Bilevel positive airway pressure therapy in a patient with myotonic dystrophy and postoperative respiratory failure: A case report.

Respiratory failure is a common complication in patients with myotonic dystrophy (MD) and might be a presenting symptom in the perioperative setting. We report the case of a 59-year-old woman with MD who underwent open cholecystectomy and developed postoperative respiratory failure. Without reintubation, the patient was successfully managed with bilevel positive airway pressure (BiPAP) and was discharged uneventfully. BiPAP may be considered as an alternative for postoperative respiratory failure in patients with MD. Careful observation of patients' postoperative condition and an earlier application of BiPAP are instrumental in avoiding retracheal intubation, which may cause further serious problems in patients with MD.

Effect of endotracheal tube lubrication on cuff pressure increase during nitrous oxide exposure: a laboratory and prospective randomized controlled trial.

BACKGROUND: We previously demonstrated that lubrication of an endotracheal tube (ETT) cuff with K-Y™ jelly strongly and significantly inhibited the increase in cuff pressure during nitrous oxide (N2O) exposure in vitro. However, in our previous study, we identified critical differences between some influential factors, such as the amount of lubricant retained on the cuff, and studied temperature differences between laboratory and clinical conditions. Therefore, it remained unclear whether this effect holds true in clinical settings. METHODS: We first sought to study how changes in the amount of K-Y™ jelly and temperature influence the inhibitory effects of the lubricant on the increase in N2O-induced cuff pressure in vitro. Furthermore, we aimed to determine whether the application of K-Y™ jelly inhibits the increase in ETT cuff pressure during general anesthesia using N2O in adult patients. RESULTS: In the laboratory studies, we found that K-Y™ jelly inhibited the cuff pressure increase dose-dependently when the dose of K-Y™ jelly was varied (P = 0.02), and that such an inhibitory effect decreased with an increase in the studied temperature (P = 0.019). In the clinical study, lubrication with K-Y™ jelly slightly, but significantly, delayed the increase in ETT cuff pressure during general anesthesia with N2O (P = 0.029). However, the inhibitory effect in the clinical settings was smaller than that in vitro. CONCLUSIONS: Lubrication of the ETT cuff with K-Y™ jelly may delay the increase in cuff pressure during general anaesthesia with N2O. However, the clinical significance of this effect may be limited. TRIAL REGISTRATION: UMIN Clinical Trials Registry: UMIN000031377 on March 1, 2019.

Supraglottic airway device versus a channeled or non-channeled blade-type videolaryngoscope for accidental extubation in the prone position: A randomized crossover manikin study.

BACKGROUND: It is very rare but challenging to perform emergency airway management for accidental extubation in a patient whose head and neck are fixed in the prone position when urgently turning the patient to the supine position would be unsafe. The authors hypothesized that tracheal intubation with a videolaryngoscope would allow effective airway rescue in this situation compared with a supraglottic airway device and designed a randomized crossover manikin study to test this hypothesis. METHODS: The authors compared airway rescue performances of the 3 devices-the ProSeal laryngeal mask airway (PLMA; Teleflex Medical, Westmeath, Ireland) as a reference; the Pentax AWS (AWS; Nihon Kohden, Tokyo, Japan) as a channeled blade-type videolaryngoscope; and the McGRATH videolaryngoscope (McGRATH; Medtronic, Minneapolis, MN) as a nonchanneled blade type in a manikin fixed to the operating table in the prone position. Twenty-one anesthesiologists performed airway management on the prone manikin with the 3 devices, and the time required for intubation/ventilation and the success rates were recorded. RESULTS: The median (range) intubation/ventilation times with the PLMA, AWS, and McGRATH were 24.5 (13.5-89.5) s, 29.9 (17.1-79.8) s, and 46.7 (21.9-211.7) s, respectively. There was no significant difference in intubation/ventilation times between the PLMA and AWS. The AWS permitted significantly faster tracheal intubation than did the McGRATH (P = 0.006). The success rates with the PLMA (100%) and AWS (100%) were significantly greater than that with the McGRATH (71.4%). Airway management performance of the PLMA and AWS was comparable between devices and better than that of the McGRATH in the prone position. CONCLUSIONS: Considering that tracheal intubation can provide a more secure airway and more stable ventilation than the PLMA, re-intubation with a channeled blade-type videolaryngoscope such as the AWS may be a useful method of airway rescue for accidental extubation in patients in the prone position.

