Cardiac arrest in a patient with polyarteritis nodosa.

Polyarteritis nodosa (PAN) is a rare necrotizing vasculitis that occurs in small- to medium-sized muscular arteries. A 71-year-old man was admitted to our hospital for an evaluation of a sustained fever and he later died of cardiac arrest. The autopsy revealed that the patient had PAN and severe coronary vasculitis with no signs of cardiac ischemia. The reason for the patient's sudden death remains unclear; however, cardiac arrest due to coronary vasculitis was suggested. This is a rare case of PAN coronary vasculitis that led to sudden death without either stenosis or any embolism.

Histopathologic profiles of coronary atherosclerosis by myocardial bridge underlying myocardial infarction.

OBJECTIVE: Anatomic properties of myocardial bridge (MB) are sometimes responsible for myocardial infarction (MI) through the changes in the atherosclerosis distribution in the left ascending coronary artery (LAD). The purpose of this study was to investigate histopathologic profiles of atherosclerotic lesions resulting from the MB presence in the LAD in the MI cases. METHODS: In 150 consecutive autopsied MI hearts either with MBs [MI(+)MB(+); n = 67] or without MBs [MI(+)MB(-); n = 83] and 100 normal hearts with MBs [MI(-)MB(+)], LADs were consecutively cross-sectioned at 5-mm intervals. The most advanced intimal lesion and unstable plaque-related lesion characteristics (UPLCs) in each section were histopathologically evaluated in conjunction with the anatomic properties of the MB, such as its thickness, length, location, and MB muscle volume burden (MMV: the total volume of MB thickness multiplied by MB length). RESULTS: The MB showed a significantly greater thickness (P = 0.0090), length (P = 0.0300), and MMV (P = 0.0019) in MI(+)MB(+) than in MI(-)MB(+). Mean age of acute MI cases was significantly younger (P = 0.0227) in MI(+)MB(+) than in MI(+)MB(-). Frequency of plaque fissure/rupture in the proximal LAD was significantly higher in acute MI cases of MI(+)MB(+) than in MI(+)MB(-). UPLCs tended to be located proximally in MI(+)MB(+) and frequent 2.0 cm or more proximal to the MB entrance in MI(+)MB(+). CONCLUSION: In MI(+)MB(+), UPLCs tend to be located more proximally, and a plaque in the LAD proximal to the MB is prone to rupture, resulting in MI at younger age.

[Assessment of clinical features in solitary minimum size (<1 cm) bladder cancer].

We retrospectively evaluated primary non-muscle-invasive bladder cancer diagnosed between 1999 and 2008 at 2 facilities (Kawasaki Municipal Hospital and Yokohama Minami Kyosai Hospital). Size (< 1 cm) solitary bladder cancer statistically evaluated the characteristics. Out of 463 bladder cancers, 52 were minimum-size solitary pTa bladder cancers less than 1 cm in diameter. The average follow-up period was 50.9 months. The recurrence rate of the minimum-size bladder cancer was significantly lower than that of bladder cancers of other sizes (1 to 3 cm or ≥ 3 cm). The 3-year non-recurrence rate was 80.7,71.0,and 62.9% in each group (< 1, 1 to 3, ≥ 3 cm). High-grade minimum size bladder cancer (pTa) showed a significantly higher recurrence rate than the low-grade cases (P = 0.0101). Intravesical chemotherapy with anti-cancer drugs significantly reduced the intravesical recurrence rate in the low-grade minimum-size bladder cancer group (P = 0.0418). There was no statistically significant difference in either the average recurrence number or the rate of multiple recurrences between the minimum-size tumor group and the 1 to 3 cm tumor group. Minimum size bladder cancer had a lower recurrence rate than tumors of other sizes; however, there were no differences in other characteristics between the groups. Therefore, sufficient treatment, in accordance with the guidelines, should be administered for minimum size tumors as well as tumors of other sizes.

[Assessments of progression in non-muscle-invasive bladder cancer].

