Objective Left ventricular (LV) functions assessed by echocardiography and cardiac biomarkers are strong predictors of mortality in patients with systemic light chain (AL) amyloidosis. However, most previous studies have been conducted in Western countries, and the predictors of mortality in Asian patients with AL amyloidosis have not been characterized. To address this issue, we aimed to determine the predictors of mortality in Asian patients with biopsy-confirmed AL amyloidosis. Methods We retrospectively enrolled 31 patients (59±11 years, 55% men) in whom AL amyloidosis was confirmed by biopsies from cardiac or non-cardiac tissues. Of these patients, 15 (48%) met the international echocardiographic criteria for cardiac amyloidosis (mean LV wall thickness >12 mm without other causes of LV hypertrophy). Results During a mean follow-up period of 21±20 months, 15 patients died. Non-survivors had a higher number of involved organs, lower e', and higher rates of E/e' >15, pericardial effusion (PE), low voltage on an electrocardiogram and a New York Heart Association (NYHA) functional class ≥ III, compared with survivors. In multivariate analysis, a NYHA functional class ≥ III (p=0.024) and cardiac involvement (p=0.032) were independent predictors of PE in patients with AL amyloidosis. Multivariate Cox proportional hazard analysis indicated that PE (hazard ratio: 21.9, p=0.025) and the number of involved organs (hazard ratio: 2.8, p=0.015), but not LV diastolic parameters of tissue Doppler echocardiography, independently predict mortality in patients with AL amyloidosis. Conclusion PE and multiple organ involvement, compared with e' and E/e', are stronger predictors of mortality in patients with AL amyloidosis. The advanced disease stage of AL amyloidosis might underlie the strong association between PE and a poor outcome.
Objective Analyses of arterial blood gas parameters, including pH, HCO3- and lactate, play an important role in assessing the clinical status of patients with heart failure. In the present study, we evaluated the degree of agreement in the pH, HCO3- and lactate levels between arterial and venous blood samples according to the subset of the Forrester classification. Methods The study population consisted of 128 patients with known or suspected heart failure. The subjects were divided into four groups based on the Forrester classification. Arterial blood samples were drawn from the radial or brachial artery, and venous blood samples were drawn from the pulmonary artery. Results There were 59 patients with a Forrester subset I status, 32 patients with a subset II status, 21 patients with a subset III status and 16 patients with a subset IV status. The pH and HCO3- levels were similar between the four subsets. In all subsets, the pH values were significantly higher and the HCO3- values were significantly lower in the arterial blood samples than in the venous blood samples. There was good correlation and agreement in the pH and HCO3- levels between the arterial and venous blood samples. In contrast, there was a significant difference in the lactate levels in both the arterial and venous blood samples between the four subsets, and the lactate levels were highest in subset IV. In all subsets, there was good correlation and agreement in the lactate levels between the arterial and venous blood samples. Conclusion These data suggest that the venous pH, HCO3- and lactate levels are acceptable substitutes for arterial parameters, regardless of the cardiac hemodynamics.
