Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Over the past 40 years, a small but increasing number of fetal genetic and congenital anomalies has become amenable to in utero treatment. Successful fetal therapies have included open procedures for congenital diaphragmatic hernia, cystadenomatoid malformation of the lung and saccrococygeal teratoma, shunts for uropathies and thoracic fluids, pharmacological therapies for congenital adrenal hyperplasia and neural tube defect prevention, and the stem cell treatment of severe combined immunodeficiency disorder.
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PMID:Fetal therapy. 1247 47

Neonatal clitoromegaly is mainly attributed to in utero androgen exposure secondary to congenital adrenal hyperplasia. We report on 2 extremely premature girls with clitoromegaly, increased androgen levels, no salt wasting syndrome, and ovarian cyst. In case 1, the cyst liquid was aspired during ovarian hernia surgery and revealed high androgen levels. After aspiration, serum androgen levels decreased, as did clitoral size. In case 2, an ovarian cyst was seen on pelvic ultrasound. Aspiration was not indicated. The cyst regressed spontaneously on iterative pelvic ultrasounds, and her clitoromegaly decreased. Case 1 demonstrates the ovarian origin of this transient virilization. Cyst formation seems to be linked to the physiologic maturation of the hypothalamic-pituitary-ovarian axis. Thirteen cases of clitoromegaly with hyperandrogenism, without salt wasting syndrome, have been reported in extremely premature infants. In the context of clitoromegaly, we recommend ruling out in utero androgen exposure, adrenal hyperandrogenism, and disorders of sex development. We further recommend affirming hyperandrogenism by androgen assay and confirming ovarian origin with gonadotrophin assays and pelvic ultrasound. Drug therapy abstention and clinical and ultrasound monitoring are recommended because spontaneous regression of clitoral hypertrophy seems to be the most common outcome in the literature, as it was in our 2 observations.
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PMID:Case Report of Clitoral Hypertrophy in 2 Extremely Premature Girls With an Ovarian Cyst. 2893 75