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Neonatal cholestasis underpowered to detect portal hypertension in kids with AAT deficiency

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While the need for liver transplantation is not urgent and the risk of death is low, portal hypertension nevertheless disrupts growth measures in children with alpha-1-antitrypsin (AAT) deficiency, a recent study has shown.

Moreover, having a history of neonatal cholestasis appears to be a weak predictor of portal hypertension, suggesting that there is a need for continuous monitoring and guidance of young AAT patients, regardless of such a disease history.

Of the 350 participants (median age, 4.2 years; 60 percent male), 21 percent had definite or possible, clinically evident portal hypertension at entry. Of the remaining 278 children without such condition at baseline, 18 eventually developed it over a median 2.5 years of follow-up. The resulting incidence rate was 2.1 per 100 person-years of follow-up.

Thirty-two patients either needed liver transplantation or died over 1,077 person-years of follow-up, yielding an annual incidence rate of 3.0 transplants per 100 person-years. Almost all the transplanted patients entered the study with definite or possible portal hypertension.

For all participants, the 2-year probability of developing definite or possible portal hypertension is 5 percent. Over the same time scale, the incidence of death or liver transplantation increased slowly, reaching a peak probability of 8 percent.    

Kaplan-Meier curves for portal hypertension-free survival further showed that the likelihood of such an event was comparable between children who did vs did not have a history of neonatal cholestasis. The same was true for liver transplant-free survival rates.

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Most Read Articles
Stephen Padilla, 23 Nov 2020
Fetoscopic laser ablation (FLP) is an effective treatment for twin-to-twin transfusion syndrome (TTTS) in monochorionic twin pregnancies, but it requires a tertiary neonatal facility and a highly specialized team in performing intervention therapies for the treatment of potential complications, according to a Singapore study.
18 Nov 2020
Serum folate levels during pregnancy correlate with the child’s neurodevelopment at 2 years of age, particularly in terms of language development, a recent study has shown. Folate supplementation may be a valuable nutritional intervention to consider.
18 Oct 2020
Girls with higher body fat percentage and whose parents smoke are likely to undergo precocious puberty, as reported in a study.