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Biomedical subjects

M Marx

Publications and source records attributed to M Marx.

At least 55 records · Page 3Linked to original sources

Acute clinical deterioration in a patient with end-stage dilated cardiomyopathy and left ventricular thrombus formation.

The case of a 17-year-old male patient with severe end-stage dilated cardiomyopathy and a large thrombus formation within the cavum of the left ventricle is reported. After an acute thrombectomia combined with a partial left ventriculectomy (Batista procedure), the patient was successfully treated with an appropriate left ventricular assist device (LVAD) system using a centrifugal nonocclusive pump (Biomedicus, Medtronic, Anaheim, CA, U.S.A.). Mechanical support was removed on Day 9, and the patient was discharged from the hospital on Day 19. The effectiveness of emergency mechanical support in patients with very unfavorable prognoses is discussed.

Adolescent↗

Pulmonary failure after Norwood procedure: indication for extracorporeal membrane oxygenation? A case report.

Today some authors consider univentricular repair a contraindication for postoperative cardiac extracorporeal membrane oxygenation (ECMO). The question is whether or not ECMO is indicated as pulmonary support in case of an overwhelming pulmonary infection during the postoperative course after a Norwood procedure. During the prolonged weaning period after a Norwood procedure using a 4 mm aortopulmonary shunt, proven respiratory syncytial virus (RSV) bronchiolitis occurred at the time of expected weaning from artificial ventilation. Venovenous ECMO was able to improve oxygenation, but when pulmonary opacification failed to resolve, ECMO was terminated after 12 days.

Aorta↗

Aortopulmonary collateral artery embolization during postoperative extracorporeal membrane oxygenation after arterial switch procedure.

Aortopulmonary collateral arteries sometimes complicate cyanotic congenital heart defects. Combined with a relevant left-right shunt, this could result in massive airway bleeding during and after corrective surgery. A preoperatively diagnosed 1.2 mm small aortopulmonary collateral artery in a newborn suffering from transposition of the great arteries caused life-threatening airway bleeding during surgery. Postoperative extracorporeal membrane oxygenation (ECMO) was necessary, and coil embolization was performed on ECMO to terminate pulmonary bleeding.

Aorta↗

Extracorporeal membrane oxygenation discontinuation despite technically successful reoperation: A case report.

Death remains a probable outcome of pediatric cardiac extracorporeal membrane oxygenation (ECMO) despite increasing efforts to improve the results. On venoarterial ECMO, in an obviously hopeless situation, the decision to withdraw a life supporting measure resulting in the sudden death of a child places a heavy burden on the team. After valvulotomy of critical aortic stenosis in a prenatally diagnosed term neonate, ECMO had to be installed during postoperative resuscitation. Despite technically successful homograft implantation while on ECMO complicated by postoperative bleeding, advancing multiorgan failure resulted in ECMO withdrawal. As shown in this case report, exact termination criteria are lacking but are necessary to prevent increasing team and resource related conflicts in pediatric cardiac ECMO.

Aortic Valve↗

Valve-preserving treatment of Ebstein's anomaly: perioperative and follow-up results.

BACKGROUND: Ebstein's anomaly is a rare congenital cardiac defect of the tricuspid valve (TV) leading to severe tricuspid insufficiency (TI). METHODS: In ten patients, 6 adults (39-53 years) and 4 children (5-10 years), operated between 1989 and 1995 echocardiography was performed pre and post repair and at follow-up. Patients were assessed in our institution at two cut-off points, resulting in a mean first follow-up of 17 +/-15 months and a mean second follow-up of 53+/-23 months. All patients had additional congenital cardiac defects (ASD,VSD). In all patients the TV was repaired by techniques described by Carpentier et al. with some modifications. The goal of this reparative attempt is to mobilize restricted leaflet tissue and aid coaptation through implantation of a ring. RESULTS: Echocardiographically we were able to identify significant characteristics for the successful repair of Ebstein's anomaly. The severity of the disease is represented by the size and function of the right ventricle and the atrialized chamber, the most advanced cases exhibiting a dilated right ventricle with poor contractility. There was severe preoperative TI ( mean grade 3.2 +/- 0.3). Postoperatively TI was significantly reduced to a mean grade of 2+/-0.2. 60% of the patients demonstrated an improvement in the ratio of atrialized chamber to functional right ventricle. Right-ventricular function was improved, the mean score being 2.8+/-0.1. At follow-up I and II right-ventricular function and tricuspid insufficiency was improved in most patients and all patients benefited in quality of life. CONCLUSION: These results suggest that surgical correction should not be delayed until severe right heart failure develops as, particularly in children, good results are achieved, improving the quality of life.

