Prenature ovarian failure is a condition causing amenarrhea, hypoestrogenism, and elevated genadotropins in women younger than 40 years. A karyotype should be performed as part of basic laboratory evaluation for all patients with premature ovarian failure and prodromal premature ovarian failure. Development of a malignancy in a dysgenetic gonad is of major concern. The presence of a fragment of the Y chromosome is thought to be a key to the oncogenic potential of these gonads. The search for the testicular determining factor(TDF) has engendered much confusion about which part of the Y chromosome plays a role in malignancy. This was initially postulated to be the H- Y antigen. More recent data, however, localize the area near the centromere of the Y Chromosome, on the long arm(Yq). Malignant potential is clearly not linked to the testicular determining factor itself(SRY). This is a critical point in clinical medicine. Feilure to display SRY or a closely related sequence does not rule out the presence of the segment of the Y chromosome postulated to be associated with the development of malignancies. We have experienced a case of premature ovarian failure with chtomosomal abnormality involving Y chromosome fragment. So we report this case with a brief review of literatures.
Centromere ; Clinical Medicine ; Female ; Gonadal Dysgenesis* ; Gonads* ; Humans ; Karyotype ; Primary Ovarian Insufficiency* ; Y Chromosome

Polycystic ovary disease is a heterogenous endocrinopathy with many interacting causal factors. One potential such factor is chronic hyperinsulinemia. multiple, independent lines of evidence suppart the contention that chronic hyperinsulinemia causes ovarian hyperandragenism. This evidence includes: (1) mutations in the insulin receptor gene that cause severe hyperinsulinemia appear to be associated with ovarian hyperandrogenism, (2) insulin stimulates ovarian thecal and sttomal androgen seaetion in vitro, and (3) in some experimental models, manipulation of circulating insulin concentrations results in changes in circulating androgens. Although the association between hyperinsulinemia and hyperandrogenism remains to be fully explained at the molecular level, chronic hyperinsulinemia appears to be an important cause of hyperandrogenism. We have experienced a case of HAIR AN syndrome showing hyperandrogenism, insulin resistance and acanthosis nigricans in infertile patient. So we report this case with a brief review of literatures.
Acanthosis Nigricans ; Androgens ; Female ; Fibrinogen ; Hair* ; Humans ; Hyperandrogenism ; Hyperinsulinism ; Insulin ; Insulin Resistance ; Models, Theoretical ; Ovary ; Receptor, Insulin

Familial benign chronic pemphigua is a rare disease characterized by a localized recurrent eruption of grouped vesicles and exudative lesions involving the neck, groin, axillae and intertriginous areas. There are no systemic manifestations but chronicity and pruritic lesions creates a significant social and psychological handicap to those afflicted. We presented a case of familial benign chronic pemphigus in a 39 years old man He has had recurrent vesicles, fissures and crust formation in inguinal area for 10 years. Light microscopic findings showed suprabasal vesiculation with acantholysis and villi formation. And electron-microscopic findings showed as follows: Bizzare microvilli changes with widened intercellular spaces, followed by loss of desmosome and complete acantholysis. Tonofilament-desmosome complex is disrupted. Many of acantholytic cells are associated with abnormal keratinization which is repreaented by perinuclear thickened bundles of tonofilament arranged in whorls or lumps. Authors diagnosed with clinical symptoms, light and electron-microscopic findings as Hailey-Hailey disease. The lesions were improved by systemic antibiotics and steroid with cold soaking.
Acantholysis ; Adult ; Anti-Bacterial Agents ; Axilla ; Desmosomes ; Extracellular Space ; Groin ; Humans ; Intermediate Filaments ; Microvilli ; Neck ; Pemphigus, Benign Familial* ; Rare Diseases

Fibrous histiocytomas are characterized by a variable combination of cells with fibroblastic and histiocytic features. They exhibit a distinctive but inconstant cellular arrangement referred to as a storiform or cartwheel pattern. Considerable corfusion has enveloped these tumors as a result of their various terminology-fibrous histiocytoma, dermatofibroma, sclerosing hemangioma, fibroxanthoma, subepidermal nodular fibrosis, xanthogranuloma, giant cell tumor of tendon sheath. A histologically benign but locally infiltrating, 15 * 20cm sized, fibrous histiocytoma was found in the posterior aspect of left thigh of a 17-year-old man. The tumor was incompletely excised due to adhesion of the tumor mass to sciatic nerve and then radiation therpy was combined, Histogenesis and prognostic aspects of the tumor are discussed.
Adolescent ; Fibroblasts ; Fibrosis ; Giant Cell Tumors ; Histiocytoma ; Histiocytoma, Benign Fibrous* ; Humans ; Sciatic Nerve ; Tendons ; Thigh

Primary signet ring cell carcinoma of the prostate is extremely rare and about 18 cases have been reported in the literature. We report two cases of primary signet ring cell carcinoma of the prostate, arising in 79-year-old and 65-year-old men. Both cases were the poorly differentiated adenocarcinoma of the prostate with many signet ring cells. Signet ring cells were positive for prostatic specific antigen and prostatic acid phosphatase but negative for neutral and acid mucins. In summary, the signet ring cell carcinoma of the prostate is a rare variant of poorly differentiated adenocarcinoma of the prostate. The orgin of the prostate should be considered in cases of metastatic signet ring cell carcinoma, particularly when the signet ring cells are negative for neutral and acid mucins. Prostatic specific antigen and prostatic acid phosphatase should also be performed to confirm the primary signet ring cell carcinoma of the prostate.
Acid Phosphatase ; Adenocarcinoma ; Aged ; Carcinoma, Signet Ring Cell* ; Humans ; Male ; Mucins ; Prostate*

