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Prolidase deficiency is a rare autosomal recessive disorder characterized by cutaneous ulcers, facial dysmorphism, recurrent infections, and intellectual disability. We report a unique case of a 6‐year‐old boy with prolidase deficiency and Crohn's disease who presented with lower extremity ulcers. Cutaneous ulcers due to prolidase deficiency are historically resistant to treatment, and we report success with the novel use of topical tacrolimus. 相似文献
3.
Exposure to high pressure produces neurologic changes in humans which manifest as tremor, EEG changes, and convulsions. Since previous studies have implicated the involvement of the serotoninergic system in these symptoms, it was of interest to study serotonin release at high pressure. Synaptosomes isolated from guinea pig striatum were used to follow serotonin efflux at 68 ATA. The major observation was a decrease in [3H]serotonin release from depolarized striatal synaptosomes at 68 ATA. In view of the role of serotonin as an inhibitory neurotransmitter in this area, the observed decrease in synaptic release leads us to conclude that decreased serotoninergic activity in striatal neurons probably is contributing to the hyperexcitability associated with HPNS. 相似文献
4.
Sera of successive littermates of mothers producing anti-allotype antibodies (Ab) were analysed for altered a locus or b locus allotype expression. We measured the allotype concentration in sera of 66 individuals (17 litters) of seven mothers producing anti-a1 Ab, and 63 individuals (15 litters) of seven mothers producing anti-b4 Ab, in an enzyme-linked immunosorbent assay (ELISA). We confirmed that the ability to induce allotype suppression in utero increases with the number of antigen boosts applied to the mother, even though the Ab titre in the maternal serum may be decreased. All individuals of a litter expressed the allotype in about equal concentration. This contrasts the results we obtained when newborn rabbits were injected with anti-allotype antiserum. Injection of the same amount of anti-allotype antiserum into nine offspring of two mothers caused allotype suppression in only five individuals, showing no effect in the others. No suppression was observed when IgG-depleted antiserum was injected into newborn rabbits. As expected, maternal antibodies to a paternal allotype do not affect the Mendelian distribution of the progeny phenotypes. 相似文献
5.
Michael Dietrich Christoph Meier Daniela Zeller Patrick Grueninger Roger Berbig Andreas Platz 《European journal of trauma and emergency surgery》2007,33(5):512-519
Abstract
Background: Primary shoulder hemiarthroplasty is an established treatment modality for complex fractures of the proximal humerus. Long-term
functional outcome is often disappointing. However, little is known about social implications particularly in the elderly.
Methods: A single-institution case series of consecutive geriatric patients (age > 70 years) treated with shoulder hemiarthroplasty
for complex fractures of the proximal humerus between 1994 and 1997 was analysed. Postoperative morbidity, long-term function,
radiological outcome and social implications were evaluated.
Results: Seventy-seven patients fulfilled the study criteria. Median age at the time of operation was 80 years (range 70–93 years).
Systemic and local postoperative complications were observed in 8% including 2 patients (3%) with revision surgery. Postoperative
mortality was 1%. Forty-eight patients (62%) were available for follow-up (median 49 months, range 25–80 months), 22 (29%)
died from causes unrelated to hemiarthroplasty before follow-up and 7 patients (9%) did not attend follow-up examination.
Median Constant-Murley score was 41 points (range 17–77 points). Long-term results concerning pain were satisfying. The Oxford
shoulder score ranged from 14 to 40 (median 30). Forty-one patients (85%) still lived in their original environment and managed
their daily life independently despite poor shoulder function. Four patients (8%) lived in a retirement home and 3 (6%) in
a nursery home. Eighty percent of our patients were still able to use public transportation, do the daily shopping and wash
their whole body by themselves.
Conclusion: Most patients managed their daily life independently despite poor shoulder function. 相似文献
6.
J. H. Meier Dr. W. L. C. Rutten H. B. K. Boom 《Medical & biological engineering & computing》1995,33(3):409-417
Experimentally, during electrical nerve stimulation, the influence is examined of two intrafascicular anodes on the force
recruitment with one intrafascicular cathode. It is found that the anodes suppress recruitment and that this effect is more
pronounced when the distance of the anodes to the cathode is decreased, or when the anodal currents are increased. The measured
recruitment curve patterns can be qualitatively explained by a nerve stimulation model that calculates theoretical recruitment
curves for intrafascicular multi-electrode configurations. Discrepancies between the experimental and the theoretical recruitment
curves are seen, but these can be understood by taking into account a non-uniform fibre distribution. 相似文献
7.
C. Specker S. Bläß C. Meier H.-J. Lakomek M. Schwochau M. Schneider 《Zeitschrift für Rheumatologie》1997,56(2):63-70
Zusammenfassung Die Diagnostik vieler rheumatischer Systemerkrankungen wird heute durch den Nachweis von Autoantik?rpern unterstützt und
erleichtert. Für die Serodiagnostik der Rheumatoiden Arthritis (RA) stehen nur die doch wenig spezifischen Rheumafaktoren
zur Verfügung. Mit dem Ziel, neue krankheitsspezifische Autoantik?rper nachzuweisen, erfolgte eine besondere Proteinaufarbeitung
aus Synovialisbiopsien und anderen Geweben. Western Blots der gewonnenen Proteine wurden eingesetzt, um Seren von RA-Patienten
und solchen mit anderen rheumatischen Erkrankungen zu untersuchen. Die signifikanteste Immunreaktion von RA-Patienten richtete
sich gegen ein 68k-Antigen, welches vermutlich ubiquit?r exprimiert wird, da es nicht nur in Synovialis, sondern in allen
weiteren untersuchten Humangeweben und HeLa-Zellen nachgewiesen werden konnte. Der isoelektrische Punkt liegt bei 5,1, das
Protein ist O-glykosyliert und im endoplasmatischen Retikulum und/oder Cytoplasma lokalisiert. Antik?rper gegen dieses 68k-Antigen
waren bei 110 von 167 RA-Patienten nachzuweisen, was einer Sensitivit?t von 66% entspricht. Ihr Vorkommen war unabh?ngig vom
Rheumafaktornachweis, da sie auch bei 7 von 12 seronegativen RA-Patienten zu finden waren, dagegen nur bei einem Patienten
aus einer Kontrollgruppe von 98 Patienten mit anderen rheumatologischen Krankheitsbildern, bei einem von 22 HIV-Patienten
und überhaupt nicht bei 55 Gesunden. Daraus resultiert eine RA-Spezifit?t für diesen Antik?rper von 99%. Wegen der auff?lligen
Krankheitsspezifit?t der anti-68k-Antik?rper liegt es nahe, nach korrespondierenden autoreaktiven T-Zellen zu suchen, um
die Rolle dieser neuen Autoreaktivit?t in dem Pathomechanismus der RA zu analysieren.
Eingegangen: 20. Mai 1996 Akzeptiert: 13. Februar 1997 相似文献
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