Adult↗

[Side effects of postoperative radiochemotherapy with amifostine versus radiochemotherapy alone in head and neck tumors. Preliminary results of a prospective randomized trial].

PURPOSE: Experimental and clinical data suggest a reduction of radiation-induced acute toxicity by amifostine. We investigated this issue in a randomized trial comparing radiochemotherapy (RCT) versus radiochemotherapy and amifostine (RCT + A) in patients with head and neck cancer. PATIENTS AND METHODS: Forty-seven patients with pharyngeal or laryngeal cancer (T1-2 N1-2 G3, T3-4 N0-2 G1-3) were randomized to receive RCT alone (21 patients) or RCT + A (21 patients). Patients were irradiated up to 60 Gy (R0) or 70 Gy (R1/2). Chemotherapy consisted of 70 mg/m2 carboplatin and was administered over 5 days in the 1st and 5th week of the radiotherapy course. 250 mg amifostine were applied daily just before each radiotherapy session. Acute toxicity was evaluated according to the Common Toxicity Criteria (CTC). As for xerostomia no patients with laryngeal cancer were assessed because in these cases only small volumes of the salivary glands were within the treatment volume. To evaluate the overall toxicity a summarized CTC score of all observed side effects was calculated. RESULTS: Forty-two patients were evaluable. Clinical characteristics (age, sex, Karnofsky index, tumor-staging) were well balanced in both treatment groups (Tables 2 and 3). Amifostine provided reduction in xerostomia and mucositis (Figures 5 and 6) but had no obvious influence on Karnofsky index, body weight, cutaneous side effects and alopecia (Figures 1 to 4). CONCLUSIONS: According to our preliminary results amifostine has a radioprotective effect on salivary glands. Mucositis can be reduced during radiochemotherapy. At this point of patient accrual the difference between both groups are statistically not significant. To improve the radioprotective effects of amifostine in clinical practice the application of a higher dose (> 250 mg) seems to be necessary.

Adenocarcinoma↗

Differential expression of osteopontin, PC4, and CEC5, a novel mRNA species, during in vitro angiogenesis.

The formation of new capillaries from preexisting blood vessels, a process termed angiogenesis, plays a key role in many physiological and pathological conditions such as wound healing, embryogenesis, and tumor growth. The aim of this study was to identify changes in endothelial cell gene expression specifically associated with angiogenesis. Using an in vitro model and the differential display strategy, we compared gene expression patterns of rat microvascular endothelial cells cultured in two (2D) and three-dimensional (3D) culture. In 2D culture, the cells express actin and proliferate, whereas in 3D culture actin expression is downregulated, and the cells are mitotically quiescent and reorganize into vascular tubes. We identified three differentially expressed genes, osteopontin, PC4, and CEC5, a novel mRNA species, with homology to calmodulin-dependent protein kinases. The expression patterns were confirmed by Northern blot analysis. In conclusion, the analysis of gene expression in endothelial cells in 3D and 3D culture allows the identification of genes differentially expressed during angiogenesis. These genes or proteins may serve as targets for therapeutic modulation of angiogenesis.

Actins↗

Regulation of osteopontin expression in rat mesangial cells.

Hypercellularity and accumulation of extracellular matrix are common responses of renal glomeruli to inflammatory stimuli. Using the differential display approach, we compared the gene expression patterns of proliferating and differentiating rat mesangial cells in two- and three-dimensional cultures. Osteopontin, an extracellular matrix protein, was found to be transcribed, synthesized, and secreted by rat mesangial cells. Osteopontin transcription was not associated with cell proliferation and was found to be FCS-inducible in proliferating cells. Osteopontin expression was independent of exogenously supplied FCS in differentiating cells. The presented data indicate that osteopontin is differentially regulated in proliferating and differentiating mesangial cells.

Animals↗

Clinical use of a simulation-multileaf collimator.

