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Multiple defects in the retinal nerve fiber layer in glaucoma.

We investigated the parameters that correlated with multiple defects in the retinal nerve fiber layer in 77 human eyes with normal-tension glaucoma, 110 with chronic high-tension glaucoma, and 102 control eyes. All 187 glaucomatous eyes had a nerve defect that was multiple in 20 cases. Correlation was significant between the multiple defect and the type of defect in the nerve fiber layer (P less than 0.002), size of the disc (P less than 0.02), and oblique insertion of the disc (P less than 0.02), as evaluated by the chi-square test. By multivariate analysis, refractive error was a high-ranking risk factor for multiple defect. Eyes with multiple defects tended to have moderate myopia, a focal nerve fiber layer defect and a small optic disc (category scores 0.0932, 0.0878 and 0.0697) and were less likely to have a diffuse defect in the nerve fiber layer, emmetropia or hyperopia, and a normal disc size (category scores -0.1077, -0.0705, and -0.548). The multiple defect in the retinal nerve fiber in glaucoma was frequently focal and correlated with myopia and a small optic disc.

Adult↗

Ataxia-telangiectasia: an inherited disorder of ionizing-radiation sensitivity in man. Progress in the elucidation of the underlying biochemical defect.

This review summarizes the current research on the biochemical defect leading to ataxia-telangiectasia (AT). A DNA repair defect has been linked to AT, although the precise defect has not been found. A critical examination of the evidence for and against a DNA repair defect in AT is presented. Consideration of other recent data on AT raises the possibility that AT may not primarily be the result of a DNA repair defect. Therefore, in this review AT is approached as a syndrome which is defective in the ability to respond to ionizing-radiation-type damage, rather than defective in the ability to repair this damage. However, this does not necessarily exclude the potential involvement of a DNA repair defect in some of the genetically distinct subsets present in AT. Other recent anomalies found in AT, including an altered cell cycle and DNA synthesis profile following ionizing-radiation damage, are also assessed. A suggestion to account for the underlying defect in AT, based on the various research reports, is presented.

Ataxia Telangiectasia↗

Retinal nerve fiber layer defects and automated perimetry evaluation in ocular hypertensives.

Preliminary results of a larger investigation on correlation between Retinal Nerve Fiber Layer Defects (RNFL) and Automated Perimetry (AP) are presented. A group of ocular hypertensive patients was recruited and a cross-section, randomized study was carried out. The group was composed of 54 eyes from 28 patients; 15 men and 13 women, mean age 42.76 year, mean visual acuity 0.95 (Snellen cart) and mean visual defect expressed in diopters was +0.514 (from -2.5 to +5). A total of 17 (31.48%) refractive fields were pathologic according to our criteria, one from a normal RNFL and the others from nerve fiber defects. We observed 6 no reliable RNFL photographs, 18 normal, 11 slits, 6 wedge, 11 diffuse and 2 mixed defects. Comparing RNFL and AP, 53.33% of eyes with fiber defects had abnormal perimetries. The more evolutioned the defect, the larger percentage of abnormal visual fields were observed: 36.36% in slit defects, 66.66% in wedge, 54.55% in diffuse and 100% in mixed ones. Comparing location of defects with location of cluster of decreased sensibility points in visual field, a higher correlation in inferior than superior defects was found.

Adult↗

Identification of a heterozygous compound individual with familial hypercholesterolemia and familial defective apolipoprotein B-100.

Familial defective apolipoprotein B-100 (FDB) is a recently identified dominantly inherited genetic disorder, which leads to increased serum levels of low density lipoprotein (LDL) cholesterol with reduced affinity for the LDL receptor. This genetic disorder is characterized by defective binding of the apolipoprotein B-100 (apo B-100), which is virtually the sole protein constituent of LDL, to the LDL receptor. The defective binding results from a G to A mutation at amino acid 10,708 in exon 26 of the apolipoprotein B (apo B) gene creating a substitution of glutamine for arginine in the codon for amino acid 3500. It is postulated that FDB can exhibit the same clinical features as familial hypercholesterolemia (FH) caused by a defective LDL receptor. The purpose of this paper is to report on an individual with a defective LDL and a defective LDL receptor. The clinical features of this individual were the same as in the family members with either defective LDL or a defective LDL receptor: premature arcus lipoides, tendon xanthomata, and premature atherosclerosis. Although the clinical features were present to the same degree as in individuals with either defect the prognosis and treatment of such an individual could be different.

