Preterm Infants With Biliary Atresia

Wessel, Daan B E van; Boere, Thomas; Hulzebos, Christian V.; Kleine, Ruben H J de; Verkade, Henkjan J.; Hulscher, Jan B F

doi:10.1097/mpg.0000000000001692

Cited by 29 publications

(15 citation statements)

References 29 publications

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“…We analyzed our data from seasonal aspects but could not identify significant time clustering in line with most of the Western and East Asian studies (29); however, this has been controversial as few studies from the United States showed significant clustering of BA from August to October ( 29 ) and December to March ( 14 ). Other demographic characteristics in Saudi Arabia, such as the rate of BASM (10.8%) and prematurity (10.2%), were comparable with the findings of previous studies that showed BASM in 8–14% ( 26 ) and prematurity in 13% ( 30 ). We observed a slight but insignificant female predominance in contrast to most of the Western and East Asian studies that showed significant female predominance ( 16 , 31 ).…”

Section: Discussionsupporting

confidence: 88%

“…In our study, of the 53 patients who underwent KPE between the ages of 60 and 90 days, 33% were alive with the native liver at 5 and 10 years. In addition, our data, supported by others ( 30 , 33 – 36 ), clearly show that undergoing an initial KPE did not impact negatively on the overall LT survival rate. Hence, BA patients presenting between the ages of 60 and 90 days should not be denied KPE.…”

Section: Discussionsupporting

confidence: 87%

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The Epidemiology and Outcome of Biliary Atresia: Saudi Arabian National Study (2000–2018)

et al. 2022

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BackgroundThe epidemiology and outcomes of biliary atresia (BA) have been well-documented in national cohorts from two main ethnicities, namely, the Asian Orientals and Caucasians, with incidence ranging from 1 in 5,000 to 1 in 9,000 live births in East Asia and 1 in 15,000 to 19,000 live births in Europe and North America.ObjectiveWe report the first nationwide BA study outside North America, Europe, and East Asia to describe the epidemiology and outcomes of BA in Saudi Arabia.MethodsA national database of BA cases diagnosed between 2000 and 2018 was analyzed. We assessed clearance of jaundice (bilirubin <20 μmol/L) in all cases that underwent Kasai portoenterostomy (KPE). We then estimated survival using the Kaplan–Meier method with endpoints of liver transplantation (LT), death, or survival with native liver (SNL).ResultsBA was diagnosed in 204 infants (106 females; 10% pre-term). The incidence of BA was 1 in 44,365, or 2.254 in 100,000 live births (range, 0.5–4 in 100,000). Polysplenia was diagnosed in 22 cases (11%). The median age at referral was 65 days. A total of 146 children (71.5%) underwent KPE at a median age of 70 days. Clearance of jaundice was achieved in 66 of the 146 (45%) infants. The 10-year SNL after KPE was 25.5%, and the overall 10-year estimated survival was 72.5%. The Kaplan–Meier survival curves for patients undergoing KPE at the age of <60, 61–90, and >90 days showed a SNL rate at 51.6, 33, and 12.5%, respectively, at 5 years (P < 0.001). The 2-, 5-, and 10-year post-LT survival rates were 92.5, 90.6, and 90%, respectively. Undergoing an initial KPE did not impact negatively on the overall LT survival rate when compared to BA cases that underwent primary LT (P = 0.88).ConclusionThe incidence rate of BA in Saudi Arabia is lower than the incidence reported elsewhere. Late referral of BA cases remains a problem in Saudi Arabia; as a result, the SNL rate was lower than reported by other national registries. Hence, national policies devoted to timely referral and earlier age at KPE are needed.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionsupporting

confidence: 87%

The Epidemiology and Outcome of Biliary Atresia: Saudi Arabian National Study (2000–2018)

et al. 2022

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“…Moreover, some pediatric patients with life-threatening conditions are subject to complex surgical treatments and prolonged anesthetic exposure during the first 3 years of life. 24,[29][30][31][32][33][34] The NHP model has been crucial in describing the risks associated with early life anesthesia in some of the most vulnerable patients.…”

Section: Anesthesia and Analgesiamentioning

confidence: 99%

Application of Nonhuman Primate Models in the Studies of Pediatric Anesthesia Neurotoxicity