BACKGROUND: At the University of Lübeck, radiotherapy is delivered by a 6/18-MV linear accelerator. Using the integrated multileaf collimator, irradiation of individually shaped treatment fields is possible in place of alloy blocks. Due to unsatisfactory pretherapeutic review of the radiation-field-specific multileaf collimator (MLC) configuration, we developed a simulation-multileaf collimator (SMLC) and assessed its feasibility at different tumor sites. MATERIAL AND METHODS: The SMLC is made of a perspex carrier with 52 horizontal sliding leaves. The position of each leaf is calculated by a 3D treatment-planning computer. The technician manually adjusts the leaves according to the beams-eye-view plot of the planning computer. Consequently, the SMLC is mounted on the therapy simulator at a distance of 64.8 cm from the focus. The treatment fields and the position of the leaves are documented by X-ray films. RESULTS: Using the SMLC, radiation oncologists are able to review exactly the leaf configuration of each MLC-shaped radiation field and to correlate the MLC-shaped radiation field with the treated volume, the organs at risk and the port films acquired by the Portal Vision system. CONCLUSION: The SMLC is a new tool to review radiation planning that uses an MLC in daily routine. The use of the SMLC improves the documentation and the quality assurance. It accelerates the treatment field review at the linear accelerator by comparing the SMLC simulator films with the portal images.

Calibration↗

Echocardiographic evidence of aortopulmonary collaterals in premature infants after closure of ductus arteriosus.

Aortopulmonary collaterals occur in a variety of congenital heart diseases, in chronic pulmonary infection and abscesses, in association with lung tumors, and after multiple pulmonary emboli. In patients with congenital cyanotic heart disease aortopulmonary collaterals mainly occur in conditions with reduced pulmonary blood flow. We investigated 12 preterm low-birth-weight infants, gestational age 29.3+/-3.3 weeks, with respiratory failure who suffered from moderate to severe chronic lung disease after a period of mechanical ventilation. All patients developed aortopulmonary collaterals after closure of a patent ductus arteriosus. Aortopulmonary collaterals could be displayed clearly by color Doppler echocardiography and originated mainly from the descending aorta or the aortic arch. Hypoxic and hypercapnic episodes favored the development of aortopulmonary collaterals, which disappeared after pulmonary hemodynamics and respiratory function had improved. In only one patient coiling of a large col lateral vessel had to be performed. Systemic-to-pulmonary collateral vessels potentially aggravate chronic lung disease by increasing collateral pulmonary blood flow and reducing lung compliance. We conclude that aortopulmonary collaterals occur in bronchopulmonary dysplasia and can cause major problems in ventilated premature infants. Echocardiographic evaluation is important to prevent aggravation of chronic lung disease of infants at risk.

Aorta, Thoracic↗

Therapeutic options for critically ill patients suffering from progressive lupus nephritis or Goodpasture's syndrome.

Systemic lupus erythematosus is a chronic disease with many clinical features, while Goodpasture's syndrome usually becomes manifest with progressive glomerulonephritis and pulmonary hemorrhage. Rapidly declining renal function and even pulmonary hemorrhage may be the common feature. Early and precise diagnosis is most important as it may provide general prognostic information and serve as a guideline for initial therapy. Immunosuppression with oral cyclophosphamide and high dose corticosteroids together with plasmapheresis is used in Goodpasture's syndrome. Progressive lupus nephritis requires high dose corticosteroids together with i.v. pulses of cyclophosphamide for at least six months, followed by maintenance immunosuppression. The benefits of therapy must always be weighed against the risks. Nevertheless, current therapy remains less than optimal. A better understanding of the pathogenesis of systemic lupus erythrematosis (SLE) and Goodpasture's syndrome may provide more specific information about the nature and the role of the immune response and thus lead to new treatment strategies.

Anti-Glomerular Basement Membrane Disease↗

Intratracheal furosemide in infants after cardiac surgery: its effects on lung mechanics and urinary output, and its levels in plasma and tracheal aspirate.

OBJECTIVE: Recent studies have suggested direct pulmonary effects of furosemide in asthmatics and infants with bronchopulmonary dysplasia. We tested the hypothesis that intratracheally administered furosemide also increases respiratory compliance in children after cardiac surgery, and investigated whether furosemide has a topical and/or systemic action. STUDY DESIGN: Prospective study with intra-individual control. In twelve infants and toddlers (age: 10 +/- 8 months, weight: 6.9 +/- 3 kg) mechanically ventilated for compromised lung mechanics after cardiac surgery, 0.5 mg/kg furosemide was intratracheally administered to the lungs. Lung mechanics were serially assessed using a computerised system (Sensormedics 2600) during a 2 h control and 2 h intervention period. Urine output was measured by an indwelling bladder catheter and levels of furosemide were determined in blood and tracheal aspirates. RESULTS: Static compliance improved within 30 min in all patients, reached a maximum of 44 (20-85)% above baseline and remained improved throughout the study (p < 0.05). An immediate, short and significant diuretic effect of intratracheally applied furosemide was observed. Furosemide levels 1 h after intervention were 795 ng/ml in the blood and 431 micrograms/ml (i.e. 1000-fold higher) in the tracheal aspirate. Changes in compliance were correlated only to urine output values over the 2 h (r = 0.82, p = 0.044, n = 9) after furosemide administration. CONCLUSION: We conclude that intratracheally applied furosemide improves static compliance in infants and toddlers with compromised lung mechanics after cardiac surgery. We demonstrated that furosemide is absorbed from the lung and has a systemic effect within 15 min after its intratracheal instillation.

