The clinical manifestations and treatment outcomes of Behçet's disease: A single-center experience.

Background and Aims: Behçet's disease is a chronic, multisystemic, and relapsing inflammatory disorder. It lacks a permanent cure, the focus of treatment is on mitigating symptoms, decreasing the frequency and severity of relapses, and preventing life-threatening complications. This study aims to report the experience of a single center in managing patients with Behçet's disease and discuss the treatment outcomes. Methods: This study was a retrospective case series conducted over 2 years. All cases were clinically diagnosed according to the International Criteria for Behçet's Disease. The extracted data were demographics, family history, clinical findings, criteria scores, treatment, and outcomes. Results: A total of 31 patients were included, consisting of 13 males (42%) and 18 females (58%). Most cases were over the age of 30, and both genders were nearly equally distributed among age groups. The most commonly affected site was the oral cavity, observed in 96.77% of cases. Genital, cutaneous, and vascular involvements were more common in males, while females were more likely to have oral, ocular, and musculoskeletal involvements. For various treatment regimens, oral, cutaneous, vascular, and musculoskeletal involvements showed complete response in all cases. Among cases with genital involvement, complete response was achieved in seven cases (41.2%), while four cases (23.5%) showed only partial response, and six cases (35.3%) experienced recurrence. In cases with ocular involvement, only partial responses were observed. Conclusion: Oral, cutaneous, vascular, and musculoskeletal involvements may have a higher likelihood of a complete response to treatment regimens. However, genital involvement may be the most recurrent manifestation, followed by ocular involvement.

Point mutation in a family with hyperproinsulinemia detected by single stranded conformational polymorphism.

We previously described a case of familial hyperproinsulinemia, the fifth to be reported. In the present study we characterized the genetic defect carried by this family and demonstrated that it could be detected by polymerase chain reaction-single stranded conformational polymorphism. Since the serum proinsulin molecule from the propositus, a 63-yr-old Japanese man, was eluted on the same fraction of human proinsulin intermediate cleaved only at the B-C junction, we sequenced exon 3 of his insulin gene, including the C-A junction. A point mutation was discovered that changed codon 65 from arginine (CGT) to histidine (CAT) in one allele. This was the same point mutation as that described previously in three unrelated kindreds representing two races, consistent with the hypothesis that the dinucleotide sequence CpG may be a "hot spot" for mutations. Recently, developed polymerase chain reaction-single stranded conformational polymorphism proved useful in detecting this mutation in the family members. The daughter of the propositus and one of his two grandsons were also demonstrated to be heterozygous for this point mutation by this method.

A phase II trial of oral UFT plus cisplatin (CDDP) in patients with non-small cell lung cancer (NSCLC).

Based on the results of our previous pilot study, we conducted a multi-institutional phase II study of combination chemotherapy consisting of oral UFT (Taiho Pharmaceutical Co. Ltd, Tokyo) plus cisplatin (CDDP) in patients with advanced non-small cell lung cancer (NSCLC). UFT capsule containing 100 mg tegafur and 224 mg uracil was orally administered in two divided doses on days 1 through 21 making the total tegafur dose 400 mg/m(2)/day (maximum 600 mg/body). CDDP was administered by drip infusion at a dose of 20 mg/m(2) on a 5-day schedule from day 8 to 12. Treatment was repeated every 4 weeks as long as the criteria for initiation of therapy were still met. Between April 1995 and March 1997, 51 patients were entered into the study. The mean age of all 50 eligible patients was 64 years(range: 40-78). There were 21 patients with clinical stage IIIB disease and 29 patients with IV disease. Thirty-two patients had adenocarcinoma, 14 had epidermoid carcinoma, and four had large cell carcinoma. Of the 47 assessable patients, 18 achieved a partial response with an overall response rate of 38.3% (95% confidence interval: 24.4-52.2%). The median response duration was 113 days. The median survival time of the eligible patients was 12.8 months, and the 1-year survival rate was 54%. Among the 51 patients enrolled, grade 3 or 4 leukopenia developed in one patient (2%), neutropenia in six patients (11. 8%), thrombocytopenia in six patients (11. 8%), and anemia in three patients (5. 9%). Non-hematological grade 3 or 4 toxicities included anorexia in 10 patients (19.6%), nausea in ten (19.6%), vomiting in two (3.9%), and diarrhea in two (3. 9%). Grade 3 abnormal laboratory data included bilirubinemia in four (7. 8%), GPT elevation in one (2.0%), and hematuria in one (2.0%). In conclusion, combination of CDDP plus oral UFT is efficacious, with low toxicity, in the treatment of advanced NSCLC. In particular, the low hematological toxicity may warrant application of this regimen to the treatment of elderly patients and in trials of concurrent chemoradiotherapy in patients with locally advanced NSCLC.

