Objective The purpose of the present study is to describe our experience with patients who have a transmural myocardial infarction (MI) in the presence of a normal coronary artery. The clinical profile, demographic characteristics and outcomes of these patients are discussed. Methods Between January 2006 and August 2011, 21 patients who presented with a Q-wave myocardial infarction were found to have normal coronary arteries. The prevalence rate of this entity was 1.5% (21 out of 1,400 Q wave MI patients). These patients were characterized by their young age (the mean age=44.95±14.86), male dominance (90.47%), and a high prevalence of smoking (85.71%). In this study, 4 patients have an evident spontaneous spasm shown on coronary angiography which disappeared after intracoronary injection of nitrates. Coagulation Disorders, such as activated protein C resistance (APC) resistance, protein C deficiency and antiphospholipid antibody syndrome were found in 4 of 12 patients who underwent systematic examination. One patient had a history of lung cancer which may be associated with a hypercoagulable state and may explain the occurrence of myocardial infarction with a normal coronary artery. The mean left ventricle ejection was 56. 5±12. The mean follow-up was 24±10 months. Six patients developed residual chest pain which was generally easily controlled by anti-spastic therapy and no patient had a major cardiovascular event. Conclusion Patients with Q-wave MI and with normal coronary arteries seem to have a good short and long-term prognosis especially when they are treated with an exclusive medical strategy.
Objective Anti-myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)- related nephritis constitutes 60% of rapidly progressive glomerulonephritis (RPGN) in Japan. The reported 1-year survival rate is over 80%, however, the long-term prognosis remains unknown. We therefore investigated the prognosis and factors affecting the clinical course of patients. Methods We retrospectively investigated 74 patients (female, n=42; median age, 73.0 years) with MPO-ANCA-related nephritis. The patients were admitted to Fukushima Medical University and two affiliated hospitals between 2000 and 2010. Results Median estimated GFR (eGFR) was 12.1 mL/min/1.73 m2 at admission. The Birmingham Vasculitis Activity Score (BVAS version 3: max 63 points) at diagnosis and at 4 weeks after start of treatment were 15.0 and 5.0, respectively. Twenty-three patients (31%) died during a median observation period of 30.5 months. Sixteen patients (22%) presented with end-stage renal disease (ESRD) at the initial phase, and needed regular dialysis therapy. Multivariate Cox proportional hazards model analysis revealed that renal death at the initial phase was a significant risk factor for all-cause death (Hazard ratio, 5.72; 95% confidence interval, 2.49-13.09; p<0.001). Furthermore, BVAS>6, evaluated 4 weeks after start of treatment, is an independent risk factor for ESRD and patient survival. Conclusion This is the first investigation to demonstrate clinical features focusing on MPO-ANCA-related nephritis. Renal death at the initial phase of treatment is a powerful risk factor for all-cause death in patients with MPO-ANCA-related nephritis. Patients at high risk of death and ESRD could be stratified according to BVAS.
Objective Japanese chronic lymphocytic leukemia (CLL) provides a diagnostic dilemma due to the low incidence and the heterogeneity shown in its morphology and immunophenotype. We clarified the diagnostic problems in Japanese CLL through our retrospective observation. Methods Between 2006 and 2011, we found a total of 48 cases with CLL and other indolent B-cell leukemias. We made a diagnosis of true CLL based on clinical, laboratory, immunophenotypic and cytogenetic data. Results Among the 48 cases, only 28 cases (58.3%) were diagnosed with true CLL. Morphologic evaluation using a forced-air dried preparation alone is not helpful to distinguish CLL from other indolent B-cell leukemias, including hairy cell leukemia, mantle cell lymphoma, lymphoplasmacytic lymphoma, and splenic marginal zone lymphoma. CLL immunophenotypic score should be more strictly applied in Japan than in Western countries. Conclusion Fluorescence in situ hybridization for CCND1/IGH, the presence of leukocytosis and lymphadenopathy at diagnosis, and the morphological evaluation using naturally air dried preparations are important clues to make a correct diagnosis of Japanese CLL.
Tuberculous aortitis is a rare disease entity indicative of disseminated tuberculosis. We report a case who presented with newly diagnosed hypertension one year after her last admission for pulmonary and pericardial tuberculosis. Chest CT and MRA, performed due to marked differences in pulse amplitude between upper and lower limbs, demonstrated long and severe segmental stenosis of the thoracic aorta. After the operation of bypass graft for the thoracic aorta, the patient recovered uneventfully. Tuberculous aortitis should be included in the list of differential diagnosis for secondary hypertension, especially if the patient has a recent disease history of pulmonary tuberculosis.
