Familial hemiplegic migraine and autosomal dominant arteriopathy with leukoencephalopathy (CADASIL)

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a recently described familial cerebrovascular disorder shown to map to chromosome 19q12. Familial hemiplegic migraine has also been shown in some families to map close to the CADASIL locus. The fully developed CADASIL phenotype consists of recurrent strokes developing in the fourth decade, progressing to a pseudobulbar palsy, spastic quadriparesis, and subcortical dementia. In an Irish family 15 members were fully investigated by magnetic resonance scanning; 10 had typical magnetic resonance features of CADASIL. Five members of this family had familial hemiplegic migraine and 4 of these had magnetic resonance evidence of CADASIL. Two other members had migraine with and without aura as a presenting clinical symptom of CADASIL. This disorder has been shown by linkage analysis to map to the CADASIL locus at chromosome 19. The phenotype at presentation of CADASIL in this family was variable and age related and included familial hemiplegic migraine, migraine with and without aura, transient ischemic attacks, strokes, and spinal cord infarction. This family study increases our understanding of the spectrum of clinical manifestations of this underrecognized familial cerebrovascular disorder.     

Migratory, invasive and metastatic capacity of NCAM transfected rat glioma cells

A cDNA encoding a transmembrane 140 kDa isoform of the neural cell adhesion molecule, NCAM, was transfected into the rat glioma cell line BT4Cn. Transfectants with a homogeneously high expression of NCAM-B showed a decreased capacity for penetration of an artificial basement membrane when compared to cells transfected with expression-vector alone or untransfected cells. However, when injected subcutaneously into nude mice, both NCAM expressing cells and control cells produced invasive tumors. Nude mice injected with NCAM positive cells developed tumors with slower growth rates as compared to those induced by NCAM negative cells. This implies that NCAM may not only be involved in adhesive and motile behaviour of glioma cells, but also in their growth regulation.     

Effect of positioning on oxygenation in single-lung transplant recipients.

BACKGROUND: Many benefits and adverse effects of positioning are related to changes in ventilation and perfusion. A number of unique factors related to the allograft make the effects of positioning difficult to determine in single-lung transplant recipients. OBJECTIVES: To determine the effect of 3 body positions (supine, lateral with allograft lung down, and lateral with native lung down) on oxygenation and blood flow in single-lung transplant recipients in the 24 hours immediately after surgery. METHODS: A quasi-experimental repeated-measures design with stratified assignment to 1 of 3 different sequencing patterns for turning group was used to study 15 transplant recipients, 9 with emphysema and 6 with fibrosis. Oxygenation, ventilation, and blood flow measures (heart rate, blood pressure) were assessed after each turn. The effect of ischemic reperfusion injury was also explored. RESULTS: The oxygenation, ventilation, and bloodflow variables did not differ significantly across group, diagnosis, or time. Oxygenation variables measured when the allograft lung was dependent did not differ significantly from such measurements obtained when the native lung was dependent. CONCLUSIONS: No single position maximizes oxygenation in the immediate postoperative period in single-lung transplant recipients. Although a single standard protocol for positioning cannot be supported, the study does support the idea that transplant recipients can be safely turned in the immediate postoperative period without compromising oxygenation or hemodynamic status.     

Phage-displayed recombinant single-chain antibody fragments with high affinity for cholesteryl ester transfer protein (CETP): cDNA cloning, characterization and CETP quantification.

Cholesteryl ester transfer protein (CETP) greatly affects the metabolism of all lipoprotein classes including low-density lipoprotein (LDL) and high-density lipoprotein (HDL), both known to constitute powerful risk factors for coronary artery disease (CAD). We now report the successful first cloning and characterization of single-chain antibody fragments specific for CETP. A recombinant phage display library was generated using spleen mRNA isolated from BALB/c mice that had been immunized with highly purified CETP. Screening of the library yielded two single-chain antibody fragments with high affinity for CETP, termed 1CL8 and 1CL10, displaying respective KD values of 4.36 x 10(-9) M and 4.64 x 10(-9) M as determined by affinity sensor technology. Amino acid sequence comparison indicated the complementarity-determining regions of the respective heavy chains to be responsible for CETP high affinity binding. Fragment 1CL8 was successfully employed in clinical chemical quantification systems that uncovered an association in humans between plasma CETP concentration and total body fat mass (r=0.50, p<0.002). Because of the demonstrated superb CETP capturing capacity, combined with high binding affinity to CETP, ready access and unlimited supply, 1CL8 and 1CL10 are expected to prove powerful tools for studies on the role of CETP in atherogenesis.     

