Exacerbation of cryoglobulinemia associated with intravenous radiographic contrast medium.
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Biomedical subjects
Publications and source records attributed to Y Kikuchi.
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A 15-year-old male was admitted to our hospital for treatment of an anterior mediastinal tumor. The tumor was visualized by chest radiography 3 months prior to admission. Computed tomography (CT) revealed a heterogeneous solid tumor located in the anterior mediastinum. Although CT-guided needle biopsy had been performed twice, histologic diagnosis could not be confirmed. We believed this tumor to be nonseminomatous mediastinal germ cell tumor (NSGCT) and started intensive chemotherapy with cisplatin (CDDP) without histologic diagnosis because his serum AFP level was rapidly increasing. After 2 courses of chemotherapy, his serum AFP level returned to the normal range and surgical resection of the tumor with part of right lung was performed. Histopathological examination revealed that the tumor consisted of mature teratoma and yolk sac tumor. He underwent 1 course of chemotherapy post-operatively because a small number of viable cells were histopathologically recognized in the yolk sac component. At the time of writing, the patient is alive without any evidence of recurrence.
A 56-year-old male was admitted to our hospital to treat for an abnormal lung shadow. Computed tomography (CT) revealed the shadow with ground-glass opacity that was 30 mm in diameter at left S6 lesion. Although transbronchial lung biopsy had been performed, histological diagnosis could not be made. We had expected the tumor could not be resected completely with a partial lung resection. Additionally, he refused being done a lower lobectomy without a histological diagnosis. So we performed a S6+S* segmentectomy with No. 7-12 lymph node dissection. Although the intraoperative frozen section diagnosis was an atypical adenomatous hyperplasia, the tumor was finally diagnosed as bronchioloalveolar carcinoma (BAC) because of its nuclear atypia. We did not resect the residual part of left lower lobe because he refused the additional operation and might have histopathologically no residual tumor and lymph node metastasis. At present, he is alive without any evidence of recurrence.
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the activation of autoreactive B lymphocytes, which are supposed to carry aberrant signal transduction after the stimulation of B-cell receptor (BCR). To investigate abnormalities in BCR-mediated signaling pathway in lupus B lymphocytes, we analyzed HS1, a molecule downstream of BCR, in 80 Japanese SLE patients. We identified 37 amino acid deletion of HS1 in a 25-year-old female patient, and the aberrant HS1 lacked a part of a functional motif. Analysis of genomic DNA revealed that the aberrant HS1 was caused by exon skipping. Family study showed that the patient as well as her father and sister are heterozygous for the abnormality. WEHI-231 cell, a mouse B cell line, transfected with the aberrant HS1 displayed a significantly increased cell death upon cross-linking of BCR. Additionally, peripheral B lymphocytes from the patient exerted increased apoptosis after BCR stimulation compared to those from control SLE patients. These data suggest that the aberrant HS1 molecule may transmit an accelerated signal after BCR stimulation and may play a role in the activation of autoreactive B lymphocytes.
A 58-year-old man, who had undergone emergency graft replacement of ascending and total aortic arch using gelatin-resorcin-formalin (GRF) glue 1 year before, complained of progressive shortness of breath. Ultrasound cardiography revealed severe aortic regurgitation and dissecting aneurysm of aortic root. He underwent composite graft replacement with the aid of the right axillary artery perfusion and deep hypothermic circulatory arrest. The redissected brownish intima was identified in the area of noncoronary cusp. There was no special finding in the subsequent histopathological examination. The use of GRF glue for reconstructing the dissected aortic root is associated with a certain amount of risk of aortic wall redissection. Therefore, care should be taken to ensure proper use of GRF glue.