BACKGROUND: Osteopontin (OPN) is a cytokine associated with a cell-matrix via integrins. Fibroblast specific protein-1 (FSP-1), known as S100A4, has been implicated in cell migration by non-muscle myosin. We investigated whether the role of OPN and FSP-1/S100A4 expression in their contribution to the podocyte phenotype change to form podocyte bridge and cellular crescent. METHODS: Glomerular expression of OPN and FSP-1/S100A4 in renal biopsies of 16 patients with crescentic glomerulonephritis (CrGN) and 13 normal renal biopsies were studied by immunohistochemistry. RESULTS: The expression of OPN and FSP-1/S100A4 was increased in the podocytes of glomeruli, with and without crescents, in patients with CrGN. Neither OPN nor FSP-1/S100A4 was expressed in glomeruli from the normal controls (p<0.01). A significant positive correlation was found between the expression of OPN in glomerular tufts and cellular crescents, and the expression of OPN and FSP-1/S100A4 in glomerular tufts (p<0.05). CONCLUSIONS: The results suggest that OPN plays a role in early podocyte attachment to Bowman's capsule, and FSP-1/S100A4 potentiate podocyte contribution to cellular crescent formation by inducing cellular migration and growth.
Biopsy ; Bowman Capsule ; Cell Movement ; Fibroblasts ; Glomerulonephritis ; Humans ; Integrins ; Myosins ; Osteopontin ; Phenotype ; Podocytes

BACKGROUND: Osteopontin (OPN) is a cytokine related to cell-matrix adhesion and cell survival and is expressed in the distal convoluted tubules in normal adult kidneys. Only one in vitro study has investigated the role of OPN in mechanically stretched podocytes and their actin cytoskeleton rearrangement. METHODS: Glomerular OPN expression was investigated in biopsies from patients with human idiopathic focal segmental glomerulosclerosis (FSGS) (n = 25) and in normal renal biopsies (n = 16) by immunohistochemistry. RESULTS: OPN was expressed in the podocytes from patients with FSGS. OPN expression increased in podocytes from both non-sclerotic hypertrophic and sclerotic glomerular tufts in patients with FSGS compared to the podocytes in normal controls. CONCLUSIONS: The results suggest that OPN plays a role in the early adaptive response of podocytes to the increased mechanical load caused by glomerular hypertrophy preceding FSGS. OPN was involved in cell-matrix adhesion and influenced the detachment delay of podocytes from the glomerular basement membrane and apoptosis.
Actin Cytoskeleton ; Adult ; Apoptosis ; Biopsy ; Cell Survival ; Glomerular Basement Membrane ; Glomerulosclerosis, Focal Segmental ; Humans ; Hypertrophy ; Immunohistochemistry ; Kidney ; Osteopontin ; Podocytes

No abstract available.
Female ; Hypertension, Pregnancy-Induced* ; Pregnancy ; Pregnancy* ; Prolactin* ; Umbilical Cord*

Infertility, defined as 1 year of unprotected coitus without conception, affects approximately 10 to 15% for couples of reproductive age. Approximately 35% of these cases are attributable to male factor infertility. A major cause of male infertility is chromosome abnormality, such as 47 chromosomes with an XXY karyotype. Early surveys of infertile males showed that the incidence of major chromosome abnormality in infertile males in azoospermic patients. When patients are treated for male infertility, a chromosome analysis including a search for abnormality at the DNA level, should be performed. We have experienced a case of autosomal reciprocal translocation in azoospermic patient. So we report this case with a brief review of literatures.
Azoospermia ; Chromosome Aberrations ; Coitus ; DNA ; Family Characteristics ; Fertilization ; Humans ; Incidence ; Infertility ; Infertility, Male ; Karyotype ; Male

Aneuploidy results from nondisjunction in either the meiotic division of the parents or the early cleavage divisions of the affected individuals. The sex chromosomes show a wide range of viable aneuploidy than do the autosomes. The incidence of 47,XXY and 47,XYY children increases with maternal age, as does that of autosomal trisomies, whereas the incidence of 45,X children does not increase with maternal age. In the group of sex chromosome aueuploidies, the 47,XXY and 47,XYY conditions occur with nearly equal hequency at birth. Translocations between X or Y chromosomes and an autosome or between an X chromosome and the Y chromosome cause sterility in human males. It has been assumed that a translocation involving either(or both) of the sex chromosomes would interfere with inactivation of the XY bivalent and thaeby disturb spermatogenesis. We bave experienced a case of Y-autosome translocation in azoospermic patient. So we report this case with a brief review of literatures.
Aneuploidy ; Azoospermia* ; Child ; Humans ; Incidence ; Infertility ; Male ; Maternal Age ; Parents ; Parturition ; Sex Chromosomes ; Spermatogenesis ; Trisomy ; X Chromosome ; Y Chromosome