Case of primitive neuroectodermal tumor of the kidney

A 28-year-old woman presented with right flank pain. A large, firm, fixed mass was palpable in the right side of the abdomen. Computed tomography revealed a solid mass of the right kidney with extension into the renal vein and inferior vena cava. The patient underwent right radical nephrectomy with en bloc resection of the inferior vena cava containing tumor thrombus and right adrenalectomy. Histologically the tumor consisted of small tumor cells with rosette formation. Immunohistochemical staining was positive for CD99 and NSE. Analysis with polymerase chain reaction (PCR) demonstrated the EWS/FLI1 fusion products resulting from a chromosomal translocation. These findings were consistent with primary renal primitive neuroectodermal tumor (PNET). Two months after surgery, multiple lung, liver and lymph node metastases were found. The patient received 2 cycles of chemotherapy with cisplatin, ifosfamide, etoposide, resulting in a partial remission. She subsequently received 1 cycle chemotherapy with paclitaxel and carboplatin, resulting in no response. The metastatic lung and liver diseases progressed and she died 5 months after diagnosis.     

p53 antibodies in the serum of patients with prostate cancer

We assessed the potential clinical utility of levels of p53-specific antibodies as a novel serum biomarker of prostate cancer that could be used in conjunction with level of PSA. Material and methods Serum levels of p53-specific antibodies in patients with relapsed, newly diagnosed prostate cancer and in patients with benign prostate hyperplasia were quantified by an enzyme-linked immunoabsorbent assay. Result There was no significant difference (P = 0.96) between the serum levels of p53-specific antibodies in patients with newly diagnosed prostate cancer and with benign prostatic hyperplasia. In the newly diagnosed prostate cancer group, stage T1c (n = 8) showed the lowest p53-specific antibody level. However, the difference between T1c group and benign prostatic hyperplasia group was not significant (P = 0.686). The relapsed cancer group tended to have low levels of the antibodies, and, there was no significant difference between the relapsed prostate cancer group and the benign prostatic hyperplasia group (P = 0.14). The serum levels of p53-specific antibodies in patients with metastatic and with localized prostate cancer showed no significant difference (P = 0.68). Conclusion The use of titers of p53-specific antibodies to make differential diagnosis between prostate cancer and benign prostatic hyperplasia might have no role, and the antibodies should not be used as a marker of prostate cancer by itself. Because our study is based on small number of patients, further studies are necessary before its absolute validity can be determined.     

Cecal volvulus in cerebral palsy: Report of a case

A rare case of cecal volvulus in cerebral palsy that was preoperatively diagnosed and surgically treated without complications is herein reported. A 45-year old man, who had been treated for cerebral palsy as a result of a neonatal cerebral hemorrhage, was admitted to our hospital because of abdominal pain and vomiting. A plain abdominal X-ray film showed evidence of a huge quantity of gas in the left abdomen. Using a gastrographin enema from the colonoscope, an obstruction of the ascending colon was revealed with tapering of the lumen. A computed tomography scan showed a grossly dilated air-distended bowel in the left abdomen and soft tissue with internal architecture containing swirling strands of soft tissue and fat attenuation. An emergency laparotomy was performed. During the laparotomy the ileocecal region, which was unfixed at the retroperitoneum, was found to be twisted counterclockwise by 360° around the mesentery with the terminal ileum, thus resulting in a diagnosis of cecal volvulus. We therefore conducted an ileocecal resection. Cecal volvulus is an uncommon form of intestinal obstruction with a high mortality rate and may present considerable difficulty in diagnosis. Although cecal volvulus is rare as a cause of intestinal obstruction, it should be included in the differential diagnosis of bowel obstruction in cerebral palsy.     