Lobar atelectasis: diagnostic pitfalls on chest radiography.

This pictorial review looks at the pitfalls in the diagnosis of lobar atelectasis on chest radiographs. Lobar atelectasis with marked volume loss is hard to recognize and may be easily missed. Lobar atelectasis presenting as a mass-like opacity may be misdiagnosed as mediastinal or lung tumour. Lobar atelectasis in an unusual location may also be misdiagnosed as other entities. Familiarity with such manifestations and consideration of anatomical alterations as the signs of lobar atelectasis are important in making the correct diagnosis.

[An autopsy case of AIDS complicated with cytomegalovirus infection and multiple Kaposi's sarcoma].

Cutaneous Kaposi's sarcoma (KS) is a well-known complication of the acquired immunodeficiency syndrome. KS in the internal organs, however, is rare in Japan. We present here a 33-years-old Japanese homosexual man who had AIDS complicated with cytomegalovirus (CMV) infection and KS. He was found to be HIV-seropositive, when he was 31-years-old. He visited our hospital in June 1996 because of high fever. The peripheral blood CD4+ lymphocyte counts were 2 per cubic millimeter, and CMV antigenemia was noted (p65 antigen positive cells; 240/50,000 white blood cells). Thereafter he was successfully treated with parental ganciclovir. On admission, some brown-colored flat nodules were found on the skin, and the diagnosis of KS was made by skin biopsy. We administrated human chorionic gonadotropin (hCG) for the treatment of KS, but had no clinical response. In September 1996, he complained of severe cough, shortness of breath, and vomiting. A chest radiogram showed nodular lesions and pleural effusion in bilateral lungs. A computed tomography of his chest also revealed nodular and linear densities distributed along the bronchovascular bundles. The ultrasonic examination of his abdomen revealed a duodenal nodule. Both nodules in the lungs and duodenum were proved to be KS based on the autopsy findings. Intranuclear inclusionbodies pathognomonic for CMV infections were detected in the stomach and the colon.

[A clinical study of skin flora in patients with MRSA colonization after introduction of preventive measures of hospital infection in a geriatric ward].

In early 1980's methicillin-resistant Staphylococcus aureus (MRSA) was reported as a major pathogenic organism of geriatric hospital infection in Japan. At the same time in the geriatric hospital MRSA infection was prevalent. To decrease nosocomial infections some active preventive measures against hospital infection were taken since Oct. 1991. After introduction of preventive measures of hospital infection in the geriatric ward (190 beds) nosocomial bacteremia and pneumonia were markedly decreased in comparison of episode number before introduction of prevention. However several patients with MRSA colonization were observed every month. The aim of this clinical study was to clear how frequent MRSA was isolated from skin. Consequently 3 strains (3.4%) of MRSA were observed in 86 cultured skin. In conclusion we considered frequency of MRSA colonization frequency of MRSA colonization on skin was not so high but rather very low under the preventive measures.

[Findings of MR and MR cholangiopancreatography in acute cholecystitis].

We retrospectively reviewed magnetic resonance cholangiopancreatography (MRCP) of 25 patients with acute cholecystitis based on clinical, sonographic and surgical findings. Intramural high signal intensity on MRCP was demonstrated in 22 of the 25 patients (88%), and pericholedochal high signal intensity was observed in 6 of the 25 patients (24%). Pericholecystic or perihepatic fluid was demonstrated in 6 of the 25 patients (24%). Gallbladder stones were identified in all 21 patients (100%) by sonography, in 19 of the 21 (90%) by MRCP and in 11 of 18 patients by CT (CT was not performed in other 3 patients). Common bile duct calculi were detected in all 6 patients (100%) by MRCP, in 2 of the 6 (33%) by sonography, and in 5 of the 6 (50%) by CT with confirmation of surgical finding or endoscopic retrograde cholangiography (ERC). MRCP had a high accuracy in diagnosing acute cholecystitis with the finding of intramural high signal intensity. MRCP is an excellent method to evaluate acute biliary disease and may replace CT and ERC in the preoperative evaluation of acute cholecystitis.

