Biomedical subjects

M Hironaka

Publications and source records attributed to M Hironaka.

At least 19 recordsLinked to original sources

PubMed2006

Complete remission of minimal-change nephrotic syndrome induced by apheresis monotherapy.

We report a case of a 17-year-old male with relapse of minimal-change nephrotic syndrome (MCNS), in whom apheresis monotherapy without steroids or immunosuppressants resulted in complete remission. The patient initially developed nephrotic syndrome in February 1998. The first renal biopsy confirmed the diagnosis of MCNS. The patient was also found to be a carrier of hepatitis B virus. Steroid therapy was started with oral prednisolone 60 mg/day. Complete remission was achieved in 3 months, and the steroid treatment was tapered off in May 2001. During the steroid tapering, temporal exacerbation of liver function was noted. In July 2002, the patient was admitted to our hospital again due to relapse of nephrotic syndrome. Second biopsy reconfirmed the diagnosis of MCNS. Since the serum titer of HBV was elevated, apheresis monotherapy was selected to avoid the risk of steroid-induced fulminant hepatitis. Four sessions of low-density lipoprotein apheresis (LDL-A) and 5 sessions of double-filtration plasmapheresis (DFPP) reduced the proteinuria from 9.2 g/day to 0.2 g/day over 38 days without any additional medication. Proteinuria remained suppressed below 0.2 g/day for more than 12 months and no exacerbation of liver function was observed up to the final follow-up in September 2003. The present case suggested the potential of apheresis monotherapy to induce and maintain complete remission of MCNS and an important role of circulating factors in the pathogenesis of MCNS.

Adolescent↗

PubMed2006

Fanconi's syndrome and distal (type 1) renal tubular acidosis in a patient with primary Sjögren's syndrome with monoclonal gammopathy of undetermined significance.

Tubulointerstitial nephritis is a well-recognized complication in primary Sjögrens syndrome. Fanconi's syndrome is a far less frequent complication compared with distal tubular dysfunction. We here describe a 49-year-old woman with primary Sjögren's syndrome. In 1997, she was diagnosed with primary Sjögren's syndrome with tubulointerstitial nephritis, and was then treated with oral prednisolone for the tubulointerstitial nephritis. In 2002, she was referred to our hospital because of progressive fatigue. At that time, biclonal spike on serum protein (IgG-kappa and IgA-kappa) and Bence-Jones protein in urine were found. Bone marrow aspiration showed 1.0% plasma cell infiltration. Thus, a diagnosis of monoclonal gammopathy of undetermined significance (MGUS) was made. In 2004, she was again admitted to our hospital because of mild renal dysfunction and hypokalemia. Laboratory evaluation showed inappropriate, alkaline urine in hyperchloremic metabolic acidosis and a positive urine anion gap, indicating the presence of distal (Type 1) renal tubular acidosis (RTA). The urine concentration defect was also found. Further studies revealed proximal tubular dysfunction, including renal glycosuria, generalized aminoaciduria, phosphaturia, uricosuria and proximal RTA. The kidney biopsy represented diffuse and severe tubulointerstitial nephritis with dense infiltrates of lymphocytes and IgA and K light chain-positive plasma cells. No findings of multiple myeloma or malignant lymphoma were observed. In conclusion, our patient had Sjögren's syndrome with MGUS and exhibited dysfunction of both proximal tubule (Fanconi's syndrome) and distal tubule, which may be attributed to diffuse tubulointerstitial nephritis.

Acidosis, Renal Tubular↗

PubMed2003

Distinct methylated profiles in Helicobacter pylori dependent and independent gastric MALT lymphomas.

BACKGROUND: Gastric mucosa associated lymphoid tissue (MALT) lymphomas are clinically subdivided into Helicobacter pylori dependent and independent, according to H. pylori infection and the therapeutic course. In previous reports it has been suggested that H. pylori independent cases develop from H. pylori dependent cases, and sometimes transform into high grade diffuse large B cell lymphomas (DLBCLs). METHODS: To better understand the pathogenesis of H. pylori dependent and independent MALT lymphomas, we analysed the methylation profiles of eight independent CpG islands, including p15, p16, p73, hMLH1, death associated protein kinase, MINT1, MINT2, and MINT31 in H. pylori dependent and independent MALT lymphomas, DLBCLs, and H. pylori associated chronic gastritis. RESULTS: We first confirmed that H. pylori independent cases had a high incidence of t(11;18)(q21;q21) (4/8 cases) and aberrant BCL10 expression (7/8 cases) compared with H. pylori dependent cases and gastric DLBCLs. In the methylation pattern study, all 13 H. pylori dependent MALT lymphomas had more than four methylated loci while H. pylori independent cases had less than two. According to the previous criterion, all H. pylori dependent MALT lymphomas (13/13, 100%) and five of 10 (50%) DLBCLs were classified as CpG island methylator phenotype positive (CIMP+). In contrast, all H. pylori independent MALT lymphomas were CIMP-. CONCLUSION: The distinct methylation pattern together with lack of chromosomal translocation in H. pylori dependent MALT lymphomas suggest that H. pylori dependent and independent MALT lymphomas have a different pathogenesis.

