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1 -Feil syndrome, renal agenesis or esophageal atresia.
2 mild pulmonary stenosis to severe pulmonary atresia.
3 bile ducts of mice with experimental biliary atresia.
4 ng sphincter-of-Oddi dysfunction and biliary atresia.
5 ion, showed a limited effect on ovulation or atresia.
6 rapid increase of macrophages and a surge of atresia.
7 ious adverse events in children with biliary atresia.
8 before the surge of macrophage migration and atresia.
9 mimicking the defects associated with canal atresia.
10 coscopic to thoracotomy repair of esophageal atresia.
11 esis and possible therapy for infant biliary atresia.
12 6 years (IQR 0.58-3.0) and 64.3% had biliary atresia.
13 amprey thrives despite developmental biliary atresia.
14 role in pathogenesis in experimental biliary atresia.
15 thout tracheo-esophageal fistula or tracheal atresia.
16 and embryos predominantly develop pulmonary atresia.
17 drives the phenotype of experimental biliary atresia.
18 ion to primary biliary cirrhosis and biliary atresia.
19 mice a partial rupture, resulting in choanal atresia.
20 ith isolated SMCP and/or unexplained choanal atresia.
21 mune response in the pathogenesis of biliary atresia.
22 ion of apoptosis in a mouse model of biliary atresia.
23 have furthered our understanding of biliary atresia.
24 in the pathogenesis of experimental biliary atresia.
25 ant clinical problem in infants with biliary atresia.
26 tion, or to die by programmed cell death, or atresia.
27 ai portoenterostomy for treatment of biliary atresia.
28 mice using an experimental model of biliary atresia.
29 omes, screening, and pathogenesis of biliary atresia.
30 ntributing factor in the etiology of biliary atresia.
31 bility to induce the murine model of biliary atresia.
32 es, and structural proteins at all phases of atresia.
33 jury and obstruction in experimental biliary atresia.
34 ogy of Fallot with complete pulmonary artery atresia.
35 heds light on the underlying causes of canal atresia.
36 of kidney and urinary tract) and esophageal atresia.
37 might be developed for treatment of biliary atresia.
38 ices (EV) in infants with congenital biliary atresia.
39 g thoracoscopy to thoracotomy for esophageal atresia.
40 , resulting in an attenuated form of biliary atresia.
41 e surrounding follicular cells did not begin atresia.
42 the early pathogenesis of congenital biliary atresia.
43 ng laryngeal atresia and the other, tracheal atresia.
44 e balance between follicular development and atresia.
45 s after liver transplantation due to biliary atresia.
47 logy of Fallot (15 patients, 25%), tricuspid atresia (12 patients, 20%), Ebstein's anomaly (4 patient
49 dence interval, 45.8 to 64.4); and tricuspid atresia, 74.6% (95% confidence interval, 62.4 to 83.4).
52 est in whether rotavirus could cause biliary atresia, an idiopathic, obliterative infantile disease o
53 congenital malformations comprising choanal atresia, anal abnormalities, post-axial polydactyly, hea
54 e for neonatal disorders such as oesophageal atresia and biliary atresia through clinical trials beca
58 litis in milder cases with severe intestinal atresia and immunodeficiency in cases with complete loss
59 rs in response to Shh knockdown is pulmonary atresia and is directly related to the abnormal developm
65 ct remnants, and peripheral blood of biliary atresia and other cholestatic disease controls were char
66 ar and humoral autoimmunity in human biliary atresia and possible interventional strategies therefore
67 formation for assessing prognosis in biliary atresia and primary biliary cirrhosis; and important cli
68 affect infants and children-such as biliary atresia and Reye's syndrome; and (3) redefinition of the
72 hat affect outcomes of patients with biliary atresia and there are no medical therapies that increase
73 virus (RRV)- induced murine model of biliary atresia and whether the T cells are sufficient to result
75 (48%) with tetralogy of Fallot and pulmonary atresia, and 4 of 54 (8%) with prosthetic valve endocard
78 ngiomas, pyriform aperture stenosis, choanal atresia, and laryngeal webs, may also have adverse effec
81 , familial intrahepatic cholestasis, biliary atresia, and primary sclerosing cholangitis, and clinica
82 sis, primary sclerosing cholangitis, biliary atresia, and progressive familial intrahepatic cholestas
85 ents of autoimmunity exist in murine biliary atresia, and the progressive bile duct injury is due in
87 curring events such as ovulation and ovarian atresia are accompanied with tissue destruction and repa
88 , primary sclerosing cholangitis and biliary atresia are thought to be immune-mediated cholangiopathi
89 ons include traversing occlusions (pulmonary atresia, arterial and venous occlusion, and iatrogenic g
90 etically programmed animal model for biliary atresia, as it loses its bile ducts and gallbladder duri