Molecular spin resonance in the geometrically frustrated magnet MgCr2O4 by inelastic neutron scattering.

We measured two magnetic modes with finite and discrete energies in an antiferromagnetic ordered phase of a geometrically frustrated magnet MgCr2O4 by single-crystal inelastic neutron scattering, and clarified the spatial spin correlations of the two levels: one is an antiferromagnetic hexamer and the other is an antiferromagnetic heptamer. Since these correlation types are emblematic of quasielastic scattering with geometric frustration, our results indicate instantaneous suppression of lattice distortion in an ordered phase by spin-lattice coupling, probably also supported by orbital and charge. The common features in the two levels, intermolecular independence and discreteness of energy, suggest that the spin molecules are interpreted as quasiparticles (elementary excitations with energy quantum) of highly frustrated spins, in analogy with the Fermi liquid approximation.

Kagomé ice state in the dipolar spin ice Dy2Ti2O7.

We have investigated the kagomé ice behavior of the dipolar spin-ice compound Dy2Ti2O7 in a magnetic field along a [111] direction using neutron scattering and Monte Carlo simulations. The spin correlations show that the kagomé ice behavior predicted for the nearest-neighbor interacting model, where the field induces dimensional reduction and spins are frustrated in each two-dimensional kagomé lattice, occurs in the dipole interacting system. The spins freeze at low temperatures within the macroscopically degenerate ground states of the nearest-neighbor model.

Observation of modulated quadrupolar structures in PrPb3.

Neutron diffraction measurements have been performed on the cubic compound PrPb(3) in a [001] magnetic field to examine the quadrupolar ordering. Antiferromagnetic components with q = (1/2 +/- delta 1/2 0), (1/2 1/2 +/- delta 0) (delta approximately 1/8) are observed below the transition temperature T(Q) (0.4 K at H = 0) whose amplitudes vary linear with H and vanish at zero field, providing the first evidence for a modulated quadrupolar phase. For H < 1 T, a nonsquare modulated state persists even below 100 mK suggesting quadrupole moments associated with a Tau(3) doublet ground state to be partially quenched by hybridization with conduction electrons.

Studies on serum immunoreactive elastase in experimental pancreatic rats. II. In chronic pancreatitis.

In this study using radioimmunoassay for rat, the relationship between the changes of elastase levels and the histological changes of pancreas demonstrated in two different cases of experimental chronic pancreatitis induced by dl-ethionine or PDL technique, and the resulting elastase levels were compared with amylase levels. In some of the Ethionine rats, serum elastase showed a high level, whereas serum amylase exhibited a significant decrease. In PDL rats at 3 days after ligation, elastase levels were higher than in other control rats. In PDL rats at 2, 4, and 8 weeks after ligation, serum elastase levels had significantly decreased, whereas amylase levels had not changes significantly when compared with the control rats. The changes of serum elastase levels in two different cases of experimental chronic pancreatitis showed some relation to the histological changes of the pancreas, while changes in serum amylase levels were not exhibited.

Adenosine deaminase in plasma of patients with adult T-cell leukemia (ATL): correlation between enzyme activity and the ATL subtypes.

Adenosine deaminase (ADA) was assayed in plasma from 14 patients with adult T-cell leukemia (ATL) (eight with acute ATL and six with smoldering or chronic ATL), 20 male family members (ten were anti-ATLA antibody positive and the other ten negative), and 25 normal individuals. ADA activity was uniformly higher in plasma from patients with ATL than normal controls. This enzyme activity significantly increased in acute ATL in comparison to smoldering or chronic ATL. In families of ATL patients, no statistical difference in ADA activity between the anti-ATLA antibody-positive group and -negative group could be discerned. The enzyme activity in a patient with acute ATL, after a bone-marrow transplant, rapidly increased as leukemic cells increased in peripheral blood. These findings indicate that the levels of ADA activities in plasma from ATL patients reflect the condition of this disease. Thus, measurement of this enzyme activity offers a further parameter to distinguish subtypes of ATL, and is of prognostic and therapeutic value.