Administration, Topical↗

Preoperative ECMO in congenital cyanotic heart disease using the AREC system.

BACKGROUND: In cyanotic congenital heart disease, oxygen delivery is impaired either by reduced pulmonary perfusion or by limited entry of oxygenated blood into the systemic circulation. Additional impairment of oxygen delivery (eg, in pulmonary hypertension) leads to hypoxic cerebral damage. Preoperative extracorporeal membrane oxygenation enables oxygenation in otherwise untreatable cases. METHODS: In 3 neonates suffering from cyanotic congenital heart disease (1 with tricuspid atresia and 2 with transposition of the great arteries) with arterial desaturation despite application of prostaglandins, balloon atrioseptostomy, and eventually inhaled nitric oxide during intermittent positive-pressure ventilation with an inspired oxygen fraction of 1, oxygenation could only be established by means of preoperative extracorporeal membrane oxygenation. We used a venovenous single-lumen cannula tidal-flow extracorporeal membrane oxygenation system described by Chevalier and associates that has previously been used for extracorporeal lung support. In this system, called AREC (assistence respiratoire extra-corporelle), alternating clamps and a nonocclusive roller pump were used. RESULTS: All 3 survived. CONCLUSIONS: We conclude that the AREC system enables sufficient preoperative oxygenation in patients with cyanotic congenital heart disease and hypoxia in spite of all conventional therapeutic means. This provides a stable preoperative condition for elective palliation or correction.

Cyanosis↗

Myoadenylate deaminase deficiency, hypertrophic cardiomyopathy and gigantism syndrome.

We report a 20-year-old man with gigantism syndrome, hypertrophic cardiomyopathy, muscle weakness, exercise intolerance, and severe psychomotor retardation since childhood. Histochemical and biochemical analysis of skeletal muscle biopsy revealed myoadenylate deaminase deficiency; molecular genetic analysis confirmed the diagnosis of primary (inherited) myoadenylate deaminase deficiency. Plasma, urine, and muscle carnitine concentrations were reduced. L-Carnitine treatment led to gradual improvement in exercise tolerance and cognitive performance; plasma and tissue carnitine levels returned to normal, and echocardiographic evidence of left ventricular hypertrophy disappeared. The combination of inherited myoadenylate deaminase deficiency, gigantism syndrome and carnitine deficiency has not previously been described.

AMP Deaminase↗

Auditory evoked potentials in young patients with Down syndrome. Event-related potentials (P3) and histaminergic system.

Subjects with Down syndrome exhibit various types of cognitive impairment. Besides abnormalities in a number of neurotransmitter systems (e.g. cholinergic), histaminergic deficits have recently been identified. Brainstem auditory evoked potentials (BAEPs) and auditory event-related potentials (ERPs), were recorded from 10 children (aged 11-20 years) with Down syndrome and from 10 age- and sex-matched healthy control subjects. In Down subjects, BAEPs revealed shortened latencies for peaks III and V with shortened interpeak latencies I-III and I-V. ERPs showed a delay of components N1, P2, N2 and P3. In addition, subjects with Down syndrome failed to show P3 amplitude reduction during repeated stimulation. To evaluate the cognitive effects of histaminergic dysfunction, ERPs were recorded from 12 healthy adults (aged 20-28 years) before and after antihistaminergic intervention (pheniramine) compared to placebo. Whereas components N1, P2, N2 remained unchanged after H1-receptor antagonism, P3 latency increased and P3 amplitude showed no habituation in response to repeated stimulation. The results suggest that the characteristic neurofunctional abnormalities present in children with Down syndrome must be the consequence of a combination of structural and neurochemical aberrations. The second finding was that antihistaminergic treatment affects information processing tested by ERPs similar to that seen with anticholinergic treatment.

Adolescent↗