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1.
Early Hum Dev ; 180: 105754, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-37030125

RESUMO

AIM: To assess neurodevelopment in young patients with biliary atresia (BA) and to determine the predictive value of General Movement Assessment (GMA) at infant age for neurodevelopmental impairments at toddler age. METHOD: Infants diagnosed with BA were prospectively included in a longitudinal study. Neurodevelopmental status was previously assessed before Kasai porto-enterostomy (KPE) and one month after KPE using Prechtl's GMA, including motor optimality scores. At 2-3 years, neurodevelopment was assessed using the Bayley Scales of Infant Development, and compared to the Dutch norm population. The predictive value of GMA at infant age for motor skills and cognition at toddler age was determined. RESULTS: Neurodevelopment was assessed in 41 BA patients. At toddler age (n = 38, age 29 ± 5 months, 70 % liver transplantation), 13 (39 %) patients scored below-average on motor skills, and 6 (17 %) patients on cognition. Abnormal GMA after KPE predicted both below-average motor skills and cognitive score at toddler age (sensitivity, 91 % and 80 %; specificity 83 % and 67 %; negative predictive value, 94 % and 94 %; and, positive predictive value, 77 % and 33 %, resp.). INTERPRETATION: One-third of toddlers with BA show impaired motor skills. GMA post-KPE has a high predictive value to identify infants with BA at risk of neurodevelopmental impairments.


Assuntos
Atresia Biliar , Transplante de Fígado , Lactente , Humanos , Pré-Escolar , Atresia Biliar/diagnóstico , Atresia Biliar/cirurgia , Estudos Longitudinais , Destreza Motora , Movimento
2.
J Clin Med ; 12(3)2023 Jan 18.
Artigo em Inglês | MEDLINE | ID: mdl-36769422

RESUMO

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.

3.
J Int Neuropsychol Soc ; 29(1): 80-91, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-34974853

RESUMO

OBJECTIVE: Preterm birth poses a risk to cognition during childhood. The resulting cognitive problems may persist into young adulthood. The early motor repertoire in infancy is predictive of neurocognitive development in childhood. Our present aim was to investigate whether it also predicts neurocognitive status in young adulthood. METHOD: We conducted an explorative observational follow-up study in 37 young adults born at a gestational age of less than 35 weeks and/or with a birth weight below 1200 g. Between 1992 and 1997, these individuals were videotaped up until 3 months' corrected age to assess the quality of their early motor repertoire according to Prechtl. The assessment includes general movements, fidgety movements (FMs), and a motor optimality score (MOS). In young adulthood, the following cognitive domains were assessed: memory, speed of information processing, language, attention, and executive function. RESULTS: Participants in whom FMs were absent in infancy obtained lower scores on memory, speed of information processing, and attention than those with normal FMs. Participants with aberrant FMs, that is, absent or abnormal, obtained poorer scores on memory, speed of information processing speed, attention, and executive function compared to peers who had normal FMs. A higher MOS was associated with better executive function. CONCLUSIONS: The quality of the early motor repertoire is associated with performance in various cognitive domains in young adulthood. This knowledge may be applied to enable the timely recognition of preterm-born individuals at risk of cognitive dysfunctions.


Assuntos
Nascimento Prematuro , Adulto , Feminino , Humanos , Lactente , Recém-Nascido , Adulto Jovem , Peso ao Nascer , Cognição , Seguimentos , Movimento
4.
Children (Basel) ; 9(4)2022 Apr 07.
Artigo em Inglês | MEDLINE | ID: mdl-35455569

RESUMO

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 < 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.

