The forced oscillation technique (FOT) is a noninvasive method with which to measure respiratory system resistance and reactance during tidal breathing. Recently, its clinical application has spread worldwide with the expansion of commercially available broadband frequency FOT devices, including MostGraph and Impulse Oscillometry. An increasing number of reports have supported the usefulness of the FOT in the management of asthma and chronic obstructive pulmonary disease (COPD). However, the FOT is not a surrogate test for spirometry, but should be used complementarily. Furthermore, reference values are not necessarily available and the interpretation of some measured data is controversial. There is a need to update the international statement for not only technical aspects but also the clinical use of the FOT. In this review, we summarize the previously published studies and discuss how to use the FOT in a clinical setting.
We mainly refer to the acute setting of meningococcemia. Meningococcemia is an infection caused by Neisseria meningitidis, which has 13 clinically significant serogroups that are distinguishable by the structure of their capsular polysaccharides. N. meningitidis, also called meningococcus, is a Gram-negative, aerobic, diplococcus bacterium. The various consequences of severe meningococcal sepsis include hypotension, disseminated intravascular coagulation (DIC), multiple organ failure, and osteonecrosis due to DIC. The gold standard for the identification of meningococcal infection is the bacteriologic isolation of N. meningitidis from body fluids such as blood, cerebrospinal fluid (CSF), synovial fluid, and pleural fluid. Blood, CSF, and skin biopsy cultures are used for diagnosis. Meningococcal infection is a medical emergency that requires antibiotic therapy and intensive supportive care. Management of the systemic circulation, respiration, and intracranial pressure is vital for improving the prognosis, which has dramatically improved since the wide availability of antibiotics. This review of the literature provides an overview of current concepts on meningococcemia due to N. meningitidis infection.
Objective Mesenteric phlebosclerosis (MP) is a disease characterized by calcification of the mesenteric vein, which causes chronic mesenteric ischemia. Recently, the long-term intake of gardenia fruit ('Sanshishi' in Japanese) has been attracting attention as a possible cause. Usually, only advanced, severe MP cases get reported. However, we suspected that some latent cases of this disease may exist. We performed this study in order to determine the prediagnostic cases at our outpatient departments of herbal (Kampo) medicine, with particular attention paid to the initial changes, such as any slight color change of the colon, as shown in colonoscopy. Methods We recommend colonoscopy and computed tomography (CT) scans for patients with a long-term history of taking herbal medicines containing gardenia fruit. Clinical examinations were performed upon receiving patients' consent from December 2013 to November 2014. Results Of the 103 patients who took gardenia fruit long-term, 29 agreed to be checked for MP. 14 patients underwent colonoscopy. Four patients were confirmed to have MP due to the presence of fibrotic deposition of the colonic membrane on histological inspection. Twenty-one patients underwent abdominal CT screening. Characteristic calcification of the mesenteric vein was observed on CT scans in 2 patients. All 4 MP patients took Kampo formulas containing gardenia fruit for more than 6.8 years. The other patients did not develop MP, despite long-term gardenia fruit intake. Conclusion We detected the latent and undiagnosed MP cases. All diagnoses were made while paying careful attention to any slight changes in colonoscopy and CT scans.
Objective Steroid pulse therapy is used to relieve pancytopenias in our hospital and is effective in some patients. However, it is unclear which patients will benefit from such therapy. Thus, we retrospectively analyzed the clinical features of patients undergoing allogeneic hematopoietic stem cell transplantation who received steroid pulse therapy to facilitate recovery in their blood cell counts. Methods Between 2004 and 2012, 24 patients underwent steroid pulse therapy and the medical records of 17 of these evaluable patients (11 men, 6 women) were retrospectively reviewed. Bone marrow smears were assessed to calculate the proportion of hemophagocytic macrophages just prior to receiving pulse therapy. Results Steroid pulse therapy was started at a median of 15 days after transplantation (range, 10-28 days). The median white blood cell count was 0.02×103/μL (range, 0.01-0.4×103/μL). Eight patients responded to pulse therapy and subsequent engraftment was achieved in all responders. None of the patients who underwent cord blood transplantation responded to the pulse therapy. Among the non-responders, only two patients achieved engraftment and four of nine non-responders died within one month. When evaluating the efficacy of steroid pulse therapy according to the ferritin level and proportion of hemophagocytic macrophages among patients undergoing bone marrow or peripheral blood stem cell transplantation, both values were higher in responders than in non-responders. Conclusion We speculate that responders have a hemophagocytic syndrome which is responsive to steroid pulse therapy. Thus, our results imply that the use of ferritin levels in combination with the proportion of hemophagocytic macrophages may be useful for the early detection of potential hemophagocytic syndrome after hematopoietic stem cell transplantation.
