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At least 343 records · Page 19Linked to original sources
PubMed2005

[Elimination of mandibular defects with dosed distraction. Part III. Use of dosed distraction for restricted osteoplasty in subtotal and end defects of the mandible].

Two variants (3(rd) and 4(th)) of non-free mandible osteoplasty with the use of compression-distraction apparatus (CDA) elaborated during the war in Afghanistan are described by the author. The 3(rd) variant was used in cases of vast combined defects of lower part of the face when two-sided osteotomy of both mandible stumps in order to create mobile mandible fragments of the length of 2.5 cm and their follow-up shift to the middle line of the face with the help of CDA is made. Distraction of callosity and soft tissues stimulated histogenesis that led to substitution of big osseous-soft tissue defect of lower part of the face. The 4(th) variant had two stages: 1(st) stage resulted in reconstruction of mandible branch, the 2(nd) -- in reconstruction of mandible head. In such a way the new temporomandibular joint using own tissues of the patient was created without application of allotransplant or titanium explant.

Afghanistan↗
PubMed1976

Defective initiation of the metabolic stimulation in phagocytizing granulocytes: a new congenital defect.

Two patients suffering from recurrent bacterial infections were studied: a boy and a girl from one family, children of apparently healthy parents. The granulocytes of these patients were capable of normal ingestion of latex particles and DNA-anti-DNA immune complexes. When the metabolic changes in these granulocytes during phagocytosis of latex particles were studied, however, no stimulation of oxygen consumption, superoxide production, or hexose monophosphate shunt activity could be observed. Moreover, zymosan particles were not iodinated. These findings are comparable to those found in chronic granulomatous disease. In sharp contrast to the observations in this latter disease, however, a completely normal stimulation of cell metabolism was found after phagocytosis of IgG-coated latex particles or IgG aggregates. Since latex and IgG-coated latex were equally well ingested, this means that the absence of metabolic stimulation after uptake of tatexf metabolic stimulation after uptake of latex must be due to a defect in the triggering of the oxidative burst. As far as we know, this is the first time that a defect in the triggering of the metabolic stimulation during phagocytosis could be demonstrated. Moreover, these finding suggest that adherence and subsequent ingestion of particles are in themselves not sufficient for the metabolic stimulation of granulocytes.

Adult↗
PubMed1990

[Correlation between the retinal nerve fiber layer defects (RNFLD) and visual field defects in primary open-angle glaucoma].

The RNFLD and the visual field in 73 cases (124 eyes) of primary open-angle glaucoma were studied. It was found that the early RNFLDs were usually local, as combed-hair, slit-like, or wedge shaped defects, while in the medium or late stage, diffuse RNFL atrophy or mixed RNF-LD was the rule. In the group of localized RNFLDs, 8.8% of the patients showed no visual field anomalies, indicating that RNFL damages preceded visual field damages; in the rest of the patients, visual field defects corresponded with the RNFLD in 86.7% of the cases. In patients of the medium or late stage with mixed or diffuse RNFLD, nasal steps and isopter constriction were usually found that corresponded perfectly with the RNFLD.

Adult↗
PubMed1989

[The Bebié curve (cumulative defect curve) for differentiating local and diffuse visual field defects].

Damage to the visual field can be diffuse as well as local. In the absence of local defects, diffuse damage can easily be recognized with the help of the visual field indices. In the presence of scotomas, diffuse damage in the remaining part of the visual field which is better or "normal" is more difficult to recognize and quantify. Bebié et al. have published a new method of assessing the visual field in relation to normal values. They present the results with a cumulative defect curve, a method which we have called the "Bebié curve". This method allows an easy recognition of diffuse as well as local damage. In our study we evaluate the clinical application of the Bebíe curve in different diseases and for the follow-up of the visual field.

Computer Graphics↗
PubMed1988

[Defects in the prostaglandin system. VII. (Generalized, inherited [?]) cyclooxygenase defect].

Investigation of platelet function in a 55 year-old male suffering from peripheral vascular disease revealed platelet cyclooxygenase deficiency. Examination of femoral artery tissue at a later date likewise showed the presence of a cyclooxygenase defect. Investigated relatives were not affected. A 39 year-old male smoker admitted with an acute myocardial infarct without prodromal symptoms exhibited similar laboratory findings. In contrast to the cases reported in the literature so far, both our patients suffered from severe atherosclerosis, a relatively high platelet activity and an additional cyclooxygenase defect of cells other than the platelets. The patient with peripheral vascular disease died from sudden cardiac arrest.

Adult↗
PubMed1977

[Covering of skin defect wounds with a synthetic skin substitute. Conditioning of defect wounds of various origins with a synthetic skin substitute as a preparation for skin transplantation].