5.
Ann Surg ; 275(2): e503-e510, 2022 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-32324690

RESUMO

OBJECTIVE: To assess whether regional intestinal oxygen saturation (rintSO2) and regional cerebral oxygen saturation (rcSO2) measurements aid in estimating survival of preterm infants after surgery for NEC. SUMMARY OF BACKGROUND DATA: Predicting survival after surgery for NEC is difficult yet of the utmost importance for counseling parents. METHODS: We retrospectively studied prospectively collected data of preterm infants with surgical NEC who had available rintSO2 and rcSO2 values measured via near-infrared spectroscopy 0-24 hours preoperatively. We calculated mean rintSO2 and rcSO2 for 60-120 minutes for each infant. We analyzed whether preoperative rintSO2 and rcSO2 differed between survivors and non-survivors, determined cut-off points, and assessed the added value to clinical variables. RESULTS: We included 22 infants, median gestational age 26.9 weeks [interquartile range (IQR): 26.3-28.4], median birth weight 1088 g [IQR: 730-1178]. Eleven infants died postoperatively. Preoperative rintSO2, but not rcSO2, was higher in survivors than in non-survivors [median: 63% (IQR: 42-68) vs 29% (IQR: 21-43), P < 0.01), with odds ratio for survival 4.1 (95% confidence interval, 1.2-13.9, P = 0.02) per 10% higher rintSO2. All infants with rintSO2 values of >53% survived, whereas all infants with rintSO2 <35% died. Median C-reactive protein [138 mg/L (IQR: 83-179) vs 73 mg/L (IQR: 12-98), P < 0.01), lactate [1.1 mmol/L (IQR: 1.0-1.6) vs 4.6 mmol/L (IQR: 2.8-8.0), P < 0.01], and fraction of inspired oxygen [25% (IQR: 21-31) vs 42% (IQR: 30-80), P < 0.01] differed between survivors and non-survivors. Only rintSO2 remained significant in the multiple regression model. CONCLUSIONS: Measuring rintSO2, but not rcSO2, seems of added value to clinical variables in estimating survival of preterm infants after surgery for NEC. This may help clinicians in deciding whether surgery is feasible and to better counsel parents about their infants' chances of survival.


Assuntos
Encéfalo/metabolismo , Enterocolite Necrosante/cirurgia , Intestinos/metabolismo , Oxigênio/metabolismo , Estudos de Coortes , Enterocolite Necrosante/mortalidade , Feminino , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Masculino , Estudos Retrospectivos , Taxa de Sobrevida
7.
J Pediatr Surg ; 57(4): 649-654, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-34210523

RESUMO

PURPOSE: To determine anxiety, stress, and quality of life (QoL) in parents of children who are diagnosed with biliary atresia (BA). METHODS: Parents of BA patients (0-3 years) completed validated questionnaires at three time points: at first hospitalization (T0); 1-2 months post diagnosis (T1); and 2-3 years post diagnosis (T2). Results are presented in medians (min-max). RESULTS: We included 52 parents (age 31 [24-51 y], 31 females) of 30 BA patients. In fathers, neither anxiety nor stress levels significantly differed from reference values. Mothers reported significantly higher anxiety levels compared to reference values (T0: 48 vs 35, p = 0.001; T1: 43 vs 35, p = 0.03; T2: 37 vs 35, p = 0.04), which significantly decreased over time (-23% between T0 and T2: p = 0.04). Stress in mothers was significantly higher at T1 than at T2 (+35%, p = 0.02), but was not significantly different from reference values at each time point (T0: 17 vs 14, p = 0.07; T1: 18 vs 14, p = 0.09; T2: 13 vs 14, p = 0.52).The overall QoL in mothers and fathers was rather unaffected. CONCLUSIONS: Particularly mothers of infants diagnosed with BA report high anxiety levels up to three years after diagnosis. The overall QoL of parents is rather unaffected after diagnosing BA in their child. LEVEL OF EVIDENCE: Level 2.


Assuntos
Atresia Biliar , Qualidade de Vida , Adulto , Ansiedade/diagnóstico , Ansiedade/etiologia , Atresia Biliar/diagnóstico , Atresia Biliar/cirurgia , Criança , Feminino , Humanos , Lactente , Pais , Estudos Prospectivos
8.
J Pediatr Gastroenterol Nutr ; 72(4): 592-596, 2021 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-33346571