Adolescent↗

Defects in oxidative phosphorylation. Biochemical investigations in skeletal muscle and expression of the lesion in other cells.

Mitochondria are very vulnerable to genetic and environmental damage. If a patient is suspected of having a mitochondrial disease, elevated blood lactate, lowered blood free carnitine, abnormal urinary organic acids and carnitine esters and tissue histopathology may help with the diagnosis. For biochemical assessment of the defect, muscle is the tissue of choice even when involvement of other organs like heart or brain is more prominent. We have studied isolated muscle mitochondria and homogenates from muscle biopsies in 250 patients, and have detected in more than one third mitochondrial defects in oxidative phosphorylation, dehydrogenases, non-redox enzymes catalyzing synthesis of fuel molecules and in the carnitine system. Several patients showed more than one defect. We have selected eight patients to illustrate how a relatively simple series of investigations in both isolated mitochondria and homogenate can be used for the identification of defects in oxidative phosphorylation in a small amount of muscle (200 mg or more). Identification of the defect(s) is important since it may provide the basis for rational treatment. A minority of the patients recovered partly or completely, which is unique in treatment of inborn errors of subcellular organelles. An important aspect of mitochondrial dysfunction is the tissue specificity. The defect may be systemic but is often clinically expressed in only one or a few tissues. Rarely, tissue-specific defects can be understood on the basis of tissue-specificity of mitochondrial (iso-)enzymes. Mitochondrial deficiencies of all biotin enzymes and most CoA-linked enzymes are expressed in fibroblasts; most respiratory chain defects are not. When mitochondrial ATP synthesis has been compromised by a mitochondrial defect, secondary lesions may be generated by changes in mitochondrial protein synthesis, activated proteases and phospholipases, increased matrix CoA and resulting carnitine deficiency, decrease in Krebs cycle intermediates and increased free radical formation and lipid peroxidation.

Adolescent↗

Unsuspected sphincter defects shown by anal endosonography after anorectal surgery. A prospective study.

PURPOSE: Anorectal surgery can lead to fecal soiling and incontinence. Whether surgery changes the anatomy and causes symptoms is unknown. Anatomic changes can be visualized by anal endosonography. METHODS: We studied 50 patients after hemorrhoidectomy (24), fistulectomy (18), and internal sphincterotomy (8). Symptoms were assessed, and anal endosonography, anal manometry, mucosal electrosensitivity, and neurophysiologic tests were performed. RESULTS: In 23 (46 percent) patients, a defect of the anal sphincter was found (13 patients had an internal sphincter defect, 1 had an external sphincter defect, and 9 had a combined sphincter defect), 3 after hemorrhoidectomy, 13 after fistulectomy, and 7 after internal sphincterotomy. Seven patients had symptoms, and they all had a sphincter defect. In the other 16 of 23 patients (70 percent), the sphincter defect did not produce symptoms. An internal sphincter defect lowered maximum basal pressure and shortened sphincter length. CONCLUSION: Anal endosonography can reveal sphincter defects after anorectal surgery. Seventy percent of the patients in this group had no complaints; therefore, defects were unsuspected. This has clinical implications in the evaluation of patients with fecal incontinence.

Adult↗

Reconstruction of calvarial defects by bioresorbable ceramics: an experimental study in rats.

