Laurence-Moon-Biedl syndrome: Scintigraphic appearance of kidneys

We report a 7-year-old child with Laurence-Moon-Biedl syndrome, an autosomal recessive syndrome, with impaired renal function detected by means of technetium-99m diethylenetriamine-pentaacetic acid (Tc-99m DTPA), technetium-99m dimercaptosuccinic acid (Tc-99m DMSA) scintigraphy, and ultrasonography. The altered renal morphology and decreased renal functions are documented.     

Synthesis and anticonvulsant activity of some new hexahydropyrimidine-2,4-dione derivatives

In this study, twelve new hexahydropyrimidine-2,4-dione derivatives were synthesized and screened for their anticonvulsant activities. All the compounds (7a-1) which have 6-arylhexahydropyrimidine-2,4-dione and N,N-disubstituted dithiocarbamate structures were prepared by the reaction with appropriate 3-(2-chloroethyl)-6-arylhexahydropyrimidine-2,4diones and the corresponding N,N-disubstituted dithiocarbamate potassium salts. The structure of the synthesized compounds was confirmed by UV, IR, 1H-NMR and elemental analysis. Their anticonvulsant activities were determined by maximal electroshock (MES), subcutaneous pentetrazol (metrazol, scMet) and rotorod toxicity tests for neurological deficits. According to the activity studies, 6-(4-chlorophenyl)hexahydropyrimidine-2,4-dione derivatives (7e-h) were found to be highly protective against MES and scMet. Neurotoxicity was not observed in any of the tested compounds.     

Effect of fibroblast growth factor-2 on pannus formation in replacement prosthetic heart valves

BACKGROUND AND AIM OF THE STUDY: Fibroblast growth factor-2 (FGF-2) is a multifunctional protein which plays a role in smooth muscle cell growth, wound healing, tissue repair and angiogenesis. FGF-2 is also released by mechanically wounded cells. Herein, the importance of FGF-2 release from periannular tissue in the mechanism of pannus formation in obstructed mechanical prostheses was investigated. METHODS: Between January 1993 and December 2002, 35 patients with an obstructed bileaflet prosthetic mitral valve were classified according to the nature of obstruction as either thrombus or pannus. Data were related to patient age and gender, prosthesis model and size, intraoperative and pathology findings, and interval between implant and thrombosis. FGF-2 release was monitored immunohistochemically in all cases. RESULTS: Thrombus formation was found in 19 patients, and pannus formation in 16. Patients were reoperated on after 3.10 +/- 0.7 years in the thrombus group, and after 6.3 +/- 0.46 years in the pannus group (p = 0.04). A foreign body reaction was found 78.9% of thrombus patients and 81.2% of pannus patients (p = 0.602), chronic inflammation in 31.5% and 50%, respectively (p = 0.317), and FGF-2 release in 78.9% and 87.5%, respectively (p = 0.582). CONCLUSION: As FGF-2 release was similar in both patient groups, the duration of FGF-2 release from injured periannnular tissue was considered to form part of the chronic healing process, and was not attributed to mitral valve obstruction by pannus formation.     

Fibrotic lesions of the breast: radiological findings and core-neddle biopsy results

Objectives

To evaluate the incidence of breast lesions with a histopathological diagnosis of focal fibrosis based on imaging guided core biopsy, to review the radiologic findings and to assess the diagnostic reliability of 14 G core needle biopsy.

Materials and methods

723 patients, who had undergone 14 G core biopsy and/or surgical excisions, were retrospectively analyzed. Overall, 43 lesions were diagnosed as focal fibrosis. Physical examination, mammography, ultrasonography, and follow-up findings were all reviewed.

Results

Radiological evaluation revealed that 35 (81%) lesions were solid masses. Of 35 mass lesions, 24 (69%) were well circumscribed, the remaining 11 (31%) lesions were ill defined on mammograms or sonograms. None of the lesions had pathological microcalcifications. Three lesions were surgically excised because of radio-pathological discordance after core needle biopsy.

Conclusions

Focal fibrosis of the breast is a benign condition and reflects the ductal and lobular atrophy secondary to stromal proliferation. The radiological findings of this entity may vary and sometimes mimic those of malignant lesions. The incidence of focal fibrosis among our study population is 6% and a well-defined mass lesion is the most frequent finding. Core needle biopsy is a safe and reliable diagnostic procedure in the management of these cases.     

Variation in sources of clinician-rated and self-rated instrumental activities of daily living disability

BACKGROUND: It is unclear how well self-reports and clinician ratings of performance in the instrumental activities of daily living (IADLs; household maintenance tasks) correspond and why they may differ. METHODS: We assessed clinician-rated IADL performance using an occupational therapy protocol, the Assessment of Motor and Process Skills (AMPS). AMPS and self-rated IADL disability were compared in two groups of nondemented elderly persons without ADL limitation: a group with functional limitation only (self-reported difficulty in some area of upper or lower body function, n = 139) and a group that reported functional limitation plus IADL disability (difficulty in at least one IADL task, n = 49). Occupational therapists were blind to self-reports, and all assessments were conducted in respondent homes. RESULTS: Self-rated IADL disability was significantly associated with the AMPS motor skill score (r = -.34, p <.001), but the motor skill score was only moderately sensitive (61%) and specific (67%) in identifying self-rated disability. In adjusted logistic regression models, clinician-rated performance and self-rated IADL disability shared some physical predictors, but only clinician-rated performance was related to cognitive status. AMPS process skill scores did not relate to self-rated IADL disability or physical or cognitive status. CONCLUSIONS: In this sample of older adults without dementia or ADL disability, clinician ratings of IADL motor skill and self-rated IADL disability were correlated. Physical deficits appear to be more salient in self-ratings than is cognitive ability, because cognitive ability (in particular, verbal fluency) was associated only with clinician-rated IADL performance.     

The effects of shock waves on lung tissue in acute period: an in vivo study

The acute effects of extracorporeal shock waves on lung morphology were studied by light microscopy in 30 New Zealand rabbits. The left kidneys of the ten animals were exposed to 2,000 shockwaves at 18 kV under anesthesia (ESWL group). Ten rabbits were in the control group; an intramuscular anesthetic agent (ketamine) and intravenous contrast media were administered to ten animals in the sham group. Localization of the left kidneys was achieved following contrast medium injection through an ear vein under fluoroscopic control. The animals were killed after the ESWL procedures and the lungs were removed. Edema, emphysema, hemorrhage, congestion, inflammation, loss of normal structure, and epithelial desquamation were determined and graded between 0 and 3 in all areas of both lungs. In order to determine whether proximity has any effect on the histopathological changes, left and right lungs were evaluated separately as well as upper and lower lobes. We found that ESWL exposed to kidney also affects all areas of the lungs in a rabbit model.