Objective In an effort to reduce mortality from gastric cancer, endoscopic screening was introduced in 2016 as a nationwide screening program in Japan. Recent developments in high-definition endoscopic imaging and diagnostic strategies have enabled the simultaneous detection of other upper gastrointestinal (U-GI) malignancies. Therefore, we conducted a study to evaluate the feasibility of endoscopic screening for U-GI malignancy in a comprehensive health checkup.
Methods We retrospectively reviewed the data of 13,120 participants who had received a comprehensive health checkup in a single institution between April 2012 and March 2018. Participants were divided into two groups [gastrointestinal endoscopy (GIE) group (n=9,142) and gastrointestinal X-ray (X-ray) group (n=3,978)] and compared with regards to the screening results, adverse events, and detection rate of U-GI malignancies (gastric cancer or other) using a propensity-score matched analysis.
Results The gastric cancer detection rate was significantly higher in the GIE group [34/9,142 (0.48%)] than in the X-ray group [3/3,978 (0.08%)] (p=0.003). Other U-GI malignancies were found only in the GIE group and comprised two hypopharyngeal cancers, five esophageal cancers, two duodenal cancers, and one duodenal gastrointestinal stromal tumor. Adverse events occurred in 6/9,142 (0.07%) participants in the GIE group and 18/3,978 (0.45%) participants in the X-ray group (p<0.0001). A propensity-score matched analysis yielded 1,551 matched pairs, and the detection rate of gastric cancer and other U-GI malignancies remained significantly higher in the GIE group than in the X-ray group.
Conclusion This study indicated that not only gastric cancer but also other U-GI malignancies can be detected by endoscopic screening.
Objective This study evaluated the efficacy associated with switching to rifaximin in patients with hepatic cirrhosis receiving kanamycin sulfate for the treatment of hepatic encephalopathy and hyperammonemia.
Methods We included 37 patients who switched from kanamycin sulfate to rifaximin at our institution from January 2017 to December 2018. The onset of hepatic encephalopathy and changes in blood ammonia values during a six-month period were retrospectively evaluated.
Results There were 4 (11%) patients with hepatic encephalopathy at the time of switching from kanamycin sulfate to rifaximin. The cumulative incidence of hepatic encephalopathy was 3% and 16% at 3 and 6 months later, respectively. The blood ammonia levels at the time of switching to rifaximin and at 3 and 6 months later were 94 (range, 20-243) μg/dL, 95 (range, 33-176) μg/dL, and 81 (range, 32-209) μg/dL, respectively, and no significant changes were observed. However, in the 11 patients receiving an oral dose of <1,500 mg/day of kanamycin sulfate, the blood ammonia levels at the time of switching and at 3 and 6 months later were 136 (range, 35-243) μg/dL, 95 (range, 33-150) μg/dL, and 63 (range, 43-124) μg/dL, respectively. Furthermore, the blood ammonia levels significantly decreased at the time of the switching to rifaximin and at three and six months later (p=0.043 and p=0.011, respectively).
Conclusion Switching to rifaximin in hepatic cirrhosis patients receiving kanamycin sulfate to treat hepatic encephalopathy and hyperammonemia showed effects that were equivalent to or greater than the original therapy, thereby demonstrating the clinical efficacy.
Objective Current clinical guidelines have proposed heart failure (HF) with mid-range ejection fraction (HFmrEF), defined as a left ventricular ejection fraction (LVEF) of 40-49%, but the proportion and prognosis of patients transitioning toward HF with a reduced LVEF (LVEF <40%, HFrEF) or HF with a preserved LVEF (LVEF ≥50%, HFpEF) are not fully clear. The present study prospectively evaluated the changes in the LVEF one year after discharge and the outcomes of hospitalized patients with HFmrEF.
Methods We prospectively studied 259 hospitalized patients with HFmrEF who were discharged alive at our institutions between 2015 and 2019. Among them, 202 patients with HFmrEF who underwent echocardiography at the one-year follow-up were included in this study. Patient characteristics, echocardiographic data and all-cause death were collected.
