Necrotizing fasciitis (NF) is a necrotizing soft tissue infection that can cause rapid local tissue destruction, necrosis and life-threatening severe sepsis. Predisposing conditions for NF include diabetes, malignancy, alcohol abuse, and chronic liver and kidney diseases. NF is classified into two categories (types 1 and 2) based on causative microorganisms. The initial clinical picture of NF mimics that of cellulitis or erysipelas, including fever, pain, tenderness, swelling and erythema. The cardinal manifestations of NF are severe pain at onset out of proportion to local findings, hemorrhagic bullae and/or vital sign abnormality. In such cases, NF should be strongly suspected and immediate surgical intervention should be considered, along with broad-spectrum antimicrobials and general supportive measures, regardless of the findings of imaging tests.

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Background Transcatheter arterial chemoembolization (TACE) and transcatheter arterial infusion chemotherapy (TAI) are increasingly used to treat inoperable liver malignancies. It has not been determined whether standard oral and intravenous administration of antibiotics have different prophylactic effects against post-TACE/TAI infection. We compared the efficacy of oral levofloxacin (LVFX) and intravenous cephazolin (CEZ) in patients receiving TACE/TAI for hepatocellular carcinoma (HCC) using a prospective design.
Patients and Methods One hundred twenty-nine eligible subjects with HCC treated by TACE/TAI were analyzed in this study. Patients were randomly assigned by the envelope method to groups who received either intravenous infusion of CEZ at 2 g/day or oral administration of LVFX at 300 mg/day for 5 days. Laboratory data, changes in antibiotic administration from the standard ones, duration of hospital stay, side effects of antibiotics, and infectious complications were assessed.
Results There were no significant differences in the WBC counts and serum CRP levels between the groups; there were also no significant inter-group differences in the numbers of infectious and other adverse events.
Conclusion Our study findings suggest that the results of peroral administration of LVFX for the prevention of post-procedure infectious complications in patients receiving TACE/TAI for HCC are not inferior to those of intravenous administration of CEZ.

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Objective The aim of this study was to investigate the outcome of overlap/switch to adefovir dipivoxil (ADV) monotherapy for chronic hepatitis B (CHB) patients with lamivudine (LAM)-resistant HBV, who responded to LAM plus ADV combination therapy.
Methods In 29 of 35 LAM-resistant CHB patients, serum HBV-DNA levels decreased to <3.7 log genome equivalent (LGE)/mL at 12 months after LAM plus ADV combination therapy, defined as complete virological response (CVR). The 29 CVR patients were randomly allocated to continuation of combination therapy or switch to ADV monotherapy within 12 months. The cumulative rates of sustained CVR were compared between the two groups.
Results The follow-up duration after randomization was 19.3-36.7 months (median, 28.2 months) for the combination group and 21.0-36.4 months (29.0 months) for the overlap/switch group. The cumulative rate of sustained CVR during the follow-up period was 100% in all patients of both groups. The total medical expenses during follow-up after randomization were median US$20,949 for the combination group and US$16,107 for the overlap/switch group (p=0.012). Overlap/switch to ADV monotherapy sufficiently repressed the replication of LAM-resistant mutants without the development of ADV-resistant mutants. The rate of sustained CVR was not influenced by treatment regimen (continuation of combination therapy or switching to ADV monotherapy), the duration of the overlap period, or patient and virological characteristics.
Conclusion In LAM-resistant CHB patients who achieved CVR to LAM plus ADV combination therapy, CVR was maintained after overlap/switch to ADV monotherapy, suggesting that it could be a useful regimen for such patients.

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Objective The diagnostic criteria for primary biliary cirrhosis (PBC) in Japan were revised in 2004. The prevalence and prognosis of PBC using the revised criteria have not been reported. This study investigated the prevalence and prognosis of "newly-diagnosed" symptomatic-PBC (ns-PBC), which was defined as asymptomatic PBC (a-PBC) in Japan until 2004.
Patients and Methods The clinical features and the prognosis of 207 patients with PBC were retrospectively investigated according to clinical stage.
Results The prevalence of ns-PBC was 3.4% and 9.7%, at the time of diagnosis and final evaluation, respectively. The prognosis of ns-PBC was poorer than a-PBC. A total of 7.2% of the patients with a-PBC progressed to ns-PBC during the observation period. These patients had a poorer prognosis than patients who remained asymptomatic.
Conclusion Approximately 10% of the PBC patients presented with signs of portal hypertension as an initial symptom. The signs of portal hypertension should therefore be carefully investigated in patients with PBC at the time of diagnosis and during the observation.

