A case of juvenile-onset ankylosing spondylitis effectively treated with tumour necrosis factor-alpha inhibitor agents.

A 15-year-old girl had experienced hip pain at 11 years of age. At 15 years of age, the patient complained of persistent generalised pain. Her rheumatoid factor and serum matrix metalloproteinase-3 levels were below standard values; there were no inflammatory responses, and the human leukocyte antigen test was negative for B27 and positive for B52 and B62. The bath ankylosing spondylitis disease activity index (BASDAI) value was 8.0 at the time of induction and 3.1 at 6 months after the introduction of adalimumab (at a dose of 40 mg). The BASDAI value improved with an increase in the dose of adalimumab to 80 mg at 8 months after the initial introduction of adalimumab (at 40 mg), although it remained at 4.8 at 16 months after the dose increase. The BASDAI value was 2.6 at 6 months, 2.7 at 1 year, and 1.8 at 1.5 years after the introduction of infliximab, indicating that the patient had progressed well without any adverse events. Based on this case, juvenile ankylosing spondylitis is a differential diagnosis for low back pain and generalised pain since childhood. Tumour necrosis factor (TNF) inhibitors were promptly introduced in this case, although it took 4 years from the initial presentation. TNF inhibitors were effective in treating juvenile ankylosing spondylitis in the present case without any adverse events. This case is notable because juvenile onset ankylosing spondylitis is one of the reasons for severe lumbago since childhood and because TNF inhibitors were administered promptly after diagnosis.

Percutaneous aspiration embolectomy is beneficial for pulmonary thromboembolism in adult patients with Fontan circulation.

In patients with acute pulmonary thromboembolism (PTE), increased pulmonary vascular resistance (PVR) due to thrombus sometimes causes hemodynamic instability, requiring reperfusion therapy with drugs, surgery, or catheterization. In particular, patients with Fontan circulation, which is strongly affected by PVR, are prone to hemodynamic instability. Moreover, these patients sometimes have bleeding complications such as hemoptysis and intrathoracic adhesions, following multiple prior thoracotomies, making it difficult to choose pharmacotherapy. Percutaneous aspiration embolectomy (PAE) is a useful treatment option because it minimizes bleeding complications, is less invasive, and more rapid than surgery, and is easy to perform. Herein, we report two cases of Fontan patients having PTE treated with PAE. In Case 1, a 21-year-old man with a history of a Fontan procedure suddenly developed acute PTE, for which anticoagulants were administered immediately. However, his condition was refractory to treatment and he underwent PAE on the third day of illness. In Case 2, a 28-year-old woman with a history of Fontan procedure who had been on anticoagulants, developed acute PTE on the ninth postpartum day, and underwent PAE on the day of onset. In both cases, the respiratory condition improved, and re-treatment for PTE was not required. Learning objective: Fontan patients with acute pulmonary thromboembolism often require reperfusion therapy because they can easily become hemodynamically unstable due to increased pulmonary vascular resistance. For them, who often have adhesions following multiple prior thoracotomies and bleeding complications, percutaneous aspiration embolectomy may be effective as it has minimal bleeding risk and is minimally invasive, rapid, and straightforward.

Ankle arthrodesis using a retrograde intramedullary rod with fins for highly destructive joints in elderly patients with rheumatoid arthritis: A report of two cases.

Herein, we present two cases of older adult patients with highly destructive changes in ankle joints (Larsen grade IV) who underwent retrograde intramedullary ankle nail fixation with fins. In both patients, bony union was achieved, and full weight-bearing was attained at 3 months after surgery.

Clinical characteristics and treatment status of pustulotic arthro-osteitis: A single-center study involving 51 cases.

Pustulotic arthro-osteitis (PAO) is a major complication of palmoplantar pustulosis (PPP). In orthopedic surgery outpatient clinics, PPP patients with osteoarticular symptoms are seen frequently, but PAO's clinical features remain not well known. To determine Japanese patients' clinical features and treatment status with PAO, we conducted a single-center retrospective epidemiologic survey. Clinical features, including gender, age, smoking habit, the onset pattern, interval between skin manifestation and osteoarticular symptoms, and the incidence of sternoclavicular joint lesions, axial and peripheral joint lesions, were examined. The association between physical status and image findings by X-ray, computed tomography, bone scintigraphy with Technetium99 , or magnetic resonance imaging was evaluated. The distribution pattern of peripheral joint lesions and the treatment status were evaluated. We identified 51 patients, 10 men and 41 women, with PAO. The average age was 48 years and 59% were smokers. The frequency of onset patterns was skin-leading type (63%), simultaneous onset (18%), and osteoarticular leading type (16%). The average interval between skin involvement and osteoarticular involvement in skin-leading type was significantly longer than that in osteoarticular leading type (7.1 years vs. 2.0 years). A sternoclavicular joint (SCJ) lesion was detected in 65% cases, and the physical findings of SCJ were significantly related to the image findings. Axial and peripheral joint lesions were detected in the same ratio (23 cases, 45%). In the peripheral joints, the finger joint was the most common (26%), followed by the shoulder joint (21%). Patients were treated with nonsteroidal anti-inflammatory drugs (76%), followed by conventional synthetic disease-modifying antirheumatic drugs (DMARDs) (29%) and biological DMARDs (9.8%). Tonsillectomy was performed in 11 cases. In conclusion, PAO more frequently involves SCJ in middle-aged women who smoke. Given that osteoarticular leading type was detected in 16% cases, seronegative oligoarthritis patients should be monitored for PPP, leading to a diagnosis of PAO.

