Die Conjunctivitis lignosa, eine noch immer ungeklärte Erkrankung

Nover, A; Knieper, P

doi:10.1055/s-2008-1055233

Cited by 8 publications

(5 citation statements)

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“…The clinical and histological findings in our patients were consistent with the data on other patients with ligneous conjunctivitis available in the literature (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). We were unable to find detailed information on growth, general development and wound healing in such patients, however.…”

Section: Clinicul Jindingssupporting

confidence: 90%

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Human homozygous type I plasminogen deficiency and ligneous conjunctivitis

Mingers

Philapitsch

Zeitler

et al. 1999

APMIS

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Mingers A-M, Philapitsch A, Zeitler P, Schuster V, Schwarz HP & Kreth HW. Human homozygous type I plasminogen deficiency and ligneous conjunctivitis. APMIS 1999: 107:62-72.On the basis of a questionnaire sent to the ophthalmology departments of hospitals throughout Germany, 10 patients with ligneous conjunctivitis or pseudomembranous disease, ranging in age from 1 to 71 years were identified. All 10 patients had severely reduced plasminogen levels. Genetic analysis revealed homozygous type I plasminogen deficiency (which had not previously been described in humans) in 7 patients and compound heterozygous plasminogen deficiency in 1 patient. Clear differentiation was not possible in 2 patients. Most of the parents had heterozygous plasminogen deficiency. None of the patients had experienced any episodes of thrombosis. Additionally, the following observations were made: 1) Levels of polymorphonuclear (PMN)-elastase protein were markedly elevated in 6 of 6 patients and 10 of 1 1 parents tested, and levels were higher in homozygotes than in heterozygotes. 2) Hereditary factor XI1 deficiency was found in 3 of 6 patients tested. 3) CI-inhibitor was elevated in 2 of 4 patients, prekallikrein was elevated in 1 of 4 patients, and plasminogen activator inhibitor type 1 was elevated in 1 of 4 patients. Infusions of lys-plasminogen concentrate induced pronounced fibrinolytic activity as indicated by high levels of D-dimer, increases in plasmin-antiplasmin complex and decreases in polymorphonuclear elastase. C 1 -inhibitor, prekallikrein and PAI-1 normalized after repeated infusions of lys-plasminogen. In contrast to dysplasminogenemia, severe type I plasminogen deficiency might be seen as a problem of extravascular space, in particular of the mucous membranes, possibly triggered by mechanically induced or inflammatory lesions of the vessels supplying the tissue.We diagnosed homozygous type I plasminogen deficiency for the first time in 1994. This defect of the fibrinolytic system had not previously been seen in humans. The patient was an 18-month-old female infant who had ligneous conjunctivitis ( 1 4 ) as well as congenital hydrocephalus. Occlusions had repeatedly occurred at the tips of shunts inserted for drainage, but there was no other evidence of thrombus formation. The etiology of ligneous conjunctivitis was unclear at that time (5-17), and we hypothesized that it was related to the severe plasminogen deficiency. We therefore decided to study this condition in more detail.A questionnaire focusing on genetic aspects was

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Section: Clinicul Jindingssupporting

confidence: 90%

“…The symptoms in our patients are comparable to those seen in other patients with ligneous conjunctivitis as described in the literature (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). The most characteristic feature consists of the formation of ligneous pseudomembranes on the tarsal conjunctivae.…”

Section: Clinicul Picturvsupporting

confidence: 88%

Human homozygous type I plasminogen deficiency and ligneous conjunctivitis

Mingers

Philapitsch

Zeitler

et al. 1999

APMIS

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“…As summarized in Table 1, the clinical and histological findings in our patients were consistent with the data available in the literature on other patients with ligneous conjunctivitis. [5][6][7][8][9][10][11][12][13][14][15][16][17] We were unable to find detailed information on growth, general development, and wound healing in such patients, however.…”

Section: Patientsmentioning

confidence: 99%

Polymorphonuclear Elastase in Patients with Homozygous Type I Plasminogen Deficiency and Ligneous Conjunctivitis

Mingers¹,

Philapitsch²,

Schwarz³

et al. 1998

Semin Thromb Hemost

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Laboratory studies were performed on six female patients (ranging in age from 1 to 31 years) with ligneous conjunctivitis, which we regard as a systemic condition consisting of ligneous conjunctivitis and other pseudomembranous lesions. Plasminogen levels were severely reduced in all six patients; five patients were homozygous, and one patient was double heterozygous for type I plasminogen deficiency. Of family members tested, 11 of 12 parents and two of six siblings tested were diagnosed as heterozygous. No thrombotic episodes had occurred in any of the patients. Polymorphonuclear (PMN) elastase protein levels were markedly elevated in all, significantly more so in the homozygous patients (range 88 to 335 ng/mL; normal range, 20+/-10 ng/mL) than in the heterozygous patient (58 ng/mL). Of 11 parents examined, only 1 mother had normal PMN elastase (27 ng/mL, with plasminogen antigen 60% and plasminogen functional activity 86%), whereas values were moderately elevated (range 42 to 110 ng/mL) in the other 10 parents examined. After plasminogen substitution, PMN elastase levels consistently decreased but did not reach normal values. We interpret our findings as indicating that non-plasmin-induced fibrinolytic processes, possibly mediated via elastase, may be intensified in patients with plasminogen deficiency.

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“…[4][5][6][7][8]12 Ligneous conjunctivitis that usually manifests itself early in life, occurs sporadically and is found more often in girls than boys. [5][6][7][8][9][13][14][15][16] However, in some fami lies the disease was clearly shown to be inherited as an autosomal recessive trait. Some children with ligneous conjunctivitis also suffer from congenital occlusive hy drocephalus.…”

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confidence: 99%

Homozygous Type I Plasminogen Deficiency

Mingers¹,

Heimburger²,

Zeitler³

et al. 1997

Semin Thromb Hemost

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Homozygous type I plasminogen (Plg) deficiency has not been described in human subjects so far. Ligneous conjunctivitis is a rare and unusual form of chronic pseudomembranous conjunctivitis of unknown etiology. Here we report for the first time on homozygous type I Plg deficiency in three unrelated female patients who suffered from ligneous conjunctivitis and additional pseudomembranous lesions of other mucous membranes. The disease is caused by massive fibrin depositions within the "extravascular space" of mucous membranes because of absent clearance by plasmin. Infusions of albumin, fresh frozen plasma, or Lys-plasminogen (Lys-Plg) into two of the three patients revealed normal Plg activation capacity in these patients. The absence of fibrinolytic activity could therefore be shown to be due to Plg deficiency. Similar studies in the third patient have not been completed. In the two patients studied so far, infusions of Lys-Plg resulted in prompt and adequate Plg recovery with a short half-life and high amounts of plasmin-antiplasmin complexes and D-dimer. One patient additionally revealed an inherited partial factor XII deficiency. Functionally, this factor XII deficiency did not interfere with Plg activation. However, there may be a pathway of Plg activation in this patient via the prekallikrein C1-INH system.

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Die Conjunctivitis lignosa, eine noch immer ungeklärte Erkrankung

Cited by 8 publications

References 0 publications

Human homozygous type I plasminogen deficiency and ligneous conjunctivitis

Human homozygous type I plasminogen deficiency and ligneous conjunctivitis

Polymorphonuclear Elastase in Patients with Homozygous Type I Plasminogen Deficiency and Ligneous Conjunctivitis

Homozygous Type I Plasminogen Deficiency

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