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At least 973 records · Page 54Linked to original sources
PubMed1987

[Dominant autosomal humeroperoneal syndrome with early contractures and cardiomyopathy (Emery-Dreifuss syndrome)].

At least seven members of a family coming from southern Germany (Franconia) have manifested Emery-Dreifuss syndrome within four generations. This syndrome is characterized by slowly progressing atrophic pareses, generally in a humeroperoneal distribution, premature joint contracture, and cardiomyopathy with reduction of functional capacity. Up to now four members of this family have died from cardiac causes between the age of 39 and 46 years. Three family members have now been thoroughly examined; they showed typical muscular atrophies and contractures. The two older patients were diagnosed as having a cardiomyopathy with a complete atrioventricular block and ventricular tachycardia. Electromyographic and histological findings indicate a primary neurogenic process. Regular cardiological examinations combined with effective therapy (for example, implantation of a cardiac pacemaker and treatment with antiarrhythmic drugs) will be necessary in treating the serious arrhythmias. Progressive myocardial insufficiency required heart transplantation in one of our patients, which was successfully performed. Macroscopic and microscopic examination of the explanted heart showed a dilatative cardiomyopathy with hypertrophy and dilatation of both atria and ventricles, differences in the diameters of individual heart muscle cells, and a predominately focal interstitial fibrosis.

Adolescent↗
PubMed2006

Muscle changes in brachial plexus birth injury with elbow flexion contracture: an MRI study.

BACKGROUND: Muscle pathology of the arm and forearm in brachial plexus birth injury (BPBI) with elbow flexion contracture has not been evaluated with MRI. OBJECTIVE: To determine whether limited range of motion of the elbow in BPBI is correlated with specific patterns of muscular pathology. MATERIALS AND METHODS: For 15 BPBI patients, total active motion (TAM) of the elbow (extension-flexion) and the forearm (pronation-supination) were measured. MRI of the elbow joints and musculature allowed assessment of elbow congruency. Fatty infiltration and size reduction of the muscles were graded semiquantitatively. RESULTS: Mean TAM of the elbow was 113 degrees (50 degrees-140 degrees) and that of the forearm 91 degrees (10 degrees-165 degrees). The greater the size reduction of the brachioradialis muscle, the more diminished was elbow TAM. The more extensive the BPBI and muscle pathology of the pronator teres muscle, the more limited was the TAM of the forearm. Pathology of the supinator and brachialis muscles was evident in every patient. CONCLUSION: Extensive BPBI may result in marked limitation of TAM. Elbow flexion contracture seems to be caused mainly by brachialis muscle pathology. Prosupination of the forearm is better preserved when the pronator teres is not severely affected. MRI can reliably show the extent of muscle pathology in BPBI.

Adolescent↗
PubMed2004

Neonatal spinal muscular atrophy with multiple contractures, bone fractures, respiratory insufficiency and 5q13 deletion.

We present the case of a floppy neonate with marked and generalized weakness, respiratory insufficiency and fetal akinesia deformation sequence. The infant showed multiple joint contractures, two bone fractures and needed mechanical ventilation from birth to death at 16 days of age. Electrophysiological assessment showed electrically unexcitable motor and sensory nerves. Muscle biopsy showed diffuse atrophy of type I and type II fibers. Necropsy confirmed the diagnosis of infantile spinal muscular atrophy (SMA) with severe loss of motor neurons in anterior horns and motor nuclei of brainstem. There were also neuronal loss, gliosis, chromatolysis, ballooned cells, empty cell beds and neuronophagia figures in other brainstem and brain nuclei. Genetic analysis of the patient revealed homozygous deletions of survival motor neuron gene 1 (SMN1) and a single copy of SMN2 in region 5q13. This case confirms that the loss of spinal motor neurons underlies the muscular atrophy in severe cases of 5q SMA. This case also shows that the presence of multiple joint contractures, bone fractures and respiratory insufficiency in SMA in the neonatal period does not necessarily exclude the occurrence of classical deletions in the SMA 5q13 region. Rather, these atypical clinical findings show the extreme severity and prenatal onset of the disease in these SMA cases, which may be related with the occurrence of a single copy of SMN2 gene. More reports of clinically, pathologically and genetically well-documented cases are essential to define the different types of this disease.

Chromosomes, Human, Pair 5↗
PubMed2003

Posttraumatic contracture of elbow treated with intraarticular technique.

