Cancer-prone syndrome of mosaic variegated aneuploidy and total premature chromatid separation: report of five infants.

Five infants (two girls and three boys) from four families all had severe pre- and postnatal growth retardation, profound developmental delay, microcephaly, hypoplasia of the brain with Dandy-Walker complex or other posterior fossa malformations, and developed uncontrollable clonic seizures. Four infants developed Wilms tumors, and one showed cystic lesions in bilateral kidneys. All five infants showed variegated mosaic aneuploidy in cultured lymphocytes. In two infants whose chromosomes were prepared by us, 48.5%-83.2% lymphocytes showed total premature chromatid separation (PCS). Their parents had 3.5%-41.7% of their lymphocytes in total PCS. The remaining three infants and their parents, whose chromosomes were prepared at outside laboratories, tended to show lower frequencies of total PCS. Another five infants reported with the disorder were reviewed together with the five infants we described. Together, their clinical and cytogenetic manifestations were similar enough to suggest a syndrome. Seven of the 10 infants developed proven or probable Wilms tumors. The age at diagnosis of the tumors was younger than usual at 2-16 months. The tumors were bilateral in four infants and unilateral in three infants, and cystic changes were present in six infants. Two infants developed botryoid rhabdomyosarcoma. The carriers of the syndrome are thus liable to tumorigenesis. The possible role of mitotic checkpoint defects, proven in two infants with the syndrome (Matsuura et al. [2000: Am J Hum Genet 69:483-486]), was discussed in connection with tumor development and progression.

Radical hysterectomy: An anatomic evaluation of parametrial dissection.

OBJECTIVES: This study was designed to demonstrate a reduction in the amount of blood loss for vesicouterine ligament dissection and to investigate the intrapelvic autonomic nerve pathway and its preservation by means of anatomic analysis. METHODS: The anchoring mechanism of the pelvic viscera to the pelvic wall was divided into a supporting system facing laterally and a suspensory system facing dorsoventrally. An operative procedure was designed in which both systems were separated and dissected independently. RESULTS: Between the two systems, an artificial space was developed, which required a new dissection method for the parametrium and revealed a new anatomic pathway for the ureter and autonomic nerve. The amount of blood loss (mean +/- SD) during dissection of the vesicouterine ligament was ultimately 260.1 +/- 114.8 ml. Postoperatively, the maximum capacity of the bladder was 393.9 +/- 40.4 ml, maximum detrusor pressure 6.3 +/- 4.1 cm H(2)O, mean compliance >10 ml/cm H(2)O, residual urine 23.8 +/- 9.4 ml, and maximum flow rate 25. +/- 8 2.2 ml/s, respectively. CONCLUSION: A new classification for the parametrium and its dissection method have been established. Development of this new operative procedure has also contributed to a decrease in blood loss and preservation of bladder function.

[Decreasing seropositivity of cytomegalovirus of pregnant women in Japan].

We examined seroprevalence of cytomegalovirus (CMV) of pregnant women for 18 years since 1980 with complement-fixing antibody (CF) and specific IgG antibody. CF seropositive rate decreased gradually from 93.2% to 66.7%. CMV-IgG Seropositive rates were 87.4% in 1985, and 75.2% in 1996 to 1997. Age and parity of pregnant women associated with the immune status; 35.6% of recent young primipara were susceptible.

A new proposal for radical hysterectomy.

