Spontaneous neck hematoma associated with parathyroid adenoma.

We should consider parathyroid extraglandular bleeding for patients with acute neck pain and swelling. Evaluation of serum calcium and parathyroid hormone levels is crucial for a suspected neck hematoma associated with parathyroid adenoma.

Amyloid deposition through endocytosis in vascular endothelial cells.

No mechanistic lead is known for establishing AL amyloid deposits in organs. We here report an electron microscopic (EM) analysis in a case of intestinal AL amyloidosis before initiating treatment for amyloidosis. The dense deposits of amyloid fibrils are concentrated around the small blood vessels in the submucosal area of intestinal tissue. Surprisingly, we observed endothelial cells (ECs) of blood vessels containing plenty of endocytotic (pinocytotic) and transcytotic vesicles at the luminal side and above the basement membrane, indicating the one-way active trafficking of either the immunoglobulin (Ig) light chain or preassembled amyloid fibrils from the luminal side of ECs to the extraluminal area of ECs. Immunoelectron microscopy displayed that the immuno-gold signals were observed in the vascular cavity and the subendothelial area of amyloid deposits. However, there is no sign of an Ig light chain in pinocytotic vesicles. Therefore, the intestinal ECs may actively pump out mainly the preassembled amyloid fibrils (not light chains) from the blood stream into the subendothelial area as a physiologic function.

Glucocorticoid in Combination with a TNF-α Inhibitor: Treatment of Deep Vein Thrombosis in a Patient with Behçet's Disease.

A 38-year-old man with deep vein thrombosis associated with Behçet's disease (BD) was admitted to our hospital due to worsening symptoms despite the initiation of direct oral anticoagulants (DOACs). Administration of oral prednisolone and an intravenous anti-tumor necrosis factor-alpha (TNF-α) monoclonal antibody dramatically improved his symptoms. In addition, he was incidentally diagnosed with autosomal dominant polycystic kidney disease, which increases the risk of aortic aneurysms. BD also increases the risk of aortic aneurysms. This case suggests that immunosuppressive treatment is effective in patients with inflammation-related DOAC-refractory venous thrombosis who also suffer from BD.

Panhypopituitarism Mimicking Acute Coronary Syndrome.

A 59-year-old man suspected of having myocardial infarction with sinus bradycardia, a decreased blood pressure, and ST-change on an electrocardiogram was referred to our hospital's emergency department. Emergent coronary angiography revealed no significant findings. However, the patient experienced shock and required intensive care. Curiosity rose when his urination volume was not disturbed; we suspected hormonal abnormalities. A hormonal examination and imaging analysis revealed panhypopituitarism caused by a Rathke's cyst. Appropriate hormonal replacement therapy improved his symptoms and led to normalization of his electrocardiogram findings. Acute coronary syndrome (ACS) is a fatal disease; however, clinicians must not discount panhypopituitarism, as it may mimic ACS symptoms.

Acute Onset of Remitting Seronegative Symmetrical Synovitis With Pitting Edema (RS3PE) Two Weeks After COVID-19 Vaccination With mRNA-1273 With Possible Activation of Parvovirus B19: A Case Report With Literature Review.

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare clinical entity characterized by "remitting," "seronegative," and "symmetrical" synovitis with pitting edema on the dorsum of the hands and feet. Although rheumatic or malignant diseases are diseases that are known to coexist with RS3PE, other factors such as medication, infection, and vaccination have been reported to be associated with RS3PE. Here, we present a case of RS3PE syndrome that satisfied all four diagnostic criteria of RS3PE (pitting edema in the limbs, acute onset, age ≥ 50 years, and/or rheumatoid factor negativity) after mRNA-1273 SARS-CoV-2 vaccination.

Hemophagocytic Syndrome-Like Tuberculosis-Immune Reconstitution Inflammatory Syndrome After the Initiation of Hepatic Tuberculosis Treatment.

A 25-year-old Nepalese woman was referred to our hospital because of fever and intermittent abdominal pain with inguinal lymphadenopathy, which had lasted for several months. A liver biopsy of the positron emission tomography-positive lesion led to a diagnosis of hepatic tuberculosis. After the initiation of antituberculosis treatment, her symptoms resolved. However, 11 days after treatment initiation, chest and back pain, high-grade fever, and vomiting appeared and gradually worsened. She developed anemia and her serum ferritin level was elevated. Hemophagocytic syndrome due to the initial deterioration of tuberculosis was suspected and steroid therapy was initiated with the continuation of the antituberculosis drugs. Thereafter, the patient's condition improved remarkably.

Nontraumatic bladder rupture: The images before and after.

We should consider the complication of bladder rupture for patients with worsened abdominal pain and inability to pass urine following acute cystitis. A CT scan is a useful first-line modality when evaluating for a suspected bladder rupture.

Association between abnormal myocardial scintigraphy findings and long-term outcomes for elderly patients 85 years or older: a retrospective cohort study.