A 71-year-old man was admitted to our hospital for treatment of an endobronchial tumor. Although he was asymptomatic and chest radiography showed no tumor shadow, bronchoscopic findings demonstrated an endobronchial tumor with a villous surface occluding the bronchus of the left upper division. Endobronchial resection was abandoned because we could not directly observe the cervix with a bronchoscope. In addition, because we could not diagnose the tumor histologically as benign by biopsy specimens, we elected to perform a segmentectomy of the left upper division. To reduce surgical stress, the operation was carried out using thoracoscopy. The tumor was finally diagnosed as endobronchial chondromatous hamartoma. The patient's postoperative course was uneventful. When endobronchial resection is not feasible, a less invasive surgical approach and method should be taken in patients with suspected endobronchial hamartoma.
Environmental streptococci are frequently isolated from bovine mastitis in dairy cows with only limited information available on the antimicrobial susceptibility of these organisms. A total of 362 environmental streptococci isolated from cases of bovine mastitis from the central San Joaquin Valley of California over a 3-yr period were used in the study. Overall, 39.9% of the strains tested were Streptococcus uberis, 42.2% were Streptococcus dysgalactiae, and 11.1% were Enterococcus spp. The antimicrobial susceptibility for these organisms was determined for the following antimicrobial agents: penicillin, ampicillin, cephalothin, ceftiofur, penicillin + novobiocin, erythromycin, pirlimycin, tetracycline, and sulfadimethoxine. Results demonstrate substantial differences in the susceptibility patterns for the various organisms collectively referred to as the environmental streptococci. The MIC90 for penicillin was 0.06 microg/ml for 152 strains of S. dysgalactiae compared with 0.25 microg/ml for 133 strains of S. uberis. However, the Enterococcus spp. were the most resistant organisms tested. These data also indicate that the use of interpretive criteria based on human data may provide misleading results. In conclusion, these data confirm that the environmental streptococci are a diverse group of organisms comprised of several different genera and species and that identification of environmental streptococci to the species level is needed to appropriately modify control methods. Moreover, the use of the agar disk diffusion (Kirby-Bauer) susceptibility test for agents with human-based interpretive criteria is contraindicated, and these tests should only be performed with agents with mastitis specific interpretive criteria.
A 67-year-old female was admitted to our hospital with eruption and cervical lymphadenopathy which occurred one week after pneumococcal vaccination. Polyclonal hyperimmunoglobulinemia (IgG 6,620 mg/dl) and mild plasma cell proliferation (6.4%) in a bone marrow specimen were found, but a lymph node aspiration biopsy showed no specific findings. Normochromic and normocytic anemia with a positive direct Coombs test were also confirmed. Short-term intensive steroid therapy was given, but the systemic eruption and lymphadenopathy continued. About 4 months after vaccination, she suffered from edema in her face and legs and visual disturbance. When massive proteinuria (10.4 g/day) was found, she was admitted to our ward. A renal biopsy specimen showed a minor glomerular abnormality with mild interstitial plasmacytic infiltration. An abdominal CT scan showed hepatosplenomegaly and para-aortic lymphadenopathy. Uveitis was also found by ophthalmoscopy. These abnormalities completely disappeared after intensive steroid therapy including pulse therapy. On the basis of her clinical course and laboratory findings, it was suggested that minimal change nephrotic syndrome might be induced after vaccination, possibly due to hypersensitivity syndrome.
Between October, 1991, and October, 2001, 60 patients underwent aortic arch replacement with or without an aortic arch branched graft for atherosclerotic arch aneurysms. Their mean age was 70.1 +/- 8.6 years. Eight (13.3%) patients were operated on an emergency basis because of rupture or impending rupture of aneurysms. All operations were performed with hypothermic extracorporeal circulation. Selective cerebral perfusion for cerebral protection during aortic arch repair and systemic circulatory arrest during distal graft anastomosis was used in 56 patients. Mean selective cerebral perfusion time was 86.1 +/- 12.1 minutes. A total of 14 concomitant procedures were done. Overall in-hospital mortality was 3.3%. Postoperative temporary and permanent neurologic dysfunction were 1.6% and 1.6%. Long-term follow-up was 100% complete. There were 6 late deaths with the cumulative survival rate was 74.6 +/- 8.8%. A subsequent aortic operation was necessary for the treatment of an aortic abnormality distal to the arch in 6 patients. Reoperation free rate was 85.2 +/- 5.8%. In conclusion, cerebral protective effect of antegrade selective cerebral perfusion and total arch replacement with an aortic arch branched graft could substantially reduce in-hospital mortality and postoperative neurologic dysfunction and a satisfactory long-term results could be obtain in patients with atherosclerotic arch aneurysms.
