Three autopsy cases of arthrogryposis multiplex congenita are studied. They were two deadborns and one neonatal death. All of them had characteristic abnormalities involving multiple joints. Neither primary myopathy nor abnormalities of anterior horn cells of the spinal cord were detected in our cases. However, two cases had minor central nervous system anomalies. All four cases showed pulmonary hypoplasia of varying degree. Two of three cases showed facial dysmorphism such as micrognathia and low set ears, and one showed cleft lip and palate. Ventricular septal defect, umbilical hernia and ureteral anomalies were also associated.
Infant, Newborn ; Humans

Body stalk anomaly represents an extreme maldevelopment of embryonic body folding and is characterized by absence of the umbilicus and umbilical cord. The failure of complete obliteration of the extraembryonic coelom is responsible for the absence of the umbilical cord formation and the wide-based insertio of the amnioperitoneal membrane onto the placental chorionic plate. We have analyzed 10 autopsy cases of various midline anomalies of the body that could best be classified into body stalk anomaly. All cases were either stillborns or dead immediately after birth. The pregnancy was interrupted due to this anomaly in 6 cases, and their gestational ages varied from 17 weeks to 37 weeks. The affected fetuses were characterized bt absent or vestigial umbilical cord, and ruptured amnion with direct amnioperitoneal connection without the mediation of the umbilical cord. Exomphalos with abdominal wall defect and serve scoliosis were characteristic components of this anomaly, that provided important clues in differentiating other similar anomalies. Other associated anomalies included neural tube defect, intestinal atresia, genitourinary and skeletal defects, pulmonary hypoplasia, single umbilical artery and narrow-spaced chest and abdomen, etc. These findings strongly suggest that anomaly of body stalk represents mechanical teratogenesis due to early amnion repture and subsequent effect, and should be categorized into amniotic band disruption syndrome.
Pregnancy ; Female ; Humans ; Teratogens

There had been a continuous evolution of lymphoma classification and recently a Revised European-American Lymphoma Classification was proposed by the International Lymphoma Study Group. This new classification often requires information on immunophenotypic and molecular biologic markers in addition to the usual histologic findings. Recent advances in the production of commercially available monoclonal antibodies reactive on formalin-fixed paraffin-embedded tissues provide us a great help to classify the non-Hodgkin's lymphoma. We have analyzed 31 low grade B-cell lymphomas by the schemes proposed by the International Lymphoma Study Group using antibodies to CD3, CD5, CD20, CD23, CD43, cyclin D1, and bcl-2 protein, and have analyzed the immunophenotypic features. Among 31 low grade B-cell lymphomas, 8 small lymphocytic lymphomas, 5 mantle cell lymphomas, 7 follicle center lymphomas (2 grade I, 3 grade II, and 2 grade III), and 11 marginal zone B-cell lymphomas (all of which were extranodal) were identified. Among 8 small lymphocytic lymphomas, 5 cases were positive for CD5; 6 cases were positive for CD23; 7 cases were positive for CD43; all 8 cases were negative for cyclin D1; and 7 cases were positive for bcl-2. Among 5 mantle cell lymphomas, 4 cases were positive for CD5 and CD43; all five cases were negative for CD23; 4 cases were positive for cyclin D1 and bcl-2. All 7 follicle center lymphomas were negative for CD5, CD43 and cyclin D1 and 2 cases were positive for CD23; and 6 cases were positive for bcl-2. All marginal zone B-cell lymphomas were negative for CD5, CD23 and cyclin D1; 3 cases were positive for CD43 and 9 cases were positive for bcl-2. Diagnostic utility for CD5 antigen detection on paraffin embedded tissue has a limitation due to weak antigen expression in tumor cells of B-cell lymphomas; however, still be useful in differentiating small lymphocytic lymphoma and mantle cell lymphoma from other B-cell lymphomas when applied in conjunction with CD43. CD23, CD43, and cyclin D1 appear to be of great help in differentiating subgroups of low grade B-cell lymphomas. Bcl-2, as known, is found to be useful to rule out reactive follicular hyperplasia.
Antibodies ; Antibodies, Monoclonal ; Antigens, CD5 ; B-Lymphocytes* ; Biomarkers ; Classification ; Cyclin D1 ; Hyperplasia ; Immunohistochemistry* ; Leukemia, Lymphocytic, Chronic, B-Cell ; Lymphoma ; Lymphoma, B-Cell* ; Lymphoma, B-Cell, Marginal Zone ; Lymphoma, Mantle-Cell ; Lymphoma, Non-Hodgkin ; Paraffin*

No abstract available.
Encephalitis* ; Encephalitis, Herpes Simplex*

A 63-year-old male patient with extensive carcinoma in situ of the urinary bladder was found to have unsuspected transitional cell carcinoma of the prostate. Mapping of the totally embedded radical cystectomy specimen demonstrated diffuse, multifocal, epithelial abnormalities, ranging from mucosal atypia to the nonpapillary carcinoma in situ with extension to the urethra, prostatic ducts and glands, seminal vesicles and ureter, probably reflecting individual urothelial susceptibility in reaction to carcinogenic stimulus. The importance of prostatic assessment in the evaluation of the patient with carcinoma in situ of the urinary bladder is emphasized.
Male ; Humans

