In patients with type 2 diabetes (T2DM), the coexistence of hypertension enhances the cardiovascular risk, and the prevention of future cardiovascular disease is an important component of T2DM management. Antihypertensive therapy has been shown to be an effective method of reducing the micro- and macrovascular complications of T2DM, however, the optimal target blood pressure (BP) levels are still under debate. Most of the international guidelines have raised the target clinic BP from 130/80 to 140/90 mmHg, however, the Japanese Society of Hypertension 2014 guidelines kept the target BP level at 130/80 mmHg. However, individualized BP-lowering treatments should be considered in patients with T2DM, especially in high-risk individuals such as those with a history of stroke or retinopathy, and aggressive antihypertensive therapy below 130 mmHg should be initiated even when the initial systolic BP level is <140 mmHg. The authors performed two studies concerning the BP target levels of home BP. In the first study, the authors found that a home BP target <125/75 mmHg was effective in improving the measures of vascular stiffness and kidney damage. In the second study, when the clinic BP target was set at 130/80 mmHg, the home BP could be approximately 130/80 mmHg. More data are needed to individualize the target BP levels of T2DM patients.
Objective The Friedewald equation has been used as the standard formula for the estimation of very low-density lipoprotein cholesterol (VLDL-C) to calculate the serum levels of low-density lipoprotein cholesterol (LDL-C). However, the usefulness of this formula in non-Caucasians has been challenged in recent years. The aim of this study was to assess the validity of the conventional and modified Friedewald equation in an ethnic Chinese adult population. Methods We prospectively recruited 938 subjects from the Lipid Clinic of the National Taiwan University Hospital. The fasting lipids, including cholesterol, high-density lipoprotein cholesterol, LDL-C, VLDL-C, and triglycerides (TGs), of each participant were measured and we constructed a prediction model for the estimation of LDL-C by the modified Friedewald equation. Results The constant values, used to estimate the VLDL-C concentration, were different in the Friedewald equation in the subgroups exhibiting different cardiovascular characteristics, ranging from 4.45 to 6.63, if we calculated the equation by a direct LDL-C measurement. According to the results of a stepwise multiple linear regression analysis excluding the TG levels ≥400 mg/dL, an equation for the individual constant Y estimation was as follows: Constant Y = 4.39 + (-0.59) if the age ≥50 years + 0.98 if men + TG group (2.06 if the TG levels were between 150-399 mg/dL; 1.07 if the TG levels were between 100-149 mg/dL) + (-0.49) if the cholesterol levels ≥240 mg/dL + the body mass index (BMI) group (0.8 if BMI ≥27 kg/m2; 0.48 if BMI =24-27 kg/m2). Conclusion This modified Friedewald formula provides a new and simple equation for calculating the LDL-C levels in ethnic Chinese people.
Objective Periprocedural myocardial injury (PMI) remains a relatively common complication even after successful procedures. In-stent restenosis (ISR) may be involved in lesion-related factors for PMI. We compared the incidence of PMI between patients with ISR and those with de novo stenosis. Methods The study population consisted of 121 patients with coronary artery disease who had been treated with statins and subsequently underwent angiographically successful percutaneous coronary intervention (PCI). Blood samples for troponin I were collected 18 to 24 hours after PCI. PMI was defined as an increase in the troponin I levels greater than 0.15 ng/mL. Major PMI was defined as an increase in the troponin I levels greater than 0.75 ng/mL. Results There were 34 patients with ISR and 87 patients with de novo stenosis. The incidence of PMI was similar between the two groups (47.1 % vs. 55.2 %, p=0.42). Among the patients with ISR, the incidences of PMI were 33.3 %, 60.0 % and 66.7 % in patients with focal ISR, diffuse ISR and diffuse proliferative ISR, respectively, although these differences were not statistically significant. The incidence of major PMI was significantly less frequent in patients with ISR than those with de novo stenosis (5.9 % vs. 25.3 %, p=0.03). A multivariate logistic regression analysis showed that ISR [odds ratio (OR) 0.22, 95% confidence interval (CI) 0.03-0.90; p=0.03] and the maximum inflation pressure (OR 1.15, 95% CI 1.04-1.30; p=0.009) were independent predictors of major PMI. Conclusion Our results suggest that while PMI occurs in patients with ISR as commonly as those with de novo stenosis, major PMI occurs less frequently in patients with ISR.