BACKGROUND: Posttraumatic contracture of the elbow (either flexion or extension) is sometimes very disabling. However, an absolutely convincing surgical technique has not yet been defined in the literature. We developed an intraarticular technique to concomitantly treat both intraarticular and extraarticular lesions with one posterior incision. METHODS: Twenty consecutive adult patients were treated. After the olecranon was osteotomized, all intraarticular pathologies and the anteroposterior capsule were corrected completely. The olecranon was then stabilized with the modified tension band wiring technique. Immediately postoperatively, continuous passive movement was performed, and range-of-motion exercise of the elbow was encouraged continuously. RESULTS: All 20 patients were followed up for a median of 3.8 (range 2.1-6.6) years. The satisfactory rate was 95% (19 of 20, p<0.001). The flexion contracture improved from an average of 42 to 13 deg (p<0.001), and the maximal flexion improved from an average of 89 to 131 deg (p<0.001). The arc of motion improved from an average of 47 to 118 deg (p<0.001). The sole unsatisfactory patient still had 20-110 deg of arc of motion. There were no evident complications noted. CONCLUSION: Compared with other techniques, we recommend this one due to its high satisfactory rate and low complication rate.

Adult↗
PubMed2005

[Therapy of bony and fibrous contractures and buccal defects after noma using the temporalis muscle flap].

METHODS: A prospective pilot study was conducted on 12 patients to establish whether, under the difficult conditions prevailing in a developing country, the temporalis muscle flap is suitable as a safe and single-stage procedure for the repair of buccal defects combined with fibrous or osseous contractures of the temporomandibular joint. RESULTS: The surgical procedures were performed from 1998 to 2000 in the Hôpital National du Niamey (Republic of Niger). Primary healing was achieved with all grafts. Local clinical and radiological follow-up (in Niger) was performed on seven patients after 12 and 24 months. Mouth opening of at least 1.5 cm was achieved in all cases, although there was virtually no use of physiotherapy to prevent scar contracture. DISCUSSION: By contrast with expensive and surgically more complex reconstructions, our use of this procedure shows that even where medical conditions are less than ideal, a satisfactory functional and cosmetic result can be achieved in the long-term.

Adolescent↗
PubMed2003

Emery-Dreifuss muscular dystrophy in the evaluation of decreased spinal mobility and joint contractures.

In this report we present three patients who had complaints primarily related to joints and flexibility. Two had no specific diagnosis and one was thought to have ankylosing spondylitis. Extensive evaluation revealed Emery-Dreifuss muscular dystrophy (EDMD) in all. EDMD is a muscular dystrophy where joint contractures and spinal limitation occur before any overt muscle weakness, and the syndrome may be combined with serious cardiac pathology. We wish to call the attention of professionals involved in rheumatology and physical medicine to the existence of this syndrome, which may only present with joint contractures and spinal limitation but which may end with fatal cardiac problems if not diagnosed in time.

Adolescent↗
PubMed1986

Sclerema neonatorum and joint contractures at birth as a potential complication of chronic in utero hypoxia.

Three term neonates presented with an unusual combination of skin changes (sclerema) and joint contractures at birth. In each case a prolonged period of fetal hyporeactivity was reported by the mother. Each child demonstrated a number of clinical findings consistent with chronic fetal hypoxia. Each child recovered with normal joint function. We postulate that sclerema neonatorum and joint contractures are another, though poorly recognized sequela of chronic in utero hypoxia and are preceded by a prolonged period of diminished fetal activity.

Contracture↗
PubMed1976

Scar contracture after neck dissection. Causes, prevention, and treatment.

Every effort should be made to employ neck incisions that allow for the accomplishment of surgical extirpative goals and at the same time provide maximal functional and aesthetic rehabilitation. Incisions parallel to the normal skin tension lines best fulfill these criteria. When vertical incisions are used, they should be anteriorly directed so as to cross the skin crease lines at an acute angle rather than a perpendicular angle. Such incisions perpendicular to the normal skin crease lines usually result in the greatest degree of scar contracture. Significant improvement in both major and minor scar contractures can be achieved with conventional Z-plasty technics to redistribute the skin tension. A wide resection of the subcutaneous cicatrix in such cases is imperative for a successful result.

Cicatrix↗
PubMed1976

Proximal and distal releasing incisions for the treatment of flexion contracture of the popliteal region.

Proximal and distal releasing incisions and skin grafting have been found to be useful as an alternative procedure to the standard transverse incision for dealing with flexion contractures of the popliteal region. In this region important structures underlying the deep fascia may readily be exposed when the deep fascia itself is involved in the scar process and is cut across during the release. Chronic ulcer formation in the centre of hypertrophic scar contracture is a fairly frequent accompanying feature. Proximal and distal release incisions help solve the problem by providing protection for the deep vessels, nerves and tendons, without jeopardising the take of a skin graft.