91 ent hepatoportoenterostomy (HPE) for biliary atresia at 9 U.S. pediatric centers between 1997 and 200
92 ai portoenterostomy as treatment for biliary atresia at the region's largest pediatric hepatology cen
93 omplications of portal hypoplasia in biliary atresia (BA) and acute rejection (AR) are still major co
94 ing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these p
96 ent in infants with cirrhosis due to biliary atresia (BA) as early as the time of evaluation for live
112 theory regarding the pathogenesis of biliary atresia (BA) is that bile duct injury is initiated by a
115 e, rhesus rotavirus (RRV) can induce biliary atresia (BA), a disease resulting in inflammatory obstru
117 th rhesus rotavirus (RRV) results in biliary atresia (BA), and this condition is influenced by the ho
118 iteria to define CCM associated with biliary atresia (BA), or BA-CCM, and correlate presence of BA-CC
121 asonography (US) in the diagnosis of biliary atresia (BA), with surgery as the reference standard.
125 e three main human cholangiopathies (biliary atresia [BA], primary biliary cholangitis [PBC], and pri
127 for children with spina bifida, oesophageal atresia, biliary atresia, diaphragmatic hernia, gastrosc
128 ular septum, single ventricle, and tricuspid atresia born in 1996 to 2003 were identified from the Te
129 populate the livers of infants with biliary atresia, but it is unknown whether neonatal lymphocytes
132 s with features of vertebral anomalies, anal atresia, cardiovascular anomalies, trachea-oesophageal f
133 ance to multiple diseases, including biliary atresia, choledochal cysts and gallbladder agenesis.
134 ed immunodeficiency with multiple intestinal atresias (CID-MIA) is a rare hereditary disease characte
136 d anomalies include ocular coloboma, choanal atresia, cranial nerve defects, distinctive external and
139 h spina bifida, oesophageal atresia, biliary atresia, diaphragmatic hernia, gastroschisis, and Down s
140 IM 600992), a condition including esophageal atresia (EA) and tracheoesophageal fistula (TEF), in whi
141 orted in patients after repair of esophageal atresia (EA), but the basis of this association is unkno
143 imary repair (aged <3 months) with pulmonary atresia (early-PA group; n=49), and primary repair betwe
146 sis, including organ and digit duplications, atresia, fistulas, hypertelorism, cleft palate and hamar
147 and haematocervix with cervical and vaginal atresia found on the left side (classification 1.2) with
149 operative hemodynamics and overall pulmonary atresia growth were similar, although right pulmonary ar
151 s of ocular coloboma, heart defects, choanal atresia, growth retardation, genital abnormalities, and
152 ere bilateral microphthalmia and oesophageal atresia has a de novo missense mutation, R74P, that alte
153 very of the pathogenic mechanisms of biliary atresia has been limited by the inability to study extra
155 and neonatal mice with experimental biliary atresia have shown increased expression of proapoptosis
156 , reintervention was associated with virtual atresia (hazard ratio [HR], 0.51; 95% confidence interva
157 dition is characterized by bilateral choanal atresia, hearing loss, cleft lip and/or palate, and othe
158 Relatively common conditions such as biliary atresia, however, remain largely unexplained and the vir
159 mimic of Tetralogy of Fallot with pulmonary atresia; however, subsequent reports describe only a sin
160 uctal stent were less likely to have virtual atresia (HR, 0.36; 95% CI, 0.15-0.85; P=0.02) and more l
162 neonatal conditions reported were intestinal atresia in 28 (54.9%) studies, abdominal wall defects in
164 re-examine whether rotavirus causes biliary atresia in children are discussed based on concepts from
168 toxin, biliatresone, responsible for biliary atresia in naturally-occurring animal models, that cause
170 Using a model of rotavirus-induced biliary atresia in newborn mice, we found that activated NK cell
173 ents detected all known infants with biliary atresia in the study population, although the 95% CI aro
174 lts in intestine after developmental biliary atresia, in addition to known mechanisms, such as the re
175 dies in the rotavirus mouse model of biliary atresia indicate that infection of biliary epithelium is
176 onfidence interval, 49.9 to 61.7); pulmonary atresia intact ventricular septum, 55.7% (95% confidence
177 h hypoplastic left heart syndrome, pulmonary atresia intact ventricular septum, single ventricle, and
178 ial intrahepatic cholestasis type 1, biliary atresia, intrahepatic cholestasis of pregnancy, and prim
187 ween ovarian folliculogenesis and follicular atresia is critical for female fertility and is strictly
188 tment typically occurs later because biliary atresia is difficult to detect during its early stages.