This study evaluated bioresorbable ceramics in the reconstruction of calvarial defects. Full-thickness defects were made in the calvaria of 40 adult Sprague-Dawley rats (350-450 g) with a standard 8-mm trephine drill. Three different materials were used for defect repair: (a) pure alpha-tricalcium phosphate (TCP), (b) surface-treated glass ceramic, (c) surface-treated glass ceramic plus 70/30 L/DL polylactic acid (volume ratio 45/55). The implants were pellets of 7.9 mm diameter and 2 mm thickness and were inserted press fit into the calvarial defects. Each of these materials was inserted into ten animals. Five animals were evaluated each after 6 weeks and 26 weeks. For each interval there was a control group of five animals. After 6 weeks the control defects exhibited negligible bone regeneration at the defect margins but showed substantially more bone regeneration after 26 weeks extending up to 2.5 mm into the defect space. The TCP specimens showed a number of multinuclear cells on the material surface and direct bone/implant contact in a few locations but no signs of gross degradation or volume reduction after 6 weeks. The amount of bone ingrowth and cellular behavior had not changed after 26 weeks with resorption still going on. Glass ceramic implants by contrast appeared to be even better tolerated after 6 weeks with remarkable bone ingrowth and broad osseous fixation of the pellet to the defect margins and beyond, while highly vascular connective tissue filled the remaining pores of the implant. After 26 weeks the material had been extensively degraded, leaving behind only a few remnants, which were surrounded by seams of highly vascularized and cell-rich resorptive connective tissue with newly formed bone tissue nearly bridging the defect. The polylactic acid/ceramic composite implants showed hardly any tissue ingrowth or degradation either after 6 or after 26 weeks. Hence all tested materials appeared to be well tolerated at the site of implantation. However, gradual replacement by bone ingrowth tended to occur only in implants without polylactic acid.

Animals↗

[Functional and esthetically oriented reconstruction of lip defects].

Reconstruction of lip defects presents a challenge to the surgeon. Apart from attaining a good aesthetic result, preserving the function of the oral sphincter is an essential objective. A variety of techniques and modifications of procedures for reconstructing lip defects have been reported in the literature. It is up to the surgeon to select the reconstruction procedure ensuring optimum functional and cosmetic results, depending on the location, size, and depth of the defect. The division of the lips into aesthetic subunits can prove very beneficial in designing lip reconstruction procedures. The upper lip is divided into three aesthetic subunits: two lateral subunits and a median subunit, the philtrum. The lower lip constitutes a single subunit. In this study, established reconstruction techniques are discussed and a concept serving to facilitate the appropriate choice of technique is introduced. This concept differentiates between vermilion defects on the one hand, and partial-thickness and full-thickness lip defects on the other hand. For the lower lip, the full-thickness defects are classified as defects involving one-third, one-third to two-thirds of the lower lip width, and between two-thirds and complete loss of lip tissue. For the upper lip, a distinction is made between isolated defects of the central and lateral subunits and combined defects of the central and lateral subunits. Important principles of surgery and the advantages or disadvantages of different reconstruction techniques are discussed.

Basal Cell Carcinoma↗

[Radiologic evaluation of acetabular defects on acetabular loosening of hip alloarthroplasty].

In 18 human pelvis specimens we produced different acetabular defects according to Paprosky. Standardized X-rays of these specimens were taken, and these X-rays were evaluated by six orthopaedic surgeons with different levels of surgical experience. A total of 350 evaluation sheets were analyzed. The X-rays of the acetabular rim were correctly interpreted in only 66% of cases. The interpretation of plates showing bone loss was even worse. A medial wall defect was correctly assessed in just 49% of cases, giving a correlation (r) or 0.60 between the real defect and the radiological estimation. While massive defects were recognized in most cases, moderate defects were correctly estimated in about 50%. Moderate defects included migration of the cup, which was overlooked in between 46% and 69% of cases. Similar results were achieved in the judgement of bone loss. Only in 25% was direct classification according to Paprosky successful; it was better in small defects and worse in massive defects, with 16%. The interobserver correlation was only 0.54. In general, in most cases the defects were underestimated. This study raises the question of whether classification systems can provide the information needed for clinical useful therapeutic algorithms. The fact that the real bone loss is generally underestimated must receive due considerations when operations are being planned.

Acetabulum↗

Time course of transient cortical scintigraphic defects associated with acute pyelonephritis.