We studied distribution of fibronectin in paraquat-induced adult fibrotic lung by indirect immunofluorescence and indirect immunoperoxidase methods, using affinity-purified antifibronectin IgG peroxidase conjugates and antifibronectin IgG FITC conjugates. In contrast to the relative paucity of staining in normal lung, there was a marked increase in interstitial staining for fibronectin in this fibrotic lung. This marked alterations in the apparent amounts and distribution of fibronectin in fibrotic human lung suggest its involvement in the cellular events accompanying human lung fibrosis.
Adult ; Male ; Female ; Humans

Gastric lesion in familial polyposis coli is commonly presented with fundic gland hyperplastic polyps, but duodenal and gastric adenomas together with their carcinomatous transformation have been rarely described in familial polyposis coli mostly by case reports. We present three cases of gastric adenomas in familial polyposis coli with one in synchronous development of gastric adenocarcinoma in Korea. All three cases had the family history related to familial polyosis coli and received proctocolectomy because of synchronous development of colonic adenocarcinoma. One patient developed gastric polyposis and adenocarcinoma 8 years after colectomy, and the remaining two presented with multiple polyps either in the stomach or duodenum synchronously at the time of the diagnosis of familial polyposis coli with colonic adenocarcinoma. None disclosed any evidence of Gardner's syndrome. We conclude that association of gastric adenomas in familial oplyposis coli is not uncommon and gastric adenoma-carcinoma sequence is an another important participating mechanism to understand the histogenesis of gastric carcinoma in Korea.
Adenocarcinoma ; Adenoma

No abstract available.
Poisoning*

OBJECTIVE: To evaluate the clinical effect and reproductive outcome of pelviscopic ovarian drilling in infertile patients with polycystic ovarian syndrome resistant to clomiphene citrate. METHODS: Twenty two patients involved in this study were taken pelviscopic ovarian drilling with needle electrocautery. Change of characteristic LH/FSH ratio before and after operative treatment, ovulation and pregnancy success were followed up. Paired t-test was applied for hormonal change (p<0.05). RESULTS: Thirteen of 14 patients (93%) treated only with pelviscopic ovarian drilling showed successful ovulation with or without clomiphene citrate and among these 10 patients (71%) were success in pregnancy and 9 patients delivered full term babies. When including patients who needed other assisted reproductive technique and specific medication of endometriosis 17 of 22 patients (77%) were successfully pregnant and 15 patients (68%) delivered healthy babies. The mean LH/FSH ratio 2.0+/-0.5 before treatment were decreased to 1.26+/-0.37 after treatment (p<0.05). Interestingly, among 22 patients, various degree endometriosis were found during pelviscopy and specific medication with GnRH analogue were followed. CONCLUSION: Pelviscopic ovarian drilling showed successful ovulation rate and effective reproductive outcome in clomiphene-resistant infertile patients with PCO. This surgical technique seems to be an alternative step for the management of clomiphene-resistant infertile patients with PCO and also for the diagnosis of possible high rate of endometriosis in these patents.
Clomiphene* ; Diagnosis ; Electrocoagulation ; Endometriosis ; Female ; Gonadotropin-Releasing Hormone ; Humans ; Needles ; Ovulation ; Polycystic Ovary Syndrome* ; Pregnancy ; Reproductive Techniques, Assisted

Beckwith-Wiedemann syndrome is a rare clinical entity characterized by exomphalos, macroglossia, macrosomia, and renal hyperplasia/dysplasia. Although its entity is established, its etiology and obligatory features have not been settled. We report an autopsy case with the unusual involvement of the salivary gland. This infant was born to a 37-year-old mother as a normal full-term spontaneous delivery. At 11 days of age she developed with purulent eye discharge and weak sucking, and died suddenly. At autopsy the baby weighed 2,630 gm and the head circumference was 35 cm. She showed thick and prominent skin folds, bilateral aural fissures, macroglossia, hepatomegaly, cardiomegaly, dysmorphic kidneys, and nesidioblastosis. Both kidneys showed dysplastic tubules and hyperplastic cortical tissue enclosing the medulla. In this case there were characteristic findings in major and minor salivary glands with both acinar and ductal hyperplasia, and hypertrophy of mammary glands. Besides, she had generalized depletion of subcutaneous fat, immature buccal fat, patent ductus arteriosus, hyperlobation of the right lung, two accessory spleens, and hyperplasia of basophils and chromophobes in the pituitary gland. The lungs showed diffuse interstitial pneumonia and multiple fibrin thrombi. There were no adrenal cytomegaly, umbilical hernia and exophthalmos.
Infant ; Male ; Female ; Humans