The JNK pathway represents a novel target in the treatment of rheumatoid arthritis through the suppression of MMP-3.

BACKGROUND AND AIM: The pathophysiology of rheumatoid arthritis (RA) is characterized by excess production of pro-inflammatory cytokines, including tumor necrosis factor-α (TNF-α), interleukin-1ß (IL-1ß), and interleukin-6 (IL-6) by neutrophils and macrophages in synovium. Additionally, these cytokines promote the production of reactive oxygen species (ROS), and increased production of matrix metalloproteinases (MMPs), including MMP-3, in synoviocytes that result in joint destruction. There is limited information on how proteolytic enzymes such as MMP-3 can be regulated. We evaluated the effect of the antioxidant N-acetylcysteine (NAC) on RA and identified the relationship between the c-Jun N terminal kinase (JNK) pathway and MMP-3. We hypothesized that elucidating this relationship would lead to novel therapeutic approaches to RA treatment and management. METHODS: We investigated the effect of administering a low dose (1000 µM or less) of an antioxidant (NAC) to human rheumatoid fibroblast-like synoviocytes (MH7A cells). We also investigated the response of antioxidant genes such as nuclear factor erythroid -derived 2-related factor 2 (Nrf2) and Sequestosome 1 (p62). The influence of MMP-3 expression on the JNK pathway leading to joint destruction and the mechanisms underlying this relationship were investigated through primary dispersion culture cells collected from the synovial membranes of RA patients, consisting of rheumatoid arthritis-fibroblast-like synoviocytes (RA-FLS). RESULTS: Low-dose NAC (1000 µM) increased the expression of Nrf2 and phospho-p62 in MH7A cells, activating antioxidant genes, suppressing the expression of MMP-3, and inhibiting the phosphorylation of JNK. ROS, MMP-3 expression, and IL-6 was suppressed by administering 30 µM of SP600125 (a JNK inhibitor) in MH7A cells. Furthermore, the administration of SP600125 (30 µM) to RA-FLS suppressed MMP-3. CONCLUSIONS: We demonstrated the existence of an MMP-3 suppression mechanism that utilizes the JNK pathway in RA-FLS. We consider that the JNK pathway could be a target for future RA therapies.

Usefulness of Transcranial Doppler for Detecting Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia.

The aim of this study was to assess transcranial Doppler (TCD) as a screening test for pulmonary arteriovenous malformation (PAVM) in patients with hereditary hemorrhagic telangiectasia (HHT). This retrospective study included suspected patients with HHT who were screened for PAVM with a TCD and a chest computed tomography (CT) study. The results of TCD and CT were compared to evaluate the usefulness of TCD for detecting PAVM. A TCD Spencer grade ≥3 was defined as positive for a significant right-to-left shunt (RLS). The diameter of the pulmonary arteries feeding the PAVM was measured by calipers from the CT study. In 86 subjects from 74 families with HHT, the sensitivity of TCD for identifying a PAVM at rest was 98% and post-Valsalva was 100%. Specificity was 58% and 35%, respectively, presumably due to pulmonary shunts too small to recognize on CT. Of the patients with HHT who were referred for embolization therapy for their PAVMs, all 20 had TCD grade ≥3. In patients who were diagnosed with a PAVM by chest CT, patients with TCD grade ≥5 had a significantly larger sum of artery diameters feeding the PAVMs compared to those with grade ≤4 (5.0 ± 3.2 mm vs 2.6 ± 1.9 mm, p = 0.01). In conclusion, a TCD examination for evaluating RLS is sensitive for identifying PAVM in patients with HHT and is useful in quantitating the degree of RLS flow. The sensitivity of the TCD examination makes it a useful screening test without radiation in HHT subjects to determine which patients need to undergo CT evaluation to identify PAVMs.

Three-dimensional echocardiography guided closure of complex multiple atrial septal defects.