Objective Adiponectin is an adipokine which is known to decrease in individuals associated with obesity and insulin resistance. In this study, we aimed to investigate the serum adiponectin levels and glucose metabolism in patients with thyroid dysfunction before and after treatment. Methods Newly diagnosed overt hypothyroid (n=20) and thyrotoxic (n=23) patients and healthy controls (n=20) with a body mass index of <30 kg/m2 were evaluated prospectively. Patients with a known state of insulin resistance, including prediabetes and overt diabetes, and individuals with chronic diseases were excluded. Thyroid function and fasting plasma glucose (FPG), insulin, homeostatic model assessment (HOMA) insulin resistance (HOMA-IR) and HOMA-beta cell function (HOMA-beta), lipid and adiponectin levels were investigated in the basal state and after the restoration of euthyroidism. Results The basal fasting FPG levels were lower in the hypothyroid patients than the control subjects (p=0.02) and similar between the thyrotoxic patients and control subjects (p=0.127). The basal HOMA-beta levels were higher in the patients with hypothyroidism than in those with thyrotoxicosis (p=0.015). Following the restoration of euthyroidism, the FPG levels significantly increased in the hypothyroid patients (p=0.002) and decreased in the thyrotoxic (p=0.001) patients. The basal plasma adiponectin levels were 14.55±8.4 mcg/mL, 13.79±9.13 mcg/mL and 11.68±6.0 mcg/mL in the hypothyroid and thyrotoxic patients and healthy controls, respectively (p=0.503). The adiponectin levels decreased significantly in the patients with hypothyroidism (p=0.047), whereas they did not change in the patients with thyrotoxicosis (p=0.770) after achieving euthyroidism. Conclusion In this study, following the restoration of euthyroidism, the FPG levels increased in the hypothyroidism patients and decreased in the thyrotoxicosis patients, despite the lack of changes in the HOMA-IR and HOMA-beta levels. Meanwhile, the hypothyroid, thyrotoxic and euthyroid subjects had similar basal adiponectin levels, and a significant decrease in the adiponectin levels was observed after treatment for hypothyroidism, despite the absence of changes after treatment for thyrotoxicosis, indicating the need for further studies with a larger sample size.
Objective This study was conducted to determine whether a policy of not attempting to resuscitate patients who are 80 years of age and older with cardiopulmonary arrest on arrival is justifiable. Methods The protocol of this study was approved without the need for informed consent by the research ethics board of Gunma University Hospital. We prospectively analyzed patients with cardiopulmonary arrest on arrival that was due to an intrinsic reason who were taken to Gunma University Hospital between January 2013 and March 2014. The patients were divided into two groups: patients who were less than 80 years of age (L group) and those aged 80 years and older (H group). We compared the patients' characteristics, including the etiology of cardiac arrest, and the prognosis between the two groups. Results A total of 103 patients with cardiopulmonary arrest on arrival were enrolled. There were no significant differences in the patients' characteristics, such as age, sex, witness and bystander cardiopulmonary resuscitation, and transportation time between the two groups. The return of spontaneous circulation was obtained in 14 patients (25.5%) in the L group and in 9 patients (18.8%) in the H group; however, no significant difference was seen between the two groups. Two patients in the L group were in good neurological condition when they were discharged; however, the other patients did not survive. Conclusion Even patients 80 years of age and older can be resuscitated to spontaneous circulation. We do not endorse a policy that recommends not performing cardiopulmonary resuscitation based solely on the age of the patient.
An 80-year-old woman was referred to our hospital for the treatment of advanced gastric cancer which extended from the antrum to the bulbus of the duodenum. Although the patient did not struggle or retch during endoscopy, multiple mucosal lacerations were observed in the proximal stomach by Mallory-Weiss tears. No evidence of perforation was identified at the sites. The day after endoscopy, computed tomography revealed free air close to the gastric cardia, but the patient did not complain of any symptoms; she was able to consume a normal diet and did not require any treatment.
We encountered a case of localized IgG4-cholecystitis mimicking gallbladder cancer with focal/segmental type1 autoimmune pancreatitis (AIP). In this case, we were unable to exclude a diagnosis of gallbladder cancer and thus performed radical cholecystectomy. Type1 AIP is often associated with gallbladder lesions, accompanied by generally diffuse, circumferential thickening of the gallbladder wall. Although localized IgG4-related cholecystitis is extremely rare, differentiating this condition from gallbladder cancer is often very difficult.
A 17-year-old Japanese male athlete presented to the emergency department at our hospital with a chief complaint of exertional dyspnea. Although there were no significant findings in the right and left upper extremities on a physical examination, a chest computed tomography scan showed bilateral multiple thrombosis in the pulmonary arteries, indicating pulmonary thromboembolism, and deep vein thrombosis in the left subclavian vein. Upper limb venography showed interruption of the left subclavian vein (so-called Paget-Schroetter syndrome; PSS). We herein report this rare case of PSS that led to pulmonary thromboembolism in a young, male field athlete.