RESUMO

OBJECTIVES: The aim of the study was to assess the neurological status in infants with biliary atresia (BA) at time of diagnosis, using Prechtl's validated General Movement Assessment. METHODS: Infants diagnosed with BA were prospectively included in a nationwide cohort study. From birth to approximately 46 weeks of postmenstrual age (PMA), general movements (GMs) are defined as "writhing movements." At 46 to 49 weeks PMA, "'fidgety movements" emerge. The infant's early motor repertoire was recorded on video before Kasai portoenterostomy. We scored GM optimality scores (min-max 5-42) or motor optimality scores (MOS, min-max 5-28) as appropriate. We defined GM optimality scores <36 and MOS <26 as atypical, and compared the results with 2 reference groups of healthy peers. RESULTS: We assessed GMs in 35 infants with BA (11/35 boys, gestational age 40 weeks [36-42], birth weight 3370 g [2015-4285]). At time of diagnosis (PMA 47 weeks [42-60]), 16 infants (46%) showed atypical GMs. The proportion of infants with atypical GMs was significantly higher in BA (46%) than in 2 reference groups of healthy infants (vs 10%, P < 0.001; vs 18%, P < 0.001). Total and direct bilirubin levels were 165 µmol/L (87-364) and 134 µmol/L (72-334), respectively, height z score was 0.05 (-2.90, 1.75), weight z score -0.52 ([-2.50, -0.20) and mean upper arm circumference z score -1.80 (-2.50, -0.20). We found no statistically significant relation between atypical GMs and clinical variables. CONCLUSIONS: Almost half of the infants with BA showed atypical GMs at time of diagnosis, suggesting neurological impairment. Close monitoring of these infants is warranted to determine their individual neurodevelopmental trajectories.


Assuntos
Atresia Biliar , Atresia Biliar/diagnóstico , Pré-Escolar , Estudos de Coortes , Idade Gestacional , Humanos , Lactente , Masculino , Movimento , Estudos Prospectivos
9.
J Pediatr Gastroenterol Nutr ; 72(2): 202-209, 2021 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-32833894

RESUMO

OBJECTIVES: Environmental factors may be involved in the pathogenesis of biliary atresia (BA). This epidemiological study aimed to analyze the relationships between the incidence of BA, the incidence of confirmed viral or bacterial infections and population density, and geographical and temporal clustering of BA in the Netherlands. STUDY DESIGN: Correlations between the monthly incidence of BA and the number of confirmed infections were assessed. BA incidence per province was calculated and compared to the province with highest population density. Birthplaces were classified as rural or urban. Temporal clustering of month of birth and month of conception were analyzed. We performed analyses for isolated BA (IBA) and syndromic BA (SBA) separately. Chi2, logistic regression, and Walter and Elwood test were used. RESULTS: A total of 262 IBA and 49 SBA patients, born between 1987 and 2018, were included. IBA incidence correlated to the number of confirmed infections of, for example, Chlamydia trachomatis (R = 0.14; P = 0.02) and adenovirus (R = 0.22; P = 0.005). We observed a higher incidence of IBA (0.75/10,000; odds ratio [OR] = 1.86; P = 0.04) and SBA (0.27/10,000; OR = 6.91; P = 0.001) in Groningen and a higher incidence of SBA in Gelderland (0.13/10,000; OR = 3.35; P = 0.03). IBA incidence was 68% higher in rural (0.67/10,000) versus urban areas (0.40/10,000) (P = 0.02). The estimated month of conception of patients with SBA clustered in November (85% increase compared to average SBA incidence [0.09/10,000; P = 0.04]). CONCLUSIONS: IBA incidence correlated weakly with national confirmed infections. IBA and SBA incidence varied geographically in the Netherlands. IBA incidence was higher in rural than in urban areas, which may be explained decreased exposure to pathogens. Our results provide support for a role of environmental factors in the pathogenesis of IBA.


Assuntos
Atresia Biliar , Atresia Biliar/epidemiologia , Atresia Biliar/etiologia , Estudos Epidemiológicos , Humanos , Incidência , Países Baixos/epidemiologia , População Rural
10.
J Pediatr Gastroenterol Nutr ; 71(5): 641-646, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-33093371

RESUMO

OBJECTIVES: The aim of the study was to determine quality of life (QoL), stress, and anxiety levels in parents of children with biliary atresia (BA), and to assess factors associated with parental QoL. METHODS: Parents of children (6-16 years) with BA were included in this cross-sectional study. We used validated questionnaires to assess parental QoL, stress, and anxiety levels. We compared the results with reference data from the general population and determined associated factors using generalized linear mixed model analysis. Results are given as mean ±â€ŠSD or median [min-max]. RESULTS: We included 61 parents of 39 children (aged 11 ±â€Š3 years). Thirty-one children (79%) had undergone a liver transplantation (LTx). Parents reported reduced family activities (88 [8-100] vs 95 [30-100], P = 0.002) and more emotional worry (83 [17-100] vs 92 [95-100], P < 0.001) compared with reference data, but a stronger family cohesion (85 [30-100] vs 60 [30-100], P = 0.05). Scores on parental QoL, anxiety and stress were similar to reference data. Fathers (16.0 [11-19]) and mothers (15.4 ±â€Š1.4) scored higher on the psychological domain compared with reference data (vs 14.7 ±â€Š2.2, P < 0.01). There was no significant difference in QoL of parents with children with native liver or those who had undergone LTx. Older age and high anxiety trait in parents were adversely associated with physical QoL. Household income below &OV0556;35 000/year and high anxiety trait were adversely associated with environmental QoL. CONCLUSIONS: QoL in parents of school-aged children with BA appears to be unaffected. Parents with high-anxiety personality trait, older age, and low household income are at increased risk of impaired QoL.


