ABSTRACT
Peritoneal membrane dysfunction in peritoneal dialysis (PD) is primarily attributed to angiogenesis; however, the integrity of vascular endothelial cells can affect peritoneal permeability. Hyaluronan, a component of the endothelial glycocalyx, is reportedly involved in preventing proteinuria in the normal glomerulus. One hypothesis suggests that development of encapsulating peritoneal sclerosis (EPS) is triggered by protein leakage due to vascular endothelial injury. We therefore investigated the effect of hyaluronan in the glycocalyx on peritoneal permeability and disease conditions. After hyaluronidase-mediated degradation of hyaluronan on the endothelial cells of mice, macromolecules, including albumin and ß2 microglobulin, leaked into the dialysate. However, peritoneal transport of small solute molecules was not affected. Pathologically, hyaluronan expression was diminished; however, expression of vascular endothelial cadherin and heparan sulfate, a core protein of the glycocalyx, was preserved. Hyaluronan expression on endothelial cells was studied using 254 human peritoneal membrane samples. Hyaluronan expression decreased in patients undergoing long-term PD treatment and EPS patients treated with conventional solutions. Furthermore, the extent of hyaluronan loss correlated with the severity of vasculopathy. Hyaluronan on endothelial cells is involved in the peritoneal transport of macromolecules. Treatment strategies that preserve hyaluronan in the glycocalyx could prevent the leakage of macromolecules and subsequent related complications.
Subject(s)
Peritoneal Dialysis , Peritoneal Fibrosis , Humans , Animals , Mice , Hyaluronic Acid/metabolism , Endothelial Cells , Peritoneal Dialysis/adverse effects , Peritoneum/metabolism , Biological Transport , Dialysis Solutions/metabolism , Peritoneal Fibrosis/etiology , Peritoneal Fibrosis/metabolismABSTRACT
Various cationic polymers are used to deliver polyplex-mediated antisense oligonucleotides (ASOs). However, few studies have investigated the structural determinants of polyplex functionalities in polymers. This study focused on the polymer hydrophobicity. A series of amphiphilic polyaspartamide derivatives possessing various hydrophobic (R) moieties together with cationic diethylenetriamine (DET) moieties in the side chain (PAsp(DET/R)s) were synthesized to optimize the R moieties (or hydrophobicity) for locked nucleic acid (LNA) gapmer ASO delivery. The gene knockdown efficiencies of PAsp(DET/R) polyplexes were plotted against a hydrophobicity parameter, logD7.3, of PAsp(DET/R), revealing that the gene knockdown efficiency was substantially improved by PAsp(DET/R) with logD7.3 higher than -2.4. This was explained by the increased polyplex stability and improved cellular uptake of ASO payloads. After intratracheal administration, the polyplex samples with a higher logD7.3 than -2.4 induced a significantly higher gene knockdown in the lung tissue compared with counterparts with lower hydrophobicity and naked ASO. These results demonstrate that the hydrophobicity of PAsp(DET/R) is crucial for efficient ASO delivery in vitro and in vivo.
Subject(s)
Oligonucleotides, Antisense , Polymers , Polymers/chemistryABSTRACT
We present a single-center retrospective analysis of 228 Japanese patients with peritoneal dialysis, in which we examined whether reduced left ventricular ejection fraction (LVEF) is a risk factor for peritonitis development. Time-dependent multivariable-adjusted Cox proportional hazards models revealed that reduced LVEF (LVEF < 50% vs. preserved LVEF ≥ 50%, hazard ratio (HR) 2.10; 95% confidence interval (CI) 1.16-3.82) was associated with peritonitis. Qualitatively, similar associations with reduced LVEF (< 50%) were observed for enteric peritonitis (adjusted HR 7.68; 95% CI 2.51-23.5) but not for non-enteric peritonitis (adjusted HR 1.15; 95% CI 0.54-2.44). Reduced LVEF is associated with a significantly higher risk of subsequent peritonitis, particularly enteric peritonitis. These results indicate that patients with reduced LVEF may be at risk of enteric peritonitis from bowel sources caused by intestinal involvement due to cardiac dysfunction.
