Health-Related Quality of Life in Biliary Atresia Patients with Native Liver or Transplantation

Introduction We aimed to assess health-related quality of life (HrQoL) in biliary atresia (BA) patients, based on original data and a literature review, and to determine factors associated with their HrQoL. Materials and Methods We reviewed available studies describing HrQoL in BA patients. We assessed HrQoL in Dutch BA patients (6-16 years) using the validated Child Health Questionnaire. We compared HrQoL scores in BA patients with healthy peers and with children who had undergone major surgery in infancy or children with chronic conditions. We determined the relationship between specific patient-related factors and HrQoL. Results Literature data indicated that HrQoL in children with BA is lower than in healthy peers. In Dutch BA patients (n = 38; age 10 ± 3 years), parent-proxy physical HrQoL (48 ± 11) was significantly lower compared with two reference groups of healthy peers (59 ± 4 and 56 ± 6, respectively, each p < 0.001), and lower than in children with attention deficit hyperactivity disorder (60 ± 5), asthma (54 ± 6), attending a cardiology clinic (52 ± n / r), congenital diaphragmatic hernia (53 ± 7) or D-transposition of the great arteries (54 ± 6; all p < 0.05). Psychosocial HrQoL (50 ± 9) was lower than in healthy peers (54 ± 6, p = 0.02, and 53 ± 6, p = 0.07) and children with asthma (54 ± 6, p = 0.02), and largely comparable to children with other chronic conditions. Parent-proxy physical HrQoL was adversely related to adverse medical event in the past year, special education, and motor impairments; psychosocial HrQoL was adversely related to behavioral problems. Conclusion Children with BA are at risk of impaired HrQoL. Special attention is warranted for children with adverse medical events and special education

Long-Term Neurodevelopmental Outcomes in Children with Biliary Atresia

Objective: To assess long-term neurodevelopmental outcomes in school-aged children with biliary atresia. Study design: All Dutch children (6-12 years of age) diagnosed with biliary atresia were invited to participate in this study. We used validated neurodevelopmental tests to assess motor skills and cognition, and questionnaires to assess behavior. Scores were compared with the Dutch norm population, by means of 1-sample tests. Results are given as number and percentage or mean ± SD. Results: We included 46 children, with a median age of 11 years (range, 6-13 years); 36 children had undergone a liver transplantation (78%). Twelve children (26%) received special education (vs 2.4% in the norm population; P <.01). Motor outcomes were significantly affected compared with the norm population (P <.01), with 25% normal (vs 85%), 25% borderline (vs 10%), and 50% low scores (vs 5%). Total IQ was lower in patients with biliary atresia, compared with the norm population (91 ± 18 vs 100 ± 15; P <.01). There were no significant differences in test scores between children with native liver and after liver transplantation. Conclusions: School-aged children with biliary atresia show neurodevelopmental impairments compared with the norm population, especially in motor skills. Our data strongly warrant evaluation of neurodevelopmental intervention programs to assess whether long-term outcomes could be improved

Surgical Outcome of Children with a Malignant Liver Tumour in The Netherlands:A Retrospective Consecutive Cohort Study

INTRODUCTION: Six to eight children are diagnosed with a malignant liver tumour yearly in the Netherlands. The majority of these tumours are hepatoblastoma (HB) and hepatocellular carcinoma (HCC), for which radical resection, often in combination with chemotherapy, is the only curative treatment option. We investigated the surgical outcome of children with a malignant liver tumour in a consecutive cohort in the Netherlands. METHODS: In this nationwide, retrospective observational study, all patients (age &lt; 18 years) diagnosed with a malignant liver tumour, who underwent partial liver resection or orthotopic liver transplantation (OLT) between January 2014 and April 2021, were included. Children with a malignant liver tumour who were not eligible for surgery were excluded from the analysis. Data regarding tumour characteristics, diagnostics, treatment, complications and survival were collected. Outcomes included major complications (Clavien-Dindo ≥ 3a) within 90 days and disease-free survival. The results of the HB group were compared to those of a historical HB cohort. RESULTS: Twenty-six children were analysed, of whom fourteen (54%) with HB (median age 21.5 months), ten (38%) with HCC (median age 140 months) and one with sarcoma and a CNSET. Thirteen children with HB (93%) and three children with HCC (30%) received neoadjuvant chemotherapy. Partial hepatic resection was possible in 19 patients (12 HB, 6 HCC, and 1 sarcoma), whilst 7 children required OLT (2 HB, 4 HCC, and 1 CNSET). Radical resection (R0, margin ≥ 1.0 mm) was obtained in 24 out of 26 patients, with recurrence only in the patient with CNSET. The mean follow-up was 39.7 months (HB 40 months, HCC 40 months). Major complications occurred in 9 out of 26 patients (35% in all, 4 of 14, 29% for HB). There was no 30- or 90-day mortality, with disease-free survival after surgery of 100% for HB and 80% for HCC, respectively. Results showed a tendency towards a better outcome compared to the historic cohort, but numbers were too small to reach significance. CONCLUSION: Survival after surgical treatment for malignant liver tumours in the Netherlands is excellent. Severe surgical complications arise in one-third of patients, but most resolve without long-term sequelae and have no impact on long-term survival