Results Eighty-seven (43%) patients transitioned to HFpEF (improved group), and 35 (17%) transitioned to HFrEF (worsened group). During a median follow-up of 33 months, 27 (13%) patients died. After adjustment, patients in the worsened group had an increased risk of all-cause mortality compared with those in the improved group [hazard ratio 7.02, 95% confidence interval (CI) 1.13-43.48]. The baseline LVEF (per 1% decrease) and tricuspid annular plane systolic excursion (per 1 mm decrease) were independent predictors of the worsened LVEF category (odds ratio 2.13, 95% CI 1.25-3.63 and odds ratio 1.31, 95% CI 1.01-1.70, respectively).
Conclusion Our study showed that a worsened LVEF one year after discharge was associated with a poor prognosis in hospitalized patients with HFmrEF.
Objective The coronavirus disease 2019 (COVID-19) pandemic has resulted in a shortage of medical resources, including ventilators, personal protective equipment, medical staff, and hospital beds. We investigated the impact of COVID-19 in amyotrophic lateral sclerosis (ALS) patients, their families, caregivers, and medical experts.
Methods We conducted a nationwide ALS webinar about COVID-19 in May 2020 and sent a questionnaire to those enrolled.
Results A total of 135 participants (31 ALS patients; 23 families and caregivers of ALS patients; 81 medical experts) responded to this cross-sectional self-report questionnaire. The results showed that tracheostomy and invasive ventilation (TIV) was used in 22.6% of ALS patients, whereas 77.4% of ALS patients were not under TIV. Among non-TIV patients (n=24), 79.2% did not want TIV in the future. However, 47.4% of non-TIV patients not wanting a tracheostomy in advanced stages replied that they would want an emergency tracheostomy if they developed COVID-19-related pneumonia. These results suggest that ALS patients may be receptive to emergency treatments for reasons other than ALS. In addition, approximately half of the ALS patients agreed with the policy of not ventilating the elderly or ALS patients in case of a ventilator shortage. Furthermore, compared with medical experts, few ALS patients reported that the chance for ALS patients to obtain work was higher due to the increasing availability of remote work.
Conclusions This survey indicates that the COVID-19 pandemic might be associated with increased distress about access to care and work, inducing contradictory responses and potential hopelessness among ALS patients.
Duodenal stenting has gradually been established as the first-line treatment for malignant gastric outlet obstruction (GOO). We encountered a case of duodenal stent fracture in a 76-year-old woman with gastric cancer and GOO. She underwent self-expandable metallic stent (SEMS) placement. The SEMS was found to be fractured 4 weeks after its placement. We removed the broken part of the stent and placed a second SEMS. SEMS fracture is a rare and - to the best of our knowledge - unreported complication; hence, clinicians and their patients should be aware of this possibility.
A 72-year-old man without any symptoms was referred to our hospital. Esophagogastroduodenoscopy revealed an elevated esophageal lesion that was covered with normal mucosa. The examination of biopsy specimens from the lesion revealed amyloid light-chain (AL) (λ) type amyloid deposits, but there were no amyloid deposits elsewhere in the gastrointestinal tract. Further examinations did not indicate systemic amyloidosis. Thus, this case was diagnosed as a localized esophageal amyloidosis. As the clinical outcome of localized amyloidosis is favorable, this case was scheduled for close follow-up. Localized amyloidosis should be considered in the differential diagnosis of esophageal submucosal tumors.
A 79-year-old man with lymphoma who tested negative for anti-hepatitis C virus (HCV) antibody received rituximab-containing chemotherapy. Liver dysfunction of unknown cause had persisted since the second cycle of chemotherapy. Ten months after treatment, he rapidly developed massive ascites and atrophy of the liver, and we detected HCV RNA in his serum using real time polymerase chain reaction. Furthermore, medical interviews showed that the patient had no episodes for acute HCV infection, but he did have a history of unspecified liver dysfunction. These findings support the possibility of the reactivation of seronegative occult HCV infection due to chemotherapy in a cancer patient.