Treatment of large skin defects with a synthetic substitute for homograft skin is described. Defect wounds of different genesis, particularly with heavy infections, were treated with the synthetic skin substitute Epigard (Parke-Davis). An advantage is an easy wound dressing and good condition of the wound ground with development of a sufficient vascularization and cleaning of the wound bed for the reconstructive procedures keeping the wound ready for autotransplantation.

Adult↗
PubMed1983

A defect in the suppressor circuits among OKT4+ cell populations in patients with systemic lupus erythematosus occurs independently of a defect in the OKT8+ suppressor T cell function.

The autologous mixed lymphocyte reaction (MLR) is thought to be part of a regulatory role of T cells on B cell function. OKT4+, but not OKT8+, cells can proliferate in response to autologous non-T cells. Moreover, the OKT4+ cell population activated early in the course of autologous MLR functioned as inducer cells for the differentiation of B cells, whereas later in the response, the activated OKT4+ cells were particularly enriched in suppressor cells. A part of the autologous MLR appears to be an important pathway for the activation of feedback suppression mechanisms among cells contained within the OKT4+ populations. Patients with systemic lupus erythematosus (SLE) were studied with regard to the following OKT4+ cell functions in vitro after activation in the autologous MLR: a) proliferative response, and b) helper and suppressor activities for differentiation of B cells. A marked reduction in the proliferative response of OKT4+ cells was observed in SLE patients. SLE OKT4+ cells activated in the autologous MLR could function as helper cells but could not exert any suppressor activity. This OKT4+ cell abnormality was present regardless of the disease activity, and occurred in the absence of autoantibodies including anti-T cell antibodies. Instead, SLE anti-T cell antibodies could preferentially eliminate cells bearing the OKT8+ phenotype characteristic of suppressor cells in populations of normal T cells. These results suggest that the defect in the suppressor circuits among OKT4+ cell populations is intrinsic to SLE lymphocytes and that the OKT8+ suppressor T cell defect is caused by antibodies produced by the B cells of SLE patients.

Adult↗
PubMed1994

Skeletal defects. A comparison of bone grafting and bone transport for segmental skeletal defects.

To evaluate two different methods of managing segmental skeletal defects, 15 patients treated with the open bone graft (Papineau) technique were compared with 17 patients who had intercalary bone transport (Ilizarov) management. The treatment time was identical for both groups: 1.9 months in fixation for each centimeter of defect reconstructed. Both techniques shared several fixator-associated problems such as implant site sepsis and patient discomfort. Each method of treatment, however, had its own unique problems. For the bone grafted group, limited graft availability, donor site morbidity (three patients), and graft fractures (two patients) occurred. For the bone transport group, the main problems were failure of the docking site to unite without a supplementary graft (seven patients) and joint contractures (seven patients). A new synthesis of both techniques is described.

Adolescent↗
PubMed1993

A Chinese hamster ovary cell line with a temperature-conditional defect in receptor recycling is pleiotropically defective in lysosome biogenesis.

We have previously described the isolation of a Chinese hamster ovary cell line, TfT1.11, that has a pleiotropic, temperature-conditional defect in receptor recycling (Cain, C. C., Wilson, R. B., and Murphy, R. F. (1991) J. Biol. Chem. 266, 11746-11752). These cells show a rapid loss of cell surface receptors upon temperature shift due to a reduction in the rate of receptor recycling. We show here that, in addition to altered receptor recycling, TfT1.11 cells show three defects in lysosome biogenesis. At the nonpermissive temperature, they 1) redistribute at least one lysosomal enzyme from lysosomes to endosomes, 2) fail to transfer fluid-phase material from early endosomes to later compartments, and 3) fail to accumulate fluid-phase markers due to increased efflux of internalized material. The results suggest that the processes of recycling from the endosome and movement of material from endosomes to lysosomes are tightly linked.

Animals↗
PubMed1996

Human platelet signaling defect characterized by impaired production of inositol-1,4,5-triphosphate and phosphatidic acid and diminished Pleckstrin phosphorylation: evidence for defective phospholipase C activation.