Survival and sinus rhythm maintenance after modified Cox/maze procedure and mitral valve operation in patients with chronic atrial fibrillation

OBJECTIVE: Sinus rhythm gained after the Cox/maze procedure concomitant with mitral valve operation has demonstrated long-term attrition during the follow-up, no information exists on whether the type of mitral valve operation--(repair vs. replacement)--affects this sinus rhythm maintenance rate. We retrospectively studied patients undergoing concomitant mitral valve operation and Cox/maze procedure to answer this question. METHODS: Between April 1993 and August 1995, 87 consecutive patients--35 men and 52 women (mean age: 59.3 years)--with chronic atrial fibrillation and mitral valve disease underwent the modified Cox/maze procedure and concomitant mitral valve operation, with 56 having mitral valve repair (repair group) and 31 mitral valve replacement (replacement group). Patients were followed up and changes in rhythm studied retrospectively. RESULTS: Follow-up for a mean 51.3 +/- 11.6 months was completed in 82 of 83 long-term survivors (99%). Repair group surgery survival was 98.1 +/- 1.9% at 1 year and 94.2 +/- 3.2% at 5 based on the Kaplan-Meier method. Replacement group surgery survival was 85.7 +/- 5.9% at 1 year and 82.9 +/- 6.4% at 5. Probability in sinus rhythm maintenance for the repair group at 1 year was 88.6 +/- 5.4% and at 5 years was 67.6 +/- 9.1%. Probability in sinus rhythm maintenance for the replacement group at 1 year was 95.7 +/- 4.3% and at 5 years was 65.0 +/- 11.1%. CONCLUSIONS: Medium-term results after the Cox/maze III procedure concomitant with mitral valve operation are good. The attrition of sinus rhythm maintenance appears similar by the completion of 5-year follow-up.     

Women with mitochondrial haplogroup N9a are protected against metabolic syndrome

To identify mitochondrial haplogroups that confer resistance against or susceptibility to metabolic syndrome, we performed a large-scale association study on 1,337 unrelated Japanese individuals, including 871 subjects with metabolic syndrome and 466 control subjects. Metabolic syndrome was diagnosed according to modified National Cholesterol Education Program Adult Treatment Panel III guidelines, using the cutoff point for obesity as a BMI of >/=25 kg/m(2) instead of waist circumference. The genotypes for 25 polymorphisms in the coding region of the mitochondrial genome were determined, and the haplotypes were classified into 10 major haplogroups, i.e., F, B, A, N9a, M7a, M7b, G1, G2, D5, and D4. Multivariate logistic regression analysis revealed that the haplogroup N9a was significantly associated with resistance against metabolic syndrome in women with an odds ratio (OR) of 0.21 (95% CI 0.07-0.58, P = 0.0042). Women with haplogroups G1 and D5 tended to be resistant against metabolic syndrome with an OR of 0.22 (0.06-0.68, P = 0.0129) for G1 and with an OR of 0.32 (0.10-0.96, P = 0.0469) for D5, respectively. These results indicate that mitochondrial haplogroup N9a may be a protective factor against metabolic syndrome in Japanese women.     

Anesthetic management for cleft palate plasty in a patient with Pierre-Robin syndrome

A girl (15 months-old) with Pierre-Robin Syndrome was scheduled for cleft palate plasty. She had a past history of difficulty feeding, mild airway obstruction during sleeping and mental retardation. After induction of anesthesia with an inhalational anesthetic technique, conventional tracheal intubation was impossible. We introduced a laryngeal mask airway (LMA) and successfully intubated through the LMA. After extubation of the tracheal tube, she developed upper airway obstruction with arterial desaturation. We ventilated her lungs in the lateral position with an inhalation of epinephrine and injection of methylprednisolone. Airway obstruction then improved gradually. In this case, LMA was a valuable device as a guide for the tracheal intubation. Because airway obstruction after extubation is a common complication in a patient with Pierre-Robin syndrome, we need to observe the patient closely.     

Clinical impact of amyloid PET using 18F-florbetapir in patients with cognitive impairment and suspected Alzheimer’s disease: a multicenter study

Annals of Nuclear Medicine - Amyloid positron emission tomography (PET) can reliably detect senile plaques and fluorinated ligands are approved for clinical use. However, the clinical impact of...     

