A case with dicentric translocation between chromosome 9 and 18: Confirmation by fluorescent in situ hybridization on metaphase spread

A female child with dicentric translocation between chromosome 9 and chromosome 18 presented non-specific minor anomalies with laryngomalacia. Chromosomal analyses were performed by the G-banding method and a fluorescence in situ hybridization (FISH) technique with a specific probe for the centromeric region of chromosome 18 and the painting probe for the chromosomes 9 and 18. Her full karyotype was confirmed as 45, XX, tdic (9;18)(p24;p11). This is the first case of dicentric translocation between chromosomes 9 and 18. The FISH technique is an important tool in chromosome diagnostics.     

Immunohistochemical Study of Serum Proteins in Normal and Cadmium-Treated Mouse Testis by in vivo CryotechniqueImmunohistochemical Study of Serum Proteins in Normal and Cadmium-Treated Mouse Testis by in vivo Cryotechnique

Objective To investigate time-dependent changes of serum proteins permeability in the normal and cadmium（Cd）-treated mouse testis, reflecting tight junctional （TJ） barriers of Sertoli cells Methods The serum proteins, albumin and immunoglobulin-G（IgG）, in the seminiferous tubules were firstly immobilized by the “in vivo cryotechnique”, in which the dynamic blood circulation was always kept. The cryofixed testicular tissues were then processed for the freeze-substitution method, and embedded in the paraffin wax. Serial sections of 5μm thickness were immunostained by anti-mouse albumin or IgG antibody with peroxidase immunostaining, and also stained with hematoxylin-eosine （HE）for morphological observation. Results In normal seminiferous tubules, albumin immunoreaction products were localized around peritubular myoid cells and among Leydig cells, as well as in blood vessels. They were also localized as arch-like patterns around some spermatogonia in basal compartments. The number of the immunopositive arch structures was different according to developmental stages of the seminiferous cycle, judging from the arrangement of germ cells by HE-staining. The patterns of localization of IgG immunostaining in normal mouse testis were similar to that of albumin. In 24 h after Cd-treatment, some enlarged spaces and vesicular formation in the seminiferous epithelium were observed on the paraffin sections by HE-staining. The albumin or IgGimmunolocalization was seen not only in the basal compartments, but also in the adluminal compartments between Sertoli.cells and germ cells. Conclusion The structural changes of inter-Sertoli TJ barriers in vivo, such as different immunostaining patterns of serum proteins between the normal and Cd-treated mouse seminiferous tubules, could be clearly detected by the “in vivo cryotechnique” with albumin or IgG immunohistochemistry.     

Inflammatory esophagogastric polyp and fold in an adolescent

The case of a 13 year old boy with an inflammatory esophagogastric polyp and ulcerative colitis is described. Endoscopy revealed a typical polyp and gastric fold complex at the esophagogastric junction and a hiatal hernia. Histology of a biopsy specimen confirmed an inflammatory polyp covered by hyperplastic squamous and gastric foveolar epithelium. Continuous 24 hour esophageal manometry suggested gastroesophageal reflux, which may be related to the pathogenesis of the lesion. Follow-up endoscopy showed marked regression of the polyp with medication for reflux eosphagitis. This clinical entity is rare in childhood and adolescence, and the manifestations may not be readily recognized. Therefore, endoscopic biopsy is important in children with esophageal polyps. However, polypectomy is unnecessary except when malignancy is suspected or when symptoms persist.     

Acute childhood leukemia in a patient with hemophilia: First report in Japan

This study reports an unusual case of acute leukemia which was diagnosed as hemophilia A on initial admission for leukemia. A 3 year old boy was admitted to Kagoshima University Hospital with anemia. He was diagnosed as acute lymphoblastic leukemia. At the same time he was revealed to have severe hemophilia A⁻ without any previous episodes of severe bleeding tendency or family history of this disease. The laboratory investigation showed his mother to be a carrier of hemophilia A. Although there are many cases of hemophilia which have developed malignant tumors, most of them were caused by association with human immunodeficiency virus (HIV) infection. Only five cases with coexistence of leukemia and hemophilia without HIV infection have been reported and the present case is the first one in Japan. At this stage, hemophiliacs are not necessarily regarded to be a population at risk for the development of leukemia. Furthermore, no particular subtype of leukemia was characterized among these patients in the literature.     

Intraspinal Metastasis of Neuroblastoma -- Report of a Case Detected at Autopsy --

Metastasis to the brain or spinal cord parenchyma is extremelyrare in cases of neuroblastoma. We present a 3-year-7-month-old boy with neuroblastoma, stageIV, with intraspinal metastasis. He had no neurologic manifestationexcept incontinentia urinae and ataxia at the terminal stage.His cranial computed tomography scan showed high density areasin both cerebellar hemispheres which seemed to be distant metastaticmasses. At autopsy, the metastases in the cerebellum were foundto be due to dural infiltration but in the spinal cord parenchymaof the lumbar spine metastases were detected macroscopically.There were multiple distant metastatic areas near the roots,anterior and posterior. The neuroblastoma seemed to have spreadalong the roots by direct invasion from the meninges. In the future, the number of patients with metastasis into thebrain or spinal cord parenchyma will increase because patientswith progressive disease could live for a long time as a resultof intensive chemotherapy. Observation of these cases will behelpful to clarify the routes of metastasis to these areas.     