BACKGROUND: Acute pyelonephritis is distinguished from renal scarring using repeat cortical scintigraphy. The defects of acute pyelonephritis resolve, while those of scars persist. OBJECTIVE: To determine the duration of reversible cortical defects following acute pyelonephritis and the time interval required to differentiate infection from scars. MATERIALS AND METHODS: An observational prospective study of 193 children (386 kidneys) aged less than 5 years following their first proven urinary tract infection (UTI). Renal cortical scintigraphic defects were detected in 112 (29%) kidneys within 15 days of diagnosis. Of these, 95 underwent repeat renal cortical scans 2 years after the UTI, including 50 with additional scans performed within 2-6 months of infection. RESULTS: Of the 50 kidneys undergoing a second renal cortical scan within 2-6 months of the first UTI, 22 (44%) had persistent defects. A third scan was performed on 17 (77%) kidneys after 2 years, by which time defects had resolved in another 8 (47%) kidneys. The predictive value of defects detected within 2-6 months of UTI representing scars is 53% (95% CI 28, 77). Overall, nine (18%) kidneys with initial renal cortical abnormalities had permanent defects. In the 45 kidneys undergoing a second cortical scan more than 6 months after the UTI, 11 (24%) had persistent defects. None of the 95 kidneys undergoing serial scans developed new or larger defects. CONCLUSIONS: Renal scars may not be reliably diagnosed by cortical scintigraphy performed within 6 months of UTI because the inflammatory lesions may not have fully resolved.

Acute Disease↗

Concordance between rest MIBG and exercise tetrofosmin defects: possible use of rest MIBG imaging as a marker of reversible ischaemia.

Perfusion imaging combined with pharmacological stress is the study of choice in patients with ischaemic heart disease who are incapable of exercising. Some medical conditions, however, can preclude the use of pharmacological stress. In these particular situations, availability of a diagnostic test which allows for the assessment of ischaemic territory at rest would be desirable. With the purpose of providing a marker of reversible ischaemia, we evaluated myocardial iodine-123 metaiodobenzylguanidine (MIBG) uptake in regions with fixed and reversible defects defined by exercise/rest perfusion study. Fifty-four male patients with ischaemic heart disease and previous myocardial infarction were studied by means of exercise/rest tetrofosmin and MIBG single-photon emission tomography (SPET). Regional tracer uptake was quantified and expressed as a percentage of maximum peak activity. Areas with denervated but perfused myocardium and areas with ischaemic myocardium were calculated. Regions with<75% of peak activity in the exercise perfusion study were divided into two groups according to whether the increase in peak activity in the respective rest study was >10% (reversible regional defect) or <10% (fixed regional defect). These percentages were compared with the percentages of the innervation study. The area of the innervation defect was significantly larger when the perfusion defect was reversible than when it was fixed. In regions with reversible perfusion defects, the size of the area of denervated but perfused myocardium was similar to the size of the area of ischaemic myocardium. In regions with reversible defects, the percentage of myocardial MIBG uptake was not significantly different from the percentage of tetrofosmin uptake at exercise, while it was significantly lower than the percentage of tetrofosmin uptake at rest. In regions with fixed defects, the percentage of myocardial MIBG uptake was significantly lower than the percentage of tetrofosmin uptake at exercise and at rest. In patients who developed angina during exercise test, the area of denervated but perfused myocardium was significantly larger than in patients without angina (4.1+/-2.4 vs 3.4+/-2.5, P=0.02). The same trend was observed with regard to the size of the innervation defect (8.6+/-2.4 vs 5.7+/-2.2, P=0.02). It is concluded that when the use of pharmacological stress is not possible in patients incapable of exercising, rest studies with MIBG combined with rest myocardial perfusion studies may be useful as a marker of reversible ischaemia.

3-Iodobenzylguanidine↗

Cell-based treatment of osteochondral defects in the rabbit knee with natural and synthetic matrices: cellular seeding determines the outcome.