We retrospectively studied 463 patients with primary non-muscle-invasive bladder cancer diagnosed between 1999 and 2008 at two facilities (Kawasaki Municipal Ida Hospital and Yokohama Minami Kyosai Hospital). In this study, disease progression was defined as invasion to the muscle or further (upstage) and presence of metastasis (metastasis). We detected progression in 22 cases, including 18 upstages and 4 metastasis. Univariate analysis showed that factors associated with progression were T category (pT1 p< 0.0001), grade (high grade p< 0.0001, G3 p< 0.0001) and number of tumors (multiple p=0.0213). Multivariate analysis showed that the only equivocal factor associated with progression was T category (T1). Use of a second tansurethral resection for high-grade pT1 cases was unrelated to progression. Among the patients with progression, many had a more advanced T category at the time of radical treatment, and the results of treatment were poor. The factors associated with progression of bladder cancer should be investigated in more detail, so that early radical treatment can be initiated in eligible patients.

Polymyositis associated with autoimmune hepatitis, primary biliary cirrhosis, and autoimmune thrombocytopenic purpura.

We describe a 40-year-old woman with polymyositis (PM) who developed autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and autoimmune thrombocytopenic purpura (AITP) concurrently. About 4 years earlier, she suffered from muscle weakness probably due to PM. When she visited our hospital, she had polyarthritis, myalgia, symmetrical proximal limb-muscle weakness, elevated muscle enzymes, and myogenic abnormalities on electromyogram. Pathological findings obtained by muscle biopsy showed histological findings consistent with PM. Her serum liver enzymes were also elevated. The histology obtained by liver biopsy revealed the mixture findings of chronic active hepatitis and biliary cirrhosis. As antibodies to mitochondria M2 and liver/kidney microsome type 1 (LKM-1) were present, we concluded her liver disease was due to an overlap of AIH and PBC. Furthermore, purpura on the legs with thrombocytopenia appeared in parallel with liver dysfunction. She was diagnosed as having AITP by clinical and laboratory findings. Her serum showed a speckled pattern in immunofluorescence antinuclear antibody testing, but the antigen specificities were distinct from those of the known myositis-related autoantigens. This is a first case report of PM accompanied by AIH, PBC, and AITP. It was notable that there was an overlap of disease-associated immunological findings and immunogenetic backgrounds. This case provides a possible insight into the mechanisms and interplay of autoimmune diseases.

[A case of Creutzfeldt-Jakob disease diagnosed with a combination of ER visit by the patient and a home visit by the physician].

In case of home care patient's health gets deteriorated, an admission is often requested due to a nursing care need rather than a medical need. However, it is often inevitable to transfer a patient to other nursing home, or to observe the patient at home, is caused by a limitation of medicalre sources and the capacity of the patient to adapt environmental changes. It may be criticized that a medical care of the elderly at home is left unattended as a consequence of the oversight of critical pathophysiologic changes. We have experienced a case diagnosed as Creutzfeldt-Jakob disease, which was not suspected at the initial visit of our ER unit. A grave hidden disease was recognized by next day's emergency visit and a follow-up elaboration examination at our hospital. Such cooperation may have a potency maintaining a home medical treatment level by grasping the precise pathophysiology of home elderly patients.

Anatomic properties of myocardial bridge predisposing to myocardial infarction.

BACKGROUND: A myocardial bridge (MB) that partially covers the course of the left anterior descending coronary artery (LAD) sometimes causes myocardial ischemia, primarily because of hemodynamic deterioration, but without atherosclerosis. However, the mechanism of occurrence of myocardial infarction (MI) as a result of an MB in patients with spontaneously developing atherosclerosis is unclear. METHODS AND RESULTS: One hundred consecutive autopsied MI hearts either with MBs [MI(+)MB(+) group; n=46] or without MBs (n=54) were obtained, as were 200 normal hearts, 100 with MBs [MI(-)MB(+) group] and 100 without MBs. By microscopy on LADs that were consecutively cross-sectioned at 5-mm intervals, the extent and distribution of LAD atherosclerosis were investigated histomorphometrically in conjunction with the anatomic properties of the MB, such as its thickness, length, and location and the MB muscle index (MB thickness multiplied by MB length), according to MI and MB status. In the MI(+)MB(+) group, the MB showed a significantly greater thickness and greater MB muscle index (P<0.05) than in the MI(-)MB(+) group. The intima-media ratio (intimal area/medial area) within 1.0 cm of the left coronary ostium was also greater (P<0.05) in the MI(+)MB(+) group than in the other groups. In addition, in the MI(+)MB(+) group, the location of the segment that exhibited the greatest intima-media ratio in the LAD proximal to the MB correlated significantly (P<0.001) with the location of the MB entrance, and furthermore, atherosclerosis progression in the LAD proximal to the MB was largest at 2.0 cm from the MB entrance. CONCLUSIONS: In the proximal LAD with an MB, MB muscle index is associated with a shift of coronary disease more proximally, an effect that may increase the risk of MI.