Subject(s)
Peritoneal Dialysis , Peritonitis , Ventricular Dysfunction, Left , Humans , Stroke Volume , Ventricular Function, Left , Retrospective Studies , Japan/epidemiology , Ventricular Dysfunction, Left/etiology , Peritoneal Dialysis/adverse effects , Risk Factors , Peritonitis/epidemiology , Peritonitis/etiologyABSTRACT
The fabrication of artificial structures using a twisted van der Waals assembly has been a key technique for recent advancements in the research of two-dimensional (2D) materials. To date, various exotic phenomena have been observed thanks to the modified electron correlation or moiré structure controlled by the twist angle. However, the twisted van der Waals assembly has further potential to modulate the physical properties by controlling the symmetry. In this study, we fabricated twisted bilayer WTe2 and demonstrated that the twist angle successfully controls the spatial inversion symmetry and hence the spin splitting in the band structure. Our results reveal the further potential of a twisted van der Waals assembly, suggesting the feasibility of pursuing new physical phenomena in 2D materials based on the control of symmetry.
ABSTRACT
Cardiovascular disease is the most common comorbidity in patients with chronic kidney disease (CKD), affecting both their prognosis and quality of life. Cardiac fibrosis is common in patients with CKD with left ventricular diastolic dysfunction, and it is associated with increased risk of heart failure and mortality. Recent evidence suggests that high salt intake activates immune responses associated with local accumulation of sodium. We reported that high salt intake promotes cardiac inflammation in subtotal nephrectomized (Nx) mice. We investigated the effects of administration of MR16-1, a rat anti-mouse monoclonal interleukin (IL)-6 receptor antibody, in Nx mice with salt loading (Nx-salt). Expression of monocyte chemoattractant protein-1, tumor necrosis factor-α, IL-1ß, and IL-6 mRNAs and macrophage infiltration was significantly reduced in the heart of Nx-salt mice treated with MR16-1 (Nx-salt-MR16-1) compared with Nx-salt mice treated with control rat rat IgG1 (Nx-salt-rat IgG1). Correspondingly, cardiac fibrosis was significantly attenuated in Nx-salt-MR16-1 mice compared with Nx-salt-rat IgG1 mice. Furthermore, in the heart of Nx-salt-MR16-1 mice, expression of mRNA for nicotinamide adenine dinucleotide phosphate oxidase-2, an oxidative stress marker, was significantly downregulated compared with Nx-salt-rat IgG1 mice. Increases in cardiac metabolites, including histidine and γ-butyrobetaine, were also reversed by IL-6 blockade treatment. In conclusion, IL-6 blockade exerts anti-inflammatory, antifibrotic, and partial antioxidative effects in the heart of Nx-salt mice.NEW & NOTEWORTHY In the present study, IL-6 blockade exerted anti-inflammatory, antifibrotic, and partial antioxidative effects on the hearts of mice with CKD on a high-salt diet. Therefore, IL-6 potentially mediates cardiac fibrosis induced by high salt intake in patients with CKD, a finding with therapeutic implications. Of note, the next therapeutic implication may simply be the reinforcement of low-salt diets or diuretics and further research on the anti-inflammatory effects of these measures rather than IL-6 blockade with high-salt diet.
