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Selected AbstractsCardiac amyloidosis: MR imaging findings and T1 quantification, comparison with control subjectsJOURNAL OF MAGNETIC RESONANCE IMAGING, Issue 6 2007Gabriele A. Krombach MD Abstract In cardiac amyloidosis an interstitial deposition of amyloid fibrils causes concentric thickening of the atrial and ventricular walls. We describe the results of tissue characterization of the myocardium by T1 quantification and MRI findings in a patient with cardiac amyloidosis. The T1 time of the myocardium was elevated compared to that in individuals without amyloidosis. The T1 time of the myocardium was 1387 ± 63 msec (mean value obtained from four measurements ± standard deviation [SD]) in the patient with cardiac amyloidosis, while the reference value obtained from the myocardium of 10 individuals without known myocardial disease was 1083 ± 33 msec (mean value ± SD). In combination with other MR findings suggestive of amyloidosis, such as homogeneous thickening of the ventricular and atrial walls, thickening of the valve leaflets, restrictive filling pattern, and reduction of systolic function, T1 quantification may increase diagnostic confidence. J. Magn. Reson. Imaging 2007;25:1283,1287. © 2007 Wiley-Liss, Inc. [source] Recurrence of primary sclerosing cholangitis after liver transplantationLIVER TRANSPLANTATION, Issue 7 2002Ivo W. Graziadei MD Orthotopic liver transplantation (OLT) has become the only effective therapeutic option for patients with end-stage liver disease caused by primary sclerosing cholangitis (PSC). Excellent long-term outcome has been reported, with 5-year patient survival rates of approximately 80%. In the last few years, increasing evidence has emerged that PSC recurs after OLT. The diagnosis of PSC is based on well-defined cholangiographic features combined with biochemical and histological findings. However, none of these features is specific for PSC, particularly after OLT, because biliary strictures in the liver allograft can occur from a variety of causes other than recurrence. Therefore, PSC recurrence remains a controversial issue, especially because of a lack of a gold standard for diagnosis and well-established diagnostic criteria. Some reports provided cholangiographic evidence that post-OLT biliary strictures occurred more frequently in patients with PSC than in those who underwent OLT for other liver diseases (including patients with a Roux-en-Y biliary reconstruction). Because no other possible cause of biliary strictures could be invoked to explain the greater prevalence of these strictures, recurrent disease has been implicated. There also is histological evidence suggesting that PSC recurs after OLT. Histological findings suggestive of PSC were found more often in PSC allografts compared with a control group. Furthermore, histological features typical for PSC (fibro-obliterative lesions) were seen exclusively in liver biopsy specimens from patients with PSC. Recurrence of PSC was defined in a recent study from the Mayo Clinic by means of strict cholangiographic and histological criteria in a large cohort of patients with PSC in whom other causes of biliary strictures were excluded. PSC recurrence was found in 20% of patients. No risk factor for PSC recurrence could be found, and recurrent disease did not influence patient or graft survival after a mean follow-up of 4.5 years. In conclusion, several studies provided convincing evidence that PSC recurs after OLT, with an incidence of 5% to 20% and an interval to diagnosis of at least 1 year after OLT. To date, patient and graft survival do not appear to be negatively affected by disease recurrence in the intermediate term of follow-up. (Liver Transpl 2002;8:575-581.) [source] Pallidopyramidal disease: A misnomer?,MOVEMENT DISORDERS, Issue 9 2010Martin W.I.M. Horstink MD Abstract The combination of recessive early-onset parkinsonism and pyramidal tract signs caused by pallidopyramidal degeneration is known as pallidopyramidal disease or syndrome (PPD/S). We investigated whether patients diagnosed as Davison's PPD/S showed any definite proof of pyramidal and pallidal involvement, without findings suggestive of other nosological entities. Since Davison's original description, 15 other PPD/S cases have been reported, yet all lack proof of pyramidal or pallidal degeneration. Because of the dopa-responsiveness in all patients subsequent to Davison's report, we argue that these patients probably suffered from early-onset