OBJECTIVE: The authors revised the surgical procedure for radical hysterectomy utilizing detailed observation of the venous system, connective fascial sheets, and neural pathways within the uterine supports. STUDY DESIGN: The anterior, middle, and posterior uterine supports were reclassified into two systems, supporting or fascial and drainage or areoral. Supporting system consisted of the superficial layer of the vesicouterine ligament, fascial part of the Mackenrodt ligament, sacrouterine ligament, and rectovaginal ligament, whereas drainage system consisted of the deep layer of the vesicouterine ligament, vascular part of the Mackenrodt ligament, and the so-called mesoureter. The operative procedure was planned according to the continuity of these ligaments and executed first by excising the fascia and then dissecting the denuded areoral tissue. RESULTS: Among the 15 patients who underwent surgery for uterine cancer during a 2-year period the mean (SD) time required for the operation was 305.5 +/- 30.5 min and the mean (SD) total volume of blood loss 592.0 +/- 238.2 ml. A mean (SD) period of 14.3 +/- 3.8 postoperative days was required until the volume of the residual urine decreased to less than 50 mL. CONCLUSION: The present operation has been structured more three-dimensionally and systematically than before. Further, safety of the operation was significantly improved including prevention of hemorrhage and preservation of bladder function.

[Simplified avidity assay of rubella IgG antibody in rubella virus infection].

Simplified avidity assay of rubella IgG antibody with urea was evaluated to distinguish primary rubella from reinfection. In this method urea washing was done once for 10 minutes. The avidity index (AI) was calculated as the optical density percentage for the urea-washed well when compared to that of the non-treated well. We examined 292 sera from 50 patients with primary infection collected 6 to 2,259 days after the rash appeared, 29 sera from 11 patients with rubella reinfection and 69 sera from 68 pregnant women without fetal infection and having a high hemagglutination inhibition (HI) antibody. In primary infection AI increased gradually from 0%, and reached a plateau of about 60% four months after the rash appeared, whereas the mean AIs of patients after reinfection and with high HI antibody were as high as 87.1% and 89.9%, respectively. These results indicate that the simplified avidity assay in rubella IgG antibody is also valuable in diagnosing recent primary rubella in pregnant women with a high HI antibody.

Predicting long-term results of trabeculectomy from early postoperative intraocular pressure levels.

BACKGROUND AND OBJECTIVES: The authors retrospectively examined the potential for early postoperative intraocular pressure to predict the long-term results of initial trabeculectomies. PATIENTS AND METHODS: The records of 173 patients (207 consecutive eyes) who underwent initial trabeculectomies, which were performed by one of the authors between 1983 and 1991, with a minimum follow-up of 150 days were reviewed. Cases of trabeculectomy combined with cataract extraction were excluded. RESULTS: The intraocular pressure during postoperative week 1 was the same for patients with successful initial trabeculectomies (success group) as it was for patients with unsuccessful trabeculectomies (failure group). However, the intraocular pressure during the second, third, and fourth weeks was significantly higher in the failure group (P < .001). Laser suture lysis and 5-fluorouracil had no influence on the fact that high intraocular pressure during the first postoperative week had no prognostic significance. CONCLUSION: Positive results on postoperative Seidel tests did not predict a poor prognosis. However, high intraocular pressure after the first week may require intervention because it predicts a poor prognosis.

A retrospective study of the effects of laser suture lysis on the long-term results of trabeculectomy.

We retrospectively examined the effects of laser suture lysis on the long-term results of initial trabeculectomies in 130 consecutive eyes that had an intraocular pressure greater than 21 mm Hg during the first 4 postoperative weeks. After the high intraocular pressure was noted, 46 eyes underwent laser suture lysis; the other 84 did not. The 2-year cumulative probability of success in the laser suture lysis group was .84 and, in the control group, .82 (P > .05). Intraocular pressures in the two groups 1 and 2 years after trabeculectomy were similar. Laser suture lysis had no discernible effect on the long-term success rate of trabeculectomy in these patients.

[Avidity of rubella IgG antibody in rubella virus infection].

