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The use of therapeutic apheresis in very low weight patients is generally thought to have limitations, because of possible severe adverse reactions, potential risk related to the extracorporeal procedure, due to the low weight of the young patients. A careful therapeutic approach using appropriate precautions, and also introducing modifications to the standard procedure, can minimise the risk without compromising the efficacy of the plasmapheresis. The aim of the study was to evaluate apheresis tolerance and acceptability in children [Artif. Organs. 21 (1997) 1126] and infants [J. Clin. Apheresis 5 (1989) 21] with inherited lipid metabolism disorder, familial hypercholesterolemia (FH), primary hyperlipoproteinemia (lipoprotein phenotype I), and acute leukemia, weighing on average 20.55 kg. One thousand one hundred twenty three aphereses were completed. Three types of apheresis were performed: leukapheresis, plasma exchange, dextran sulphate cellulose (DSC) low density lipoprotein (LDL)-apheresis. Three different types of continuous flow systems were used. Technical adaptation depending on patients blood volume, body mass index, hematocrit, type of system used, permitted us to perform complete aphereses, obtaining a high degree of tolerance and acceptability of the treatment. The use of plasmapheresis is regarded to be an extreme therapeutic measure in children. However, when the need for such treatment is undebatable, plasmapheresis must be done. A well-trained and experienced team can overcome the technical difficulties in order to complete the procedures without complications. The most frequently observed adverse effects are vascular relative access insufficiency (2.0%), and mild hypotension (2.0%).  相似文献   
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Extended reverse dorsal metacarpal artery (RDMA) flaps use the skin from the dorsum of the hand to cover defects of the long fingers up to the distal phalanx. The authors present a series of 12 patients who underwent closure of defects of the long fingers with these flaps. As relevant literature is scarce, special emphasis was put on donor site morbidity. Active and passive total range of motion (TRM) and pinch grip strength of the finger neighbouring the reconstructed one were evaluated and compared to the corresponding finger of the contralateral hand. The donor site was furthermore evaluated for cosmetic appearance and pain. There was no statistically significant difference for active and passive TRM. The difference for pinch grip reached statistical significance (p=0.04). Subjective evaluation of pain and cosmetic appearance by the patients revealed a mean pain value of 1.25 on a visual analogue scale (0=no pain, 10=maximal imaginable pain) and a mean estimation of cosmetic appearance of 8 (visual analogue scale, 0=worst cosmetic result, 10=best cosmetic result). In conclusion, compared to other flaps, the extended RDMA flap is a fast, secure and single-stage procedure for defect coverage on the long fingers with low donor site morbidity.  相似文献   
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Conclusions The peroneal nerves and their blood supply are at risk during high tibial osteotomies. Fixation of nerves by fibrous tissues, compression by tendinous arcades of the peroneus longus tendon, and narrow passages for nerves crossing dense fibrous septa are all factors which favor the development of peroneal nerve lesions. Intraoperative soft tissue retraction and pull by retractors may damage nerves and vessels. The muscle branch for the extensor hallucis longus muscle is particularly at risk during the fibular osteotomy since it runs directly on the bone.  相似文献   
27.
Zusammenfassung Die Typ I-Allergien gegen Latex sind in den vergangenen Jahren zu einem zunehmenden berufsdermatologischen Problem geworden, zumal mindestens 10% der Angestellten im Gesundheitswesen betroffen sind. In der Dermatologischen Klinik der Universit?t Erlangen-Nürnberg stieg die Anzahl der j?hrlich diagnostizierten Patienten mit Latexallergien von 1989 bis 1995 auf das 12fache, wobei der Anteil der schweren, generalisierten Formen der Erkrankung von 10,7% (1989/1990) auf 44% (1994/1995) zunahm. Unter den m?glichen Ausl?sern der Latexallergie (wasserl?sliche Proteine mit Molekulargewichten von 2 bis 200 kD) sind mindestens 5 Hauptproteine mit bereits bekannter Prim?rstruktur zu berücksichtigen. Zus?tzlich gibt es Hinweise für Markerproteine, die in bestimmten Risikogruppen geh?uft zur Ausl?sung spezifischer IgE-Antik?rper führen (z.B. 46 kD-Protein in medizinischen Berufen, 14,6 kD- und 27 kD-Proteine bei Kindern mit Spina bifida). Das Vorkommen von Kreuzreaktionen zwischen Latex und unterschiedlichen Früchten (besonders Avocado, Kiwi, Banane, E?kastanie) bei 60 bis 70% der Latexallergiker ist bei der allergologischen Abkl?rung und Beratung dieser Patienten zu beachten. Wesentliche Aspekte der Prophylaxe umfassen die konsequente Umstellung medizinischer Einrichtungen auf ungepuderte Latexhandschuhe mit niedrigem Proteingehalt. Eine Zusammenstellung von OP- und Untersuchungshandschuhen, welche Angaben über die von uns ermittelten Proteinkonzentrationen (modifizierte Lowry-Methode und Hochdruck-Flüssigkeits-Chromatographie, HPLC) enth?lt, soll ein Leitfaden bei der Auswahl allergologisch geeigneter Handschuhe sein. Eingegangen am 10. August 1996 Angenommen am 21. August 1996  相似文献   
28.