Objective Antiphospholipid syndrome (APS) is a well-known complication of habitual abortion and/or thrombosis and is frequently associated with autoimmune diseases. Methods We retrospectively investigated the relationships between the presence of antiphospholipid antibodies (aPLs) and the incidence of thrombotic events (THEs) in 147 patients with various connective tissue diseases (CTD) suspected of having APS and 86 patients with idiopathic thrombocytopenic purpura (ITP). THEs were observed in 41 patients, including 14 cases of venous thrombosis, 21 cases of arterial thrombosis and eight cases of complications of pregnancy. Results The prevalence of THE was significantly high in the systemic lupus erythematosus (SLE) patients compared with the other CTD patients and ITP patients. The frequency of lupus anticoagulant (LA), anticardiolipin antibodies (aCL)-β2-glycoprotein (GPI) complex IgG and aPL was significantly high in the SLE patients compared with the ITP patients. Subsequently, the rate of development of THE was significantly high in the patients with aPLs. In particular, the incidence of THE was significantly high in the SLE or ITP patients with LA, aCL-β2GPI IgG or aPL. The optimal cut-off values for LA, aCL IgG and aCL-β2GPI complex IgG for the risk of THEs were higher in the SLE patients in comparison to the values obtained when using the kit provided by the manufacturer. Conclusion Although aPLs is frequently associated with SLE and is a causative factor for thrombosis, the optimal cut-off value for aPL for predicting the occurrence of THEs varies among different underlying diseases.
Objective To identify the pathogens responsible for neonatal sepsis in a high-volume women and children's hospital in Southwest China. Methods We retrospectively studied 133 neonates who were admitted to the West China Women and Children's Hospital between 2008 and 2012 for sepsis. The clinical characteristics of the patients were recorded, and the antibiotic sensitivities of the isolated bacteria were determined. Results All of the included patients had clinical symptoms of sepsis, and subsequent blood cultures confirmed the infection. Almost 80% of patients were infected with coagulase-negative staphylococci (52.8%), Escherichia coli (23.6%), Klebsiella pneumoniae (16.0%) or Staphylococcus aureus (7.5%). Neonates who were infected with gram-negative bacteria, particularly K. pneumoniae, had lower birth weights and were admitted to hospital within 24 hours of birth. Additionally, 87.5% of the isolated K. pneumoniae strains were resistant to third generation cephalosporins. Conclusion Coagulase-negative staphylococci were the most common pathogens found in neonatal sepsis. Moreover, neonatal sepsis caused by gram-negative bacteria was more often observed in newborns of low birth weight. The isolated strains of gram-negative bacteria were highly resistant to cephalosporins. This observation highlights the issue of antibiotic-resistant pathogens in the clinical setting, which poses an added risk to infants presenting with sepsis.
Objective To clarify the current situation concerning drug fever (DF) in Japan, we retrospectively analyzed patients undergoing infectious disease consultation at our institution. Methods Between April 2014 and May 2015, we extracted the records of DF patients from among 388 patients who had obtained infectious disease consultations in Kurume University Hospital. We reviewed their medical charts and summarized the characteristics of DF. Results This study included the records of 16 patients. Clinical signs (relative bradycardia, the duration of the drug administration before becoming febrile, and the interval between the discontinuation of a drug and the alleviation of a fever), and laboratory tests (varied white blood cell count, low level of C-reactive protein, and a mild elevation of transaminases) were compatible with those from previous reports. Among the drug-confirmed cases, five involved the use of glycopeptides (vancomycin: 3, teicoplanin: 2), which were considered to be uncommon causes, and the another five cases involved the use of β-lactams. In addition, the procalcitonin levels were either negative or low (≤0.25 ng/mL) in 10 of the 11 procalcitonin-measured cases. Conclusion Our findings demonstrated that glycopeptides, similar to β-lactams, may be the origin of DF. Furthermore, procalcitonin may be helpful in the diagnosis of DF, but only in combination with other detailed examinations.
