Cases reported "Chronic Disease"

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1/211. Dermal vessels in acrodermatitis chronica atrophicans.

    An ultrastructural investigation of two patients suffering from acrodermatitis chronica atrophicans revealed in the small dermal vessels swelling of the endothelial cells, material of probably plasmatic origin accumulated in the subendothelial area, and sleeves of basement membrane-like material on a concentric perivascular layout.
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keywords = membrane
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2/211. Visualization of functional improvement by 123I-IMP lung SPET after thromboendarterectomy for chronic pulmonary embolism.

    We report on six patients with chronic pulmonary embolism who underwent 123I-IMP and 99Tcm-MAA lung SPET before and after thromboendarterectomy. 123I-IMP lung SPET can assess the viability of lung parenchyma, because it is a non-particulate agent that accumulates in the endothelial membranes of pulmonary capillaries. Chronic pulmonary thromboembolism accompanied by pulmonary hypertension has a poor prognosis that may be improved only by thromboendarterectomy. We compared 123I-IMP and 99Tcm-MAA lung SPET in terms of functional improvement after such surgery. After thromboendarterectomy, all six patients were functionally improved, according to the criteria of the new york heart association. The pre- and post-surgery percentage of vascular obstruction did not differ significantly with 99Tcm-MAA lung SPET (44.8 /- 11.2% and 32.5 /- 15.6% pre- and post-surgery, respectively). In contrast, 123I-IMP lung SPET revealed a significant pre- versus post-surgery difference (15.5 /- 9.5% and 3.3 /- 5.9% pre- and post-surgery, respectively). 123I-IMP lung SPET could be useful for evaluating thromboendarterectomy because pulmonary parenchymal viability owing to arterial microvasculature can be estimated.
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3/211. Management of chronic posttraumatic radial head dislocation in children.

    We describe a new procedure for the management of chronic posttraumatic radial head dislocation, which uses two drill holes in the proximal ulna. The holes are placed at the original attachments of the annular ligament and thereby allow repair of the annular ligament (frequently avulsed from one attachment and impinged within the joint) or reconstruction of the annular ligament with whatever tissue or material desired (triceps tendon is convenient). It secures the radial head in its normal position from any dislocated position. It also allows for osteotomy of any accompanying deformity of the ulna or radius. This operation developed gradually between 1967 and 1995 while we treated seven female patients. The average age at time of injury was 5 years 10 months (range, 3 years 4 months to 8 years 11 months). The interval between injury and operation averaged 30 months (range, 3 months to 7 years). The age at time of surgery averaged 8 years 4 months (range, 5 years 4 months to 13 years 5 months). The only criterion for surgery was a normal concave proximal radial articular surface. Follow-up averaged 48 months. At final follow-up, all patients were fully active and had no elbow pain or instability. Analysis of these cases suggests that the criteria for surgical repair should be based on two features: (a) normal concave radial head articular surface, and (b) normal shape and contour of the ulna and radius (deformity of either should be corrected by osteotomy). The age of the patient and duration of the dislocation are unimportant.
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ranking = 22.824136291357
keywords = ligament
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4/211. prenatal diagnosis in a family with severe type I plasminogen deficiency, ligneous conjunctivitis and congenital hydrocephalus.

    Severe type I plasminogen deficiency may cause severe ligneous conjunctivitis, a rare and unusual form of chronic pseudo-membranous conjunctivitis that usually starts in early infancy, but also pseudo-membranous lesions of other mucous membranes in the mouth, nasopharynx, trachea and female genital tract, and in rare cases congenital occlusive hydrocephalus. The index patient, the daughter of a consanguineous marriage, had suffered from severe ligneous conjunctivitis and had died from decompensated congenital hydrocephalus despite numerous shunt revisions. She was found to be homozygous for a non-sense mutation in exon 15 of the plasminogen gene (Trp597->Stop). In her next pregnancy, the mother asked for prenatal diagnosis of the plasminogen deficiency. Chorionic villus biopsy was performed at 12 weeks of gestation. dna analysis of the plasminogen gene by PCR and single-strand conformation polymorphism (SSCP) revealed that the fetus exhibited an identical heterozygous band pattern as observed in the healthy mother. Therefore, the fetus was heterozygous for the Trp597->Stop mutation in plasminogen exon 15. In addition, the fetus was found to be male by cytogenetic analysis and by multiplex PCR analysis using two polymorphic X-chromosomal markers (DXS424, HPRT). These findings excluded the possibility of contamination by maternal dna. It was concluded that the fetus was not at risk for ligneous conjunctivitis and its associated complications. After the birth of a healthy boy, plasminogen functional activity was shown to be 38 per cent. dna analysis confirmed prenatal molecular genetic results.
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5/211. Spontaneous chronic spinal epidural hematoma of the lumbar spine.

    We report an exceptional description of a spontaneous chronic spinal epidural hematoma presenting as lumbar radiculitis. The computed tomographic, magnetic resonance imaging, and intraoperative findings are presented. We discuss anatomical and pathophysiological considerations that could lead to such a condition. We estimate that spontaneous spinal epidural hematomas located in the ventral space are in fact premembranous or posterior longitudinal ligament hematomas.
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ranking = 7.6080454304522
keywords = ligament
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6/211. association of rickettsia helvetica with chronic perimyocarditis in sudden cardiac death.