Assuntos
Atresia Biliar , Qualidade de Vida , Idoso , Ansiedade/epidemiologia , Ansiedade/etiologia , Atresia Biliar/cirurgia , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Pais , Inquéritos e Questionários
11.
J Pediatr Gastroenterol Nutr ; 71(4): 440-445, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32694399

RESUMO

OBJECTIVES: Biliary atresia (BA) causes neonatal cholestasis that requires hepatoportoenterostomy or liver transplantation (LT) for long-term survival. Nutritional optimization is necessary as sarcopenia and sarcopenic obesity have been associated with adverse clinical outcome. Currently, mid upper arm circumference (MUAC) is considered the most accurate indicator. The aim of the study was to determine computed tomography (CT)-based body metrics in infants with BA and to evaluate its correlation with MUAC. METHODS: We retrospectively analyzed all BA infants below 2 years of age who underwent CT as part of LT screening at our hospital between 2006 and 2019. Measured variables were indexed with length and included: MUAC, total psoas muscle surface area (tPMSA), cross-sectional skeletal muscle area (CSMA), and total abdominal fat area. Intraclass correlation coefficients and Pearson coefficients were calculated. CSMA-to-abdominal fat area ratio was divided in quartiles, the lowest quartile group was considered sarcopenic obese. RESULTS: Eighty infants with a median age of 4.6 months at LT screening were included. Intraclass correlation coefficients were: tPMSA = 0.94, CSMA = 0.92, and total abdominal fat area = 0.99. Correlation between MUAC z-score and indices of tPMSA, CSMA, and total abdominal fat area were r = 0.02, r = 0.06, and r = 0.43, respectively. The cut-off for sarcopenic obesity was CSMA-to-abdominal fat area ratio below 0.93. CONCLUSIONS: In BA infants, it is possible to determine CT-based body metrics during LT screening with very strong interobserver agreement. Poor correlation between CT-based body metrics and MUAC suggests that CT-based body metrics provide additional information on body composition in BA infants, such as relative muscle mass.


Assuntos
Atresia Biliar , Braço , Benchmarking , Atresia Biliar/complicações , Atresia Biliar/diagnóstico por imagem , Composição Corporal , Estudos Transversais , Humanos , Lactente , Recém-Nascido , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
12.
Eur J Pediatr Surg ; 30(3): 261-272, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32629498

RESUMO

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.


Assuntos
Atresia Biliar/psicologia , Qualidade de Vida , Adolescente , Atresia Biliar/cirurgia , Estudos de Casos e Controles , Criança , Feminino , Humanos , Transplante de Fígado/efeitos adversos , Transplante de Fígado/psicologia , Masculino , Pais/psicologia , Estudos Prospectivos , Autorrelato
13.
J Pediatr ; 217: 118-124.e3, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31831162

RESUMO

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.


Assuntos
Atresia Biliar/complicações , Cognição/fisiologia , Destreza Motora/fisiologia , Transtornos do Neurodesenvolvimento/etiologia , Adolescente , Atresia Biliar/cirurgia , Criança , Feminino , Seguimentos , Humanos , Incidência , Transplante de Fígado , Masculino , Países Baixos/epidemiologia , Transtornos do Neurodesenvolvimento/epidemiologia , Transtornos do Neurodesenvolvimento/psicologia , Prognóstico , Fatores de Tempo
14.
J Pediatr Gastroenterol Nutr ; 67(6): 689-694, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30095577