INTRODUCTION: Matrix-associated transplantation of cartilage constructs is an appealing method in cartilage repair. Three different matrices seeded with allogenic chondrocytes were compared in an osteochondral defect model in the rabbit. An investigation was conducted to identify the best matrix for cell-based treatment of osteochondral defects in the rabbit knee joint. MATERIALS AND METHODS: Osteochondral defects (diameter 3 mm) were created in the trochlea and the femoral condyles of 33 New Zealand White rabbits, which were then treated with bioartificial cartilage constructs. The cartilage constructs were created in vitro using three different resorbable carrier materials (two fleece matrices: one of PLLA, and one composite of polydioxanon/ polyglactin, as well as one consisting of lyophilized dura) cultured with isolated allogenic chondrocytes. The defects were evaluated macroscopically, by histological and immunhistological techniques, and by scanning electron microscopy after 6 weeks, 6 months, and 12 months. The chondrocyte-seeded constructs were compared to defects treated with carrier material alone as well as to untreated control defects. RESULTS: There was a significant improvement in defect repair quality in the transport materials, which were cultured with chondrocytes prior to implantation (P < 0.0005). No significant differences were observed between the three carrier matrices, and no significant differences were seen between the unseeded matrices and the untreated control defects. CONCLUSION: There is no difference in the outcome between the three tested matrices in the treatment of osteochondral defects in the rabbit knee. The results of this in vitro experiment are promising and with refinement may lead to useful clinical therapies.

Animals↗

Visual field defect and perfusion of the juxtapapillary retina and the neuroretinal rim area in primary open-angle glaucoma.

BACKGROUND: At this time little information is available about the relationship between glaucomatous visual field defects and impaired blood flow in the optic nerve head. The purpose of this study was to examine blood flow of the juxtapapillary retina and the rim area of the optic nerve head in primary open-angle glaucoma with a borderline visual defect. METHODS: Juxtapapillary retinal and neuroretinal rim area blood flow was measured by scanning laser Doppler flowmetry (SLDF). The visual field was evaluated by static perimetry (Octopus-G1). The optic nerve head was assessed on 15 degrees color stereo photographs. We examined 116 eyes of 91 patients with POAG with controlled IOP and 66 eyes of 44 healthy individuals. The POAG group was divided into eyes with a mean defect lower than 2 dB (POAG group I) and in eyes with a mean defect equal to or greater than 2 dB (POAG group II). The mean age of POAG group I and POAG group II was 55 +/- 11 years and 57 +/- 10 years, respectively. The mean age of the control group was 45 +/- 15 years. The eyes of POAG group I had an average C/D ratio of 0.71 +/- 0.18 with an average mean defect of the visual field of 0.97 +/- 0.68 dB; the eyes of POAG group II had an average C/D ratio of 0.80 +/- 0.17 with an average mean defect of the visual field of 8.2 +/- 6.0 dB. The intraocular pressure on the day of measurement in POAG group I was 18.2 +/- 3.7 mmHg, in POAG group II 17.6 +/- 4.0 mmHg, and in the control group 15.1 +/- 2.5 mmHg. For statistical analysis, age-matched groups of 32 normal eyes of 32 subjects (mean age 52 +/- 10 years) were compared to 18 glaucomatous eyes of 18 patients (POAG group I, mean age 55 +/- 11 years) and 59 glaucomatous eyes of 59 patients (POAG group II, mean age 55 +/- 10 years). RESULTS: In the eyes of POAG group I and POAG group II, both juxtapapillary retinal blood flow and neuroretinal rim area blood flow were significantly decreased compared to an age-matched control group: neuroretinal rim area "flow" POAG group I -65%, POAG group II -66%; juxtapapillary retina "flow" POAG group I -52%, POAG group II -44%. All eyes of the POAG group I (MD < 2 dB) and 56 of 61 eyes of the POAG group II (MD > = 2 dB) showed a retinal perfusion lower than the 90% percentile of normal blood flow. We found no correlation between reduction of juxtapapillary or papillary blood flow and mean defect in POAG eyes. CONCLUSION: Glaucomatous eyes with no defects or borderline visual field defects as well as glaucomatous eyes in an advanced disease stage show significantly decreased optic nerve head and juxtapapillary retinal capillary blood flow.

Blood Flow Velocity↗

Defect repair after somite removal in avian embryos is not true regeneration.

