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)

A 15-year-old, legally male patient came to our department with chief complaint of gynecomastia. Serum testosterone was at a low level of 1.6 ng/ml, and prolactin a high level of 23 ng/ml. Blood type was a mixed type of both type A and type B, and a chromosomal analysis with peripheral blood lymphocytes demonstrated a mosaic of 46, XX/46, XY. During the follow-up, he complained a painful swelling in his right scrotum, and received an emergent surgery. A large amount of blood was noted in the right scrotum. Unicorn uterus, Fallopian tube and finbriae were observed, and a thumb-sized gonad with hemorrhage and fissure was also seen in the upper part of the scrotum. The right gonad was an ovary and no testicular tissue was confirmed in the right scrotum, whereas the right vas deferens was noted. The left testis was accompanied by an induration on its upper pole which was histologically found to be ovarian tissue. The patient was diagnosed as a true hermaphroditism with 46, XX/46, XY chimera that had an ovary with inguinal uterus hernia and an unusual vas deferens in the right scrotum and an ovotestis in the left. It was considered that an adequate amount of testosterone secreted from the left testis during the early embryonal period might have affected the descent of the right ovary into the scrotum and on the development of the right vas deferens.
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PMID:[True hermaphrodite with ipsilateral vas deferens and intrascrotal ovary]. 177 99

True hermaphroditism, a very rare cause of intersex, is usually diagnosed during the newborn period in the course of evaluating ambiguous genitalia. As an exception we report an unusual case of a 14.5 year-old boy with phenotypically near-normal male genitalia and bilaterally descended gonads, who was seen for evaluation of gynecomastia and hematuria. His eunuchoid body habitus and mild mental retardation were compatible with Klinefelter's syndrome. He had a low level of free testosterone (15.2 pmol/l), and high level of estradiol (264.3 pmol/l) for his age. The patient was diagnosed as true hermaphroditism with 46,XX /47,XXY karyotype causing an ovotestis with inguinal uterus hernia in the left scrotum and a dysgenetic testis in the right scrotum.
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PMID:True hermaphroditism with characteristics of Klinefelter's syndrome: a rare presentation. 1604 29

An 18-year-old phenotypic male presented with an irreducible left inguinal mass, gynecomastia, and hypospadias. This mass on exploration was found to be a nonfunctional uterus with ipsilateral ovary and was excised. Further investigation confirmed the presence of a contralateral testis and a genotype of 46, XX. This confirmed the diagnosis of ovotesticular disorder of sexual differentiation (formerly true hermaphroditism) with obstructed hernia uteri inguinalis. The patient was raised as a male. Subcutaneous mastectomy for gynecomastia and neourethra construction with full thickness skin graft for hypospadias were performed. Hernia uteri inguinalis is rarely seen in this condition with only 2 cases being reported worldwide thus far, including our case.
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PMID:Hernia uteri inguinalis in a case of ovotesticular disorder of sexual differentiation. 2333 99