Adaptor Proteins, Signal Transducing↗

PubMed2001

Neuroendocrine small cell carcinoma of the uterine cervix showing polypoid growth and complicated by pregnancy.

BACKGROUND: Neuroendocrine small cell carcinoma of the uterine cervix is an aggressive disease, and it rarely is complicated by pregnancy. CASE: A polypoid tumor was found in the uterine cervix in a 27-year-old Japanese woman at 27 weeks of gestation. No polyp had been detected at 14 weeks of gestation. The polyp was excised and diagnosed as neuroendocrine small cell carcinoma by histological examination, including Grimelius, neuron-specific enolase, and chromogranin staining. A healthy infant was born by cesarean section at 29 weeks of gestation: this was followed by radical hysterectomy with pelvic lymphadenectomy. After surgery, four cycles of combination chemotherapy with cisplatin and etoposide were administered, and the patient is disease-free as of 13 months after surgery. CONCLUSION: When a polypoid lesion is found, especially when it demonstrates rapid growth, it may be necessary to excise and histologically examine the polyp even during pregnancy.

Adult↗

PubMed2001

Pulmonary gangliocytic paraganglioma: case report and comparative immunohistochemical study of related neuroendocrine neoplasms.

The authors report a case of gangliocytic paraganglioma of the lung, which has not yet been described in a pulmonary neoplasm. A 75-year-old man underwent right middle and lower lobe lobectomy. A slightly yellowish mass was located at the bifurcation between the lower and middle lobe bronchus, protruding into the truncus intermedius. The neoplastic cells were composed of three cellular elements: uniform endocrine cells in a Zellballen arrangement, large ganglion-like cells within the nests of endocrine cells, and spindle-shaped cells arranged in streams to surround the nests. Each component exhibited the characteristic immunohistochemical properties, which were similar to those of the corresponding neuroendocrine neoplasms: Endocrine cells were positive for CAM 5.2, chromogranin A, and synaptophysin, like carcinoid tumor; ganglion-like cells were positive only for neurofilament, like ganglioneuroma; and spindle-shaped cells were positive for neurofilament and S-100 protein, like paraganglioma. These results agreed with those in gangliocytic paraganglioma of the duodenum. Pulmonary gangliocytic paraganglioma is similar to that in the duodenum, and is a hamartomatous proliferation of epithelial endocrine and neuronal cells of the bronchus.

Aged↗

PubMed2001

Mutations in c-kit gene exons 9 and 13 in gastrointestinal stromal tumors among Japanese.

Gain-of-function mutation in c-kit proto-oncogene exon 11 has been described in about 20 -- 50% of gastrointestinal stroma tumor (GIST). Recently, additional mutational hot-spots in exon 9 and exon 13 of the c-kit gene have been reported in GISTs without mutations of exon 11, but a subsequent report in a Western population indicated that only a small portion of GISTs (eight of 200 GISTs, 4%) showed mutations in these regions. In this study, we evaluated mutations in exon 9 and exon 13 of the c-kit gene by both polymerase chain reaction-single strand conformation polymorphism analysis and direct sequencing in 48 GISTs in a Japanese population, for which the clinicopathological and immunohistochemical features and mutations in exon 11 had previously been reported. C-kit gene mutation in exon 9, representing insertion of GCC TAT, was identified in only 4 of 48 GISTs (8%), and none of the GISTs had mutations in exon 13. All four GISTs with mutation in exon 9 were high-risk, and the patients died of multiple tumor metastasis. Mutations in exon 9 and exon 13 of the c-kit gene were also rare events in Japanese GISTs and were related to a poor prognosis. These results in Japanese are consistent with those in Western populations, although a preferential occurrence of GISTs with exon 9 mutation in the small intestine, which was suggested in a previous report, was not observed.