Endoscopic ultrasound-guided transmural drainage for pancreatic fistula or pancreatic duct dilation after pancreatic surgery

Background

Endoscopic ultrasound (EUS)-guided drainage is widely used to manage pancreatic pseudocysts. Several studies have reported the use of EUS-guided drainage for pancreatic fistula and stasis of pancreatic juice caused by stricture of the pancreatic duct after pancreatic resection.

Methods

At the authors’ hospital, 262 patients underwent surgery involving pancreatic resection from April 2005 to March 2010. In 90 of these patients (34%), a grade B or C postoperative pancreatic fistula developed that required additional treatment. The authors performed EUS-guided transmural drainage (EUS-TD) for six patients (2.1%) with a pancreatic fistula or dilation of the main pancreatic duct visible by EUS. Percutaneous drainage was provided for 18 patients (6.8%). The success rates for EUS-TD and percutaneous drainage were compared in a retrospective analysis.

Results

In all six cases, EUS-TD was performed successfully without complications. Five of the six patients were successfully treated with only one trial of EUS-TD. The final technical success rate was 100% for both EUS-TD and percutaneous drainage. Both the short- and long-term clinical success rates for EUS-TD were 100% and those for percutaneous drainage were 61.1 and 83%, respectively. The differences in these rates were not significant (short-term success, P?=?0.091 vs. long-term success, P?=?0.403). However, the time to clinical success was significantly shorter with EUS-TD (5.8?days) than with percutaneous drainage (30.4?days; P?=?0.0013) in the current series.

Conclusions

The EUS-TD approach appears to be a safe and technically feasible alternative to percutaneous drainage and may be considered as first-line therapy for pancreatic fistulas visible by EUS.     

A case of minimal change nephrotic syndrome with immunoglobulin A nephropathy transitioned to focal segmental glomerulosclerosis

A 50-year-old woman with a 1-month history of lower extremity edema and a 5 kg weight increase was admitted to our hospital with suspected nephrotic syndrome in October 1999. Urine protein level was 3.5 g per day, 10-15 erythrocytes in urine per high-power field, and serum albumin level 2.5 g/dl. Furthermore, an accumulation of pleural effusion was confirmed by chest X-ray. The results of a renal biopsy indicated slight mesangial proliferation in the glomeruli by light microscopy, and an immunofluorescence study confirmed the deposition of immunoglobulin (Ig) A and C3 in the mesangial area. Diffuse attenuation of foot processes and dense deposits in the mesangial area were observed by electron microscopy. Treatment with 40 mg/day of prednisolone was effective, and proteinuria was negative 1 month later. Because of this course, we diagnosed minimal change nephrotic syndrome complicated by mild-proliferative IgA nephropathy. In November 2000, there was a relapse of nephrotic syndrome, which was believed to be induced by an influenza vaccination, but response to increased steroid treatment was favorable, and proteinuria disappeared on day 13 of steroid increase. A second relapse in May 2001, showed steroid resistance with renal insufficiency, and an increase in the selectivity index to 0.195. Light microscopy revealed focal sclerotic lesions of the glomeruli, and an immunofluorescence study revealed attenuation of mesangial IgA and C3 deposition. These findings led to the diagnosis that minimal change nephrotic syndrome had transitioned to focal segmental glomerulosclerosis, whereby mesangial IgA deposition was reduced by immunosuppressive treatment. Subsequently, her renal function gradually worsened to the point of end-stage renal failure by 27 months after the second relapse of nephrotic syndrome.     

Mitral valve repair for double-orifice mitral valve with flail leaflet: the usefulness of real-time three-dimensional transesophageal echocardiography

We describe a 66-year-old man who required an operation for severe mitral regurgitation associated with a double-orifice mitral valve. Real-time 3-dimensional transesophageal echocardiography clearly demonstrated a double-orifice mitral valve with a central fibrous bridge. A flail posterior leaflet was observed on the anterolateral mitral valve orifice. Mitral valve repair using P1 triangular resection, anterolateral commissure plication, and ring annuloplasty with Duran band (Medtronic, Minneapolis, MN) was successfully performed. Postoperative real-time 3-dimensional transesophageal echocardiography demonstrated a double-orifice mitral valve without regurgitation or stenosis.