Uterus didelphys with unilateral imperforate vagina and ipsilateral renal agenesis

Uterus didelphys with unilateral imperforate vagina is rare. Interestingly, in all reported cases in which the urinary tract was investigated renal agenesis was found on the side of the obstructed hemivagina. We report 2 cases of this unusual and interesting condition, and review the embryogenesis of müllerian duct abnormalities associated with renal agenesis, as well as the clinical findings, diagnosis and management. We stress the importance of prompt and accurate diagnosis, and treatment as a means to avoid unnecessary surgical procedures.     

Prostatic carcinoma in a young adult: a case report]

We report a case of carcinoma of the prostate in a 30-year-old man. Serum acid phosphatase was normal. A transrectal biopsy of the prostate demonstrated an undifferentiated carcinoma. Total prostatocystectomy was performed and subsequent pathologic report stated that the mass was an undifferentiated carcinoma of the prostate gland. Metastases to the intrapelvic lymph node were present. Although immunohistochemical prostatic acid phosphatase (PAP) activity was not demonstrated, prostatic specific antigen (PSA) staining revealed a positive reaction within the tumor cells, confirming prostatic carcinoma. The patient's course has been uneventful without any recurrence by the intermittent adjuvant chemotherapy 8 months postoperatively. Review of the literature in Japan disclosed 16 cases (including our case) of carcinoma of the prostate in patients under 40 years of age.     

Cataractogenesis in neonatal Sprague-Dawley rats by N-methyl-N-nitrosourea

Cataract was induced by a single intraperitoneal injection of 100 mg/kg N-methyl-N-nitrosourea (MNU) to 0-, 5-, 10-, 15-, or 20-day-old male and female Sprague-Dawley rats. In day 0, 5, 10, and 15 MNU-treated rats, mature cataracts were constantly seen 7, 14, 14, and 30 days after dosing, respectively. In the day 20 MNU-treated rats, only subcapsular cataract was seen 30 days after dosing. Therefore, the rats exposed to MNU at an earlier age caused cataract more rapidly and severely. In the day 0 MNU-treated rats, 7-methyldeoxyguanosine DNA adduct was detected in the lens epithelial nuclei 12 hours after MNU dosing, followed by apoptosis, which was confirmed by morphology, by TUNEL signals, and by DNA ladder and peaked 3 days after MNU dosing. In the apoptosis cascade, upregulation of Bax, downregulation of Bcl-2, and increased CPP32 protease (caspase-3) activity were seen 12 hours after MNU dosing. Therefore, the pathogenesis of MNU-induced cataract was associated with DNA adduct formation in the lens epithelial cell nuclei leading to apoptosis by upregulation of Bax protein, downmodulation of Bcl-2 protein, and activation of caspase-3.     

Pigmentary degeneration indwed by N-methyl-N-nitrosourea and the fate of pigment epithelial cells in the rat retina

Pigmentary degeneration of the retina was induced by a single intraperitoneal Injection of 75mgkg of N-methyl-N-nitrosourea (MNU) In female Brown-Norway colored rats at 50 days of age, which were then observed at 24, 48 and 72 h and 7, 21,35 and 150 days after the treatment. MNU-treated rats showed selective destruction of the photoreceptor cells by an apoptotic mechanlsm 24 h after the treatment, and the destruction was completed by day 7. During the photoreceptor cell degeneration, proliferation of Miller cells and infiltratlon of macrophages was prominent 72h and 21 days aRttr the treatment, respectively. Müller cell proliferation and macrophage infiltratbn corresponded to degenerative photo-receptor cell phagocytosis, and prollferating Müller cell processes responded to stabilize the damaged retina. Pigment epithelial cell detachment from the Bruch's membrane was seen 72 h after the treatment, and migration within all layers of the retina was seen at day 7 when photoreceptor Cells were lost. At 21, 35 and 150 days after the treatment, lack of photoreceptor cells and deposition of pigment epithelial cells within the retina but not in contact to vascular endothe-lial cells were characteristic. MNU-induced photoreceptor apoptosis followed by Miiller cell and macrophage reaction then pigment epithellal cells deposition withln the retina partially resembles retinitis pigmentosa in humans.     

Juvenile hyaline fibromatosis: A report of two unrelated adult sibling cases and a literature review

Two unrelated adult sibling cases (36- and 32-year-old females) of Juvenile hyaline fibromatosis are presented. The parents of one of these patients were non-consanguineous but natives of a small Island, and one elder sister among four siblings was affected with the same disease. The parents of the other patient were consanguineous, and one other sibling suffered from the identical disease. Both patients presented with multiple subcutaneous nodules, which they had had since infancy, and had undergone numerous surgical excisions. Light microscopy examination of skin lesions from both patients showed identical histology; an abundance of a homogenous, amorphous, eosinophlllc extracellular matrix in which spindle-shaped cells were embedded. Electron microscopically, the spindle-shaped cells had hypertrophic Golgi apparatus and dilated, rough endoplasmlc reticulum. Fine flbrillar and granular material-filled structures, the contents of which were occasionally released into the extracellular matrix, were also seen, immunohistochemically, the spindle-shaped cells were vlmentin-positive but negative for α-smooth muscle actln and S-100 protein, and the hyaline ground substance was positive for type I and type III collagen but negative for type II and type IV collagen and tenascin. Matrix metalloprotelnase-1, -2, and -9, and tissue inhibitor of matrix metalloproteinase (TlMP)-2 was positive but TIMP-1 was negative. A review of 39 cases of juvenile hyaline fibromatosis In the literature is also presented. In summary, skin lesions may be the most outstanding symptoms of juvenile hyaline fibromatosis, but joint contracture and gingival hypertrophy precede the skin manifestation.     