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Objective The data from the Fulminant Type 1 Diabetes Committee suggested that patients with fulminant type 1 diabetes are a subgroup at high risk for diabetic microangiopathy in the first 5 years after diagnosis associated with the lack of endogenous insulin secretion from the onset of diabetes. The aim of this study was to assess the development of microangiopathy in patients with fulminant type 1 diabetes followed in our diabetes center.
Methods Sixteen patients with fulminant type 1 diabetes and 60 age-matched patients with non-fulminant type 1 diabetes were recruited as subjects. The existence or lack of diabetic retinopathy and nephropathy, average HbA_1C level, serum C-peptide level, average blood pressure, insulin level, whether or not they were taking antihypertensive agents, and smoking history were investigated retrospectively based on medical records.
Results The 5-year incidence of microangiopathy was lower in fulminant than in non-fulminant type 1 diabetes patients; retinopathy cases occurred in 0% vs. 8.3% of patients, and nephropathy occurred in 0% vs. 1.7% of patients. The 10-year incidence of retinopathy was 0% vs. 24.1%, and that of nephropathy was 11.1% vs. 3.4%. The cumulative incidence of microangiopathy did not differ between the fulminant and non-fulminant type 1 diabetes patients. Mean HbA_1C levels and systolic blood pressure were significantly lower in fulminant type 1 diabetes patients.
Conclusion No difference between the patients visiting the center with fulminant type 1 diabetes and those with non-fulminant type 1 diabetes was observed in the development of microangiopathy complications.

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Objective The incidence of the gastrointestinal adverse effects is important to determine as these effects are the reason for lower compliance of α-glucosidase inhibitors (αGIs). There has been no direct investigation of the adverse effects with acarbose or miglitol, therefore we compared them in healthy subjects.
Methods Twenty-two healthy men were administered 75 mg of miglitol or 100 mg of acarbose per every meal for three days. After four drug-free washout days, they were administered 100 mg of acarbose or 75 mg of miglitol per every meal, respectively. They reported the state of their stool, borborygmi, abdominal bloating, flatus, and abdominal pain on the 1st and 3rd day.
Results Stool tended to be soft when miglitol was administered and to be firm when acarbose was administered. The flatus score of acarbose was greater than that of miglitol. The abdominal bloating score of acarbose was greater than that of miglitol on the 1st day.
Conclusion Our results suggest that if diabetic patients have constipation, firm stool, or flatus they may be administered miglitol and if they have diarrhea or soft stool they may be administered acarbose.

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Objective Several reports have described adverse events resulting from sternal bone marrow aspiration procedures. We hypothesized about inevitable lacerations to the ascending aorta that occur from structural abnormalities in the needle passage.
Methods We evaluated dimensions in the thorax related to the sternal bone marrow aspiration, using a chest CT scan on a display terminal.
Patients Among the 3,848 patients who visited our institution and underwent a chest CT scan between September 1, 2007 and December 31, 2008, a total of 153 subjects showing no particular chest CT findings were enrolled.
Results Significant variation was observed in the distance from the sternum to the ascending aorta, from a minimum of 4.2 to a maximum of 47.6 mm (median 23.6) in men and a minimum of 5.2 to a maximum of 38.4 mm (median 21.3) in women. There were three men (3.6%) and women (4.3%) each having a distance within 10 mm.
Conclusion Our findings showed for the first time that an anterior chest approach to sternal bone marrow aspiration involves an inevitable risk of laceration of the ascending aorta, when the needle tip penetrates the sternum. Our analysis of dimension also suggested that the sternal marrow aspiration procedure involved a greater risk among older subjects of short stature.