Subject(s)
Interleukin-6 , Renal Insufficiency, Chronic , Animals , Mice , Rats , Anti-Inflammatory Agents , Fibrosis , Immunoglobulin G , Inflammation/metabolism , Interleukin-6/genetics , Interleukin-6/metabolism , Quality of Life , Renal Insufficiency, Chronic/chemically induced , Renal Insufficiency, Chronic/drug therapy , Sodium Chloride , Sodium Chloride, DietaryABSTRACT
BACKGROUND: Reconstruction after wide resection of a large sarcoma arising in the posterior trunk may require free-flap transfer to reduce the postoperative complications. Here, we describe the recipient vessels on the whole posterior trunk. Moreover, to show the reliability of these vessels, we describe an institutional series of free-flap reconstruction. METHODS: In the cadaveric study, 20 posterior trunk regions from 10 fixed cadavers were dissected. The location and the diameter of the perforating artery and vein on the posterior were documented. In the clinical study, 54 patients undergoing immediate reconstruction surgery with only a pedicled flap (n = 45) and with a free flap (n = 9) after sarcoma resection on the posterior trunk between July 2005 and September 2021 were identified. One-to-one propensity score matching was performed to compare the postoperative complications. RESULTS: In the cadaveric study, a total of 178 perforators were identified. The average diameter of the superficial cervical artery (SCA) and vein, dorsal scapular artery (DSA) and vein, medial branch of dorsal intercostal artery perforator (m-DICAP) and vein, lateral branch of dorsal intercostal artery perforator (l-DICAP) and vein, and dorsolateral intercostal artery perforator (DLICAP) and vein were 1.03, 1.67, 1.38, 1.84, 1.28, 1.84, 1.01, 1.60, 1.11, and 1.70 mm. In the clinical study, the propensity score-matched analysis involving eight pairs showed a significantly higher occurrence of total complications in the pedicled-flap group than the free-flap group (62.5 vs. 0%, p = 0.03). CONCLUSION: The cadaveric study showed that the perforators from the SCA, DSA, and posterior intercostal artery are constantly present. The clinical study demonstrated the feasibility and reliability of a free-flap transfer.
Subject(s)
Free Tissue Flaps , Perforator Flap , Plastic Surgery Procedures , Sarcoma , Soft Tissue Neoplasms , Humans , Reproducibility of Results , Sarcoma/surgery , Cadaver , Postoperative Complications , Perforator Flap/blood supplyABSTRACT
Symptomatic large pineal cyst (PC) remains a rare entity. The stable natural course of asymptomatic PCs is well established. However, large cysts may cause pressure-related symptoms necessitating surgical intervention. The surgical strategy for symptomatic PCs is still controversial. Regardless of the approach, total resection of the cyst is not mandatory. The endoscopic approach allows cyst fenestration in patients with associated obstructive hydrocephalus. On the other hand, the necessity of simultaneous endoscopic third ventriculostomy (ETV) is still debatable. Here, we report a case of a woman who underwent endoscopic cyst fenestration, biopsy, and third ventriculostomy for a large symptomatic PC and discuss the surgical strategy. A 30-year-old woman presented with headache and diplopia, MRI showed a large PC and accompanying obstructive hydrocephalus. Simultaneous cyst fenestration, biopsy and ETV with endoscopy was successfully completed. She had an uneventful recovery period with immediate relief of symptoms. Although, the aqueduct was communicated due to cyst shrinkage, the patency of the third ventricular stoma was demonstrated in long-term follow-up scans. Based on clinical course of the present case, we concluded that ETV in addition to cyst fenestration should be considered necessary and beneficial in cases of large symptomatic PC with associated hydrocephalus whenever an endoscopic intraventricular approach is considered.
