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1 esis and possible therapy for infant biliary atresia.
2 6 years (IQR 0.58-3.0) and 64.3% had biliary atresia.
3 amprey thrives despite developmental biliary atresia.
4 role in pathogenesis in experimental biliary atresia.
5 thout tracheo-esophageal fistula or tracheal atresia.
6 and embryos predominantly develop pulmonary atresia.
7 drives the phenotype of experimental biliary atresia.
8 ion to primary biliary cirrhosis and biliary atresia.
9 mice a partial rupture, resulting in choanal atresia.
10 ith isolated SMCP and/or unexplained choanal atresia.
11 mune response in the pathogenesis of biliary atresia.
12 e surrounding follicular cells did not begin atresia.
13 ion of apoptosis in a mouse model of biliary atresia.
14 have furthered our understanding of biliary atresia.
15 the early pathogenesis of congenital biliary atresia.
16 in the pathogenesis of experimental biliary atresia.
17 ant clinical problem in infants with biliary atresia.
18 tion, or to die by programmed cell death, or atresia.
19 mice using an experimental model of biliary atresia.
20 omes, screening, and pathogenesis of biliary atresia.
21 ntributing factor in the etiology of biliary atresia.
22 bility to induce the murine model of biliary atresia.
23 es, and structural proteins at all phases of atresia.
24 jury and obstruction in experimental biliary atresia.
25 ogy of Fallot with complete pulmonary artery atresia.
26 ng laryngeal atresia and the other, tracheal atresia.
27 disease progression in patients with biliary atresia.
28 reserve by early adulthood without inducing atresia.
29 e balance between follicular development and atresia.
30 g a mouse model of rotavirus-induced biliary atresia.
31 athogenesis of the embryonic form of biliary atresia.
32 utor to the pathogenesis of neonatal biliary atresia.
33 s after liver transplantation due to biliary atresia.
34 esophagus did not correlate with esophageal atresia.
35 as not sufficient for diagnosis of pulmonary atresia.
36 ngiography in depicting extrahepatic biliary atresia.
37 es within the livers of infants with biliary atresia.
38 cular hypoplasiawith aortic and mitral valve atresia.
39 , resulting in an attenuated form of biliary atresia.
40 mild pulmonary stenosis to severe pulmonary atresia.
41 bile ducts of mice with experimental biliary atresia.
42 ng sphincter-of-Oddi dysfunction and biliary atresia.
43 ion, showed a limited effect on ovulation or atresia.
44 rapid increase of macrophages and a surge of atresia.
45 ious adverse events in children with biliary atresia.
46 before the surge of macrophage migration and atresia.
48 logy of Fallot (15 patients, 25%), tricuspid atresia (12 patients, 20%), Ebstein's anomaly (4 patient
50 dence interval, 45.8 to 64.4); and tricuspid atresia, 74.6% (95% confidence interval, 62.4 to 83.4).
