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Biomedical subjects

G Fabry

Publications and source records attributed to G Fabry.

At least 55 records · Page 3Linked to original sources

Treatment of nonunion of the humerus using the Ilizarov external fixator.

Ilizarov's method of monofocal compression was used in 30 humeri with a diaphyseal pseudarthrosis. Twenty-one patients had previous surgery but had loosening of the osteosynthesis material. Nine patients initially were treated with a hanging cast, resulting in interfragmentary distraction. Fourteen nonunions were hypertrophic, and 16 were atrophic, of which six were infected. A complete circular frame was used only in the first nine patients, whereas the remaining 21 patients were treated with the modified semicircular fixator. Union was obtained in all but two patients, with an average consolidation time of 4.5 months (range, 2.5-10 months). No patient required additional bone grafting. Apart from superficial pin tract infection seen in most of the patients, three had a minor temporary sensory neurologic problem. Four patients experienced a second fracture after removal of the fixator that required a second application of an Ilizarov frame. Although similar results with regard to union are reported after plate osteosynthesis, there was no radial nerve palsy or deep infection in this series, indicating that the treatment by the Ilizarov technique is associated with less complications. The authors' findings suggest that the Ilizarov method is a reliable treatment for humeral nonunions, even after multiple previous operations or in the event of infection.

Adolescent↗

Treatment of hip subluxation in Dyggve-Melchior-Clausen syndrome.

We report a case of Dyggve-Melchior-Clausen syndrome, a skeletal dysplasia with short trunk dwarfism and mental retardation. A Chiari pelvic osteotomy was performed to halt hip subluxation on both sides. The patient was evaluated after 9 years and 10 months. The progressive lateral migration of the femoral head seemed to have been unaffected by this type of osteotomy.

Abnormalities, Multiple↗

Characteristics of patients with symbrachydactyly.

The clinical and radiologic spectrum of symbrachydactyly has been extended, on the basis of experimental embryology, into four categories: typical symbrachydactyly, atypical cleft hand, monodactyly, and adactyly. The origin of this embryologic failure is probably a vascular disruption. A series of 64 hands and 3 feet are reported, indicating associated anomalies and possible etiologic features.

Child↗

Distraction bone healing versus osteotomy healing: a comparative biochemical analysis.

This study investigates the biochemical changes in a canine tibia lengthening model in comparison with a nonlengthened osteotomy model. The lengthened and the osteotomized callus and a contralateral corresponding segment were analyzed for their mineralization profile, collagen content, osteocalcin, insulin-like growth factor I (IGF-I), and transforming growth factor beta1 (TGF-beta1). Examinations of bone samples were performed using specimens excised at different time intervals (respectively at 3, 5, 7, 9, and 13 weeks postoperatively). Several serum parameters (alkaline phosphatase [ALP], osteocalcin, IGF-I, and TGF-beta1) were also measured during the experimental period. A progressive increase in mineral parameters was noticed in both the lengthened and the osteotomized areas. A higher level of hydroxyproline and TGF-beta1 was observed in the lengthened area compared with the osteotomized area. IGF-I showed a significant increase in both the lengthened and contralateral control area at the later stage of the experimental period in the lengthened group. In serum, a high level of TGF-beta1 and a progressively increasing osteocalcin concentration were observed in the lengthened dogs in comparison with the osteotomized dogs. Serum ALP was significantly increased in both models during the experimental period. Serum IGF-I was increased in the lengthened models during the distraction period and decreased in the osteotomized models at the early stage of the experimental period. These results suggest that the mechanical strain induced by the Ilizarov distraction procedure stimulates osteoblast proliferation and promotes biosynthesis of bone extracellular matrix in distracted callus. Our data furthermore show that this process is different compared with normal fracture healing.

Alkaline Phosphatase↗

Symbrachydactyly involving hands and feet.

Two patients are described with a symbrachydactyly of the hand and foot. This is a rare combination which cannot be explained by the subclavian artery supply disruption sequence.

Abnormalities, Multiple↗

Grip strength in tennis elbow : long-term results of operative treatment.

In a prospective study the grip strength was measured preoperatively in 17 patients with chronic tennis elbow and postoperatively after a minimum follow-up of 1 year. The grip strength measured with a flexed and extended elbow was significantly less on the involved side (28.4 and 17.2 kg) than on the normal side (36.4 and 36.5 kg) ; postoperatively it increased significantly (33.9 and 34.2 kg) and almost reached the level of the normal contralateral side. An increase of grip strength measured with an extended elbow was correlated significantly with a satisfactory clinical outcome.

Adult↗

Popliteal cysts in children: a retrospective study of 62 cases.

Popliteal cysts in children are usually asymptomatic and are usually found fortuitously by the parents. This study presents a series of 62 children with popliteal cysts. Among the 51 patients who received no treatment, the cysts had already disappeared at the time of the study in 43 patients; the remaining 8 patients are under 14 years of age and are without complaints. This study confirms the hypothesis that the primary popliteal cyst in children usually disappears before the age of 18. Surgery presents unnecessary risks for the patient ; moreover, the chance of recurrence after surgery is real.

