Endocrine studies of the ovulatory disturbances in Wilson’s disease (hepatolenticular degeneration)

Kaushansky, Arieh; Frydman, Moshe; Kaufman, H.; Homburg, Roy

doi:10.1016/s0015-0282(16)50004-1

Cited by 51 publications

(36 citation statements)

References 11 publications

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“…In the present study—compared to women on treatment for WD—women with undiagnosed WD had a significantly increased rate of spontaneous abortions. This is in line with seminal work highlighting oligomenorrhea or amenorrhea as a disease‐associated symptom in untreated female WD patients with WD . Nonetheless, Tarnaka et al reported a relatively low spontaneous abortion rate of 19.5% in 31 untreated WD patients with 82 pregnancies .…”

Section: Discussionsupporting

confidence: 76%

“…Overall, our data support the conclusion that the spontaneous abortion rate is high in undiagnosed WD patients. Proposed pathophysiological explanations for diminished fertility and increased spontaneous abortions are decreased levels of estradiol and increased testosterone concentrations secondary to liver dysfunction and finally leading to impaired ovarian function . This is supported by the observation of diminished ovarian follicular aromatase activity, whereas an ovarian copper overload is not evident in rodent WD models (copper content in ovaries of LEC rats was shown to be not elevated; Schilsky ML, unpublished observation).…”

Section: Discussionmentioning

confidence: 99%

“…Other disease manifestations in females include amenorrhea or oligomenorrhea, possibly related to disturbed hormonal activity. These are common findings in female patients and may even be the first symptoms leading to the diagnosis of WD . Conception can be impaired and recurrent miscarriages have been reported …”

mentioning

confidence: 99%

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Pregnancy in Wilson's disease: Management and outcome

Pfeiffenberger¹,

Beinhardt

Gotthardt³

et al. 2018

Hepatology

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Pregnancy in WD patients on anticopper therapy is safe. The spontaneous abortion rate in treated patients was lower than that in therapy-naïve patients. Although the teratogenic potential of copper chelators is a concern, the rate of birth defects in our cohort was low. Treatment for WD should be maintained during pregnancy, and patients should be monitored closely for hepatic and neurological symptoms. (Hepatology 2018;67:1261-1269).

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Section: Discussionsupporting

confidence: 76%

Section: Discussionmentioning

confidence: 99%

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Pregnancy in Wilson's disease: Management and outcome

Pfeiffenberger¹,

Beinhardt

Gotthardt³

et al. 2018

Hepatology

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“…Menstrüel düzensizlikler ve amenore sık karşılaşılan komplikasyonlardır. Tedavi almayan olgularda gebelik çoğunlukla düşükle sonuçlanmaktadır (5). Menstrüel bozukluklar ve kronik karaciğer hastalığının erken başlangıçlı olmasından dolayı güvenli ve başarılı gebelik nadirdir (6).…”

Section: Introductionunclassified

Wilson Hastalığı Olan Gebelerin Obstetrik Sonuçları / Obstetric Outcomes in Pregnant Women with Wilson’s Disease

Akduman¹,

Oral²,

Özerkan³

2016

Anadolu Kliniği Tıp Bilimleri Dergisi

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Wilson Hastalığı bulunan annelerin gebeliğine ait obstetrik sonuçları sunmak. Gereç ve Yöntemler: Kliniğimizde takip edilmiş olan 4 gebe kadının geçirmiş olduğu toplam 6 gebeliğe ait kayıtlar retrospektif olarak tarandı. Bu gebelerin özellikleri, gebeliklerinin klinik seyirleri, gebeliklerinde uygulanan tedavi yöntemleri ve fetal-maternal sonuçlar literatür eşliğinde değerlendirildi. Bulgular: Gebelerin medyan yaşı ve doğum anındaki medyan gebelik haftası sırasıyla 33,5 yıl (25-37) ve 19 hafta (10-38) olarak saptandı. Üç gebelik 1. trimesterde, bir gebelik 2. trimesterde ve iki gebelik de 3. trimesterde sonlandırıldı. Sonlandırılma sebepleri ablasyo plasenta, akut fetal distres, missed abortus ve terapötik abortus olarak gözlendi. Tartışma ve Sonuç: Wilson hastalığı biliyer bakır atılım bozukluğu sonucu özellikle karaciğer, beyin, böbrek ve korneada toksik miktarda bakır birikmesi ile karakterize otozomal resesif genetik bir hastalıktır. İnfertilite ve düşüklere neden olmaktadır. Wilson hastalığı gebelikte komplikasyonlarla seyredebilen bir hastalıktır. Maternal ve fetal komplikasyonları en aza indirmek için hastalar tedavi ve takipleri açısından yakın gözlem altında bulundurulmalıdır.

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“…Therapy aims at a reduction of tissue copper deposits and a binding of copper to the protein metallothionein in the liver. Until specific treatments became available successful pregnancy was a rare occur rence except in patients who remained asymptomatic well into adult life [1,2]. Most of the patients treated for Wil son's disease become clinically asymptomatic and they can live a normal reproductive life.…”

Section: Introductionmentioning

confidence: 99%