This case report describes agranulocytosis immediately after oral administration of cibenzoline and dabigatran in a 70-year-old woman with paroxysmal atrial fibrillation (AF). No blasts were found in peripheral blood and bone marrow, and the white blood cell count increased abruptly by intravenous administration of granulocyte colony-stimulation factor, suggesting an allergic response caused by cibenzoline or dabigatran, or both. Though antiarrhythmic drugs with anticoagulation therapy are commonly used to treat paroxysmal AF, caution has to be paid to drug-induced agranulocytosis.
We report a 66-year-old man with chronic hepatitis caused by hepatitis type C virus of genotype-1b and high-viral-load combined with cryoglobulinemia and advanced diabetic nephropathy in whom we successfully achieved viral removal and eradication by DFPP (VRAD). The dose of PEG-interferon was reduced to 70 mg/week due to thrombocytopenia. Rivavirin was discontinued at day 21 due to anemia. Even with treatment of PEG-interferon alone, the condition was judged to be sustained viral remission at the end of the observation. This is a successful report of VRAD in a combined case of diabetic and HCV-related cryoglobulin-nephropathy with nephrotic syndrome. The therapeutic effect of IFN seemed to be efficiently enhanced by concomitant DFPP (VRAD therapy).
A 43-year-old diabetic woman on peritoneal dialysis, developed left upper abdominal pain and culture-negative cloudy peritoneal dialysate. The dialysate had WBC counts of 1,532/μL with 90% polymorphonuclear cells. The patient did not respond well to anti-bacterial therapy. Abdominal CT scan revealed diffuse atherosclerosis in the abdominal vessels and wedge-shaped splenic infarction. Anticoagulation therapy was initiated and an improvement in peritonitis was observed without peritoneal catheter removal. Thus, in peritoneal dialysis patients with diffuse atherosclerosis or the risk of systemic embolization, symptoms of unexplained left upper quadrant pain and culture-negative peritonitis should be evaluated to rule out splenic infarction.
A 62-year-old splenectomized woman was admitted because of upper respiratory tract symptoms, general fatigue, and purpura. Laboratory data demonstrated microangiopathic hemolytic anemia, thrombocytopenia, acute renal failure, and a positive Streptococcus pneumoniae (SP) urinary antigen test. A renal biopsy showed thrombotic microangiopathic changes. She was diagnosed with hemolytic uremic syndrome (HUS) secondary to SP infection. Methylprednisolone pulse therapy in addition to antibiotic therapy led to prompt improvement of her symptoms and laboratory abnormalities. This is the first adult case of SP-associated HUS successfully treated without hemodialysis. SP infection should be considered as a causative etiology in all splenectomized patients with HUS.
Birt-Hogg-Dubé syndrome is a rare autosomal dominant condition caused by a germline mutation in the folliculin gene, which is characterized by skin fibrofolliculomas, multiple lung cysts and renal cancer. The clinical expression of the syndrome is highly variable, with recurrent pneumothoraces due to ruptured lung cysts in many cases. We report a patient with pneumomediastinum and cervico-facial emphysema after severe coughing without pneumothorax, skin lesions or renal tumour, but a striking family history of lung abnormalities.
Pneumothorax associated with chronic graft-versus-host disease (cGVHD) after stem cell transplantation is a rare complication. Autologous blood has been used successfully for pleurodesis, which was less toxic than chemical agents. However, when pneumothorax is resistant to pleurodesis, no other procedure is more effective and conservative. Here, we describe a case of myelodysplastic syndromes complicated with cGVHD-related pneumothorax. His pneumothorax has been resistant to pleurodesis using autologous blood and was treated successfully with fibrin glue sealant. In our limited experience, we believe the best success could be achieved when this method is used to treat persistent pneumothorax with cGVHD.
Here we report the first case of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), who initially presented with peripheral neuropathy. Nerve conduction, cerebral spinal fluid studies and his clinical course were compatible with sub-acute demyelinating polyradiculoneuropathy. In addition, left cervical lymph node swelling was observed on admission. Diagnosis of PTCL-NOS was made by the histological, immunohistochemical, and Southern blot analyses on the biopsy specimen from the enlarged lymph node. Combination chemotherapy composed of cyclophosphamide, vincristine, doxorubicin and prednisolone (CHOP) was effective for polyneuropathy as well as for lymphoma. Several antibodies relating to paraneoplastic syndrome such as Ma1, Ma2, Amphiphysin, CV2, Ri, Yo and Hu were all negative. Because sural nerve biopsy performed prior to CHOP therapy revealed no infiltration of lymphoma cells, immune dysfunction mediated by some cytokine or unidentified autoantibody related to PTCL-NOS was thought to be involved in the polyradiculoneuropathy.
