1/381. Polymorphonuclear elastase in patients with homozygous type I plasminogen deficiency and ligneous conjunctivitis. 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. ( info) |
2/381. Conjunctival lymphoma masquerading as chronic conjunctivitis. OBJECTIVE: Malignant lesions of the conjunctiva may present with slowly evolving signs resembling inflammation. The authors describe the clinical and histopathologic findings of two patients with bilateral conjunctival lymphoma who presented with a history of chronic conjunctivitis without clinically noticeable subconjunctival nodules. DESIGN: Case report. PARTICIPANTS: Two patients. INTERVENTION: Both patients underwent conjunctival biopsy for evaluation of persistent conjunctival inflammation that did not respond to various medical treatment methods. RESULTS: Histopathologic examination revealed extranodal marginal zone B-cell lymphoma (mucosa-associated lymphoid tissue type) in one patient and follicle center lymphoma in the other patient. Both patients subsequently received radiation therapy and achieved a complete remission with no evidence of recurrence in the follow-up period of 20 and 16 months, respectively. CONCLUSIONS: Conjunctival lymphoma should be included in the differential diagnoses of chronic conjunctivitis. Persisting signs and symptoms of conjunctivitis not responding to standard treatment should prompt biopsy. ( info) |
3/381. Sebaceous carcinoma presenting as a unilateral papillary conjunctivitis. PURPOSE: To describe a previously unreported presentation of sebaceous carcinoma, an aggressive tumor that often presents insidiously with minimal symptoms and nonspecific signs. methods: We report a 71-year-old man who presented with unilateral ocular irritation and ipsilateral, idiopathic, papillary changes of the superior palpebral conjunctiva. The patient underwent incisional biopsy of the palpebral conjunctiva followed by full-thickness excision of the involved eyelid. RESULT: Histopathologic examination established the diagnosis of sebaceous carcinoma. CONCLUSION: Unexplained asymmetric, papillary changes of the palpebral conjunctiva should arouse suspicion of sebaceous carcinoma. ( info) |
4/381. Compound-heterozygous mutations in the plasminogen gene predispose to the development of ligneous conjunctivitis. Homozygous type I plasminogen deficiency has been identified as a cause of ligneous conjunctivitis. In this study, 5 additional patients with ligneous conjunctivitis are examined. Three unrelated patients (1 boy, 1 elderly woman, and 1 man) had plasminogen antigen levels of less than 0.4, less than 0.4, and 2.4 mg/dL, respectively, but had plasminogen functional residual activity of 17%, 18%, and 17%, respectively. These subjects were compound-heterozygotes for different missense mutations in the plasminogen gene: Lys19 --> Glu/Arg513 --> His, Lys19 --> Glu/Arg216 --> His, and Lys19 --> Glu/Leu128 --> Pro, respectively. The other 2 patients, a 14-year-old boy and his 19-year-old sister, who both presented with a severe course of the disease, exhibited plasminogen antigen and functional activity levels below the detection limit (<0.4 mg/dL and <5%, respectively). These subjects were compound-heterozygotes for a deletion mutation (del Lys212) and a splice site mutation in intron Q (Ex17 1del-g) in the plasminogen gene. These findings show that certain compound-heterozygous mutations in the plasminogen gene may be associated with ligneous conjunctivitis. Our findings also suggest that the severity of clinical symptoms of ligneous conjunctivitis and its associated complications may depend on the amount of plasminogen functional residual activity. ( info) |
| A case of newcastle disease virus infection in a female laboratory technician is reported for the first time in malaysia. infection was acquired by droplet infection of the eye while grinding infected chicken in the laboratory. The case was confirmed by isolation of newcastle disease virus from an eye swab taken from the subject on the first day of clinical signs. A four-fold rise of haemagglutination-inhibition titre was shown when sera on the third day of infection and 15 days later were compared. ( info) |
6/381. Radiculomyelitis following acute haemorrhagic conjunctivitis. The clinical manifestations and natural history of radiculomyelitis following a newly reported disease--acute haemorrhagic conjunctivitis (AHC)--have been studied in 33 patients in taiwan, and the following observations made: All the patients in this series were adults at ages ranging from 21 to 55 years; the salient initial neurological manifestations were radicular pains and acute flaccid paralysis which developed from five to thirty-seven days after the onset of AHC. In some patients, signs and symptoms indicating involvement of the meninges, cranial nerves and the white matter of the cord were observed; motor paralysis was the most striking feature during the whole clinical course; it