51 rvival was obtained in children with biliary atresia (82%, 79%, and 78% at 1, 5, and 10 years, respec
54 patients with complex pulmonary stenosis or atresia, a detailed delineation of all sources of pulmon
55 est in whether rotavirus could cause biliary atresia, an idiopathic, obliterative infantile disease o
56 congenital malformations comprising choanal atresia, anal abnormalities, post-axial polydactyly, hea
57 e for neonatal disorders such as oesophageal atresia and biliary atresia through clinical trials beca
58 patients with complex pulmonary stenosis and atresia and can be considered a noninvasive alternative
62 rs in response to Shh knockdown is pulmonary atresia and is directly related to the abnormal developm
65 ring from tetralogy of Fallot with pulmonary atresia and multiple aortopulmonary collaterals and fami
69 ct remnants, and peripheral blood of biliary atresia and other cholestatic disease controls were char
70 ar and humoral autoimmunity in human biliary atresia and possible interventional strategies therefore
71 formation for assessing prognosis in biliary atresia and primary biliary cirrhosis; and important cli
72 cedures such as portoenterostomy for biliary atresia and repair of esophageal atresia and tracheoesop
73 affect infants and children-such as biliary atresia and Reye's syndrome; and (3) redefinition of the
78 virus (RRV)- induced murine model of biliary atresia and whether the T cells are sufficient to result
80 that, based on rates of oocyte degeneration (atresia) and clearance, are needed to continuously reple
82 (48%) with tetralogy of Fallot and pulmonary atresia, and 4 of 54 (8%) with prosthetic valve endocard
84 ngiomas, pyriform aperture stenosis, choanal atresia, and laryngeal webs, may also have adverse effec
87 , familial intrahepatic cholestasis, biliary atresia, and primary sclerosing cholangitis, and clinica
88 sis, primary sclerosing cholangitis, biliary atresia, and progressive familial intrahepatic cholestas
90 ents of autoimmunity exist in murine biliary atresia, and the progressive bile duct injury is due in
92 curring events such as ovulation and ovarian atresia are accompanied with tissue destruction and repa
93 on, embryonic and perinatal forms of biliary atresia are distinguished by gene expression profiling.
95 , primary sclerosing cholangitis and biliary atresia are thought to be immune-mediated cholangiopathi
96 etically programmed animal model for biliary atresia, as it loses its bile ducts and gallbladder duri
97 ent hepatoportoenterostomy (HPE) for biliary atresia at 9 U.S. pediatric centers between 1997 and 200
98 = 5) and perinatal (n = 6) forms of biliary atresia at the time of diagnosis and hybridized them aga
99 omplications of portal hypoplasia in biliary atresia (BA) and acute rejection (AR) are still major co
100 ing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these p
101 ent in infants with cirrhosis due to biliary atresia (BA) as early as the time of evaluation for live
113 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
119 asonography (US) in the diagnosis of biliary atresia (BA), with surgery as the reference standard.
123 e three main human cholangiopathies (biliary atresia [BA], primary biliary cholangitis [PBC], and pri
125 ular septum, single ventricle, and tricuspid atresia born in 1996 to 2003 were identified from the Te
126 populate the livers of infants with biliary atresia, but it is unknown whether neonatal lymphocytes
127 duces a disease similar to infantile biliary atresia, but previous attempts to correlate reovirus inf
130 s with features of vertebral anomalies, anal atresia, cardiovascular anomalies, trachea-oesophageal f
131 ed immunodeficiency with multiple intestinal atresias (CID-MIA) is a rare hereditary disease characte
133 d anomalies include ocular coloboma, choanal atresia, cranial nerve defects, distinctive external and
134 defects, tetralogy of Fallot, and tricuspid atresia, defects that resemble those associated with mut
135 st 60 years, successful repair of esophageal atresia (EA) and distal tracheoesophageal fistula (TEF)
136 IM 600992), a condition including esophageal atresia (EA) and tracheoesophageal fistula (TEF), in whi
137 imary repair (aged <3 months) with pulmonary atresia (early-PA group; n=49), and primary repair betwe
140 sis, including organ and digit duplications, atresia, fistulas, hypertelorism, cleft palate and hamar
141 and haematocervix with cervical and vaginal atresia found on the left side (classification 1.2) with
