1/137. Congenital neutropenia. Report of a case and a biorationale for dental management.Congenital neutropenia is characterized by a marked decrease in or lack of circulating PMN's in children with no prior history of drug intake. The neutropenia is persistent and the clinical course is one of early onset of severe, recurrent, and eventually fatal infections. bone marrow studies show a maturation arrest of neutrophilic precursors. Because of their greatly increased susceptibility to infection, patients with congenital neutropenia present a difficult dental management problem. A case of congenital neutropenia has been presented, as well as a biorationale for dental treatment. On the basis of reports in the literature, the following recommendations for the management of patients with congenital neutropenia are made: 1. The prevention and control of infection and the interception of dental disease before surgical intervention becomes necessary should be the overriding considerations in the management of patients with congenital neutropenia. 2. The carious breakdown of teeth should be prevented by the daily application of a 0.4 per cent stannous fluoride gel in addition to oral hygiene and limitation of sucrose intake. 3. Periodontal therapy should be palliative only, since alveolar bone loss is progressive despite frequent oral hygiene instruction and prophylaxis. The goal of periodontal therapy for patients with congenital neutropenia should therefore be a decrease in gingival inflammation to make the patient's mouth more comfortable and to slow down alveolar bone loss. Periodontal surgery is contraindicated. 4. bacteremia and subsequent septicemia should be prevented since a minor infection can become life threatening in patients with congenital neutropenia. The patient should rinse for 30 seconds and the gingival sulci should be irrigated with a phenolated antiseptic mouthwash prior to all dental manipulations of the soft tissue. This will significantly reduce the incidence of bacteremia. 5. Surgery should be avoided if at all possible because of the high risk of post-operative infection. All surgery sholld be performed in the hospital, and the patient should be given antibiotics as determined by his physician. Primary closure should be done with fine polyglycolic acid sutures to reduce the chance of infection. If postoperative infection can be prevented, wound healing will progress normally despite the complete absence of PMN's.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
2/137. The use of recombinant human G-CSF in the treatment of propylthiouracil-induced agranulocytosis.A 43-year-old female patient with Basedow-Graves' disease developed agranulocytosis in the eighth month of propylthiouracil therapy. After discontinuing the drug, a broad spectrum antibiotic regimen plus recombinant human granulocyte colony-stimulating factor (G-CSF), a human haematopoietic growth factor, were started. Her granulocyte count returned to normal with the second dose of G-CSF, and ulcerating pharyngitis improved rapidly. We think that in patients with propylthiouracil-induced agranulocytosis, G-CSF will reduce the risk and severity of infection, and should be accepted as a part of the standard therapy.- - - - - - - - - - ranking = 0.14285714285714keywords = infection (Clic here for more details about this article) |
3/137. Chronic neutropenia: Response to plasma with high colony-stimulating activity.A child with repeated infections was immunologically normal but was found to have neutropenia with periodic elevations of the absolute mature polymorphonuclear count at 21-day intervals. Immediately following the PMN rise, bone marrow morphology and in vitro cultures demonstrated a maturation arrest at the myelocyte stage with an increase in proliferative capacity. His cycle was not altered by infusions of normal plasma or by injections of epinephrine or typhoid vaccine. Infusion of 10 ml/kg of "stimulated" plasma from donors reactive to TV, obtained 60 minutes following immunization, resulted in an out-of-phase rise in PMN cells and clinical improvement. in vitro assays, using normal or patient marrow, detected high levels of colony-stimulating activity only in those plasma samples that were effective in the patient. These observations support a role of CSA as a physiologic regulator of granulopoiesis in man.- - - - - - - - - - ranking = 0.14285714285714keywords = infection (Clic here for more details about this article) |
4/137. Alteration of colony-stimulating factor output, endotoxemia, and granulopoiesis in cyclic neutropenia.Cellular and humoral factors involved in the regulation of granulopoiesis were evaluated in two patients with cyclic neutropenia by utilizing the agar-gel marrow culture technique to serially study marrow granulocytic colony-forming capacity (CFC) and the urinary output of colony-stimulating factor (CSF). CSF output varied inversely with peripheral neutrophil counts and directly with monocyte counts and evidence for infection (endotoxemia and/or staphylococcal abscesses). Following autologous infusion of one patient's plasma obtained during a period of neutropenia, increased urinary excretion of CSF occurred concomitant with increments in both marrow CFC and the proportion of granulocytic progenitor cells in dna synthesis. Neutrophil periodicity was not altered by the administration of the neutropenic plasma. These findings are consistent with the hypothesis that cyclic neutropenia is caused by a quantitatively decreased entry of stem cells or granulocytic progenitor cells into granulopoiesis.- - - - - - - - - - ranking = 0.14285714285714keywords = infection (Clic here for more details about this article) |