Subject(s)
Brain Neoplasms , Cysts , Hydrocephalus , Third Ventricle , Adult , Brain Neoplasms/complications , Brain Neoplasms/surgery , Cysts/complications , Cysts/diagnostic imaging , Cysts/surgery , Female , Humans , Hydrocephalus/etiology , Hydrocephalus/surgery , Third Ventricle/diagnostic imaging , Third Ventricle/surgery , Treatment Outcome , VentriculostomyABSTRACT
Vascular endothelial cells are covered with glycocalyx comprising heparan sulfate, hyaluronan, chondroitin sulfate, and associated proteins. Glomerular endothelial glycocalyx is involved in protecting against induction of proteinuria and structural damage, but the specific components in glycocalyx that represent therapeutic targets remain unclear. Anti-vascular endothelial growth factor (VEGF) therapy is associated with an increased risk of glomerular endothelial injury. This study investigated whether hyaluronan could provide a therapeutic target to protect against proteinuria. We conducted ex vivo and in vivo experiments to explore the effects of degrading glomerular hyaluronan by administering hyaluronidase and of supplementation with hyaluronan. We investigated hyaluronan expression using biotin-labeled hyaluronan-binding protein (HABP) in human kidney specimens or serum hyaluronan in endothelial injuries under inhibition of VEGF signaling. We directly demonstrated hyaluronan in glomerular endothelial layers using HABP staining. Ex vivo and in vivo experiments showed the development of proteinuria after digestion of hyaluronan in glomerular capillaries. Supplementation with hyaluronan after hyaluronidase treatment suppressed proteinuria. Mice in the in vivo study developed albuminuria after intraperitoneal injection of hyaluronidase with decreased glomerular hyaluronan and increased serum hyaluronan. In human kidneys with endothelial cell dysfunction and proteinuria due to inhibition of VEGF, glomerular expression of hyaluronan was reduced even in normal-appearing glomeruli. Serum hyaluronan levels were elevated in patients with pre-eclampsia with VEGF signaling inhibition. Our data suggest that hyaluronan itself plays crucial roles in preventing proteinuria and preserving the integrity of endothelial cells. Hyaluronan could provide a therapeutic target for preventing glomerular endothelial glycocalyx damage, including VEGF signaling inhibition.
Subject(s)
Endothelial Cells/metabolism , Glycocalyx/metabolism , Hyaluronic Acid/biosynthesis , Kidney Glomerulus/metabolism , Proteinuria/metabolism , Animals , Cattle , Endothelial Cells/drug effects , Endothelial Cells/pathology , Female , Glycocalyx/drug effects , Glycocalyx/pathology , Humans , Hyaluronoglucosaminidase/administration & dosage , Kidney Glomerulus/drug effects , Kidney Glomerulus/pathology , Male , Mice , Mice, Inbred C57BL , Organ Culture Techniques , Pregnancy , Proteinuria/pathology , Rats , Rats, Inbred LewABSTRACT
BACKGROUND: Intraoperative flash stimulation visual evoked potential (VEP) monitoring has been used for endoscopic endonasal approach (EEA). Recently, off-response VEP, which is recorded when the light stimulus is turned off, was introduced to monitor visual function intraoperatively. OBJECTIVE: To evaluate off-response VEP monitoring in comparison with the conventional flash stimulation VEP monitoring for EEA. METHODS: From March 2015 to March 2020, 70 EEA surgeries with intraoperative VEP monitoring (140 eyes) were performed. Light stimuli were delivered by a pair of goggle electrodes. Recording electrodes were placed on the scalp over the occipital region. The warning signal was prompted by a reduction of the peak-to-peak amplitude of the VEP by more than 50% compared to the initial amplitude. Visual function was assessed pre- and postoperatively. Results of flash and off-response VEP monitoring were compared. RESULTS: VEP was recorded in 134 eyes. Warning signal occurred in 23 eyes (transient in 17 eyes and permanent in 6 eyes). Two eyes showed permanent VEP attenuation for flash VEP monitoring, in which one patient had postoperative visual function deterioration. Four eyes showed permanent VEP attenuation for off-response VEP monitoring, where 2 patients had postoperative visual function deterioration. Sensitivity, specificity, positive predictive value, and negative predictive value were 100%, 98.9%, 50%, and 100%, respectively, for flash stimulation VEP, and 100%, 97.8%, 50%, and 100%, respectively, for off-response VEP. CONCLUSION: VEP monitoring was useful to monitor visual function in EEA surgery. Off-response VEP monitoring was not inferior to conventional flash stimulation VEP monitoring.