142 s of ocular coloboma, heart defects, choanal atresia, growth retardation, genital abnormalities, and
143 ere bilateral microphthalmia and oesophageal atresia has a de novo missense mutation, R74P, that alte
144 very of the pathogenic mechanisms of biliary atresia has been limited by the inability to study extra
147 and neonatal mice with experimental biliary atresia have shown increased expression of proapoptosis
148 , reintervention was associated with virtual atresia (hazard ratio [HR], 0.51; 95% confidence interva
149 dition is characterized by bilateral choanal atresia, hearing loss, cleft lip and/or palate, and othe
150 ound in 11 independent patients with biliary atresia, hepatitis BC, alcohol, primary biliary cirrhosi
151 Relatively common conditions such as biliary atresia, however, remain largely unexplained and the vir
152 mimic of Tetralogy of Fallot with pulmonary atresia; however, subsequent reports describe only a sin
153 uctal stent were less likely to have virtual atresia (HR, 0.36; 95% CI, 0.15-0.85; P=0.02) and more l
155 neonatal conditions reported were intestinal atresia in 28 (54.9%) studies, abdominal wall defects in
158 re-examine whether rotavirus causes biliary atresia in children are discussed based on concepts from
159 f four patients, unilateral transverse sinus atresia in eight, and a narrowed superior sagittal sinus
160 nce 1999, all infants with suspected biliary atresia in England and Wales, UK, have been referred to
161 pathology analogous to that found in biliary atresia in humans, and high levels of T3SA+ antigen in b
165 Using a model of rotavirus-induced biliary atresia in newborn mice, we found that activated NK cell
166 falcine sinus associated with straight sinus atresia in one patient and suggested transverse sinus th
167 indications for transplantation were biliary atresia in seven, fulminant hepatic failure in six, chro
169 lts in intestine after developmental biliary atresia, in addition to known mechanisms, such as the re
170 dies in the rotavirus mouse model of biliary atresia indicate that infection of biliary epithelium is
171 onfidence interval, 49.9 to 61.7); pulmonary atresia intact ventricular septum, 55.7% (95% confidence
172 h hypoplastic left heart syndrome, pulmonary atresia intact ventricular septum, single ventricle, and
173 ial intrahepatic cholestasis type 1, biliary atresia, intrahepatic cholestasis of pregnancy, and prim
181 ween ovarian folliculogenesis and follicular atresia is critical for female fertility and is strictly
190 and are associated with extrahepatic biliary atresia, lead to a loss of membrane recognition, but do
191 Sox17 haploinsufficiency causes biliary atresia-like phenotypes and hepatitis in late organogene
192 iary disease, including extrahepatic biliary atresia, liver disease and transplantation in cystic fib
194 gh much is known about management of biliary atresia, many aspects are poorly understood, including i
195 opment of 46XX females can result in vaginal atresia, masculinization of the urethra, a single urogen
197 irty-two patients with pulmonary stenosis or atresia (median age: 4.7 years, range: 1 day to 46.9 yea
200 astic left heart syndrome (n=346), tricuspid atresia (n=103), tetralogy of Fallot (n=127), pulmonary
201 septal defects (n=22), tricuspid hypoplasia/atresia (n=13), and coronary artery fistulas (n=16).
202 ded tetralogy of Fallot (TOF) with pulmonary atresia (n=13), TOF with pulmonary stenosis (n=4), post-
203 103), tetralogy of Fallot (n=127), pulmonary atresia (n=177), heterotaxy syndrome (n=38), and other (
205 and had the underlying diagnoses: intestinal atresia, necrotizing enterocolitis, gastroschisis, and m
206 tions in CHD7 cause Coloboma, Heart Disease, Atresia of Choanae, Retardation of Growth and/or Develop
208 knockouts), including abnormal stenosis and atresia of the aorta, defective arterial branching from
209 CHARGE (coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and/or dev
210 yndrome (coloboma of the eye, heart anomaly, atresia of the choanae, retardation, and genital and ear
211 acterized by ocular Coloboma, Heart defects, Atresia of the choanae, Retarded growth and development,
212 acterized by ocular coloboma, heart defects, atresia of the choanae, retarded growth and development,
213 defects (Coloboma of the eye, Heart defects, Atresia of the choanae, severe Retardation of growth and
214 velopment of the mesonephros in males causes atresia of the homolateral ejaculatory duct that results
216 was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.