nigral parkinsonism or dopa-responsive dsystonia, rather than pallidal parkinsonism; in such cases, the presumed pyramidal Babinski could be a pseudobabinski ("striatal toe"). Secondary pallidopyramidal syndromes do occur, for example, in multiple system atrophy or Wilson's disease, but in these patients additional findings indicate diseases other than Davison's PPD/S. We conclude that the existence of PPD/S as a distinct clinico-pathological nosological entity, as proposed by Davison, is doubtful. In cases reported as Davison's PPD/S, the description "pallidopyramidal" seems to be a misnomer. © 2010 Movement Disorder Society [source] Chronic obstructive pulmonary disease and interstitial lung disease in patients with lung cancerRESPIROLOGY, Issue 3 2009Satoko MIZUNO ABSTRACT Background and objective: Although lung cancer is frequently accompanied by COPD and interstitial lung disease (ILD), the precise coincidence of these diseases with lung cancer is not well understood. The objectives of this study were to determine the prevalence of abnormal CT and spirometric findings suggestive of COPD or ILD in a population of patients with untreated lung cancer, and to estimate the lung cancer risk in this population. Methods: The study population consisted of 256 patients with untreated lung cancer and 947 subjects participating in a CT screening programme for lung cancer. Semi-quantitative analysis of low attenuation area (LAA), fibrosis and ground glass attenuation (GGA) on CT was performed by scoring. Gender- and age-matched subpopulations, with stratification by smoking status, were compared using the Mantel,Haenszel projection method. Results: Inter-observer consistency was excellent for LAA, but not as good for fibrosis or GGA scores. Pooled odds ratios for lung cancer risk using LAA, fibrosis, GGA scores and reduced FEV1/FVC and %VC were 3.63, 5.10, 2.71, 7.17 and 4.73, respectively (P < 0.0001 for all parameters). Multivariate regression analyses confirmed these results. Conclusion: Abnormal CT and spirometric parameters suggestive of COPD and ILD were strong risk factors for lung cancer, even after adjusting for gender, age and smoking status. [source] A case of discoid lupus erythematosus of the eyelidTHE JOURNAL OF DERMATOLOGY, Issue 5 2006Monji KOGA ABSTRACT A 39-year-old man was first evaluated 12 years ago for erosive erythema of the left lower eyelid. The response to topical therapy was poor. The eyelid lesion was excised on the patient's demand 6 years ago and blepharoplasty was performed. However, recurrence of erythematous plaques at the same site prompted referral to our department. Examination clinically revealed atrophic erythematous plaques and several white papules on the left lower eyelid, in addition to an atrophic erythema of the upper back. The histopathological findings of both plaques and papules were typical of discoid lupus erythematosus. Immunoserological findings suggestive of systemic lupus erythematosus were absent. The lesions improved with administration of low-dose corticosteroids. Discoid lupus erythematosus involving the eyelid is rare, and definitive diagnosis often takes considerable period of time. In patients with lesions of the eyelid such as erythema or papules that are refractory to treatment, skin biopsy and careful physical examination of the entire body for similar lesions are important. [source] C4d and C3d Staining in Biopsies of ABO- and HLA-Incompatible Renal Allografts: Correlation with Histologic FindingsAMERICAN JOURNAL OF TRANSPLANTATION, Issue 8 2006M. Haas Biopsies of ABO-incompatible and positive crossmatch (HLA-incompatible) renal allografts were retrospectively examined to compare results of C4d and C3d staining, and the correlation between such staining and histologic findings suggestive of antibody-mediated rejection (AMR). A total of 75 biopsies (55 protocol, 17 for graft dysfunction, 3 for other indications) of 24 ABO-incompatible grafts and 244 biopsies (103 protocol, 129 for graft dysfunction, 12 for other indications) of 66 HLA-incompatible grafts were examined; all were stained for C4d and ,40% for C3d. In ABO-incompatible grafts, 80% of protocol biopsies and 59% performed for graft dysfunction showed C4d staining in peritubular capillaries (PTC); this staining was not correlated with neutrophil margination in PTC. In HLA-incompatible grafts, PTC C4d was present in 26% of protocol biopsies and 60% of biopsies for graft dysfunction; 92% of biopsies with >1+ (0,4+ scale), diffuse PTC C4d