Action observation facilitates motor cortical activity in patients with stroke and hemiplegia.

Motor imagery (MI) is a mental practice that reproduces the visual- and/or kinesthetic-modality brain activations accompanying movement. It is a useful rehabilitation technique as the affected motor cortex can be stimulated in patients with stroke and hemiplegia. However, most patients with stroke have difficulty with MI owing to advanced age and/or higher-cognitive dysfunction, thus impairing their ability to internally simulate the action. We therefore investigated whether action observation (AO), an alternative form of motor stimulation that works via the mirror-neuron system, could facilitate motor cortical activity in such patients. Combined AO and physical training of the observed actions has been reported to have a positive impact on motor deficits after stroke. Eleven patients with stroke and hemiplegia affecting the hand performed MI and AO with verbal and video instructions under 19 channels of electroencephalogram (EEG) recording. The event-related desynchronization (ERD) was measured as an electroencephalographic marker of motor cortical activity. The ERD power in the AO condition (30.0±5.0%) was significantly higher than that in the MI condition (12.2±3.9%). These results suggest that AO could be a good option for patients with stroke who have difficulty using MI to effectively stimulate and reestablish cortical-peripheral motor pathways.

Syndrome of inappropriate secretion of antidiuretic hormone induced by tacrolimus in a patient with systemic lupus erythematosus.

We describe the first reported case of a syndrome of inappropriate secretion of antidiuretic hormone (SIADH) induced by low-dose tacrolimus in a patient with autoimmune disease. A 41-year-old man with systemic lupus erythematosus (SLE) developed hyponatremia induced by SIADH after administration of tacrolimus (0.06 mg/kg per day). In this case, the hyponatremia promptly resolved upon withdrawal of tacrolimus. This case strongly suggests that SIADH is a potentially important complication of tacrolimus administration, irrespective of dosage, and should be borne in mind whenever the drug is used.

Derivative (1;18)(q10;q10) in essential thrombocythemia.

This study reports the association of the chromosomal abnormality derivative (1;18)(q10;q10) with essential thrombocythemia (ET) occurring in a 75-year-old woman. Allele-specific polymerase chain reaction also revealed a V617F mutation in the Janus Kinase 2 gene (JAK2) in the platelet compartment in this patient. The der(1;18)(q10;q10) abnormality has previously been reported in two cases of myeloid disorders. The etiological implications for ET of this combination of chromosomal abnormality and JAK2 mutation still remain elusive. This is a novel report of derivative (1;18)(q10;q10) abnormality in ET.

IK cytokine ameliorates the progression of lupus nephritis in MRL/lpr mice.

OBJECTIVE: IK cytokine has been isolated as a factor that inhibits interferon-gamma (IFNgamma)-induced expression of class II major histocompatibility complex (MHC) antigens. Aberrant expression of class II MHC antigens has reportedly been recognized in the target organs of autoimmune diseases and been associated with disease activity. In this study, we investigated whether IK cytokine can ameliorate the progression of lupus nephritis in MRL/lpr mice. METHODS: A truncated IK analog was prepared and transfected into a nonmetastatic fibroblastoid cell line, and then injected subcutaneously into MRL/lpr mice at ages 8 weeks (before the onset of lupus nephritis) and 12 weeks (at the early stage of the disease). RESULTS: An IK cytokine, when it was translated from methionine at position 316, acted as a secretory protein. This truncated IK cytokine (tIK) reduced IFNgamma-induced class II MHC expression in various cells through decreased expression of class II MHC transcription activator. Treatment of MRL/lpr mice with tIK significantly reduced renal damage as compared with control mice. A significant decrease in macrophage and T cell infiltration was found in the kidneys of tIK-treated mice, resulting in decreased production of IFNgamma and interleukin-2. Mice treated with tIK also showed significant reduction of anti-DNA antibodies and circulating immune complexes. A specific reduction of class II MHC expression was observed on B cells and monocytes as well as in the kidney. CONCLUSION: We prepared a potent IK analog and demonstrated its ability to ameliorate the progression of lupus nephritis. This agent may therefore provide a new therapeutic approach for lupus nephritis.

