Synovial osteochondromatosis of the hip treated through a surgical dislocation

We report a case of osteochondromatosis in the hip joint treated through a surgical dislocation and describe the use and safety of the procedure confirmed by postoperative magnetic resonance imaging. A 44-year-old man had right hip pain with moderate limitation of the range of motion. Plain radiographs and computed tomography showed calcified loose bodies in the hip with mild joint degeneration. Magnetic resonance imaging showed remarkable expansion of the capsule containing joint fluid and loose bodies below the femoral head. Complete removal of loose bodies and synovectomy were performed through a surgical dislocation. Three months after the operation, the patient had fully recovered and returned to his job. Magnetic resonance imaging performed 5 months after surgery revealed full removal of the loose bodies and no recurrence of the disease including synovial proliferation. It also showed no evidence of avascular necrosis, meaning that the procedure had been performed safely.     

Bcl-2 protects tubular epithelial cells from ischemia reperfusion injury by inhibiting apoptosis

Ischemia followed by reperfusion leads to severe organ injury and dysfunction. Inflammation is considered to be the most important cause of graft dysfunction in kidney transplantation subjected to ischemia. The mechanism that triggers inflammation and renal injury after ischemia remains to be elucidated; however, cellular stress may induce apoptosis during the first hours and days after transplantation, which might play a crucial role in early graft dysfunction. Bcl-2 is known to inhibit apoptosis induced by the etiological factors promoting ischemia and reperfusion injury. Accordingly, we hypothesized that an augmentation of the antiapoptotic factor Bcl-2 may thus protect tubular epithelial cells by inhibiting apoptosis, thereby ameliorating the subsequent tubulointerstitial injury. We examined the effects of Bcl-2 overexpression on ischemia-reperfusion (I/R) injury using Bcl-2 transgenic mice (Bcl-2 TG) and their wild-type littermates (WT). To investigate the effects of I/R injury, the left renal artery and vein were clamped for 45 min, followed by reperfusion for 0-96 h. Bcl-2 TG exhibited decreased active caspase protein in the tubular cells, which led to a reduction in TUNEL-positive apoptotic cells. Consequently, interstitial fibrosis and phenotypic changes were ameliorated in Bcl-2 TG. In conclusion, Bcl-2 augmentation protected renal tubular epithelial cells from I/R, and subsequent interstitial injury by inhibiting tubular apoptosis.     

Focal segmental glomerulosclerosis with intramembranous vesicle-like microstructures and podocytic infolding lesion

A 42-year-old woman was admitted to Kyushu University hospital because of 6 months' history of bilateral leg edema. Upon admission, ascites and pleural effusion as well as systemic edema were noted. Laboratory tests revealed hypoalbuminemia of 1.5 g/dl and massive proteinuria of 10 g/day. She was diagnosed with nephrotic syndrome. Renal biopsy revealed diffuse thickening of the glomerular basement membrane (GBM) and a crescent-like extracapillary lesion with segmental sclerosis in four of 11 glomeruli. Immunoglobulins and complements were negative by immunofluorescence examination. Therefore, we diagnosed this as focal segmental glomerulosclerosis (FSGS) rather than membranous nephropathy. Using an electron microscope, we observed a thickening of the GBM with numerous intramembranous vesicle-like microstructures and an infolding of the podocyte into the GBM. Since the microstructures were partly demarcated by a unit membrane and some of them were located very closely to the infolded podocyte, we speculated that the microstructures were derived from the podocyte. The unique electron microscopic finding of our case is a disease entity rather than a reactive phenomenon.     

Adrenal ganglioneuroma: a case report

A patient with an incidentally diagnosed adrenal ganglioneuroma is reported. A 37-year-old man who underwent abdominal computed tomography (CT) in the course of evaluating liver dysfunction was found to have a right adrenal tumor. Laboratory data including results of endocrinologic tests were normal except for a slight elevation of plasma aldosterone. With a preoperative diagnosis of non-functioning right adrenal tumor, resection was performed. The tumor specimen was noncystic weighing 150 g and measuring 10 x 8 x 3 cm. The histopathologic diagnosis was ganglioneuroma originating from the adrenal gland. Adrenal ganglioneuroma is relatively rare, 147 cases including ours have been reported in Japan. Increasing numbers of these tumors are being found incidentally by ultrasonography or CT. Ganglioneuroma is a benign tumor, and disagreement exists concerning diagnosis and indications for surgery.     

Experience of temporary inferior vena cava filters inserted in the perinatal period to prevent pulmonary embolism in pregnant women with deep vein thrombosis

OBJECTIVE: We placed temporary inferior vena cava filters to prevent pulmonary thromboembolism in patients with deep vein thrombosis (DVT) who were presumed to have an increased risk of pulmonary embolism in the perinatal period. These experiences of using temporary inferior vena cava filters in pregnant women are reported. METHODS: We reviewed 11 patients with DVT who underwent placement of a temporary inferior vena cava filter and delivered in our hospital between 1998 and 2004. All of the filters were placed at the suprarenal inferior vena cava before delivery. During filter placement, anticoagulant therapy was routinely performed, and we stopped the administration of anticoagulant agents intrapartum. RESULTS: No complications occurred at filter insertion or during placement. No symptomatic pulmonary thromboembolism occurred during or after delivery. All of the filters were successfully removed, one of which was exchanged for a permanent filter because the temporary filter captured a large thrombus. CONCLUSION: Intrapartum temporary inferior vena cava filters may reduce the incidence of pulmonary thromboembolism in pregnancy with DVT. Temporary inferior vena cava filters appear to be safe for pregnant women.     

