Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0241908 (BFH)
 20 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

For 4 cephalometric criteria (the angles ANB and AB-OCC and the distances AFH-BFH and "Wits"), which all are measured to show the relation between the maxilla and the mandible (i.e. the A- and B-points), average values, standard errors, standard deviations and errors of the method were determined in a sample of 298 schoolchildren of Zurich, who were selected on the basis of their date of birth only. It could be demonstrated that the angle ANB is dependent on both the angle SNA and NA to Frankfort horizontal, and that this dependence is linear. The angle AB to occlusal plane however seems to be independent of the relation between the A-point and the Nasion. Both ANB and AB-OCC could be determined with the same accuracy.
 ...
PMID:[Comparative study on 4.cephalometric measurements for the demonstration of the sagittal intermaxillary relation with special reference to Swiss health insurance criteria]. 27 84

A total of nineteen children were studied because of asymptomatic hematuria. all had normal renal function, and immunologic and urologic studies. Renal tissue was obtained by percutaneous biopsy and examined by light, immunofluorescence and electron microscopy. On electron microscopy, thickness of the glomerular basement membrane (BGM) was found to distinguish benign familial hematuria (BFH - 10 cases) from non familial idiopathic recurrent hematuria (IRH - 9 cases). Measurements were taken in areas of peripheral glomerular capillary loops (minimum of five) where both cell membranes could be resolved to insure tht determinations were uniform and represented ideal cross sections. The mean thickness +/- SE of GBM in IRH of 303.3 +/- 16.9 nM was greater than that noted in BFH of 207.1 +/- 11.99 nM (P less than .002). The mean thickness +/- SE of the lamina densa in IRH of 236 +/- 15.36 nM was significantly greater than that observed in BFH of 128.5 +/- 11.7 nM (P less than .001). In both BFH and IRH the GBM and LD were uniform in thickness (CV = 20%) and qualitatively normal. The uniform attenuation observed in BFH can be used to distinguish this condition from IRH.
 ...
PMID:The glomerular basement membrane in benign familial hematuria. 705 92

Benign familial hematuria (BFH) is characterized by autosomal dominant inheritance, thinning of the glomerular basement membrane (GBM) and normal renal function. It is frequent in patients with persistent microscopic hematuria, but cannot be clinically differentiated from the initial stages of Alport syndrome, a severe GBM disorder which progresses to renal failure. We present here linkage of benign familial hematuria with the COL4A3 and COL4A4 genes at 2q35-37 (Zmax = 3.58 at theta = 0.0). Subsequently, a glycine to glutamic acid substitution was identified in the collagenous region of the COL4A4 gene. We conclude that type IV collagen defects cause both benign hematuria and Alport syndrome. Furthermore, our data suggest that BFH patients can be carriers of autosomal recessive Alport syndrome.
 ...
PMID:Benign familial hematuria due to mutation of the type IV collagen alpha4 gene. 878 73

Transurethral electrovaporization of the prostate (TVP) has been introduced as an alternative to standard transurethral resection of the prostate (TURP) with lesser morbidity. However, the efficacy of this new technique has not been well known. To compare the results of standard TURP and TVP, 76 patients with symptomatic benign prostatic hyperplasia (BFH) were divided into two groups in a randomized clinical trial. Preoperative assessment included AUA Symptom score, maximum flow rates (Qmax), digital rectal examination, serum prostate specific antigen, and transrectal ultrasonography, with biopsy if the patient was randomized to vaporization. Transrectal temperature measurements and the hemoglobin concentration of the irrigation fluid were investigated in all the patients during the procedure. Although the transrectal temperature was higher in the TVP group (0.53-1.27 degrees C; mean 0.83 degrees C), no associated complication were determined. However, blood loss was significantly lesser than with TURP (340 mL v 60 mL). Two patients in the TURP group required blood transfusions, and one had sphincteric incontinence, whereas one postoperative retention, one reoperation with bladder perforation, and one sphincteric incontinence were seen in the TVP group. On the other hand, 12-month follow-up demonstrated that the uroflow rates improved in a similar manner. The Qmax increased in the TURP and TVP groups from 8.8 and 8.3 mL/sec to 19.6 and 17.2 mL/sec, respectively. The mean AUA Symptom Score decreased from 13.7 to 7.9 and 6.1 at 6 and 12 months, respectively. In the TVP group and from 14.6 to 7.3 and 7.0 at 6 and 12 months, respectively, in the TURP group. There were significant differences in the mean catheterization time (P < 0.0001) and hospital stay (P < 0.0001) in favor of TVP. Our results suggest that TVP is a safe and effective alternative treatment for symptomatic BPH.
 ...
PMID:Efficacy of transurethral electrovaporization of the prostate with respect to standard transurethral resection. 989 67

