scholarly journals VACTERL-H SYNDROME – A CASE REPORT

2019 ◽  
Vol 3 (9) ◽  
Author(s):  
Dr. Priya Singh ◽  
Prof. Dr. Ghazi Sharique Ahmad ◽  
Prof. Dr. Ahmad Rizwan Karim
Keyword(s):  
Congenital Malformations ◽  
Failure To Thrive ◽  
Facial Asymmetry ◽  
Postnatal Period ◽  
The Body ◽  
Surgical Correction ◽  
Vacterl Association ◽  
Anal Atresia ◽  
Vater Association ◽  
Ear Malformations

The exact cause of VACTERL association is unknown; most cases occur randomly, for no apparent reason. In rare cases, VACTERL association has occurred in more than one family member. VACTERL association is an association of birth defects that affects multiple parts of the body. It includes vertebral or vascular anomalies, anal atresia, cardiac defects, tracheoesophageal – fistula/esophageal atresia, renal defects, and limbs defects. Other features may include (less frequently) growth deficiencies and failure to thrive; facial asymmetry (hemifacial microsomia); external ear malformations; intestinal malrotation; and genital anomalies. Intelligence is usually normal. The diagnosis of VACTERL-H Syndrome is majorly based upon the complete physical examination and a few specialized tests to ascertain the features of the syndrome. The treatment of VACTERL-H is directed towards the specific symptoms that are apparent in each individual, which often varies greatly. The management of patients with VACTERL/VATER association typically centers around surgical correction of the specific congenital anomalies (typically anal atresia, certain types of cardiac malformations, and/or tracheo-esophageal fistula) in the immediate postnatal period, followed by long-term medical management of sequelae of the congenital malformations. If optimal surgical correction is achievable, the prognosis can be relatively positive. Hence early diagnosis and early interventions are needed to prevent morbidity and mortality. Key words: VACTERL-H syndrome, congenital malformations, clinical examinations

scholarly journals Analysis of risk factors for different types of anesthetic support of newborns and infants with surgical pathology

2020 ◽  
pp. 28-34
Author(s):  
V.I. Snisar ◽  
◽  
O.O. Vlasov ◽  
I.A. Makedonskyy ◽  
◽  
...  
Keyword(s):  
Risk Factors ◽  
Blood Pressure ◽  
Logistic Regression ◽  
Surgical Treatment ◽  
Postoperative Period ◽  
Congenital Malformations ◽  
Fatal Case ◽  
The Body ◽  
Surgical Correction ◽  
Simple Logistic

High-quality anesthetic support during surgical correction of congenital malformations of internal organs and the postoperative period in newborns and infants is complicated by concomitant diseases, significantly affects the processes of metabolism, gas exchange, homeostasis, cerebral, peripheral hemodynamics, etc. before, during and after the surgical intervention. Purpose — to identify the leading risk factors associated with death in various types of anesthetic support for newborns and infants during surgical correction of congenital malformations. Materials and methods. The retrospective study included newborns with congenital malformations of the surgical profile, as well as infants who received and continued stepwise surgical treatment for congenital malformations, depending on the chosen combined anesthetic accompaniment (inhalation + regional anesthesia and inhalation + intravenous anesthesia). The study was carried out in the following stages: 1) to conduct surgical treatment and anesthetic support, 2) introduction of the child into anesthesia, 3) the traumatic stage of the operation, 4) within 1 hour after the operation, 5) 24 hours after the operation. Risk factors were determined by simple logistic regression with the calculation of the odds ratio, 95% confidence interval (95% CI). Results. Among the functional indicators of the vital functions of the body of children with congenital disorders before, during, immediately and 24 hours after surgery against the background of combined anesthesia, with a simple logistic regression analysis, the chance of a fatal case increases with deviations from the norm of peripheral saturation — at all stages of surgical support (7.8–15.0 times); cerebral oxygenation of the child — at the moments of induction into anesthesia and in the postoperative period (10.8 at the stage 2, 72.0 times at the stage 4); increased diastolic blood pressure at the stage of induction of the child into anesthesia (1.6 times). Conclusions. To prevent the chances of death under various types of anesthesia for children with congenital malformations during surgical treatment, it is advisable to more closely monitor blood pressure, cerebral, peripheral oximetry and promptly correct the impaired condition of the child. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of these Institutes. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: newborns, infants, congenital malformations, anesthesia, risk factors.

