1/40. sarcoidosis with selective involvement of a second liver allograft: report of a case and review of the literature.A case of sarcoidosis recurrent in a patient's second liver allograft is described. There was no granulomatous disease seen in the patient's first liver allograft. After the second orthotopic liver transplantation (OLT), the patient was successfully treated for acute rejection, aspergillus infection, and cytomegalovirus viremia. Approximately 2 months after the second OLT, the patient was treated with long-term interferon-alpha for recurrent hepatitis c. Five years after the operation, he experienced liver failure secondary to recurrent hepatitis and underwent a third OLT. This is only the second reported case of sarcoidosis recurrent in the liver parenchyma of a transplanted organ and the first in which interferon-alpha might have played a role.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
2/40. Prepubertal periodontitis associated with chronic granulomatous disease.BACKGROUND: Generalised prepubertal periodontitis is a rare entity that is usually a consequence of severe systemic diseases. Chronic granulomatous disease is one of the extremely rare inherited immunodeficiency diseases, which predisposes the patient to recurrent severe bacterial and fungal infections. AIM: The purpose of this report is to describe a 5-year old male patient suffering from prepubertal periodontitis associated with chronic granulomatous disease, who was referred to the Department of Periodontology for treatment of severe gingival inflammation. methods: A detailed past history was obtained and thorough clinical and laboratory examinations were performed. RESULTS: Medical tests revealed the only immunodeficiency sign as the extremely low burst test result. The patient was diagnosed as having an autosomal recessive (AR) form of chronic granulomatous disease. He was put on prophylactic treatment with trimethoprim-sulfamethoxazole (TMP-SMX) and also a periodontal maintenance regimen with regular 1-month intervals. CONCLUSION: This case report emphasises the importance of the differential diagnosis of severe immunodeficiency in the background of prepubertal periodontitis.- - - - - - - - - - ranking = 7keywords = granulomatous disease (Clic here for more details about this article) |
3/40. Rosai-Dorfman disease manifesting as relapsing uveitis and subconjunctival masses.Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy, SHML) is a rare, non-hereditary, benign histiocytic proliferative disorder mainly affecting young people with common clinical characteristics such as painless bilateral cervical lymphadenopathy, fever, leukocytosis and polyclonal gammopathy. Extranodal manifestations have been reported in 28-43% of cases. Eye involvement is relatively uncommon (8.5%), and most of cases have presented as lymphoproliferation in the soft tissues of the orbit and eyelids. uveitis is an even more rare presentation as a review of all the literature. We describe a 63-year-old man with SHML with unusual ophthalmic manifestations of relapsing uveitis and bilateral subconjunctival masses. The results of biopsies were compatible with the characteristic histopathological findings of SHML: focal aggregations of S100-positive foamy histiocytes and the existence of lymphocytophagocytosis. During the clinical course, the patient relapsed but the relapse was relatively benign and the patient showed fair response to topical as well as systemic corticosteroid treatment. Here we describe this unusual presentation of SHML to inform physicians of the possibility for this systemic granulomatous disease to contribute to relapsing uveitis.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
4/40. A case of impaired chemotaxis and lymphocyte transformation.The report describes the clinical syndroms of a 14-year-old boy which suffered from recurrent infections since early infancy. The clinical and general laboratory findings were similar to "the granulomatous disease of childhood" as described by Bridges et al. (8). The following serum factors were determined: immunoglobulins, complement factors, isoagglutinins. The following assays with normal or patient's granulocytes were done: Chemotactic activity, nitroblue-tetrazolium test, bactericidal assay, fungicidal assay, myeloperoxidase, monocytes fungicidal assay. Immunological studies include kinetics of phytohaemagglutinin response, effect of serum of the patient on lymphocytic reactivity in vitro and skin tests. The following results were obtained: 1) Assays with normal or patient's granulocytes showed an impaired chemotatic activity, when serum of the patient was added. There was strong indication by treatment of the patient with plasma infusions, that the chemotactic defect is a serum dependent factor. 2) It could be demonstrated that the patient's serum also inhibited the response of lymphocytes to tuberculin and phytohaemagglutinin. Therefore the patient report focuses attention upon the possibility of serum related abnormalities that may influence granulocytic as well as lymphocytic functions leading to recurrent bacterial, fungal and viral infections.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
5/40. rhinosporidiosis presenting as recurrent nasal polyps.rhinosporidiosis is a chronic granulomatous disease of the mucous membrane, predominantly of the nose and nasopharynx. It is uncommon in malaysia but has been seen in immigrant workers from endemic areas like india and sri lanka. A case seen in Johor is reported here to highlight the need of awareness among clinicians at a time where there is increasing numbers of immigrant workers in our country. The causative organism of this disease is rhinosporidium seeberi, which is found in stagnant waters. sporangia and endospores of R. seeberi are seen in the granulomatous polypoidal lesions. The patients commonly present with epistaxis and nasal blockage. Complete excision is the treatment of choice for this disease. Recurrences are common despite anti-microbial treatment.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