had ,1+ margination and/or thrombotic microangiopathy (TMA), compared with 12% of C4d-negative biopsies. C3d was somewhat more predictive of margination than C4d in ABO-incompatible, but not HLA-incompatible, grafts. In summary, while PTC C4d deposition indicates probable AMR in biopsies of HLA-incompatible grafts, including protocol biopsies, there is no histologic evidence that C4d deposition is correlated with injury in most ABO-incompatible grafts. [source] Is detrusor hypertrophy in women associated with voiding dysfunction?AUSTRALIAN AND NEW ZEALAND JOURNAL OF OBSTETRICS AND GYNAECOLOGY, Issue 6 2009Orawan LEKSKULCHAI Background:, In men, bladder wall thickness ,5 mm seems to be a useful predictor of outlet obstruction, with a diagnostic value exceeding that of free uroflowmetry indices. There are no data in the literature examining whether this may also apply in women. Aims:, To identify the relationships between detrusor wall thickness (DWT) and symptoms and urodynamic findings suggestive of voiding dysfunction. Methods:, This is a retrospective study analysing data sets of 686 women seen for urodynamic testing in a tertiary urogynaecological unit. Hesitancy, poor stream and stop,start voiding were recorded as symptoms of voiding dysfunction. All women underwent free uroflowmetry and multichannel urodynamic testing. We used the urethral resistance factor (URA) and the obstruction coefficient (OCO), methods employed to quantify bladder outlet resistance in men. Transperineal ultrasound for DWT was performed after voiding and catheter removal. Statistical analysis was carried out by using the statistical software (spss 15.0; SPSS Inc., Chicago, IL, USA). Results:, Symptoms of voiding dysfunction were reported by 33.1% of patients and 22.4% had urodynamically diagnosed voiding dysfunction. The mean DWT in symptomatic women was not statistically different from the mean DWT in those without symptoms. URA and OCO of symptomatic women were significantly different from those of asymptomatic women (P < 0.01). DWT was not associated with parameters of voiding function, URA or OCO. Conclusions:, Contrary to the situation in men, increased DWT in women does not seem to be associated with symptoms or signs of voiding dysfunction. Therefore, DWT cannot be used as a predictor of voiding difficulty in women. [source] Is Hospital Admission and Observation Required after a Normal Abdominal Computed Tomography Scan in Children with Blunt Abdominal Trauma?ACADEMIC EMERGENCY MEDICINE, Issue 10 2008Smita Awasthi MD Abstract Objectives:, The objective was to determine if hospital admission of children with blunt abdominal trauma for observation of possible intraabdominal injury (IAI) is necessary after a normal abdominal computed tomography (CT) scan in the emergency department (ED). Methods:, The authors conducted a prospective observational cohort study of children less than 18 years of age with blunt abdominal trauma who underwent an abdominal CT scan in the ED. Abdominal CT scans were obtained with intravenous contrast but no oral contrast. The decision to hospitalize the patient was made by the attending emergency physician (EP) with the trauma or pediatric surgery teams. An abnormal abdominal CT scan was defined by the presence of any visualized IAI or findings suggestive of possible IAI (e.g., intraperitoneal fluid without solid organ injury). Patients were followed to determine if IAI was later diagnosed and the need for acute therapeutic intervention if IAI was present. Results:, A total of 1,295 patients underwent abdominal CT, and 1,085 (84%) patients had normal abdominal CT scans in the ED and make up the study population. Seven-hundred thirty-seven (68%) were hospitalized, and 348 were discharged to home. None of the 348 patients discharged home and 2 of the 737 hospitalized patients were identified with an IAI after a normal initial abdominal CT. The IAIs in patients with normal initial CT scans included a 10-year-old with a mesenteric hematoma and serosal tear at laparotomy and a 10-year-old with a perinephric hematoma on repeat CT. Neither underwent specific therapy. The negative predictive value (NPV) of a normal abdominal CT scan for IAI was 99.8% (95% confidence interval [CI] = 99.3% to 100%). Conclusions:, Children with blunt abdominal trauma and a normal abdominal CT scan in the ED are at very low risk of having a subsequently diagnosed IAI and are very unlikely to require a therapeutic intervention. Hospitalization of children for evaluation of