VATER Association

2003 ◽  
Vol 22 (3) ◽  
pp. 71-75 ◽  
Author(s):  
Janie Spoon
Keyword(s):  
Ventricular Septal Defect ◽  
Tracheoesophageal Fistula ◽  
Umbilical Artery ◽  
Septal Defect ◽  
Vacterl Association ◽  
Anal Atresia ◽  
Single Umbilical Artery ◽  
Vater Association ◽  
Vertebral Defects ◽  
Cardiovascular Anomalies

VATER ASSOCIATION WAS FIRST described in 1973 as a nonrandom group of related defects, as follows: V = vertebral defects, A = anal atresia, TE = tracheoesophageal fistula, R = radial limb dysplasia.1 Since that time, various expansions of the acronym have been suggested in an effort to further define the scope of the VATER association. Temtamy and Miller expanded the V in VATER to include vascular anomalies, specifically, ventricular septal defect and single umbilical artery.2 An expansion of the acronym to VACTERL has been suggested, with the C denoting cardiovascular anomalies and the L denoting limb anomalies.3 The designation of VATER or VACTERL association can be made for infants with at least three of the defects designated in the acronym.4

scholarly journals Vestibular Derangement and Motion Intolerance in VATER Association

2017 ◽  
Vol 2017 ◽  
pp. 1-2
Author(s):  
Orit Samuel ◽  
Avi Shupak ◽  
Ayelet Eran ◽  
Dror Tal
Keyword(s):  
Congenital Malformations ◽  
Tracheoesophageal Fistula ◽  
Laboratory Diagnosis ◽  
Anal Atresia ◽  
Lateral Semicircular Canal ◽  
Vater Association ◽  
Bone Anomalies ◽  
Vertebral Defects ◽  
Field Defect ◽  
Radial Bone

VATER association is a nonrandom occurrence of congenital malformations: vertebral defects, anal atresia, tracheoesophageal fistula, renal defects, and radial bone anomalies. We report the case of a 19-year-old man with a childhood diagnosis of VATER association, who presented to the motion sickness clinic with severe seasickness. We discuss the clinical and laboratory diagnosis of vestibular pathophysiology, which was confirmed by MRI of lateral semicircular canal and vestibule dysplasia. We suggest the possibility of vestibular involvement as part of the developmental field defect associated with VATER syndrome, which hitherto has rarely been reported.

scholarly journals Neonate with VACTERL association: a rare entity

2017 ◽  
Vol 4 (4) ◽  
pp. 1551
Author(s):  
Sandhya Chauhan ◽  
Ashok Garg ◽  
Pancham Kumar ◽  
Ambika Sood
Keyword(s):  
Congenital Malformations ◽  
Genetic Factors ◽  
Developmental Defect ◽  
Rare Entity ◽  
Vacterl Association ◽  
Anal Atresia ◽  
Rare Association ◽  
Multiple Organs ◽  
Healthy Mother ◽  
Random Occurrence

VACTERL (Vertebral, anal, cardiac, tracheoesophageal, renal and limb) is an acronym for cluster of congenital malformations including Vertebral or Vascular anomalies, Anal atresia, Cardiac defects, Tracheoesophageal fistula/Esophageal atresia, Renal and Limb defects. It is a rare association with sporadic and non-random occurrence where multiple organs are affected due to developmental defect during blastogenesis. Exact cause is unknown but multiple environmental and genetic factors have been implicated. For diagnosis, three components of VACTERL association are needed however patient may have other congenital malformations also. Here we report a newborn with VACTERL association born to a healthy mother by cesarean section for polyhydramnios with unstable lie.

The supine position of postnatal human infants

2009 ◽  
Vol 10 (2) ◽  
pp. 252-269 ◽  
Author(s):  
Hideko Takeshita ◽  
Masako Myowa-Yamakoshi ◽  
Satoshi Hirata
Keyword(s):  
Supine Position ◽  
Postnatal Period ◽  
The Body ◽  
Body Parts ◽  
Human Infants ◽  
Face To Face ◽  
Developmental Continuity ◽  
Exploratory Movements ◽  
Locomotor Ability ◽  
Development And Evolution

In this review, we discuss the implications of placing an infant in the supine position with respect to human cognitive development and evolution. When human infants are born, they are relatively large and immature in terms of postural and locomotor ability as compared with their closest relatives, the great apes. Hence, human mothers seemingly adopt a novel pattern of caring for their large and heavy infants, i.e., placing their infants in the supine position; this promotes face-to-face communication with their infants. Moreover, infants in the supine position can interact with other nearby individuals in the same manner from an early age. In addition, the infants can also explore their own body parts and/or objects with their hands since the hands are not required to support the body and are therefore, free to move. These activities are considered to be fundamental to the early development of human social and nonsocial cognition, including knowledge of self, in the first six months after birth. Further, developmental continuity in the voluntary exploratory movements in the prenatal period (in utero) to the early postnatal period is also discussed.