Tension pneumomediastinum after severe vomiting in a 21-year-old female.

A 21-year-old female with chronic membranoproliferative nephritis was admitted for suspected esophageal disruption and asthma after severe, prolonged vomiting. At the time of admission she presented with dyspnea, tachypnea, arterial hypotension and tachycardia. Physical examination showed discrete signs of ectopic air at the neck and distended cervical veins. CT-scan of the chest showed severe mediastinal emphysema with compression of the right atrium. After cervical mediastinotomy the cardiorespiratory parameters normalized immediately. Esophagoscopy showed multiple longitudinal mucosal tears between 25 and 45 cm; fluoroscopically, there was no leakage of contrast medium. Following conservative treatment the patient recovered completely and was discharged on day 8.     

Hyperphosphataemia and related mortality.

Sir, With great interest we read the editorial review of Jean etal. [1] on the relationship between hyperphosphataemia and mortalityin end-stage renal disease patients. The authors summarize resultsfrom the large USRDS and DOPPS studies in which associationsof hyperphosphataemia and increased mortality risks were     

Messung der psychischen Belastung nach akutem Myokardinfarkt mit Hilfe der SCL-90-R

Zusammenfassung. Ziel: Die psychische Belastung bei Patienten mit akutem Herzinfarkt innerhalb der ersten 48 h auf der Intensivstation des Städtischen Krankenhauses Kiel wird mit Hilfe der Symptom-Checkliste (SCL-90-R) gemessen. Patienten und Methodik: 134 hauptsächlich wegen eines akuten Herzinfarkts aufgenommene Patienten wurden zwischen August 1996 und Juli 1999 randomisiert ausgewählt. Sie füllten einen der Intensivstation angepassten Fragebogen aus, der u. a. neben Fragen zu sozioökonomischen Daten die standardisierten SCL-90-R enthält. Ergebnisse: Erhöhte Mittelwerte zeigten sich vor allem für die Skalen Somatisierung, Ängstlichkeit, Depressivität und GSI (Global Severity Index) bei solchen Patienten, die wenig soziale Unterstützung bekamen und über ein niedriges Einkommen verfügten. Schlussfolgerung: Da für Patienten mit schlechtem sozialen Netzwerk, niedrigem sozialen Status, erhöhter Angst und Depressivität eine schlechtere Prognose hinsichtlich der Mortalität und Wiedereingliederung in den Beruf beschrieben wurde, ist es im prospektiven Verlauf dieser Studie notwendig, gerade diese Patienten hinsichtlich der Entwicklung ihrer Angst zu erfassen und zu behandeln. Abstract. Objective: 134 patients with acute myocardial infarction, who were treated in an intensive care unit (ICU) of a German hospital in Kiel between 1996 and 1999 were selected for the study to examine their psychologic symptoms. Patients and Methods: The patients were asked to fill in the self-report inventory, the Symptom Checklist-90-Revised (SCL-90-R) within the first 48 h after their arrival in the ICU. Results: Patients with a low social status were found to have elevated mean values of the following scales: somatization (0.93), depression (0.66), and anxiety (0.59). The Global Severity Index (GSI) as a global measure of psychologic distress was elevated as well (0.46). Conclusion: Since patients with a limited social network and low social status, increased anxiety and depressive scores have a poor prognosis as to their mortality and their professional reintegration, it it considered necessary for the prospective setting of this study to register particularly those patients in order to treat their anxiety.     

Significance of the glenoid labrum for stability of the glenohumeral joint. An experimental study

In human cadavers we were able to show that the glenohumeral joint is comparable to the model of a physical piston. The labrum glenoidale functions like a valve against atmospheric pressure. It is possible to characterize the behavior of intraarticular negative pressure by the equation (formula; see text). The calculations of the force F of atmospheric pressure tending to resist distraction of the joint surfaces leads to a 95% confidence interval from 6.9 to 22.9 kp. Under a general anesthetic, distraction of the healthy glenohumeral joint also produces negative intraarticular pressure in the area of the fossa glenoidalis in vivo. Joints with a labral tear (Bankart defect) and a chronic instability are not characterized by this phenomenon. A change in intraarticular pressure might stimulate intraarticular pressure receptors. This could be important in functioning as a neuromuscular protection reflex for the joint.