Incidence of Isolated Biliary Atresia during the COVID Lockdown in Europe: Results from a Collaborative Project by RARE-Liver

Background: Biliary atresia (BA) is a rare cholangiopathy where one of the proposed aetiological mechanisms is an infectious viral trigger. Coronavirus disease-19 (COVID) lockdown restrictions were implemented to reduce the transmission of infections. Strictness of lockdown varied across European countries. This study aimed to investigate if there was an association between strictness of lockdown and change in isolated BA (IBA) incidence in Europe. Methods: We approached European centres involved in the European Reference Network RARE-LIVER. We included IBA patients born between 2015 and June 2020. We calculated the number of IBA patients born per centre per month. The Stringency Index (SI) was used as lockdown strictness indicator. The association between percentage change of mean number of IBA patients born per month and the SI was assessed. Results: We included 412 IBA patients from thirteen different centres. The median number of patients per month did not change: 6 (1–15) pre-lockdown and 7 (6–9) during lockdown (p = 0.34). There was an inverse association between SI and percentage change in IBA (B = -0.73, p = 0.03). Median age at Kasai portoenterostomy (days) did not differ between time periods (51 (9–179) vs. 53 (19–126), p = 0.73). Conclusion: In this European study, a stricter COVID-lockdown was seemingly accompanied by a simultaneous larger decrease in the number of IBA patients born per month in the lockdown. Results should be interpreted with caution due to the assumptions and limitations of the analysis

The Early Motor Repertoire of Children Born Preterm Is Associated With Intelligence at School Age

OBJECTIVE: The goal was to determine whether the quality of general movements (GMs) for preterm children had predictive value for cognitive development at school age. METHODS: In this prospective cohort study, 60 preterm infants (gestational age, median: 30.0 weeks [range: 25-33 weeks]; birth weight, median: 1130 g [range: 595-1800 g]) without cerebral palsy were studied. The quality of GMs was assessed prospectively as normal or abnormal, from video recordings that were made at regular intervals until 17 weeks after term. At 7 to 11 years, intelligence was tested by using the Wechsler Intelligence Scale for Children III, Dutch version. Total IQ (TIQ), verbal IQ (VIQ), and performance IQ (PIQ) scores were calculated. RESULTS: The median TIQ was 93 (range: 67-113), VIQ 96 (range: 68117), and PIQ 92 (range: 65-119). Fifteen children (25%) had low TIQ scores ( CONCLUSIONS: The quality of GMs during the early postterm period is a marker for intelligence at school age. Abnormal GMs during the early postterm period may reflect injury or developmental disruptions of brain areas involved in cognitive development. Pediatrics 2010; 125: e1356-e136

Early motor repertoire is related to level of self-mobility in children with cerebral palsy at school age

Aim To determine the predictive value of the early motor repertoire for the level of self-mobility in children with cerebral palsy (CP) at school age. Method Video recordings were made at 11 to 17 weeks post-term of 37 preterm infants (20 males, 17 females) who later developed CP. The early motor repertoire was assessed by obtaining a motor optimality score. At 6 to 12 years, children were classified according to the Gross Motor Function Classification System (GMFCS). Results Of 37 children (mean gestational age 29.1wks, SD 1.9; mean birthweight 1273g, SD 324), nine had unilateral and 28 had bilateral spastic CP. Twelve children were in GMFCS level I, three level II, 10 level III, four level IV, and eight level V. The absence of the age-adequate motor repertoire, a cramped motor repertoire, an abnormal kicking pattern, and a non-flat supine posture were associated with lower levels of self-mobility (KH2 for trend test, p <0.05). Predictive for a low level of self-mobility was a cramped motor repertoire/non-flat supine posture (positive predictive values [PPV] 100%, negative predictive values [NPV] 54%). Predictive for a high level of self-mobility was a non-cramped repertoire/flat supine posture (PPV 80%, NPV 74%). Interpretation Several aspects of the motor repertoire at 11 to 17 weeks post-term predicted the degree of functional limitations in children with CP at school age