The question of regeneration after experimental somite extirpation has been controversial in the literature. While all workers agree that repair of the defects occurs, results concerning the extent and mechanism of this process, as well as the origin of the cells filling the defect, show great discrepancies. Our approach towards a re-examination of this question involved microsurgical removal of individual somites in 2-day-chick embryos in combination with grafting of quail somites and lateral plate. We show that the defect in the paraxial mesoderm is filled within a day after extirpation and that the reconstituting cells are derived only from the cranial and caudal somites, but not from the contralateral somites or from the lateral plate. There are no indications of an increase of proliferation in the neighbouring somites. In order to examine the differentiation capacities of the cells that fill the defect, we used immunohistochemistry and in situ-hybridization. We show that the cells in the defect are mesenchymal in morphology and express Pax-1 and Twist. There are a few desmin-positive cells in the defect that can be shown to derive from adjacent somites. An epithelial dermomyotome and myotome are absent at the operation site. Neural crest cells do not participate in the reconstitution of the defect. We conclude that cells in the defect either already have or adopt a ventral somitic (sclerotomal) identity, whereas derivatives with dorsal identity are absent from the defect except for a few individual cells.

Animals↗

Defective antibody production in patients with rheumatoid arthritis and bronchiectasis.

Bronchiectasis (BR) occurs in about 3% of patients with rheumatoid arthritis (RA). Defective antibody production is a rare but well-recognised cause of both BR and inflammatory arthritis. We examined the hypothesis that subtle specific antibody defects might play a role in the pathogenesis of BR associated with RA. Identification of defects in antibody production is important because substantial benefits may be gained from immunoglobulin replacement. Specific antibody production was assessed in 20 patients with RA and BR, 20 with BR alone, 20 with RA alone and 20 healthy controls (all groups matched for age and sex). All had normal total IgG. IgA and IgM and IgG subclass levels. Specific antibody production was assessed by assay of antibodies to representative polysaccharide and protein antigens. Subjects with subprotective titres were challenged with the appropriate vaccine. Defective antibody production was defined as a subprotective level despite immunisation. Three out of 20 patients with RA and BR had a defective IgG2 response to the polysaccharide antigen, but normal responses to the protein antigen. All of the subjects in the BR alone or healthy control group had normal antibody production. Two out of 20 patients with RA alone had defective production of antibodies against both protein and polysaccharide antigens; both were receiving gold therapy, a recognised cause of functional antibody defects. It was concluded that some patients with RA and BR have functional antibody defects and may benefit from antibody replacement. An unexpectedly high proportion of patients with RA alone also have functional antibody defects, possibly secondary to gold therapy.

Adult↗

Do internal anal sphincter defects decrease the success rate of anal sphincter repair?

BACKGROUND: Anatomic anal sphincter defects can involve the internal anal sphincter (IAS), the external anal sphincter (EAS), or both muscles. Surgical repair of anteriorly located EAS defects consists of overlapping suture of the EAS or EAS imbrication; IAS imbrication can be added regardless of whether there is IAS injury. The aim of this study was to assess the functional outcome of anal sphincter repair in patients intraoperatively diagnosed with combined EAS/IAS defects compared to patients with isolated EAS defects. METHODS: The medical records of patients who underwent anal sphincter repair between 1988 and 2000 and had follow-up of at least 3 months were retrospectively assessed. Fecal incontinence was assessed using the Cleveland Clinic Florida incontinence score wherein 0 equals perfect continence and 20 is associated with complete incontinence. Postoperative scores of 0-10 were interpreted as success whereas scores of 11-20 indicated failure. RESULTS: A total of 131 women were included in this study, including 38 with combined EAS/IAS defects (Group I) and 93 with isolated EAS defects (Group II). Thirty-three patients (87%) in Group I had imbrication of a deficient IAS, compared to 83 patients (89%) in Group II. All patients had either overlapping EAS repair (n=121) or EAS imbrication (n=10). Mean follow-up was 30.9 months (range, 3-131 months). There were no statistically significant differences between the two groups relative to age (48.3 vs. 53.0 years; p=0.14), preoperative incontinence score (16.1 vs. 16.7; p=0.38), extent of pudendal nerve terminal motor latency pathology (left, 11.1% vs. 8%; p=0.58; right, 8.6% vs. 15.1%; p=0.84), extent of pathology at electromyography (54.8% vs. 60.1%; p=0.43), and length of follow-up (26.9 vs. 32.5 months; p=0.31). The success rates of sphincter repair were 68.4% for Group I versus 55.9% for Group II (p=NS). Both groups were well matched for incidence of IAS imbrication as well as age, follow-up interval, and physiologic parameters. The success rates of anal sphincter repair were not statistically significant between the two groups. CONCLUSION: A pre-existing IAS defect does not preclude successful sphincteroplasty as compared to repair of an isolated EAS defect. Thus, patients with combined anal sphincter defects should not be considered as poor candidates for sphincter repair.

