Congenital hepatic fibrosis in children

Álvarez, Fernando; Bernard, Olivier; Brunelle, Françis; Hadchouel, M; Leblanc, A.; Odièvre, M; Alagille, D

doi:10.1016/s0022-3476(81)80320-4

Cited by 129 publications

(72 citation statements)

References 10 publications

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“…In a prospective study in which 18 patients (mean age of 16 years) were followed up for 6 years, Perisic (16) reported that CHF with Fatal Cholestatic Liver Damage severe cholangitis and renal failure werethe major causes of death in CHF. In the present patient, there were no clinical findings of Calori syndrome (13) or polycystic kidney (15), which are frequent complications of CHFand may contribute to cholangitis and renal failure. NRH,identified in the second biopsy, is a pathological concept that includes any diseases presenting this type of hyperplasia (17), and various cases have been reported (1 8-24).…”

Section: Case Reportcontrasting

confidence: 44%

“…The characteristic finding of CHFis irregular fibrous expansion of Glisson's sheath with ductular proliferation ( 12). The pathological findings in the first liver biopsy were compatible with the initial stage of CHE It has been reported that cholestasis, cholangitis, and intestinal bleeding due to portal hypertension are the most common clinical signs in CHF (13). There are also some atypical cases with refractory ascites from the early clinical stage or without portal hypertension (14).…”

Section: Case Reportmentioning

confidence: 69%

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Congenital Hepatic Fibrosis with Fatal Cholestatic Liver Damage.

et al. 2000

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A 23-year-old male with congenital hepatic fibrosis died because of progressive cholestatic liver damage. Pathologically, marked extension of fine fibers along the sinusoids in addition to fibrosis in Glisson's sheath, miniaturization and pseudo-glandular formation of hepatocytes as parenchymal damage, and nodular regenerative hyperplasia were considered as the cause of rapid aggravation of liver damage or portal hypertension. (Internal Medicine 39: 930-935, 2000)

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Section: Case Reportcontrasting

confidence: 44%

Section: Case Reportmentioning

confidence: 69%

Congenital Hepatic Fibrosis with Fatal Cholestatic Liver Damage.

et al. 2000

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“…All ARPKD patients manifest some degree of congenital hepatic fibrosis (CHF) caused by ductal plate malformation of the developing portobiliary system; some patients also have macroscopic dilations of the intrahepatic bile ducts, a combination termed Caroli's syndrome (7,13,14). Portal hypertension complicates CHF and often results in esophageal varices and hypersplenism (15)(16)(17)(18). Early-onset severe hypertension, often requiring multiagent therapy, occurs in most ARPKD patients (5).…”

mentioning

confidence: 99%

Correlation of Kidney Function, Volume and Imaging Findings, and PKHD1 Mutations in 73 Patients with Autosomal Recessive Polycystic Kidney Disease

Gunay‐Aygun¹,

Font–Montgomery²,

Lukose³

et al. 2010

Clinical Journal of the American Society of Nephrology

109

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Background and objectives: Renal function and imaging findings have not been comprehensively and prospectively characterized in a broad age range of patients with molecularly confirmed autosomal recessive polycystic kidney disease (ARPKD).Design, setting, participants, & measurements: Ninety potential ARPKD patients were examined at the National Institutes of Health Clinical Center. Seventy-three fulfilled clinical diagnostic criteria, had at least one PKHD1 mutation, and were prospectively evaluated using magnetic resonance imaging (MRI), high-resolution ultrasonography (HR-USG), and measures of glomerular and tubular function.Results: Among 31 perinatally symptomatic patients, 25% required renal replacement therapy by age 11 years; among 42 patients who became symptomatic beyond 1 month (nonperinatal), 25% required kidney transplantation by age 32 years. Creatinine clearance (CrCl) for nonperinatal patients (103 ؎ 54 ml/min/1.73 m 2 ) was greater than for perinatal patients (62 ؎ 33) (P ‫؍‬ 0.002). Corticomedullary involvement on HR-USG was associated with a significantly worse mean CrCl (61 ؎ 32) in comparison with medullary involvement only (131 ؎ 46) (P < 0.0001). Among children with enlarged kidneys, volume correlated inversely with function, although with wide variability. Severity of PKHD1 mutations did not determine kidney size or function. In 35% of patients with medullary-only abnormalities, standard ultrasound was normal and the pathology was detectable with HR-USG.Conclusions: In ARPKD, perinatal presentation and corticomedullary involvement are associated with faster progression of kidney disease. Mild ARPKD is best detected by HR-USG. Considerable variability occurs that is not explained by the type of PKHD1 mutation.Clin J Am Soc Nephrol 5: 972-984, 2010.

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“…A portosystemic shunt surgical intervention has the least side effects and contraindications [15,16]. Alvares et al describe a population of 27 children with CFL in which a portosystemic shunt was performed in 16 patients aged 3-16 years [17]. During a 3 month to 12 year follow-up, liver functioning did not worsen, and signs of hepatic encephalopathy were absent.…”

Section: Discussionmentioning

confidence: 99%