[Esophageal stricture following complete remission after chemotherapy for malignant lymphoma in an elderly patient].

A 75-year-old woman was given a diagnosis of malignant lymphoma (non-Hodgkin, diffuse large B cell type, stage IIA) at our hospital on August 2003. She received six courses of rituximab-based chemotherapy (R-CHOP regimen) and then she achieved complete remission. On August 16, 2004, she was readmitted in our hospital for difficulty in swallowing. Upper gastrointestinal endoscopy reveled esophageal stricture and an ulcerative lesion on the esophageal mucosa. The X-ray examination of the upper gastrointestinal tract reveled a severe esophageal stricture with niches and hiatus hernia. No malignancy was seen on CT scanning, gallium radioisotope scanning and histological examination of biopsy specimens with the upper gastrointestinal endoscopy. The physical examination showed gibbosity, and MR imaging showed multiple compression spined fractures. Finally, we diagnosed benign esophageal stricture with reflux esophagitis. She underwent laparoscopic partial esophagectomy in September 21, 2004, and the postoperative course was satisfactory. The pathological findings showed benign esophageal stricture caused by esophagitis. We report here a case of esophageal stricture following complete remission after chemotherapy for malignant lymphoma in an elderly patient.

Identification of N-arachidonylglycine as the endogenous ligand for orphan G-protein-coupled receptor GPR18.

An orphan G-protein-coupled receptor, GPR18, was cloned on the basis of degenerate-oligonucleotide PCR analysis of HUT 102 cells using primers designed from the conservative regions of the human chemokine receptor. GPR18 was expressed significantly in lymphoid cell lines, but not in non-lymphoid hematopoietic cell lines. Moreover, the expression of the GPR18 gene was higher in peripheral lymphocyte subsets (CD4(+), CD4(+)CD45RA(+), CD4(+)CD45RO(+), CD8(+), and CD19(+)) than in monocytes and lymphoid cell lines, and was increased after stimulation with phytohemagglutinin. By screening using a lipid library, N-arachidonylglycine (NAGly) induced an increase in intracellular Ca(2+) concentration in GPR18-transfected cells, which was significantly greater than that in mock-transfected cells. NAGly also inhibited forskolin-induced cAMP production in a pertussis toxin-sensitive manner in the GPR18-transfected CHO cells. This is the first study to demonstrate that NAGly is a natural ligand for GPR18.

Antagonist of interferon-inducible protein 10/CXCL10 ameliorates the progression of autoimmune sialadenitis in MRL/lpr mice.

OBJECTIVE: Mononuclear cell infiltration of the salivary glands is a major feature of Sjögren's syndrome (SS) and its animal model. Local generation of chemokines and the presence of chemokine receptors on the infiltrating cells may be involved in this process. We undertook the present study to investigate the expression of chemokines during the development of autoimmune sialadenitis in MRL/lpr mice and the therapeutic effect of chemokine antagonists on sialadenitis. METHODS: NH2-terminal-truncated interferon-inducible protein 10 (IP-10)/CXCL10 analogs were transfected into a nonmetastatic fibroblastoid cell line, MRL/N-1, and injected subcutaneously into MRL/lpr mice, and the effects on sialadenitis were monitored. RESULTS: IP-10 analogs truncated by 5 or more amino acid residues from the N-terminal failed to induce chemotaxis and calcium influx by CXCR3-expressing cells. Of these, the most potent antagonist (AT) (IP-10-AT) was a molecule with methionine added after removal of the 5 N-terminal amino acid residues. Significantly increased expression of the Th1-associated chemokines IP-10, monokine induced by interferon-gamma/CXCL9, and interferon-inducible T cell chemoattractant/CXCL11 was induced in the ductal epithelium by interferon-gamma produced in the salivary glands, whereas expression of the Th2-associated chemokines thymus and activation-regulated chemokine (TARC)/CCL17 and monocyte-derived chemokine/CCL22 was almost undetectable during sialadenitis. Inoculation of IP-10-AT into MRL/lpr mice during the early stage of sialadenitis significantly reduced periductal mononuclear cell infiltration and parenchymal destruction compared with these features in control and TARC-AT-bearing mice. This was due to a significant reduction in infiltration of CXCR3+ T cells, predominantly Th1 cells, resulting in decreased interferon-gamma production. CONCLUSION: We prepared a novel potent IP-10 antagonist and demonstrated its ability to ameliorate the progression of autoimmune sialadenitis. This agent may provide a new therapeutic approach to SS.

