Congenital Heart Diseases associated with Identified Syndromes and Other Extra-Cardiac Congenital Malformations in Children in Lagos

Ekure, Ekanem N.; Animashaun, A.; Bastos, Marcus Gomes; Ezeaka, Chinyere

doi:10.4314/wajm.v28i1.48422

Cited by 20 publications

(23 citation statements)

References 9 publications

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“…These individuals often have structural abnormalities of these organs [21, 24, 34, 70]. In a study of Apert syndrome, it was found that cardiac anomalies and genitourinary anomalies were present in 10 and 9.6 % of patients, respectively [24].…”

Section: Managementmentioning

confidence: 99%

Impact of genetics on the diagnosis and clinical management of syndromic craniosynostoses

2012

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Purpose More than 60 different mutations have been identified to be causal in syndromic forms of craniosynostosis. The majority of these mutations occur in the fibroblast growth factor receptor 2 gene (FGFR2). The clinical management of syndromic craniosynostosis varies based on the particular causal mutation. Additionally, the diagnosis of a patient with syndromic craniosynostosis is based on the clinical presentation, signs, and symptoms. The understanding of the hallmark features of particular syndromic forms of craniosynostosis leads to efficient diagnosis, management, and long-term prognosis of patients with syndromic craniosynostoses. Methods A comprehensive literature review was done with respect to the major forms of syndromic craniosynostosis and additional less common FGFR-related forms of syndromic craniosynostosis. Additionally, information and data gathered from studies performed in our own investigative lab (lab of Dr. Muenke) were further analyzed and reviewed. A literature review was also performed with regard to the genetic workup and diagnosis of patients with craniosynostosis. Results Patients with Apert syndrome (craniosynostosis syndrome due to mutations in FGFR2) are most severely affected in terms of intellectual disability, developmental delay, central nervous system anomalies, and limb anomalies. All patients with FGFR-related syndromic craniosynostosis have some degree of hearing loss that requires thorough initial evaluations and subsequent follow-up. Conclusions Patients with syndromic craniosynostosis require management and treatment of issues involving multiple organ systems which span beyond craniosynostosis. Thus, effective care of these patients requires a multidisciplinary approach.

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Section: Managementmentioning

confidence: 99%

Impact of genetics on the diagnosis and clinical management of syndromic craniosynostoses

2012

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“…[5] Congenital heart disease (CHD) accounts for 8 to 12/1,000 live births. [13] In Egypt despite the routine recognition about Discharge care provided after heart surgery to help mothers in caring for their children, the mothers still needs program care to help them after open heart surgery with support, guidance, and prepare to care for their children to overcome this period successfully which provide the mothers with knowledge and practice for improving quality of care of their children after discharge from the hospital.…”

Section: Introductionmentioning

confidence: 99%

The effect of pre-hospital discharge care program on mothers’ knowledge and reported practice for children with congenital heart surgery

Elsobky

Amer²,

Sarhan³

2018

JNEP

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Heart surgery in children is done to repair heart defects for a child born with congenital heart defects and heart diseases. The aim of the study was to evaluate the effect of pre-hospital discharge care program on mothers' knowledge and reported practice regarding discharge care of children after congenital heart surgery. The study was conducted in the Heart Surgery Department at Abo-EL Riesh University Children Hospital. The sample included 30 mothers and their children that were available during the period of data collection. Three tools were used to collect data including: Tool I: Interviewing questioner sheet of the participants categorized into two parts. Part I and part II: Personal characteristics of the children and their mothers. Tool II: Assessment of mothers' knowledge regarding care of children after congenital heart surgery. Tool III: Mother's reported care practice checklist. Results: The results of the present study revealed that there was a highly significant difference in all items of care knowledge (activity, food, medication and follow-up) before, after immediately and after one month from discharge program with highly significant statistically (p < .001). Discharge program improve mothers' knowledge and practice regarding care of their children after congenital heart surgery. Recommendations: Comprehensive, multidisciplinary discharge planning should deign early and should include the mothers and children contain education regarding congenital heart surgery care.

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“…[6,7]. This predicts the true prevalence of congenital heart defects in the southwestern zone of Nigeria as both studies were in same geopolitical zone and state.…”

Section: Discussionmentioning

confidence: 99%

“…The most prevalent heart defects detected in each syndrome/malformations were documented; however, their Page 2 of 5 associations were not statistically stated. Also, the study did not assess risk factors associated with congenital malformations [6].…”

Section: Introductionmentioning

confidence: 99%

Structural Cardiac Abnormalities in Children with Congenital Malformations in Lagos

Animasahun¹,

Oa²,

Oy³

2017

J Mol Genet Med

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Congenital Heart Diseases associated with Identified Syndromes and Other Extra-Cardiac Congenital Malformations in Children in Lagos

Abstract: Maladies cardiaques congénitales associées à des syndromes identifiés et extra-Malformations cardiaques congénitales chez les enfants à Lagos

Cited by 20 publications

References 9 publications

Impact of genetics on the diagnosis and clinical management of syndromic craniosynostoses

Impact of genetics on the diagnosis and clinical management of syndromic craniosynostoses

The effect of pre-hospital discharge care program on mothers’ knowledge and reported practice for children with congenital heart surgery

Structural Cardiac Abnormalities in Children with Congenital Malformations in Lagos

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