Objective High-flow nasal cannula (HFNC) therapy is an oxygen delivery system. However, evidence regarding the clinical applications of HFNC is still emerging. We herein evaluated the clinical predictors of HFNC therapy success for adult patients with acute hypoxemic respiratory failure. Methods We retrospectively reviewed the medical records of the subjects with acute hypoxemic respiratory failure supported by HFNC therapy in the medical intensive care unit between July 2011 and March 2013. Therapy success was defined as the avoidance of intubation. The patients' baseline characteristics and the serial changes in the respiratory parameters after HFNC therapy at 1 and 24 hours were measured. Results Of the 75 eligible patients, 62.7% successfully avoided intubation. Overall, HFNC therapy significantly improved the physiologic parameters, such as partial pressure of arterial oxygen (PaO2), saturation of arterial oxygen (SaO2), respiratory rate (RR), and heart rate (HR), throughout the first 24 hours. After the adjustment for the other clinical variables, Acute Physiology and Chronic Health Evaluation II (APACHE II), Sequential Organ Failure Assessment (SOFA), cardiogenic pulmonary edema, and PaO2 improvement at 1 and 24 hours were associated with therapy success. The overall intensive care unit (ICU) mortality was 25.3%. However, out of 37.3% of the patients who required intubation, the ICU mortality in this proportion of patients was 67.9%. The ICU mortality in the therapy failure group was associated with the use of a vasopressor and a limited PaO2 improvement at 1 hour. Conclusion HFNC therapy showed a good compliance and the improvement of the physiologic parameters in an adult population. The failure to improve oxygenation within 24 hours was a useful predictor of intubation. Among the failure group, the vasopressor use and failed oxygenation improvement were associated with ICU mortality.
Objective In the 1950s, a high-dose (40-70 mg/kg/day) of pyrazinamide (PZA), was reported to cause drug-induced liver injury (DILI) at an unacceptable frequency. It remains unclear whether adding PZA (Z) at the currently accepted low-dose (20-25 mg/kg/day) for two months to a regimen of isoniazid (H) + rifampicin (R) + ethambutol (E) actually increases the risk of DILI. Method Smear-positive tuberculosis patients were treated with daily HRE or HRZE regimen under direct observation. We used three independent models. Model 1 was analyzed with a multivariate Cox-analysis using a pre-matched cohort. Next, propensity score matching was conducted using the nearest neighbor method with caliper of 0.03. Models 2 and 3 were analyzed by univariate and multivariate Cox-analyses, respectively, with the matched cohort. DILI was assessed based on the guidelines of the American Thoracic Society. Results We reviewed the records of 383 patents (male, n=260; female n=123; mean age, 64±20 years). Among these patients, 75 patients were treated with HRE and 308 were treated with HRZE. DILI occurred in the first two months in 24% (18/75) and 8% (24/308) of the HRE-treated and HRZE-treated cases, respectively. In all three of the models, DILI was less frequent in patients treated with the HRZE regimen: Model 1, HR of 0.30 (95% confidence interval (CI) 0.14-0.68, p=0.004); Model 2, HR of 0.37 (95%CI 0.14-0.96, p=0.041); and Model 3, HR of 0.34 (95%CI 0.12-0.94, p=0.038). Conclusion The addition of the currently accepted low dose (20-25 mg/kg/day) of PZA to the HRE regimen did not increase the incidence of DILI during the first two months of treatment.
We herein report the case of a 72-year-old man who underwent photodynamic therapy (PDT) with talaporfin sodium for recurrent cholangiocarcinoma after surgical resection. Endoscopic retrograde cholangiography (ERC) showed severe stenosis with an irregular surface measuring approximately 1 cm in length from the anastomotic site, and a recurrent nodular lesion was observed at the anastomotic site of the right anterior intrahepatic bile duct on gastrointestinal endoscopy. ERC after PDT revealed a dramatic improvement in the bile duct stenosis, and the nodular lesion had disappeared. No adverse events from the PDT were detected. PDT using talaporfin sodium may be a safe alternative treatment for cholangiocarcinoma.
