Persistent T-cell activation and clinical correlations in patients with ANCA-associated systemic vasculitis.

BACKGROUND: Although in antineutrophil cytoplasmic autoantibodies (ANCA)-associated systemic vasculitis (AASV) patients, activation of T-cells has been described, persistence of these alterations has not been well characterized. This study was conducted to define persistent T-cell activation (PTA) in AASV patients and to assess whether this correlates with disease activity, disease severity, age or therapy. METHODS: The expression of CD4, CD45RO, CD25, CD26, CD28, CCR7 and HLA-DR was examined longitudinally in 38 consecutive AASV patients. Clinical parameters were compared by univariate and multiple analysis and Kaplan-Meier curves for relapse-free survival were calculated. RESULTS: PTA could be defined as either of two activation phenotypes, i.e. a low percentage of CD4+ CD45RO- T-cells or a high percentage of CD25 in the na?ve CD4+ population (n = 26), since only these phenotypes were stable over time and were not associated with active disease. In patients with PTA, major organ involvement was significantly more often found than in patients without PTA. Moreover, the cumulative cyclophosphamide dose (26.86 vs 8.53 P < 0.01) was significantly increased in these patients, suggesting that PTA was associated with disease severity. In general, patients with PTA were older than those without (62.92 +/- 9.4 years vs 48.42 +/- 16.9 years respectively, P < 0.01). PTA was independent of disease duration. Interestingly, patients with a low percentage of CD4+CD45RO- T-cells were significantly more often diagnosed as microscopic polyangiitis (P < 0.01). CONCLUSION: We identified two independent phenotypes of T-cell activation in AASV patients. These phenotypes are persistent and do not reflect disease activity. PTA predominantly occurs in patients with severe disease. This might explain the higher cumulative cyclophosphamide dose found in these patients.     

Clinical investigation of 9 cases of urachal carcinoma]

Recently, we encountered 9 cases of urachal tumor and conducted clinical studies on its diagnosis and treatment. The chief complaint was gross hematuria in all cases. Computed tomographic scan was performed in 7 cases, and in all cases it revealed a tumor between the dome of the bladder and the Retzius' cavity. This finding strongly suggested a urachal tumor before pathological study. Magnetic imaging resonance (MIR) was performed in one of the 9 cases, and it indicated the extent of the tumor more clearly than computed tomography. Partial cystectomy + en bloc removal of the umbilicus and urachus was performed in 5 cases, radical cystectomy + en bloc removal of the umbilicus and urachus was performed in 2 cases, and radical cystectomy was performed in 2 cases. As postoperative therapy, radiotherapy was administered to 4 cases, and chemotherapy was administered to 4 cases. One case died from cancer after 4 years and 10 months, while the other 8 cases are being treated on an outpatient basis without any local recurrence. All 5 patients who underwent partial cystectomy are alive (mean survival time: 97 months). Thus we believe that cure can be achieved by partial cystectomy and adjuvant therapy. In addition, serum carcinoembryonic antigen is thought to be useful as an indicator of residual tumor or local recurrence.     

Synchronous and multiple transitional cell carcinoma of the bladder and urachal cyst

Abstract:   Incomplete involution of the allantoic duct can result in different pathological forms of urachus which can give rise to inflammation or late malignant changes. Among urachal tumors, adenocarcinoma is most frequent, although other histological types can also be found. The synchronous presentation of a urachal transitional cell tumor, along with recurrent superficial bladder tumors has not been reported previously. We are reporting a 49-year-old male patient in whom transitional cell carcinoma of a urachal cyst was found with recurrent, multiple bladder tumors. The diagnosis of urachal cyst tumor was established according to ultrasonography and computed tomography. Most of the bladder tumors were resected transurethrally while open surgical excision of the urachal cyst with en bloc resection of the bladder dome was performed. Recurrent bladder tumors were afterwards treated with Bacillus Calmette Guerin (BCG) instillations. A year after surgery the patient has no signs of local recurrence or distant metastases of transitional cell carcinoma.     

Extra-intracranial anastomosis: radio-clinical correlations]

25 patients have been operated on by means of extra-intracranial anastomosis (22 with STA-MCA anastomosis, 3 with occipital-MCA anastomosis). 23 patients underwent an angiographic study early after surgery (two weeks). The patency rate is 14 out of 23 (13 STA, 1 occipital anastomosis). In comparison of its pre-operative size, the afferent artery has enlarged in the most cases, particularly in patients with complete obliteration, either of the carotid artery, or of the middle cerebral artery. In every case, only a limited part of the MCA territory is visualized through the anastomosis. In no case was the complete MCA field visualized; the frontal branches, particularly, are not supplied through a temporal anastomosis. In the case of occipital anastomosis, both upper and lower branches of MCA are supplied through the new channel. 11 patients underwent a second angiographic study, from one year through 28 months after the first one. In three patients with no patency on the first angiography the anastomosis remained non patent. So, in this series, no anastomosis was seen to became patent secondarily. In 8 patients with patency on the first control, the anastomosis remained patent on the second angiography. In patients with a pre-operative stenosis, no increasing of the size of the vessel could be noticed. The filling of the MCA branches is difficult to be discussed, for in these cases, the angiographies were not performed selectively through the external carotid artery. In patients with a pre-operative thrombosis, an enlarging of the vessels was seen, as well as an extension of the intra-cranial filling through the anastomosis. Clinical correlations are the following ones: the most patients with TIA's had a stenosis. They presented no increasing of the size of the vessels. They were doing well after operation, as if a little more of blood supply was sufficient to improve the general blood perfusion. Every patient with stroke had a pre-operative thrombosis and presented an enlarging of the vessels with a better filling on the second angiography, as if a great deal of additional blood supply was required; the clinical improvement is slow (3 out of 5) and remains often incomplete.     