Signal transduction on platelet activation involves phosphoinositide-specific phospholipase C (PLC)-mediated hydrolysis of phosphatidylinositides and formation of inositol-1,4,5-triphosphate [I(1,4,5)P3], which mediates Ca2+ mobilization, and diacylglycerol (DG), which activates protein kinase C (PKC) to phosphorylate a 47-kD protein (Pleckstrin). We studied these events in two related patients previously reported (Blood 74:664, 1989) to have abnormal aggregation and 14C-serotonin secretion, and impaired intracellular Ca2+ mobilization in response to several agonists. Thrombin-induced I(1,4,5)P3 and phosphatidic acid formation were diminished. Pleckstrin phosphorylation was impaired on activation with thrombin, platelet-activating factor, and ionophore A23187, but was normal with PKC activator 1,2-dioctonyl-sn-glycerol (DiC8). Ca2+ mobilization induced by guanosine triphosphate (GTP) analog guanosine 5'-0-(3 thiotriphosphate) (GTP gamma S) was diminished. Pretreatment with either A23187 or DiC8 did not correct the impaired adenine diphosphate-induced secretion; however, upon stimulation with A23187 plus DiC8, pleckstrin phosphorylation and secretion were normal, indicating that both PKC activation and Ca2+ mobilization are essential for normal secretion. We conclude that these patients have a unique inherited platelet defect in formation of two key intracellular mediators [I(1,4,5)P3 and DG] and in the responses mediated by them due to a defect in postreceptor mechanisms of PLC activation.

Adenosine Diphosphate↗
PubMed1996

Abrogation of c-kit/Steel factor-dependent tumorigenesis by kinase defective mutants of the c-kit receptor: c-kit kinase defective mutants as candidate tools for cancer gene therapy.

The growth and survival of many types of cancer cells are known to be supported by specific growth factor/cytokine systems. Among these, the activation of c-kit receptor and its ligand steel factor participates in several types of human carcinogenesis. W mutations of laboratory mouse strains are loss of functional mutations of the c-kit receptor. To examine the validity of these mutants in investigating c-kit-mediated carcinogenesis and in the treatment of c-kit-dependent tumors, we introduced various W mutations (W, Wv, and W42) into a transgenic mouse strain carrying human papillomavirus oncogenes, in which c-kit/Steel-mediated tumorigenesis occurs with a very high incidence. In all transgenic strains carrying a W mutation, the c-kit deficiency affected the tumorgenic process to various degrees. Tumor development was markedly suppressed in transgenic strains carrying kinase defective mutations (Wv and W42) in a heterozygous condition. In null-type (W) heterozygous transgenic mice, tumorigenesis was suppressed at a lower level. Moreover, minimal focal legions or, in some cases, no focal legions were found in the testes of W/Wv heterozygous transgenic mice, showing a close relationship between tumor cell growth and the degree of c-kit inactivation. These results indicated that c-kit activity is a pivotal determinant of testicular tumor development and that the kinase defective mutants of c-kit are valuable for treating c-kit-dependent cancer, as well as for clarifying the c-kit-mediated carcinogenesis.

Animals↗
PubMed2004

Device closure of muscular ventricular septal defects using the Amplatzer muscular ventricular septal defect occluder: immediate and mid-term results of a U.S. registry.

OBJECTIVES: We sought to report the results of a U.S. registry of device closure of congenital muscular ventricular septal defects (VSDs) using the new Amplatzer mVSD occluder (AGA Medical Corp., Golden Valley, Minnesota). BACKGROUND: Muscular VSDs pose a significant surgical challenge with increased morbidity and mortality. METHODS: Data were prospectively collected from 83 procedures involving 75 patients who underwent an attempt of percutaneous (70 [93.3%] of 75) and/or perventricular (surgical) (6 [8.0%] of 75) device closure of hemodynamically significant muscular VSDs. The patients' median age was 1.4 years (range 0.1 to 54.1 years). Outcome parameters were procedural success, evidence of residual shunts on echocardiography, and occurrence of procedure-related complications. The median follow-up was 211 days (range 1 to 859 days). RESULTS: The median size of the primary VSD was 7 mm (range 3 to 16 mm) and in 34 of 78 (43.6%) procedures, patients had multiple VSDs (range 2 to 7). The device was implanted successfully in 72 of 83 (86.7%) procedures. In 17 of 83 (20.5%) procedures, multiple devices were implanted (range 2 to 3). Procedure-related major complications occurred in 8 of 75 (10.7%) patients. Device embolization occurred in two patients and cardiac perforation in one patient. There were two (2.7%) procedure-related deaths. The 24-h postprocedural complete closure rate was 47.2% (34 of 72 patients), increasing to 69.6% (32 of 46 patients) at 6 months and 92.3% (24 of 26 patients) at 12 months. Six patients underwent successful closure using the perventricular surgical (beating heart) approach, with complete closure at day 1 in three patients and trivial/small residual shunts in the remainder of the patients. CONCLUSIONS: The Amplatzer mVSD device (AGA Medical Corp.) offers excellent closure rates and low mortality when used to close congenital muscular VSDs. The device appears to be safe and effective.

Adolescent↗
PubMed1993

Mental health and psychosocial functioning in adolescents with congenital heart disease. A comparison between adolescents born with severe heart defect and atrial septal defect.

