[A surgical case of abdominal aortic aneurysm accompanied by hamartomatous changes in the vascular wall].

The current patient was a 22-year-old woman with dyspnea on exertion that had exacerbated over the past two to three years. Chest X-ray indicated pneumothorax in the right lung, and chest computed tomography showed diffuse microcysts in both lungs. The patient was diagnosed with lymphangiomyomatosis based on pleural biopsy by intrathoracic endoscopy.

Gene therapy/cell therapy for lysosomal storage disease.

Lysosomal storage diseases (LSD) are considered to be appropriate disorders for gene therapy/cell therapy. We are attempting to treat one of these disorders using a mouse model, the Sly mouse. This is an authentic model for human beta-glucuronidase deficiency, MPS VII.

Presence of sonographically detectable parathyroid glands can predict resistance to oral pulsed-dose calcitriol treatment of secondary hyperparathyroidism.

Oral pulsed-dose calcitriol administration has been shown to be effective therapy for patients with secondary hyperparathyroidism. However, this effect is not consistently observed in the clinical setting. This study was undertaken to examine whether enlarged parathyroid glands can serve as a clinical marker that predicts the suppressive effect of calcitriol.

Suppression of N(epsilon)-(carboxymethyl)lysine generation by the antioxidant N-acetylcysteine.

Objective: Accumulated evidence suggests that N(epsilon)-(carboxymethyl)lysine (CML), which is a dominant antigen of advanced glycation end-products (AGEs), is generated in the peritoneal cavity of patients undergoing continuous ambulatory peritoneal dialysis (CAPD), and that this process may be involved in the pathophysiology of the peritoneal injury found with CAPD treatment. Since CML is a sequential product of glycation and oxidation processes, CML generation could be suppressed by antioxidants. The aim of this in vitro study was to clarify the effect of N-acetylcysteine (NAC), an antioxidant, on CML generation from proteins under high glucose settings mimicking peritoneal dialysis solutions.

[Twelve cases of systemic lupus erythematosus in boys].

We compared clinical findings in 12 cases of systemic lupus erythematosus (SLE) in boys with those in 49 cases in girls. The age at which SLE developed in boys was consistent with that of infantile SLE and there was no age specificity. Momy cases in boys were diagnosed earlier as compared with cases in girls.

[A case of primary transitional cell carcinoma of the prostate responsive to combination chemotherapy with methotrexate, epirubicin and cisplatin].

A 72-year-old man with the chief complaint of macrohematuria was referred to our hospital in February 1996. On transrectal examination, the prostate was stony hard without nodules. Cystoscopic findings and biopsies did not confirm a transitional cell carcinoma or carcinoma in situ in the bladder.

Enhanced magnetic resonance imaging of leptomeningeal angiomatosis.

We present two patients with unilateral occipital gyriform calcification and seizures. Gyriform or serpentine calcification as revealed by computed tomography (CT) scan is rare and is a characteristic finding of Sturge-Weber syndrome (SWS) and celiac disease (CD). These patients had neither the facial nevus flammeus or neurological deficits characteristic of SWS, nor the gastrointestinal symptoms characteristic of CD.

Molecular and biochemical events within the brain subjected to cerebral ischemia (targets for therapeutical intervention).

We review the molecular and biochemical events that occur within the brain during cerebral ischemia, based on recent investigations of focal cerebral ischemia models. Occlusion of the middle cerebral artery in rats produces focal ischemia. In contrast to the core where ischemia is severe and infarction develops rapidly, areas surrounding the core (called the penumbra) show a more moderate decrease of blood flow and can tolerate longer durations of ischemic stress.

Immunohistochemical detection of advanced glycosylation end-products in the peritoneum and its possible pathophysiological role in CAPD.

It has recently been suggested that advanced glycosylation end-products (AGEs) are formed in the peritoneum in patients on CAPD. However, the exact location of AGE accumulation, the relation with the duration of CAPD and its pathophysiological role in CAPD remain unclear. If the peritoneum is glycosylated, it could bring about altered peritoneal function.