Adult↗
PubMed1976

Reversed dynamic slings. A new concept in the treatment of post-traumatic elbow flexion contractures.

Following the successful treatment of knee-flexion contractures in haemophiliacs using an external corrective system with reversed dynamic slings, these have been adapted to treat post-traumatic elbow contractures. A case is described in which 90 degrees of fixed flexion was corrected in 1 week without discomfort. Clearly there is no need to resort to an internally applied hinge-distractor apparatus or capsulectomy if a simple external sling system is successful without complications.

Adult↗
PubMed1987

Purification of contracture-inducing insect toxins from Buthinae scorpion venoms by immunoaffinity and high pressure liquid chromatography.

A method of rapid and selective detection and purification of contracture-inducing insect toxins from Buthinae scorpion venoms is described in this paper. It consists of two main steps: the first one is specific as it uses immunoaffinity chromatography with antibodies against Androctonus australis Hector IT; the second, reverse phase high pressure liquid chromatography, allows the final separation of the different toxins present in each of the three venoms studied. Two, three and four insect toxins have been purified, respectively, from the venoms of Androctonus australis Hector, Buthus occitanus mardochei and Leiurus quinquestriatus quinquestriatus. This work demonstrates that, in Buthinae venoms, contracture-inducing insect toxins antigenically related to AaH IT, the first one purified, constitute the most important and, in some cases, the only toxins present.

Amino Acids↗
PubMed1976

Open palm technique for Dupuytren's contracture.

A study of the pre-operative condition of seventy-five hands afflicted with Dupuytren's contracture is followed by an analysis of the results of limited fasciectomy approached by midlateral digital and transverse palmar excisions. The palmar wounds were left open and healing occurred with minimal complications. The correction of contractures compares favourably with that achieved using other techniques of access and wound closure.

Adult↗
PubMed1979

Transurethral resection combined with steroid injection in treatment of recurrent vesical neck contractures.

Vesical neck contractures occur following 5 to 10 per cent of transurethral prostatectomies and an even smaller number of enucleative prostatectomies. Recurrences following both conservative and surgical treatment are common and present management problems to the urologist. Seven patients with recurrent vesical neck contractures were managed with reresection and steroid injection. One hundred per cent of patients responded to this although 1 patient required two procedures. The use of hydrocortisone sodium succinate (Solu-Cortef) versus triamcinolone acetonide (Kenalog) preparations are discussed.

Contracture↗
PubMed1986

Fibrin/fibrinogen and fibrinolytic activity of the palmar fascia in Dupuytren's contracture.

Considering the proved interaction of fibrin with fibroblasts and the seemingly decisive role of structural and functional changes ("modulation") of these cells in the evolution of Dupuytren's contracture, research has been carried out in order to investigate the fibrinolytic capacity and the possible presence of fibrin/fibrinogen in the palmar fascia of subjects operated upon for Dupuytren's Disease. Fibrin/fibrinogen were detected by a direct immunofluorescence technique and fibrinolytic activity was assessed by a fibrin plate method. A remarkable decrease of fibrinolytic activity and the presence of fibrin/fibrinogen were observed in small nodules in the early stage of disease, whereas large nodules showed a high amount of plasminogen activator enzymes. Small nodules seem to form and increase by progressive adhesion of fibroblasts to the polymerizing fibrin, while high fibrinolytic activity of large nodules probably results from "modulation" of many fibroblasts into contractile myofibroblasts and could therefore be considered as a biochemical sign of the evolutionary phase of Dupuytren's contracture.

Adult↗
PubMed1991

Dupuytren's contracture: the long-term results after day surgery.

220 operations in 211 hands in 160 consecutive patients suffering from Dupuytren's contracture were performed from 1976 through 1984 at the Kuopio University Central Hospital in Finland. 179 operations were carried out as day cases. The mean follow-up was 3.2 years. Sub-total fasciectomy resulted in good immediate and long-term improvement in 86% of patients. The recurrence rate was 27%. Surgical treatment of Dupuytren's contracture was well-suited to day surgery. A properly planned follow-up programme is suggested because of the relatively high rate of recurrence, which usually appeared within two years after operation.

Adult↗
PubMed1988

The long-term results of closed palmar fasciotomy in the management of Dupuytren's contracture.

Twenty-three patients were reviewed an average of 5.3 years after a closed palmar fasciotomy for the correction of Dupuytren's contracture. Results suggest that this procedure is best suited to those whose contracture is mainly at the MP joint and that approximately 55% of such patients can expect the correction to be maintained for at least five years. In those patients with severe deformity affecting the PIP joint in addition to the MP joint, this may be a useful preliminary procedure, but is unlikely to be of any long term benefit.

Dupuytren Contracture↗