1/23. Effectiveness of IFN-gamma for liver abscesses in chronic granulomatous disease.In chronic granulomatous disease, interferon-gamma (IFN-gamma) significantly reduces the incidence and severity of recurrent infections, but its effectiveness administered ex novo during acute infection has been reported in only one case. In this report, we describe two adult brothers with chronic granulomatous disease treated successfully with IFN-gamma for acute liver abscesses. Two brothers with severe recurrent infections of unknown origin were hospitalized for septic fever, malnutrition, and ultrasonographic evidence of liver abscess. Autosomal recessive chronic granulomatous disease was diagnosed based on lack of superoxide anion production by phagocytes and absence of p47-phox protein. An antibiotic regimen specifically directed against staphylococcus aureus was ineffective, whereas treatment with 50 microg/m2 IFN-gamma s.c. thrice weekly induced complete healing with scarring within 3 months. No septic recurrence was observed during a 4-year follow-up period. In chronic granulomatous disease, IFN-gamma is effective not only in preventing but also in healing life-threatening acute infections.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
2/23. keratitis caused by candida glabrata in a patient with chronic granulomatous disease.PURPOSE: To report an unusual ocular presentation of candida glabrata in a patient with chronic granulomatous disease. methods: Interventional case report. A 15-year-old boy with chronic granulomatous disease presented with bilateral limbal infiltrates. He had been receiving broad-spectrum systemic antibiotics for recurrent liver abscesses. The keratitis did not respond to antibiotics and did not resolve after a course of topical steroids. RESULTS: Corneal cultures revealed candida glabrata. The same species was simultaneously isolated from the surgical drainage of the liver abscesses. The ocular and hepatic findings resolved on intravenous amphotericin b. CONCLUSION: candida glabrata has recently emerged as an important nosocomial pathogen. It may present as a limbal keratitis in the setting of systemic infection.- - - - - - - - - - ranking = 0.75keywords = granulomatous disease (Clic here for more details about this article) |
3/23. Percutaneous transhepatic alcoholization: a new therapeutic strategy in children with chronic granulomatous disease and liver abscess.Children affected with chronic granulomatous disease develop severe infections, which frequently evolve to abscess. In most instances the liver is involved. We report a case of liver abscess successfully treated by percutaneous transhepatic alcoholization after antibiotic therapy and percutaneous drainage had failed. This procedure avoided the need for hepatic resection, which is associated with high morbidity in such patients.- - - - - - - - - - ranking = 0.625keywords = granulomatous disease (Clic here for more details about this article) |
4/23. Actinomycotic liver abscess. Case report and review of the literature.actinomycosis is a chronic suppurative, granulomatous disease, which is characterized clinically by extensive necrosis and abscess formation, and histologically by the presence of the so-called "sulphur granules". actinomycosis is generally classified as being of cervicofacial, thoracic or abdominal type. The objective of this study is the presentation of a case of actinomycotic liver abscess that we encountered in our department 5 years ago, along with a brief review of this rare disease. A 39-year-old male non-Greek Caucasian patient presented with high fever, malaise, anorexia, vomiting after food ingestion and right upper quadrant pain. Leucocytosis with a left shift was present, and ultrasonography demonstrated a mass of mixed composition in the anterior part of the right hepatic lobe, measuring 6.8 x 4.6 cm, suggestive of an abscess or haemangioma. MRI scan confirmed the presence of a space-occupying lesion, suggestive of an abscess. The patient was submitted to surgical drainage of the hepatic abscess. The culture of the purulent material was found to be sterile, while the histochemical examination of the specimen demonstrated the presence of actinomycosis. The patient had an uneventful postoperative course and after discharge received prolonged chemoprophylaxis. Actinomycotic liver abscess is a very rare clinical entity, and only 57 cases have been reported in the English literature. Due to the rarity of the disease and the limited number of reported cases, we considered it useful to report this case.- - - - - - - - - - ranking = 0.125keywords = granulomatous disease (Clic here for more details about this article) |
