scholarly journals Reversal of portal gastropathy with partial internal biliary diversion in a case of progressive familial intrahepatic cholestasis

2021 ◽  
Vol 2021 (3) ◽  
Author(s):  
Arkadeep Dhali ◽  
B Rathna Roger ◽  
Elaina Pasangha ◽  
Christopher D'Souza ◽  
Gopal Krishna Dhali
Keyword(s):  
Liver Disease ◽  
Early Diagnosis ◽  
Liver Transplant ◽  
Intrahepatic Cholestasis ◽  
Genetic Disorder ◽  
Disease Diagnosis ◽  
End Stage Liver Disease ◽  
Rare Genetic Disorder ◽  
Biliary Diversion ◽  
End Stage

Progressive intrahepatic cholestasis is a rare, genetic disorder causing bile acid secretion or transport defects. It can result in intrahepatic cholestasis that can progress to end-stage liver disease. Diagnosis is made using a combination of clinical and biochemical approaches. Genetic testing is currently the gold standard for investigation. We report a case of an 18-month-old male child with cholestatic pattern of jaundice from 16 months of life, which was associated with features suggestive of portal gastropathy. Detailed workup led to the diagnosis of progressive intrahepatic cholestasis (type 2). Early diagnosis prevented the need for liver transplant, and the child underwent surgical treatment with partial internal biliary diversion. Portal gastropathy and disease progression dramatically changed with corrective surgery. The patient was symptom-free at 10-week follow-up. Detecting this rare genetic disorder early has very good therapeutic implications from the patient's perspective and their morbidity and mortality profile; if untreated, it has a high propensity to progress to end-stage liver disease. The requirement of surgical interventions and liver transplantation is individualized on a case-to-case basis. An early diagnosis and initiation of treatment can prevent the need for a liver transplant as shown in the present case.

scholarly journals Hepatic encephalopathy expands the predictivity of model for end-stage liver disease in liver transplant setting: Evidence by means of 2 independent cohorts

2016 ◽  
Vol 22 (10) ◽  
pp. 1333-1342 ◽  
Author(s):  
Cristina Lucidi ◽  
Stefano Ginanni Corradini ◽  
Juan G. Abraldes ◽  
Manuela Merli ◽  
Puneeta Tandon ◽  
...  
Keyword(s):  
Liver Disease ◽  
Hepatic Encephalopathy ◽  
Liver Transplant ◽  
End Stage Liver Disease ◽  
End Stage

scholarly journals Language impairment in adults with end-stage liver disease: application of natural language processing towards patient-generated health records

2019 ◽  
Vol 2 (1) ◽  
Author(s):  
Lindsay K. Dickerson ◽  
Masoud Rouhizadeh ◽  
Yelena Korotkaya ◽  
Mary Grace Bowring ◽  
Allan B. Massie ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Natural Language Processing ◽  
Liver Disease ◽  
Natural Language ◽  
Liver Transplant ◽  
Language Processing ◽  
Word Length ◽  
Post Transplant ◽  
End Stage Liver Disease ◽  
End Stage

Abstract End-stage liver disease (ESLD) is associated with cognitive impairment ranging from subtle alterations in attention to overt hepatic encephalopathy that resolves after transplant. Natural language processing (NLP) may provide a useful method to assess cognitive status in this population. We identified 81 liver transplant recipients with ESLD (4/2013–2/2018) who sent at least one patient-to-provider electronic message pre-transplant and post-transplant, and matched them 1:1 to “healthy” controls—who had similar disease, but had not been evaluated for liver transplant—by age, gender, race/ethnicity, and liver disease. Messages written by patients pre-transplant and post-transplant and controls was compared across 19 NLP measures using paired Wilcoxon signed-rank tests. While there was no difference overall in word length, patients with Model for End-Stage Liver Disease Score (MELD) ≥ 30 (n = 31) had decreased word length in pre-transplant messages (3.95 [interquartile range (IQR) 3.79, 4.14]) compared to post-transplant (4.13 [3.96, 4.28], p = 0.01) and controls (4.2 [4.0, 4.4], p = 0.01); there was no difference between post-transplant and controls (p = 0.4). Patients with MELD ≥ 30 had fewer 6+ letter words in pre-transplant messages (19.5% [16.4, 25.9] compared to post-transplant (23.4% [20.0, 26.7] p = 0.02) and controls (25.0% [19.2, 29.4]; p = 0.01). Overall, patients had increased sentence length pre-transplant (12.0 [9.8, 13.7]) compared to post-transplant (11.0 [9.2, 13.3]; p = 0.046); the same was seen for MELD ≥ 30 (12.3 [9.8, 13.7] pre-transplant vs. 10.8 [9.6, 13.0] post-transplant; p = 0.050). Application of NLP to patient-generated messages identified language differences—longer sentences with shorter words—that resolved after transplant. NLP may provide opportunities to detect cognitive impairment in ESLD.

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