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1/10. Microvascular submandibular gland transfer for severe cases of keratoconjunctivitis sicca.

    Free submandibular salivary gland transfer was investigated as a surgical method for the treatment of severe keratoconjunctivitis sicca. In an animal model, we examined the tolerance of warm ischemia of the submandibular gland. After temporary interruption of the blood supply (1 to 6 hours), the morphologic changes in the submandibular gland were analyzed histologically and immunohistochemically in 41 rabbits. From 1.5 hours ischemia onward, an increasing structural damage of the parenchyma with emphasis on the secretory cells was seen. Six hours of ischemia caused total necrosis of the salivary gland. Our clinical experience includes 24 highly selected patients suffering from keratoconjunctivitis sicca, in whom we transferred 31 autologous submandibular glands to the temple for permanent autologous tear substitution within the past 4 years. The glands were implanted into a pocket prepared in the temporalis muscle, and the nourishing vessels were anastomosed to the superficial temporal artery and vein. The submandibular duct was implanted into the upper lateral conjunctival fornix. The transferred glands were left denervated. In addition to the clinical examination, scintigraphy with Tc 99m pertechnetate was used to document the graft's viability after the transfer. Viable incorporation with longstanding secretory function occurred in 26 of the 30 transplanted denervated salivary glands. The resulting lubrication of the treated eyes was irregular for up to 3 months in almost even case. One year after surgery, all patients with a viable transplant developed at least occasional epiphora, which was surgically managed by reducing the size of the graft in 10 patients. No severe side effects were seen in this series. The ophthalmologic evaluation of the method included the assessment of dry eye symptoms and of the volume and quality of ocular lubrication (Schirmer test, fluorescein break-up time), the pathology of the ocular surface (rose bengal staining), and the need for pharmaceutical tear substitutes. One year after surgery, 18 of 27 cases assessed were judged as significantly improved by these tests.
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keywords = submandibular gland, submandibular, gland
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2/10. Primary and secondary sjogren's syndrome in children--a comparative study.

    sjogren's syndrome is a chronic inflammatory systemic autoimmune disease mainly affecting the exocrine and, particularly, the salivary and lacrimal glands. The condition usually occurs in adults. In 1994, the criteria for this syndrome were redefined in a multicenter European study. In children, sjogren's syndrome is a rare and probably underdiagnosed disease. To date, sjogren's syndrome in children has only been described in case reports and in the comparative presentation of various study results. So far, no study of a comparative classification into primary and secondary sjogren's syndrome has been carried out in a patient population of any size. sjogren's syndrome should be considered in the differential diagnosis of children with recurrent parotitis, keratoconjunctivitis sicca, or pronounced and early tooth decay associated with xerostomia. In this study of 23 children and adolescents under the age of 16 with the clinical symptoms and laboratory findings of sjogren's syndrome, we differentiate between primary and secondary sjogren's syndrome. The value of the individual methods of assessing the oral and the ophthalmological components and the manifestation of the underlying rheumatic condition are discussed on the basis of the EULAR criteria. The EULAR diagnostic criteria are of limited applicability in children because reliable anamnestic data are frequently lacking. Another problem in diagnosing sjogren's syndrome is the short-term detection of serological alterations and clinical symptoms. Even if young patients do not completely fulfill the required criteria, sjogren's syndrome can be assumed or confirmed in the presence of positive testing for oral and ocular manifestations and recurrent salivary gland enlargement.
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ranking = 0.010457553076365
keywords = gland
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3/10. Treatment of keratoconjunctivitis sicca with topical androgen.

    OBJECTIVE: androgens have been reported to influence lipid production of sebaceous glands and even many ocular tissues. The effect of topical androgen therapy on a 54-year-old patient with keratoconjunctivitis sicca (KCS) and decreased lipid phase of the tear film is reported. methods: For assessment of the lipid phase of the tear film, break up time (BUT) and lipid layer thickness (LLT) were monitored during 6 months before treatment as well as 3 months while using a daily topical androgen therapy. RESULTS: During the topical androgen therapy the pathological lipid phase of the tear film was completely restored indicated by the normalisation of the values of BUT and LLT. CONCLUSION: These findings are consistent with animal experiments indicating that topical administered androgen can restore the decreased lipid phase of the tear film. This may open up new therapeutic strategies for KCS.
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ranking = 0.0052287765381826
keywords = gland
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4/10. Epstein-Barr virus dacryoadenitis resulting in keratoconjunctivitis sicca in a child.