Antagonist of fractalkine (CX3CL1) delays the initiation and ameliorates the progression of lupus nephritis in MRL/lpr mice.

OBJECTIVE: Lupus nephritis is characterized by immune complex deposition and inflammatory cell infiltration into the renal glomeruli. Local generation of chemokines and the presence of chemokine receptors on the infiltrating cells may be involved in this process. Fractalkine (Fkn)/CX3CL1 and its receptor, CX3CR1, form one such chemokine system. We therefore undertook this study to investigate whether Fkn antagonist inhibits the initiation and progression of lupus nephritis in MRL/lpr mice. METHODS: NH(2)-terminally truncated Fkn/CX3CL1 analogs were transfected into a nonmetastatic fibroblastoid cell line, MRL/N-1, and injected subcutaneously into MRL/lpr mice. RESULTS: Fkn analogs truncated by >/=4 amino acid residues from the N-terminus failed to induce chemotaxis and calcium influx by CX3CR1-expressing cells. Of these, the most potent antagonist (Fkn-AT) lacked the 4 N-terminal amino acid residues. Fkn expression in the glomerulus was significantly increased in 12-week-old MRL/lpr mice. Expression was localized predominantly in the glomerular endothelial cells, but was occasionally observed in the mesangial cells and, to a lesser extent, in the interstitial microvasculature. Inoculation of MRL/lpr mice with Fkn-AT before the onset or during the early stages of lupus nephritis significantly reduced glomerular hypercellularity, glomerulosclerosis, crescent formation, and vasculitis compared with control mice. This seemed to be due to a marked reduction in macrophage accumulation. In contrast, Fkn antagonist did not affect pneumonitis, sialadenitis, lymphadenopathy, or splenomegaly. CONCLUSION: We prepared a novel potent Fkn antagonist and demonstrated its ability to delay the initiation and ameliorate the progression of lupus nephritis. This agent may therefore provide a new therapeutic approach to lupus nephritis.

Existence of leukemic clones resistant to both imatinib mesylate and rituximab before drug therapies in a patient with Philadelphia chromosome-positive acute lymphocytic leukemia.

Imatinib mesylate and rituximab are molecularly targeted drugs against the BCR-ABL fusion protein and the CD20 antigen, respectively. Although these drugs have excellent anticancer effects, a major concern is drug resistance. We have investigated the case of a patient with Philadelphia chromosome-positive and CD20+ acute lymphocytic leukemia who acquired resistance to imatinib and rituximab. Imatinib therapy resulted in prompt cytogenetic remission, but resistance developed shortly thereafter. Sequencing of the kinase domain of the ABL gene and allele-specific polymerase chain reaction analysis revealed a point mutation resulting in an E255V substitution that was present before the therapy. After the patient received mild chemotherapy followed by rituximab administration, hematologic and cytogenetic remission was sustained for 5.5 months. The recurrent leukemic cells after the rituximab therapy showed not only the E255V mutation in the ABL gene but also loss of the CD20 antigen due to impaired transcription of the CD20 gene. The results of 2-color flow cytometry analysis showed that a small population of CD20(-) leukemic cells existed before the imatinib therapy. These results suggest that leukemic subclones carrying a genetic perturbation of the targeted molecules for both imatinib and rituximab were present before the therapies. The preexistence of primary resistant clones suggests the inability of combination therapy with 2 molecularly targeted drugs to overcome drug resistance in leukemia.