Clinical significance of endothelial vasodilatory function evaluated by EndoPAT in patients with systemic sclerosis

Endothelial dysfunction is a hallmark of vasculopathy associated with systemic sclerosis (SSc). Reactive hyperemia peripheral arterial tonometry is a rapid and non-invasive technique to assess peripheral microvascular endothelial function by measuring changes in digital pulse volume during reactive hyperemia. Low scores of the reactive hyperemia index (RHI) imply an impaired vasodilatory response and, accordingly, impaired endothelial and vascular health. To investigate the clinical significance of the RHI in SSc patients, RHI values were measured in 43 SSc patients and 10 healthy controls. In diffuse cutaneous SSc (dcSSc) patients, RHI values were significantly decreased compared with healthy controls, and inversely correlated with disease duration. In total SSc patients, there was a significant inverse correlation between RHI values and skin score, and interstitial lung disease was associated with the decrease in RHI values. Among vascular symptoms, the current and past history of digital ulcers was seen more frequently in patients with decreased RHI values than in those with normal RHI values. Although no SSc patients had pulmonary arterial hypertension, an inverse correlation was evident between RHI values and mean pulmonary arterial pressure measured by right heart catheterization. These results indicate that the decrease in RHI values is associated with skin fibrosis, interstitial lung disease, digital ulcers and pulmonary vascular involvement leading to pulmonary arterial hypertension, supporting the canonical idea that endothelial dysfunction is a critical event underlying the development of tissue fibrosis and vascular complications in SSc.     

Effects of long-term administration of oxybutynin hydrochloride (KL007) for the treatment of neurogenic bladder and unstable bladder

The efficacy and safety of long-term administration of oxybutynin hydrochloride in patients with neurogenic bladder and unstable bladder, who complained of urinary frequency, urgency and incontinence, and whose bladder was proved to be uninhibited, reflex, and/or low compliant, were studied at the multi-center hospitals, and the following results were obtained. This study comprised 149 cases but 8 of them were excluded because of the incomplete protocol. Thus, 141 cases (104 neurogenic bladder patients, 33 unstable bladder patients and 4 others) were investigated. The daily dosage of Oxybutynin hydrochloride ranged from 1 to 18 mg, and averaged at 6.6 mg. Since a small daily dosage of 3 mg was administered in 32 cases to examine the minimum effective dosage of the drug, the low average dosage possibly resulted. The clinical optimal dosage seemed to be more than 6.6 mg. The average period of administration in all cases was 161.7 days (1-336 days), and the drug was discontinued in 46 cases (33.6%) on average 62.1 days (1-141 days). The rate of global improvement by this drug estimated at the time of completion was found to be 65.6% with excellent and good and 87.8% with excellent, good and fair. The efficacy of this drug was stable and not decreased during the long-term test period. As to the objective findings studied before, during and after the drug administration, cystometric bladder capacity was significantly increased both first desire to void and the total capacity. Voided volume and residual urine were also increased, but there was no change in the rate of residual urine. Side effects were observed in 37 (26.2%) of 141 cases, and mainly gastrointestinal signs such as dry mouth and constipation. Urological signs such as dysuria and urinary retention were experienced in 8 cases. Regarding the findings of laboratory tests, there were no abnormality except for small changes of some items in normal range. Six children who were younger than 15 years old were subjected to this study. The results of evaluation were similar to those obtained on adults and no side effects were observed. From these findings, oxybutynin hydrochloride is considered an effective and useful drug in patients suffering from neurogenic bladder and unstable bladder with over active bladder condition.     

Neoadjuvant chemoradiotherapy in locally invasive transitional cell carcinoma of the urinary bladder: early results of PVB and CAP therapy

A total of 22 patients with locally invasive transitional cell carcinoma of the urinary bladder were treated with neoadjuvant cis-diamminedichloroplatinum (CDDP), vincristine, peplomycin (PVB) or cyclophosphamide, doxorubicin, CDDP (CAP) combined with radiation therapy in our institutes between June, 1982 and May, 1988. Twelve patients were entered into the PVB regimen and the remaining 10 patients into the CAP regimen. In the PVB treated group, clinical response was obtained in 2 complete response (CR) and 6 partial response (PR), 8 out of 12 patients (66.7%). Downstaging was noted in 8 out of 12 patients (66.7%). In the CAP treated group, a clinical response was obtained in 1 of CR and in 2 of PR out of the 9 patients with evaluable lesions (33.3%). Downstaging was noted in 6 out of 9 patients (66.7%). There were no significant side effects in either the PVB or CAP treated groups, and these neoadjuvant therapies were well-tolerated. These results indicated that neoadjuvant PVB or CAP combined with radiation therapy would be useful in the management of invasive bladder cancer.