Large pulmonary arteriovenous malformation with hyperammonemia

A 45-year-old female presented with generalized fatigue, unaccompanied by other symptoms. Investigation revealed severe anemia due to gastric bleeding, and hereditary hemorrhagic telangiectasia accompanied by a large pulmonary arteriovenous malformation (PAVM). Additionally, the presence of hepatic arteriovenous and portovenous shunts indicated hepatic involvement. In addition to hypoxemia due to right-to-left shunting in PAVM, hyperammonemia with normal hepatic function was detected. The large PAVM was successfully managed with surgical resection. Hyperammonemia, persisting despite the hemostasis of gastric bleeding, improved postoperatively in the absence of treatment directed at hepatic involvement. We believe that resection of large PAVM contributed to the improvement of hyperammonemia.     

Clinical effectiveness of pipemidic acid on gonorrhoeal urethritis

Twenty cases of gonorrhoeal urethritis were orally treated with pipemidic acid (PPA). The results are as follows. The clinical effect of PPA was excellent in 9 cases and good in 6 cases, the efficacy rate being 75%. The usefulness of PPA was satisfactory in 12 cases (60%). No side effects were observed. The minimal inhibitory concentrations against 14 isolated Neisseria gonorrhoeal strains ranged from 0.78 to 3.13 micrograms/ml with a median value of 1.56 micrograms/ml. A beta-lactamase-producing strain was observed among the 14 strains and the case infected by this strain was successfully treated with PPA. These results indicate that PPA is a useful drug for the treatment of gonorrhoeal urethritis.     

Hepatic Encephalopathy and Reversible Cortical Blindness

We report a case of recurrent hepatic encephalopathy accompanied by transient cortical blindness. The patient with cryptogenic liver cirrhosis had six attacks of hepatic encephalopathy of grades II to III during 1 yr after admission. In the beginning of each episode of encephalopathy, when the patient was conscious, a complete loss of vision occurred, but with a normal pupillary reflex to light. At the same time, the visual evoked potential recorded the second negative wave with a prolonged latency and a diminished amplitude. His sight completely returned, and the electroencephalogram tracing and the visual evoked potential response normalized after treatment for the encephalopathy. The loss of vision was thought to be cortical blindness accompanied by hepatic encephalopathy. Thus, in rare cases of hepatic encephalopathy, the visual cortex may be affected, and cortical blindness may occur before the loss of consciousness.     

In vitro effect of hyaluronate on lysosomal enzyme activities in the bovine ciliary body and iris

The effect of hyaluronate on lysosomal enzyme activities in bovine ciliary body and iris was studied in vitro. Hyaluronates from both the human umbilical cord and bovine vitreous inhibited the activities of cathepsin b and acid phosphatase. Cathepsin d, β-N-acetyl-glucosaminidase, β-d-glucuronidase and α-l-fucosidase activities were not affected by the hyaluronate.     

Gastroduodenal Complications in Patients with Adult T-Cell Leukemia

Gastroduodenal endoscopic examinations were performed on 15patients with adult T-cell leukemia (ATL). Twelve had the diseasein acute form, two in chronic form and one patient was in crisis.Eight patients had gastroduodenal lesions, four esophageal candidiasis,three gastric infiltration and two duodenal ATL-cell infiltration.Four out of the five patients who had the gastroduodenal ATL-cellinfiltration complained of gastroduodenal symptoms such as anorexia,upper abdominal pain, diarrhea and melena. Our observationssuggested that these gastroduodenal symptoms were related tothe gastroduodenal ATL-cell infiltration. Esophageal candidiasisin ATL could be related to immunodeficiency.     

WORINGER-KOLOPP DISEASE

A case of Woringer-Kolopp disease was studied using electron microscopy. The patient was a 45-year-old Japanese woman with a plaque lesion of six years duration on the left upper thigh. The pathological features of the lesion included abnormal cells, mainly in the epidermis and compatible with those of Woringer-Kolopp disease. Electron microscopic examination indicated that the abnormal cells within the epidermis and passing through the basal lamina had features indicating a histiocytic origin, including abundant and protruded cytoplasm and lysosomal dense bodies.     

Second primary cancers in patients with stage III non-small cell lung cancer successfully treated with chemo-radiotherapy

BACKGROUND: Patients successfully treated for non-small cell lung cancer (NSCLC) remain at risk for developing second primary cancer (SPC). The purpose of the current study is to assess the incidence of SPC and the impact of smoking status on the SPC in long-term survivors with stage III NSCLC after chemo-radiotherapy. METHODS: Using the database from the Japan National Hospital Lung Cancer Study Group between 1985 and 1995, information was obtained on 62 patients who were more than 3 years disease-free survivors. Details of clinical information and most smoking history were available from the questionnaire. RESULTS: Nine of the 62 patients developed SPC 3.9-12.2 years (median, 6.2 years) after the initiation of the treatment. The site of SPC was 2 lung, 1 esophagus, 2 stomach, 1 colon, 1 breast, 1 skin and 1 leukemia. Among these nine, three cancers occurred inside the radiation field. The relative risk of any SPC was 2.8 [95% confidence interval (CI) 1.3-5.3]. The risk changed with the passage of time and it increased significantly (5.2 times at or beyond 7 years) after the treatment. In univariate analysis, the patients who were male, had more cumulative smoking and continued smoking, had an increased risk of SPC [relative risk (RR) 2.7, CI 1.1-5.3; RR 3.0, CI 1.2-6.2; RR 5.2, CI 1.6-11.7, respectively]. In multivariate analysis, factors including smoking status and histological type had no effect on the development of a SPC. CONCLUSION: The patients with stage III NSCLC successfully treated with chemo-radiotherapy were at risk for developing SPC and this risk increased with time.     

Molecular targeting therapy for renal cell carcinoma

Metastatic renal cell carcinoma (RCC) is currently one of the most treatment-resistant malignancies. However, significant advances in understanding the molecular mechanisms underlying RCC have led to the development of rationally designed therapies, which are now being tested clinically. To date, the vascular endothelial growth factor receptor (VEGFR) pathway has been the most promising target, and two agents (BAY 43–9006 and SU 11248) that inhibit not only VEGFR but also other receptors, including platelet-derived growth factor receptor (PDGFR), FMS-like tyrosine kinase 3 (FLT3), KIT, and Raf kinase, were recently approved by the FDA for advanced RCC. In addition, a phase III trial investigating the addition of VEGF inhibition to interferon alpha (IFN-α) in RCC is also now going on. Although the clinical activity of existing agents is to be further defined in ongoing trials, the exciting clinical response data with VEGF inhibition in RCC have demonstrated a key role in the treatment of this historically resistant malignancy.