6/40. Recurrent eosinophilic cystitis in a child with chronic granulomatous disease.Eosinophilic cystitis is an uncommon disease in children, and its association with chronic granulomatous disease (CGD) has been previously reported in only five patients. In all those patients the disease showed either a self-limited benign course or a rapid response to corticosteroid treatment. The authors describe a child with X-linked CGD who developed eosinophilic cystitis with a recurrent course and difficult therapeutic management. The authors also discuss the pathogenesis of granuloma formation in CGD and review the literature for current therapies for these complications.- - - - - - - - - - ranking = 5keywords = granulomatous disease (Clic here for more details about this article) |
7/40. Presumed ocular sarcoidosis.BACKGROUND: sarcoidosis is a multisystemic granulomatous disease of unknown etiology. Ocular manifestations commonly occur in patients with sarcoidosis, with a granulomatous anterior uveitis as the most-prevalent ocular sign. Acute symptoms of uveitis, such as pain, photophobia, lacrimation, or redness, may be absent. Without early detection and timely treatment, this "silent uveitis" may cause permanent ocular damage. case reports: Two patients came in for routine eye examinations, with no symptoms of anterior uveitis. The first, a 36-year-old man, had a bilateral granulomatous anterior uveitis. The uveitis was treated with topical corticosteroids, with no complications. The second case involved a 44-year-old woman with chronic, recurrent, bilateral, non-granulomatous anterior uveitis. Despite treatment with topical and oral corticosteroids. the patient had a prolonged course with recurrent episodes leading to secondary sequelae, including glaucoma and cataract. Both patients were co-managed with internal medicine, and in each case, systemic workups were consistent with sarcoidosis. CONCLUSION: Anterior uveitis often manifests as the initial presentation of sarcoidosis. Without acute symptoms, the detection and diagnosis may be delayed, leading to visual deterioration. The diagnosis of sarcoidosis may be difficult, owing to the lack of definitive diagnostic criteria and a variety of presentations. Histologic confirmation may not always be possible or practical. However, a range of serological and radiological tests, when combined with physical and ophthalmic evaluation, can lead to the presumed diagnosis of sarcoidosis. Aggressive treatment is imperative in order to prevent permanent structural damage to the eye resulting from this idiopathic inflammation.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
8/40. paecilomyces lilacinus infection in a child with chronic granulomatous disease.Chronic granulomatous disease (CGD) is a rare inherited disorder in which neutrophils do not appropriately generate cytotoxic superoxide anion, the respiratory burst, in response to invading bacteria or fungi as a part of normal host defence. We report the case of a child with CGD who had two abdominal wall abscesses caused by paecilomyces lilacinus, an organism not previously known to cause infections in patients with CGD. The abdominal wall is a location that is rarely associated with paecilomyces infections. Parenteral amphotericin b eradicated the infection in an immunocompromised child whereas this regimen has heretofore largely been unsuccessful in the treatment of this infection. paecilomyces species and other fungi from immunocompromised hosts and thought to be laboratory contaminants, need to be carefully investigated for they may become pathogens in this clinical setting.- - - - - - - - - - ranking = 5keywords = granulomatous disease (Clic here for more details about this article) |
9/40. X-linked chronic granulomatous disease: report of one case.Chronic granulomatous disease (CGD) is an inherited dysfunction of phagocytic cells secondary to a defect in the respiratory burst to kill catalase-positive microorganisms. This leads to recurrent life-threatening bacterial and fungal infections. We report a 1 year-10 month-old boy with X-linked CGD who was noted to have recurrent suppurative lymphadenitis since one and half month old. failure to thrive, lymphadenitis and generalized skin lesions with multiple scar and dimples were found. Immunological data of patient, his mother and father were as follows: PMN phagocytosis (%): 98, 88, 92 (control, >80), PMN chemotaxis: 0.6, 1.0, 1.3 (control, >1.2), PMN bactericidal function test over a period of 2 hours (%): 28.1, 28.5, 84.8 (control, 82.1). PMN chemiluminescence (delta mV): 0.612, 364.1, 1131 (control, 614.1), H202 production (ug/mL): 1.6, 7.2, 16.8 (control, 14.1), NBT test: negative, mixed, positive (control, positive). His mother was a carrier. The patient expired with invasive salmonella infection before the availability of gamma-interferon prophylaxis.- - - - - - - - - - ranking = 5keywords = granulomatous disease (Clic here for more details about this article) |
10/40. Recurrent multiple hepatic abscesses, hepatic calcification and congenital hearing loss in a child with chronic granulomatous disease.Chronic granulomatous disease (CGD) is a rare inherited primary immunodeficiency in which phagocytes cannot destroy catalase-positive bacteria and fungi. In this article, we describe a 6-year-old boy with CGD associated with recurrent multiple hepatic abscesses, hepatic calcification and congenital hearing loss because of rare presentation. To the best of our knowledge, congenital hearing loss in CGD has not been reported in the literature. In the treatment of our patient, a combination of antibiotherapy, percutaneous drainage and surgical intervention was used, but multiple hepatic abscesses recurred during the follow-up. On account of this case, we would like to reemphasize that recurrent hepatic abscesses are frequently observed and difficult to treat in patients with CGD. Additionally, we would like to state that congenital hearing loss may be a part of the disease, or it may be coincidental. To clarify the last point, we suggest that all patients with CGD should be tested for hearing impairment.- - - - - - - - - - ranking = 5keywords = granulomatous disease (Clic here for more details about this article) |
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