Subject(s)
Evoked Potentials, Visual , Eye Diseases , Endoscopy , Humans , Monitoring, Intraoperative , Neurologic ExaminationABSTRACT
BACKGROUND: Lymphaticovenular anastomosis (LVA) is a challenging procedure and requires a sophisticated supermicrosurgical technique. The aim of this study was to evaluate and establish a discrete supermicrosurgical anastomosis method using the "suture-stent technique". METHODS: Forty-eight LVA sites of twenty patients with lower extremity lymphedema who had undergone LVA between July 2020 and January 2021 were included in this study. LVA was performed with the conventional technique or with the suture-stent technique. The patency of the anastomoses was evaluated using an infrared camera system intraoperatively. The success rate on the first try and the final success rate for each group were compared. RESULTS: After full application of the exclusion criteria, 35 LVAs of 16 patients including 20 limbs were included in the analysis. The ratio of good patency findings after anastomosis in the suture-stent technique group was 100%. The incidences of leakage or occlusion on the first try were statistically greater in the conventional technique group (29.4%) than in the suture-stent technique group (0%) (p = 0.0191). All anastomoses achieved good patency in the final results. CONCLUSION: With its minimal risk of catching the back wall during the anastomosis, the suture-stent technique can be considered an optimal anastomosis option for LVA.
ABSTRACT
A 52-year-old man experienced sudden-onset global amnesia and left limb ataxia. An embolism of the right anterior cerebral artery resulted in anterior cingulate cortex (ACC) infarction, and working memory dysfunction persisted. The ACC, prefrontal cortex, and bilateral superior parietal lobule exhibited decreased activity on single-photon emission computed tomography (SPECT). The ACC handles working memory formation and is essential for the executive function. The areas showing a decreased activity on SPECT were responsible for the working memory, which corresponded to the observed symptoms. This is the first case in which limited ACC infarction resulted in permanent working memory dysfunction, and SPECT revealed the decreasing working memory in the associated region.
Subject(s)
Gyrus Cinguli , Memory, Short-Term , Cerebral Infarction/complications , Cerebral Infarction/diagnostic imaging , Gyrus Cinguli/diagnostic imaging , Humans , Magnetic Resonance Imaging , Male , Memory Disorders/etiology , Middle Aged , Tomography, Emission-Computed, Single-PhotonABSTRACT
INTRODUCTION: Albeit uncommon, hydrothorax is an important complication of peritoneal dialysis (PD). Due to paucity of evidence for optimal treatment, this study aimed to evaluate the effectiveness and safety of computed tomographic (CT) peritoneography and surgical intervention involving video-assisted thoracic surgery (VATS) for hydrothorax in a retrospective cohort of patients who underwent PD in Japan. METHODS: Of the 982 patients who underwent PD from six centers in Japan between 2007 and 2019, 25 (2.5%) with diagnosed hydrothorax were enrolled in this study. PD withdrawal rates were compared between patients who underwent VATS for diaphragm repair (surgical group) and those who did not (non-surgical group) using the Kaplan-Meier method and log-rank test. RESULTS: The surgical and non-surgical groups comprised a total of 11 (44%) and 14 (56%) patients, respectively. Following hydrothorax diagnosis by thoracentesis and detection of penetrated sites on the diaphragm using CT peritoneography, VATS was performed at a median time of 31 days (interquartile range [IQR], 20-96 days). During follow-up (median, 26 months; IQR, 10-51 months), 9 (64.3%) and 2 (18.2%) patients in the non-surgical and surgical groups, respectively, withdrew from PD (P = 0.021). There were no surgery-related complications or hydrothorax relapse in the surgical group. CONCLUSIONS: This study demonstrated the effectiveness and safety of CT peritoneography and VATS for hydrothorax. This approach may be useful in hydrothorax cases to avoid early drop out of PD and continue PD in the long term. Further studies are warranted to confirm these results.