Expression and localization of matrix metalloproteinases and tissue inhibitors of metalloproteinases as a prognostic factor in advanced colorectal carcinomas

To clarify the usefulness of matrix metallo-proteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) as prognostic factors in advanced colorectal carcinoma, the immunohistochemical expressions of MMP-1, MMP-2, MMP-3, MMP-9, TIMP-1 and TIMP-2 were examined. Specimens were selected from 67 consecutive patients undergoing surgery for advanced colorectal carcinoma. The patterns of expression were compared with the prognoses of the patients. The patients with TIMP-2 expression in stroma adjacent to the tumor mass had better prognoses than those of the patients who had no TIMP-2 expression in normal stroma adjacent to the tumor (p<0.05), which probably acted as a block of cancer cell invasion. However, the expression of MMP-2, presumably acting as an antagonist to TIMP-2 was not related to the prognosis, and the MMP-1, MMP-2, MMP-3, MMP-9 and TIMP-1 expressions were not related to any clinicopathological factors examined.     

Lectin bindings in non-neoplastic esophageal epithelium and esophageal carcinomas

Lectin binding was examined histochemically in 22 cases of primary esophageal carcinomas (10 well differentiated, 8 moderately differentiated and 3 poorly differentiated squamous cell carcinomas, and 1 undifferentiated carcinoma) and was compared with the adjacent non-neoplastic epithelium by means of a panel of 10 different lectins (RCA-I, WGA, Con A, LCA, SEA, UEA-I, HPA, PNA, DBA and GS-I) on formalin-fixed paraffin-embedded sections. In the non-neoplastic epithelium, RCA-I and WGA showed basal/parabasal binding, Con A, LCA, SEA, UEA-I, HPA and PNA revealed prickle cell binding, while DBA and GS-I only stained the surface cells of the squamous cell layer. In squamous cell carcinomas, no clear difference was evident regarding the grade of differentiation. However, basal/parabasal specific lectins were expressed in all the cases, the prickle cell-specific lectins were expressed less frequently, whereas lectins expressed at the surface cells of the squamous cell layer were only infrequently expressed. Therefore, basal/parabasal cell specific lectins were widely preserved in squamous cell carcinomas. One case of undifferentiated cancer tested was devoid of all the lectins.     

Correlation of anatomic and hemodynamic features with aortic valve leaflet deformity in doubly committed subarterial ventricular septal defect

Summary The records of 153 patients with doubly committed subarterial ventricular septal defect (DCVSD) who underwent intracardiac repair were analyzed to evaluate factors responsible for aortic valve leaflet deformity. The patients were divided into two groups according to their echocardiographic and angiographic features as well as anatomic findings at operation: DCVSD without (17/153, 11.1%) and with arterial valve offsetting (136/153, 88.9%). Aortic regurgitation (AR) was much more prevalent in the patients with (50.0%) than in those without leaflet deformity (2.2%,P < 0.01). Arterial valve offsetting is one of the major contributing factors to the development of leaflet deformity, accounting for 5.9% in the patients without offsetting and 46.3% in those with offsetting (P < 0.01). Among the patients with arterial valve offsetting, the pulmonary-to-systemic pressure ratio was significantly higher (P < 0.01) in the patients without (0.76 ± 0.14) than in those with leaflet deformity (0.36 ± 0.12), suggesting that pulmonary hypertension might prevent the aortic valve leaflet from prolapsing in DCVSD. In addition, increased severity of aortic valve leaflet deformity and subsequent AR were observed with increasing age. These results suggest that aging and the presence of arterial valve offsetting as well as the absence of pulmonary hypertension might be factors responsible for aortic valve leaflet deformity and subsequent AR in DCVSD. The anatomic and hemodynamic features in DCVSD have a great impact on the development of aortic valve leaflet deformity and subsequent AR.     

Prevalence,implication, and determinants of worsening renal function after surgery for congenital heart disease

Accumulating data in adults indicate the prognostic importance of worsening renal function (WRF) during treatment of acute heart failure. Venous congestion appears to play a dominant role in WRF; however, data regarding WRF in children with congenital heart disease (CHD) are limited. The present study was conducted to elucidate the prevalence and characteristics of WRF after surgery for CHD in children. We also tested our hypothesis that, similar to adult heart failure, venous congestion is an important determinant of WRF independent of cardiac output in this population. Fifty-five consecutive pediatric patients who underwent cardiovascular surgery for CHD were studied (median age 0.7 years; range 3 days to 17 years). The degree of WRF was assessed by the difference between the maximum levels of postoperative serum creatinine (Cr) and preoperative serum Cr. There was a high prevalence of WRF in the present cohort: an increase in Cr level was observed in 47 patients (85 %) and a Cr increase ≥0.3 mg/dL was seen in 23 (42 %). Importantly, WRF was significantly associated with a worse clinical outcome of a longer stay in the intensive care unit and hospital (both p < 0.05), even after controlling for age and operative factors. In addition, multivariate regression analysis revealed that central venous pressure, rather than cardiac output, was an independent determinant of WRF. Postoperative management to relieve venous congestion may help ameliorate or prevent WRF and thereby improve outcomes in patients with CHD.