Wang

Liu

et al. 2022

Anesthesia &Amp; Analgesia

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Numerous animal models have been used to study developmental neurotoxicity associated with short-term or prolonged exposure of common general anesthetics at clinically relevant concentrations. Pediatric anesthesia models using the nonhuman primate (NHP) may more accurately reflect the human condition because of their phylogenetic similarity to humans with regard to reproduction, development, neuroanatomy, and cognition. Although they are not as widely used as other animal models, the contribution of NHP models in the study of anesthetic-induced developmental neurotoxicity has been essential. In this review, we discuss how neonatal NHP animals have been used for modeling pediatric anesthetic exposure; how NHPs have addressed key data gaps and application of the NHP model for the studies of general anesthetic-induced developmental neurotoxicity. The appropriate application and evaluation of the NHP model in the study of general anesthetic-induced developmental neurotoxicity have played a key role in enhancing the understanding and awareness of the potential neurotoxicity associated with pediatric general anesthetics.

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“…The association of prematurity and biliary atresia is, therefore, possible by chance but obviously infrequent. There are three large-scale studies from Sweden [ 18 ], Taiwan [ 19 ] and, most recently, The Netherlands [ 20 ] in which the incidence of BA was reportedly higher in the premature population than in term infants with an odds ratio of 2.9 and 1.65 in the first two studies, respectively. Despite this reported increased incidence, outcomes and characteristics have been rarely reported, consisting largely of case reports (total reported = 5) [ 21 – 23 ].…”

Section: Discussionmentioning

confidence: 99%

Prematurity and biliary atresia: a 30-year observational study

2017

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Aim of studyThe diagnosis of biliary atresia (BA) remains challenging and delay can lead to significant morbidity with time to surgery a key factor in determining outcome. Prematurity may impact on outcome potentially delaying diagnosis. We sought to assess whether the premature BA infants (PBA) have a delayed time to surgery and as such, worse outcomes?MethodsReview of a single-centre prospectively maintained database. Prematurity was defined as delivery < 37/40 gestation. PBA was compared with date-matched term biliary atresia controls on a 2:1 basis. Primary outcomes were clearance of jaundice (< 20 μmol/L) and native liver survival. A retrospective assessment of liver fibrosis was made on biopsies at diagnosis and at Kasai portoenterostomy (KPE) in both premature and term cohorts. Data are quoted as median (range) unless indicated. A P value of ≤ 0.05 was considered statistically significant.Results21 (female n = 14, 67%) premature infants with BA were treated in the period Jan. 1988–Dec. 2016 and compared with 41 contemporaneous term BA controls. Median gestation was 33 (29–36) weeks and birth weight 1930 (948–4230)g. Twin pregnancy (n = 10) was the leading cause for prematurity and significantly higher than the controls (48 vs. 0%; P < 0.0001). Maternal co-morbidity was high (n = 10, 48%) including pre-eclampsia (19%) and diabetes (14%). Liver biopsy was performed in 19 (90%) patients (all diagnostic) at a median of 57 (4–266) days. Delayed diagnosis (> 50 days) was seen in n = 13 but not associated with parenteral nutrition use (46 vs. 33%, P = 0.59) or phototherapy (50 vs. 83%, P = 0.19). Both BASM (33 vs. 7.5%; P = 0.01) and duodenal atresia (19 vs. 0%; P = 0.01) were seen more frequently in the PBA cohort. Mean fibrosis scores (Ishak) from diagnostic biopsies were lower in the premature group than the control group (2.71 vs. 3.53, P = 0.043) indicating less fibrosis but this equalized by time of subsequent KPE (P = 0.17). Primary surgery was Kasai portoenterostomy (n = 20) at an older median age than controls (65 vs. 56 days; P = 0.06). Liver transplantation was the primary procedure in one late-presenting child. There was an increased but non-significant clearance of jaundice in the PBA group [n = 12/20 (60%) vs 20/41 (48%); P = 0.23] post-KPE. Native liver survival and true survival were not different (P = 0.58 and 0.23).ConclusionsPBA infants have similar outcomes to term infants, despite delayed diagnosis and higher frequency of the syndromic form. The high incidence of discordant twins supports the theory that epigenetic modifications could contribute to the pathogenesis of BA.Level of evidenceIIIc Retrospective Matched Cohort Study.

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Preterm Infants With Biliary Atresia

Cited by 29 publications

References 29 publications

The Epidemiology and Outcome of Biliary Atresia: Saudi Arabian National Study (2000–2018)

The Epidemiology and Outcome of Biliary Atresia: Saudi Arabian National Study (2000–2018)

Application of Nonhuman Primate Models in the Studies of Pediatric Anesthesia Neurotoxicity

Prematurity and biliary atresia: a 30-year observational study

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