A man diagnosed with alcoholic liver cirrhosis complained of abdominal distention due to massive ascites. The ascites did not resolve with diuretic agents. The serum-ascites albumin gradient value of 1.9 g/dL and the total protein level in the ascites of 3.1 g/dL indicated the ascites to have been caused by portal hypertension. Hypothyroidism was detected, and the patient received supplementation with levothyroxine. The ascites dramatically decreased after supplementation with levothyroxine. We herein conclude that the ascites in the present case had thus been strongly influenced by portal hypertension, which was induced by liver dysfunction associated with liver cirrhosis and hypothyroidism.
Streptococcus agalactiae (Group B streptococcus, GBS) is the major pathogen encountered in the perinatal period, although the incidence of GBS infection has recently increased among non-pregnant adults. Nevertheless, GBS infective endocarditis (IE) is uncommon and often accompanies aortic embolism. We experienced two cases of GBS IE. In Case 1, mobile vegetation of the aortic valve caused an infective cerebral aneurysm. In Case 2, the patient experienced an acute aortic embolic episode. Generally, early surgery for large mobile sites of vegetation is recommended as a class IIb therapy in the guidelines. GBS IE often exhibits a severe clinical course and specificity of vegetation. Therefore, early surgery should be considered in such cases.
IgG4-related pericardial involvement has rarely been reported and its clinical features remain unknown. We herein report a case of a 50-year-old woman with pericarditis who presented with a fever, elevated C-reactive protein levels, elevated serum IgG4 concentrations, and thickened pericardium with a patchy 18F-fluorodeoxyglucose (FDG) uptake. A biopsy specimen of 18F-FDG accumulated in the mediastinal lymph nodes revealed an abundant infiltration of IgG4-bearing plasma cells without fibrosis. Moderate-dose glucocorticoids promptly resolved the physical, serological, and imaging abnormalities, thus indicating a relatively acute and reversible nature of IgG4-related pericardial involvement.
A 33-year-old woman experienced near-syncope at a hospital. Electrocardiography revealed an intermittent ventricular rhythm. The echocardiogram at admission indicated mild hypokinesis and severe diffuse hypokinesis with reverse takotsubo cardiomyopathy on the following day. The patient experienced abdominal pain on the admission day, and computed tomography identified a large left adrenal mass. Her catecholamine levels increased remarkably on the third day. The wall motion improved on the twelfth day. The tumor was successfully resected and the patient was diagnosed with an ectopic pheochromocytoma. The ventricular rhythm with myocardial damage and hypotension induced by the reverse takotsubo cardiomyopathy masked the characteristic symptoms of pheochromocytoma.
An 83-year-old woman who complained of dizziness and nausea visited our hospital. An electrocardiogram showed ST-segment elevation in multiple leads and an echocardiogram showed severe hypokinesis of the anteroseptal wall of the left ventricle. However, emergency coronary angiography showed no stenotic lesions in any coronary arteries. A laboratory examination showed thrombocytopenia, renal dysfunction, and hemolysis. We therefore diagnosed the patient with thrombotic thrombocytopenic purpura (TTP). While we were preparing to initiate plasma exchange therapy, she suddenly developed cardiopulmonary arrest. A postmortem examination revealed microthrombi in the small vessels of the myocardium. We herein report a case of ischemic cardiomyopathy with a rapid progression from TTP.