A 71-year-old man was admitted to our hospital with a small protrusive lesion at the lingular orifice of the left upper bronchus. He had undergone a right lower lobectomy and mediastinal dissection for lung carcinoma (large cell carcinoma, pT1N0M0) 14 months earlier. Early hilar squamous cell carcinoma was diagnosed by chest radiograph, CT and transbronchial biopsy. We performed a lingular segmentectomy with wedge resection of the left upper bronchus and N 1 lymph node dissection. The tumor was histopathologically diagnosed as early hilar second primary lung carcinoma. The patient's postoperative course was uncomplicated. At present, he is alive with good respiratory condition and without any evidence of recurrence. Segmentectomy is appropriate for a patient with contralateral second primary lung carcinoma as well as a patient with early hilar lung carcinoma. Bronchoplasty seems to increase the likelihood that such a patient will be a candidate for segmentectomy.
The natural history of patients with Marfan syndrome is depressing, however, cardiovascular surgery can improve the prognosis. We reviewed the results of this surgical procedure for 10 years. Fourteen Marfan patients underwent cardiovascular surgery for a total of 21 times. Hospital mortality was 4.8% (1 case died on the 105th day after entire thoraco-abdominal aortic replacement because of pyothorax and sepsis), and the 5- and 10-year Kaplan-Meier survival was 92.9% and 77.4% respectively. Reoperation free rate was 51.4% and 38.6% respectively. Cardiovascular surgery in Marfan syndrome can be performed with good result. Aggressive surgical management and close follow-up of patients who undergo surgery is important.
It has been demonstrated that the cytomegalovirus (CMV)-specific CD4(+) T cell response could be restored after ganciclovir and highly active antiretroviral therapy (HAART) in AIDS patients. In this study, we first confirmed the above observation cross-sectionally. We then performed a prospective longitudinal study over a period of 48 weeks. The second study included nine patients. All patients had received HAART. Five patients had a history of retinitis that was, however, under control after discontinuation of anti-CMV therapy more than 1 year before this study (group A). The other four had active CMV retinitis at the start of this study and anti-CMV therapy was required to control retinitis (group B). Median periods between commencement of HAART and the start of this study in group A and in group B were 27 and 4.5 months, respectively. Within both groups, the number of CD4(+) T cells that produced tumor necrosis factor alpha in response to CMV antigen did not vary throughout the observation period (Friedman test; p > 0.05). However, the median number of responsive CD4(+) T cells in group A patients was significantly higher than in group B (p < 0.05). Our results demonstrate that the number of CMV-responsive CD4(+) T cells increased when HIV was well controlled with HAART and was then maintained, and suggest that these cells may play an important role in the control of retinitis in patients with AIDS.
SUMO1/Smt3, a ubiquitin-like protein modifier, is known to conjugate to other proteins and modulate their functions in various important processes. Similar to the ubiquitin conjugation system, SUMO/Smt3 is transferred to substrate lysine residues through the thioester cascade of E1 (activating enzyme) and E2 (conjugating enzyme). In our previous report (Takahashi, Y., Toh-e, A., and Kikuchi, Y. (2001) Gene 275, 223-231), we showed that Siz1/Ull1 (YDR409w) of budding yeast, a member of the human PIAS family containing a RING-like domain, is a strong candidate for SUMO1/Smt3 ligase because the SUMO1/Smt3 modification of septin components was abolished in the ull1 mutant and Ull1 associated with E2 (Ubc9) and the substrates (septin components) in immunoprecipitation experiments. Here we have developed an in vitro Smt3 conjugation system for a septin component (Cdc3) using purified recombinant proteins. In this system, Ull1 is additionally required as well as E1 (Sua1.Uba2 complex), E2 (Ubc9), and ATP. A cysteine residue of the RING-like domain was essential for the conjugation both in vivo and in vitro. Furthermore, a region containing the RING-like domain directly interacted with Ubc9 and Cdc3. Thus, this SUMO/Smt3 ligase functions as an adaptor between E2 and the target proteins.