    PURPOSE: To describe a case of severe dry eye syndrome in a child. methods: Observational case report. The authors describe a 10-year-old male with severe dry eyes who was profoundly disabled by pain and photophobia despite aggressive conventional therapy. Lacrimal gland histology was consistent with the primary Sjogren syndrome, and serologic and immunohistologic evidence supported the hypothesis of Epstein-Barr virus causality. RESULTS: Treatment with systemic acyclovir and cyclosporin A resulted in dramatic and rapid reversal of the profound sicca syndrome and enabled the patient to resume his normal activities. CONCLUSION: Epstein-Barr virus dacryoadenitis should be considered in the differential diagnosis of keratoconjunctivitis sicca in children. Epstein-Barr virus can cause keratoconjunctivitis sicca, which can be treated successfully with acyclovir therapy in addition to suppression of the inflammatory response.
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keywords = gland
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5/10. A diffuse infiltrative CD8 lymphocytosis syndrome in human immunodeficiency virus (hiv) infection: a host immune response associated with HLA-DR5.

    STUDY OBJECTIVE: To describe the clinical, immunologic, and immunogenetic features of a diffuse infiltrative lymphocytic disorder resembling Sjogren syndrome in persons infected with human immunodeficiency virus (hiv). DESIGN: Clinical case study. SETTING: University-affiliated hospitals and outpatient clinics. patients: Consecutive sample of 17 patients. MEASUREMENTS AND MAIN RESULTS: All of the 17 patients had bilateral parotid gland enlargement; 14 had xerostomia and 6 had xerophthalmia. Of the 17 patients, 14 had generalized lymphadenopathy, 10 had histologically proved lymphocytic interstitial pneumonitis, 4 had neurologic involvement, and 3 had lymphocytic infiltration of the gastrointestinal tract. gallium scanning in all of 11 tested patients showed abnormal salivary gland uptake. Minor salivary gland biopsies showed more than 2 lymphocytic foci per 4 mm2 tissue in all of 11 tested patients, the infiltrate consisting predominantly of CD8 cells. Fifteen patients had circulating CD8 lymphocytosis; the principal phenotype of these cells was CD8 CD29 . Rheumatoid factor and antinuclear antibodies were infrequent, and none of the patients had anti-Ro/SS-A or anti-La/SS-B antibodies. HLA-DR5 was significantly more frequent in the black patients (10 of 12) compared with controls (13 of 45). Only one patient developed an opportunistic infection during 544 patient-months of study, and none has died of AIDS. CONCLUSIONS: A distinct syndrome primarily characterized by parotid gland enlargement, sicca symptoms, and pulmonary involvement occurs in hiv infection. This disorder is associated with CD8 lymphocytosis and the presence of HLA-DR5, and appears to be a genetically determined host immune response to hiv.
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ranking = 0.02091510615273
keywords = gland
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6/10. Ocular manifestations of patients with circulating antineutrophil cytoplasmic antibodies.

    Antineutrophil cytoplasmic antibodies are seen in patients with systemic vasculitides, especially Wegener's granulomatosis. Antineutrophil cytoplasmic antibodies are helpful laboratory markers for these disease. We report on the ocular findings of six patients with systemic vasculitis who had antineutrophil cytoplasmic antibodies. Four patients had systemic Wegener's granulomatosis, one had microscopic polyarteritis, and in one a specific histopathologic diagnosis could not be made. Two patients were first evaluated for systemic vasculitis because of their ocular manifestations. Ocular findings included ptosis, bilateral lacrimal gland masses, proptosis, choroidal folds, episcleritis, phlebitis, retinal and vitreous hemorrhage, keratitis sicca, and bilateral central scotomas. It was difficult to make a systemic diagnosis in all cases. If systemic vasculitis is in the differential diagnosis of a patient with suggestive ocular findings, antineutrophil cytoplasmic antibody testing should be considered. A prospective study of antineutrophil cytoplasmic antibody testing should be considered in patients with ocular findings that suggest the possibility of vasculitis.
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keywords = gland
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7/10. Amylase in mare lacrimale in patients with submandibular salivary gland transplantation to the lacrimal basin.

    Six patients with dry eyes of different etiologies underwent transplants of 1.0 to 1.6 ml of submandibular salivary gland tissue, five of them to one eye and one to both eyes. In the four cases in which the transplants survived, the amylase activity in tear fluid sampled from the cisterna lacrimalis (temporal canthal meniscus) had a mean value of 5,147 U/l in contrast with the mean value 943 U/l of the fellow control eyes. In the two eyes in which the transplants failed to survive, the average value was 635 U/l. The small sample size does not enable calculation of statistical significance to the results but suggests that salivary amylase determinations in tear fluid would facilitate assessment of the functional status of the transplanted salivary tissue.
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ranking = 0.20652783179893
keywords = submandibular, gland
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8/10. diagnosis of MALT lymphoma by conjunctival biopsy: a case report.

