Objective The rate of platelet count reduction appears to differ among different liver diseases. In the present study, we investigated the difference in the platelet counts of patients with nonalcoholic fatty liver disease (NAFLD) and those with chronic liver disease due to hepatitis C virus (CLD-HCV).
Methods The study population included 620 patients with NAFLD and 405 patients with CLD-HCV, all of whom were diagnosed by liver biopsy. The relationships between the grade of fibrosis and the platelet count in the two diseases were compared. The optimal cut-off value for the diagnosis of liver cirrhosis (LC) was measured. The relationships between the platelet count and anti-platelet antibodies, the serum thrombopoietin level, the grade of splenomegaly and liver stiffness were also investigated in both LC groups.
Results In NAFLD patients, the platelet count was significantly higher at each grade of fibrosis in comparison to CLD-HCV. The optimal cut-off value for the diagnosis of LC was 16.0×104/μL [sensitivity, 86.7%; specificity, 87.6%; area under the curve (AUC), 0.930] in NAFLD and 12.7×104/μL (sensitivity, 57.8%; specificity, 88.2%; AUC, 0.863) in CLD-HCV. No anti-platelet antibodies were detected in patients with either type of LC. The serum thrombopoietin levels, the distribution of splenomegaly grades, and liver stiffness did not differ between the two LC groups to a statistically significant extent. As the splenomegaly grade increased, the platelet count decreased.
Conclusion The optimal cut-off values for diagnosing LC differed between the two diseases and should be determined separately. The reason for the difference in platelet reduction is still unclear, and requires further investigation.
Objective In 2010, candid advice concerning the low rate of typhoid vaccination among Japanese travelers was received from Nepal. Recently, progressive Japanese travel clinics have encouraged Japanese travelers to be vaccinated against typhoid fever in conjunction with officially approved vaccines, such as hepatitis A vaccine. We herein report the status of typhoid vaccinations for Japanese travelers to the most endemic area (South Asia) and describe the factors associated with compliance.
Methods In the travel clinic at Kurume University Hospital, we used the following criteria to retrospectively extract the records of new pre-travel Japanese clients between January 2011 and March 2015: hepatitis A vaccine administered, traveling to South Asian countries, and ≥2 years of age. We first summarized the participants and then divided them into typhoid-vaccinated and typhoid non-vaccinated groups for a comparative analysis.
Results This study included 160 clients. A majority (70.0%) of these clients traveled for business. The duration of trips was long (≥1 month) (75.0%), and India was a popular destination (90.6%). A comparative study between the vaccinated group (n=122) and the non-vaccinated group (n=38) revealed that the two factors most positively associated with typhoid vaccination were business trips [adjusted odds ratio (aOR) 3.59, 95% confidence interval (CI) 1.42-9.06] and coverage by a company/organization payment plan (aOR 7.14, 95% CI 2.67-20.3).
Conclusion The trend toward typhoid vaccination among Japanese travelers to South Asia with pre-travel consultation is correlated with business trips and coverage by a company/organization payment plan. If problems concerning the cost of vaccines were resolved, more travelers would request typhoid vaccination.
Objective Mamushi (Gloydius blomhoffii) snakebite is the most common type of snake injury in Japan and is also seen in China and Korea. Although the components of Mamushi venom have been investigated, epidemiological and clinical descriptions still remain limited in the English literature. The aim of this study was to review the clinical features and management of patients with injuries related to Mamushi snakebites.
Methods We conducted a retrospective examination of 114 Mamushi snakebite cases encountered at a general hospital in Japan from January 2004 to November 2016. Data were collected from the medical records.
Results We found that Mamushi snakebites commonly occurred during summer and the daytime, with elderly men typically being affected. The symptom grade at initial consultation was significantly worse in the walk-in group than in the ambulance admission group, probably due to treatment delay. The number of fangs that pierced the skin was not related to the severity of the symptoms. The group treated with a tourniquet more frequently exhibited exacerbation of symptoms than those that received other treatments (p<0.001).
Conclusion The delay between patients being bitten and arriving at hospital as well as the number of fangs that pierced the skin did not affect the duration of hospitalization; however, proximal tourniquation should be avoided in such cases, as significant exacerbation of local symptoms was observed when this procedure was applied.