Munchausen syndrome with foreign-body granuloma mimicking rheumatic diseases.

A 37-year-old man was admitted to our hospital because of fever, polyarthralgia, and subcutaneous tumors. There was swelling of the bilateral wrists and ankles, and subcutaneous tumors over the bilateral elbow joints. Despite his complaints of multiple symptoms, clinical investigations failed to reveal any abnormality. Although laboratory parameters improved rapidly after steroid therapy, the symptoms remained unchanged, and there was an enormous discrepancy between the laboratory data and his symptoms. A biopsy specimen from one of the subcutaneous tumors revealed foreign-body granuloma associated with a foreign body fragment. Because the nursing staff later discovered that the patient had been carrying out self-injection, a diagnosis of Munchausen syndrome was made. Munchausen syndrome should be included in the differential diagnosis of rheumatic diseases.

Clinical usefulness of multiplanar reconstruction images obtained by multi-slice computed tomographic angiography for early-stage Takayasu's arteritis.

We describe a case of Takayasu's arteritis discovered during the early stage (pre-pulseless stage). Cervical ultrasonography and multiplanar reconstruction (MPR) images obtained by computed tomography (CT) showed thickening and stenosis of the walls of the common carotid arteries. However, magnetic resonance arteriography (MRA), volume rendering (VR) imaging CT, and maximum intensity projection (MIP) imaging CT could detect no obvious abnormality. We emphasize the importance of noninvasive vascular investigation with cervical ultrasonography and MPR imaging CT to support the diagnosis of early-stage Takayasu's arteritis.

Antagonist of monocyte chemoattractant protein 1 ameliorates the initiation and progression of lupus nephritis and renal vasculitis in MRL/lpr mice.

OBJECTIVE: To examine whether chemokine antagonists inhibit the initiation and progression of lupus nephritis in MRL/lpr mice. METHODS: NH(2)-terminal-truncated monocyte chemoattractant protein 1 (MCP-1)/CCL2 or thymus and activation-regulated chemokine (TARC)/CCL17 analogs were inserted into the pCXN2 expression vector and transfected into a nonmetastatic fibroblastoid cell line, MRL/N-1, established from an MRL/gld mouse. RESULTS: MCP-1 antagonist- or TARC antagonist-transfected MRL/N-1 cells were injected subcutaneously into MRL/lpr mice ages 7 weeks (before the onset of lupus nephritis) and 12 weeks (at the early stage of the disease). After 8 weeks, mice bearing the MCP-1 antagonist showed markedly diminished infiltration of macrophages and T cells, glomerular hypercellularity, glomerulosclerosis, crescent formation, and vasculitis compared with control mice. This seemed to be due to decreased production of interferon-gamma and interleukin-2 in the kidney. In contrast, there was no significant difference in renal damage between mice bearing TARC antagonist and control mice. CONCLUSION: We established a new system using MRL/N-1 cells that allows long-term observation of the effects of chemokine antagonists on lupus nephritis in MRL/lpr mice. We also showed that the MCP-1 antagonist ameliorated the initiation and progression of lupus nephritis and of renal vasculitis, which might provide a new approach to the treatment of the disease.

[A case of pyomyositis following influenza virus infection].

A 45-year-old man visited the first hospital complained of high fever on January 2003. He was diagnosed as having Influenza virus type A infection and prescribed of Oseltamivir. He was afebrile next day, but severe myalgia of neck, shoulder, lumbar region and right femoral region was appeared. His illness was considered as polymyalgia rheumatica and started of oral steroid therapy. His symptom was deteriorated and transferred to our hospital. Echography, Ga scintigraphy, computed tomography and magnetic resonance imaging revealed the multiple abscesses and the diagnosis of pyomyositis was made. Pyomyositis following Influenza virus infection must be considered as a differential diagnosis of myalgia after Influenza virus infection.

Antagonist of secondary lymphoid-tissue chemokine (CCR ligand 21) prevents the development of chronic graft-versus-host disease in mice.

