A 60-year-old man was admitted to our hospital because of dyspnea, cough, and fever two weeks after participating demolition work. Computed tomography of the chest showed multiple diffuse interstitial shadows. While fibrotic bronchoscopy and transbronchial lung biopsy showed alveolitis. We diagnosed hypersensitivity pneumonitis (HP) and treated with mPSL-pulse therapy then got better soon, and also chest abnormal shadow disappeared. Two month later he was admitted because of the same symptom. His chest CT-scans showed diffuse interstitial shadows and we diagnosed recurrence of HP. Treatment with mPSL pulse-therapy was not effective. Acute respiratory failure appeared and progressed daily. The patient died 3 weeks after admission. The final diagnosis of autopsy is angiotrophic large cell lymphoma, a very rare type of lymphoma (<1% of NHL). It is important to consider angiotrophic large cell lymphoma in the differential diagnosis of diffuse interstitial lung disease. 相似文献
BACKGROUND: Staphylococcus aureus alpha-toxin is a major virulence factor, but its mechanism of action in vivo is incompletely understood. METHODS: We examined the role of alpha-toxin in S. aureus pneumonia using the mouse model of intranasal lung infection with S. aureus strain 8325-4 (hla(+) S. aureus) and an alpha-toxin-deficient mutant strain made on the 8325-4 background (hla(-) S. aureus). RESULTS: Intranasal infection of mice with hla(-) S. aureus resulted in substantially less lung injury and inflammation, pulmonary edema, and tissue bacterial burden than did infection with hla(+) S. aureus. Furthermore, fewer mice infected with hla(-) S. aureus died of the infection, compared with those infected with hla(+) S. aureus. Levels of the CXC chemokines keratinocyte-derived chemokine and macrophage inflammatory protein-2 were significantly lower in the airways of mice infected with hla(-) S. aureus, and this difference was the result of reduced secretion of newly synthesized chemokines into the airway. Consistent with these data, significantly fewer neutrophils were present in the airways and lungs of mice infected with hla(-) S. aureus, compared with those infected with hla(+) S. aureus. CONCLUSIONS: These data suggest that alpha-toxin enhances virulence by facilitating the generation of CXC chemokine gradients and stimulating chemokine-induced neutrophil influx in S. aureus pneumonia. 相似文献
OBJECTIVE: Despite multiple and repeated exposures to HIV-1, some individuals possess no detectable HIV genome and show T-cell memory responses to the viral antigens. HIV-1-reactive mucosal IgA detected in such uninfected individuals suggests their possible immune resistance against HIV. We tested if the above HIV-1-exposed but uninfected status was associated with genetic markers other than a homozygous deletion of the CCR5 gene. METHODS: Based on our mapping in chromosome 15 of a gene controlling the production of neutralizing antibodies in a mouse retrovirus infection, we genotyped 42 HIV-1-exposed but uninfected Italians at polymorphic loci in the syntenic segment of human chromosome 22, and compared them with 49 HIV-1-infected and 47 uninfected healthy control individuals by a closed testing procedure. RESULTS: A significant association was found between chromosome 22q12-13 genotypes and a putative dominant locus conferring anti-HIV-1 immune responses in the exposed but uninfected individuals. Distributions of linkage disequilibrium across chromosome 22 also differed between the exposed but uninfected and two other phenotypic groups. CONCLUSIONS: The data indicated the presence of a new genetic factor associated with the HIV-1-exposed but uninfected status. 相似文献
A 57-year-old man was admitted with fever and epigastralgia, and presented with splenomegaly and pancytopenia. A CT scan revealed splenic infarctions. There were no lymphadenopathies, skin lesions, or neurological abnormalities. A splenectomy was performed. Bone marrow involvement with hemophagocytosis was noted. The diagnosis of Asian variant of intravascular diffuse large B-cell lymphoma was based on intravascular and sinusoidal distribution of large CD5+ B cells. The patient died of the disease 11 months after onset. To our knowledge, this is the first report of AIVL that presented with splenic infarction. This distinct lymphoma should be included in the differential diagnosis of splenic infarction. 相似文献
Objective: To investigate the potential beneficial effects of guideline-based pharmacological therapy on pulmonary function and quality of life (QOL) in Japanese chronic obstructive pulmonary disease (COPD) patients without prior treatment.
Research design and methods: Multicenter survey, open-label study of 49 Japanese COPD patients aged ≥ 40 years; outpatients with >10 pack years of smoking history; ratio of forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) < 70%; predicted FEV1 < 80%; treated with bronchodilators and/or inhaled corticosteroids as maintenance therapy until week 48.