Adolescent↗

Ganglia of the superior tibiofibular joint: report of three cases and review of the literature.

Ganglion cysts that compress the common peroneal nerve frequently arise from the proximal tibiofibular joint. We report three cases of such ganglia. In one case the ganglion cyst arises from the proximal tibiofibular joint, finds its course along the common peroneal nerve causing pain, but no neurological deficit. In the two other cases the cysts have the same origin but they are the cause of a progressive peroneal nerve palsy. Treatment in these three cases was successful resection of the cyst with non mutilating surgery for the nerve and ligation of the stalk connecting the ganglion to the proximal tibiofibular articulation. A review of the controversial pathogenesis of the intra- and extraneural ganglia is exposed, and the anatomy, the diagnosis, the surgical treatment and the prognosis is discussed in light of relevant literature.

Adult↗

Core decompression for avascular necrosis of the femoral head.

The results of core decompression as the sole treatment for Ficat stages 1, 2 and 3 avascular necrosis of 51 femoral heads in 39 patients were studied. The mean follow-up time was 24 months (6-47 months); 19 hips (38.8%) were good and 30 hips (61.2%) had failed (two hips were lost to follow-up). The good results as stated in the literature could not be obtained, but we believe core decompression can delay the need for total hip replacement in avascular necrosis of the femoral head.

Adolescent↗

Surgical treatment of trigger fingers.

The surgical treatment of 55 trigger fingers resulted in 87% patient satisfaction, without major complications. The mean incapacity was 1.4 weeks. Three recurrences were noted; four patients had a slight extension lag and three still experienced some local pain.

Adult↗

Biochemical and density assessment of the new bone in late remodeling after callus distraction.

Biochemical changes in a canine bone-lengthening model were characterized 5 months after surgery. The mineral content and the total amount of EDTA-extractable noncollagenous proteins, insulin-like growth factor-I, and osteocalcin were determined for the lengthened callus, and a gradient density fractionation analysis of bone powder particles was performed. The results were compared with two other areas of the lengthened tibia and one region of the contralateral tibia. The mineral and osteocalcin contents showed significant decreases, whereas the hydroxyproline concentration was significantly increased. Neither the insulin-like growth factor-I content nor the concentration of EDTA-extractable proteins was significantly different in any of the examined regions.

Animals↗

Simple trapezectomy for treatment of trapeziometacarpal osteoarthritis of the thumb.

This is a review of 20 patients (22 operations) with symptomatic osteoarthritis of the first carpometacarpal joint who were treated by simple trapezectomy without interposition material. They were all women with a mean age of 60 years (46-77). The average follow-up was 2 years, ranging from 8 months to 3 years. Pain relief is the main contributor to the good clinical results of this procedure. Sixteen patients professed to be very satisfied, two had a fair result and four cases claimed to be unsatisfied. Range of motion (opposition and counter opposition) and first web space were very well preserved. Mean values of grip strength showed reduction of grip power on the operated side though not statistically. However, we did find a statistically significant difference in pinch strength between operated and non-operated side. In general, simple trapezectomy is our preferred surgical therapy in the treatment of carpometacarpal osteoarthritis in the elderly population.

Aged↗

Graft selection in anterior cruciate ligament reconstruction--prospective analysis of patellar tendon autografts compared with allografts.

A prospective study of 73 arthroscopic anterior cruciate ligament reconstructions using either a patellar tendon autograft or an allograft was made to assess any difference in clinical outcome. Allocation was by availability of an allograft. There were 48 autografts and 25 allografts. Evaluation was by clinical examination and physical tests. At follow-up 2 years after operation, there were no statistically significant differences between the two groups in mobility or in physical tests, but KT-1000 evaluation showed a slightly greater anterior translation in the autografts at 6 months and one year, although at 2 years the allografts developed greater anterior laxity. Cybex testing showed greater quadriceps strength at 6 months and one year in the allografts, but at 2 years the strength was greater in the autografts. Re-rupture occurred in 3 allografts. ACL reconstruction with a patellar tendon allograft does not produce a significant functional deficit. Full quadriceps recovery takes 2 years. Allografts are not recommended as stability deteriorates with time.

Adolescent↗

Pedodynographic measurements after forefoot reconstruction in rheumatoid arthritis patients.

From January 1987 to December 1992, 38 patients (59 feet) with rheumatoid arthritis underwent reconstruction of the forefoot using Keller-Lelièvre arthroplasty of the first metatarsophalangeal joint and Hoffman resection of the lesser metatarsal heads. The average age of the patients was 61.3 years, with both feet involved in 21 patients and 17 with single foot involvement. The aim of our study was to evaluate the results both on a functional and an objective basis using dynamic and static pedodynographic measurements. Attention was given to dynamic pressure measurements under the metatarsal heads, the center of pressure distribution, gait analysis, and peak loads taken on different areas of the forefoot during normal walking. Correlations were made between these measurements and symptoms. After a mean follow-up time of 35 months, the clinical results were satisfactory in 54%, satisfactory with some reservations in 39%, satisfactory with major reservations in 3%, and unsatisfactory in 3% of patients.

Adult↗