BACKGROUND: Normal findings of cardiac scintigraphy predict good outcomes. However, a paucity of the data exists for elderly patients 85 years or older. In the present study, we aimed to demonstrate the association between the abnormal findings of cardiac scintigraphy and the risk of all cause death in patients 85 years or older. METHODS: We enrolled 143 consecutive patients 85 years or older with known or suspected coronary artery disease who underwent stress scintigraphy under adenosine or an exercise test and a 99mTechnetium (Tc)-labeled tracer or thallium 201 (201Tl), dual tracer rest scintigraphy using 201Tl and 123I-ß-methyl iodophenyl pentadecanoic acid (123I-BMIPP), or 123I-BMIPP single tracer scintigraphy. Ischemia was defined by an induced perfusion abnormality according to a provocation test with recovery at rest or decreased uptake of 123I-BMIPP despite normal perfusion at rest. Infarction was defined by perfusion abnormalities assessed by images at rest on 201Tl or 99mTc-labeled tracer. We defined these findings as abnormal when at least one of these aforementioned characteristics was observed. RESULTS: Patients in the abnormal findings group (N = 62) were more likely to have undergone prior coronary angiography and to have decreased ejection fraction than those in the normal findings group (N = 81). The median follow-up duration was 797 days (interquartile range, 635-1045 days), with follow-up rates of 90% at 1 year and 73% at 2 years. The 2-year mortality rate were significantly higher in the abnormal findings group than in the normal findings group (26.8% vs. 10.9%; p = 0.01). The risk of abnormal findings relative to normal findings remained significant for the mortality (adjusted hazard ratio, 5.99; 95% CI, 1.37-42.8; P = 0.015). CONCLUSION: Abnormal myocardial scintigraphy findings were associated with the increased risk for mortality, even for patients 85 years or older.

Acute Pulmonary Hypertension Crisis after Adalimumab Reduction in Rheumatoid Vasculitis.

Rheumatoid vasculitis is a rare etiology for pulmonary hypertension (PH) in patients with connective tissue disease. We encountered a case of acute PH crisis in a case with rheumatoid vasculitis eight months after undergoing adalimumab reduction. Since no repetition of arthralgia occurred after the adalimumab reduction, we decided to not increase the dose of adalimumab. However, hemodynamic collapse thereafter developed and even though steroid pulse therapy was administered, the patient nevertheless died. The autopsy showed clusters of acute and chronic inflammation around the remodeled pulmonary arteries along with micro-thrombi in the vessel lumen. We should consider the possibility of critical worsening of PH as a phenotype of vasculitis related to immunosuppressive therapy reduction.

Progression of calcific aortic valve sclerosis in WHHLMI rabbits.

BACKGROUND AND AIMS: Aortic valve stenosis (AS) is the most common valvular heart disease and can be life-threatening. The pathogenesis of aortic valve calcification remains largely unknown, primarily due to the lack of an adequate animal model. The high-cholesterol diet-induced AS model in rabbits is one of the established models, but it has the significant limitation of liver dysfunction leading to low survival rates. We hypothesized that a myocardial infarction-prone Watanabe heritable hyperlipidemic (WHHLMI) rabbit, an animal model of familial hypercholesterolemia and atherosclerosis, is a useful animal model of AS. METHODS: WHHLMI rabbits, aged 20 months and 30 months (n = 19), and control Japanese White rabbits (n = 4), aged 30 months, were used and evaluated by echocardiography under anesthesia. Pathological evaluation and quantitative analyses by polymerase chain reaction (PCR) were also performed. RESULTS: The lipid profile was similar between 20 months and 30 months. Two rabbits died due to spontaneous myocardial infarction during the study. Thirty-month-old WHHLMI rabbits exhibited significantly smaller aortic valve area (0.22 ±â€¯0.006 cm2vs. 0.12 ±â€¯0.01 cm2, p < 0.05) and higher maximal transvalvular pressure gradient (7.0 ±â€¯0.32 vs. 9.9 ±â€¯0.95 mmHg, p < 0.05) than 20 month-old rabbits. Macroscopic examination of excised aortic valves demonstrated thickened and degenerated valve leaflets at 30 months. Histological evaluation confirmed thickened leaflets with calcified nodules at 30 months. Real-time PCR of resected aortic valve also showed increased expression level of calcification-related molecules including osteopontin, Sox9, Bmp2, RANKL, osteoprotegerin, and Runx2 (p < 0.05 each) in 30-month-old rabbits. CONCLUSIONS: WHHLMI rabbits may be useful models of early-stage AS in vivo.

Targeted Disruption of JCAD (Junctional Protein Associated With Coronary Artery Disease)/KIAA1462, a Coronary Artery Disease-Associated Gene Product, Inhibits Angiogenic Processes In Vitro and In Vivo.

OBJECTIVE: Recent genome-wide association studies newly identified the human KIAA1462 gene as a new locus for coronary artery disease. However, the function of the gene product, named JCAD (junctional protein associated with coronary artery disease), is unknown. Because JCAD is expressed at cell-cell junctions in endothelial cells, we hypothesized and tested whether JCAD regulates angiogenic processes in vitro and in vivo. APPROACH AND RESULTS: Cell culture experiments revealed impaired angiogenic ability (proliferation, migration, and cord formation) by the knockdown of JCAD with siRNA (P<0.05 versus control siRNA). We have generated mice lacking JCAD (mKIAA1462-/-) by gene-targeted deletion of JCAD to address in vivo angiogenic function. mKIAA1462-/- mice did not show morphological differences in development of retinal vasculature. Ex vivo aortic ring model demonstrated impaired neovascularization in aorta from mKIAA1462-/- mice than control wild-type mice (P<0.05). Tumor growth was assessed by monitoring tumor volume after the subcutaneous injection of melanoma, LLC (Lewis lung carcinoma), and E0771 cells into the mice. mKIAA1462-/- mice exhibited significantly smaller tumor volume compared with wild-type mice (P<0.001). Histological assessment of the tumor exhibited less smooth muscle actin-positive neovascularization determined by CD31-positive vascular structure in tumor of mKIAA1462-/- mice than wild-type mice, indicating that knockdown of JCAD inhibited the vascular maturation in pathological angiogenic process. CONCLUSIONS: These in vitro and in vivo studies suggest that JCAD has a redundant functional role in physiological angiogenesis but serves a pivotal role in pathological angiogenic process after birth.