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Background The present study was aimed to evaluate the role and safety of surgical lung biopsies (SLB) in patients with interstitial lung disease (ILD) in China.
Methods A retrospective analysis of 418 patients with ILD undergoing SLB from Chinese literature and the data of our hospital during the past ten years was performed.
Results A total of 418 cases underwent SLB, including open lung biopsy (OLB) in 229 cases and video-assisted thoracoscopic lung biopsy (VATLB) in 189 cases. SLB yielded a specific diagnosis for 88.0% cases, and unclassifiable idiopathic interstitial pneumonia diagnosis was seen in 9.1% cases. The total postoperative complication rate was 12.0% and mortality rate was 1.9%. The diagnostic yield, post-operative complication rate, and mortality rate between VATLB and OLB had no significant difference.
Conclusion SLB is a very useful and relatively safe procedure for diagnosis of ILD.

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Background With the wide use of antibiotics, antibiotic-resistant Helicobacter pylori strains are becoming increasingly prevalent. It has been hypothesized that culture-guided therapy might help to increase treatment success. But the effects and the costs still remain controversial.
Aims To systematically review the efficacy and the cost of culture-guided triple therapy, compared to standard triple regimen for first-line treatment of Helicobacter pylori infection.
Methods A search of the Cochrane Library, PubMed, EMBASE, Science Citation Index Expanded and CBM was performed. Randomized controlled trials comparing culture-guided triple therapy to standard triple therapy in the first-line treatment of Helicobacter pylori infection were selected for meta-analysis. Relative risk was used as a measure of the effect of two regimens mentioned above with a fixed-effects model using the methods of DerSimonian and Laird.
Results Five randomized controlled trials totaling 701 patients were included. The meta-analysis showed that culture-guided triple therapy was superior referring to a higher eradication rate from intention-to-treat analyses (RR, 0.84; 95% CI,0.77, 0.90; p<0.00001) and a lower overall cost.
Conclusion Culture-guided triple therapy was more effective than standard triple therapy for first-line treatment of Helicobacter pylori infection. Based on the only paper focused on the overall cost, the culture-guided triple therapy was also more cost saving. Antimicrobial susceptibility testing is necessary before first-line treatment for Helicobacter pylori infection.

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Objective Due to its high morbidity rates, brucellosis -a systemic inflammatory disease, is still an important health problem, particularly in Mediterranean regions. One-third of the patients are characterized with musculoskeletal involvement. Principally in chronic cases, there are difficulties in the follow-up of therapy success. Radiological imaging methods are used in musculoskeletal brucellosis in addition to standard serological tests. Two macrophage products, namely neopterin (NPT) and chitotriosidase (ChT), are used as novel markers in order to reflect the status of inflammatory diseases. In this study, we aimed to test the validity of these markers in follow-up of patients with brucellosis.
Patients and Methods A total of 40 brucellosis cases were included in the study and 27 healthy individuals were used as controls. Twenty of the brucellosis patients were presented with sacroiliac joint involvement. A 6-week treatment of doxycycline combined with rifampicin or streptomycin was used to treat brucellosis. Clinical observations and serological outcome were used to determine whether treatment was successful or not.
Results All of the 20 brucellosis patients without musculoskeletal involvement healed with the first cure of treatment, but all of the brucella-sacroiliitis patients had to be retreated. In addition to routine testing, serum NPT and ChT levels were evaluated after each treatment. The results presented a clear fall in both NPT and ChT levels in parallel with the serological data of the patients.
Conclusion In conclusion, NPT as well as ChT seems to be a useful marker in the follow-up of brucellosis patients and for evaluating the success of therapy.

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We describe a 72-year-old woman with chronic hepatitis C and autoimmune thrombocytopenic purpura (AITP) during pegylated interferon (PEG-IFN) α. Immunoglobulin G and antinuclear antibody were 2,113 mg/dL and 1,280 at the start, respectively. A liver biopsy negated autoimmune hepatitis. After a 48-week combination therapy with ribavirin, PEG-IFN α-2a was administered. At the 30th month, the platelet count was decreased to 1.1×10⁴/μL. Bone marrow biopsy disclosed normocellular marrow compatible with AITP. The platelet-associated IgG (PAIgG) titer rose to 500 ng/10⁷ cells. Corticosteroid therapy was successful, and the platelet count and PAIgG titer reverted to 6.4×10⁴/μL and 57.3 ng/10⁷ cells, respectively.