Disseminated cryptococcal infection with eosinophilia in a healthy person.

A 23-year-old man with no recent medical history was hospitalized complaining of high fever and cough. In addition to very marked eosinophilia, chest X-ray revealed extensive bronchovascular bundle thickening. Transbronchial lung biopsy (TBLB) showed moderate eosinophil infiltration. Cryptococcus neoformans infection was diagnosed, based on blood culture, cerebrospinal fluid culture, urine culture, and lung biopsy specimens. The eosinophilia was successfully alleviated by treatment for cryptococcal meningitis. Furthermore, cryptococcal sepsis resolved with amphotericin B and 5-flucytosine treatment. Eosinophilia commonly occurs following chronic Aspergillus infection, but the present case suggests the involvement of Cryptococcus in another mechanism for eosinophilia.

[Case of pulmonary MALT lymphoma with follow-up imaging for 10 years].

Although abnormal shadow in the left upper lung of an 84-year-old male patient was confirmed in an examination in November 1996, follow-up observation was discontinued. In July 2006, he first visited our department with a chief complaint of shortness of breath, and was hospitalized because of an abnormal shadow in the left upper lung field and left pleural effusion. Since atypical lymphocytes were found in the pleural effusion, and positive cellular surface markers CD19 and 20, and chromosomal aberration of t (11 ; 18) (q22 ; q21) were confirmed, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) was diagnosed. Transbronchial lung biopsy of the left upper lobe confirmed small lymphocyte-like cellular infiltration, as seen in the pleural effusion, and CD20 immunostaining was positive, leading to the diagnosis of MALT lymphoma. In addition, serum immunoelectrophoresis demonstrated the development of macroglobulinemia as a complication. This case is valuable as changes diagnostic image over 10 years can be compared.

Rapidly progressive invasive pulmonary aspergillosis in a diabetic man.

A 45-year-old man was admitted to our hospital with high fever and a large amount of gray sputum. His initial chest X-ray showed broncho-bronchiolitis and thickening of the large bronchus, and he was subsequently diagnosed with pulmonary aspergillosis based on his sputum culture, polymerase chain reaction for Aspergillus fumigatus, and mannan antigen for Aspergillus. His immune responses, including neutrophil phagocytosis function and neutrophil sterilizing function, were normal as far as we could determine. He was treated with itraconazole, amphotericin B, and meropenem trihydrate, but died of respiratory failure on the twenty-fifth hospital day. Chest X-ray showed rapidly progressive invasive shadows in both lung fields, resulting in multiple cavity formation. This was a rare case of invasive pulmonary aspergillosis in a diabetic man with normal neutrophil phagocytosis function and neutrophil sterilizing function.

Intradiploic epidermoid cyst with focal internal enhancement.

We report a case of intradiploic epidermoid cyst with focal internal enhancement. The patient was a 55-year-old woman presenting with right temporal head protrusion and head heaviness. Skull radiography and computed tomography demonstrated an expansile mass in the right diploic space. On magnetic resonance imaging, the mass showed makedly high signal T2-weighted images (T2WI) and intermediate to low signal on T1WI. On contrast-enhanced T1WI, there was nodular internal enhancement, which corresponded to neovascularity on pathology, as well as rim-like enhancement. It should be noted that internal enhancement, an indicator of malignant transformation, may be seen even in a benign epidermoid cyst.

Increased occurrence of caspase-dependent apoptosis in unfavorable neuroblastomas.