Mucosa-associated lymphoid tissue (MALT) lymphoma and reactive inflammatory lymphoid changes are frequently difficult to distinguish based on a routine histological differential diagnosis. We were unable to diagnose gastric MALT lymphoma histologically using specimens obtained by endoscopy, although a flow cytometry (FCM) analysis demonstrated clonality of neoplastic cells by separating cells by CD45 gating. Furthermore, a fluorescence in situ hybridization (FISH) analysis showed trisomy 18. We therefore diagnosed gastric MALT lymphoma with trisomy 18. We recommend that FCM and FISH analyses of biopsy specimens be considered for diagnosing gastric MALT lymphoma if this diagnosis is suspected based on endoscopic findings.
Glycogenic hepatopathy (GH) is a rare complication of poorly controlled type 1 diabetes mellitus (T1DM), and is characterized by elevated liver enzymes, hepatomegaly, and glycogen accumulation. We herein present the case of a 23-year-old man with poorly controlled T1DM who had liver dysfunction. Imaging studies showed severe hepatomegaly and fatty liver. The examination of a liver biopsy specimen revealed fatty droplets, ballooning, inflammation, and mild fibrosis. Subsequent periodic acid-Schiff (PAS) staining after diastase digestion confirmed GH. In this case, the improvement of hyperglycemia, not HbA1c, resulted in the improvement of the patient's liver function. This is the first report on the use of continuous glucose monitoring in patients with GH to show that continuous hyperglycemia may worsen GH.
We herein report the case of a 74-year-old man who underwent surgery 9 years after his initial visit and who was pathologically diagnosed with signet-ring cell carcinoma (SRCC) derived from a main-duct-type intraductal papillary mucinous neoplasm (MD-IPMN). At the first imaging examination, only a small pancreatic cyst with mild dilation of the main pancreatic duct (MPD) was detected in the pancreatic head. Eventually, MD-IPMN with mural nodules and MPD dilation (30 mm) developed in the pancreatic body, while the pancreatic head cyst remained unchanged. Total pancreatectomy was performed and the MD-IPMN was pathologically diagnosed as SRCC derived from an intestinal-type MD-IPMN.
Combination therapy with ledipasvir and sofosbuvir (LDV/SOF), direct-acting antiviral agents, is highly effective against hepatitis C virus genotype 1 infection. Although LDV/SOF is safer than conventional treatment, reports have indicated that LDV/SOF was discontinued in certain cases due to severe skin disorders. A 68-year-old woman presented with a rash after starting LDV/SOF treatment. We interrupted LDV/SOF and began the oral administration of prednisolone (PSL). After the rash improved, we re-started LDV/SOF with PSL. After treatment, the rash clearly improved; we checked for a sustained virologic response 12 weeks after treatment. Steroids may therefore be an effective treatment option for controlling the side effects of LDV/SOF.
A 75-year old man with a history of inferior myocardial infarction was admitted with symptoms of progressive heart failure 3 months after undergoing transapical transcatheter aortic valve implantation (TAVI). Echocardiography revealed severe mitral regurgitation (MR) caused by posterior leaflet tethering, without traumatic injury of the mitral valve or chordae. The patient was successfully treated by percutaneous edge-to-edge mitral valve repair (MitraClip®). This case highlights the role of MitraClip® in high-risk patients suffering from MR, and suggests that apical contractile loss or adhesion caused by apical puncture and suturing in transapical TAVI may be one of the mechanisms of worsening MR.
Even in modern clinical cardiology, basic auscultation skill is not obsolete and is still important because it can always provide a clue to an underlying pathophysiology. We demonstrate an unusual mechanism of pathological wide splitting of the second heart sound due to external compression of the pulmonary trunk in a patient with a giant coronary arterial aneurysm of the proximal left anterior descending artery. Echocardiography, when combined with a three-dimensional anatomical analysis with cardiac computed tomography, was useful for elucidating the mechanism of the abnormal heart sounds.
