What is Neonatal Cholestasis?

Neonatal Cholestasis
Histological and immunohistochemical findings in 1HR1H4 mutant livers.Expression of BSEP, MDR3, and FXR in the liver (diaminobenzidine chromogen and hematoxylin counterstain) in patients with NR1H4-related cholestasis; normal controls in insets. Hematoxylin/eosin views for orientation. Original magnification, all images, × 200. BSEP and FXR are absent in all four patients when compared with the positive control (inset). MDR3 is present in all four patients although reduced in patients 2 and 4. H&E, hematoxylin, and eosin. Mutations in the nuclear bile acid receptor FXR cause progressive familial intrahepatic cholestasis: Gomez-Ospina N, Potter CJ, Xiao R, Manickam K, Kim MS, Kim KH, Shneider BL, Picarsic JL, Jacobson TA, Zhang J, He W, Liu P, Knisely AS, Finegold MJ, Muzny DM, Boerwinkle E, Lupski JR, Plon SE, Gibbs RA, Eng CM, Yang Y, Washington GC, Porteus MH, Berquist WE, Kambham N, Singh RJ, Xia F, Enns GM, Moore DD - Nature communications (2016). Not altered. CC.

Neonatal cholestasis is prolonged jaundice beyond 2 weeks of age.

What is the Pathology of Neonatal Cholestasis?

The pathology of neonatal cholestasis is:

-Etiology: The cause of neonatal cholestasis is intrahepatic neonatal cholestasis.

-Genes involved: None.

-Pathogenesis: The sequence of events that lead to neonatal cholestasis includes elevated conjugated bilirubin levels in the newborn period.

-Histology: The histology associated with neonatal cholestasis shows lobular changes: giant cell transformation (hepatocytes containing 4 – 10 nuclei), variable lobular inflammatory infiltrate and necrosis (spotty, confluent to bridging), canalicular ± hepatocellular bilirubinostasis, extramedullary hematopoiesis.

How does Neonatal Cholestasis Present?

Patients with neonatal cholestasis typically affect newborns. The symptoms, features, and clinical findings associated with neonatal cholestasis include jaundice, cirrhosis, hepatomegaly, ascites, and itching.

How is Neonatal Cholestasis Diagnosed?

Neonatal cholestasis is diagnosed through ultrasonography, and hepatobiliary.

How is Neonatal Cholestasis Treated?

Neonatal cholestasis has no specific treatment, only symptomatic relief is provided

What is the Prognosis of Neonatal Cholestasis?

The prognosis of neonatal cholestasis is fair depending upon the severity of the disease. 

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Last updated on May 24, 2022