possible undiagnosed IAI after a normal abdominal CT scan has a low yield and is generally unnecessary. [source] Evidence of placental haemorrhage and preterm deliveryBJOG : AN INTERNATIONAL JOURNAL OF OBSTETRICS & GYNAECOLOGY, Issue 4 2010JW Gargano Please cite this paper as: Gargano J, Holzman C, Senagore P, Reuss M, Pathak D, Williams M, Fisher R. Evidence of placental haemorrhage and preterm delivery. BJOG 2010;117:445,455. Objective, To evaluate evidence of placental haemorrhage (PH) obtained through maternal interviews, patient charts and placental pathology examinations as potential indicators of a ,bleeding pathway' to preterm delivery (PTD). Design, Prospective cohort. Setting, Fifty-two clinics in five communities in Michigan, USA (1998,2004). Population, A subset (n = 996) of cohort participants with complete placental pathology data. Methods, First-trimester bleeding and placental abruption were ascertained by mid-trimester interviews and chart review, respectively. Disc-impacting blood clot was defined as a gross placental examination finding of a blood clot impacting adjacent tissue. Microscopic haemorrhage was defined as ,high' (top quintile) scores on an aggregate measure of placental pathology findings suggestive of atypical maternal vessel haemorrhage. These four PH indicators were compared with one another and with risk of PTD assessed by logistic regression analyses. Main outcome measures, Preterm delivery and PTD subtypes (i.e. <35 weeks, 35,36 weeks; spontaneous, medically indicated) compared with term deliveries. Results, Placental abruption cases had 2.3-fold to 5.5-fold increased odds of the other three PH indicators. Disc-impacting blood clots and microscopic haemorrhage were associated with one another (odds ratio [OR] = 4.6), but not with first-trimester bleeding. In a multivariable model that included all four PH indicators and confounders, risk of PTD < 35 weeks was elevated with first-trimester bleeding (OR = 1.9 [1.0, 3.4]), placental abruption (OR = 5.2 [1.7, 16.2]), disc-impacting blood clots (OR = 2.3 [1.0, 5.0]) and microscopic haemorrhage (OR = 2.4 [1.4, 4.2]). Conclusions, Multiple clinical and subclinical PH indicators are associated with PTD, particularly early PTD. [source] The role of indirect radionuclide cystography during the acute phase of pyelonephritis in young womenBJU INTERNATIONAL, Issue 4 2005Jehonathan H. Pinthus Authors from Israel have investigated the use of dynamic renal scans in young female patients with acute pyelonephritis, combined with indirect radionuclide cystography. They found that using these techniques may avoid up to half of the delayed voiding cysto-urethrograms, preventing the related inconvenience and cost. OBJECTIVE To review our experience using dynamic 99mTc-diethylenetriamine penta-acetic acid renal scintigraphy combined with indirect radionuclide cystography (IRC) in the acute phase of pyelonephritis, as a possible alternative to the conventional imaging, as investigating acute pyelonephritis usually includes imaging the upper urinary tract during the acute phase, to exclude obstruction, and delayed voiding cysto-urethrography (VCUG) when underlying vesico-ureteric reflux (VUR) is suspected. PATIENTS AND METHODS Between 1997 and 1999, 47 young women (median age 22 years, range 18,37) were hospitalized for acute pyelonephritis. The combined study was used during the acute phase of the disease, usually within 24 h of hospitalization. The principle of IRC is based on the reappearance of radioactivity in the ureters or kidneys after previously detecting renal clearance of an intravenously injected radioisotope. The increase in radioactivity over the ureters or kidneys indicates VUR. The subsequent follow-up included VCUG, after recovery and at least 6 weeks after discharge. RESULTS Overall, 47 patients had early IRC studies; obstruction of the urinary tract during the acute phase of the disease was excluded in all. In 13 (28%) of the patients early IRC studies showed VUR involving 21 upper tract units. The renal parenchymal scan was impaired in 17 (36%) patients, and six of these 17 also had detectable concomitant reflux on IRC. Overall, 24 IRC studies (51%) were considered positive, showing VUR, renal parenchymal pathology or both; 23 (49%) were normal. Follow-up VCUG was used in 32 patients (68%); only three (9%) detected VUR. All of the patients with VUR on follow-up VCUG had also had an abnormal early IRC study, showing either reflux (two) or findings suggestive of pathological