Hereditary hemorrhagic telangiectasia (HHT) is a rare disorder characterized by telangiectasias and arteriovenous malformations (AVMs), which can involve multiple organ systems. Although hepatic involvement is common, the development of portosystemic encephalopathy is extremely rare. We herein report a 72-year-old woman with HHT-induced portosystemic encephalopathy secondary to hepatic arteriovenous malformations. She presented with disturbance of consciousness, and her serum ammonia level was elevated at 270 mg/dL. Color Doppler ultrasonography and contrast-enhanced computed tomography showed hepatic AVMs and shunts, which were useful for making the definite diagnosis. Portosystemic encephalopathy should be considered as a differential diagnosis in HHT patients presenting with disturbance of consciousness.
We experienced a case of acute multiple organ ischemia and multiple organ failure due to atypical aortic coarctation (AAC). Since the patient's hemodynamics were too unstable to perform surgical revascularization, we performed urgent endovascular therapy (EVT) with a stent. Eventually, the patient achieved remission from multiple organ failure and a satisfactory clinical outcome. We feel that EVT for AAC is a sufficiently effective treatment option if the purpose of EVT is to save a patient's life in the acute phase. In the present case, spontaneous retroperitoneal bleeding (SRB) occurred after EVT of AAC, but this is a rare incident, although noteworthy in the clinical course.
A 71-year-old man complained of nausea and loss of appetite for eight months prior to admission. He was transported to a hospital with disorientation and diagnosed with primary hyperparathyroidism by laboratory examinations. However, ultrasonography, computed tomography, and technetium-99m labeled methoxyisobutyl isonitrile (99mTc-MIBI) with single-photon emission computed tomography did not yield definite results. In contrast, somatostatin receptor scintigraphy successfully identified the lesion responsible for the over-secretion of parathyroid hormone within the middle mediastinum. The tumor was successfully resected by surgery, and a histopathological analysis confirmed the parathyroid adenoma nature of the tumor.
Fabry disease (FD) is an X-linked lysosomal storage disorder resulting from a lack of alpha-galactosidase A (AGALA) activity in lysosomes. We herein report a patient with FD revealed by a renal biopsy who survived seven years after the introduction of peritoneal dialysis despite having severe heart failure due to left ventricular hypertrophy (LVH). FD was diagnosed based on a renal biopsy and biochemical analysis showing a low enzymatic activity of AGALA. A microscopic examination at the autopsy revealed marked hypertrophy and vacuolation of cardiac muscle cells. In our case, cardiac involvement determined the prognosis. Peritoneal dialysis is the modality of choice in the long-term management of dialysis patients with FD.
The eligibility for kidney donation and long-term post-donation renal prognosis of patients with Gitelman syndrome (GS) are unknown. We herein report a 44-year-old woman with GS who donated her kidney for transplant. A gene sequence analysis revealed compound heterozygous mutations of T180K and L858H in the SLC12A3 gene. Since transplantation, the renal function and serum potassium and magnesium levels of the donor and recipient have remained stable for seven years with careful monitoring and supplementation. Patients with asymptomatic GS who have no complications can be considered eligible to donate their kidney for transplant with proper monitoring after transplantation.
Drug-induced lung injury is defined as a respiratory disorder. The usefulness of the basophil activation test (BAT) for drug allergy-related cases was recently reported. The patient was an 82-year-old woman who had been taking Daisaikoto and Yokukansan (herbal medicines) 3 months before developing dry cough. She was admitted to our hospital with an initial diagnosis of pneumonia with elevated serum LDH, KL-6, and IgE. Chest CT showed bilateral ground-glass opacities. Her bronchoalveolar lavage fluid showed increased eosinophils. Finally, a BAT was positive for both medications. Based on the findings, the patient was diagnosed with Daisaikoto- and Yokukansan-induced lung injury. The current case suggests that the BAT may be useful for the diagnosis of drug-induced lung injury.
Paraneoplastic neurological syndromes (PNS), such as sensory polyneuropathy, are rare, and serum neuronal antibodies that are used for diagnosing this syndrome are occasionally positive. Similarly, neurological immune-related adverse events due to immune checkpoint inhibitors (ICIs) are also rare. However, their etiologies and the relationship between them remain unclear. We herein report a patient with suspected small cell lung cancer who showed sensory polyneuropathy after treatment with atezolizumab in combination with cytotoxic agents (carboplatin and etoposide) and was doubly positive for serum anti-Hu and anti-SOX-1 antibodies. Treatment with ICI and cytotoxic agents may sometimes lead to the development of PNS.