[Successful direct embolectomy for acute massive pulmonary thromboembolism].

A 35-year-old woman was bedridden because of a fall and developed pulmonary embolism after venous thromboembolism of lower extremity. Although anticoagulation therapy was immediately carried out, hemodynamics deteriorated gradually into the state of class IV in Green-field's classification. As a result, an urgent surgical procedure was performed.

[A 12-year-old girl with acute respiratory disease with pneumonia caused by adenovirus type 7].

We have encountered a 12-year-old girl with acute respiratory disease including pneumonia caused by adenovirus type 7. While being followed-up for bronchial asthma, she developed this disease with dyspnea accompanied by high fever, pharyngalgia and a dry cough subsequent to a slight fever. Adenovirus type 7 was separated and identified from the pharyngeal smear.

[Three cases with juvenile rheumatoid arthritis who showed hypocomplementemia related to disease activity].

We encountered three cases of juvenile rheumatoid arthritis which showed a depressed serum complement level during the clinical course. Circulating immune complex and increased secondary fibrinolysis were detected in all cases. The laboratory data suggested the presence of vesculitis.

[Generalized angitis and recurrent infection associated hemophagocytic syndrome in a girl with XXX syndrome].

We have encountered a case of a girl with XXX syndrome who had infection associated hemophagocytic syndrome (HPS) recurrently. The patient presented hyper-gammaglobulinemia during the clinical course and developed IAHS probably because of infection with Rubella virus and EB virus each as a trigger. Diseases that cause abnormality in X chromosome are said to present immune abnormality such as SLE in many cases.

[5 cases of clostridium difficile enteritis in infants].

We encountered 5 cases of Clostridium difficile enteritis that is rate in infancy. Its clinical symptoms consisted mainly of diarrhea, fever and vomiting. Also progressive abdominal enlargement was characteristically noted.

[Chronic cytomegalovirus infection that present specific clinical course--a case of a boy with common variable immunodeficiency].

We describe an 8-year-old boy with CVID and chronic CMV infection. Although at onset he was diagnosed as IgA deficiency, 4 years after his clinical manifestations because compatible to CVID. During his clinical course he had suffered from various disorders as follows; AIHA, interstitial pneumonia, hemophagocytic syndrome, chronic gastroenterocolitis and so forth.

Vascular endothelial dysfunction in patients on regular dialysis treatment.

Circulating plasma immunoreactive thrombomodulin (i-TM) reflects the injury of vascular endothelium, and desmopressin stimulates tissue plasminogen activator (tPA) release from endothelium. Therefore, in order to estimate the damage of vascular endothelium in patients on regular dialysis treatment (RDT), we studied 1) basal plasma levels of i-TM and 2) a capability to release tPA by desmopressin. The basal plasma i-TM levels of the patients (n = 23) were significantly higher than those of the normal controls (p < 0.

Anti-hypertensive effect of low Na connection (120 mEq/l) solution for CAPD patients.

In patients undergoing continuous ambulatory peritoneal dialysis (CAPD), transperitoneal sodium (Na) flux plays a crucial role in the control of blood pressure (BP), as it determines the net Na balance of the patient. However, with the conventional solution, Na removal into the effluent greatly depends on the drained ultrafiltration (UF) volume, and this can hinder the success of adequate Na removal in some patients. Therefore, we developed a low Na concentration solution to increase Na removal through diffusion force, and studied the clinical effect of this solution in patients with Na overload.

[Clinical psychopathologic study of aged chronic schizophrenics in residential homes for the elderly--relapsed and non-relapsed].

Residential homes for the elderly are expected to play an important role in the future as places for the social rehabilitation of inpatients with chronic schizophrenia in psychiatric hospitals. A study was performed to investigate the state of adjustment of schizophrenics currently staying in residential homes for the elderly from the viewpoint of clinical psychopathology. The subjects, consisting of 26 schizophrenics (10 males and 16 females) with a mean age of 76.