Transcatheter aortic valve implantation (TAVI) has been validated as a reliable therapy for aortic stenosis (AS), similar to surgical aortic valve replacement. Due to the methodological differences between the two therapeutic options, each has unique complications. We experienced a hitherto unreported complication of TAVI. An 81-year-old man underwent TAVI for severe AS. Acute mitral regurgitation (MR) occurred during valve deployment. Interference of the guidewire with the mitral subvalvular structure caused transient severe MR, leading to the development of pulmonary hemorrhaging. During TAVI, careful attention should be paid to the position of the guidewire, changes in hemodynamics and degree of MR.
A 63-year-old man was hospitalized due to an abdominal pulsatile mass. Computed tomography revealed a saccular type abdominal aortic aneurysm, the diameter of which was 52 mm. A physical examination revealed prominent Achilles tendon thickness and plantar xanthomas. He was born in a family of consanguineous marriage, where his parents were second cousins. He had no familial history of high low-density lipoprotein cholesterol, tendon xanthomas, or premature atherosclerosis. Whole-exome sequencing assuming recessive inheritance determined his genetic diagnosis to be cerebrotendinous xanthomatosis caused by homozygous mutations (c.410G>A or p.Arg137Gln) in the cytochrome P450 subfamily 27 A1 (CYP27A1) gene.
Nephrotic syndrome can be caused by various diseases, from primary kidney diseases to systemic diseases. A kidney biopsy is useful for confirming the causes of nephrotic syndrome and in its management. We herein describe a case of nephrotic syndrome with thrombocytopenia, lymphadenopathy, systemic inflammation, splenomegaly, kidney enlargement, and progressive renal insufficiency. A kidney biopsy showed endothelial swelling with mild interstitial fibrosis and tubular atrophy. This case met the diagnostic criteria for TAFRO syndrome. Little is known about TAFRO syndrome, especially in relation to the associated kidney pathophysiology. The accumulation of a greater number of cases in which the kidney biopsy findings are investigated is needed to clarify the pathogenesis of kidney involvement in this condition.
We herein report a rare case of mucinous tubular and spindle cell carcinoma (MTSCC) in an 80-year-old woman. A well circumscribed tumor located on the right kidney was discovered incidentally as a result of screening non-contrast CT. Fluorodeoxyglucose positron emission tomography (FDG PET)/CT showed the increased tracer accumulation in the tumor. The histological diagnosis was MTSCC, which is a rare and only recently established subtype of the malignant renal cell carcinoma (RCC). The present case suggests the clinical benefit of a high uptake of FDG combined with enhanced contrast CT in the differentiation of MTSCCs and other RCCs.
A 67-year-old man presented with a fever and general malaise. Computed tomography showed multiple nodules in the lungs and liver, associated with mediastinal and para-aortic lymphadenopathy. Bone marrow aspiration revealed diffuse large B-cell lymphoma (DLBCL). Renal and liver dysfunction and pancytopenia inhibited chemotherapy administration; the patient subsequently died of multiorgan failure. An autopsy revealed pulmonary adenocarcinoma with metastases to the lungs, liver, and adrenal glands; the DLBCL spread to the liver, spleen, and bone marrow. Adenocarcinoma and DLBCL collision was observed in the mediastinal and para-aortic lymph nodes. This was a rare case of collision metastasis occurring in the lymph node.
Salmonella spp. are food-borne pathogens that usually cause gastroenteritis, although bacteremia and subsequent focal metastatic infection can also occasionally occur. Of the known Salmonella spp., Salmonella houtenae is a rare subspecies, comprising less than 1% of all Salmonella strains. We herein report the first case of S. houtenae-induced empyema complicated with chronic tuberculous empyema, which was successfully treated by antibacterial agents alone. We wish to highlight the importance of being aware that Salmonella spp. can cause empyema in cases suffering from chronic tuberculous empyema; moreover, despite the successful completion of treatment with antibacterial agents, periodical follow-up is mandatory in such cases.