consisted of flaccid asymmetrical weakness in one or more limbs, usually being more severe in the lower limbs than in the upper, and often more proximal than distal. Atrophy in the severely affected muscles usually became apparent in the second or third week of the weakness; the prognosis regarding the return of function in the affected muscles was dependent on the severity of the involvement. Permanent incapacitation due to paralysis and muscular atrophy in the affected proximal muscles of lower limbs was the main sequel in severe cases. The pattern and prognosis of flaccid motor paralysis were reminiscent of acute poliomyelitis in which the anterior horn cells of the spinal cord are mainly involved. Pleocytosis ranging from 11 to 270 per mm3 was noted in the majority of the patients when the cerebrospinal fluid was examined within the first three weeks from the onset of neurological symptoms; the total protein level was raised invariably from the second week onwards in all specimens, and remained so throughout the subsequent course as long as the seventh week or later. Tissue culture neutralization tests were performed on the sera from 9 patients; significant rises in the antibody titres (greater than or equal to 1:16) to AHC virus antigens were found in 8 cases, and in 2 of them a fourfold rise in the paired sera was noted. The differentiation of this syndrome from poliomyelitis and from guillain-barre syndrome, the relative freedom of children from neurological complications of AHC and the aetiological relationship of AHC virus to the syndrome have been discussed. It is concluded that this unusual neurological syndrome is caused by the neurovirulent properties of the AHC virus. ( info) |
7/381. Hypertrophic discoid lupus erythematosus of the conjunctiva. PURPOSE: To report the ophthalmic manifestations of hypertrophic discoid lupus erythematosus of the conjunctiva. METHOD: Case report and review of biopsy results. RESULTS: A 58-year-old woman with a history of chronic blepharoconjunctivitis presented with an unusual raised conjunctival lesion. Previous biopsy slides were reviewed and interpreted as diagnostic of discoid lupus erythematosus, hypertrophic or verrucous type. Both blepharoconjunctivitis and the raised conjunctival lesion resolved with hydroxychloroquine therapy. CONCLUSIONS: A raised conjunctival mass in the context of refractory blepharoconjunctivitis should elicit suspicion for discoid lupus erythematosus. The hypertrophic variant of this disease can affect the conjunctiva. ( info) |
8/381. Ocular surface neoplasia masquerading as chronic blepharoconjunctivitis. PURPOSE: To present the clinical characteristics and difficulties in the diagnosis of various ocular surface malignancies mimicking features of chronic blepharoconjunctivitis and to summarize the current therapeutic approach and prognosis of patients. methods: Six patients with slowly evolving signs of persistent inflammation underwent a conjunctival biopsy after a prolonged course of medical treatment. The medical records of the patients were reviewed. RESULTS: Histopathologic examination of the biopsy specimens revealed intraepithelial squamous neoplasia (one patient), invasive squamous cell carcinoma (one patient), sebaceous carcinoma (two patients), and conjunctival lymphoma (two patients). CONCLUSION: Although uncommon, ocular surface malignancies may involve the conjunctiva diffusely and present as chronic conjunctivitis. A high index of suspicion and an early histopathologic examination are essential to not delay diagnosis. ( info) |
| 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. ( info) |
10/381. Respiratory allergy to mushroom spores: not well recognized, but relevant. BACKGROUND: Although basidiospores are a major component of the air spora in many parts of the world, their clinical significance as triggers of respiratory allergy has rarely been demonstrated. Therefore, the class of basidiomycetes as an aeroallergen is not well known. OBJECTIVE: To demonstrate a cause and effect relationship between respiratory allergy and basidiospores, we illustrate this case report of a 38-year-old housewife. methods: skin prick test, immunoblot, and active anterior rhinomanometry were used as diagnostic tools to verify specific reactivity of a pleurotus pulmonalis spore extract. Two atopic subjects served as controls. RESULTS: The skin prick test positive study subject reacted with subjective and objective signs including a significant drop of the FEV1 by nasal challenge at a concentration of 0.1 mg/mL of the pleurotus spore extract while both controls were negative even at a higher test concentration. IgE-immunoblot revealed several distinct bands in the serum of the pleurotus-sensitized subject. CONCLUSION: spores of pleurotus pulmonalis, a common mushroom of the fungal class of basidiomycetes, can cause specific, IgE-mediated acute rhinoconjuncivitis and asthma in sensitized individuals. ( info) |