Subject(s)
Hydrothorax/surgery , Kidney Failure, Chronic/complications , Peritoneal Dialysis/adverse effects , Thoracic Surgery, Video-Assisted , Adult , Aged , Female , Humans , Hydrothorax/etiology , Japan , Male , Middle Aged , Retrospective StudiesABSTRACT
BACKGROUND: Acromegaly has been reported in adolescents and young adults, but it is unusual in preadolescence. Diagnosis and management pose different challenges in this age group. Here, we present a rare case of acromegaly in preadolescence. CASE DESCRIPTION: A 9-year-old boy presented with acromegalic features and MRI revealed a pituitary tumor. He was diagnosed as having growth hormone secreting pituitary adenoma based on the multidisciplinary assessment, and underwent gross total tumor resection through an endoscopic endonasal approach (EEA) with subsequent normalization of the hormonal parameters. CONCLUSIONS: Advances in EEA have made safe removal and cure possible even in children. However, long- term follow-up is needed in such younger patients with multidisciplinary management by neurosurgeons, endocrinologists, pediatricians, and ENT surgeons.
ABSTRACT
To tackle the rising healthcare expenditure in an ageing society in Japan, home healthcare has been promoted over the past several years. However, there is a dearth of literature on total costs incurring for home healthcare. In this study, we conducted a cross-sectional study among patients, who received home healthcare in the month of May, 2018. Direct healthcare costs and patients' clinical characteristics were collected from medical records and long-term care databases (n = 166). Indirect costs were estimated using a questionnaire survey which obtained information on job absenteeism and care time from the caregiver. A total of 112 patients responded to the survey. The median age was 82 years (interquartile range: 74-88). Total per-person per month home-care costs averaged USD 6,163 with direct costs (USD 2,547) and indirect costs (USD 3,596) accounted for 41.3% and 58.3% of the total costs, respectively. The largest components of direct costs were long-term care costs (48%) and medical costs (47%). Multivariable adjusted model showed that those with heavy healthcare were more likely to incur higher total as well as direct and indirect home healthcare cost (p<.05 for each). Patients aged >75 years (p = .041) were less likely and those who used oxygen at home were more likely to incur direct home healthcare cost (p = .001) than their counterpart. Our study findings show that indirect cost is a major contributor to total home healthcare costs in Japan. Also for patients who need heavy healthcare, both direct and indirect costs are large burden.
Subject(s)
Caregivers/economics , Cost of Illness , Direct Service Costs/statistics & numerical data , Home Care Services/economics , Aged , Aged, 80 and over , Cross-Sectional Studies , Female , Health Expenditures/statistics & numerical data , Home Care Services/organization & administration , Humans , Japan , Male , Outcome Assessment, Health CareABSTRACT
BACKGROUND: Superficial siderosis (SS) associated with craniopharyngioma is an extremely rare occurrence. To our knowledge, only 3 cases have been reported in literature. Two of the cases occurred following resection of the tumor. Similar to the present case, 1 of the cases was discovered before surgical intervention, "pure tumor-related" SS. The clinical presentation, diagnosis, management, and outcome are discussed in this article. CASE DESCRIPTION: A 50-year-old man presented with an 11-month history of left-sided tinnitus, hearing impairment, and a 2-month history of visual disturbance. Cerebellar ataxia and gait disturbance were found on examination. Brain magnetic resonance imaging findings were consistent with SS. Cerebrospinal fluid (CSF) analysis before surgery revealed xanthochromia confirming an existing chronic sustainable subarachnoid hemorrhage. He underwent a tumor resection in endoscopic endonasal approach followed by stereotactic radiosurgery. On follow-up, the visual symptoms resulting from craniopharyngioma subsided, whereas hearing impairment, cerebellar ataxia, and gait disturbance associated with SS did not progress but were unchanged despite subsequent evidence of watery-clear CSF. CONCLUSIONS: In the present case, SS could be associated with craniopharyngioma on the basis of CSF findings and clinical symptoms. Detection and early treatment aimed at eliminating the bleeding source offer the best chance of halting the disease process.