A 59-year-old man with an 18-year history of rheumatoid arthritis who had been treated with steroids, methotrexate, and infliximab presented with a high-grade fever, cervical lymphadenopathy, and hepatosplenomegaly. Epstein-Barr virus (EBV) hepatitis was diagnosed based on the liver histology and EBV antibody titer. The symptoms improved temporarily, but five months later, the fever, skin rash, jaundice, and thrombocytopenia relapsed. Bone marrow and liver biopsies demonstrated infiltration with Reed-Sternberg cells. Based on these findings, the patient was diagnosed with other iatrogenic immunodeficiency-associated lymphoproliferative disorder (OIIA-LPD), Hodgkin lymphoma type. This case followed a rare clinical course, in that acute hepatitis preceded the diagnosis of OIIA-LPD.
Rituximab is a highly effective agent that is used in the treatment of B-cell lymphoma. Rituximab-induced acute thrombocytopenia is a rare side effect that has previously been reported in a small number of patients with malignant lymphoma; its mechanism is still unknown. We herein report the case of a 74-year old man who was diagnosed with follicular lymphoma and who developed severe acute thrombocytopenia the day after the administration of rituximab. Coagulation abnormality, which mimicked disseminated intravascular coagulation, also appeared. When physicians use rituximab to treat high-risk patients, the platelet count should be closely monitored to avoid possible adverse events.
Isolated sarcoma with features of mixed-phenotype acute leukemia (MPAL) is an extremely rare disease and it can be easily misdiagnosed as lymphoma or other malignancies. We herein report the case of a 61-year-old woman with non-leukemic sarcoma of the right pleura, pretracheal lymph node, and supraclavicular lymph node with features of MPAL, B/myeloid, not otherwise specified, which was first misdiagnosed as diffuse large B cell lymphoma. After performing a detailed re-examination of the biopsy specimens, few scattered eosinophilic myelocytes allowed us to reach a correct diagnosis of MPAL and the patient was thereafter successfully treated by intensified chemotherapy followed by cord blood transplantation.
A small cortical infarction confined to the medial location of the precentral knob on the precentral gyrus may cause isolated shoulder palsy. However, there are no reports indicating intracerebral hemorrhage as a cause of isolated shoulder palsy. We herein report the case of a 48-year-old man who presented with isolated shoulder palsy as an initial symptom of subcortical hemorrhage at the precentral gyrus. Such cases may be easily misdiagnosed as shoulder or cervical spine problems. The distribution of muscle weakness is the key to an accurate diagnosis.
We herein report a case of dural arteriovenous fistula (DAVF) at the cavernous sinus that was diagnosed by arterial spin-labeled imaging (ASL). A 67-year-old woman was referred to our hospital due to double vision and bilateral conjunctival injection. Conventional magnetic resonance imaging findings were normal. However, abnormal hyperintense signals on ASL were detected. Furthermore, the abnormality disappeared after successful endovascular embolization. Although conventional digital subtraction angiography is the standard tool for diagnosing DAVF, we speculated that ASL might be useful to this end as well.
Central nervous system (CNS) involvement in granulomatosis with polyangiitis (GPA), including pachymeningitis and CNS vasculitis, is uncommon. Although intracerebral hemorrhage (ICH) has been reported in GPA, simultaneous multiple ICH (SMICH) is rare. We describe the case of a 50-year-old woman with a history of a limited form of GPA with chronic pachymeningitis who presented with acute-onset headache accompanied by nausea and vomiting, and who developed consciousness impairment. Computed tomography revealed bilateral subcortical ICH. Sinus thrombosis was not apparent on angiography. The patient was treated with high-dose corticosteroid therapy. The cause of the steroid-responsive SMICH in this case was unknown, but it might have been CNS vasculitis. Patients with GPA may present with SMICH, which is considered an indication for immunosuppressive therapy.
Nivolumab shows promising efficacy against metastatic melanoma. However, immune-related adverse events are of great concern. We herein report a case of persistent colitis that developed during nivolumab monotherapy and nivolumab readministration. An 82-year-old Japanese woman with recurrent melanoma developed Grade 3 colitis after 6 cycles of nivolumab. She was treated with corticosteroid for 28 days. Follow-up by computed tomography and colonoscopy after corticosteroid treatment revealed persistent pancolitis. Her symptoms ameliorated spontaneously in two months. Given the amelioration, nivolumab was restarted and resulted in the maintenance of stable disease for 21 months without recurrence of colitis. Even in cases of persistent colitis over several months, nivolumab readministration should be considered.