Secondary immune thrombocytopenic purpura (ITP) with non-Hodgkin lymphoma (NHL) is a rare disease. Although some treatment regimens are available for primary ITP, the treatment strategy for secondary ITP remains unconfirmed. We herein report a 79-year-old man who was diagnosed with secondary ITP with mantle cell lymphoma. Although intravenous immunoglobulin (IVIG) has been considered an effective option for secondary ITP, similar to the treatment of primary ITP, our patient did not benefit from IVIG. A literature review including the current report revealed that IVIG was ineffective in all treated patients. Secondary ITP with NHL should be treated differently from primary ITP.
TAFRO syndrome and POEMS syndrome are lymphoproliferative disorders with elevated interleukin-6 and vascular endothelial growth factor (VEGF) levels; however, their underlying pathogenic mechanisms remain unclear. Similarities have been reported in the pathological findings of the lymph nodes of TAFRO syndrome, Multicentric Castleman disease (MCD), and some cases of POEMS syndrome. However, there is no consensus on the relationship among them. We encountered a case of lymphoproliferative disorder that presented with manifestations of both TAFRO syndrome and POEMS syndrome. This case may be a subtype of idiopathic MCD and will be very important for establishing the disease concept of TAFRO syndrome and POEMS syndrome.
Mild encephalitis/encephalopathy with reversible splenial lesion (MERS) has not been described in lymphoma patients. A 65-year-old man with refractory mantle cell lymphoma (MCL) presented typical MRI features of MERS. The patient's cerebrospinal fluid contained an increased number of reactive T-cells; a small number of MCL cells were detected by immunoglobulin heavy chain-polymerase chain reaction (IGH-PCR). His symptoms and the splenial lesion resolved in response to ibrutinib treatment, although the patient eventually died of progressive MCL with overt leptomeningeal disease. We suggest that central nervous system involvement in MCL can present clinicoradiological features of MERS and that ibrutinib could be a choice of treatment.
Cronkhite-Canada syndrome (CCS) is a rare polyposis disorder accompanied by alopecia and onychodystrophy. A 63-year-old man with a history of CCS and repeated embolism developed progressive thrombocytopenia and mild anemia. Laboratory testing, a bone marrow examination, and magnetic resonance imaging of the spine resulted in a diagnosis of concurrent aplastic anemia (AA). Paroxysmal nocturnal hemoglobinuria (PNH)-type cells were detected in a peripheral blood specimen. In addition, human leukocyte antigen (HLA) included DRB1*15:01 and DRB1*15:02. Mesalazine was discontinued in consideration of possible drug-induced pancytopenia. Immunosuppressive therapy ameliorated both the gastrointestinal symptoms of CCS and pancytopenia. A common autoimmune abnormality might underlie both CCS and AA.
A 69-year-old man was admitted to our hospital for progressive muscle weakness in both lower limbs and limb ataxia (day 0). Nerve conduction studies showed low compound muscle action potential amplitudes at rest and increased amplitudes after maximum voluntary contraction. Blood testing revealed SOX-1 antibodies. He was diagnosed with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome (PCD-LEMS). He died from aspiration pneumonia on day 9. Small-cell lung carcinoma (SCLC), which had not been obvious on computed tomography, was found during the autopsy. Patients with PCD-LEMS who test positive for SOX-1 antibodies should be carefully evaluated for SCLC.
Charcot-Marie-Tooth disease type 1A (CMT1A) is a hereditary peripheral neuropathy, and its involvement in the central nervous system (CNS) is very rare. We herein report a 51-year-old woman with CMT1A who suffered from recurrent optic neuritis and myelopathy. Under the diagnosis of anti-aquaporin-4 (anti-AQP4) antibody positive neuromyelitis optica spectrum disorder (NMOSD), we treated her successfully with corticosteroids. This is the first report of CMT1A complicated with anti-AQP4-positive NMOSD. Although the coexistence of the two disorders may simply be a coincidence, we speculated that immune cross-reaction between overexpressed peripheral myelin protein 22 and CNS myelin may have caused concomitant CMT1A and NMOSD.
