Biosynthesis of collagen crosslinks in rabbit articular cartilage in vivo.
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Biomedical subjects
Publications and source records attributed to F Shapiro.
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Gross and histological abnormalities were demonstrated in a club-foot talus from a boy with multiple congenital anomalies who died when he was nine days old. Both tali were studied, the one from the club foot and the one from the normal foot. The gross anomalies involved the smaller size of the club-foot talus and the increased medial deviation of a stunted, misshapen head and neck region. Serial histological sections of both tali allowed for a three-dimensional geometric appreciation of both bones and an assessment of the nature and extent of histological and cytological features. The ossification center of the club-foot talus was absolutely and relatively smaller than that of the normal talus. It was eccentrically positioned, being more lateral and anterior than that of the normal talus. The marked histological abnormalities seen in the head and neck region of the club-foot talus involved extensive breaching of the endochondral sequence by vessels. The posterior aspect of the endochondral sequence and ossification center was normal. The extra-osseous and intra-osseous blood supply of the two tali was normal with the exception of the increased and irregular breaching of the endochondral sequence in the club-foot talus. This study demonstrates histological abnormalities in the head and neck region of the club-foot talus, which was most abnormal grossly. The eccentric position of the secondary ossification center as well as its related vascular abnormalities do not support a theory of developmental arrest of the talus but appear sufficiently abnormal to support the theory of a primary defect in the cartilage anlage. Clinically, one must bear in mind that early open reduction of the talocalcaneal navicular joint in a foot such as this would have served to reposition the navicular onto a talus that still was structurally abnormal.
Early experience with the treatment of patients with insulin-dependent diabetes and renal failure by chronic hemodialysis indicated a high mortality and increased incidence of medical complications. Since 1972, a marked improvement in survival and reduction in incidence of complications has been attributed to more rigorous control of fluid overload, hypertension, and blood sugar levels by insulin therapy and careful dietary management. A diet has been developed which combines the diet used by dialysis patients with suitable modifications for the insulin-dependent patient with diabetes. The importance of patient education is stressed in an attempt to improve patient compliance.
The present paper describes some of the technical inadequacies in the clinical practice of dialysis. Problem areas of hemodialysis, i.e., those related to blood access, dialyzer and delivery system, are treated in depth. Corollaries with peritoneal dialysis are obvious. Research priorities, in particular development of a more sophisticated delivery system, are suggested. The intent of this discussion is to outline a rational progression in improvement of care of the end-stage renal disease patient.
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The perichondrial ossification groove of Ranvier, a circumferential groove in the periphery of the epiphyseal cartilage, was studied in rabbits whose ages ranged from one week to eight months using light and electron microscopy, autoradiography after labeling with 3H-thymidine, 3H-proline, and 3H-glucosamine, and histochemical staining for proteoglycans and alkaline phosphatase. By these methods, three groups of cells were identified within the groove: 1. A group of densely packed cells deep in the groove, which are the progenitor cells for the osteoblasts that form the bone bark, a cuff of bone surrounding the epiphyseal growth-plate region and the adjacent part of the metaphysis. 2. A group of more widely dispersed, relatively undifferentiated mesenchymal cells and fibroblasts, some of which are chondroblast precursors that probably contribute to appositional chondrogenesis and growth in width of the epiphyseal cartilage. 3. Fibroblasts and fibrocytes among sheets of highly oriented and organized collagen fibers which form a fibrous layer that is continuous with the outer fibrous layer of the periosteum and with the perichondrium. This layer also sends fibers into the epiphyseal cartilage and anchors the periosteum firmly to the epiphyses as bone growth proceeds.
Carbenicillin appears to produce clinically important inactivation of aminoglycoside antibiotics in patients with severe renal failure. If combination carbenicillin/aminoglycoside therapy is used in patients with severe renal failure, the dose of carbenicillin must be adjusted to renal function and serum levels of both drugs should be monitored. Even with therapeutic serum carbenicillin levels (100-200 mug/ml), large doses of aminoglycosides may be required to achieve therapeutic serum levels and this may increase the risk of toxicity.
