Gastrointestinal Malformations, Associated Congenital Abnormalities, and Intrauterine Growth

Tárnok, András; Méhes, K

doi:10.1097/00005176-200204000-00017

Cited by 13 publications

(8 citation statements)

References 7 publications

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“…GISM's in neonatal babies are frequently accompanied by other congenital anomalies and they usually require surgical interventions and intensive care (1,8). Therefore, they must be examined for additional anomalies at early periods.…”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal system malformations in children are associated with congenital heart defects

Örün¹,

Bilici²,

Demirçeken³

et al. 2011

Anadolu Kardiyol Derg

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ÖZETAmaç: Gastrointestinal sistem malformasyonlu (GİSM) çocuklarda doğumsal kalp hastalıklarının sıklığını ve mortalite ile ilişkisini saptamak. Yöntemler: Hastanemizin, Çocuk Cerrahisi Kliniğinde GİSM nedeniyle izlenen ve kardiyolojik inceleme yapılan ardışık 242 çocuk hastanın kayıt-ları retrospektif olarak incelenerek, GİSM olan çocuk hastalarda doğumsal kalp defektlerinin (DKH) insidansı araştırılmıştır. Verilerin istatistiksel değerlendirmesinde Ki-kare testi kullanılmıştır. Bulgular: GİSM saptanan, yaşları 0 gün ile 15 yıl arasında değişen 242 hasta değerlendirildi. Hastaların 135'i (%55.8) erkek, 107'si kız (%44.2) idi. En sık görülen gastrointestinal malformasyonları anorektal malformasyon (%43.2), mide, ileum veya kolon atrezisi (%21) ve özofajiyal atrezi/ trakeoözo-fajiyal fistül (%18.3) idi. Hastaların %28.5'inde doğumsal kalp hastalığı saptanmış olup, en sık gözlenen anomaliler; olguların 31'inde (%44.9) atriyal septal defekt, 17'sinde (%24.6) ventriküler septal defekt, 5'inde (%7.2) patent duktus arteriyozus idi. GİSM nedeni ile ameliyat edilen doğumsal kalp hastalığı olan (%16.7) ve olmayan (%13.3) hastalar arasında mortalite açısından anlamlı fark saptanmadı (p>0.05). Sonuç: Gastrointestinal sistem malformasyonu saptanan hastaların erken dönemde kardiyolojik açıdan değerlendirilmesi gerektiği vurgulanmak istenmiştir. (Anadolu Kardiyol Derg 2011; 2: 146-9) Anahtar kelimeler: Gastrointestinal sistem malformasyonu, doğumsal kalp defekti, ekokardiyografi, çocuk, hastalık insidansı ABSTRACT Objective: To determine the frequency of congenital heart defects (CHD) in children with gastrointestinal malformations (GISM) and mortality rates in patients with GISM. Methods: Two hundred and forty two consecutive children patients with GISM followed up in Pediatric Surgery Clinics of our hospital were examined for cardiovascular anomaly by the Department of Pediatric Cardiology, and the CHD incidence was investigated by examining the records of the patients retrospectively. Chi-square test was used for the statistical analysis of data. Results: Two hundred and forty two patients with gastrointestinal system malformations were included in the study. Of 242 patients, 135 (55.8%) were male and 107 (44.2%) were female, and their age range was 0-15 years. The most frequent GISM were anorectal malformations (43.2%), atresia involving stomach, ileum or colon (21%) and esophageal atresia/tracheoesophageal fistula (18.3%). Congenital heart defects were observed in 28.5% of the participants. The most frequent defects were as follows; atrial septal defect (31 patients, 44.9%) a, ventricular septal defect (17 patients, 24.6%) and patent ductus arteriosus (5 patients, 7.2%). There was no significant difference (p>0.05) in mortality rate in patients with CHD (16.7%) and without CHD (13.3%) undergoing operations for GISM. Conclusion: We would like to emphasize the importance of the earliest possible cardiological evaluation of all patients with gastrointestinal system malformations. (Anadolu Kardiyol Derg 2011; 2: 146-9)

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Section: Discussionmentioning

confidence: 99%

Gastrointestinal system malformations in children are associated with congenital heart defects