Two Autopsy Cases of Diffuse Gastrointestinal Polyposis with Ectodermal Changes

Two autopsy cases of Cronkhite-Canada syndrome were reported. The caused of hypoproteinemia, electrolyte imbalance and ectodermal changes were discussed with reference to previously reported cases. The mechanism of protein loss was probably due to outflow into the intestinal lumen of the mucous substance in the cystically dilated glands, directly and/or indirectly followed by loss of mucosal surface. Electrolyte imbalance probably developed from gastrointestinal loss as well as poor substitution. The ectodermal changes were probably not a subsequent part of the emaciation or hypoproteinemis, but an inherent part of this disease. Therapy, whether substitution or surgical procedure, should be selected in order to control the general condition of the patient.     

STUDIES ON PEPTIDES

The tetradecapeptide amide corresponding to the entire amino acid sequence of a wasp venom (polistes mastoparan), isolated from Polistes jadwagae, was synthesized using the trifluoroacetic acid-thioanisole deprotecting procedure.     

Efficacy and safety of laparoscopic surgery for pheochromocytoma

OBJECTIVE: Laparoscopic surgery for primary aldosteronoma and Cushing's syndrome is well established. We report on our experiences with laparoscopic adrenalectomy for pheochromocytoma, and assess the efficacy and safety of the laparoscopic approach. METHODS: Between April 1998 and April 2003, a total of 23 patients underwent laparoscopic adrenalectomy for pheochromocytoma at Chiba University Hospital and Yokohama Rosai Hospital, Japan. We compared the surgical outcomes of these patients with those of 106 patients with adrenal tumors due to other pathologies who underwent laparoscopic adrenalectomy during the same period. RESULTS: The mean tumor size of pheochromocytoma was 4.96 cm. Mean operative time was 192.7 min, and mean estimated blood loss was 130 mL. Neither mean operative time nor mean estimated blood loss was greater for patients with pheochromocytoma. Intraoperative hypertension (systolic blood pressure > 180 mmHg) occurred in 39.1% (9/23) of patients with pheochromocytoma. During the follow-up period, there were no mortalities or recurrences of endocrinopathy. CONCLUSIONS: Laparoscopic adrenalectomy for pheochromocytoma is a safe and minimally invasive procedure.     

Influence of piecemeal necrosis on the compensatory increase of mitochondrial respiratory enzymes of the tumour-bearing liver: Clinical study of 53 surgical cases

The relationships between histological findings, adaptively increased cytochrome a(+a3) levels in chronic liver disease and complications after hepatectomy were studied in order to clarify the mechanism of mitochondrial derangement. The liver specimens of 53 hepatectomized patients were randomly evaluated by three independent hepatopathologists and were compared with cytochrome a(+a3) levels in the biopsied liver, the extent of operation and postoperative complications. The cytochrome a(+a3) concentrations did not show any significant difference between cases of chronic hepatitis and liver cirrhosis nor groups classified by regeneration. Severity of piecemeal necrosis was categorized into three groups: group A--minimal (n = 20); group B--moderate (n = 19); and group C--severe (n = 14). There were significant differences (P less than 0.01) in cytochrome a(+a3) concentrations between the groups (A: 99 +/- 9; B: 135 +/- 6; C: 155 +/- 10 pmol/mg of mitochondrial protein). Extensive hepatectomy, involving segmentectomy or more, was frequently complicated (four of nine, 44.4%) in group C, whereas there were few complications (two of 16, 12.5%) in group A cases in which extensive hepatectomy was performed. Evidence will be presented which will show that deranged liver function, as indicated by cytochrome a(+a3) levels, is closely correlated with piecemeal necrosis. This may be attributed to the damage of periportal hepatocytes which are the main sites of oxidative phosphorylation.     

Clinicopathological and Endoscopic Studies on Gastric Cancer Located; the Cardia

Abstract: In this study the definition of cardiac cancer (histologically adenocarcinoma) was taken to be a cancerous lesion, the size of which is less than 4 cm and the center of which is located within 2 cm from the esophagocardiac junction. 42 patients (0.8%) fit the above definition of cardiac cancer out of 4,958 patients with solitary gastric cancer operated on at the National Cancer Center Hospital daring the period between 1962 and 1988. The clinicopathological and endoscopic findings of these 42 patients were compared with those of 132 patients with gastric cancer located in the upper third of the stomach other than in the cardia (cancer in the other C-area). In the group with cardiac cancer, the male patients (M/F ratio: 3.7), the elderly patients (mean age: 61.3 years), a differentiated type of adenocarcinoma (90%), lesions located at the lesser curvature (62%) and the elevated type of lesion were found to predominate. The incidence of early cancer (60%) and the incidence of small lesions less than 2 cm (32%) in diameter were lower than in the patients with cancers in the other C-area. In the group with cardiac cancer, however, the incidence of such types of early cancer as the depressed lesion, a small lesion less than 2 cm in diameter and lesions difficult to diagnose endoscopically, have been increasing. In recent years, cardicac cancers have been found not only on the lesser curvature, but also in different areas of the cardia. These results suggest an improvement in endoscopic diagnosis for cardiac cancer. To make early detection more precise, it will be indispensable not to overlook even faint or indistinct mucosal abnormalities at the area adjacent to the esophagocardiac junction.