Many of the fair and poor results are directly traceable to errors in technique. Patient selection is also important. The operation can be done in adolescents since there is no growth plate at the distal end of the first metatarsal. We do not currently recommend this procedure for those over 60, for those with first metatarsophalangeal osteoarthritis or hallux rigidus or for those with moderate or severe rheumatoid arthritis. For these patients we usually do a Keller excisional arthroplasty. Our use of the osteotomy-bunionectomy operation for the patient with hallux valgus with an associated metatarsalgia or short first metatarsal has now become more cautious. We feel that the operation is not indicated for those with significant preoperative metatarsalgia, especially if the first metatarsal is shorter than the second, or for those whose first metatarsal is more than 4 or 5 millimeters shorter than the second, regardless of preoperative metatarsalgia. In these patients a McBride procedure or a proximal opening-wedge osteotomy is done.
Those planning provision of medical services should consider the type of music, the anticipated size of the crowd, the locale, the available local resources, the length of the festival, the numbers and types of patients to be treated, and especially the potential criminal and civil liability.
1) On log-log-coordiantes a direct correlation has bee shown to exist between plasma cyclic AMP and plasma Cr levels. 2) hemodialysis results in a significant reduction in the arterial plasma cyclic AMP levels, but a return of plasma cyclic AMP to pre-dialysis levels is seen within 30 mins post-dialysis. 3) the dialyzer clearance of cyclic AMP, both in vitro and vivo, is commensurate with its M.W. 4) In vitro platelet aggregation responses, to ADP, EPI, and COLL are not influenced by PRP cell counts between 150,000 and 300,000/mm3. 5) the BT of NC and CHDP are not significantly different, indicating that the in vivo hemostatic properties of the CHDP are otherwise intact. 6)the CHDP have PVPC significantly lower than the NC, a finding commensurate with the usual mold thrombocytopenia of renal failure. 7)the aggreation responses of the CHDP to COLL and to both Lo and Hi concentrations of ADP and EPI are significantly less than those of the NC. 8)A statistically significant inverse correlation between aggregation response and plasma cyclic AMP is observed.
Allele-specific molecular diagnosis of Duchenne and Becker muscular dystrophies (DMD and BMD) has been largely dependent upon muscle biopsy for dystrophin protein assay. We performed lymphocyte DNA mutation analysis by polymerase chain reaction on 14 boys presenting with a clinical picture compatible with DMD or BMD. DNA analysis revealed that 12 of 14 boys had a deletion of the dystrophin gene, thus establishing the diagnosis of DMD/BMD. Furthermore, genotypes for 9 of 12 deletion patients permitted prediction of the specific allelic disorder (i.e., DMD or BMD). Subsequent dystrophin testing confirmed all of the DNA-based diagnoses. We propose that DNA mutation analysis be included in the initial evaluation of patients suspected of having DMD/BMD, thus potentially eliminating the need for muscle biopsy in the majority of patients.
Duchenne muscular dystrophy (DMD) is an X-linked recessive genetic disorder for which the biochemical defect is as yet unknown. Recently, two cloned segments of human X-chromosome DNA have been described which detect structural alterations within or near the genetic locus responsible for the disorder. Both of these cloned segments were described as tightly linked to the locus and were capable of detecting deletions in the DNA of boys affected with DMD. In an attempt to determine more precisely the occurrence of these deletions within a large population of DMD patients and the accuracy of one of the segments, DXS164 (pERT87), in determining the inheritance of the DMD X chromosome, the subclones 1, 8 and 15 were made available to many investigators throughout the world. Here we describe the combined results of more than 20 research laboratories with respect to the occurrence of deletions at the DXS164 locus in DNA samples isolated from patients with DMD and Becker muscular dystrophy (BMD). The results indicate that the DXS164 locus apparently recombines with DMD 5% of the time, but is probably located between independent sites of mutation which yield DMD. The breakpoints of some deletions are delineated within the DXS164 locus, and it is evident that the deletions at the DMD locus are frequent and extremely large.
Orthopedic deformities in Emery-Dreifuss muscular dystrophy are discussed based on a study of four patients and an extensive literature review. The condition is characterized by slowly progressive humeroperoneal muscle weakness; ankle equinus, elbow flexion, and neck extensor muscle contractures; paravertebral muscle tightness; and cardiac abnormalities involving bradycardia and atrioventricular conduction defects. Tendo Achilles lengthening is warranted, since patients remain ambulatory for several decades. Scoliosis occurred in three patients but stabilized in the absence of treatment. Recognition of the condition is important to allow for heart pacemaker insertion because the usually asymptomatic cardiac abnormalities are associated with a high incidence of sudden death in mid-adult life.