Benign familial hematuria (BFH: MIM141200) is an autosomal-dominant disease accounting for one-fifth of all hematuria of unknown cause in children. Previous observations suggest that BFH may be allelic to recessive Alport syndrome (AS: MIM 203780) with a mutation in the COL4A3/COL4A4 locus. However, it is not clear whether all cases of BFH are due to heterozygous mutation of COL4A3/COL4A4 genes. We report here the exclusion of linkage between BFH and COL4A3/COL4A4 loci at 2q35-37 in a restricted population from Sicily (Italy). Total lod score is -9.6 at theta 0. Furthermore, in some cases exclusion of linkage is evident even considering single families. We conclude that BFH is genetically heterogeneous.
 ...
PMID:Evidence for genetic heterogeneity in benign familial hematuria. 1046 Sep 35

p53 mutation is one of the major results of ultraviolet (UV) radiation. UV photoproducts of cyclobutane pyrimidine dimers (CPDs) and pyrimidine-pyrimidone (6-4) photoproducts (64PPs) also play an important role in skin cancer development. Atypical fibroxanthoma (AFX), which mimics malignant fibrous histiocytoma (MFH) histologically, occurs in the sun-exposed skin of the elderly, and therefore, an association with UV has long been suspected. Eighteen fibrohistiocytic skin lesions comprising AFX (n = 7), storiform-pleomorphic type MFH centered in the subcutis (superficial MFH; S-MFH; n = 4) and benign fibrous histiocytoma (BFH; n = 7) were used for immunohistochemical and molecular analysis. Eight cases of deep MFH (D-MFH) were also analyzed for UV photoproduct expression for the purposes of comparison. Immunohistochemically, the CPD scores of AFX (3.6 +/- 0.4) were significantly higher than those of S-MFH (1.3 +/- 0.8), D-MFH (0.8 +/- 0.5), or BHF (1.4 +/- 0.7); however, the 64PP scores were extremely low in all these tumors (AFX, 0.1 +/- 0.1; S-MFH, 0.0 +/- 0.0; D-MFH, 0.0 +/- 0.0; and BHF, 0.0 +/- 0.0). AFX, S-MFH, and BFH showed immunoexpression for p53 (2/7, 2/4, and 0/7), respectively. p53 mutations were detected in AFX (4/6; 67%) and S-MFH (1/4; 25%), but not in BFH (0/5; 0%) using polymerase chain reaction-single-strand conformation polymorphism, and all of the mutations in AFX were either C-T transitions or at dipyrimidine sites. In conclusion, AFX and S-MFH are both similar fibrohistocytic lesions; however, AFX has high immunoreactivity for CPDs compared with S-MFH, D-MFH, or BFH. These data suggest that CPDs may play an important role in the pathogenesis of AFX.
 ...
PMID:Immunoexpression of ultraviolet photoproducts and p53 mutation analysis in atypical fibroxanthoma and superficial malignant fibrous histiocytoma. 1140 60

Fibrohistiocytic tumors are characterized by the presence of fibroblast like spindle cells and histiocytes. The benign fibrous histiocytoma (dermatofibroma, BFH) as well as the malignant dermatofibrosarcoma protuberans (DFSP) and the malignant fibrous histiocytoma (MFH) belong to this group. A recurrent painful, hard 2 cm tumor on the left hallux of a 54-year-old woman led to an erosion of the underlying phalanx. The patient had suffered from ingrown toenails for more than 10 years. Histologically there was a deep penetrating fibrohistiocytic tumor that grew in a storiform pattern with interspersed foam cells. The tumor was CD34 negative and mitoses were scarce. The diagnosis was benign cellular fibrous histiocytoma (BZFH). BZFH belong to the group of BFH with a high recurrence rate especially after incomplete removal. Damage to the underlying bone has not been reported so far.
 ...
PMID:[Benign cellular fibrous histiocytoma with erosion of the phalanx]. 1271 66