RESUMO

OBJECTIVES: The aim of the study is to determine the prognosis of patients with biliary atresia after 2 years of native liver survival (NLS) and to identify prognostic factors for continued NLS after 2 years of age. METHODS: We retrospectively analyzed perioperative, laboratory, and outcome parameters of all biliary atresia patients in The Netherlands between January 1987 and June 2015 with NLS of at least 2 years. We compared parameters between patients who continued to have their native liver (NLS+) to those who did not, either by transplant or death (NLS-). RESULTS: We included 100 patients. Upon a median follow-up of 16.4 years, NLS ended in 37% by liver transplantation (LTx) and in 6% by (pre-transplant) mortality. NLS rates at 5, 10, 15, 18 years of age were 89%, 72%, 60%, 54%, respectively. Corresponding overall survival rates were 98%, 90%, 87%, 87%, respectively. Six months post-Kasai, NLS+ patients had higher clearance of jaundice (COJ) rate, significantly lower total and direct serum bilirubin, aspartate-aminotransferase and alkaline phosphatase levels, compared with NLS- patients (each P < 0.05). Cox regression could only assess a significant effect of COJ on continued NLS. Main indications for LTx after the age of 2 were irreversible jaundice and portal hypertension. CONCLUSIONS: Eighty-seven percent of patients with 2-year NLS reach adult age and more than 50% with their native liver. A pre-transplant mortality of 6%, however, exists among patients who reach the age of 2 years with their native livers. Early life parameters, other than COJ, did not have a significant effect on continued NLS after 2 years of age.


Assuntos
Atresia Biliar/mortalidade , Portoenterostomia Hepática/mortalidade , Adolescente , Adulto , Atresia Biliar/fisiopatologia , Atresia Biliar/cirurgia , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Seguimentos , Humanos , Fígado/fisiopatologia , Masculino , Países Baixos , Prognóstico , Sistema de Registros , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Adulto Jovem
15.
J Pediatr Gastroenterol Nutr ; 67(2): 157-168, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29601439

RESUMO

OBJECTIVE: The aim of the study was to determine the neurodevelopmental outcomes of children with liver diseases based on a systematical review of the literature. METHOD: A literature search according to the PRISMA statement was conducted using predefined search terms in PubMed, Cochrane Library, and PsycINFO. The inclusion criterion was studies published from 2000 onwards that reported on the neurodevelopmental outcomes of term-born children with liver diseases. A narrative synthesis was done to appraise the studies. RESULTS: Twenty-five studies were included (1913 children), 19 of which described children after liver transplantation (LTx; 1372 children). Sixty-seven percentage of the studies on children with liver diseases who survived with their native livers showed low-average or abnormal scores on specific subscales of cognitive and behavioral measures. In studies on children after LTx, this was 82%. After LTx, 83% of studies demonstrated impaired outcomes on behavior, whereas 42% of children received special education. Motor development was impaired in 82% of studies in children with native liver and after LTx. LIMITATIONS: Studies were heterogenic because of sample sizes, etiology of liver disease and type of assessment tools used. CONCLUSIONS: More than two-third of included studies showed neurodevelopmental deficits in children with liver diseases, affecting all neurodevelopmental areas. Knowledge on risk factors for impaired neurodevelopment is limited and lack of long-term follow-up is worrying, especially considering the increasing survival rates, resulting in more at-risk patients. Studying early predictors and risk factors of abnormal developmental trajectories of children with liver diseases is indicated to assess strategies to improve their long-term neurodevelopmental outcomes.


Assuntos
Deficiências do Desenvolvimento/complicações , Hepatopatias/complicações , Sistema Nervoso/crescimento & desenvolvimento , Criança , Humanos
16.
Semin Vasc Surg ; 24(4): 182-90, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22230672

RESUMO

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.


Assuntos
Aorta/cirurgia , Implante de Prótese Vascular/efeitos adversos , Prótese Vascular/efeitos adversos , Infecção Hospitalar/diagnóstico , Diagnóstico por Imagem , Infecções Relacionadas à Prótese/diagnóstico , Implante de Prótese Vascular/instrumentação , Infecção Hospitalar/etiologia , Infecção Hospitalar/terapia , Diagnóstico por Imagem/métodos , Humanos , Valor Preditivo dos Testes , Prognóstico , Infecções Relacionadas à Prótese/etiologia , Infecções Relacionadas à Prótese/terapia
17.
Pediatrics ; 126(5): e1134-42, 2010 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-20921067