A 35-year-old man was diagnosed to have gastric cancer by endoscopic and histological examinations. Staging laparoscopy detected peritoneal metastasis. Systemic chemotherapy was started, but the patient complained of severe headache. Subsequently, a lumbar puncture demonstrated adenocarcinoma cells in the spinal fluid, suggesting the occurrence of meningeal carcinomatosis (MC) from gastric cancer. MC occurs only rarely in patients with gastric cancer, but the prognosis is invariably poor. However, this patient nevertheless survived for 12 months after receiving intrathecal MTX/Ara-C together with systemic chemotherapy. Therefore, the early detection of meningeal irritation sign and intrathecal chemotherapy might greatly improve the prognosis of gastric cancer patients with MC.
Localized hepatic tuberculosis (LHTB) is difficult to diagnose preoperatively, and most cases of LHTB are diagnosed based on pathological findings. A relationship between imaging features and the pathological stage of hepatic tuberculosis (TB) has recently been reported, which could aid in the diagnosis of hepatic TB. We herein present a case study of a patient with LHTB diagnosed postoperatively who demonstrated imaging changes due to the progression of TB. An awareness of the presence of LHTB might have permitted a preoperative diagnosis. This is the first report of an LHTB patient who exhibited imaging changes during the course of the disease.
We herein describe a case of somatostatinoma coexisting with a gastrointestinal stromal tumor (GIST) in the duodenum of an 81-year-old woman with Von Recklinghausen's disease (VRD) and common bile duct stone who presented with diarrhea of three months in duration. Gastroduodenoscopy revealed an ulcer on the second part of the duodenum. A 2.1-cm enhancing tumor was observed to extend from the ulcer on an abdominal computed tomography scan. Subtotal stomach-preserving pancreaticoduodenectomy revealed a somatostatinoma on the papilla of the vater and duodenal GIST. There have been only eight reports on VRD associated with ampullary somatostatinoma and GIST. An awareness of this possibility in patients with gastrointestinal lesions is necessary for proper treatment and patient management.
Sorafenib has been approved to increase the survival in patients with advanced hepatocellular carcinoma. Acute pancreatitis is an uncommon complication of sorafenib treatment. Only a few cases of sorafenib-induced acute pancreatitis have been reported in the English literature. We herein present the case of a 56-year-old man with hepatocellular carcinoma treated with sorafenib at 200 mg once daily. After six days of treatment, he suffered epigastric pain. Laboratory tests showed markedly elevated serum amylase and lipase levels. Imaging studies demonstrated negative findings. Sorafenib-induced acute pancreatitis was diagnosed after reviewing his history. The sorafenib treatment was discontinued, and his symptoms were resolved seven days later. To date, this case had the shortest duration and the lowest dosage of sorafenib to have induced acute pancreatitis.
von Hippel-Lindau (VHL) disease, caused by germline mutations in the VHL gene, is a hereditary autosomal-dominant disorder which predisposes the individual to various malignant and benign tumors. VHL acts as a tumor suppressor, mainly through the negative regulation of hypoxia-inducible factors. Molecular-targeted drugs against vascular endothelial growth factor-signaling pathways, a target of hypoxia-inducible factors, have recently been introduced into clinical practice for the treatment of patients with sporadic renal cell carcinoma and pancreatic neuroendocrine tumors. However, whether such treatments are effective in patients with VHL disease remains to be elucidated. We herein report a Japanese patient with VHL disease who was successfully treated with sunitinib for approximately 5 years.
Primary cardiac lymphoma (PCL) only rarely occurs and it is defined as a lymphoma in which the bulk of the tumor is located within the heart and pericardium. A 53-year-old woman was referred due to dyspnea, and an electrocardiogram exhibited atrial fibrillation (AF). Echocardiography revealed no abnormal findings. Scintigraphy and a lymph node biopsy led to a diagnosis of PCL. After the start of chemotherapy, AF was converted to atrial tachycardia prior to sinus rhythm with a first-degree atrioventricular block, which was finally restored to a normal sinus rhythm. PCL is only rarely encountered, but it should be included in the differential diagnosis as a possible cause of AF, and such AF could be reversible if the patient can be treated in a timely manner.