Main outcome measures: The primary endpoint was change in pulmonary function (trough FEV1, trough FVC); secondary endpoints were QOL and physical activity at 48 weeks after initiation of therapy.
Results: Airway reversibility was confirmed in untreated patients. Significant changes over time were not observed for FEV1 and FVC, indicating lung function at initiation of treatment was maintained during the observation period. COPD assessment test scores showed statistical and clinical improvements. Cough, sputum, breathlessness, and shortness of breath were significantly improved.
Conclusions: Lung function and QOL of untreated Japanese COPD patients improved and improvements were maintained by performing a therapeutic intervention that conformed to published guidelines. 相似文献
Results of pediatric lymphoma treatment have improved markedly over the past 30 years. In Hodgkin's lymphoma, the 5 year event‐free survival (EFS) was 81.5% in a retrospective study. In the ALB‐NHL03 study, the 5 year EFS according to clinical stage in patients with lymphoblastic T‐cell lymphoma (T‐LBL) was 70.6% for stage III and 88.9% for stage IV. In mature B‐cell lymphoma, the B‐NHL03 study indicated that the 4 year EFS according to treatment group was 94% for group 1, 98% for group 2, 84% for group 3, and 78% for group 4. Moreover, the 2 year EFS rate was 81% in Japanese advanced stage patients based on the international ALCL99 study. Thus, EFS >80% was achieved in any subtype of pediatric lymphoma. With regard to refractory or recurrent lymphoma, however, treatment methods for improvement of the survival rate in these patients still need to be developed. Also the difference between child, and adolescent and young adult patients still needs to be clarified, and treatment protocols developed. Although lymphoma treatment does not greatly change according to country, it does differ between other countries and Japan for some subtypes of lymphoma. In particular, the results of treatment of stage III T‐LBL in Japan are worse than those in the USA and Europe. The priority in future studies will be to collect data on these differences, and the reasons for these differences. 相似文献
Peripheral T‐cell lymphoma (PTCL) is rare in children, and it has a poor prognosis compared with other types of lymphoma. We report the case of a 7‐year‐old boy with spontaneous improvement of PTCL complicated by hemophagocytic syndrome as the initial symptom. He complained of pain and swelling of the right neck and presented with high fever. Pancytopenia, liver dysfunction, elevated ferritin and soluble interleukin 2 receptor were noted on laboratory tests. Peripheral blood plasma and white blood cells were positive for Epstein–Barr virus (EBV) genome but, after several days, the fever abated and laboratory data improved. On histopathology of lymph node biopsy, he was diagnosed as having PTCL not otherwise specified (PTCL‐NOS) with EBV infection. He received no chemotherapy and was disease free at the last follow up, 6 years 8 months after onset. This is probably the first case of spontaneous improvement in PTCL‐NOS. Careful treatment planning is therefore necessary in PTCL‐NOS, given the possibility of spontaneous improvement of symptoms. 相似文献
The management of LSRS is a crucial problem to ensure a sufficient PV flow during pediatric LT. Although several techniques have been indicated to solve this problem, a more appropriate approach to LSRS is still needed in pediatric LT. We herein present a modified surgical approach to the ligation of LSRS via the left side of the IVC for a nine‐month‐old boy with severe portal hypertension and a history of Kasai portoenterostomy. LSRS was identified and exposed through the left side of the IVC and the dorsal surface of the pancreas from the superior side of the body of the pancreas. The post‐operative course was uneventful with an excellent PV flow. The central approach for the ligation of LSRS is worth considering as an alternative procedure for a patient with collateral vessels and a history of multiple laparotomies. 相似文献
PC is produced in the liver and inhibits blood coagulation by catalyzing active factors V and VIII. PC deficiency causes abnormal blood clotting that is difficult to regulate by anticoagulative treatments. Four reports of PC deficiency treated with LTx have been published; however, no report of DLT as a therapy for PC deficiency is available. We describe a case of a 23‐month‐old girl who received DLT for compound heterozygous PC deficiency. Her PC activity was below 5%. She developed intracranial lesion and frequent refractory purpura fulminans. Both her parents had heterozygous mutations of PC genes and were excluded as living donors. Furthermore, she was a low priority on the waiting list of deceased‐donor transplantation. We performed living DLT using the liver from a patient with MSUD. Activated PC concentrate safely supported the perioperative period. After DLT, she maintained normal PC activities and BCAA levels. This is the first case of PC deficiency successfully treated by living DLT with MSUD. We propose that DLT using liver from patients with MSUD is a treatment option for PC deficiency. 相似文献