RESUMO

OBJECTIVE: To determine the stability of the scores obtained on tests of motor development from birth until school age in healthy, term singletons and to determine if early motor scores are associated with more complex cognitive functions at school age, such as attention and memory. PATIENTS AND METHODS: This longitudinal, prospective cohort study included 77 infants. The motor development of these infants was assessed during the neonatal period with Prechtl's neurologic examination; in early infancy with Touwen's neurologic examination and general movement assessment; at toddler age with Hempel's neurologic examination and the Psychomotor Developmental Index from the Bayley Scales of Infant Development; and at school age with the Movement Assessment Battery for Children. Cognition was determined at toddler age with the Mental Developmental Index from the Bayley Scales of Infant Development; and at school age with an intelligence test and attention and memory tests. RESULTS: The mean absolute difference in standardized motor scores for all time points was 1.01 SD (95% confidence interval: 0.91-1.11). Only the explained proportions of variance of maternal socioeconomic status and verbal intelligence were significant for sustained attention and verbal memory (r(2) = 0.104, P = .030 and r(2) = 0.074, P = .027), respectively. The children's scores on early motor tests added little value for their motor and cognitive development at school age. CONCLUSIONS: In healthy children the stability of motor development from birth until school age is low. Maternal socioeconomic status and verbal intelligence rather than the infants' scores on early motor tests signified added value for complex cognitive functions at school age.


Assuntos
Atenção , Desenvolvimento Infantil , Memória , Destreza Motora , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Inteligência , Estudos Longitudinais , Masculino , Países Baixos , Gravidez , Valores de Referência , Fatores Socioeconômicos , Estatística como Assunto , Aprendizagem Verbal
18.
Pediatrics ; 125(6): e1356-63, 2010 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-20457678

RESUMO

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: 68-117), and PIQ 92 (range: 65-119). Fifteen children (25%) had low TIQ scores (<85). When the quality of GMs normalized before 8 weeks after term, TIQ, VIQ, and PIQ scores were in the normal range. Consistently abnormal GMs to 8 weeks after term were associated with lower TIQ, VIQ, and PIQ scores. With correction for male gender and the educational levels of the parents, the likelihood ratio of consistently abnormal GMs for a low TIQ was 4.9 (95% confidence interval: 1.3-17.6). The model explained 22.4% of the variance. 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.


Assuntos
Recém-Nascido Prematuro/fisiologia , Destreza Motora , Escolaridade , Feminino , Humanos , Lactente , Recém-Nascido , Inteligência , Masculino , Fatores de Risco
19.
Int J Cardiovasc Imaging ; 26(1): 111-9, 2010 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-19760516

RESUMO

Many apparent healthy persons die from cardiovascular disease, despite major advances in prevention and treatment of cardiovascular disease. Traditional cardiovascular risk factors are able to predict cardiovascular events in the long run, but fail to assess current disease activity or nearby cardiovascular events. There is a clear relation between the occurrence of cardiovascular events and the presence of so-called vulnerable plaques. These vulnerable plaques are characterized by active inflammation, a thin cap and a large lipid pool. Spectroscopy is an optical imaging technique which depicts the interaction between light and tissues, and thereby shows the biochemical composition of tissues. In recent years, impressive advances have been made in spectroscopy technology and intravascular spectroscopy is able to assess the composition of plaques of interest and thereby to identify and actually quantify plaque vulnerability. This review summarizes the current evidence for spectroscopy as a measure of plaque vulnerability and discusses the potential role of intravascular spectroscopic imaging techniques.


Assuntos
Aterosclerose/diagnóstico , Diagnóstico por Imagem , Diagnóstico por Imagem/métodos , Humanos , Valor Preditivo dos Testes , Prognóstico , Ruptura , Espectrometria de Fluorescência , Espectroscopia de Luz Próxima ao Infravermelho , Análise Espectral Raman
20.
Early Hum Dev ; 85(1): 25-36, 2009 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-18691834

RESUMO

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<0.05). 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.


Assuntos
Paralisia Cerebral/fisiopatologia , Recém-Nascido Prematuro/fisiologia , Atividade Motora , Sistema Nervoso/fisiopatologia , Estudos de Casos e Controles , Criança , Estudos de Coortes , Feminino , Humanos , Recém-Nascido , Masculino , Postura , Reprodutibilidade dos Testes
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