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.     

Metabolite changes with age measured by proton magnetic resonance spectroscopy in normal subjects

Abstract  To determine whether there are metabolite changes in the left medial temporal and frontal lobes with aging, we performed proton magnetic resonance spectroscopy in 36 normal subjects. The N-acetylaspartate/creatine-phosphocreatine ratio in the medial temporal lobe tended to be decreased in subjects over 60 years of age. The ratio decrease in the frontal lobe related to aging was lower than that in the medial temporal lobe. There were no significant differences in the metabolite ratios between males and females. These findings suggest that structures in the medial temporal lobe may be more susceptible to neuronal dysfunction associated with aging than those in the frontal lobe.     

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.     

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.     

Delta infection in Japan: Immunohistological and immuno-electron microscopic study of delta antigen in liver tissue

Abstract Although the vast majority of hepatitis B surface antigen (HBsAg) carriers of the world inhabit South-east Asia, very little is known about delta infection in this area. Therefore, a serological and immunohistological study was made in the Tokyo-Chiba area. One of 58 (1.7%) HBsAg carriers had anti-delta antibody in a high titre in serum. Delta antigen was immuno-histologically localized in the liver in two of 146 (1.4%) HBsAg carriers studied. The antigen was strongly stained in the nuclei, and positive cells were diffusely scattered throughout the liver in both cases. Neither subject was an illicit drug user: one had travelled to Italy 10 years earlier and the other had a blood transfusion during a 5-year residence in Bangkok in the past.
Thus, there is delta infection among non-intravenous drug users in Japan. Delta infection has been linked to severe liver damage, occasionally fatal. Once introduced, it could become epidemic in a country where hepatitis B virus infection is endemic, and might spread among non-drug users.     

Conduction Delay in Right Ventricle as a Marker for Identifying High‐Risk Patients With Brugada Syndrome

Conduction Delay as a Marker for Brugada Syndrome. Objectives: To evaluate the significance of conduction delay (CD) in the right ventricle (RV) in Brugada syndrome (BS) as a marker for risk stratification of sudden death. Methods: Twenty‐five patients with BS (7 with documented ventricular fibrillation (VF), 8 with syncope, and 10 without symptoms) and 10 control subjects were paced from the RV apex using 8 beats of drive pacing and a single extra‐stimulus. CDs in the right ventricular outflow tract (RVOT) (CD‐RV) and in the lateral left ventricle (L‐LV) (CD‐LV), and the local electrogram durations at a single extra‐stimulus in RVOT (D‐RV) and L‐LV (D‐LV) were calculated. We also evaluated changes in 12‐lead ECG parameters in 16 patients with BS after pilsicainide challenge test (Pilsicainide‐test). Results: Maximal CD‐RV and maximal D‐RV were significantly larger than maximal CD‐LV and maximal D‐LV in BS (26 ± 10 and 105 ± 15 vs 20 ± 6 and 92 ± 15 ms, P < 0.05, respectively). Maximal CD‐RV and maximal D‐RV in patients with documented VF were the largest among the 3 groups. There was a significant positive correlation between maximal CD‐RV or maximal D‐RV and changes in QRS duration in leads V2 and V5 and in S wave duration in lead II and V5 after Pilsicainide‐test (CD‐RV; r = 0.54, 0.51, 0.56, and 0.53: D‐RV; r = 0.55, 0.5, 0.57, and 0.53, P < 0.05, respectively). In control subjects, there were no significant differences. Conclusions: CD in RV was a useful marker for identifying high‐risk patients with BS. CD in the RV, especially in the RVOT epicardium, may be related to arrhythmias in BS. (J Cardiovasc Electrophysiol, Vol. 21, pp. 688‐696, June 2010)