Alagille syndrome and pregnancy: anesthetic management for cesarean section

Rahmoune, F.C.; Bruyère, M.; Técsy, M; Benhamou, Dan

doi:10.1016/j.ijoa.2011.07.012

Cited by 8 publications

(3 citation statements)

References 24 publications

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“…For these reasons (and to minimize maternal efforts), delivery via cesarean section or assisted vaginal delivery (forceps or vacuum) has been suggested as the delivery route of choice specifically in pregnant ALGS patients with intracranial vasculopathy, clinically significant portal hypertension, and severe cardiac disease [ 78 , 79 ]. Unassisted vaginal delivery however, has been successful in ALGS expectant women without these issues [ 80 , 81 ].…”

Section: Alagille Syndrome and Pregnancymentioning

confidence: 99%

Management of adults with Alagille syndrome

et al. 2023

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Alagille syndrome (ALGS) is a complex rare genetic disorder that involves multiple organ systems and is historically regarded as a disease of childhood. Since it is inherited in an autosomal dominant manner in 40% of patients, it carries many implications for genetic counselling of patients and screening of family members. In addition, the considerable variable expression and absence of a clear genotype–phenotype correlation, results in a diverse range of clinical manifestations, even in affected individuals within the same family. With recent therapeutic advancements in cholestasis treatment and the improved survival rates with liver transplantation (LT), many patients with ALGS survive into adulthood. Although LT is curative for liver disease secondary to ALGS, complications secondary to extrahepatic involvement remain problematic lifelong. This review is aimed at providing a comprehensive review of ALGS to adult clinicians who will take over the medical care of these patients following transition, with particular focus on certain aspects of the condition that require lifelong surveillance. We also provide a diagnostic framework for adult patients with suspected ALGS and highlight key aspects to consider when determining eligibility for LT in patients with this syndrome.

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Section: Alagille Syndrome and Pregnancymentioning

confidence: 99%

Management of adults with Alagille syndrome

et al. 2023

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“…Alagille syndrome. Alagille syndrome is an autosomal dominant disease caused by mutations on the JAG1 or NOTCH2 genes and is characterized by intrahepatic biliary hypoplasia and congenital cardiovascular, renal, eye and skeletal malformations (44). Case reports suggest increased risk of miscarriage, preterm labor, intrauterine growth restriction, and neonatal death, possibly related to the inheritance of Alagille syndrome by the fetus (4,44).…”

Section: Preexisting Bile Acid Disorders In Pregnancymentioning

confidence: 99%

Pregnancy and bile acid disorders

Pataia

Dixon

Williamson

2017

American Journal of Physiology-Gastrointestinal and Liver Physi

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During pregnancy, extensive adaptations in maternal metabolic and immunological physiology occur. Consequently, preexisting disease may be exacerbated or attenuated, and new disease susceptibility may be unmasked. Cholestatic diseases, characterized by a supraphysiological raise in bile acid levels, require careful monitoring during pregnancy. This review describes the latest advances in the knowledge of intrahepatic cholestasis of pregnancy (ICP), the most common bile acid disorder specific to pregnancy, with a focus on the disease etiology and potential mechanisms of ICP-associated adverse pregnancy outcomes, including fetal demise. The course of preexisting cholestatic conditions in pregnancy is considered, including primary sclerosing cholangitis, primary biliary cholangitis, biliary atresia, and Alagille syndrome. The currently accepted treatments for cholestasis in pregnancy and promising new therapeutics for the condition are described.

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“…The pulmonary artery stenosis is usually peripheral, but sometimes central, valvular, supravalvular, or even hypoplastic or atresic [ 4 ]. Local peripheral pulmonary artery stenosis accounts for 75–90%, while diffuse pulmonary artery stenosis and pulmonary artery hypoplasia are uncommon, but capable of causing pulmonary artery hypertension [ 7 ]. Complex congenital heart defects, such as tetralogy of Fallot and pulmonary atresia, have been described in Alagille syndrome, which are proven to be associated with increased morbidity and mortality [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

Pulmonary artery pathologies in Alagille syndrome: a meta-analysis

Yuan¹

2022

pwki

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Alagille syndrome, caused by mutations in the gene encoding Jagged1 (JAG1), a ligand in the Notch signaling pathway, is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, face and skeleton. The aim of the present study is try to disclose the clinical features, management and outcomes of pulmonary artery stenosis associated with Alagille syndrome. By comprehensive literature retrieval, 38 articles involving 401 patients were recruited for this study. The pertinent variables closely related to pulmonary artery stenosis in patients with Alagille syndrome were comprehensively analyzed by following the PRISMA guidelines. The management of pulmonary artery pathologies, especially a severe type of pulmonary artery stenosis in Alagille syndrome, is a concerned matter. Publications of literature retrieval of recent 3 decades were the study material of this article. The pulmonary artery pathologies, especially the severe type of pulmonary artery stenosis in Alagille syndrome, warrant surgical or interventional treatments. After the procedures, the right ventricular to left ventricular pressure ratio was reduced by 25%. There were no intergroup differences in terms of recovery, reintervention and mortality rates between interventionally and surgically treated patients. Transcatheter treatment is preferable due to less trauma. Surgical treatment of pulmonary artery stenosis can be performed currently with intracardiac defect repair.

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Alagille syndrome and pregnancy: anesthetic management for cesarean section

Cited by 8 publications

References 24 publications

Management of adults with Alagille syndrome

Management of adults with Alagille syndrome

Pregnancy and bile acid disorders

Pulmonary artery pathologies in Alagille syndrome: a meta-analysis

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