Conclusion The age of presentation of CHF typically ranges between 1.8-14 years, both cases in this report were presented at a relatively older age. [aclr.com.es]

Firm or hard hepatomegaly is present nearly in all patients, often with a prominent left lobe, and this is usually one of the presenting signs. [ncbi.nlm.nih.gov]

CHF has initial presentation may be at around 3-6 months. The presentation ranges between 1.8-14 years, Portal Hypertension is a usual accompaniment and renal involvement is seen with 9 ]. [omicsonline.org]

• Malnutrition

  Diagnoses upon admission were as follows: severe acute malnutrition, cryptogenic liver cirrhosis, grade III hepatic encephalopathy, normochromic normocytic anemia, hyperuricemia, thrombocytopenia, septic shock, and probable cholangitis. Evolution. [elsevier.es]

  Agents/circumstances to avoid: Alcohol, obesity, diabetes mellitus, malnutrition, infection with human immunodeficiency virus (HIV), immunosuppression, hepatotoxic medicines, nonsteroidal anti-inflammatory drugs (NSAIDs) in those with varices because [ncbi.nlm.nih.gov]

• Splenomegaly

  RESULTS: Sixteen patients had morphologic abnormalities in the liver, 15 had splenomegaly (three underwent splenectomy for portal hypertension), and 14 had varices or spontaneous splenorenal shunts. [ncbi.nlm.nih.gov]

• Pneumonia

  One patient died within days of admission with convulsions, coma, and aspiration pneumonia. One patient was lost to follow-up. The remaining 12 patients are all alive and receive regular follow-up. Two patients required splenorenal shunt. [ncbi.nlm.nih.gov]

• Gastropathy

  Final diagnoses are as follows: • Biliary cirrhosis probably secondary to congenital liver fibrosis, which caused portal hypertension, esophageal and fundal varices, and hypertensive gastropathy • Upper and lower GI bleeding • Acute liver failure in the [elsevier.es]

• Hypertension

  Although this study demonstrates that CHF and portal hypertension can occur with AD-PKD, CHF and portal hypertension are a characteristic finding with AR-PKD. [gutsandgrowth.wordpress.com]

  Commonly Associated Conditions Portal hypertension leading to varices and hypersplenism Hepatomegaly Systemic hypertension Renal dysfunction Cholangitis Conditions associated with the finding of ductal plate malformation/CHF: ARPKD; most frequent association [unboundmedicine.com]

  Portopulmonary hypertension is a rare complication of portal hypertension. [ncbi.nlm.nih.gov]

• Hepatomegaly

  Hepatomegaly was present in all patients and splenomegaly in all but one. Liver function and renal function tests were normal in all children, except for a raised serum alkaline phosphatase in six. [ncbi.nlm.nih.gov]

• Hepatosplenomegaly

  The patients showed early hepatosplenomegaly, portal hypertension, and no apparent kidney involvement. Clinical and laboratory findings were similar in both patients. [ncbi.nlm.nih.gov]

• Eruptions

  On examination, her upper primarily canines were loose, she had severe periodontitis, eruption of permanent teeth, diffuse eritematous and hyperkeratotic palms and soles that suggested the syndrome. [ncbi.nlm.nih.gov]

• Ulcer

  Also, because of the liver damage, in the esophagus and stomach there are dilated and congested varices associated with ulcerated areas in the esophagus. [elsevier.es]

• Renal Insufficiency

  It appears to be a particular risk in patients with prolonged survival but is rare in CHF, where portal hypertension and renal insufficiency make the long-term prognosis poor. [ncbi.nlm.nih.gov]

Laboratory workup may reveal mild elevations in liver enzymes. Patients with a predominantly cholangitic clinical picture may have marked elevations in alkaline phosphatase (ALP), γ-glutamyl transpeptidase (GGT) and bilirubin. [wjgnet.com]

• Enlargement of the Liver

  A to Z definitions A liver abnormality common in children with ARPKD; it may lead eventually lead to enlargement of the liver and spleen. [pkdcure.org]

  According to the operative findings, his liver was also grossly nodular and enlarged, so he was diagnosed with liver cirrhosis but no specific treatment was given. [kjim.org]

  Clinical findings include enlarged, abnormally shaped liver, relatively well-preserved hepatocellular function, and portal hypertension (PH) resulting in splenomegaly, hypersplenism, and gastroesophageal varices. [ncbi.nlm.nih.gov]

• Liver Biopsy

  In one patient the liver biopsy was repeated after 18 years and showed only a mild progression of fibrosis in the portal triads. Duodenal biopsies taken in infancy in two of the three patients did not show any abnormalities. [ncbi.nlm.nih.gov]

  Liver biopsy was performed but the diagnosis was difficult to make due to tissue damage. Later on liver biopsy was repeated in preparation for a porta-caval anastomosis. [jstage.jst.go.jp]

During combined drug treatment, the patient remained free of cholangitis for at least two years. [ncbi.nlm.nih.gov]

Pharmacological treatment and treatment based on sclerosis should be continued. It is important to rule out the presence of gastric varices prior to treatment of the esophageal varices. [elsevier.es]

As these infections (cholangitis) are often severe, they require long-term intravenous treatment. [ekids.hcuge.ch]

Although epoprostenol infusion, nitric oxide inhalation, isosorbide-5-mononitrate, nitroglycerin, and calcium channel blockers may reduce pulmonary artery pressure in patients with portopulmonary hypertension, the prognosis remains poor. [ncbi.nlm.nih.gov]

The management and prognosis of CHF is dependent on alimentary bleeding secondary to portal hypertension. In late childhood abdominal pain, cholangitis and features of hypersplenism complicate the problem. [scopemed.org]

Mean age of clinical presentation was 25.68±20.99, variably ranging Conclusions: CHF presented as a rare liver disease with an asymptomatic long-term period and a poor prognosis when clinically apparent. [thieme-connect.com]

METHODOLOGY: A total of 1285 patients with portal hypertension of different etiologies were studied using ultrasonography as the screening test. [ncbi.nlm.nih.gov]

[…] structures numerous epithelium-lined structures of biliary origin elongated and cystic spaces lined by regular biliary epithelium central or peripheral localization in the portal space luminal inspissated bile +/- cholangitis associated Caroli disease Etiology [humpath.com]

Epidemiology The incidence of ARPKD is 1/20,000–1/40,000 live births. Carrier frequency of PKHD1 mutation in the general population is ∼1:70. [unboundmedicine.com]

Epidemiology Frequency International Congenital hepatic fibrosis is a rare autosomal recessive disease; the exact incidence and prevalence are not known. [emedicine.com]

The associating biliary ductal disease led the authors to think that all these entities are a continuum and different reflections of the same underlying pathophysiological process. [ncbi.nlm.nih.gov]

Pathophysiology Ductal plate malformation is a characteristic histologic lesion of the liver, implying a disturbance of the normal development of the bile ducts. [unboundmedicine.com]

Only rarely is hepatic synthetic function compromised Predisposition to cholangitis may affect transplantation decisions and timing As our understanding of pathophysiology improves, frequently this results in reclassification of diseases. [gutsandgrowth.wordpress.com]

Several studies have attempted to establish the pathophysiological mechanism behind the abnormal and excessive fibrotic response associated with CHF. [wjgnet.com]

Dehydrocholic acid with meals improved hepatobiliary excretion of the radioactive isotope and lowered serum bile acid levels but did not prevent cholangitic attacks when used alone. [ncbi.nlm.nih.gov]

Fibrous foods help keep stool soft and easy to pass, which can help prevent constipation and subsequent straining to have a bowel movement. [chicstrology.com]