A Case of Pneumocytoma (So-Called Sclerosing Hemangioma) With Lymph Node Metastasis

A case of "sclerosing hemangionia" (pneumocytoma) of the lungwith lymph node metastasis is reported. A 22-year-old Japaneseman was found to have a well-defined round lesion in the rightlung (S⁷), which increased in size slightly during a 2-yearfollow-up period. He underwent right lower lobectomy with a preoperative diagnosisof a benign lung tumor. The pulmonary tumor revealed histologicalfeatures characteristic of "sclerosing hemangioma" of the lung,in addition to which there were many large polygonal foamy cells,forming tubular or papillary structures. These cells were foundby electron microscopy to contain numerous cytoplasmic lamellarbodies and showed a positive reaction with anti-surfactant apoproteinantibody immunohistochemically. Therefore, they were consideredto be cells differentiating toward type II pneumocytes. Reviewof 21 typical "sclerosing hemangionia" disclosed a few or somesuch foamy cells in 10 cases. A single hilar lymph node wasthe site of microscopic metastases, which consisted of "largeclear foamy cells" and smaller polygonal or round cells withslightly eosinophilic cytoplasm, both of which were componentsof the pulmonary "sclerosing hemangioma" This case supportsthe theory that "sclerosing hemangioma" is a neoplasm of typeII pneumocyte lineage. Although it is said to be benign, rarecases apparently show metastatic potential.     

Carnitine depletion during total parenteral nutrition despite oral L-carnitine supplementation

Carnitine (CAR) plays an important role in the β-oxidation of fatty acids. Less attention, however, has been paid to CAR compared to other nutrients even in total parenteral nutrition (TPN). To examine CAR metabolism during TPN and the effect of simultaneous oral L-CAR supplementation on CAR levels, the blood CAR level was measured in a 3-year-old boy receiving long-term TPN because of short bowel syndrome. Both the total and acyl CAR in the serum were evaluated under various nutritional conditions including oral supplementation of L-CAR. Low CAR concentrations were observed especially when lipid containing TPN regimens were in place. Oral L-CAR supplementation was not sufficient to restore the low CAR levels in the present index patient even when the dose was increased to 120 mg/kg in accordance with the result of the L-CAR absorption test that revealed poor intestinal absorption of this nutrient. Moreover, a markedly low CAR level was measured during the onset of sepsis in the patient, and the blood CAR was depleted when lipid metabolism was activated by lipid loading or sepsis. To date, the late effects of CAR depletion on child growth have not been well examined. It is recommended that the blood CAR level be maintained at normal levels before any prominent manifestations of the deficiency have developed. The intravenous administration of CAR appears to be necessary to supply a sufficient amount of CAR for patients with severe malabsorption.     

Resetting of Tachycardia Cycle by Single and Double Ventricular Extrastimuli in Recurrent Sustained Ventricular Tachycardia

In two cases with recurrent sustained ventricular tachycardia (VT) due to re-entry, the response pattern to extrastimuli during the tachycardia was studied. In each case, right ventricular extrastimuli with longer coupling intervals during VT were followed by fully compensatory pauses and with shorter coupling intervals reset the tachycardia cycle. In one case, a plateau was produced by a single extrastimulus, resembling that seen in the response curve of sinus node automaticity as well as ectopic atrial tachycardia. Two successive stimuli produced three definite zones, i.e., fully compensatory, reset producing a plateau, and progressive prolongation zones with shortening of the coupling intervals between the two stimuli, and terminated the tachycardia with further shortening of the coupling intervals. In conclusion, resetting phenomenon was confirmed on two cases with re-entrant VT. This phenomenon cannot be used as a criterion to determine the mechanism responsible for VT.     

Acute myocardial infarction due to thrombus formation in congenital coronary artery fistula

A young female patient developed acute myocardial infarctionduring physical exercise. Coronary arteriography in the acutephase demonstrated a coronary fistula originating from the leftcoronary main trunk and communicating with the right atrium.Several branches of the left coronary artery were obstructedby thrombotic material originating from the fistula, causingan acute myocardial infarction.     

Epidermolysis bullosa acquisita with oesophageal stenosis

Epidermolysis bullosa acquisita (EBA) is a rare acquired subepidermal blistering disorder associated with autoimmunity to type VII collagen. Although the full clinical spectrum of EBA is still being defined, it is now known that EBA has greater clinical heterogeneity than previously suggested. We describe a patient with EBA which closely approximated the severity of the recessive form of dystrophic epidermolysis bullosa.     

Effect of glycyrrhizin on lysis of hepatocyte membranes induced by anti-liver cell membrane antibody

Studies were made on why glycyrrhizin injection decreases the plasma aspartate aminotransferase (AST) and alanine aminotransferase activities in patients with chronic hepatitis.¹ For this, rat hepatocytes were isolated, and incubated with antibody raised against rat liver cell membranes, and the effect of glycyrrhizin on their release of transaminase was investigated. Isolated rat hepatocytes released AST on incubation with anti-liver cell antibody in the presence of complement. At this time, their endogenous phospholipase A₂ activity was increased. Cultured hepatocytes also released the transaminase in the presence of venom phospholipase A₂. Glycyrrhizin suppressed the release of transaminase in the presence of either anti-liver cell membrane antibody or phospholipase A₂. These results suggest that antibody treatment raised the phospholipase A₂ activity in liver cell membranes, resulting in release of transaminases, and that glycyrrhizin suppressed this increase in phospholipase A₂ activity and so inhibited the release of transaminase.     

Liver abscess that developed after a long interval of transcatheter arterial embolization followed by formation of hepatoduodenal fistula

A 55‐year‐old male patient with hepatocellular carcinoma underwent transcatheter arterial embolization (TAE). He became febrile and experienced pain at the right hypochondrial region 323 days later, which led to the discovery of a liver abscess that fistulated into the duodenal bulb. There have been no reports on the fistulation of liver abscesses into the digestive system following TAE. Rhodococcus equi was isolated as a causative agent, which distinguished the case further.     

Sexual function and clinical features of patients with Klinefelter's syndrome with the chief complaint of male infertility

In this report, we present the overall sexual function and clinical features of patients with Klinefelter's syndrome with the chief complaint of male infertility. The study consisted of 40 patients with a control group of 55 infertile non-azoospermic males with a normal 46,XY karyotype who visited the Reproduction Center of Toho University Hospital during the 5.5-year period between January 1991 and June 1996 with the chief complaint of male infertility. Among the 40 patients with Klinefelter's syndrome, 38 cases were pure 47,XXY, one case was 47,XXY with a pericentric inversion of chromosome 9 and one case was a mosaic of 46,XY/47,XXY(2:28). Thirty-nine of these 40 patients were azoospermic and one (47,XXY) had severe oligoasthenozoospermia. The sexual function of the patients was evaluated according to their responses to a preliminary questionnaire devised by our department. There was no significant difference in the frequency of sexual function disturbances between the patients with Klinefelter's syndrome and the control group (67.5% vs. 60.0%; χ² analysis; p = 0.454). The mean frequency of sexual intercourse per month in the patients with Klinefelter's syndrome was significantly higher than in the control group (4.4 ± 2.8 vs. 3.3 ± 1.6: Welch's t -test, p < 0.05). A possible explanation for this variation may lie in the fact that many of these patients were diagnosed with azoospermia prior to the administration of the questionnaire and may have wished to continue to have relations as a couple.     

Successful treatment of bullous pemphigoid with pulsed intravenous cyclophosphamide

Summary Pulsed therapy with intravenous cyclophosphamide was successful in a patient with bullous pemphigoid whose disease had proved resistant to other treatments.