Transcatheter closure of complex multiple atrial septal defects (ASDs) remains a challenge. We describe our clinical experience with staged device deployment in a patient with multiple ASDs using four Amplatzer septal occluder devices. Three-dimensional transesophageal echocardiography imaging contributed not only to the therapeutic guidance of successful device deployment but also to the decision making for the staged device delivery approach in a case of morphologically complex multiple ASDs.

Cardiac erosion after catheter closure of atrial septal defect: Septal malalignment may be a novel risk factor for erosion.

Pericardial tamponade occurred 3 days after the catheter closure of an atrial septal defect (ASD) using Amplatzer Septal Occluder (St. Jude Medical, St. Paul, MN, USA). Before the closure, two-dimensional and real-time three-dimensional transesophageal echocardiography demonstrated a deficient aortic rim and atrial septal malalignment. Perforation of the right atrium toward the non-coronary sinus of the aortic root was confirmed at the emergent surgery. Cardiac erosion is one of the most catastrophic complications in ASD patients undergoing catheter closure with Amplatzer Septal Occluder. Hence, several risk factors for this complication are discussed and identified. Oversized device deployment and a deficient aortic rim are accepted factors potentially causing cardiac erosion. Besides, atrial septal malalignment, which is a morphological characteristic of ASD, may be a novel risk factor for cardiac erosion. .

Torn atrial septum during transcatheter closure of atrial septal defect visualized by real-time three-dimensional transesophageal echocardiography.

Transcatheter closure of atrial septal defects (ASDs) has become an accepted and reliable procedure. Although various complications have been recognized, tear of the atrial septal rim is a rare complication. We report a case of atrial septal rim tear that was diagnosed during the procedure by real-time three-dimensional transesophageal echocardiography (TEE). The device was successfully implanted 3 months after the initial intervention. RT3D TEE is more useful for displaying the entire shape of the defect and its spatial relationship (RT3D) with its neighboring structures compared with conventional two-dimensional echocardiography. By using both two-dimensional and RT3D TEE images, especially in cases with complicated ASD morphology, both the echocardiologist and interventionalist gain valuable information on the morphology of the ASD before and during the procedure.

Application of real-time three-dimensional transesophageal echocardiography using a matrix array probe for transcatheter closure of atrial septal defect.

BACKGROUND: The aim of this study was to demonstrate the utility of real-time three-dimensional (3D) transesophageal echocardiography (RT3D-TEE) using a matrix array 3D transesophageal echocardiographic probe for morphologic evaluation and guidance of transcatheter closure of atrial septal defects (ASDs). METHODS: Forty-eight consecutive patients scheduled for the intervention were included. Two-dimensional (2D) transesophageal echocardiography (2D-TEE) and RT3D-TEE were performed before and during the procedures. Measurements of maximal ASD diameter and surrounding rims obtained on RT3D-TEE were compared with those obtained on 2D-TEE. RESULTS: In 46 patients (96%), optimal 3D images for the morphologic evaluation of ASDs were obtained. RT3D-TEE facilitated the evaluation of ASD morphology and surrounding rims and was able to provide intraprocedural information clearly. A Bland-Altman plot showed a mean maximal diameter difference of -0.12 mm between the means (95% limits of agreement, -2.2 to 2.5 mm). CONCLUSION: RT3D-TEE is a clinically useful, complementary option to 2D-TEE for evaluation of ASD morphology and for interventional guidance.

Right atrial thrombosis as a complication of arrhythmogenic right ventricular cardiomyopathy.

A 65-year-old woman was admitted to our hospital due to palpitation. Electrocardiogram (ECG) showed ventricular tachycardia originating from the right ventricle, and transthoracic echocardiography revealed dilatations of the right atrium and ventricle. The diagnosis of arrhythmogenic right ventricular cardiomyopathy was made. Eleven months later, echocardiography revealed a solid thrombus (36x32 mm) attached to the free wall of the right atrium, and it was surgically resected. Four months after the operation, a solid thrombus (48x30 mm) appeared again at the same site despite anticoagulant treatment. The patient died of both left and right heart failure 33 months after the operation.

Temporary overdriving pacing as an adjunct to antiarrhythmic drug therapy for electrical storm in acute myocardial infarction.

A-55-year-old man with diabetes mellitus was admitted to hospital because of chest pain. He was diagnosed as anterior acute myocardial infarction and treated with stent placement. After 7 days, ventricular fibrillation occurred because of a subacute reocclusion and balloon angioplasty was performed. Despite reperfusion therapy, intraaortic balloon pumping, antiarrhythmic drugs and beta-blocker, ventricular tachycardia or fibrillation relapsed and cardioversion was performed 29 times during 32 h. Temporary overdrive atrioventricular sequential pacing was initiated and the malignant arrhythmia finally disappeared. Even after stoppage of 25 h overdride pacing, it never recurred. Temporary overdrive pacing is an easy and feasible therapy for a drug-resistant electrical storm associated with AMI and should be performed in the early stage.