Anal Canal↗

Defects in auditory sensory gating and their apparent compensation in relatives of schizophrenics.

Auditory sensory processing is defective at several stages in schizophrenics, as revealed by electrophysiological recordings. The purpose of this study was to assess the relationship between two of these defects in schizophrenics and their relatives. One defect is illustrated by the failure to gate the P50 wave of the auditory evoked potential in the conditioning-testing paradigm. In this paradigm, paired clicks are presented to the subject. Normals suppress or gate the P50 response to the second or test click. Schizophrenics fail to suppress the test response. This defect has been related to schizophrenics' inability to filter out noise in their environment. A second defect is illustrated by schizophrenics' lower than normal N100 wave, which has been related to failure to attend to particular features of interest in the stimulus. The question addressed in this study was whether these two defects inevitably occur together. While they do occur together in schizophrenics, even in very good prognosis, mildly ill subjects, they do not occur together in the relatives of schizophrenics. The defect in the gating of P50 occurs in half these relatives, but N100 amplitudes are not diminished. Instead, relatives with abnormal P50 gating have N100 amplitudes which are larger than normal. One interpretation of the data is that the relatives with the sensory gating defect can compensate for that defect at a subsequent stage of information processing, as demonstrated by their large amplitude N100 wave, whereas schizophrenic patients cannot.

Adult↗

Versatility of the sural fasciocutaneous flap in the coverage of lower third leg and hind foot defects.

BACKGROUND: Reconstruction of soft tissue defects of the lower third of the leg, the heel and the hind foot remains a challenge. The distally based sural artery fasciocutaneous flap has been used effectively to resurface these defects. In many instances, it has obviated the need for free tissue transfer. OBJECTIVE: The objective of the study is to evaluate the efficacy of reverse sural artery fasciocutaneous flap for coverage of lower third leg, posterior heel, malleoli and hind foot. STUDY DESIGN: This is a descriptive study, which was conducted on 84 patients who presented with soft tissue defects in the area of lower third leg, heel, malleoli and hind foot. PLACE AND DURATION OF STUDY: The study was conducted at department of plastic and reconstructive surgery, Federal Postgraduate Medical Institute Shaikh Zayed Hospital Lahore, over a period of 7 years from February 1997 to February 2005. PATIENTS AND METHODS: Over a period of 7 years, a total of 84 patients with Soft tissue defect of lower third leg, heel, malleoli and hind foot were included. Preoperative data, the age and sex of each patient, cause and site of defect, dimension of flap, transposition of pedicle (through a tunnel or laid open and covered with a skin graft), postoperative results and complications were recorded. All patients were followed up in out patients department for 6 months. RESULTS: Out of 84 patients, 54 were males and 30 females. Their ages ranged from 8 to 55 years with a mean of 31 years. Road traffic accidents was the cause of the defects in 53 patients, wheel spoke injury in 12 patients, trophic ulcer in five patients, osteomyelitis in five patients, marjolin ulcer in seven patients and diabetic ulcer in two patients. The site of 84 defects comprised 52 distal tibia; 20 tendo-Achillis and posterior heel defects; seven-malleolar region; three-anterior ankle and two-foot amputation stumps. The dimension of flap ranged from 5 to 15 cm in length and 4 to 12 cm in width. Postoperatively 66 flaps survived completely while marginal necrosis was seen in six patients and infection in four patients. The complete flap necrosis occurred in eight patients. There was no considerable morbidity at donor site and all patients had satisfactory functional outcome. CONCLUSIONS: The distally based superficial sural artery flap is a versatile, reliable procedure, useful in reconstruction of lower third leg, heel, malleoli and hind foot defects. The surgical technique is safe, of short duration and provides alternative to microsurgical reconstruction.

Adolescent↗