Actins↗

PubMed2001

[Induction chemotherapy (cisplatin + vinorelbine) is found to be markedly effective for squamous cell lung carcinoma with sarcoidosis--a case report].

A sixty-one-year-old man was admitted to our hospital because of a right lung tumor shadow. He had been diagnosed as having sarcoidosis at the age of fifty-seven. He was newly diagnosed as having squamous cell carcinoma by trans bronchial biopsy. He was treated with an induction chemotherapy (cisplatin 80 mg/m2 + vinorelbine 20 mg/m2) followed by right middle and lower lobectomy with a mediastinal nodal dissection, because the stage of his carcinoma was cT2N2M0. Resected lung tissue showed the disappearance of cancer cells. Dissected mediastinal and hilar lymph nodes showed many sarcoid granulomas. Cisplatin combined with vinorelbine might be an effective chemotherapy for non-small cell lung carcinoma.

Antineoplastic Agents↗

PubMed2001

[Clinical analysis of intrapulmonary lymph nodes].

We encountered 12 cases (9 men, 3 women) of intrapulmonary lymph nodes, discovered by chest radiography or chest CT and identified by thoracoscopic lung biopsy (in 10 cases), open lung biopsy (1 case) or lobectomy (1 case). We also studied the literature related to intrapulmonary lymph nodes in Japanese. Many intrapulmonary lymph nodes were found in the lower lung field, few in the upper lung field. All intrapulmonary lymph nodes were spherical and were located under the pleura, but we were not able in some cases to differentiate them from malignancies by the CT scanfindings. We could not diagnose them or rule out malignancy before surgery. Pathological findings revealed that all of them showed anthracosis. Silicotic changes were found in three cases. We consider that thoracoscopy is useful in making a definite diagnosis if peripheral pulmonary lesions cannot be diagnosed. We emphasize that intrapulmonary lymph nodes should be taken into consideration in differential diagnoses of small nodular lesions in the lung.

Aged↗

PubMed2000

Provisioned Parastrachia japonensis (Hemiptera: Cydnidae) nymphs gain access to food and protection from predators.

Females of the shield bug Parastrachia japonensis Scott progressively provision nymph-containing nests with drupes of the host tree, Schoepfia jasminodora (Olicaceae: Rosidae: Santales). The majority of nests are 5-15 m from the host tree, a distance thought to have been a major impetus for the occurrence of provisioning in this species. However, the function of provisioning is not well understood. We carried out two field experiments to determine whether provisioning is nutritionally important and whether it affords protection against predation. Development of nymphs was significantly delayed and their survival was low in the absence of provisioning, even when nests were within the area of ground on to which the drupes fell, apparently because of the poor quality of the majority of the drupes. Selective provisioning of good-quality drupes by female P. japonensis, a semelparous species, was thus necessary for young nymphs to obtain enough food for their development. Furthermore, even without a female in attendance, having drupes in the nest significantly reduced early mortality in the presence of a predator. Copyright 2000 The Association for the Study of Animal Behaviour.

Journal Article↗

PubMed2000

[Sjögren's syndrome with multiple bullae and pulmonary nodular amyloidosis].

We report a case of Sjögren's syndrome with pulmonary involvement diagnosed by open lung biopsy. The patient was a 62-year-old woman with antiphospholipid antibody syndrome. Her chest radiograph and CT scan showed multiple bullae diffusely scattered throughout the lung. The open lung biopsy specimens revealed marked inflammatory mononuclear cell infiltration and nodular amyloid deposits in the bronchiolar walls. The mechanism of bulla formation appeared to be the check valve mechanism caused by the narrowing of the airway by the bronchiolitis. The patient was treated with oral corticosteroids, and her symptoms and laboratory findings became stable.