Örün¹,

Bilici²,

Demirçeken³

et al. 2011

Anadolu Kardiyol Derg

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“…Even if infants with aneuploidy are excluded, most sporadic structural malformations are still associated with a decreased birth weight for gestational age [Khoury et al, ; Mili et al, ; Rasmussen et al, ]. This has been shown specifically for the VACTERL association [Czeizel and Ludányi, ], the prototypic blastogenetic association [Lubinsky, ], for anorectal anomalies [Wijers et al, ] and gastrointestinal abnormalities, both isolated or together with other findings [Tárnok and Méhes, ], cardiac defects ranging from the simple to the complex [Malik et al, ; Wallenstein et al, ], TEF/EA [Depaepe et al, ], radial findings alone [Czeizel et al, ], or associated with other anomalies [Evans et al, ], and hypospadias [Gatti et al, ; Brouwers et al, ].…”

Section: Hypocellularity and Malformationsmentioning

confidence: 99%

Embryonic hypocellularity, blastogenetic malformations, and fetal growth restriction

Lubinsky

2016

American J of Med Genetics Pt A

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An association between congenital malformations and fetal growth restriction (FGR) can be largely explained by a relationship with early embryonic hypocellularity. The malformations include the VACTERL association, which is exceptional as a Mendelian syndrome, but is commonly associated with monozygotic twinning, maternal diabetes, and some forms of aneuploidy, all characterized by a small embryo early in development. Parsimony suggests that these different links to VACTERL are related to the hypocellularity as a single common factor, rather than as an expression of three independent pathogenetic processes. A distinct non-genetic pathogenesis is further supported by increased frequencies in the same conditions of a single umbilical artery (SUA), which is also unusual in Mendelian disorders. SUA often involves the atrophy of one artery, which may be facilitated by altered hemodynamics in a smaller embryo, providing a direct link to hypocellularity. Hypocellularity may also explain a possible connection between VACTERL and certain mitochondrial disorders, where reduced energy might slow early cell division and growth, reducing the size of the embryo. © 2016 Wiley Periodicals, Inc.

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“…Muscle or/and skeletal defects tend to coincide with trisomy [Bollmann et al, 1995;Keeling et al, 1997;Robert et al, 1997b] and several other congenital defects [Mastroiacovo et al, 1992;Bollmann et al, 1995;Tárnok and Méhes, 2002;Harmath et al, 2006;Man and Chang, 2006].…”

Section: Introductionmentioning

confidence: 99%

“…The etiology of musculoskeletal defects is largely unknown [Hösgor and Tibboel, 2004;Anthony et al, 2005;Yang et al, 2006], but an increased risk of one or more of these defects has been reported in association with both low and high maternal age [Mastroiacovo et al, 1992;Bellovits, 2003;Reefhuis and Honein, 2004;Yang et al, 2006], pregestational diabetes [Janssen et al, 1996], nulliparity [Yang et al, 2006], tobacco smoking [Källén, 1997;Man and Chang, 2006], medication use [Giles and Bannigan, 2006;Man and Chang, 2006], fetal alcohol syndrome [Hannigan and Armant, 2000], and iatrogenic reproductive technologies [Farhi and Fisch, 2007]. Muscle or/and skeletal defects tend to coincide with trisomy [Bollmann et al, 1995;Keeling et al, 1997;Robert et al, 1997b] and several other congenital defects [Mastroiacovo et al, 1992;Bollmann et al, 1995;Tárnok and Méhes, 2002;Harmath et al, 2006;Man and Chang, 2006].…”

Section: Introductionmentioning

confidence: 99%

Maternal nickel exposure and congenital musculoskeletal defects

Vaktskjold

Talykova

Chashchin

et al. 2008

American J Industrial Med

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Gastrointestinal Malformations, Associated Congenital Abnormalities, and Intrauterine Growth

Cited by 13 publications

References 7 publications

Gastrointestinal system malformations in children are associated with congenital heart defects

Gastrointestinal system malformations in children are associated with congenital heart defects

Embryonic hypocellularity, blastogenetic malformations, and fetal growth restriction

Maternal nickel exposure and congenital musculoskeletal defects

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