Subject(s)
Craniopharyngioma/complications , Pituitary Neoplasms/complications , Siderosis/complications , Brain/diagnostic imaging , Craniopharyngioma/diagnostic imaging , Craniopharyngioma/surgery , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Neurosurgical Procedures/methods , Pituitary Neoplasms/diagnostic imaging , Pituitary Neoplasms/surgery , Siderosis/diagnostic imaging , Siderosis/surgery , Tomography Scanners, X-Ray ComputedABSTRACT
Nontuberculous mycobacteria (NTM) are rarely isolated from peritoneal dialysis (PD)-associated catheter infections. However, NTM infection is usually difficult to treat and leads to catheter loss. Prompt diagnosis is essential for appropriate treatment. A 70-year-old Japanese man who had been on PD for 2 years and with a medical history of 2 episodes of exit site infections (ESIs) due to methicillin-resistant Staphylococcus aureus was admitted to the hospital due to suspected ESI recurrence. However, Gram staining of the pus revealed no gram-positive cocci. Instead, weakly stained gram-positive rods were observed after 7 days of incubation, which were also positive for acid-fast staining. Rapidly growing NTM Mycobacterium chelonae was isolated on day 14. Despite administering a combination antibiotic therapy, ESI could not be controlled, and catheter removal surgery was performed on day 21. Although PD was discontinued temporarily, the patient did not require hemodialysis, without any uremic symptoms. The catheter was reinserted on day 48, and PD was reinitiated on day 61. The patient was discharged on day 65. Antibiotic therapy was continued for 3 months after discharge, with no indications of recurrent infections observed. It is important to consider the risk of NTM infections in patients on PD. Acid-fast staining could be a key test for prompt diagnosis and provision of an appropriate treatment.
ABSTRACT
BACKGROUND: TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal failure, and organomegaly) syndrome is a systemic inflammatory disorder and unique clinicopathological variant of idiopathic multicentric Castleman disease that was proposed in Japan. Prompt diagnosis is critical because TAFRO syndrome is a progressive and life threating disease. Some cases are refractory to immunosuppressive treatments. Renal impairment is frequently observed in patients with TAFRO syndrome, and some severe cases require hemodialysis. Histological evaluation is important to understand the pathophysiology of TAFRO syndrome. However, systemic histopathological evaluation through autopsy in TAFRO syndrome has been rarely reported previously. CASE PRESENTATION: A 46-year-old Japanese man with chief complaints of fever and abdominal distension was diagnosed with TAFRO syndrome through imaging studies, laboratory findings, and pathological findings on cervical lymph node and bone marrow biopsies. Interleukin (IL)-6 and vascular endothelial growth factor (VEGF) levels were remarkably elevated in both blood and ascites. Methylprednisolone (mPSL) pulse therapy was initiated on day 10, followed by combination therapy with PSL and cyclosporine A. However, the amount of ascites did not respond to the treatment. The patient became anuric, and continuous renal replacement therapy was initiated from day 50. However, the patient suddenly experienced cardiac arrest associated with myocardial infarction (MI) on the same day. Although the emergent percutaneous coronary intervention was successfully performed, the patient died on day 52, despite intensive care. Autopsy was performed to ascertain the cause of MI and to identify the histopathological characteristics of TAFRO syndrome. CONCLUSIONS: Bacterial peritonitis, systemic cytomegalovirus infection, and Trichosporon asahii infection in the lungs were observed on autopsy. In addition, sepsis-related myocardial calcification was suspected. Management of infectious diseases is critical to reduce mortality in patients with TAFRO syndrome. Although the exact cause of MI could not be identified on autopsy, we considered embolization by fungal hyphae as a possible cause. Endothelial injury possibly caused by excessive secretion of IL-6 and VEGF contributed to renal impairment. Fibrotic changes in anterior mediastinal fat tissue could be a characteristic pathological finding in patients with TAFRO syndrome.
