Pathophysiology of Tamm-Horsfall protein.

Tamm-Horsfall protein, a renal glycoprotein present in normal urine, is the primary constituent of urinary casts. Immunoelectron microscopy has shown that this protein is localized selectively along surface membranes of the thick ascending loop of Henle. In this surface membrane site, the unique aggregation and gel formation of Tamm-Horsfall protein in response to increasing concentrations of electrolytes within physiologic ranges may influence the permeability characteristics of this nephron segment. These aggregation characteristics also play a role in pathologic conditions and lead to the prolonged persistence of interstitial Tamm-Horsfall protein deposits in several tubulointerstitial diseases. Recent studies have demonstrated immunologic responses to this protein, including an immune complex tubulointerstitial nephritis in rats mediated by autoantibodies to Tamm-Horsfall protein.     

Zur Chemie des 6-Azabicyclo [3.2.1] octans Stickstoffhaltige Bicyclen III

6-Azabicyclo [3,2,1] octanes The synthesis of the secondary and tertiary 6-azabicyclo [3,2,1] octanes 4 and 6 via the corresponding lactam 3 and 5 is described. The stereochemistry is discussed and the course of the Hofmann-degradation of 6b clucidated.     

The high-riding superior aortic recess of the pericardium: MRI visualization in a child

We report a 4-year-old child with a high-riding superior aortic recess of the pericardium, initially misdiagnosed as a possible vascular malformation. The anatomy of the pericardial recesses is reviewed.     

Homozygous hemoglobin Abruzzo in a North American child

Hemoglobin Abruzzo is a stable hemoglobin variant with increased oxygen affinity, clinically causing compensatory erythrocytosis in affected patients. Heterozygosity of this variant with or without beta-thalassemia has been previously described in three Italian families, thereby suggesting a single origin of the mutation. The authors report the first case of homozygosity in a North American female infant, born to heterozygous parents as a product of consanguineous marriage.     

Two unstable beta chain variants associated with beta-thalassemia: Hb Miami [beta116(G18)his-->Pro], and Hb Hershey [beta70(E14)Ala-->Gly], and a second unstable Hb variant at 170: Hb Abington [beta70(E14)Ala-->Pro

We report on three previously undescribed unstable hemoglobin (Hb) variants: Hb Miami, Hb Hershey and Hb Abington. Hb Miami was associated with a beta(+)-thalassemia (thal) mutation [IVS-I-110 (G-->A)], whereas Hb Hershey was associated with a B0-thal mutation [IVS-I-1 (G-->A)]. Hb Hershey also has decreased oxygen affinity. These three Hb variants illustrate the range of clinical severity that can be seen with unstable Hb variants, particularly when combined with a thalassemic mutation.     

Micromorphological evaluation of posterior composite restorations - a 10-year report

The summarized scanning electron microscopic-supported micromorphologic data of a 10-year longitudinal clinical study of GIC/Hybrid composite posterior restorations is reported. The aim of this study was to compare the results to USPHS-compatible clinical criteria of the CPM index and to analyse the deterioration pattern and the success rate. Out of a total of 194 Class I and Class II fillings 46 restorations were at baseline, after 1, 3, 5, 7 and 10 years. The restorations were micromorphologically examined using gold-sputtered replicas and coded according to the M-criteria of the CPM index. The features for surface roughness, surface texture, marginal integrity, excess of material, marginal fracture, loss of material, marginal leakage and other filling imperfections were assessed. Eighteen fillings showed perfect margins, rapidly deteriorating after the first year of service. Despite poor micromorphologic marginal conditions all restorations retained optimal functional characteristics. Parallel longitudinal micromorphologic and clinical assessment of posterior GIC/Composite restorations revealed fine structure and deterioration patterns of the tooth-biomaterial interface. The layer-technique using GIC as dentine replacement and resin-based composite as enamel replacement results, according to previous biocompatibility testing, and clinical data, in optimal pulp protection and correct anatomic form over a 10-year period. The myth of the need for perfect marginal integrity requires further discussion.     

Using heart rate variability to stratify risk of obstetric patients undergoing spinal anesthesia

In this study, we evaluated whether point correlation dimension (PD2), a measure of heart rate variability, can predict hypotension accompanying spinal anesthesia for cesarean delivery. After the administration of spinal anesthesia with bupivacaine, hypotension was defined as systolic blood pressure 相似文献   

Medical and surgical aspects of pediatric renal transplantation using living donors

The outcomes of 19 consecutive living-donor renal transplants (LD-RTx) was compared with 41 cadaveric grafts (CD-RTx) performed at our institution using basiliximab, cyclosporine, and prednisone as standard immunosuppression. LD-RTx significantly shortened the waiting time on dialysis. However, patient survival (100% in both groups), 1-year graft survival (94.7% vs 90%), and rejection-free graft survival (76.9% vs 73.5%) was not significantly different. LD-RTx showed better glomerular filtration rates in the early phase after transplantation, a difference that faded with time. Graft function was similar after 1 and 2 years. LD grafts with double renal arteries were used successfully in four cases; heparin therapy was administered to avoid graft thrombosis. A significantly greater number of lymphoceles was observed with LD grafts (7/19 vs 1/41, P < .01). In conclusion with improved immunosuppression producing better results with CD grafts, the advantages of LD-RTx have vanished. LD grafts with double arteries may be used successfully and LD-RTx allows a shorter dialysis period. The high incidence of lymphoceles in our series awaits further evaluation.     

Pseudotumor cerebri following cyclosporine A treatment in a boy with tubulointerstitial nephritis associated with uveitis

An 11-year-old boy with recurrent nephritis due to tubulointerstitial nephritis associated with uveitis (TINU syndrome) was treated with cyclosporin A (CSA) to induce sustained remission. CSA was introduced as a steroid-sparing drug because of extreme obesity (body mass index 32 kg/m²). Although the boy did not complain of any clinical symptoms, eye inspection after 7 months revealed bilateral disk edema with retinal bleeding and the patient developed cerebrospinal hypertension. Pseudotumor cerebri was diagnosed by measuring the intracranial pressure (31 cmH₂O) and normal computer tomography and brain magnetic resonance imaging. Cessation of CSA therapy and treatment with mycophenolate mofetil led to resolution within 12 weeks.Supported by Forschungsunterstützungskreis Kindernephrologie e.V. Essen