First Case of Human Infection Caused by Pasteurella gallinarum Causing Infective Endocarditis in an Adolescent 10 Years After Surgical Correction for Truncus Arteriosus

PEDIATRICS ◽  
1995 ◽  
Vol 95 (6) ◽  
pp. 944-948
Author(s):  
Mohammad Al Fadel Saleh ◽  
Mohammad S. Al-Madan ◽  
Hashim H. Erwa ◽  
Ivy Defonseka ◽  
Saira Z. Sohel ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Infective Endocarditis ◽  
Congenital Heart ◽  
Human Infection ◽  
The Body ◽  
Surgical Correction ◽  
Truncus Arteriosus ◽  
Causative Organism ◽  
First Case

Objective. To report the first case of human infection (infective endocarditis [IEI]) caused by Pasteurella gallinarum and to review the literature regarding IE caused by the genus Pasteurella. Setting. University hospital based. Patient. An adolescent boy who underwent successful correction for truncus arteriosus 10 years before the present illness. Results. Persistent fever, pallor, and a palpable spleen suggested IE clinically. Echocardiography documented vegetation in the conduit that was used for surgical correction. Blood cultures grew P. gallinarum and confirmed its role as the causative organism for IE in the patient. Conclusion. This case illustrates that IE may develop in a child with congenital heart disease several years after surgical intervention using material that is foreign to the body (conduit), and that such a complication may involve unusual pathogens. These observations emphasize the need for careful long-term follow-up of children with congenital heart disease even after successful surgical correction.

Rehabilitation potential in the children after surgical correction of congenital heart defects in different age periods

10.12737/7238 ◽  
2014 ◽  
Vol 8 (1) ◽  
pp. 0-0
Author(s):  
Любчик ◽  
V. Lyubchik ◽  
Голубова ◽  
T. Golubova ◽  
Елисеева ◽  
...  
Keyword(s):  
Congenital Heart ◽  
Heart Defects ◽  
Heart Rhythm ◽  
The Body ◽  
Surgical Correction ◽  
Self Assessment ◽  
Sf 36 ◽  
Rehabilitation Potential

Comparative evaluation of rehabilitation potential in 20 girls at the sanatorium stage of rehabilitation after surgical correction of congenital heart disease in different age periods was carried out. Structural and functional diagnoses were specified to determine the level of rehabilitation potential. Identified violations were assessed as the loss or absence, reduction, addition or excess. Research methods included: clinical examination with determination of the level of physical development on the body mass index, functional orthostatic test, as-sessment of the estimated impact of blood volume and "double work", Doppler echocardiography, spectral analysis of heart rhythm, some indicators of emotional state on the differentiated self-assessment of functional status test and “quality of life” according to test SF-36. In children of the first group with early surgical correction regarding (under 2 years) significant changes in functionality were noted: higher initial rehabilitation potential and positive changes in central and peripheral hemodynamics, positive changes in the emotional sphere traced by the level of comfort, improved exercise tolerance under the influence of sanatorium rehabilitation. Preliminary observations suggest the possibility of compensation of impaired functions in children of the specified contingent during the sanatorium stage of rehabilitation is to improve their sano-genetic and psycho-physiological potential.

Number of nuclei in mammalian cardiac myocytes

1979 ◽  
Vol 57 (10) ◽  
pp. 1122-1129 ◽  
Author(s):  
B. Korecky ◽  
S. Sweet ◽  
K. Rakusan
Keyword(s):  
Cardiac Myocytes ◽  
Regional Distribution ◽  
Normal Growth ◽  
Nutritional Intervention ◽  
Postnatal Period ◽  
The Body ◽  
Neonatal Rat ◽  
Cardiac Muscle Cell ◽  
Serial Sectioning ◽  
Enzymatic Isolation

The numbers of nuclei per cardiac muscle cell were determined in adult mammalian hearts after previous enzymatic isolation of individual myocytes. A high percentage of binucleation was observed in rat (78 ± 1.8%), rabbit (78 ± 0.8%), guinea pig (81 ± 1.6%), cat (76 ± 1.5%), dog (47 ± 2.4%,), and beef (45 ± 1.1%), while the human myocytes were found predominantly mononucleated (90%) and only 10 ± 2.2% of them were binucleated. These findings are in agreement with our data obtained after serial sectioning of the myocardium of the above species and subsequent reconstruction of individual myocytes. We also found that in fetal and neonatal rat the cardiac myocytes are predominantly mononucleated (95% and 84%, respectively), that the binucleation develops during the early postnatal period, and that adult values are reached by 12–14 days of age. When the body growth and consequently also the heart growth were either accelerated or slowed down by nutritional intervention during the above period, the rate of appearance of binucleation correlated with the organ size rather than with the age of the rat. Early postnatal increase in binucleation was also observed in rabbit, cat, and dog during normal growth. The regional distribution of the above phenomenon was found to be uniform throughout both ventricles.

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