Rathke's cleft cysts are known to cause hormone-related abnormalities. However, the natural history of this disorder is obscure, so it is rarely associated with acute adrenal insufficiency. We herein describe a case of Rathke's cleft cyst associated with acute adrenal insufficiency in a 27-year-old man. The patient experienced severe headaches due to acute adrenal insufficiency without changes in the size of the cyst. Glucocorticoid administration improved these symptoms, and the cyst spontaneously shrank before operation. This case led us to conclude that Rathke's cleft cysts should be considered in the differential diagnosis of patients who present with adrenal insufficiency, and that the cysts can be reduced by glucocorticoids.
A 65-year-old Japanese man presented to our hospital in June 2013 with a 6-day history of fever and fatigue, a 24-h history of thirst, and polyuria. His temperature was 37.8°C and he was alert. However, laboratory tests revealed severe hyperglycemia, undetectable C-peptide levels, and diabetic ketoacidosis. Serum antibody testing confirmed a Coxsackie virus A2 infection. A variety of viral infections are reported to be involved in the development of fulminant type 1 diabetes mellitus (FT1D). Our patient is the first reported case of FT1D associated with Coxsackie virus A2 infection and supports the etiological role of common viral infections in FT1D.
We herein report the case of an 18-year-old boy who developed nephrotic syndrome and hypertension after upper airway inflammation. Post-streptococcal acute glomerulonephritis was diagnosed on the basis of a high antistreptolysin O titer, hypocomplementemia, proteinuria, and microscopic hematuria. A renal biopsy was performed due to persistent proteinuria, and the pathological diagnosis was membranoproliferative glomerulonephritis (MPGN) type I. Glomeruli showed positive staining for nephritis-associated plasmin receptor (NAPlr), a nephritogenic group A streptococcal antigen, and plasmin activity was found in a similar distribution as NAPlr deposition. This rare case of streptococcal infection-related nephritis (SIRN) manifesting MPGN type I supports the histological diversity of SIRN.
Pulmonary tumor thrombotic microangiopathy (PTTM) is a fatal cancer-related pulmonary complication. It is generally caused by gastric adenocarcinoma, and several molecules produced by tumor cells are reported to play important roles in its pathogenesis. We herein report an autopsy case of PTTM caused by urothelial carcinoma. Vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF), and osteopontin were found to be expressed in both the primary tumor cells and metastatic cells in the PTTM lesions. These findings implicate the possible involvement of VEGF, PDGF, and osteopontin in the pathogenesis of PTTM caused by urothelial carcinoma.
A man in his mid-60's with idiopathic pulmonary fibrosis and hepatitis B-related liver cirrhosis developed exertional dyspnea and a dry cough lasting for three months. High-resolution computed tomography (HRCT) showed increasing bilateral ground-glass opacity superimposed on the usual interstitial pneumonia pattern. Six months after starting pirfenidone therapy, the partial pressure of arterial oxygen at rest increased from 81 to 101 torr, the predicted forced vital capacity (FVC) value increased from 75% to 94% and the ground-glass opacity on HRCT improved. The FVC value was subsequently maintained near or above baseline for 43 months. We concluded that our patient was a super-responder to pirfenidone therapy.
A 72-year-old man with salivary gland cancer and multiple pulmonary metastases suffering from intractable pneumothorax was transferred to our institution; he was inoperable because of a low pulmonary function. A chest tube had been placed more than a month prior to this admission. A digital drainage system was used for 24-h monitoring of air leaks (Thopaz®). Using the Thopaz® system, we performed endoscopic bronchial occlusion using an endobronchial Watanabe spigot (EWS) to reduce air leaks. Finally, the air leaks ceased, and the chest tube was removed five days after EWS placement. We herein report a case of persistent pneumothorax that was successfully treated by endoscopic bronchial EWS placement with the aid of a Thopaz® system.
Inherited hemophagocytic lymphohistiocytosis (HLH) is a genetic anomaly disorder in which abnormally activated cytotoxic T lymphocytes cannot induce the apoptosis of target cells and antigen-presenting cells, leading to hemophagocytosis, pancytopenia, and a variety of symptoms such as a high fever. The present patient with adult-onset HLH developed refractory disease despite receiving immunosuppressive treatments. He underwent a reduced-intensity conditioning (RIC) regimen that comprised antithymocyte globulin (ATG) followed by cord blood transplantation (RIC-CBT). He achieved and maintained a complete donor type. The incorporation of ATG into RIC-CBT may prevent graft failure and control hemophagocytosis, however, further efforts are necessary to reduce infectious complications.
Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive T-cell lymphoma. A 63-year-old man was diagnosed with AITL. He received 6 cycles of CHOP therapy, but showed progressive disease. Subsequently, he received ESHAP chemotherapy; however, it was not effective. He received mogamulizumab (an anti-CCR4 monoclonal antibody). After 4 cycles, his respiratory condition worsened and he was diagnosed with cytomegalovirus (CMV) pneumonia. Despite antiviral and antibiotic therapy, he died. We speculate that the combination of progressive lymphoma with mogamulizumab and chemotherapy likely caused CMV pneumonia. Because mogamulizumab therapy causes immunosuppression, if CMV pneumonia is suspected, then rapid treatment should be initiated.
A 50-year-old man was admitted to our hospital for an adhesive ileus 14 years after total abdominal colectomy for ulcerative colitis (UC). The ileus decreased with conservative treatment, however, autoimmune hemolytic anemia (AIHA) was diagnosed due to worsening anemia, a positive direct Coombs test, low haptoglobin, high lactase dehydrogenase, reticulocytosis, and an increase in the erythroblastic series in a bone-marrow examination. Human parvovirus B19 (PV-B19) IgM and PV-B19 DNA were present, indicating the development of AIHA triggered by an infection with PV-B19. The patient is currently being monitored after spontaneous remission. This is the first report of UC after total abdominal colectomy complicated by AIHA triggered by PV-B19 infection.
A 61-year-old woman with rheumatoid arthritis who was undergoing hemodialysis for end-stage renal failure was transferred to our hospital due to severe thrombocytopenia and anemia. A bone marrow biopsy showed the complete absence of megakaryocytes and erythroblasts. Cyclosporine treatment resulted in the improvement of her megakaryocyte and erythroblast levels, and a decrease in her serum level of anti-c-Mpl (thrombopoietin receptor) antibodies. After this initial improvement, her anemia progressively worsened, despite the continuous administration of immunosuppressive therapy with cyclosporine. Her platelet and leukocyte counts remained stable. This is the first report of a probable case of anti-c-Mpl antibody-associated pure red cell aplasia and acquired amegakaryocytic thrombocytopenic purpura.
A 24-year-old Japanese woman developed anterocollis, weakness of the proximal arms, and subsequent cognitive impairment. A neurological examination revealed amyotrophic lateral sclerosis (ALS) without a family history. Systemic muscle atrophy progressed rapidly. Cerebral MRI clearly exhibited high signal intensities along the bilateral pyramidal tracts. An analysis of the FUS gene revealed a heterozygous two-base pair deletion, c.1507-1508delAG (p.G504WfsX515). A subset of juvenile-onset familial/sporadic ALS cases with FUS gene mutations reportedly demonstrates mental retardation or learning difficulty. Our study emphasizes the importance of conducting a FUS gene analysis in juvenile-onset ALS cases, even when no family occurrence is confirmed.
We herein present a case of tubercular spinal epidural abscess (SEA) without osseous involvement that mimicked an acute bacterial abscess. This case manifested quite unusual findings not only radiographically, but also clinically compared with previously reported cases of tubercular SEA.
Acute disseminated encephalomyelitis (ADEM) followed by optic neuritis (ON) has been reported as a distinct phenotype associated with anti-myelin oligodendrocyte protein (MOG) antibody. We herein report the case of a 37-year-old woman who was diagnosed with ADEM at 4 years old of age and who subsequently developed ON followed by recurrent ADEM 33 years after the initial onset. A serum analysis showed anti-MOG antibody positivity. This phenotype has only previously been reported in pediatric cases. Neurologists thus need to be aware that the phenotype may occur in adult patients, in whom it may be assumed to be atypical multiple sclerosis.
A 23-year-old Japanese woman was admitted to A hospital due to pneumonia. IgM for Mycoplasma pneumoniae was positive, and the patient was treated with imipenem/cilastatin, clindamycin, pazufloxacin and minocycline. However, both the chest radiological findings and the symptoms became exacerbated, and she was therefore transferred to our hospital. The bronchoalveolar lavage fluid was obtained, and a 16S rRNA gene sequencing analysis revealed a monobacterial infection of Mycoplasma pneumoniae. Therefore, corticosteroid treatment in addition to minocycline was administered, and the patients symptoms, laboratory data and chest radiographs improved. Corticosteroid therapy may therefore be considered for patients with refractory M. pneumoniae pneumonia.