5/23. Molecular characterization of a novel splice site mutation within the CYBB gene leading to X-linked chronic granulomatous disease.Chronic granulomatous disease (CGD) is a primary immunodeficiency that affects the oxidative mechanism of microbial killing of phagocytic cells. The defect is characterized by a lack or severely reduced superoxide anion (O2-) production by phagocytes. Seventy percent of CGD cases are X-linked (X-CGD) and they are caused by mutations in the gene encoding for gp91(phox), one of the two subunits of the flavocytochrome b558 of the nadph oxidase. We identified an abnormal transcript arising from a novel splice site mutation within the gene encoding gp91(phox), which suggested that the mutation affected normal mRNA splicing. Thus, the effect of this mutation leads to the complete absence of the flavocytochrome b558 in neutrophil membranes, which caused the biochemical phenotype X91 degrees-CGD in this family. These molecular findings help to explain the early onset and severe phenotype in this X-CGD kindred.- - - - - - - - - - ranking = 0.625keywords = granulomatous disease (Clic here for more details about this article) |
6/23. 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 = 0.625keywords = granulomatous disease (Clic here for more details about this article) |
7/23. Invasive chromobacterium violaceum infection in children: case report and review.A 3.3-year-old boy developed chromobacterium violaceum abscesses of lungs, liver and spleen and was successfully treated. He had chronic granulomatous disease (CGD). Twenty-five episodes of invasive C. violaceum infection in 24 children were reviewed. All 9 CGD and 10 nonbacteremic cases survived, but 12 of 16 (75%) non-CGD and 12 of 15 (80%) bacteremic patients died.- - - - - - - - - - ranking = 0.125keywords = granulomatous disease (Clic here for more details about this article) |
8/23. Granulocyte-macrophage colony stimulating factor does not improve neutrophil oxidative metabolism in a patient with variant X-linked chronic granulomatous disease.Variant X-linked chronic granulomatous disease (CGD) is characterised by a decreased but still measurable respiratory burst and cytochrome b content of phagocytes resulting in a clinically milder form of the disease. We examined the in vivo effect of recombinant human granulocyte-macrophage colony stimulating factor (rh-GM-CSF) on the neutrophil functions of a patient treated for liver abscess. The number of white blood cells was markedly increased at the highest dose of GM-CSF injected (30 micrograms/kg per day). This was mainly due to a large increase in eosinophils and to a lesser extent in neutrophils. No change in the deficient neutrophil respiratory burst nitroblue tetrazolium (NBT)-reduction, superoxide (O2-)-production and cytochrome b content was observed during 6 weeks of therapy with increasing doses of GM-CSF. No significant clinical improvement of the liver abscess was observed during treatment with GM-CSF.- - - - - - - - - - ranking = 0.625keywords = granulomatous disease (Clic here for more details about this article) |
9/23. Corticosteroid therapy for refractory infections in chronic granulomatous disease: case reports and review of the literature.BACKGROUND: Chronic granulomatous disease (CGD) is a primary phagocyte immunodeficiency. It is often accompanied by an exuberant and aberrant inflammatory response, with granulomata and obstruction of the gastrointestinal and genitourinary tracts and inflammatory bowel disease. Although corticosteroids are successful in managing the obstructive and inflammatory disorders of CGD, they are not ordinarily used for the management of infection because of the possibility of further compromising the patient's immune system. OBJECTIVES: To discuss the pros and cons of the use of corticosteroids for the treatment of infections in CGD. methods: We describe 2 patients with CGD and refractory infections who were successfully treated with systemic corticosteroids in addition to antimicrobial agents. We also review the medical literature in which corticosteroids have been used for CGD infection. RESULTS: Our cases add to 3 other reports in which antibiotics and corticosteroids were used successfully in patients with CGD. However, in the presence of a potential pathogen, notably, aspergilla, corticosteroids may mask or favor dissemination of the fungus, especially in adults. CONCLUSIONS: Corticosteroids may play an important adjunctive role in CGD refractory infections.- - - - - - - - - - ranking = 0.625keywords = granulomatous disease (Clic here for more details about this article) |
10/23. Successful management of hepatic abscesses by percutaneous catheter drainage in chronic granulomatous disease.Chronic granulomatous disease (CGD) is a disorder of polymorphonuclear leukocytes that can cause multiple recurrent hepatic abscesses in 40% of those patients with the disorder. The mortality rate from this complication of CGD is estimated at 27%. Treatment has consisted of extensive surgical debridement and drainage and prolonged antibiotic therapy; however, this approach is accompanied by high morbidity and the frequent need for reoperation. Successful percutaneous drainage of multiple hepatic abscesses in a patient who had previously undergone 10 operative procedures to manage hepatic abscesses is reported. With the development of imaging and percutaneous drainage techniques, as well as the recurrent nature of this problem, percutaneous management should be given consideration in appropriate patients with CGD with hepatic abscesses.- - - - - - - - - - ranking = 0.625keywords = granulomatous disease (Clic here for more details about this article) |
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