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Cronkhite-Canada syndrome (CCS) is a rare, noninherited gastrointestinal polyposis syndrome associated with characteristic ectodermal abnormalities. Here, we report a case of Cronkhite-Canada syndrome with cecal intussusception relieved by colonoscopy. A 52-year-old man who was diagnosed as CCS pathologically two years previously presented abdominal pain and sub fever-up. Physical examination revealed the palpable mass sized approximate 10 cm in diameter in the upper abdominal site, in addition to the symptoms of alopecia, absent fingernails and toenails. However, abdominal wall rigidity and rebound tenderness were never expressed. Abdominal plain CT showed concentric circles from the ascending to the middle of the transverse colon, and a tumor in the lumen at the middle of the transverse colon. Colonoscopic reduction was performed first because we diagnosed it as intussusception due to CCS polyps without peritoneal irritation, and his symptoms were improved dramatically after careful reduction. Therefore, he was able to undergo the laparoscopic ascending colectomy as scheduled.

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Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive sterol storage disease caused by a mutated sterol 27-hydroxylase (CYP27A1) gene. We analyzed the CYP27A1 gene in two Japanese CTX patients. The CYP27A1 gene was amplified by PCR and screened by PCR-SSCP. The nucleotide sequence was analyzed to confirm mutations. Case 1 was a compound heterozygote for Arg104Gln in exon 2 and Arg441Gln in exon 8. To our knowledge, this is the first report in which the Arg104Gln mutation is identified in CTX patients. Probably case 2 would be a compound heterozygote for Arg441Trp in exon 8 and a mutation that was not identified.

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We report the case of a 57-year-old woman with anorexia nervosa showing evidence of "tako-tsubo" cardiomyopathy complicated by several syncopes due to recurrent episodes of torsades de pointes. Prolongation of QT interval and QT dispersion have been reported both in the "tako-tsubo" cardiomyopathy and in anorexia nervosa. The QT prolongation and the QT dispersion has been linked as risk indicators for sudden cardiac death. The combination of "tako-tsubo" cardiomyopathy with a condition associated with the prolongation of QT and/or with an increase of QT dispersion, such as anorexia nervosa, makes the acute and subacute prognosis of this disease much more severe than usual.

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Chronic myelogenous leukemia (CML) is a myeloproliferative disease that originates in abnormal pluripotent bone marrow stem cells and it is consistently associated with the Philadelphia chromosome and/or BCR/ ABL fusion gene. Renal infiltration of leukemic cells is relatively rare in CML and is associated with renal impairment. We describe a patient who developed acute renal failure by tubulointerstitial nephropathy during treatment with imatinib mesylate for CML. The acute kidney injury was subsequently found to be due to direct leukemic infiltration. Treatment with hydroxycarbamide and prednisolone resulted in stabilization of the renal function for approximately 4 months. Leukemic infiltration into the kidney should always be considered when a patient with CML presents with renal impairment, regardless of the clinical stage, as the renal failure often responds well to chemotherapy.

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In the autumn two patients with general malaise were consecutively admitted to the department of internal medicine of our hospital in the northern region of Nagasaki Prefecture. Since both patients were engaged in rice farm work and showed conjunctival suffusion and pain of the gastrocnemius muscle, leptospirosis was suspected. The first patient required temporary hemodialysis for renal dysfunction, whereas liver dysfunction developed in the second patient. The disease was remitted by antimicrobial agents. A diagnosis of leptospirosis was made serologically in both patients. Leptospirosis should be considered as a differential diagnosis when a patient engaged in farm work in the autumn has typical symptoms, and an early initiation of treatment after onset is important.

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A 46-year-old woman was admitted to our hospital because of pain in the right upper quadrant and dyspnea. Abdominal and chest computed tomography (CT) scans revealed areas of low attenuation in both hepatic lobes, left pleural effusion, and multiple nodules in both lungs. Laboratory data indicated disseminated intravascular coagulation. She developed rapidly progressive respiratory and hepatic failure despite intensive treatment including mechanical ventilation and died of respiratory failure 3 weeks after admission. Immunohistochemical analysis of liver necropsy and cytology of the left plural effusion stained positive for factor VIII-related antigen and CD31. Based on these observations, a diagnosis of hemangioendothelioma (EHE), a rare vascular tumor, was made. A rapid clinical course and fatal outcome, as in the present case, are rare clinical manifestations in EHE.