Tocilizumab (TCZ), a biologic that blocks the signal transduction of interleukin-6, has been used for the treatment of various autoimmune diseases. Many of these cases are sometimes complicated by ulcerative colitis (UC). However, the effect of TCZ on UC is unclear. We experienced two cases with concomitant UC that were treated with TCZ, one for Takayasu arteritis (TAK) and the other for relapsing polychondritis (RP). TCZ did not improve UC in either of these cases. TCZ might have adverse effects on the intestinal tract, since interleukin-6 signaling plays an important role in intestinal epithelium maintenance. Treatment with TCZ should therefore be carefully provided in patients complicated with UC.
Aggregatibacter actinomycetemcomitans, an etiological agent associated with periodontitis, endocarditis, and other infections, has rarely been implicated in spondylitis. A 70-year-old man with aortic valve replacement presented with a 4-month history of lower back pain and was diagnosed with spondylitis. Prolonged incubation of blood cultures and a biopsy yielded A. actinomycetemcomitans. Concurrent infective endocarditis (IE) was probable considering the infectious organism and the patients' prosthetic valve. The patient was treated with ceftriaxone and recovered well. Pyogenic spondylitis with possible concurrent IE may be caused by A. actinomycetemcomitans. Extended incubation and repeated cultures should be considered if Haemophilus spp., Aggregatibacter spp, Cardiobacterium spp, Eikenella spp, and Kingella spp. (HACEK) infection is suspected.
A 41-year-old woman with systemic lupus erythematosus (SLE) was admitted to our hospital due to a fever at 35 weeks of pregnancy. Laboratory testing revealed a low platelet count and elevated liver enzymes. Emergency Caesarean section was performed due to the risk of SLE exacerbation or hemolytic anemia, elevated liver enzyme, and low platelet count syndrome. Based on the blood culture results, the patient was diagnosed with Listeria monocytogenes bacteremia. She was treated with ampicillin and eventually recovered, and the neonate did not have any complications. Pregnant women with SLE are at risk of complications. Listeriosis should be monitored for and, if found, managed appropriately.
Most cases of mediastinal abscess occur as a postoperative complication of a thoracic surgical procedure or following trauma. The most common causative microorganism is Staphylococcus aureus, but it can be rarely caused by unusual microorganisms, such as Gemella species. These are relatively difficult-to-identify commensal microorganisms of the upper respiratory and gastrointestinal tracts and may cause several infections. A 66-year-old man was diagnosed with Gemella bergeri mediastinal abscess by the molecular detection of bacterial genes. He was successfully treated with penicillin antibiotic for eight weeks. To our knowledge, this is the first case report of mediastinal abscess caused by G. bergeri.
A married couple of a 62-year-old woman and a 64-year-old man as well as their neighbor, an 84-year-old woman, visited the hospital complaining of a burning sensation on their hands and feet that had presented on the same day. They had consumed mushrooms that had been picked on a mountain five days before the onset of the symptoms. The symptoms were attributed to Paralepistopsis acromelalga. In conclusion, asking about the dietary history is considered essential when diagnosing the cause of erythromelalgia, which has multiple causative diseases, including food poisoning due to P. acromelalga.
Acute appendicitis is an important differential diagnosis in patients with right lower quadrant pain during pregnancy. Endometriosis, a hormone-related pathology, is another possibility. Patients with endometriosis are typically symptomatic before pregnancy. Stromal endometriosis is a variant of endometriosis that presents no symptoms before pregnancy but which occasionally presents with the new onset of symptoms during pregnancy. We report the case of a 35-year-old woman in her 8th month of pregnancy who presented with impending appendiceal rupture due to deciduosis of the appendix, a progesterone-related condition, during pregnancy. This case suggests that deciduosis/stromal endometriosis should be considered as a differential diagnosis of acute abdomen during pregnancy, even if the patient is asymptomatic before pregnancy.