The avidity assay of rubella IgG antibody with urea was evaluated to distinguish the early convalescent phase of primary rubella from the late one and reinfection. We examined 116 sera from 23 patients with primary infection collected 7 to 1,477 days after the rash appeared and 25 sera from 8 patients with rubella reinfection and 16 sera from 15 pregnant women without fetal infection having high hemagglutination inhibition (HI) antibody or weak positive rubella IgM antibody. The avidity index (AI) was calculated as the ratio of the optical density from the urea-washed well to that of the non-treated well, expressed as a percentage. In primary infection AI were lower than 30% within 30 days after the rash appeared, then gradually increased and reached a plateau of 60 to 80% three months later, whereas the AIs of patients after reinfection, with high HI antibody and weak positive IgM antibody were as high as 79.2 +/- 7.5%, 93.5 +/- 4.1% and 90.6 +/- 5.0%, respectively. These results indicate that the measurement of avidity in rubella IgG antibody is valuable in diagnosing recent primary rubella in pregnant women having a high HI antibody or IgM antibody.

Posttrabeculectomy anterior subcapsular cataract formation induced by anterior chamber air.

We retrospectively examined the effect of air, as contrasted with balanced salt solution (BSS), sodium hyaluronate, or no substance at all, used to reform the anterior chamber at the end of trabeculectomy on subsequent cataract formation in 141 consecutive patients (173 phakic eyes). The minimum follow up was 120 days. The use of air increased the incidence of anterior subcapsular cataract formation significantly. Also, the eyes that had a large air bubble on postoperative day 1 had a higher incidence of anterior subcapsular cataract than the eyes with less air on that day. We suggest that the toxicity to the lens of the oxygen in the air increased the incidence of anterior subcapsular cataract in the eyes in which air was used. We recommend that BSS or sodium hyaluronate be used instead of air to reform the anterior chamber following trabeculectomy.

Dissection of the cardinal ligament in radical hysterectomy for cervical cancer with emphasis on the lateral ligament.

Surgical experience with carcinomas of the uterus and rectum has provided new insights into the surgical anatomy of a lamina, which separates the paravesical space from the pararectal space. It has been proved that each of the lamina consists of the cardinal and lateral ligaments and pelvic splanchnic nerves, descending in the following order. The cardinal and lateral ligaments, as a connective stalk, insert into the lateral walls of the uterus and rectum extending from the inner aspect of the pelvic wall. Clarification of this structural relationship led to the development of a new procedure for the dissection of the cardinal ligament in radical hysterectomy, while still preserving the lateral ligament. This facilitated systematic dissection of the cardinal and uterosacral ligaments with posterior manipulation, leading to a reduction in blood loss and to prevention of brisk bleeding from the venous plexuses.

[Studies on the management of pregnant women having high hemagglutination inhibition (HI) antibody titer of rubella virus in the first trimester examination of rubella virus antibody in the pregnant women having high HI titer by different methods and prognosis of their infants after birth].

Several serological studies of rubella virus (RV) infection were carried out on sera of 45 pregnant women having RV hemagglutination inhibition (HI) antibody titer more than 1:512 in the first trimester, including a follow-up study of these women's pregnancies. In order to detect RV-specific IgM antibody, various procedures were employed as follows: Complement fixation test (CF), HI test after treatment of the sera with Protein-A (ProA-HI), enzyme linked immunosorbent assay (ELISA) by indirect or sandwich procedures and several tests using fractionated sera by sucrose density gradient ultracentrifugation (SDG). Positive ratios found with the CF test and ProA-HI were 26.7% and 20.0%, respectively. In indirect ELISA, 4 cases were positive for RV-specific IgM, but all sera were negative in sandwich ELISA and SDG tests. Both RV isolation from urine of some infants and detection of high RV-specific IgM antibody in cord sera failed to be positive. Of 44 pregnancies, 4 cases resulted in spontaneous abortion and all others in normal delivery. These forty normal infants had no sign of congenital rubella syndrome, but one case among them showed polysyndactyly . The results obtained here seemed to indicate that sandwich ELISA is the most valuable and convenient method among the tested procedures for clinically determined diagnosis of recent RV infection in pregnant women having a high HI antibody titer.

[Antibody response to live rubella vaccination and its clinical meanings].

