We present a case of Marfan's syndrome with acute aortic dissection during the trimester of her pregnancy, who underwent a Bentall operation 2 days after emergency cesarean section. A 24-year-old woman during the 31st week of pregnancy visited our emergency room due to sudden onset of chest and back pain, though she had no abnormality until this event. Because of her tall height, spider fingers, positive wrist sign, visual disorder and scoliosis, she was given a diagnosis of Marfan's syndrome. Enhanced CT and cardiac ultrasonography revealed that she was suffering from acute aortic dissection with annulo-aortic ectasia. Since it was difficult for her to continue with her pregnancy, she underwent emergency cesarean section and gave birth to a male baby weighted 1, 706g. Although there was little likelifood of early thrombus formation in the false lumen or significant aortic regurgitation indicating an emergency operation, fear of massive bleeding from her uterus and the exfoliated surface of the placenta after cesarean section required an observation period of 2 days. We performed a Bentall operation successfully after careful sedation, ventilation and blood pressure control for 2 days.

Protein C (PC) deficiency is an inherited thrombotic disorder with a prevalence of 0.19% among the general population. PC deficiency is associated with an increased risk of thrombosis when other risk factors are present, such as trauma, surgery, or infection, and is an important cause of mechanical valve thrombosis. We performed tricuspid valve replacement with a 29mm Carpentier-Edwards Perimount valve in a 20-year-old man with PC deficiency. The patient had corrected transposition of the great vessels with severe tricuspid insufficiency, as well as a history of cerebral infarction. In the perioperative period, we used only heparin sodium as the anticoagulant. When we restarted administration of warfarin, changing over from heparin, transient increases of serum plasmin inhibitor-plasmin complex (PIC) and thrombin antithrombin complex (TAT) levels were observed. Despite an increased dose of heparin, an appropriate activated partial thromboplastin time (APTT) was not obtained. This suggested a hypercoagulatory state, but the postoperative course was uneventful. Management of perioperative anticoagulation, prevention of late thrombotic events, and prosthetic valve selection in this particular situation are discussed.

A 56-year-old woman suffering from mitral stenosis had underwent PTMC (percutaneous transvenous mitral commissurotomy) at age 46. After she developed congestive heart failure, mitral valve replacement (MVR) with Carbomedics 29M and tricuspid annuloplasty (TAP) was carried out. Four hours after admission to the ICU, massive bleeding was noticed. Cardiopulmonary bypass was restarted in the operating room. Laceration and hematoma were found at the posterolateral wall of the left ventricle. Under cardiac arrest with removal of the prosthetic valve, an internal tear was detected about 2cm below the anterolateral commissure (Miller Type III). The tear was covered with a horse pericardial patch (2×3cm) using 6-0 running sutures with reinforcement with gelatin-resorcine-formaline (GRF) glue between the laceration and the patch. MVR sutures in the annulus above the ventricular tear were first passed through the annulus, the pericardial patch and then the prosthetic cuff. Additionally, an epicardial tear was covered and reinforced with the fibrin sheet, GRF glue and pericardial patch in turn. Cardiopulmonary bypass was weaned easily without bleeding. The patient was intentionally on respiratory support with sedation for 3 days. The subsequent postoperative course was uneventful.

Infected aortic aneurysm is very difficult to treat and is associated with a high mortality rate. A 78-year-old man had been scheduled to undergo selective endovascular repair for distal aortic arch aneurysm. While standby, however, he was admitted to our emergency room because of hemoptysis. Rapid dilatation of the aneurysm shown on serial CT and elevated of inflammatory reactions yielded a diagnosis of infected aortic aneurysm. Because the aneurysm had ruptured into the left lung, emergency surgery was performed. Six days after the first operation, critical bleeding due to anastomotic disruption of the distal aorta caused by infection and subsequent cardiac arrest occurred. We immediately started open chest massage and controlled the bleeding manually in the ICU, while an operating room was prepared. In the redo operation, anastomotic disruption was repaired using the visceral pleura under deep hypothermic circulatory arrest. Anastomotic bleeding is a potentially life-threatening condition, therefore extremely prompt measures are vital. Appropriate management based on the assumption of anastomotic bleeding was very important in the postoperative course of this case of infectious aortic aneurysm.