217 were abnormal but not suggestive of biliary atresia (one false-negative finding); 12 were consistent
220 n bile duct (CBD) disorders, such as biliary atresia or ischemic strictures, is restricted by the lac
221 owever, in contrast to patients with biliary atresia or other forms of cholestasis who develop progre
222 patients (one lost to follow-up) had biliary atresia or other surgical lesions; two were abnormal but
223 stein's anomaly (4 patients, 6.5%), pulmonic atresia or stenosis (7 patients, 11.5%), truncus arterio
224 isk factors for mortality, whereas pulmonary atresia or stenosis and pulmonary artery banding were as
225 s with primary biliary cirrhosis and biliary atresia or with Alagille syndrome, two major pediatric c
226 developed ovaries but had excess follicular atresia, ovulation of defective oocytes, and severely re
227 icantly higher occurrence of pulmonary valve atresia (P = 0.001) compared with cases without a detect
231 vating cytokine IL-33 is elevated in biliary atresia patient serum and in the livers and bile ducts o
232 onfiguration in a paediatric tricuspid valve atresia patient; (3) establish whether the widely used c
234 d compared these phenotypes to the pulmonary atresia phenotype observed following the systemic loss o
236 tic left heart syndrome and gastrointestinal atresias, probably due to haploinsufficiency for the nei
237 treated with cyclopamine exhibited pulmonary atresia, pulmonary stenosis, and persistent truncus arte
238 ulticenter, double-blind Steroids in Biliary Atresia Randomized Trial (START) was conducted in 140 in
239 r, increased apoptosis of GCs and follicular atresia, reduced ovulation rate, and a dramatic decline
241 3 low), aortopexy (7), laparoscopic duodenal atresia repair (4), and various major cardiac operations
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
245 ding ocular coloboma, heart defects, choanal atresia, retarded growth and development, genitourinary
246 E syndrome (coloboma, heart defects, choanal atresia, retarded growth, genital anomalies, and ear ano
247 ed penetrance, such as cleft palate, choanal atresia, septal defects of the heart, haemorrhages, pren
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
259 subjects from families with congenital aural atresia that were heterozygous for TSHZ1 loss-of-functio
261 ; however, a subset of patients with biliary atresia, the most common childhood cholangiopathy, exhib
262 ve finding); 12 were consistent with biliary atresia (three false-positive findings); four demonstrat
263 vered that the sea lamprey adapts to biliary atresia through a unique mechanism of de novo synthesis
264 ders such as oesophageal atresia and biliary atresia through clinical trials because of the rarity of
265 we show that androgens attenuate follicular atresia through nuclear and extranuclear signaling pathw
266 d drive the phenotypic expression of biliary atresia, thus constituting a potential therapeutic targe
267 We hypothesized that the T cells in biliary atresia tissue expressed related TCRs, suggesting that t
268 s (7.5% [95% CI, 3.5%-13.8%]) with pulmonary atresia to 497 of 801 (62.0% [58.7%-65.4%]) with coarcta
269 we used a rotavirus-induced model of biliary atresia to investigate the entire biliary transcriptome
270 fants with tetralogy of Fallot and pulmonary atresia (TOF/PA) and to prospectively validate these pre
272 ntact ventricular septum and 28 with virtual atresia) underwent RV decompression at median 3 (25th-75
278 nfants born in the UK with suspected biliary atresia were investigated in regional centres, and, if c
280 d chronic biliary disorders, such as biliary atresia, which remains the most common paediatric chroni
281 low-up was predicted by PN dependence and SB atresia, while maximal absolute SB width by birth weight
282 cts including overriding aorta and pulmonary atresia, while none of the sham-operated controls were a
284 3712]), tracheoesophageal fistula/esophageal atresia (WIQR, $39206; median, $105259 [IQR, $87335-$126
286 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 ibe the morphologic variability in pulmonary atresia with intact ventricular septum (PAIVS) within a
292 Ninety-nine patients (71 with pulmonary atresia with intact ventricular septum and 28 with virtu
295 (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely.
296 es undergoing RV decompression for pulmonary atresia with intact ventricular septum were included fro
297 a relatively common birth defect, esophageal atresia with or without tracheoesophageal fistula (EA/TE
298 al diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TEF) can be r
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