Serological antibody response for over a year to rubella live virus vaccination was examined by hemagglutination inhibition (HI) test and enzyme-linked immunosorbent assay (ELISA) of 22 seronegative and 25 seropositive sera of young women. In the seronegative group, some HI antibody response appeared first 2 weeks after the vaccination. A positive response of ELISA-IgM was first observed at 3 weeks, showing slowly declining values within the following year. Similarly, ELISA-IgG response occurred first and was positive 3 weeks later with gradual increases in both ELISA-values and positive ratios for 5 months. In the seropositive group, responses of both ELISA-IgM and -IgG were found within a few levels of ELISA-values from 2 weeks after the vaccination. Higher ELISA-IgM values observed after the natural infections than those following in the vaccination should be cited. These possibly show different amounts of viral antigens introduced in vivo. Reinfection of high HI antibody cases may be distinguished by small changes in the ELISA-value's pattern. When the above data are taken together, single measurement of ELISA-IgM does not seem to always be definitive and characteristic changes in positive patterns may be much more important in clinically diagnosing recent rubella virus infection.

Primary trabecular carcinoid of the ovary.

A case of primary trabecular carcinoid of the ovary apparently occurring from the foregut element of a mature cystic teratoma was reported. The tumor was found incidentally and resected surgically from a 56-year-old female. The cystic teratoma found in the tumor was composed of only respiratory epithelium except a colliculus which showed a picture of tonsil-like tissue. No thyroid tissue was found. The trabeculae of the tumor cells were longer and wavier near the cystic teratoma, while they were straight and short at the periphery. The tumor cells had argyrophil granules but negative in argentaffin granules. The electronmicroscopy revealed many round neurosecretory granules measuring from 170 to 290 nm in diameter. The histogenesis was also discussed.

[Clinical evaluation of sulbactam/cefoperazone in the field of obstetrics and gynecology].

One or 2 grams of sulbactam/cefoperazone (SBT/CPZ) in 250 ml of 5% glucose solution was administered twice daily by drip infusion to 10 patients with female genital organ infections. The clinical effectiveness was seen in 8 patients but not in 2 patients associated with emergence of replaced organism or non-eradicated causative organism. Antibacterial activity of SBT/CPZ vs. CPZ in the 20 clinical isolates revealed that SBT/CPZ was 1 or 3 tubes superior to CPZ alone in the beta-lactamase producing organisms, but, 1 or 2 tubes inferior to CPZ in the beta-lactamase non-producing organisms. In 1 case increases in GOT and GPT, and in another increases in GOT and GPT, and a decrease in platelets were observed even though their degrees were mild.

[Therapeutic effect of T-1982 (cefbuperazone) on the gynecologic infectious diseases].

T-1982 (cefbuperazone), a new cephamycin antibiotic with broad spectrum against Gram-positive, negative aerobic and anaerobic organisms, was clinically and bacteriologically evaluated on the gynecologic infectious diseases. Fourteen cases hospitalized at Kanazawa Medical University Hospital and the affiliated hospitals from October 1981 to July 1982 were treated with T-1982. By clinical symptoms, signs and bacteriological examinations the patients were diagnosed as pelveoperitonitis (1), intrauterine infection (3), adnexitis (3), infectious diseases of external genitalia (4), infectious abortion (1), vulvar hematoma (1), and rectovaginal fistula (1). T-1982 was administered intravenously or by drip infusion at a dose of 0.5-2.0 g twice a day after dissolved in a saline solution or a 5% glucose solution. Based on the improvement of clinical findings and antibacterial effect of T-1982, results were evaluated as excellent, good, poor and unknown. Clinical effects more than good were shown in 9 of 11 cases which could be followed up exactly on the therapeutic of T-1982. Two cases showing poor response were pelveoperitonitis and pyometra under carcinoma colli uteri stage IIIb, respectively. In 8 of 11 cases, antibacterial effect of T-1982 could be evaluated. Thirteen strains of aerobic and anaerobic bacteria were disappeared by T-1982 therapy, while no effect was seen on 5 strains. On the side effect of T-1982, exanthema was observed in 1 case but disappeared soon after the cessation of administration.