It was reported that nerve fibers were present in the inner part of lumbar intervertebral discs from patients with discogenic pain. Because there are no nerve fibers in the inner part of annulus fibrosus in normal condition, this finding suggests nerve ingrowth into the disc may be a cause of discogenic pain. Disc degeneration is often asymptomatic, thus, to understand the differences between symptomatic and asymptomatic disc, it is necessary to understand the pathogenesis of discogenic pain. We recently revealed that over 90% of the nociceptive dorsal root ganglion (DRG) neurons innervating the disc are sensitive to nerve growth factor (NGF), which is related to inflammatory pain. This indicates that discogenic pain is closely related to inflammation and NGF may play a key role. The increase of inflammatory mediators in symptomatic discs has been reported; we therefore studied the effects of disc inflammation and found that it induces sensitization of disc-innervating neurons and nerve ingrowth into the disc. More recently, it was shown that annular rupture induces nerve ingrowth, an increase of inflammatory mediators in the disc, and upregulation of calcitonin gene-related peptide, a pain-related molecule in DRGs. These findings led us to believe that annular rupture triggers inflammation and nerve ingrowth, inflammatory mediators then further promote nerve ingrowth into the disc and sensitization of disc-innervating neurons, and discogenic pain finally becomes chronic. NGF, found in symptomatic discs, may act as a key factor in generating chronic discogenic pain by sensitizing disc-innervating neurons and stimulating nerve ingrowth into the disc.
Calcitonin Gene-Related Peptide ; Diagnosis-Related Groups ; Ganglia, Spinal ; Humans ; Inflammation ; Intervertebral Disc ; Intervertebral Disc Degeneration ; Nerve Fibers ; Nerve Growth Factor ; Neurons ; Rupture ; Up-Regulation

STUDY DESIGN: Retrospective cohort study. PURPOSE: To investigate the effect of spinal correction on respiratory muscle strength in patients with Duchenne muscular dystrophy (DMD). OVERVIEW OF LITERATURE: Several studies have reported that scoliosis correction in patients with DMD does not improve pulmonary function. In these studies, pulmonary function was evaluated using the traditional spirometric values of percent vital capacity (%VC) and percent forced vital capacity (%FVC). However, traditional spirometry may not be suitable for patients with DMD because the results can be influenced by patient fatigue or level of understanding. Therefore, we evaluated respiratory function focusing on respiratory muscle strength using maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and sniff nasal inspiratory pressure (SNIP), in addition to %VC and %FVC. METHODS: We retrospectively reviewed 16 patients with DMD who underwent spinal correction surgery between 2006 and 2011 at Kitasato University Hospital. All patients were males, and the mean age was 13.5 years. Respiratory muscle strength was evaluated using MIP, MEP, and SNIP. Measurements were obtained preoperatively and at 1 and 6 months postoperatively, and %VC and %FVC were obtained preoperatively and within 6 months postoperatively. RESULTS: The mean preoperative and postoperative %VC values were 54.0% and 51.7%, whereas the mean %FVC values were 53.9% and 53.2%, respectively. The mean MIP, MEP, and SNIP values obtained preoperatively and at 1 and 6 months postoperatively were as follows: MIP, 40.5, 42.7 and 47.2 cm H₂O; MEP, 26.0, 28.0, and 29.0 cm H₂O; and SNIP, 33.4, 33.0, and 33.0 cm H₂O; respectively. The mean MIP and MEP values significantly improved postoperatively. There were no significant differences in SNIP, %VC, or %FVC preand postoperatively. CONCLUSIONS: By focusing on respiratory muscle strength, our results suggest that scoliosis correction in patients with DMD might have a favorable effect on respiratory function.
Cohort Studies ; Fatigue ; Humans ; Male ; Muscular Dystrophy, Duchenne* ; Respiratory Muscles* ; Retrospective Studies ; Scoliosis* ; Spirometry ; Vital Capacity

Background: Type 1 diabetes mellitus induced by immune-checkpoint inhibitors (ICI-T1DM) is a rare critical entity. However, the etiology of ICI-T1DM remains unclear. Methods: In order to elucidate risk factors for ICI-T1DM, we evaluated the clinical course and immunological status of patients with ICI-T1DM who had been diagnosed during 2016 to 2021. Results: Seven of 871 (0.8%, six men and one woman) patients developed ICI-T1DM. We revealed that the allele frequencies of human leukocyte antigen (HLA)-DPA1*02:02 and DPB1*05:01 were significantly higher in the patients with ICI-T1DM In comparison to the controls who received ICI (11/14 vs. 10/26, P=0.022; 11/14 vs. 7/26, P=0.0027, respectively). HLA-DRB1*04:05, which has been found to be a T1DM susceptibility allele in Asians, was also observed as a high-risk allele for ICI-T1DM. The significance of the HLA-DPB1*05:01 and DRB1*04:05 alleles was confirmed by an analysis of four additional patients. The absolute/relative neutrophil count, neutrophils-lymphocyte ratio, and neutrophil-eosinophil ratio increased, and the absolute lymphocyte count and absolute/relative eosinophil count decreased at the onset as compared with 6 weeks before. In two patients, alterations in cytokines and chemokines were found at the onset. Conclusion Novel high-risk HLA alleles and haplotypes were identified in ICI-T1DM, and peripheral blood factors may be utilized as biomarkers.