Neuroblastoma frequently shows spontaneous regression in which two distinct types of programmed cell death, ie, caspase-dependent apoptosis and H-Ras-mediated autophagic degeneration, have been suggested to play a key role. The current study was conducted to determine which of these cell suicide pathways predominated in this tumor regression. Periodic acid-Schiff (PAS) staining and immunostaining for H-Ras and for the full-length and cleaved forms of caspase-3, poly (ADP-ribose) polymerase (PARP), and lamin A were carried out on 55 archival tumor specimens. The incidence of caspase-dependent apoptosis in each tumor was quantified by cleaved lamin A staining and compared with clinicopathologic prognostic factors. Although a recent report has shown that neuroblastic cells undergoing autophagic degeneration were readily detectable by PAS and H-Ras staining, we could not confirm this result in any of our samples with the exception of one tumor. Instead, many of our neuroblastoma samples showed nonspecific PAS and Ras staining in areas of necrosis, suggesting that autophagic "degeneration" indeed corresponds to coagulation necrosis or oncosis. Unexpectedly, the incidence of caspase-dependent apoptosis was significantly correlated with indicators of a poor prognosis in these tumors, including Shimada's unfavorable histology, MYCN amplification, and a higher mitosis-karyorrhexis index, but not with factors related to tumor regression such as clinical stage and mass screening. These results indicate that neither caspase-dependent apoptosis nor autophagic "degeneration" may be involved in spontaneous neuroblastoma regression. This suggests that other mechanisms, perhaps such as tumor maturation, may be responsible for this phenomenon.

A rare case of olfactory neuroblastoma with intracranial extension.

Olfactory neuroblastoma (ONB) is a rare form of head and neck tumor, and further ONB presenting an intracranial extension is extremely rare. Therefore, at the present time there is no established therapeutic standard supported by oncological studies. Our report documents a patient who underwent a craniotomy in combination with transnasal approach for tumor excision, followed by postoperative irradiation. We conclude that the patient shows no evidence of recurrence during the 11-year follow-up involving sinus CT scans and endoscopic examinations.

A case report of difficult diagnosis in the patient with advanced laryngeal tuberculosis.

We report a patient with severe laryngeal tuberculosis (LTB) involving thyroid cartilage and combined with whole-bone metastasis. A 57-year-old male had presented only with hoarseness. Radiological findings were indicative of suspected metastasis from a malignant tumor. However, tuberculosis was considered by histopathological findings, and so sputum samples were tested for acid-fast bacilli and purified protein derivatives of tuberculin in order to detect the presence of LTB. A polymerase chain reaction confirmed the diagnosis. Anti-tuberculous medications were effective in resolving the hoarseness, and the removal of the mass in the right wing of thyroid cartilage was confirmed by computed tomography (CT).

Increased transforming growth factor-beta1 and tubulointerstitial fibrosis in rats with congenital hydronephrosis.

OBJECTIVES: Most of our knowledge concerning renal obstruction has been derived from experimental animal models, and it is not yet well defined in spontaneous hydronephrosis. The aim of our study is to evaluate the roles of transforming growth factor-beta1 (TGF-beta1) and apoptosis in congenital hydronephrotic kidneys in comparison with experimental models. METHODS: We made histological studies on kidneys from 6-week-old Wistar-Imamichi rats with congenital unilateral hydronephrosis as well as surgical models of complete or partial unilateral ureteral obstruction. The severity of hydronephrotic kidneys was evaluated on routine hematoxylin and eosin (H&E) stained sections, and the tubulointerstitial fibrosis analyzed morphometrically on Masson's trichrome stained sections. Renal tubular atrophy was assessed on periodic acid Schiff (PAS) stained sections, and tubular cell apoptosis assessed with TUNEL technique. The renal TGF-beta1 level was determined by a sandwich enzyme-linked immunosorbent assay (ELISA). RESULTS: We observed a significant loss of kidney weight with profound compensatory growth of the contralateral kidney in rats with congenital hydronephrosis. Most of the hydronephrotic kidneys were markedly enlarged with dilatation of the collecting system, renal parenchymal thinning, tubular atrophy, interstitial infiltration and fibrosis. The renal TGF-beta1 level was markedly elevated in hydronephrotic kidneys as compared with normal controls (326.01 +/- 30.64 pg/mg protein vs 227.81 +/- 11.07 pg/mg protein, P < 0.01). The tubular apoptotic score in hydronephrotic kidneys was also significantly higher than normal controls (2.17 +/- 0.50/HPF [high power field]vs 0.14 +/- 0.04/HPF, P < 0.01). The increased TGF-beta1 and apoptotic status paralleled the histological changes of tubulointerstitial fibrosis and tubular atrophy. Similar findings were also obtained in experimental obstructive models. CONCLUSION: In comparison with surgical models of partial and complete ureteral obstruction, our data provide solid morphological and molecular evidences of renal obstruction in rats with congenital hydronephrosis.