A very rare case of benign fibrous histiocytoma of the mandible is presented. A 49-year-old woman was admitted because of left buccal swelling and pain. Panoramic radiograph showed well-demarcated soap-bubble appearance without sclerotic rim in the left mandibular bone. A yellowish-white and partly brown solid tumor was noted in the excised mandibular bone specimen. The tumor histologically consisted of spindle cells, in which areas showing a storiform pattern and other areas composed of histiocytic cells with erythrophagocytosis and foam cells were mixed. Immunohistochemically, the tumor cells were positive for vimentin, CD68, alpha-1-antichymotrypsin and alpha-1-antitrypsin. From these findings the tumor was diagnosed as a primary BFH of the mandible. No recurrence has been noted 2 years and 11 months after surgery.
 ...
PMID:Benign fibrous histiocytoma of the mandible. 1568 35

Since the high costs of common large array SQUID system may hinder widespread application of fetal magnetoencephalography (fMEG) and magnetocardiography (fMCG), we intended to investigate a small non-commercial 3-channel SQUID system. The system comprises 3 axial first order gradiometers with 7 cm base length, 2 cm diameter and 2x2 windings of niobium wire, dc-SQUIDs (UJ-111), and current locked mode SQUID electronics that form an equal length triangle (22.5 mm). The system is mounted in a Cryostat BFH-7 model 16 with 5 mm "warm"-"cold" distance. System noise is about 10 fT/Hz1/2. The fMEG and fMCG were recorded between 29 - 40 weeks of gestation after sonographic localization of the fetal head and heart using a 31-channel biomagnetometer (Philips) and the 3-channel-system, both in the same magnetically shielded room. The fMEG was recorded continuously over 500 sec (500 auditory stimuli, 100 dB SPL, 500 Hz, 50 ms, ISI 0.8-1.2/1.6-2.4 sec, trigger channel, maternal ECG lead, sampling rate 1 kHz). The fMCG was recorded over a period of 5 minutes after dewar readjustment. The detection rates of cortical auditory evoked responses (CAER) reached 100 % for both systems. Cross confirmation of the components was difficult and may have uncovered false positive component detection. The fMCG was characterized by a systematic increase in SNR under application of the smaller device. The small size array provides a profitable alternative for the fetal applications.
 ...
PMID:Fetal MCG and fetal MEG measurements with a 3-channel SQUID system. 1601 61

Dislocation after total hip arthroplasty (THA) remains a problem despite many advances in technique and prosthetic design over the 5 decades since the introduction of total joint replacement. This article reports the short-term results (1 year of follow-up) of THA in 235 patients who received a large, anatomically sized femoral head (BFH Technology; Wright Medical Technology, Inc, Arlington, Tennessee) with modular necks for hip stability. The prosthesis allows a 6-mm differential between the size of the acetabular component and femoral head size. Patients also received a Conserve monoblock acetabular cup and a Profemur femoral stem (Wright Medical Technology, Inc) implanted without cement. Postoperative clinical evaluations included measurements of Harris Hip Scores and range of motion, along with assessments of pain and function and radiological evaluations. There were no complications (deep venous thrombosis, pulmonary embolism, infection, reoperations) and no dislocations. All clinical evaluations showed statistically significant improvement (P<.001) at 1-year follow-up, and radiographic evaluation has shown no evidence of osteolysis or implant loosening. This study indicates that using a large femoral head may reduce the incidence of dislocation and may enable early return to activities postoperatively. Future evaluations of this patient group will elicit longer-term follow-up data.
 ...
PMID:Hip stability in primary total hip arthroplasty using an anatomically sized femoral head. 1963 54


1 2 Next >>