PURPOSE: Sacroiliac fixation using iliac screws for highly unstable lumbar spine has been reported with an improved fusion rate and clinical results. On the other hand, there is a potential for clinical problems related to iliac fixation, including late sacroiliac joint arthritis and pain. MATERIALS AND METHODS: Twenty patients were evaluated. Degenerative scoliosis was diagnosed in 7 patients, failed back syndrome in 6 patients, destructive spondyloarthropathy in 4 patients, and Charcot spine in 3 patients. All patients underwent posterolateral fusion surgery incorporating lumbar, S1 and iliac screws. We evaluated the pain scores, bone union, and degeneration of sacroiliac joints by X-ray imaging and computed tomography before and 3 years after surgery. For evaluation of low back and buttock pain from sacroiliac joints 3 years after surgery, lidocaine was administered in order to examine pain relief thereafter. RESULTS: Pain scores significantly improved after surgery. All patients showed bone union at final follow-up. Degeneration of sacroiliac joints was not seen in the 20 patients 3 years after surgery. Patients showed slight low back and buttock pain 3 years after surgery. However, not all patients showed relief of the low back and buttock pain after injection of lidocaine into the sacroiliac joint, indicating that their pain did not originate from sacroiliac joints. CONCLUSION: The fusion rate and clinical results were excellent. Also, degeneration and pain from sacroiliac joints were not seen within 3 years after surgery. We recommend sacroiliac fixation using iliac screws for highly unstable lumbar spine.
Aged ; Arthritis/*surgery ; Bone Screws ; Female ; Humans ; Low Back Pain/diagnosis/epidemiology ; Lumbar Vertebrae/*surgery ; Male ; Middle Aged ; Pain/diagnosis/*epidemiology ; Sacroiliac Joint/*immunology/*pathology

PURPOSE: Chronic low back pain is a common clinical problem. As medication, non-steroidal anti-inflammatory drugs are generally used; however, they are sometimes non-effective. Recently, opioids have been used for the treatment of chronic low back pain, and since 2010, transdermal fentanyl has been used to treat chronic non-cancer pain in Japan. The purpose of the current study was to examine the efficacy of transdermal fentanyl in the treatment of chronic low back pain. MATERIALS AND METHODS: This study included patients (n=62) that suffered from chronic low back pain and were non-responsive to non-steroidal anti-inflammatory drugs. Their conditions consisted of non-specific low back pain, multiple back operations, and specific low back pain awaiting surgery. Patients were given transdermal fentanyl for chronic low back pain. Scores of the visual analogue scale and the Oswestry Disability Index, as well as adverse events were evaluated before and after therapy. RESULTS: Overall, visual analogue scale scores and Oswestry Disability Index scores improved significantly after treatment. Transdermal fentanyl (12.5 to 50 microg/h) was effective in reducing low back pain in 45 of 62 patients; however, it was not effective in 17 patients. Patients who experienced the most improvement were those with specific low back pain awaiting surgery. Adverse events were seen in 40% of patients (constipation, 29%; nausea, 24%; itching, 24%). CONCLUSION: Disability Index scores in 73% of patients, especially those with specific low back pain awaiting surgery; however, it did not decrease pain in 27% of patients, including patients with non-specific low back pain or multiple back operations.
Administration, Cutaneous ; Adult ; Aged ; Aged, 80 and over ; Chronic Disease ; Female ; Fentanyl/*administration & dosage/*therapeutic use ; Humans ; Low Back Pain/*drug therapy ; Male ; Middle Aged ; Young Adult

PURPOSE: Pain from osteoarthritis (OA) is generally classified as nociceptive (inflammatory). Animal models of knee OA have shown that sensory nerve fibers innervating the knee are significantly damaged with destruction of subchondral bone junction, and induce neuropathic pain (NP). Our objective was to examine NP in the knees of OA patients using painDETECT (an NP questionnaire) and to evaluate the relationship between NP, pain intensity, and stage of OA. MATERIALS AND METHODS: Ninety-two knee OA patients were evaluated in this study. Pain scores using Visual Analogue Scales (VAS), Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), painDETECT, duration of symptoms, severity of OA using the Kellgren-Lawrence (KL) system, and amount of joint fluid were evaluated and compared using a Spearman's correlation coefficient by rank test. RESULTS: Our study identified at least 5.4% of our knee OA patients as likely to have NP and 15.2% as possibly having NP. The painDETECT score was significantly correlated with the VAS and WOMAC pain severity. Compared with the painDETECT score, there was a tendency for positive correlation with the KL grade, and tendency for negative correlation with the existence and amount of joint fluid, but these correlations were not significant. CONCLUSION: PainDETECT scores classified 5.4% of pain from knee OA as NP. NP tended to be seen in patients with less joint fluid and increased KL grade, both of which corresponded to late stages of OA. It is important to consider the existence of NP in the treatment of knee OA pain.
Aged ; Aged, 80 and over ; Female ; Humans ; Knee/pathology/physiopathology ; Male ; Middle Aged ; Neuralgia/*physiopathology ; Osteoarthritis, Knee/*physiopathology