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We present a rare case of a tuberculous retropharyngeal abscess (RPA) associated with spinal tuberculosis (TB) (Pott's disease). A patient presented with RPA and collapse of the second cervical vertebra. Fine needle aspiration was performed through the pharynx, not only for diagnosis but also for reduction of the abscess. Tuberculous RPA was diagnosed by microbiological tests of the aspirated fluid from the abscess, which was likely to be extended from Pott's disease. Anti-TB chemotherapy after the aspiration proved effective, resulting in the resolution of the abscess. Early diagnosis and treatment are essential in order to prevent life-threatening complications.

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Maintaining alertness during noninvasive positive pressure ventilation (NPPV) is important, but there are no established guidelines for the use of sedation. We report our first experience of an infant with post-surgical vocal cord paralysis, severe stridor and breathing difficulties, who was reintubated after NPPV treatment without sedation, but who avoided a third reintubation through the use of sedation with the second NPPV treatment. NPPV treatment with the proper sedation can improve blood gas data in those patients with severe dyspnea, which can occur during respiratory care in several situations, and can affect not only infants, but also adults including elderly patients.

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A 62-year-old man with empyema caused by Aspergillus fumigatus was successfully treated with a combination of voriconazole (VRCZ) and micafungin (MCFG). Data regarding the penetration of antifungal agents into pleural fluid are limited. Thus, we measured the concentration of VRCZ and MCFG in his plasma and pleural fluid. Penetration of VRCZ and MCFG into the pleural fluid was excellent. Therefore, the combination therapy using VRCZ and MCFG may contribute to successful management of Aspergillus empyema.

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A 71-year-old man with a right hilar mass shadow was referred to our department. Chest computed tomography showed a small fatty area inside. Convex probe endobronchial ultrasound (CP-EBUS) images revealed a heterogeneous low-echoic mass, resembling a stone wall and showing a smooth sliding movement along the tracheal wall during respiration. Transbronchial needle aspiration (EBUS-TBNA) was performed and cartilage cells were identified, compatible with the presence of a hamartoma. The patient requested follow-up without surgical intervention. CP-EBUS images are easier to interpret than other methods for monitoring respiratory dynamics. We conclude that CP-EBUS is a useful addition to techniques both for non-invasive imaging as well as guiding pathological examination.

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A case of IgG4-related lung disease in a worker who had been exposed to asbestos is described. The patient had nocturnal cough and wheeze that responded to inhaled corticosteroid, and the radioallergosorbent test was positive against common allergens, suggesting an association with atopic asthma. IgE elevation is reported in asbestos-exposed workers, and asbestos exposure may cause atopic conditions. Predominance of Th2 cytokines and up-regulation of regulatory T lymphocytes have been reported in IgG4-related disease. IgG4-related disease may occur from hypersensitivity of the regulatory immune system to atopic conditions. Asbestos exposure may be a causal factor of IgG4-related disease.

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A standard treatment has not yet been established for elderly small-cell lung cancer patients, especially when they have end-stage renal disease. We report the first case of successful chemoradiotherapy in an elderly small-cell lung cancer patient undergoing continuous ambulatory peritoneal dialysis. A 77-year-old Japanese man on continuous ambulatory peritoneal dialysis was diagnosed as having limited disease small-cell lung cancer. He received four monthly cycles of chemotherapy consisting of carboplatin at 240 mg/m² on day 1 and etoposide at 40 mg/m² on days 1 and 3. He underwent additional hemodialysis on days 1 and 3, while continuous ambulatory peritoneal dialysis continued as usual on the other days. Following chemotherapy, he underwent hyperfractionated radiotherapy to a total dose of 45 Grey, resulting in complete remission of the disease. A pharmacokinetic study showed an area under the concentration-time curve of carboplatin of 3.41 to 4.88 mg·min/mL, increasing gradually over the first three cycles, while etoposide did not show this gradual increase. The increased area under the concentration-time curve of carboplatin may have reflected a worsened renal function during chemotherapy. Despite dose reductions and favorable areas under the concentration-ime curve of carboplatin, the patient suffered grade 3-4 hematological toxicities, necessitating transfusions and a further dose reduction. The patient died of recurrent small-cell lung cancer 19 months after diagnosis.