ABSTRACT
BACKGROUND Spontaneous spinal epidural hematoma (SSEH) occurs in the spinal epidural space in the absence of traumatic or iatrogenic causes, and is considered to be a neurological emergency, as spinal cord compression may lead to neurological deficit. Prompt diagnosis of SSEH can be difficult due to the variety of presenting symptoms, which may resemble those of stroke. Patients who undergo hemodialysis (HD) are at risk of bleeding due to anticoagulation during dialysis and uremia. However, SSEH in HD patients undergoing HD has rarely been reported. CASE REPORT A 70-year-old Japanese man, who has been undergoing maintenance HD for the previous three years, was admitted to Kariya Toyota General Hospital, Aichi, Japan, with acute chest and abdominal pain, and with complete paraplegia. The patient denied any recent trauma or medical procedures. Magnetic resonance imaging showed an extensive hematoma in the thoracic and lumbar epidural space, extending from T8 to L5. The patient's symptoms improved within three hours following hospital admission, and after three days without HD treatment, the SSEH decreased in size, and the patient successfully recovered without residual neurological deficits and without requiring surgery. CONCLUSIONS The management of SSEH in patients undergoing HD can be difficult, due to anticoagulation during dialysis and uremia. Prompt diagnosis and close neurological monitoring are important for appropriate management. In patients whose symptoms improve within a short period, conservative management may be considered.
Subject(s)
Hematoma, Epidural, Spinal/therapy , Renal Dialysis , Aged , Conservative Treatment , Hematoma, Epidural, Spinal/diagnostic imaging , Humans , Magnetic Resonance Imaging , Male , Paraplegia/etiology , Recovery of FunctionABSTRACT
A 52-year-old Japanese male professional diver was referred to our hospital for decompression illness (DCI). After 1 h of diving operation at 20 m below sea level, he complained of dyspnea, chest pain, and abdominal pain. He dove again, intending to ease the symptoms, but the symptoms were never relieved. He dove for a total of 4 h. No neurological abnormalities were observed. Computed tomography images revealed portal venous gas and mesenteric venous gas, in addition to bubbles in the femoral veins, pelvis, lumbar canal, intracranial sinuses, and joints. Hyperbaric oxygen therapy (HBOT) was immediately administered. His symptoms improved after the first course of HBOT, however, the patient had anuria for almost 36 h after admission and exhibited acute kidney injury (AKI). Serum creatinine and creatine kinase (CK) levels were increased to maximal values of 6.16 mg/dL and 18,963 U/L, respectively. Blood flow signals were not detected on kidney Doppler ultrasound. We considered that AKI was caused by blood flow impairment and capillary leak syndrome due to DCI in addition to rhabdomyolysis secondary to arterial gas embolism in the skeletal muscles. Temporary dialysis was required to correct the acidemia and electrolyte disturbance. Diuretic phase was initiated, and the patient was put off dialysis on day 3. Serum creatinine and CK levels returned to normal on day 11. He was successfully treated without any complications. Although AKI is a rare manifestation, we should consider AKI risk in patients with severe DCI.
ABSTRACT
An 89-year-old Japanese man on peritoneal dialysis (PD) was suspected of having a PD-associated catheter infection. He visited the hospital because of the discharge of pus from the exit site of his catheter. Gram staining of the pus showed Gram-positive bacilli, but these were acid-fast bacilli. The rapidly growing nontuberculous mycobacteria, Mycobacterium abscessus, was isolated. PD catheter removal and debridement were immediately performed. The patient received combination antibiotic therapy. His clinical course was good, but he required hemodialysis due to the discontinuation of PD. However, the patient and his family chose not to continue hemodialysis even when the symptoms of uremia appeared. Best supportive care was arranged by his primary care physician. M. abscessus is a rare causative organism for PD-associated catheter infections and is difficult to treat. In our case, a rapid and precise diagnosis was made using acid-fast staining and Mycobacterium culture. The risk of nontuberculous mycobacterial infections should be considered in patients on PD.