Clinically Evident Portal Hypertension

Abstract

Portal Hypertension (PHT) is a major cause of morbidity and mortality in pediatric liver diseases. Thus, research into causes and disease modifiers in PHT in these conditions is vitally important. PHT is rarely directly or indirectly measured in the assessment of children with chronic liver disease. A straightforward, reproducible definition of portal hypertension could be invaluable for consistently identifying patients with portal hypertension and for grouping these patients according to their risk of complications from their disease. We propose the term Clinically Evident Portal Hypertension (CEPH) to denote clinical findings that demonstrate evidence of elevated portal pressure. When CEPH criteria are met, PHT is highly likely to be present, although it is likely that PHT exists for variable periods of time prior to meeting CEPH criteria. Use of this research definition of CEPH will allow for consistent identification of these patients by clinicians in nearly any clinical setting and serve as a clinical milepost that may dictate future prognosis in pediatric patients with cirrhosis. Portal hypertension (PHT) is the result of complex pathophysiologic processes that can affect patients across the spectrum of liver disease. Prolonged increases in portal pressure can result in life-threatening complications and lead to an array of chronic morbidities including variceal hemorrhage, hypersplenism, hepatopulmonary syndrome, hepatorenal syndrome, and ascites. PHT occurs as a result of increased portal resistance, increased portal

Outcomes of Childhood Cholestasis in Alagille Syndrome: Results of a Multicenter Observational Study

“…For the purposes of this study, LOGIC cholestasis enrollment criteria were defined by the presence of one or more of the following: fasting total serum bile acid greater than 3 times the upper limit of normal for age, direct/conjugated bilirubin greater than 2 mg/dL, fat-soluble vitamin deficiency otherwise unexplainable, gamma-glutamyl transferase (GGT) greater than 3 times the upper limit of normal for age, and/or intractable pruritus explainable only by liver disease. The onset of definite clinically evident portal hypertension in this cohort was assessed using the following criteria, which have recently been published by the ChiLDReN (12) : (1) ascites and the use of diuretics, or (2) documented esophageal or gastric varices on endoscopy, or (3) presence of both Although example participants A, B, C, and D were enrolled and observed in the LOGIC study at various time points after birth, the subjects in groups E and F experienced liver transplant/death before the opportunity to be enrolled, and therefore were not observed in the study. In addition, not all events were observed in A, B, C, and D due to right censoring and left censoring.…”

Nonfasted Liver Stiffness Correlates with Liver Disease Parameters and Portal Hypertension in Pediatric Cholestatic Liver Disease

“…(20) In light of these issues, the described research definition of CEPH was used as a validation for the potential utility of LSM in pediatric cholestasis. (10) We found statistically and clinically relevant differences in LSMs in all three diseases in children with dCEPH versus aCEPH. In A1ATD, there were clear step-wise increases in LSMs based on progressive CEPH.…”

“…The relationship between LSM and portal hypertension was investigated using the recently developed research definition of CEPH. (10) dCEPH was more common in BA (48%) than in A1ATD (8%) or ALGS (15%) ( Fig. 3; Supporting Tables S5 and S6).…”

“…While splenomegaly on physical examination and thrombocytopenia are crude measures of portal hypertension, more precise data would be helpful in assessing the progression of pediatric liver disease over time and in predicting the risk of complications of portal hypertension, including variceal hemorrhage and ascites. (10) The National Institute of Diabetes and Digestive and Kidney Diseases/National Institutes of Healthsponsored Childhood Liver Disease Research Network (ChiLDReN) is a multicenter consortium that longitudinally follows infants and children with cholestatic liver diseases, including BA, A1ATD, ALGS, progressive familial intrahepatic cholestasis, bile acid synthetic defects, and mitochondrial disorders. Clinical history, physical examination, and laboratory findings are collected annually on participants in ChiLDReN research protocols.…”

Evaluation of the endoscopic and surgical methods of prehepatic portal hypertension treatment in children

“…Портальна гіпертензія (ПГ) -це стан, що характеризується підвищенням тиску в системі портальної вени за рахунок різних патологічних процесів, які спричиняють розвиток ускладнень, включаючи кровотечу внаслідок розвитку варикозно розширених вен (ВРВ), спленомегалії та гіперспленізму [3,17]. Етіологія та патофізіологія причинних фак- [2,6,8,14].…”