renal parenchyma (one). CONCLUSIONS In addition to the well-established role of renal scintigraphy in excluding obstruction of the collecting system, early IRC is characterized by high sensitivity and accurate negative predictive value for detecting VUR. It can therefore be used to screen adults presenting with acute pyelonephritis for the presence of VUR. Patients with an abnormal IRC require follow-up VCUG after complete recovery, while those with a negative study may be managed expectantly, with no further radiological evaluation. This proposed strategy may avoid up to half of the delayed VCUG studies, preclude the related inconvenience, and substantially reduce the costs. [source] Extramammary Paget's disease of the groin with underlying carcinoma and fatal outcomeCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 5 2008J. C. Pascual Summary Extramammary Paget's disease (EMPD) is considered to be an intraepithelial adenocarcinoma. Typically involved anatomical sites are the vulvar, perianal, perineal, scrotal and penile regions. Clinically, the lesions present as well-defined, moist, erythematous plaques usually accompanied by pruritus. An unusual feature of EMPD is its association with cutaneous, adnexal-structure adenocarcinomas and its association with internal malignancies. Histopathological examination shows epidermal acanthosis and elongated rete ridges. Paget's cells are large intraepidermal cells with a large nucleous and abundant pale cytoplasm. Recent studies of perianal and vulvar EMPD have described distinct immunohistochemical subtypes termed cutaneous and endodermal. Cutaneous EMPD is characteristically positive for cytokeratin (CK)7, negative for CK20, and positive for gross cystic disease fluid protein (GCDFP)15+, whereas endodermal EMPD shows a CK7+ CK20+ GCDFP15, phenotype. Surgery remains the treatment of choice, with either wide surgical excision or Mohs' micrographic surgery. We present a case of EMPD with an underlying carcinoma, which combined immunohistochemical findings suggestive of the cutaneous subtype (positive for CK7, GCDFP15, mucin (MUC)1, human epidermal growth factor receptor (HER)2/neu positive) and the endodermal subtype, frequently associated with internal malignancy (CK20, MUC2, CDX-2 positve); however, our patient had no associated internal malignancy. [source] Peritubular capillary C4d deposition and renal outcome in post-transplant IgA nephropathyCLINICAL TRANSPLANTATION, Issue 2 2007Jung Choi Abstract:, Backgrounds:, Immunological staining of the transplanted kidney for C4d in peritubular capillaries (C4dPTC) has emerged as a useful method to detect antibody-mediated rejection in situ. In this retrospective study, we evaluated the prevalence of C4dPTC deposition in allograft renal biopsies diagnosed of IgA nephropathy (IgAN) and analysed its clinical significance. Method:, Sixty-six biopsy specimens of post-transplant IgAN, which were obtained to evaluate azotemia and/or heavy proteinuria, were examined by immunohistochemical staining of the paraffin sections with polyclonal antibody for C4d. Results:, C4d was stained positively in peritubular capillaries in 16 (24%) of the 66 cases. The C4dPTC -negative (n=50) and C4dPTC -positive groups (n=16) were not different in recipient gender, age, donor age, type of donor (living vs. cadaveric), interval from transplantation to graft biopsy (41.6± 21.8 vs. 48.3±26.1 months) and post-biopsy follow-up period (60.3±23.3 vs. 56.9±25.4 months). During the follow-up period, 12 of 50 (24%) although the incidence of graft failure was not different by the C4d deposition in peritubular capillaries, intervals from renal biopsy to graft failure tended to be shorter in C4dPTC -positive cases than C4dPTC -negative cases. In Kaplan,Meier analysis, the renal allograft function of the C4dPTC -positive group deteriorated more rapidly than that of the C4dPTC -negative group (p<0.05). Histologically, the C4dPTC -positive group had findings suggestive of acute cellular rejection more commonly than the C4dPTC -negative group (p<0.01). Conclusions:, Evidence of humoral rejection, as demonstrated by C4dPTC deposition, was concurrently present in significant portions of post-transplant IgAN biopsy specimens and was associated with more rapid deterioration of renal function. These results suggest that C4dPTC positivity needs to be determined at the time of biopsy even in cases of post-transplant glomerulonephritis and immunosuppression may need to be modified accordingly. [source] |