Hyperglycemia rarely manifests as hemichorea-hemiballism (HH), which is characterized by simple partial motor seizures. One of the difficulties in the management of hyperglycemia-induced HH is the failure to recognize this entity due to its relatively uncommon presentation. We herein present a case series of hyperglycemia-associated dyskinesias, highlighting the different possible clinical presentations of this entity. Both hyperglycemia and hyperosmolality are probable predisposing factors, while ketoacidosis has a protective role in preventing the dyskinesias. One of our patients had ketotic hyperglycemia leading to HH, a previously unreported finding. Early recognition of this entity is crucial as prompt glycemic control leads to the resolution of symptoms and signs.
Acute poststreptococcal glomerulonephritis (APSGN) is a well-established disease. Although various immune responses are thought to be involved in the pathogenesis of APSGN, the disease has a self-limiting nature in clinical practice, despite the presence of severe acute symptoms. We herein report the case of a 78-year-old woman with APSGN who developed primary biliary cirrhosis (PBC) after achieving remission of renal manifestations, including anasarca and elevation of serum creatinine, indicating that persistent alterations in the immune system can cause extrarenal disorders. This case provides insights into the appropriate clinical management of ASPGN and pathogenesis of PBC.
A 49-year-old Japanese woman with polycystic liver disease (PLD) was admitted for right hypochondrial pain. CT showed a huge enhancing mass in the liver. She tested negative for other liver diseases, such as hepatitis B and C and alcoholic liver disease. After the patient expired due to hepatic failure, an autopsy revealed poorly differentiated hepatocellular carcinoma (HCC) surrounded by multiple hepatic cysts. The small amount of residual hepatic parenchyma showed nonalcoholic fatty liver disease (NAFLD) with severe steatosis. Severe emaciation was also apparent. This case suggests that malnutrition in patients with symptomatic PLD may contribute to the development of HCC via NAFLD.
A 52-year-old Japanese woman was admitted to our hospital with the renal pelvic mass lesion detected on a health screening examination. The surgical specimen contained a mass exhibiting the histological features of immunoglobulin (Ig)G4-related disease, including lymphoplasmacytic infiltration and sclerosis with numerous IgG4-producing plasma cells. Postoperatively, an elevation of the serum IgG4 level was confirmed at 403 mg/dL; however, there was no evidence of tubulointerstitial nephritis or glomerulopathy, including membranous nephropathy, and the urothelium of the renal pelvis was intact without inflammation. We herein report this case in which IgG4-related disease of the renal pelvic region presented with a mass lesion in the intrarenal sinus near the renal pelvis, not 'pyelitis' (as described by Stone).
A 33-year-old woman was admitted to our emergency department in a state of unconsciousness after attempting suicide with unknown substances. Severe metabolic acidosis (pH: 6.81), with a high anion gap (36.2) and high lactate level (20.2 mmol/L), was observed. After four hours of intensive medical treatment, the patient regained consciousness, with a return of the arterial pH to 7.42. Finally, cyanide intoxication was diagnosed based on the detection of a serum cyanide level of 3.5 mg/L. The presence of a high anion gap associated with severe lactic acidosis is a clue for making a rapid differential diagnosis of acute cyanide intoxication. Providing intensive and immediate supportive management is also crucial, even in cases without obtainable specific antidotes.
Tracheobronchial aspergillosis is a form of invasive aspergillosis limited to the airways. Its presence usually indicates an immunocompromised status or local dysfunction of airway immunity. We herein report a case of lung carcinosarcoma masked by tracheobronchial aspergillosis and discuss the clinical manifestations, diagnostic procedures and treatment of such cases. Tracheobronchial aspergillosis may also mask endobronchial carcinoma, leading to difficulties in determining the diagnosis and selecting subsequent treatment. Bronchoscopy is crucial for obtaining a definitive diagnosis and confirming the treatment. In particular, the combination of intravenous antifungal drug administration, localized antifungal application and interventional bronchoscopy is effective. Nevertheless, clinicians should always investigate the underlying disease carefully, including the potential occurrence of lung malignancy.