The amplitude of the Achilles tendon reflex in infants is related to body position

In this study, we investigated whether the Achilles tendon reflex (ATR) in healthy infants is modulated by changes in body position (prone vs. supine). The amplitude of the ATR was compared at postnatal day 1, months 2, 3 and 6, while infants were placed in prone and supine position. The ATR was considered "positive'' when at least 1 of 20 tendon taps evoked a visible contraction of the ankle plantar flexor muscles. At 6 months, the amplitude and latency of the ATR were evaluated by electromyographic (EMG) recordings of the (lateral and medial.) gastrocnemius muscle, with the infant in prone and supine position. At postnatal day 1, the clinical ATR response was more frequently present in prone than supine position (14/16 or 88% vs. 4/16 or 25%, respectively, p <0.01). From 2 months onwards, the frequencies of positive ATRs were no longer significantly different between prone (14/14 or 100%) and supine position (10/14 or 67%). However, based on the EMG recordings at 6 months, the ATR amplitude was higher in prone compared to supine position (n=8, median 5.5 mV, range 4.7-6.6 mV; and median 3.8 mV, range 2.3-5.6 mV, p <0.05). We conclude that the ATR response in healthy infants is more pronounced in prone than in supine position, either when clinically (visually) assessed during routine tendon tapping or when neurophysiologically assessed during surface EMG recording. (c) 2006 Elsevier Ireland Ltd. All rights reserved

Quantitative aspects of the early motor repertoire in preterm infants: Do they predict minor neurological dysfunction at school age?

Background: Qualitative aspects of the motor repertoire, at 11-16 weeks post-term are predictive for minor neurological dysfunction (MND) at 7 to 11 years of age. Predictive value of quantitative aspects is unknown so far. Aim: To investigate whether quantitative aspects of the motor repertoire between 6 and 24 weeks post-term also have predictive value for neurological outcome at 7 to 11 years of age. Study design: Prospective cohort study. Subjects: Preterm infants from whom several quantitative aspects of the motor repertoire were assessed between 6 and 24 weeks post-term. Outcome measures: Neurological outcome at 7-11 years of age was assessed according to Touwens' neurological examination. Children were classified as neurologically normal, or as having complex MND or cerebral palsy (CP). Results: Eighty-two children were included. At 7 to 11 years of age 15 children (18%) had developed CP, 49 (60%) were neurologically normal, and 18 (22%) had MND. Multiple logistic regression analysis showed that, when the qualitative aspects of the motor repertoire known to predict neurological outcome were taken into account, only the asymmetric tonic neck (ATN) posture provided additional predictive value. In case of normal fidgety movements (FMs) accompanied by an abnormal concurrent motor repertoire, the presence of an obligatory ATN increased the risk for developing complex MND to 75%; absence of an obligatory ATN reduced the risk to 15% (p Conclusions: Quantitative aspects of the motor repertoire at 11-16 weeks post-term, in particular the presence of an obligatory ATN posture, contribute to the prediction of neurological outcome at 7 to 11 years of age. (C) 2008 Elsevier Ireland Ltd. All rights reserved

Current Role of Imaging in Diagnosing Aortic Graft Infections

Vascular prosthetic graft infection is a rare but serious complication after aortic graft replacement, with high morbidity and mortality rates. Therefore, adequate diagnostics are needed to detect and treat these infections as early as possible. Several imaging modalities provide different diagnostic values for detecting prosthetic graft infection. Previous studies reported on the diagnostic value of ultrasound, computed tomography imaging, magnetic resonance imaging, fluorodeoxyglucose-positron emission tomography, and single-photon emission computed tomography. In addition, adjunctive studies on new and promising techniques to detect prosthetic graft infection (eg, bio-optical imaging and target imaging with nuclear techniques) have also been investigated. This review provides a summary of noninvasive imaging modalities and their diagnostic values in order to evaluate and treat possible vascular graft infections as early as possible. Semin Vasc Surg 24:182-190 (C) 2011 Elsevier Inc. All rights reserved