A Ro/SS-A auto-antibody positive mother's infant revealed congenital complete atrioventricular block,followed by insulin dependent diabetes mellitus and multiple organ failure

We experienced a congenital complete atrioventricular block infant who was born from a Ro/SS-A antibody positive mother. Ro/SS-A antibody was also found in this baby which was presumed to be mediated by the maternal placenta. Temporary cardiac pacing was required at birth and pacemaker implantation was performed at 9 months. At 11 months of age, the baby fell into shock and experienced multiple organ failure because of diabetes mellitus-induced coma. The association between congenital complete heart block and the Ro/SS-A antibody is well known. However, the accompaniment of insulin-dependent diabetes mellitus has not been reported previously. As the Ro/SS-A antigen appears in the cytoplasm of many tissues, the possibility of an association between Ro/SS-A antibody and diabetes mellitus is difficult to deny. We report this rare case to draw attention to the possibility that babies who are born from an Ro/SS-A antibody positive mother may develop diabetes mellitus as well as congenital complete heart block.     

SECONDARY AMYLOIDOSIS IN PATIENTS WITH RHEUMATOID ARTHRITIS: DIAGNOSTIC AND PROGNOSTIC VALUE OF GASTRODUODENAL BIOPSY

Upper gastrointestinal endoscopy was performed in patients withrheumatoid arthritis (RA) during the period 1989–1991,and biopsy specimens were obtained from the stomach and fromthe duodenum for examining amyloid deposits. Among 407 patients,gastrointestinal amyloidosis was confirmed in 54 (13.3%). Twenty-twopatients were regarded as having slight amyloid deposits, while32 patients were categorized as having marked amyloid deposits.The incidence of clinical manifestations suggestive of systemicamyloidosis was more frequent in the marked deposits group thanin the slight deposits group (47% vs 14%, P < 0.05). Amongthe patients who died of manifestations associated with amyloidosis,the survival period following endoscopy was shorter in the markeddeposits group than in the slight deposits group. These findingssuggest that gastroduodenal biopsies may be useful for diagnosingsecondary amyloidosis and that the degree of amyloid depositsseems to be correlated with the clinical manifestations of RA. KEY WORDS: Rheumatoid arthritis, Complications, Secondary amyloidosis, Diagnosis, Endoscopy, Biopsy, Prognosis     

Combined Repeat Dose and Reproductive/Developmental Toxicity Screening Test (OECD): Familiarization Using Cyclophosphamide

Combined Repeat Dose and Reproductive/Developmental ToxicityScreening Test (OECD): Familiarization Using Cyclophosphamide.TANAKA, S., KAWASHIMA, K., NAITO, K., USAMI, M., NAKADATE, M.,IMAIDA, K., TAKAHASHI, M., HAYASHI, Y., KUROKAWA, Y., AND TOBE,M. (1992A, Y., AND TOBE, M. (1992). Fundam. Appl. Toxicol. 18,89–95. A familiarization study was conducted on the "Combined RepeatDose and Reproductive/Developmental Toxicity Screening Test(ReproTox)" proposed by the OECD. Cyclophosphamide (CP) at dosesof 6.7, 4.5, 3, 2, and 0 mg/kg body wt was given daily by gavageto groups of 12 male and 12 female Sprague-Dawley rats. As aresult, anemia and leukopenia were evident in treated males.The absolute and relative thymus and spleen weights were decreasedin treated rats. Histopathologically, atrophy of the thymus,spleen, and bone marrow was observed. With respect to the reproductive/developmentaltoxicity, dose-dependent increases in postimplantation lossof fetuses and postnatal death were found in dams given CP.The body weight of pups treated with CP was significantly loweredin a dose-related manner. Thus the results demonstrated mostof the known toxicological properties of CP, except the adverseeffects on spermatogenesis and fertility. Therefore ReproToxcan be considered as a useful screening test for assessing repeatdose and reproductive/developmental toxicity of existing chemicalsof high production volume.     