The use of receptor antagonists for chemokines is an alternative approach to blocking chemokine actions and has the potential to provide novel therapeutics. We determined the receptor antagonist properties of murine N-terminally truncated secondary lymphoid tissue chemokine (SLC)/6Ckine/CCR ligand 21 analogs and evaluated the preventive effects of SLC antagonists on chronic graft-vs-host disease (GVHD) in a murine model by blocking the homing of donor CCR7-expressing T cells into the recipient's lymphoid organs. SLC analogs truncated >4 aa residues from the N terminus showed a loss of chemotaxis and Ca2+ influx of CCR7-expressing cells and also inhibited SLC-stimulated chemotaxis and SLC-induced Ca2+ influx completely. To determine whether SLC antagonist inhibits the development of chronic GVHD, chronic GVHD was induced by injecting DBA/2 spleen cells into (C57BL/6 x DBA/2) F1 mice. Total numbers of spleen cells and host B cells, serum levels of IgE, and of total IgG and IgG1 of anti-DNA Abs in SLC antagonist-treated GVHD mice were significantly lower than those in control PBS-treated GVHD mice. This was due to a reduction in the levels of activated donor CD4+ T cells and a decrease in IL-4 production, resulting in a reduction in the numbers of activated host B cells. Therefore, our results suggest that SLC antagonist has beneficial effects for the prevention of chronic GVHD.

[Endocrine cell carcinoma (atypical carcinoid) of the ileocecal region adjacent to an adenocarcinoma].

An 86-year-old woman was admitted because of right lower abdominal pain. A 7 by 6 cm tumor palpable in the right lower quadrant was poorly mobile. Abdominal CT scan showed a huge tumor with a strong enhancement effect. Barium enema and colonoscopic examination revealed a submucosal tumor located from the cecum to the ascending colon on the oral side. An undifferentiated adenocarcinoma was suspected after examination of the biopsy specimen, right hemicolectomy was performed. The tumor invaded the retroperitoneal membrane. Histological examination showed a very atypical carcinoid tumor with central necrosis invading the veins. Pathohistologically, the huge tumor was diagnosed as endocrine cell carcinoma or atypical carcinoid. Furthermore, an elevated lesion, 2.5 cm in size, was revealed in the cecum closed to the huge tumor. Histological examination showed that the polypoid lesion was early moderately differentiated adenocarcinoma. There was no transition between the two tumors. The patient was discharged, but died of local recurrence 9 months after the surgery. Endocrine cell carcinoma of the large bowel is rare, in particular of the ileocecal region. Endocrine cell carcinoma of the ileocecal region adjacent to an adenocarcinoma without transition had not been reported previously in Japan.

CD151 enhances cell motility and metastasis of cancer cells in the presence of focal adhesion kinase.

We have examined the role of the protein CD151 in cell motility, invasion and metastasis of cancer cells by using CD151-overexpressing cells prepared by transfection of CD151 cDNA into three cancer cell lines established from different origins; a human colon cancer RPMI4788, a human glioblastoma A172 and a human fibrosarcoma HT1080. Invasion into Matrigel and cell motility of all 3 CD151-overexpressing cancer cells were enhanced significantly when compared to control parental cells. Pulmonary metastasis of 2 metastatic CD151-overexpressing cancer cell lines, RPMI4788/CD151 and HT1080/CD151, was higher than that of control parental cells and was markedly inhibited by anti-CD151 monoclonal antibody (MAb), SFA1.2B4. To examine whether focal adhesion kinase (FAK) is associated with promotion of cell motility and invasion of cancer cells through CD151, we transfected human CD151 cDNA into FAK (+/+) or FAK (-/-) fibroblasts that were isolated from embryos in FAK-deficient mice and compared invasion into Matrigel and cell motility between each CD151-transfected cells and controls. The invasion into Matrigel and cell motility of CD151-transfected FAK (+/+) fibroblasts increased significantly above those of parental cells and were inhibited by anti-CD151 MAb, whereas those of CD151-transfected FAK (-/-) fibroblasts were not enhanced at all and were not blocked by anti-CD151 MAb. These findings indicate that the CD151 molecule enhances cell motility, invasion and metastasis of cancer cells and that FAK is needed for these events through CD151.