SUMO1/Smt3, a ubiquitin-like protein modifier, is known to be conjugated to other proteins and modulate their functions in various important processes. Similar to the ubiquitin system, SUMO1/Smt3 is activated in an ATP-dependent reaction by thioester bond formation with E1 (activating enzyme), transferred to E2 (conjugating enzyme), and passed to a substrate lysine. It remained unknown, however, whether any SUMO1/Smt3 ligases (E3s) are involved in the final transfer of this modifier. Here we report a novel factor Siz1 (YDR409w) required for septin-sumoylation of budding yeast, possibly acting as E3. Siz1 is a member of a new family (Miz1, PIAS3, etc.) containing a conserved domain with a similarity to a zinc-binding RING-domain, often found in ubiquitin ligases. In the siz1 mutant septin-sumoylation was completely abolished. A conserved cysteine residue in the domain was essential for this conjugation. Furthermore, Siz1 was localized at the mother-bud neck in the M-phase and physically bound to both E2 and the target proteins.
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Early endoderm formation in zebrafish requires at least three loci that function downstream of Nodal signaling but upstream of the early endodermal marker sox17: bonnie and clyde (bon), faust (fau), and casanova (cas). cas mutants show the most severe phenotype as they do not form any gut tissue and lack all sox17 expression. Activation of the Nodal signaling pathway or overexpression of Bon or Fau/Gata5 fails to restore any sox17 expression in cas mutants, demonstrating that cas plays a central role in endoderm formation. Here we show that cas encodes a novel member of the Sox family of transcription factors. Initial cas expression appears in the dorsal yolk syncytial layer (YSL) in the early blastula, and is independent of Nodal signaling. In contrast, endodermal expression of cas, which begins in the late blastula, is regulated by Nodal signaling. Cas is a potent inducer of sox17 expression in wild-type embryos as well as in bon and fau/gata5 mutants. Cas is also a potent inducer of sox17 expression in MZoep mutants, which cannot respond to Nodal signaling. In addition, ectopic expression of cas in presumptive mesodermal cells leads to their transfating into endoderm. Altogether, these data indicate that Cas is the principal transcriptional effector of Nodal signaling during zebrafish endoderm formation.
Cancer is an inheritable disorder of somatic cells. Environment and heredity both operate in the origins of human cancer. These environmental and genetic determinants of cancer can be classified into four groups designated "Oncodemes" [1]. Oncodeme 1 is the irreducible "background" level of cancer due to spontaneous mutagenesis. Oncodeme 2 is "environmentally induced" cancer, whose causative agents are chemical carcinogens, radiation and viruses. Oncodeme 3 is basically "environmentally induced" cancer, but there are genetically determined differences among persons, e.g. the activation or inactivation of carcinogenes. Most human cancers are believed to belong to Oncodemes 2 and/or 3 (about 80%), for which the probability of the occurrence of the initial carcinogenic step(s) is increased, although the number of steps is not decreased. Oncodeme 1 would contain the approximately 20% that would remain if "environmentally induced" cancers (Oncodeme 2 and/or 3) were prevented. Lastly, Oncodeme 4 is "hereditary" cancer. Hereditary cancers could prove valuable in elucidating carcinogenesis, even though only a small proportion of cancers belong to this group. Here, we present a unique animal model of Oncodeme 4 for the study of problems in carcinogenesis; e.g. cell stage and tissue/cell-type-specific tumorigenesis, multistep carcinogenesis, species-specific differences in tumorigenesis, modifier gene(s) in renal carcinogenesis and cancer prevention.