    BACKGROUND: rickettsia helvetica is the only non-imported rickettsia found in scandinavia. It was first detected in ixodes ricinus ticks, but has never been linked to human disease. We studied two young Swedish men who died of sudden cardiac failure during exercise, and who showed signs of perimyocarditis similar to those described in rickettsial disease. methods: Samples from the heart and other organs were analysed by PCR and dna sequencing. May-Grunwald-Giemsa, Grocott, and acridine-orange stains were used for histopathological examinations. Staining of R. helvetica grown on shell-vials in vero cells, and the early descriptions of R. rickettsii by H T Ricketts and S B Wohlbach served as controls. immunohistochemistry was done with proteus OX-19 rabbit antisera as the primary antibody. The structure of rickettsia-like organisms was investigated by transmission electron microscopy. Serological analyses were carried out by indirect immunofluorescence with R. helvetica as the antigen. FINDINGS: By use of a semi-nested PCR, with primers specific for the 16S rRNA and 17-kDa outer-membrane-protein genes, and sequence analysis of the amplified products, genetic material from R. helvetica was detected in the pericardium and in a lymph node from the pulmonary hilum in case 1, and in a coronary artery and the heart muscle in case 2. A serological response in case 1 revealed an endpoint titre for R. helvetica of 1/320 (1/256 with R. rickettsii as the antigen). Examination of PCR-positive tissue showed chronic interstitial inflammation and the presence of rickettsia-like organisms predominantly located in the endothelium. These organisms reacted with proteus OX-19 antisera, and their size and form were consistent with rickettsia. Electron microscopy confirmed that the appearance of the organisms was similar to that described for spotted-fever rickettsia. INTERPRETATION: R. helvetica, transmitted by I. ricinus ticks, may be an important pathogen in the aetiology of perimyocarditis, which can result in sudden unexpected cardiac death in young people.
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7/211. A technical solution for secondary arthritis due to chronic proximal tibiofibular joint instability.

    Chronic instability of the proximal tibiofibular joint is an uncommon diagnosis and not frequently reported in the literature. The management options of this joint instability, complicated with secondary arthritis, have rarely been discussed and consist mainly of fibular head resection or arthrodesis of this joint. We describe a new technical procedure for addressing both the instability and the joint secondary arthritis. Stability of the joint is achieved by ligament reconstruction using a biceps femoris split passed through the tibial metaphysis and fixated back to the fibular head using bone anchors. The arthritic changes are addressed by interposition of a vascularized fascia lata strip. The described procedure offers a firm stabilization with no need for postoperative restrictions and an alternative to the inadvisable joint arthrodesis or resection.
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ranking = 7.6080454304522
keywords = ligament
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8/211. Peroneus brevis tendon rupture with tophaceous gout infiltration.

    The authors present a patient with a history of chronic lateral ankle instability and pain. Physical and diagnostic evaluation revealed anterior ankle instability and peroneus brevis weakness. An MRI showed an attenuated anterior-talofibular ligament and a longitudinal tear of the peroneus brevis tendon. Surgical exploration exhibited tophaceous gout within the tear of the peroneus brevis as well as within the attenuated anterior-talofibular ligament. Presented is an unusual case of a longitudinal tear of the peroneus brevis tendon with tophaceous gout infiltration.
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ranking = 15.216090860904
keywords = ligament
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9/211. Is there a pathogenic link between gamma heavy chain disease and chronic arthritis?

    In 1991, gamma heavy chain disease was diagnosed in a 43-year-old female, who 3 years earlier had contracted an erosive seronegative chronic arthropathy. In 1996, her lymphoproliferative disorder required treatment with melphalan and prednisolone. Laboratory studies revealed a gamma3 heavy chain monoclonal component in serum and urine. Massive localization of plasma cells and blasts with cytoplasmic or cell membrane staining for gamma3 chains, but no staining for light chains, was observed by immunohistochemical studies of bone marrow as well as affected synovial tissue. Large amounts of extracellular gamma3-chains were also deposited in the synovial membrane. This is the first documentation of gamma heavy chain deposition disease directly affecting articular structures. Whether it represents the primary pathogenic event followed by reactive inflammatory changes in the joints, or another example of gamma heavy chain disease preceded by chronic arthritis, remains elusive. Regardless, several common cellular and molecular mechanisms discussed here suggest a pathogenic link between the two disease processes.
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10/211. Ligneous conjunctivitis: a clinicopathologic study of 3 cases.

    The clinical, histopathologic features, and treatment outcomes in 3 patients with ligneous conjunctivitis are described. Bilateral, idiopathic membranes occurred in the palpebral conjunctiva in 2 patients. In 1 patient, unilateral conjunctival changes occurred in the bulbar conjunctiva, at the site of pterygium excision. Treatment included topical hyaluronidase, chymotrypsin, heparin, and cyclosporine and surgical excision with limited or no success. In one patient, conjunctival autografting from the normal fellow eye resulted in pseudomembrane formation at the donor site in the previously unaffected eye. Histopathological evaluation of excised membranes revealed the presence of amorphous eosinophilic hyaline material and chronic inflammatory cells. Immunohistochemical study revealed a predominance of t-lymphocytes. This case series confirms the recalcitrant clinical course of ligneous conjunctivitis. Conventional treatment modalities described in literature were not useful in the management of this condition. Surgical manipulation of the unaffected fellow eye in patients with unilateral disease can result in pathologic conjunctival changes, and is best avoided.
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ranking = 3
keywords = membrane
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