hydroxyprogesterone
Sources réglementaires consultées
Indications approuvées
- Traitement palliatif de l’adénocarcinome avancé du corps utérin de stade III ou IV.
- Aménorrhée primaire ou secondaire et saignement utérin anormal dû à un déséquilibre hormonal, en l’absence de pathologie organique.
- Test de la production endogène d’œstrogènes et de la production d’un endomètre sécrétoire avec desquamation ; il ne doit pas être utilisé comme test diagnostique de grossesse.
Contre-indications
Absolues
- Cancer du sein actuel ou antérieur ou autre néoplasie hormonodépendante, sauf dans l’indication oncologique décrite.
- Saignement génital non diagnostiqué, maladie hépatique, avortement retenu, grossesse, hypersensibilité ou thrombose actuelle ou antérieure.
Mises en garde cliniques
- Mise en garde majeure · Arrêter en présence de signes de thrombose artérielle ou veineuse, de troubles visuels ou neurologiques, de réaction allergique grave ou d’altération de la tolérance au glucose. — FDA/DailyMed, set_id 687bcf42-f437-42c7-8909-a91f621f55df
- Mise en garde majeure · Le schéma oncologique est palliatif et ne remplace pas le traitement établi des stades I ou II. — FDA/DailyMed, set_id 687bcf42-f437-42c7-8909-a91f621f55df
Effets indésirables
Communs (≥1%)
Nausées ou gêne gastro-intestinale · Œdème ou variations de poids · Saignements irréguliers · Douleur mammaire · Céphalées · Douleur au site d’injection
Rares mais graves
Thrombose veineuse ou artérielle · Réaction d’hypersensibilité sévère
Grossesse et allaitement
La notice concerne les femmes non enceintes et contre-indique l’utilisation pendant la grossesse. La source locale ne fournit pas de recommandation suffisante pour l’allaitement ; la décision revient à l’équipe clinique.
Bibliographie récente (PubMed)
We describe congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, which is the most common primary adrenal insufficiency in children and adolescents. In this comprehensive review of CAH, we describe presentations at different life stages depending on disease severity. CAH is characterized by androgen excess secondary to impaired steroidogenesis in the adrenal glands. Diagnosis of CAH is most common during infancy with elevated 17-hydroxyprogesterone levels on the newborn screen in the United States. However, CAH can also present in childhood, with late-onset symptoms such as premature adrenarche, growth acceleration, hirsutism, and irregular menses. The growing child with CAH is treated with hydrocortisone for glucocorticoid replacement, along with increased stress doses for acute illness, trauma, and procedures. Mineralocorticoid and salt replacement may also be necessary. Although 21-hydroxylase deficiency is the most common type of CAH, there are other rare types, such as 11β-hydroxylase and 3β-hydroxysteroid dehydrogenase deficiency. In addition, classic CAH is associated with long-term comorbidities, including cardiometabolic risk factors, impaired cognitive function, adrenal rest tumors, and bone health effects. Overall, early identification and treatment of CAH is important for the pediatric patient.
Twins represent 3.2% of all live births; however, they account for 20.0% of all preterm deliveries, with 60.0% and 10.7% of twins delivered before 37 and 32 weeks' gestation, respectively. Twin pregnancies have 5 times higher risk of early neonatal and infant death related to prematurity. Monochorionic twins have a higher incidence of both indicated and spontaneous preterm delivery than dichorionic twins. Transvaginal ultrasound of the cervical length before 24 weeks' gestation is the best tool to predict preterm birth, independent of other risk factors. Among all evaluated therapies to decrease or prevent preterm birth in twin pregnancies, the use of vaginal progesterone in women with a transvaginal cervical length of <25 mm decreased neonatal morbidity, and physical examination-indicated cerclage in women with a cervical dilation of >1 cm showed a significant decrease in preterm birth at different gestational ages and perinatal mortality.
To compare the efficacy of bed rest, cervical cerclage (McDonald, Shirodkar, or unspecified type of cerclage), cervical pessary, fish oils or omega fatty acids, nutritional supplements (zinc), progesterone (intramuscular, oral, or vaginal), prophylactic antibiotics, prophylactic tocolytics, combinations of interventions, placebo or no treatment (control) to prevent spontaneous preterm birth in women with a singleton pregnancy and a history of spontaneous preterm birth or short cervical length. Systematic review with bayesian network meta-analysis. The Cochrane Pregnancy and Childbirth Group's Database of Trials, the Cochrane Central Register of Controlled Trials, Medline, Embase, CINAHL, relevant journals, conference proceedings, and registries of ongoing trials. Randomised controlled trials of pregnant women who are at high risk of spontaneous preterm birth because of a history of spontaneous preterm birth or short cervical length. No language or date restrictions were applied. Seven maternal outcomes and 11 fetal outcomes were analysed in line with published core outcomes for preterm birth research. Relative treatment effects (odds ratios and 95% credible intervals) and certainty of evidence are presented for outcomes of preterm birth <34 weeks and perinatal death. Sixty one trials (17 273 pregnant women) contributed data for the analysis of at least one outcome. For preterm birth <34 weeks (40 trials, 13 310 pregnant women) and with placebo or no treatment as the comparator, vaginal progesterone was associated with fewer women with preterm birth <34 weeks (odds ratio 0.50, 95% credible interval 0.34 to 0.70, high certainty of evidence). Shirodkar cerclage showed the largest effect size (0.06, 0.00 to 0.84), but the certainty of evidence was low. 17OHPC (17α-hydroxyprogesterone caproate; 0.68, 0.43 to 1.02, moderate certainty), vaginal pessary (0.65, 0.39 to 1.08, moderate certainty), and fish oil or omega 3 (0.30, 0.06 to 1.23, moderate certainty) might also redu
Crinecerfont (CRENESSITY™) is a corticotropin releasing factor type 1 (CRF1) receptor antagonist developed by Neurocrine Biosciences for the treatment of classic congenital adrenal hyperplasia (CAH) in adult and paediatric patients. In patients with classic CAH, circulating levels of adrenocorticotropic hormone (ACTH), androstenedione and 17-hydroxyprogesterone are elevated, which traditionally has required supraphysiologic doses of glucocorticoids to manage. As a CRF1 receptor antagonist, crinecerfont acts by reducing systemic ACTH secretion to subsequently decrease elevated levels of steroid precursors and adrenal androgens, thereby reducing the dosage of glucocorticoids required to manage androgen levels in patients. This article summarizes the milestones in the development of crinecerfont leading to this first approval as an adjunctive treatment to glucocorticoid replacement to control androgens in adults and paediatric patients aged ≥ 4 years with classic CAH.
The most common form of congenital adrenal hyperplasia (CAH) is secondary to 21-hydroxylase deficiency (21OHD). This review will summarize the clinical manifestations, recommended treatments, monitoring, clinical challenges and management strategy, and treatment challenges in special situations for infants and children with classic CAH due to 21OHD. Specifically, we review newborn screening and the initial diagnosis, glucocorticoid and mineralocorticoid treatment, and recommended monitoring, including anthropometric and laboratory measures. Children with CAH may have premature adrenarche, precocious puberty, and early growth plate closure and have an increased risk of hypertension and overweight/obesity. Many 46,XX individuals will also have genital differences, which may include clitoromegaly and/or a urogenital sinus. We review psychosocial and surgical considerations, including suggestions on how to talk with children, family, and caregivers about bodily difference. These suggestions may be used by families and/or providers caring for individuals with CAH.