A case of granular cell tumor of the bladder successfully managed with extraperitoneal laparoscopic surgery

Granular cell tumor is a benign neoplasm which frequently occurs in the oral cavity, skin, and subcutaneous tissue. Granular cell tumor of the bladder is an extremely rare disease, and only nine cases have been reported. We present here an additional case of granular cell tumor occurring in the bladder. Unlike the other tumors reported, this tumor extruded into the Retzius' cavity. Therefore, the tumor was successfully excised through extraperitoneal laparoscopic surgery. The patient was free from recurrence 40 months after surgery. The small tumor located in Retzius' cavity could be managed with extraperitoneal laparoscopic surgery.     

THE EFFECT OF ALTHESIN (CT1341) ON THE OXYHAEMOGLOBIN DISSOCIATION CURVE IN VITRO

Blood from non-smoking donors was incubated with Althesin andthe Po2 for half-saturation of haemoglobin at standard pH, Pco₂and temperature (P₆₀) was measured. The mean P₆₀ values of bloodwith blood concentrations of Althesin of 0.14ml/dl and 0.42ml/dl were greater (1.14 ± 0.31 (SEM) mm Hg (p< 0.005)and 2.22 ± 0.35 (SEM) mm Hg (P< 0.001), respectively)than that of control blood. These increases were dose-dependent(P< 0.05). Erythrocyte 2,3-diphosphoglycerate (2,3-DPG) concentrationswere not significantly different between the Althesin-treatedand the control blood. Although a decrease in the affinity ofhaemoglobin for oxygen in vitro in the presence of Althesinwas statistically significant, this would have no clinical significanceif the drug was being used for the induction of anaesthesiaonly.     

Hepatic encephalopathy due to a large intrahepatic communication between the portal vein and inferior vena cava

A 59 year old man with cirrhosis presented with encephalopathy and hyper-ammonaemia. T1 weighted magnetic resonance imaging demonstrated a large void tubular structure connecting the right posterior portal vein branch and the inferior vena cava through the right hepatic lobe inferiorly, and cine-mode imaging showed a flow within this channel. Clearly in this patient a significant portion of the portal venous blood was being shunted into the inferior vena cava, causing encephalopathy. The exact origin of this channel is not known, but several possibilities are discussed. It is also predicted that similar previously unknown large intrahepatic shunts will be discovered increasingly with the availability of modern imaging techniques.     

Primary liver cancer in non-cirrhotic liver. Epidemiological study based on autopsies performed in Trieste, Italy and Kurume, Japan

This study, aimed at elucidating the epidemiological features of primary liver carcinoma developing in non-cirrhotic livers, was based on 25,103 autopsies performed between 1975 and 1984 in Trieste, Italy. These autopsies correspond to approximately 70% of all deaths that occurred in this area. Various factors allegedly related to carcinomas were analysed in reference to our previous study on cirrhotic livers and in comparison with 5,603 autopsies in Kurume, Japan. There were 28 cases of hepatocellular carcinoma (HCC), 16 of cholangiocellular carcinoma (CCC) not associated with cirrhosis in Trieste, and 48 HCC and 19 CCC in Kurume. On the basis of our findings, it was concluded that cirrhosis, regardless of its cause, is the main pathogenetic factor in HCC; it is responsible for a much higher frequency (14.2:1) than in non-cirrhotic livers, as well as for early occurrence of tumours (an average of 6 years earlier in cirrhotic liver) in Trieste. Patients in Trieste were older than those in Japan, and the frequency of HCC among all autopsies was much greater in the latter. By contrast, the influence of cirrhosis on cholangiocellular carcinoma (CCC) was negligible, as such association appeared purely coincidental or absent. The incidence of CCC among autopsies was greater in Japan. Our data on CCC were not sufficient to demonstrate any clear aetiopathogenetic association between this tumour and alcohol abuse and hepatitis B virus (HBV) infection, except for a possible aetiological role of gallstones. The frequency of CCC relative to HCC was greater in Trieste than in Japan; the incidence of HCC was much less in Trieste, whereas CCC was more frequent in Japan.