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A 42-year-old man was admitted to our hospital because of exertional dyspnea. He had worked as a metal grinder for 3 years, but has quited his job 1 month before admission. Chest radiography and high-resolution computed tomography showed diffuse ground-glass opacities like hypersensitivity pneumonitis shadows. The results of high-energy dispersion X-ray microanalysis indicated that the patient had hard metal pneumoconiosis associated with tungsten. Since the histological changes distributed terminal to respiratory bronchiole and surrounding alveoli, and macrophages engulfed black granules within the alveoli, in absence of giant cells, we considered this case to be a type of hypersensitivity pneumonitis of hard metal lung.

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A 33-year-old man with myelodysplastic/myeloproliferative disease underwent allogeneic bone marrow transplantation. Around day 80 post-transplant, he complained of abdominal pain and diarrhea. Colonoscopy and esophagogastroduodenoscopy findings were unremarkable. Double-balloon enteroscopy revealed atrophic villi and mild erosions localized in the small intestine. Histological examination revealed marked proliferation of histiocytes with numerous acid-fast bacilli in their cytoplasm. The specific polymerase chain reaction for Mycobacterium tuberculosis was negative, and a diagnosis of intestinal non-tuberculous mycobacteria (NTM) was made. Physicians should recognize that NTM infection is one of the gastrointestinal infectious complications in immunocompromised patients such as bone marrow transplant recipients, and could localize in the small intestine.

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A 28-year-old man with marked eosinophilia is described. FIP1L1/PDGFRA mRNA showed multiple alternatively-spliced fusion transcripts. Sequencing analysis showed that the deduced DNA breakpoints were intron 10 in the FIP1L1 gene and exon 12 in the PDGFRA gene. Then, a diagnosis of chronic eosinophilic leukemia (CEL) was made. Whereas the response to the treatments with prednisolone and hydroxyurea were unsatisfactory, treatment with imatinib showed a rapid decrease of eosinophils. The hemoglobin level also dropped and bone marrow examination showed pure red cell aplasia. Continued administration of very low dose imatinib (100 mg every 5 days) led to and maintained complete molecular remission, with good tolerability.

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We describe a 72-year-old man who developed subacute onset parkinsonism caused by neuro-Sweet disease (NSD). Magnetic resonance imaging of this patient's brain suggested inflammation of the bilateral basal ganglia and amygdalae. Clinical symptoms and MRI findings gradually improved without medication. However, his parkinsonism recurred one month after discharge from the hospital, at which time he was treated with corticosteroids, resulting in improvement again. His clinical course and human leukocyte antigen typing suggested that he was suffering from NSD. This case is the first report of NSD presenting with self-remitting and reversible parkinsonism.

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A 71-year-old man developed postural tremor and was treated as an essential tremor patient. Nine years after the tremor onset, he developed symptoms resembling Fragile-X-associated tremor/ataxia syndrome (FXTAS), including exacerbated (increased coarseness and amplitude) tremor in the right arm, ataxic gait, and brain MRI showed lesions in the bilateral middle cerebellar peduncles (MCP). Evidence of premutation in the form of 83 CGG repeats of the Fragile-X-mental retardation 1 (FMR1) gene confirmed the diagnosis of FXTAS. FXTAS causes various neurological symptoms including in some cases tremor resembling essential tremor in the early stages. FMR1 gene premutation should be checked when the patient develops intention tremor, cerebral dysfunction and/or a brain MRI shows MCP lesions.

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We report a 52-year-old Japanese man showing both upper and lower motor neuron signs with familial amyotrophic lateral sclerosis (ALS). Analysis of the TAR DNA-binding protein of 43 kDa (TDP-43) gene (TARDBP) revealed a glycine-to-serine substitution at position 298 (G298S). Cerebrospinal fluid (CSF) level of total tau protein (CSF-tau) of our patient was found to be highly elevated compared with those of sporadic ALS cases and controls. The elevated CSF-tau level might be related to the damage of neurons exhibiting a large number of TDP-43 inclusions in familial ALS with this mutation.

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We report a 61-year-old Japanese woman with transthyretin (TTR) Val33-related familial amyloid polyneuropathy (FAP). She presented with late-onset, vitreous involvement as the initial manifestation, slow development of polyneuropathy, cardiomyopathy, and severe autonomic failure without carpal tunnel syndrome. Liver transplantation was performed and her postoperative course was stable. Taken together with previous reports, vitreous opacities seem to be common to Val33 FAP. Vitreous amyloidosis is usually seen in combination with the involvement of other visceral organs. The findings in the present case emphasize that vitreous opacities could be the first manifestation of FAP.