195 and are associated with extrahepatic biliary atresia, lead to a loss of membrane recognition, but do
196 al and critical care for long-gap esophageal atresia (LGEA) - in comparison to healthy infants - usin
197 Sox17 haploinsufficiency causes biliary atresia-like phenotypes and hepatitis in late organogene
198 iary disease, including extrahepatic biliary atresia, liver disease and transplantation in cystic fib
200 gh much is known about management of biliary atresia, many aspects are poorly understood, including i
201 opment of 46XX females can result in vaginal atresia, masculinization of the urethra, a single urogen
205 astic left heart syndrome (n=346), tricuspid atresia (n=103), tetralogy of Fallot (n=127), pulmonary
206 septal defects (n=22), tricuspid hypoplasia/atresia (n=13), and coronary artery fistulas (n=16).
207 103), tetralogy of Fallot (n=127), pulmonary atresia (n=177), heterotaxy syndrome (n=38), and other (
209 2], oesophageal atresia [n = 6,303], biliary atresia [n = 3,877], diaphragmatic hernia [n = 6,176], g
210 422], encephalocele [n = 1,562], oesophageal atresia [n = 6,303], biliary atresia [n = 3,877], diaphr
211 and had the underlying diagnoses: intestinal atresia, necrotizing enterocolitis, gastroschisis, and m
212 tions in CHD7 cause Coloboma, Heart Disease, Atresia of Choanae, Retardation of Growth and/or Develop
214 CHARGE (coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and/or dev
215 CHARGE (Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth/developmen
216 yndrome (coloboma of the eye, heart anomaly, atresia of the choanae, retardation, and genital and ear
217 acterized by ocular Coloboma, Heart defects, Atresia of the choanae, Retarded growth and development,
218 acterized by ocular coloboma, heart defects, atresia of the choanae, retarded growth and development,
219 defects (Coloboma of the eye, Heart defects, Atresia of the choanae, severe Retardation of growth and
220 velopment of the mesonephros in males causes atresia of the homolateral ejaculatory duct that results
222 was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.