A 22-year-old man was admitted following the detection of right hilar enlargement during a medical checkup. The patient's serum angiotensin-converting enzyme (ACE) level was abnormally high, and a needle aspiration biopsy showed non-caseating epithelioid cell granulomas. Surgical resection was performed, and the resected specimens showed irregularly shaped seminoma nests with intervening stroma consisting of epithelioid cell granulomas. Furthermore, immunohistochemistry demonstrated ACE expression in the epithelioid cells and some tumor cells. The patient's serum ACE level declined after the surgery and subsequent systemic chemotherapy, indicating the presence of tumor-induced sarcoid-like reactions rather than the coexistence of seminoma and sarcoidosis.
We herein describe the cases of two patients with post-irradiation lumbosacral radiculopathy. The patients underwent postoperative radiation therapy to the abdomen due to testicular neoplasms 20 and 25 years prior to the onset of weakness, respectively. On physical examinations, asymmetric lower limb weakness and areflexia without apparent sensory loss were observed in both patients. Interestingly, artificial and squared atrophy of the skin and subcutaneous tissue, thought to correspond to the radiation fields, were observed in the lower back, and electromyography revealed selective motor axon loss localized to the lower extremities. The detection of skin changes in the area being irradiated is a valuable clue for diagnosing post-irradiation lumbosacral radiculopathy.
We herein report the case of a 69-year-old woman with Charcot-Marie-Tooth Disease type 2J (CMT2J) who presented with Adie's pupil, deafness, and urinary disturbance in addition to motor symptoms. On autonomic investigation, the coefficient of variation of the R-R intervals was decreased, and a urodynamic analysis showed a hypotonic bladder. A heart rate variability analysis revealed a decreased high frequency component and low frequency/high frequency ratio. Orthostatic hypotension was not present, and the sympathetic skin response and cardiac scintigraphy using 123I-metaiodobenzylguanidine were normal. A gene analysis showed a known heterozygous mutation associated with CMT2J in myelin protein zero exon 3, resulting in the substitution of threonine to methionine at position 124. Our case suggests that mainly the parasympathetic autonomic function is disturbed in CMT2J.
A 63-year-old man revealed a four-month history of muscle weakness of the lower limbs, hypoesthesia of the L5 and S1 area and ischuria. On MRI, the spinal cord was compressed by an encircled mass, which showed hypointensity on T1- and T2-weighted images with gadolinium enhancement at the Th11 to Th12 vertebra. Because of the rapid progression of myelopathy, posterior decompression was performed and idiopathic hypertrophic spinal pachymeningitis (HSP) was finally diagnosed. The patient's neurological signs markedly improved with postoperative corticosteroid treatment. Idiopathic HSP is a clinical emergency and early surgical intervention is essential to prevent irreversible damage to the nervous system.
We describe the case of a 46-year-old man with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis with prominent parkinsonism. The patient presented with psychiatric symptoms followed by epileptic seizure and parkinsonism including micrographia. Magnetic resonance imaging (MRI) revealed lesions in the bilateral medial temporal lobes and basal ganglia on fluid-attenuated inversion recovery images. His symptoms and MRI findings were ameliorated by immunotherapy but then relapsed. After retreatment, his parkinsonism gradually improved except for the micrographia. This is an atypical case of anti-NMDAR encephalitis in that the patient showed prominent and refractory parkinsonism, thus indicating that the clinical diversity of anti-NMDAR encephalitis is greater than expected.
We herein report a case involving the systemic presentation of angiolymphoid hyperplasia with eosinophilia (ALHE) in association with membranous nephropathy (MN). A 34-year-old Japanese man presented with leg edema and bilateral temporal nodules. He had a history of Buerger's disease and recurrent coronary stenosis. A renal biopsy performed to assess nephrotic syndrome revealed MN. Furthermore, a temporal nodule was excised, and ALHE was diagnosed. We reevaluated the coronary and posterior tibial artery specimens obtained in his twenties and presumed that these lesions were also vascular tumors arising from ALHE. The association of ALHE and MN is quite rare.