Clinical images. Massive Meckel's enterolith mimicking urachal carcinoma

Meckel's enterolith is a rare clinical entity that may be found on imaging and at surgery, as seen in this case of a 68-year-old man presenting for esophagogastrectomy. Images are presented with differential diagnosis and treatment choices.     

Abscess of urachal remnant mimicking urinary bladder neoplasm.

Three cases of urachal abscess with extensive interstitial inflammation and fibrosis are reported. Clinically, all patients had a lower abdominal mass and CT scan and ultrasonic examination revealed a large tumour located anterior or superior to the bladder. They all underwent laparotomy and had either a partial cystectomy or an en bloc resection of the bladder and adjacent organs due to severe adhesions. Microscopic examination of the specimens revealed no evidence of carcinoma and only abscess formation with extensive chronic interstitial inflammation and fibrosis were seen. In one case, residual columnar epithelium suggestive of urachal origin was identified in the abscess cavity. Urachal abscess should be considered when dealing with lower abdominal masses.     

Structural correlations between dimensions of the trunk]

Almost all the great dimensions of the trunk are correlated. Some of them correspond to the social stratification of the biological characteristics, others to anatomical structures, as shown by the study of partial correlations for a given stature, or by multiple partial correlations for a given stature and weight.     

Persistent hypoglossal artery.

During embryological development, the hypoglossal artery serves as a transient anastomosis between the internal carotid artery and the vertebro-basilar system. This artery occasionally persists into adult life. Two cases of persistent hypoglossal artery we have recently encountered are reported here. The embryology, diagnosis and potential problems for carotid surgery are discussed.     

Persistent fetal ureter.

Six patients have been seen with a peculiar ureteral lesion that has not been described previously. The lesion is characterized by either local segmentation or convolution and may represent either a persistent fetal ureter or a form of ureteral dysgenesis. Proximal dilatation, when present, has been less than the original radiographic appearance of high grade obstruction would lead one to expect. The ureteral lesions have disappeared progressively, suggesting that a period of conservative observation is warranted in similar situations.     

Treatment of urachal anomalies: a minimally invasive surgery technique.

BACKGROUND: Urachal disease is uncommon. The surgical treatment consists of the resection of the urachus throughout its entire length. Our objective is to demonstrate the use of minimally invasive surgery to treat this disease. METHODS: Six patients were studied and diagnosed. The technique used three 10-mm ports on the right hemi abdomen, through which the dissection of the urachus was performed from the umbilical extreme to the bladder. We evaluated the perioperative records to assess morbidity and outcome. RESULTS: Most patients suffered from episodes of umbilical discharge. The diagnosis was made mainly through clinical history and confirmed during the laparoscopic procedure. The urachus was resected throughout its entire length, and we did not perform a segmentary bladder resection in any patient. The average operative time was 66 minutes (range, 42 to 123), and no operative complications were associated with the technique. DISCUSSION: Minimally invasive surgery is a safe and effective procedure that allows the dissection of the urachus through its entire length, providing optimal postoperative results.     

Desmoid tumor of the abdominal wall preoperatively suspected as urachal tumor: a case report]

A case of desmoid tumor of the abdominal wall which was preoperatively suspected as urachal tumor is presented. The patient was a 56-year-old man, who was referred to our clinic for further examination of the mass detected incidentally in the ventral region of the urinary bladder by computed tomography. Ultrasonography showed that the mass had a heterogenous and hypoechogenic content. An urachal tumor was suspected and surgery was performed to remove the tumor. During the operation we found that the tumor was completely separated from the urinary bladder and that it had originated from the left rectus abdominal muscle. The pathological diagnosis was desmoid tumor of the abdominal wall. Since urachal tumor has no characteristic findings on the imaging examinations, it is difficult to differentiate desmoid tumor of the lower abdominal wall from urachal tumor, preoperatively.     

Persistent endodontic lesion due to complex cementodentinal tears in a maxillary central incisor--a case report.

The cementodentinal tear is rarely detected by noninvasive procedures owing to its clinical picture simulating a root fracture or a periodontal or endodontic lesion. We present a case of complex cementodentinal tears in a 79-year-old woman who presented a repeated swelling at the labial mucosa of the left maxillary central incisor for 6 months. Periapical radiographs demonstrated a vertical radiolucent fracture line extending from the root apex along the mesial aspect of the root to near the middle portion of the root of the left maxillary central incisor. Because endodontic re-treatment failed to cure the disease, periapical surgery was performed, and 2 fractured U-shaped root fragments around the apical root surface were removed. Histologic examination showed that the 2 fractured root fragments were composed mainly of the dentin covered by a thin layer of the cementum and overlying periodontal ligament tissue, suggesting cementodentinal tears. A swelling recurred 8 months after the initial operation. Therefore, a second periapical surgery was performed. Although no obvious fracture line was observed around the root surface, the second surgery did not cure the disease, either. A persistent small swelling was noted at the alveolar mucosa of the affected tooth during the follow-up. We conclude that although a cementodentinal tear can be detected by a careful radiographic examination, its clinical outcome is not predictable by surgical removal only.