrome]. 802 4

The early detection of HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) is the basic condition for immediate therapeutic management, which mainly leads to prompt delivery. The classical symptoms despite the typical laboratory evaluation (hemolysis, elevated liver enzymes, low platelets) are epigastric or right upper quadrant pain and nausea and vomiting; the classical signs of preeclampsia (proteinuria and hypertension) may be absent in 20%. The differential diagnostic problems of HELLP syndrome arise in relation to the mimicry-symptomatic: upper abdomen pain can imitate gastroenterologic diseases (e.g. cholelithiasis, appendicitis), the elevated liver enzymes combined with hyperbilirubinemia liver diseases (e.g. viral hepatitis) and thrombocytopenia in combination with hemolytic anemia, neurological symptoms and renal failure other similar pathogenetic disorders due to the category of thrombotic microangiopathies. Regarding the common symptoms thrombocytopenia, hemolysis as well as signs of preeclampsia with or without renal failure the differentiation from various autoimmune diseases also can be difficult in special cases. Rare first manifestations and serious simultaneous diseases which can overlay the typical signs of HELLP syndrome show the variety of HELLP syndrome. Interdisciplinary detours and delay are the consequences of this differential diagnostic problems, which could imply deleterious effects on the mother and the fetus, until the final diagnosis is clear. Therefore all pregnant women with upper abdomen pain irrespective of symptoms of preeclampsia should be considered to have HELLP syndrome and immediate laboratory evaluation has to be done. If there is any doubt a interdisciplinary consultation is required!
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PMID:[Differential HELLP syndrome diagnosis]. 896 90

HELLP syndrome is a serious, life-threatening form of pre-eclampsia with a typical laboratory triad. The incidence of the disease is reported as being 0.17-0.85% of all live births. There has been, to date, neither reliable early recognition nor effective prevention of HELLP syndrome. As a result of endothelial dysfunction, activation of intravascular coagulation occurs with fibrin deposition in the capillaries and consecutive microcirculation disorders. The disease manifests itself on average between 32-34 weeks' gestation. HELLP syndrome will occur postpartum in up to 30% of the cases. The clinical cardinal symptom of the disease is right upper quadrant pain or epigastric pain accompanied with nausea, vomiting and malaise. In 20% of the cases with HELLP syndrome there is no hypertension and 5-15% of the pregnant patients present a low level of proteinuria or none at all. The early recognition of hemolysis is most sensitively managed by the determination of the serum haptoglobin. The increase of the aspartate transaminase (AST) and the alanine transaminase (ALT) often precedes a decrease in platelets. The course of HELLP syndrome is incalculable. It is universally agreed that a pregnancy from 32-34 weeks should be immediately delivered. Before 32-34 weeks, expectant management is generally possible in a perinatal center. The frequency for a repeated hypertensive disease in pregnancy ranges from 27% to 48%.
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PMID:HELLP syndrome. 1103 96

Herein, we report the case of a 52-year-old female with new-onset right upper quadrant pain and hypertension. She was found to have hemoperitoneum upon surgical exploration. Visceral angiography demonstrated a left hepatic artery aneurysm and an occluded right renal artery. The patient was treated with percutaneous angiographic embolization of the left hepatic artery, followed by right nephrectomy and cholecystectomy. Ruptured hepatic aneurysms are associated with a high mortality rate. The clinical presentation of patients with hepatic artery aneurysms is discussed. Early diagnosis and treatment are essential for a favorable outcome.
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PMID:Ruptured hepatic artery aneurysm: case report and review of literature. 1596 91

Non-alcoholic steatohepatitis (NASH) is one of the most common liver disorders. This is highly prevalent in obese and diabetic subjects. Persons with central obesity are at particular risk. Other clinical predictors are age more than 40-50 years and hyperlipidemias, but none of these factors is invariable for causation of NASH. Other reported associations are, celiac disease, Wilson's Disease and few other metabolic diseases. Drugs, particularly amiodarone, tamoxifen, nucleoside analogues and methotrxate have also been linked to NASH. The disease is evenly distributed in both sexes but advanced disease is more common in women. Ethnic variation exists and African Americans are less affected than Hispanic Americans. Specific clinical features of NASH are infrequent. Patients usually come to clinical attention by elevated liver enzymes found on routine evaluation but on history, about two third of patients will admit to have mild fatigue and about half will report right upper quadrant pain. Rarely, patient may present with a complication of cirrhosis. Physical examination may reveal hepatomegaly and splenomegaly. Research in last few years has stressed that development of steatosis, stetohepatitis, fibrosis with subsequent cirrhosis are most probably the result of insulin resistance. Therefore, clinical features may reflect existence of insulin resistance. Obesity, particularly central obesity is most important of these. Patients may have sleep apnea syndrome. Hypertension and manifestations of diabetes mellitus like polyuria, polydypsia, and neurological deficits may occur. Patients may have varying combination of obesity, diabetes, hyperlipidemia, hypertension and impaired fibrinolysis (syndrome X). Children with insulin resistance may show acanthosis nigricance. Patients with polycystic ovary syndrome, which consists of insulin resistance, diabetes, obesity, hirsutism, oligo or polymenorrha and hyperlipidemia may have NASH. Other rare manifestations of insulin resistance, which can be seen in patients of NASH are lipomatosis, lipoatrophy/lipodystrophy and panniculitis. Most other rare conditions known to cause NASH like peroxisomal diseases, mitochondialpathies, Weber-Christian disease, Mauriac syndrome, Madelung's lipomatosis and abetaliopprotenemia also have insulin resistance. This is believed that primary defect underlying insulin resistance is impairment in postreceptor pathways (through tyrosine kinase activity) of insulin action. Primary defect in insulin receptors appear uncommon. This results in down regulation of insulin receptor substance 1 (IRS-1) signaling by excess free fatty acids. In muscle, activated IRS-1 promotes translocation of glucose transporter protein 4 (GLUT4) to cell membrane. As a result, monocyte glucose uptake by GLUT4 increases glucose disposal from blood and reduced need for insulin. PKC-0 is a likely candidate as serine kinase in muscle regulated by fatty acids that can impair the activation of IRS-1. Insulin resistance is usually evaluated by fasting insulin levels, Quantitative Insulin Check Index (QUICKI) and Homeostasis Model Assessment of Insulin Resistance (HOMA), C-peptid/insulin ratio oral glucose tolerance test and hyper insulinemic euglycemic clamp. The clamp technique is considered the gold standard.
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PMID:Insulin resistance and clinical aspects of non-alcoholic steatohepatitis (NASH). 1619 20


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