    BACKGROUND: Most extranodular lymphatic tissue is found in the intestinal mucosa. Together with similarly structured lymphatic tissue at other locations it has been named mucosa-associated lymphatic tissue (MALT). Malignant transformation of such tissue to lymphoma is well known. Although MALT lymphoma has been described in tissue physiologically void of MALT, lymphoma manifestation in the conjunctiva is rare. methods: We report a case of a 47-year-old woman who was referred to our clinic for symptomatic treatment and evaluation of severe symptoms of dry eyes. She was thought to suffer from sjogren's syndrome because of xerophthalmia and xerostomia, as well as massive bilateral swelling of the parotid gland. Ophthalmological examination revealed marked hyperplasia of the conjunctiva, of which a biopsy was taken. RESULTS: Histological and immunohistochemical examination of the conjunctival biopsy, together with analysis of gene rearrangement by Southern blot, led to the diagnosis of low-grade B-cell lymphoma of the MALT. CONCLUSION: The differential diagnosis of keratoconjunctivitis sicca presenting with conjunctival swelling of unknown origin should include lymphoma, especially since sjogren's syndrome may be associated with malignant disorders of the lymphatic system. A biopsy of suspicious conjunctival changes can clarify a multisystem disease by providing a tissue diagnosis.
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ranking = 0.0052287765381826
keywords = gland
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9/10. keratoconjunctivitis sicca associated with achalasia of the cardia, adrenocortical insufficiency, and lacrimal gland degeneration: keratoconjunctivitis sicca secondary to lacrimal gland degeneration may parallel degenerative changes in esophageal and adrenocortical function.

    OBJECTIVE/DESIGN: This study aimed to examine and describe three siblings with alacrima, the eldest of whom had associated achalasia and adrenocortical insufficiency. PARTICIPANTS: Three affected siblings and four age-matched control subjects participated. INTERVENTION/MAIN OUTCOME MEASURES: The three children underwent complete ophthalmologic examinations; computed tomographic scanning of brain, orbit, chest, and abdomen; and measurement of serum cortisol. All three were subjected to a short synacthen challenge. Lacrimal gland biopsies were performed on the two younger subjects, and specimens were studied by light and electron microscopy. RESULTS: All three children showed virtually absent tear secretion as tested by the Schirmer test. The resulting keratopathy was most severe in the oldest child, who developed bilateral corneal melting. The two younger children showed interpalpebral corneal staining with rose bengal. All three children improved after punctal occlusion. Addison's disease was present in the oldest child. Computed tomographic scanning showed absent lacrimal and shrunken adrenal glands in association with achalasia of the cardia in the oldest child. The lacrimal glands were found to be reduced in size in the next eldest child. When evaluated by electron microscopy, the lacrimal gland biopsy specimens from the two younger children showed neuronal degeneration associated with depletion of secretory granules in the acinar cells. CONCLUSION: In this disease, radiologic evidence of reducing lacrimal gland size with increasing age could represent a degenerative process. This may be paralleled by other signs and the possibility of adrenocortical insufficiency and achalasia of the cardia should be investigated in all children presenting with dry eyes. These children appear to have a progressive neuronal disease.
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ranking = 0.067974094996373
keywords = gland
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10/10. CD4 mononuclear cell infiltrates and Fas/Fas ligand positive mammary gland cells in breast tissue from a patient with sjogren's syndrome.

    We describe a 49-year-old patient with lip biopsy proven sjogren's syndrome (SS) and keratoconjunctivitis sicca, who had dental caries, xerostomia, recurrent upper respiratory tract infections, arthritis in her hands, elbows and knees, and recurrent parotid inflammation. She developed bilateral breast nodules in 1988. Right breast nodules were excised in 1993 and 1995, but reappeared in 1996, requiring 2 more excisions. breast tissue samples showed remarkable intralobular and perilobular mononuclear cell infiltrates that were predominantly CD4 T cells and expressed bcl-2. A few cells stained CD20 and CD8 . SS breast glandular epithelial cells stained more intensely for Fas compared to normal cells. CD4 T cells and Fas mediated cell death may be involved in the mammary gland lesions in SS.
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ranking = 0.031372659229095
keywords = gland
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