Twenty-six adolescents, aged 13-18 years, with severe congenital heart disease were matched for sex, age and living area with 26 adolescents with repaired atrial septal defect and regarded as physically fit. These two groups were compared according to somatic condition, psychopathology, psychosocial functioning and chronic family difficulties. A higher rate of psychiatric problems in the complex group, an association between psychosocial functioning and physical capacity, as well as an association between psychosocial functioning and chronic family difficulties were observed. These findings suggest that physical capacity is of crucial importance for mental health and functioning of adolescents with congenital heart disease. The association with chronic family difficulties also suggests that a comprehensive biopsychosocial approach is necessary in the treatment and rehabilitation of these patients.

Adolescent↗
PubMed1990

[Partial closure of the atrial septal primum defect and biventricular repair for a case of hypoplastic right ventricle with partial atrioventricular canal defect].

An eight-year-old girl with partial atrioventricular canal defect and hypoplastic right ventricle was treated successfully by a palliative open-heart surgery. The preoperative right ventricular pressure was 58/7 mmHg (RVP/LVP = 0.67) and morphology of the right ventricle showed severe tricuspid stenosis and small outflow portion. The preoperative RVEDVI was 31 (41% of normal and tricuspid annulus was 17 mm (47% of normal). The right ventricular outflow was reconstructed with insertion of MVOP and the ASD was partially left open (the amplitude of the interatrial communication was 7 mm). Angiocardiogram two and half years after the operation demonstrated significant right ventricular growth with no right to left shunt through interatrial communication. The RVEDVI was 46 (56% of normal) and tricuspid annulus was 36 mm (90% of normal). This technique can be a procedure of choice in patient with right ventricular hypoplasia, who is not candidate for simple right ventricular reconstruction nor Fontan procedure, as the growth of the right ventricule is expected in the future.

Child↗
PubMed2004

Device closure of iatrogenic membranous ventricular septal defects after prosthetic aortic valve replacement using the Amplatzer membranous ventricular septal defect occluder.

Iatrogenic hemodynamically significant ventricular septal defects (VSDs) after aortic valve replacement are rare. The surgical risk to close such VSDs is substantial. Catheter closure has rarely been attempted because access to the left ventricle during cardiac catheterization in patients with a prosthetic aortic valve has been associated with a risk of catheter entrapment. We describe two cases of this rare entity after St. Jude and Medtronic valve replacement in which successful percutaneous closure of the iatrogenic VSDs was achieved using the Amplatzer membranous VSD occluder. Crossing the prosthetic valves with a delivery sheath and closure with the membranous VSD device was accomplished in both cases.

Adult↗
PubMed2005

Transcatheter atrial septal defect closure: modified balloon sizing technique to avoid overstretching the defect and oversizing the Amplatzer septal occluder.

The objective of this study was to evaluate a new technique of sizing atrial septal defects (ASDs) for transcatheter device closure. ASD closure using the Amplatzer septal occluder (ASO) device is commonly performed. Complications, including arrhythmias, pericardial effusions, and perforations, may be related to oversizing ASDs and choosing larger devices. Two methods were used to size ASDs using a compliant balloon. In some patients, the balloon was inflated until a waist was visible [(+)waist]; in others, only until no shunting was demonstrable by echocardiogram [echo; (-)waist]. The device was selected and implanted using standard procedure and echo guidance. One hundred seventeen patients underwent secundum ASD closure with an ASO device. There were 43 patients in the (-)waist group and 74 in the (+)waist group. All devices were implanted successfully. The initial echo ASD diameter was larger in the (-)waist group compared to the (+)waist group (P = 0.01). There was a smaller difference between the initial echo and balloon-sized ASD diameters in the (-)waist group (P < 0.02). ASO device size implanted (in mm greater than echo ASD diameter) was smaller in the (-)waist group (P < 0.01). There were 0/43 complications in the (-)waist group and 5/74 in the (+)waist group. The complete closure rate was the same in both groups. Sizing an ASD by inflating a compliant balloon just until shunting is eliminated, and not until a waist is visible, results in less overstretching of the ASD and selection of a smaller ASO device, achieving similar closure rates and potentially fewer complications.

Adolescent↗
PubMed1985

Maternal serum alpha-fetoprotein screening for neural tube defects. Report of a combined study in Germany and short overview on screening in populations with low birth prevalence of neural tube defects.

The basis of maternal serum alpha-fetoprotein (AFP)-screening for neural tube defects is discussed. A report is given of a large scale screening study in the Federal Republic of Germany combining the experiences in Giessen and Hannover on over 50,000 pregnant women, about evenly distributed among both centers. Published and known forthcoming data from other low incidence populations, particularly of European countries, are reviewed briefly. The conclusion is reached that general screening could effectively be instituted and in the final result should also be cost-beneficial.

Amniotic Fluid↗