The gene succinate dehydrogenase subunit B (SDHB) encodes a protein comprising part of the mitochondrial complex II, which links the Krebs cycle and the electron-transport chain. Heterozygous germ-line SDHB mutations causes familial pheochromocytoma-paraganglioma syndrome and has also been linked to gastrointestinal stromal tumors, as well as renal cell carcinomas. We herein report a patient with a germ-line SDHB mutation who presented with an atypical meningioma that was identified as originating from a somatic SDHB mutation. The 41-year-old man, who had a surgical history of extra-adrenal pheochromocytoma at 23 years of age, recently developed gait disorder and hypertension. At the radiological examination, a tumor was detected in the cervical spinal cord at the C6-7 intervertebral level. The pathological findings of the isolated tumor were atypical meningioma assessed as grade II according to the World Health Organization criteria. Inherited neoplasia syndrome was suspected because of the patient's history of early-onset extra-adrenal pheochromocytoma and the development of meningioma. We therefore performed molecular genetic analyses. A direct sequence analysis revealed a heterozygous germ-line frameshift mutation in SDHB, specifically an 11-nucleotide deletion, c.305-315delCAATGAACATC, in exon 4, resulting in a frameshift p.A102EfsX12. Additionally, the sequence analysis of the tumor DNA revealed only a mutated allele with a frameshift mutation in the germ-line SDHB. Our findings suggest that SDHB plays an important role in the pathogenesis of meningiomas as well as pheochromocytomas. Therefore, a differential diagnosis for metastatic pheochromocytoma and other new onset tumors, including meningioma, particularly in patients with germ-line SDHB mutations and a previous history of pheochromocytoma should be carefully made.
We herein report the case of a 66-year-old Japanese man with acute-onset type 1 diabetes mellitus (T1D) accompanied by pernicious anemia. After 2 weeks of polyuria, the patient developed insulin-deficient hyperglycemia with diabetic ketoacidosis in the absence of verifiable islet-related autoantibodies and began insulin therapy in 2001. Eight years later, he developed gastric autoantibody-positive pernicious anemia and began methylcobalamin treatment. Previous studies have reported cases of slowly progressive autoimmune T1D concomitant with pernicious anemia. The present case suggests that potential associations with organ-specific autoimmune disorders should be considered during the long-term follow-up of T1D patients, even though verifiable islet-related autoantibodies are undetectable.
A 66-year-old man with type 2 diabetes on hemodialysis treatment was admitted due to poor glycemic control. His serum insulin level and the 125I-insulin binding rate were extremely high with an increased eosinophil count, although he did not have an allergic reaction to insulin or an elevation of specific IgE for human insulin. A Scatchard analysis revealed that the patient's insulin antibodies had a low affinity constant and a high binding capacity. Prednisolone administration decreased the eosinophil count and 125I-insulin binding rate; accordingly, the glycemic control improved. Corticosteroid therapy may be a potent therapeutic strategy for insulin antibody-induced severe insulin resistance with eosinophilia.
A 33-year-old Japanese man was admitted with severe edema, and a renal biopsy confirmed minimal change nephrotic syndrome (MCNS). CT revealed his severe chronic sinusitis, and he first received antimicrobial therapy, which resulted in decreased proteinuria. The surgical operation for sinusitis resulted in the complete disappearance of proteinuria without corticosteroid or immunosuppressant therapy within one week. MCNS may be triggered by infection, but there are no previously reported cases of MCNS that is completely remitted by infection control alone. Therefore, we herein report the first case of MCNS that attained complete remission following therapy for chronic sinusitis alone, which suggests a strong etiology of chronic sinusitis for MCNS.
Immunoglobulin (Ig) A nephropathy is a prevalent form of primary glomerulonephritis, which leads to end-stage renal failure in a significant proportion of patients. Immunotherapy, including steroid use, is widely used to induce disease remission; however, it can cause serious side effects. We herein report 3 cases of progressive IgA nephropathy and their successful treatment with a combination of aspirin and eicosapentaenoic acid (EPA) without the use of steroids. The precise mechanism responsible for the combination therapy is still unknown; however, aspirin may potentiate the production of anti-inflammatory lipid mediators derived from EPA. Further clinical trials are required to substantiate this treatment regimen.
A 65-year-old woman was admitted with acute intramuscular hemorrhage of the left gluteus medius and piriformis muscles and associated anemia. Blood tests showed low plasma factor XIII (FXIII) antigen and activity. A cross-mixing test revealed a concave "inhibitor" pattern and anti-FXIII-A subunit antibody was detected. The patient was diagnosed with autoimmune hemorrhaphilia resulting from anti-FXIII antibody. The bleeding has not recurred since the initiation of treatment with oral immunosuppressive agents. Although hemorrhagic acquired FXIII deficiency is a rare disorder, prompt recognition of the underlying mechanism can save lives.