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Thrombotic microangiopathic hemolytic anemia (TMHA) caused by antiphospholipid syndrome (APS) is generally associated with renal or neurological complications and plasma exchange is the first line of treatment. We present the case of a 72-year-old woman with APS who had chronic TMHA and pulmonary hypertension without other major complications. TMHA and pulmonary hypertension were refractory to plasma exchange but were treated successfully with anticoagulant monotherapy. Contrast-enhanced computed tomography and perfusion scintigraphy did not detect signs of pulmonary embolism. TMHA localized in pulmonary microvasculature which causes pulmonary hypertension is a very rare complication of APS.

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A 53-year-old man was admitted to our hospital with thoracic back pain and weight loss. Computed tomography revealed inflammatory aortic aneurysm (IAA) of the descending aorta. Sealed rupture of the aneurysm occurred while the patient was under corticosteroid therapy. Endovascular aneurysm repair (EAR) was performed without postoperative complications. Periaortic fibrosis was remarkably decreased three months later while the patient was under prednisolone (20 mg) administration. We believe that EAR could become a practical alternative to open surgical repair. The possibility of an aneurysm rupturing during corticosteroid therapy for IAA should be considered.

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Histiocytosis sometimes involves the joints, and is one of the important differential diagnoses of osteoarthropathy. A 31-year-old man presented with recurrent fever and bilateral knee arthritis two years prior to admission. He also showed the hypopituitary mass lesion and partial hypopituitarism. X-ray studies showed both osteosclerotic and osteolytic lesions near the large joints. Histological findings of bone biopsy revealed foamy macrophage infiltration, which were CD68+CD1a-S100-, and Erdheim-Chester disease was diagnosed. In addition, CD68+CD1a+ Langerhans' cells also aggregated in the same lesions, and we thought this case was a rare variant of Erdheim-Chester disease with overlapping histiocytic invasion.

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Thrombotic thrombocytopenic purpura (TTP), scleroderma renal crisis (SRC), and hemolysis, elevated liver enzyme levels, and a low platelet count (HELLP) syndrome display common symptoms that include microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Therefore, it is important to distinguish between them because their treatments vary: however, the differential diagnosis is sometimes difficult. We report a 32-year-old woman who was referred to our department for further examination of microangiopathic hemolytic anemia, thrombocytopenia, and a slightly elevated serum creatinine level with anti-centromere antibody-positive Raynaud's syndrome in the early puerperal period. TTP, SRC, and HELLP syndrome were considered in the differential diagnosis, but the measurement of a disintegrin-like metalloprotease with thrombospondin type 1 motifs 13 (ADAMTS 13) activity and its inhibitor level led to the diagnosis of TTP. She was successfully treated by plasma exchange and high-dose prednisolone and angiotensin-converting enzyme inhibitor. If microangiopathic hemolytic anemia and thrombocytopenia are observed in perinatal women or patients with signs of systemic sclerosis, the measurement of ADAMTS13 activity and its inhibitor level are essential for diagnosis and therapeutic choice.

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A 17-year-old boy presented with a large cavity and bilateral nodular opacities on his chest roentgenogram. Mycobacterium avium was identified in his sputum. According to the recommendations of the American Thoracic Society, he was not strongly recommended to undergo surgery because of non-localized lesions. But since cavities can provide a means for disease to spread to other lobes, we decided to perform a lobectomy including the cavity combined with chemotherapy. Now he has been well for 4 years without exacerbation. There is a possibility of long-term remission with this combination treatment in cases a destructive lesion of airway such as a cavity which is localized to one lobe, even if other lesions such as nodular opacities exist in many other lobes.

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We herein report a case study of an HIV-1-infected 64-year-old Japanese man who presented, with an abdominal aortic aneurysm due to non-typhoidal Salmonella. He was admitted with a 7-day history of intermittent left back pain. A culture of a blood specimen yielded gram-negative bacilli, which were identified as non-typhoidal Salmonella. Computed tomography showed an abdominal aortic aneurysm due to the non-typhoidal Salmonella infection. Since such a complication is frequently fatal, its management, especially the timing of surgery, is difficult. Further studies are needed to determine the optimal treatment strategy, however, early diagnosis and prompt careful treatment can reduce mortality.

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