cholestasis/edema

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Cholestasis in infants with immune hydrops fetalis.

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Rhesus (Rh) hemolytic disease of the newborn represents a broad spectrum of symptoms in the fetus and newborn, ranging from mild to severe hemolytic anemia and hydrops fetalis. Cholestasis is a common problem in infants with immune hydrops fetalis (IHF). The aim of this study was to evaluate the

CCBE1 mutation in two siblings, one manifesting lymphedema-cholestasis syndrome, and the other, fetal hydrops.

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BACKGROUND Lymphedema-cholestasis syndrome (LCS; Aagenaes syndrome) is a rare autosomal recessive disorder, characterized by 1) neonatal intrahepatic cholestasis, often lessening and becoming intermittent with age, and 2) severe chronic lymphedema, mainly lower limb. LCS was originally described in

A 6-year-old girl diagnosed with mevalonate kinase deficiency who had hydrops fetalis and neonatal-onset cholestasis.

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We experienced a 6-year-old girl diagnosed with mevalonate kinase deficiency (MKD) who had cholestasis, anemia, and elevated inflammatory markers in neonatal period. She was admitted to our hospital because of fever and elevated inflammatory markers at 5 years 11months of age. Without using

[The potentials of echotomography in the diagnosis of diseases of the liver, biliary tract and pancreas in the presence of the extrahepatic cholestasis syndrome].

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2260 patients with gastroenterologic diseases were examined by ultrasound tomography (sector echotomograph). In 47 (2.8%) of the patients echographic signs of extrahepatic cholestasis were found: dilated ductus choledochus (65.9%), dilated ductus hepaticus (34%), dilated intrahepatic biliary ducts

[Consequences of cholestasis from the pathologist's viewpoint].

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The present review summarizes extrahepatic obstructive cholestasis and its hepatic morphological sequelae. The first part focuses on early and late changes visualized in liver biopsies. In the second part, pathogenic mechanisms involved in liver fibrosis are discussed. A third section deals with the

Pancreatic and bile duct obstruction exacerbates rat caerulein-induced pancreatitis: a new experimental model of acute hemorrhagic pancreatitis.

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BACKGROUND Pancreatic duct obstruction induces edematous but not hemorrhagic pancreatitis even when combined with maximal secretory stimulation. The aim of the present study was to test the hypothesis that pancreatic and bile duct obstruction exacerbates edematous pancreatitis induced by

[Insulin edema in hepatic glycogenosis].

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BACKGROUND Hepatic glycogenosis is a rare syndrome, which includes poorly controlled diabetes mellitus, hepatomegaly, delayed puberty, and growth delay. Insulin edema is sometimes associated. METHODS An 18-year-old woman presented with diffuse edema, hepatomegaly, amenorrhea, uncontrolled diabetes,

Intrahepatic cholestasis due to systemic mastocytosis: a case report and review of literature.

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A 35-yr-old female presented with symptoms of obstructive jaundice. Liver biopsy, bone marrow aspiration, and biopsy revealed systemic mastocytosis and acute myeloid leukemia. The liver biopsy specimen showed infiltration of mast cells within portal tracts with periductal and portal edema,

Complete resolution of arrhythmia-induced hydrops fetalis in utero

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A 21-year-old G3P2011 Caucasian woman at 27 weeks' gestation presented with fetal tachyarrhythmia between 240 and 270 beats per minute. Fetal supraventricular tachycardia, abdominal ascites, pleural effusion and pericardial effusion indicated hydrops fetalis. Management with digoxin and flecainide

Acute febrile cholestasis as an inaugural manifestation of Kawasaki's disease.

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We report a child who developed acute febrile cholestasis with jaundice and pruritus as the inaugural manifestation of Kawasaki's disease (KD). The severe obstructive icterus and hydrops of the gallbladder required cholecystectomy that was not followed by remission of the fever and cholestasis. KD

Refractory fetal supraventricular tachycardia and obstetric cholestasis.

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OBJECTIVE Supraventricular tachycardia (SVT) is the most common form of sustained fetal tachyarrhythmia in pregnancy. The development of hydrops with SVT is associated with significant worsening of prognosis. METHODS We report a case of fetal SVT with hydrops at 28 weeks' gestation that required

Pharmacokinetic Characteristics of Baicalin in Rats with 17α-ethynyl-estradiol-induced Intrahepatic Cholestasis.

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Baicalin is one of the main active ingredients of choleretic traditional Chinese medicine drug Radix Scutellariae. The aim of this study was to explore the pharmacokinetic characteristics of baicalin in rats with 17α-ethynylestradiol (EE)-induced intrahepatic cholestasis (IC) based on its choleretic

Neonatal cholestasis as initial manifestation of type 2 Gaucher disease: a continuum in the spectrum of early onset Gaucher disease.

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Gaucher disease type 2 [OMIM #230800] is a rare lysosomal storage disorder with usual onset between 3 and 6 months of age leading to progressive neurodegeneration and death within the first 2 years of life. Rarely it may lack the characteristic symptom-free period and initially manifest prenatally

Conservative management of cholestasis with and without fever in acute biliary pancreatitis.

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The presence of cholestasis in both mild and severe forms of acute biliary pancreatitis (ABP) does not justify, of itself, early endoscopic retrograde cholangiography (ERC) or endoscopic sphincterotomy (ES). Clinical support treatment of acute pancreatitis for one to two weeks is usually accompanied

Severe intrahepatic cholestasis, erythrocytosis and hypoglycemia: unusual presenting features of systemic AL amyloidosis.

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We report the case of a 68-year-old African woman who presented with jaundice, hepatomegaly and anasarca. Clinical investigation disclosed severe intrahepatic cholestasis, nephrotic syndrome, erythrocytosis and hypoglycemia. Diagnosis of systemic AL amyloidosis was established by percutaneous liver

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