225 n bile duct (CBD) disorders, such as biliary atresia or ischemic strictures, is restricted by the lac
226 owever, in contrast to patients with biliary atresia or other forms of cholestasis who develop progre
227 stein's anomaly (4 patients, 6.5%), pulmonic atresia or stenosis (7 patients, 11.5%), truncus arterio
228 s with primary biliary cirrhosis and biliary atresia or with Alagille syndrome, two major pediatric c
229 icantly higher occurrence of pulmonary valve atresia (P = 0.001) compared with cases without a detect
232 vating cytokine IL-33 is elevated in biliary atresia patient serum and in the livers and bile ducts o
233 onfiguration in a paediatric tricuspid valve atresia patient; (3) establish whether the widely used c
235 d compared these phenotypes to the pulmonary atresia phenotype observed following the systemic loss o
237 tic left heart syndrome and gastrointestinal atresias, probably due to haploinsufficiency for the nei
238 treated with cyclopamine exhibited pulmonary atresia, pulmonary stenosis, and persistent truncus arte
239 ulticenter, double-blind Steroids in Biliary Atresia Randomized Trial (START) was conducted in 140 in
240 immune function, skewed sex ratios, ovarian atresia, reduced egg production, and altered gene expres
241 r, increased apoptosis of GCs and follicular atresia, reduced ovulation rate, and a dramatic decline
242 for anastomotic strictures after esophageal atresia repair is feasible and acceptably safe and provi
243 tonsillectomy and/or adenoidectomy, choanal atresia repair, and/or treatment of gastroesophageal ref
246 ding ocular coloboma, heart defects, choanal atresia, retarded growth and development, genitourinary
247 E syndrome (coloboma, heart defects, choanal atresia, retarded growth, genital anomalies, and ear ano
248 were found for spina bifida, cleft lip, anal atresia, severe congenital heart defects in general, or
252 sia/stenosis (aOR = 2.97) including duodenal atresia/stenosis (aOR = 2.48), and atrial septal defect
253 gmatic hernia (aOR = 2.58), small intestinal atresia/stenosis (aOR = 2.97) including duodenal atresia
254 he prevalence of rectal and large intestinal atresia/stenosis was higher for ART births compared with
255 he aorta, cleft lip, cleft palate, anorectal atresia/stenosis, and limb reduction (upper limit of 95%
256 networks previously unrecognized in biliary atresia, such as the complement components C3ar-1 and C1
257 95-1.01, p = 0.19) and children with biliary atresia surviving with native liver (OR = 0.96, 95% CI 0
260 subjects from families with congenital aural atresia that were heterozygous for TSHZ1 loss-of-functio
262 ; however, a subset of patients with biliary atresia, the most common childhood cholangiopathy, exhib
263 itially designed for patients with tricuspid atresia, this procedure is now offered for a vast array
264 vered that the sea lamprey adapts to biliary atresia through a unique mechanism of de novo synthesis
265 ders such as oesophageal atresia and biliary atresia through clinical trials because of the rarity of
266 we show that androgens attenuate follicular atresia through nuclear and extranuclear signaling pathw
267 d drive the phenotypic expression of biliary atresia, thus constituting a potential therapeutic targe
268 We hypothesized that the T cells in biliary atresia tissue expressed related TCRs, suggesting that t
269 s (7.5% [95% CI, 3.5%-13.8%]) with pulmonary atresia to 497 of 801 (62.0% [58.7%-65.4%]) with coarcta
270 we used a rotavirus-induced model of biliary atresia to investigate the entire biliary transcriptome
272 ntact ventricular septum and 28 with virtual atresia) underwent RV decompression at median 3 (25th-75
278 f cholangiocyte injury in a model of biliary atresia, which is relevant to human BA and may suggest p
279 d chronic biliary disorders, such as biliary atresia, which remains the most common paediatric chroni
280 low-up was predicted by PN dependence and SB atresia, while maximal absolute SB width by birth weight
281 cts including overriding aorta and pulmonary atresia, while none of the sham-operated controls were a
283 3712]), tracheoesophageal fistula/esophageal atresia (WIQR, $39206; median, $105259 [IQR, $87335-$126
284 identified the 7 known infants with biliary atresia with a sensitivity of 100% (95% CI, 56.1%-100.0%
285 ied included omphalocele, jejunal, and ileal atresia with aberrant mesenteric blood supply, and synda
287 aling in the myocardium results in tricuspid atresia with hypoplastic right ventricle associated with
288 ith higher risk of thrombosis were pulmonary atresia with intact ventricular septum (hazard ratio [HR
289 ing catheter valve perforation for pulmonary atresia with intact ventricular septum (PAIVS) 21 years
290 ongitudinal series of fetuses with pulmonary atresia with intact ventricular septum (PAIVS) and/or cr
291 Ninety-nine patients (71 with pulmonary atresia with intact ventricular septum and 28 with virtu
294 (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely.
295 es undergoing RV decompression for pulmonary atresia with intact ventricular septum were included fro
296 -month-old female infant with type I biliary atresia with jaundice (total serum bilirubin, 22.2 mg/dL
297 leads to the common birth defect esophageal atresia with or without trachea-esophageal fistula (EA/T
298 a relatively common birth defect, esophageal atresia with or without tracheoesophageal fistula (EA/TE
299 al diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TEF) can be r