Evolution of white matter lesions in neurofibromatosis type 1: MR findings.   总被引:1,自引:0,他引:1  
To characterize further the evolution of white matter lesions in neurofibromatosis type 1, we reviewed 68 MR images in 43 patients (age, 1-31 years), including 25 follow-up studies (mean interval, 27 months). Lesion number, location, morphology, signal characteristics, and contrast enhancement were assessed. Lesion characteristics and changes thereof were correlated with the patients' ages. Thirty-four patients (79%) had white matter lesions. These lesions were hyperintense on T2-weighted images, were isointense on T1-weighted images, and showed no mass effect or contrast enhancement in 31 patients; in three patients, T1-prolongation was observed (one with significant mass effect). None of the lesions evolved into a glioma. The most common locations were the cerebellum (49%), brainstem (22%), and internal capsule (19%). Nineteen patients had white matter lesions and follow-up studies. Lesions decreased in size or number in seven patients (average age, 13 years), showed no change in three (average age, 12 years), increased in size or number in four (average age, 5 years), and showed a mixed pattern (increased/decreased size/number) in four (average age, 7 years). White matter lesions in neurofibromatosis type 1 frequently increase in size or number early in childhood; this did not indicate neoplasia in our study. The lesions tend to resolve with increasing age. Lesion progression in a child more than 10 years old warrants close follow-up to rule out a neoplasm.  相似文献   
29.
S S Pedersen  N Hiby  F Espersen    C Koch 《Thorax》1992,47(1):6-13
BACKGROUND: Chronic bronchopulmonary infection with mucoid, alginate producing Pseudomonas aeruginosa occurs characteristically in patients with cystic fibrosis. Alginate may be a virulence factor for P aeruginosa infection in such patients. METHODS: Forced vital capacity (FVC), nutritional state and the antibody response to P aeruginosa were determined at regular intervals from three years before chronic P aeruginosa infection to 10 years afterwards in 73 patients with cystic fibrosis. All patients were treated intensively with antipseudomonal chemotherapy during the study period. RESULTS: FVC was reduced in all patients who subsequently developed P aeruginosa infection before they acquired the infection, indicating significant pre-existing lung damage when compared with patients who remained free of P aeruginosa. Lung function and nutritional state remained unchanged after 10 years of infection, except in the patients who died of P aeruginosa lung infection. The FVC and height and weight of patients infected with nonmucoid strains of P aeruginosa were similar to those of uninfected patients. Patients infected with mucoid strains had poorer lung function and nutritional state for the first five years after infection compared with patients with nonmucoid strains. Such infection was also associated with greater IgG and IgA antibody responses to P aeruginosa standard antigen compared with nonmucoid infection. Concentrations of antibody to alginate were similar in patients with non-mucoid and mucoid infection. Noticeably increased concentrations of IgA antibodies to P aeruginosa standard antigen were observed early after the onset of infection in patients who subsequently died. CONCLUSION: Alginate producing P aeruginosa infection is associated with a hyperimmune response and poor clinical condition, suggesting that alginate production is a virulence factor in such infections in patients with cystic fibrosis.  相似文献   
30.
Background: diabetic patients with end-stage renal failure (ESRD) have a high cardiovascular morbidity and mortality. The underlying mechanisms are not completely elucidated. The aim of our study was to define predictors of death in diabetic patients with end-stage renal disease. Patients and methods: We preformed a prospective study in 35 dialysis centres in Germany between 1985 and 1994. To evaluate predictors and risk factors in this population we examined 412 diabetic patients at the time of admission to dialysis treatment (peritoneal dialysis (PD) or haemodialysis (HD)). Classification of the type of diabetes was done according the criteria of the National Diabetes Data Group [1,2]. Items assessed at the time of admission were coronary artery disease (CAD), peripheral occlusive disease (POD), and stroke. CAD was defined as a history of myocardial infarction with the corresponding changes in the ECG or luminal narrowing by more than 50% in at least one coronary artery upon coronarangiography; POD was defined as claudication and/or brachial-tibial ratio (BTR) less than 0.9 or a history of amputation. Assessment of the nutritional state comprised body mass index, skinfold thickness of the upper arm and lateral thorax area, and urea concentration. Cholesterol, HDL, LDL, apolipoprotein A (ApoA-I) and B (ApoB), triglycerides, lipoprotein (a) (Lp(a)), and fibrinogen were measured. As an index of disturbed cardiac innervation beat-to-beat variation was measured. Outcome measurements were causes of death (i.e. cardiac and non-cardiac) and time of survival. Results: One hundred and eighty of 412 (44%) patients died during the observation period Patients who died were older (61±12 versus 53±15 years P lt;0.0001), had lower skin fold thickness (13.1±6.0 versus 15.1±7.2 mm P <0.04), lower ApoA-I (100±35 versus 111±32 mg/dl P <0.005) and higher fibrinogen (515±156 versus 451±155 mg/dl P <0.02). Type II diabetic patients had a lower mean survival time than type I (34 versus 66 months P <0.0006). The mode of renal replacement therapy (PD or HD) had no adverse effect on survival time. Survivors less frequently had a history of CAD, POD and stroke than non-survivors. In multivariate analysis ApoA-I, fibrinogen ,age and stroke were independent predictors of cardiac and non-cardiac death in diabetic patients with end-stage renal failure. Lipid values and nutritional state did not independently predict the overall and cardiovascular mortality. Conclusion: This study in dialysed diabetic patients identified several predictors of death, some of which are susceptible to intervention.  相似文献   
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