Amyloidosis↗

PubMed1999

p16 inactivation in small-sized lung adenocarcinoma: its association with poor prognosis.

p16, an inhibitor of cell cycle machinery, is frequently inactivated in non-small cell carcinoma of the lung (NSCCL). To clarify the significance of p16 inactivation in the progression of lung adenocarcinoma, we immunohistochemically evaluated p16 protein status and Rb, p53 and cyclin D1 expression in 51 surgically resected adenocarcinomas that were less than 3 cm in diameter (median follow-up period: 52.5 months). Twenty-one of 51 adenocarcinomas showed negative immunostaining for p16. Twenty adenocarcinomas were also negative for Rb, while 31 and 13 were positive for p53 and cyclin D1, respectively. Loss of p16 expression was significantly correlated with scar grade, lymphatic permeation, lymph node metastasis and clinical stage. Rb protein expression was also inversely correlated with scar grade, pleural involvement and vascular invasion. When the cases were stratified according to the expression of both proteins, the Rb-/p16- subset (7/51) consisted of poorly differentiated adenocarcinoma with a higher grade of invasion. While Rb, p53 and cyclin D1 protein status showed no significant correlations with prognosis, p16 inactivation was significantly correlated with poor prognosis, and the prognosis of Rb-/p16- was the worst among the 4 subsets. Inactivation of p16 may play a role in accelerating scar formation and lymph node metastasis, and may contribute through these mechanisms to poor prognosis in patients with small-sized lung adenocarcinoma.

Adenocarcinoma↗

PubMed1999

MEN1 gene mutations in sporadic neuroendocrine tumors of foregut derivation.

Foregut-derived neuroendocrine (NE) tumors occur sporadically or in association with multiple endocrine neoplasia type 1 (MEN1) syndrome. Thirty-nine sporadic NE tumors of foregut derivation (six thymic, 21 bronchial, three gastric, and nine pancreatic tumors) as well as two hindgut-derived rectal carcinoids for somatic MEN1 gene mutation were analyzed by direct sequencing analysis. Five tumors showed mutations: nonsense mutations (Q393X and R98X) in thymic and pancreatic NE tumors, respectively, a 4 b.p. deletion (357del4) in a gastric NE carcinoma, and missense mutations (D172Y and S178Y) in pancreatic NE tumors. No mutation was identified in pulmonary or rectal NE tumors. In a patient with a pancreatic NE tumor (D172Y), the corresponding germline DNA showed the same mutation, suggesting that sporadic MEN1 syndrome was masked in this case. Somatic MEN1 gene mutations and deletions may play a crucial role in the tumorigenesis of a subset of foregut-derived NE tumors. Sporadic MEN1 syndrome may occur as a sporadic NE tumor of the pancreas.

Adult↗

PubMed1999

Pulmonary fibrosis and lung carcinoma: a comparative study of metaplastic epithelia in honeycombed areas of usual interstitial pneumonia with or without lung carcinoma.

Usual interstitial pneumonia (UIP), or idiopathic pulmonary fibrosis, has been considered to be associated with a high risk for lung carcinoma. To investigate this well-known but still equivocal relationship, we reviewed the clinical features of UIP autopsy cases with or without lung carcinoma (n = 32 and 38, respectively), and compared the morphology and cell kinetics of metaplastic epithelia in the honeycombed areas (n = 11, each group). Thirty-two of 70 UIP autopsy cases showed lung carcinomas. Clinically, UIP with lung carcinoma showed a male predominance (P = 0.001), a higher rate of smoking history (P = 0.001) and a later onset of UIP (P = 0.02), compared with UIP without lung carcinoma. Most of the carcinomas were peripheral in origin (90%), and 65% were topographically associated with honeycombed areas or the border between honeycombing and non-fibrotic areas. Quantitative assessment of the metaplastic epithelia in the honeycombed areas revealed that squamous metaplasia, but not cuboidal cell metaplasia or bronchial cell metaplasia, occurred more frequently in UIP with lung carcinoma than in UIP without lung carcinoma (P = 0.02). There were no significant differences between the two groups with regard to the labeling indexes of Ki-67 and p53 in the metaplastic epithelia, including squamous metaplasia. The degree of atypical squamous metaplasia was not different between the two groups. The quantitative predominance of squamous metaplasia in the honeycombed areas may not be a precursor for lung carcinoma, but might reflect a constitutional susceptibility of UIP patients to develop a lung carcinoma.

Adenocarcinoma↗

PubMed1999

Regional proliferation of HMB-45-positive clear cells of the lung with lymphangioleiomyomatosislike distribution, replacing the lobes with multiple cysts and a nodule.