We herein present a case of congenital erythrocytosis caused by haemoglobin (Hb) Bethesda in a Japanese family. A 55-year-old asymptomatic man was referred to our hospital for the investigation of erythrocytosis, which was present in other members of his family. The patient's serum erythropoietin level was normal, and the JAK2 V617F mutation was not detected. His P50 value was mildly decreased, thus we suspected the presence of an Hb variant with a high oxygen affinity. The high-performance liquid chromatography analysis showed an abnormal Hb, and by direct sequencing we identified the Hb Bethesda variant in this patient. For the differential diagnosis, we recommend the estimation of the P50 value as a practical and useful test.
Polycythemia vera (PV) is a chronic myeloproliferative neoplasm that leads to hyperviscosity and the risk of thrombosis. We encountered the case of a young male Filipino patient diagnosed with PV after the rupture of esophageal varices. The complete blood cell count showed a slight increase in white blood cells. An abdominal computed tomography scan disclosed splenomegaly and occlusion of the portal vein and collateral vessels. A blood examination demonstrated an increase in all three blood cell lines within three months. Based on the presence of severe hypercellularity of the bone marrow and positivity for the JAK2V617F mutation, we finally diagnosed the patient with PV.
A 67-year-old man was transported to our hospital and diagnosed with pneumococcal meningitis. We immediately administered ceftriaxone and vancomycin according to the guidelines, but did not administer dexamethasone to him because he had been previously administered antibiotics. His left eye became complicated by endogenous endophthalmitis on the next day, which resulted in blindness, although his meningitis rapidly ameliorated. In comparison to other patients who have been reported to recover from complications with endophthalmitis after the combination therapy of antibiotics, corticosteroids and vitreous surgery, we consider that this patient's poor visual outcome may have been caused by severe inflammation or the breakdown of the blood ocular barrier due to the action of S. pneumoniae. Corticosteroids may be able to successfully treat such inflammation or disruption of the blood ocular barrier.
An 88-year-old woman presented with fever and acute posterior neck pain. A CT scan revealed calcification of the transverse ligament and crown-like calcification around the odontoid process. According to the clinical and radiological findings, she was diagnosed with crowned dens syndrome (CDS). Her symptoms drastically improved following treatment with oral nonsteroidal anti-inflammatory medication. An X-ray of her wrist, elbow, shoulder and knee joints showed asymptomatic calcium deposits, suggesting underlying crystalline deposition disease. CDS may occur as the initial presentation of crystalline deposition disease. The measurement of procalcitonin and an X-ray survey of the major joints may be helpful for the diagnosis of CDS.
A 64-year-old Japanese woman had rapidly progressing bilateral palmar contracture associated with severe pain on both palms over the previous 8 weeks, without a history of arthritis in any joints. We suspected palmar fibromatosis or fasciitis without polyarthritis. Because palmar fibromatosis is known to be associated with cancer, we performed cancer screening and the patient was subsequently diagnosed with fallopian tube cancer. This is the first case report of palmar fibromatosis or fasciitis without polyarthritis associated with fallopian tube cancer. The characteristic rapid progression of palmar contracture is a key finding that suggests the potential existence of a malignancy.
We herein describe the first reported case of pyogenic spondylitis and diskitis caused by Helicobacter cinaedi. The results of magnetic resonance imaging and the histology of biopsied tissue were suggestive of acute infection at the lumbar spine. The pathogen was obtained by a blood culture examination and identified by 16S rRNA analysis. Eight weeks of antibiotics therapy resulted in a good clinical course. H. cinaedi infections have been increasingly reported in recent years, but the pathogen's epidemiological and pathological characteristics are still unclear. One of the difficulties in understanding the pathogenesis of H. cinaedi has been the challenges in cultivating the pathogen. Novel strategies for the diagnosis of H. cinaedi must be developed.