A 53-year-old Japanese woman presented with myoclonus during the course of Guillain-Barré syndrome. The myoclonus was characterized by relatively regular involuntary movements, starting from proximal muscles of the right lower leg, and moving almost simultaneously towards the left lower leg and upper trunk. Surface electromyography revealed rhythmic synchronous discharges with 100-200 ms duration in the agonist and antagonist muscles at approximately 4 Hz. The jerk-locked back averaging, long latency reflexes, and somatosensory evoked potentials studies were normal. We report myoclonus due to radiculitis in a patient with Guillain-Barré syndrome.
A 72-year-old man presented with dizziness and left hand muscle atrophy. Magnetic resonance imaging revealed a spinal cord cavity and descent of the cerebellar tonsils. His diagnosis was Chiari I malformation with syringomyelia. No cerebellar signs were observed on physical examination. The cause of dizziness was investigated using a video-based eye movement tracker, which revealed a downward smooth pursuit velocity gain significantly below normal when expressed relative to the horizontal pursuit velocity gain. Vestibulocerebellar damage can cause mild downward pursuit deficit. The downward to horizontal smooth pursuit velocity gain ratio may be a more sensitive means of detecting vestibulocerebellar damage early.
We report two cases of isolated unilateral pupil-sparing partial fascicular oculomotor paresis. Patient 1 was a 72-year-old man who developed left-sided palsy of the inferior rectus muscle (IR), medial rectus muscle (MR), superior rectus muscle (SR), inferior oblique muscle (IO), and levator palpebrae superioris (LP) due to infarction of the left paramedian thalamic artery. Patient 2 was a 70-year-old woman who developed right-sided palsy of MR, SR, IO and LP due to infarction of the right superior paramedian mesencephalic artery. These results suggest that the fibers to IR may be located in the most rostral portion of the oculomotor fascicles.
We encountered a disseminated sporotrichosis patient with polyarthritis and progressive skin ulcers, who had been previously treated with prednisolone, tocilizmab, tacrolims, and cyclophosphamide under the diagnosis of rheumatoid arthritis in another hospital. Making the diagnosis of leukocytoclasticvasculitis based on the clinical observation of skin ulcers, we intensified immunosuppressive therapy. Unfortunately, the patient developed septic shock. Blood culture revealed that the pathogenic organism was sporothrixschenckii. Any case of intractable arthritis or skin ulcers, which does not improve, despite adequate immunosuppressive therapy, is likely to be suspicious of sporotrichosis.
A dipeptidyl peptidase (DPP)-4 inhibitor, commonly used to treat patients with type 2 diabetes, has caused concern because of immune system side effects. We report a 48-year-old woman with type 2 diabetes who was diagnosed with rheumatoid arthritis (RA) after continued polyarthritis and an increase in rheumatoid factor up to 86 IU/mL after three months of treatment with sitagliptin, a DPP-4 inhibitor. The shared epitope (SE)-containing human leukocyte antigen (HLA)-DRB1 alleles, which are important predisposing factors for RA, were positive. RA might have been triggered by sitagliptin due to a predisposing condition.
A 50-year-old man presented with a 12 kg weight loss in 8 months. Upper gastrointestinal endoscopy findings showed strong erosion and diffuse bleeding in the duodenum. Histopathological findings showed PAS staining-positive macrophages consistent with Whipple's disease. He was treated with trimethoprim-sulfamethoxazole. His condition initially improved. However, during his 6-year course of treatment he developed a central nervous system relapse. Tropheryma whipplei DNA was detected by a polymerase chain reaction in his cerebrospinal fluid. This relapse was successfully treated with ceftriaxone sodium (CTRX). We considered that as initial therapy for Whipple's disease, it would be important to administer CTRX for at least a few months, due to its high translatability to CSF.
We report a 54-year-old man with osteomalacia due to a bladder reconstruction performed 35 years previously. He had had slowly progressive chest and back pain for 18 months. Osteomalacia due to metabolic acidosis was suspected based on hyperalkalinephosphatasemia and a high serum chloride level, and the diagnosis was confirmed by bone scintigraphy. His symptoms and blood electrolyte levels were improved by oral medication, including sodium hydrogen carbonate. Measurement of the serum chloride level is simple and useful for evaluating acidosis, for which a regular blood test is essential in patients who have undergone bladder reconstruction.