Clinical symptoms, biochemistry, and liver histology during the native liver period of progressive familial intrahepatic cholestasis type 2.
Hiroki KondouSatoshi NakanoTadahaya MizunoKazuhiko BesshoYasuhiro HasegawaAtsuko NakazawaKen TanikawaYoshihiro AzumaTatsuya OkamotoAyano InuiKazuo ImagawaMureo KasaharaYoh ZenMitsuyoshi SuzukiHisamitsu HayashiPublished in: Orphanet journal of rare diseases (2024)
Japanese PFIC2 patients had gradually worsening clinical findings since the initial visit, resulting in LDLT during infancy. NaPB therapy improved jaundice and growth retardation but was insufficient to treat pruritus and liver fibrosis.
Keyphrases
- liver fibrosis
- end stage renal disease
- ejection fraction
- newly diagnosed
- chronic kidney disease
- multiple sclerosis
- peritoneal dialysis
- prognostic factors
- early onset
- mesenchymal stem cells
- patient reported outcomes
- bone marrow
- weight gain
- physical activity
- body mass index
- sleep quality
- smoking cessation
- replacement therapy