The authors report a case of a localized lesion of the lung presenting as multiple cysts and as a tumor in the right upper and middle lobes, consisting of a diffuse proliferation of clear cells with intralysosomal glycogen granules and human melanin black (HMB)-45 immunoreactivity. A 33-year-old woman complained of dyspnea because of the enlargement of bullae in the right upper and middle lung fields without stigmata of tuberous sclerosis. Resection showed multiple, various-size air-filled cysts and a tumor. The cysts in the resected lungs were reminiscent of lymphangioleiomyomatosis (LAM), accompanied by the diffuse proliferation of clear cells in the interstitium. The tumor, 1.8 cm in diameter, resembled a clear cell tumor of the lung (CCTL) and showed proliferation of clear cells with sinusoidlike vascular spaces. Both forms of proliferation were continuous spatially, and both constituent cells showed diffuse HMB-45 immunoreactivity. The cells that comprised a nodule revealed ultrastructurally abundant cytoplasmic glycogen, which was in the form of free and membrane-bound glycogen granules. This case may represent a particular pulmonary lesion consisting of CCTL-LAM hybrid cells, which share the cytologic features with CCTL cells on one hand, and the proliferative pattern and potential with LAM cells on the other.

Adenocarcinoma, Clear Cell↗

PubMed1999

[Mucosa-associated lymphoid tissue lymphoma with Sjögren's syndrome].

A 68-year-old woman presented with Sjögren's syndrome. Chest X-ray films disclosed consolidated shadows in the right S2 and an infiltration shadow in the right S8 with small nodules. Pathological examination of transbronchial lung biopsy (TBLB) specimens revealed lymphocytic infiltrations that stained positive with UCHL-1 and L 26 in immunohistochemical studies. Lung tissue specimens obtained by video-assisted thoracic surgery showed lympho-epithelial lesions with dense lymphocytic infiltration. Southern blot hybridization and polymerase chain reaction (PCR) assays demonstrated monoclonality and immunoglobulin heavy chain gene rearrangement. These findings yielded a diagnosis of mucosa-associated lymphoid tissue (MALT) lymphoma. The detection of rear-ranged genes encoding for immunoglobulin heavy chains is useful for the diagnosis of primary pulmonary lymphoproliferative disorders, especially malignant lymphomas.

Aged↗

PubMed1999

[Multiple intrapulmonary rheumatoid nodules].

A 66-year-old woman who had been treated at a nearby hospital since 1977 for rheumatoid arthritis complained of cough. Chest X-ray films disclosed multiple nodular shadows with cavitation in the fields of both lungs. The patient was admitted to our hospital and a thoracoscopic lung biopsy was performed. Histologically, the nodule consisted of necrotizing granuloma, indicating a necrobiotic nodule. Rheumatoid nodule was diagnosed because the patient exhibited rheumatoid arthritis. The chest X-ray shadow disappeared without medication. Rheumatoid nodules without coniosis are uncommon, but should be considered in the differential diagnosis of lung nodular lesions in patients with rheumatoid arthritis.

Aged↗

PubMed1998

Multiple pulmonary metastases with cavitation from gallbladder cancer.

We report a rare case of multiple pulmonary metastases with cavitation from gallbladder cancer. A 77-year-old woman was admitted to our hospital complaining of productive cough and exertional dyspnea. Chest X-ray film showed multiple nodular shadows with some cavitation. Computed tomography showed multiple cavities, up to 2 cm in diameter, as well as nodules, in bilateral lung fields. Under a survey of primary focus, the ultrasonographic test of the abdomen revealed a hypoechoic mass in the hepatic hilum. The patient died of respiratory failure. Autopsy findings revealed that that multiple lung tumors had metastasized from papillary adenocarcinoma of the gallbladder and that cavitation of the lung was formed by bronchioloectasis.

Adenocarcinoma, Papillary↗

PubMed1998

[A case of lung abscess caused by Eikenella corrodens].

We report a case of lung abscess caused by Eikenella corrodens and reviewed the literature. A 51-year-old man was hospitalized because of an infiltrative shadow in the left upper lung on chest X-ray. The patient was intravenously given CEZ (2 g/day) followed by CEZ (4 g/day) and AMK (200 mg/day). We nevertheless deteriorated and was subsequently treated with CTM (4 g/day) and LCM (6 g/day), and the shadow improved but remained. Only Eikenella corrodens was isolated from aspiration materials obtained from the resected lung. We examined the susceptibility of 60 strains of Eikenella corrodens to various antimicrobial against. The organisms were very susceptible to PCG, ABPC, PIPC, CTM, CTX and LMOX, but were all resistant to LCM, CLDM, GM and AMK.

Aminoglycosides↗