5/137. Infantile genetic agranulocytosis, morbus Kostmann: presentation of six cases from the original "Kostmann family" and a review.In 1956 Rolf Kostmann reported on six children with severe neutropenia associated with a block in myelopoiesis at the promyelocyte/myelocyte stage and an autosomal recessive inheritance. He named the new syndrome infantile genetic agranulocytosis. Today it is known as Kostmann's syndrome or severe congenital neutropenia. In 1975 an additional 10 cases from northern sweden were published. This article reports on the only long-term survivor from the 1975 report plus another five patients born after 1975 who belong to the original "Kostmann family". Treatment and survival have changed dramatically since Kostmann's first publication. In the pre-antibiotic era, Kostmann's syndrome was inevitably fatal during the first year of life. CONCLUSION: Since the introduction of recombinant human granulocyte colony-stimulating factor (G-CSF) about 10 y ago, most patients now enjoy a normal life span and a greatly improved quality of life. Although the threat of death has disappeared, patients still have problems with infections, especially chronic gingivitis and periodontitis. In other groups of severe neutropenia, not related to the original "Kostmann family", an increased incidence of myeloid leukaemia has been observed. However, in this small cohort none of the children on chronic G-CSF therapy have developed malignancies.- - - - - - - - - - ranking = 0.14285714285714keywords = infection (Clic here for more details about this article) |
6/137. Reversible agranulocytosis associated with oral terbinafine in a pediatric patient.We report a case of terbinafine-induced agranulocytosis in a pediatric patient. This effect was noted 4 weeks after starting terbinafine and resolved within 1 week without the institution of therapy with granulocyte colony-stimulating factor, a course similar to reports in adults. With the incidence of severe side effects still unclear in children, this case argues for continued blood surveillance during courses of terbinafine and for the confirmation of infection before initiation of the drug.- - - - - - - - - - ranking = 0.14285714285714keywords = infection (Clic here for more details about this article) |
7/137. Investigation of coronary artery bypass grafting for a patient with myelodysplastic syndrome.A 79-year-old male with unstable angina, who had myelodysplastic syndrome (MDS), was treated with coronary artery bypass grafting (CABG). MDS causes refractory anemia accompanied by various degrees of granulocytopenia and thrombocytopenia. pancytopenia caused by MDS may complicate patients with major infections and bleeding during cardiac surgery. There were very few patients with MDS who had undergone open-heart surgery. Three case studies, including this study, had reported successful cases of CABG in patients with MDS and the analogous diseases of MDS. We used granulocyte colony-stimulating factor (G-CSF), red blood cells (RBCs) and platelets transfusions in peri-operative state. We did not need a large amount of transfusion of RBCs and platelets in intra-operative and postoperative states. We had prevented major bleeding and severe wound infections in the acute postoperative state.- - - - - - - - - - ranking = 0.28571428571429keywords = infection (Clic here for more details about this article) |
8/137. Multidrug-resistant trichosporon asahii infection of nongranulocytopenic patients in three intensive care units.trichosporon asahii (trichosporon beigelii) infections are rare but have been associated with a wide spectrum of clinical manifestations, ranging from superficial involvement in immunocompetent individuals to severe systemic disease in immunocompromised patients. We report on the recent recovery of T. asahii isolates with reduced susceptibility in vitro to amphotericin b (AMB), flucytosine, and azoles from six nongranulocytopenic patients who exhibited risk factors and who developed either superficial infections (four individuals) or invasive infections (two individuals) while in intensive care units. The latter two patients responded clinically and microbiologically to AMB treatment. All six isolates were closely related according to random amplified polymorphic dna studies and showed 71% similarity by amplified fragment length polymorphism analysis, suggesting a common nosocomial origin. We also review the literature pertaining to T. asahii infections and discuss the salient characteristics of this fungus and recent taxonomic proposals for the genus.- - - - - - - - - - ranking = 1.1428571428571keywords = infection (Clic here for more details about this article) |
9/137. invasive pulmonary aspergillosis in a puerperant with drug-induced agranulocytosis.invasive pulmonary aspergillosis (IPA) is an acute infection of aspergillus species to the lungs. It generally occurs in immunocompromised hosts, especially with neutropenia. We report a 30-year-old puerperant, who developed IPA from agranulocytosis. She had been treated for threatened labor with ritodrine and cefepime, one of which induced agranulocytosis. After vaginal delivery of twins, pneumonia emerged in the right lower lobe. She was diagnosed to have IPA according to the halo sign on computed tomography (CT) and positive circulating antibody against aspergillus, and was treated successfully with oral itraconazole followed by surgical resection. It is important to note that IPA might arise in otherwise immunocompetent hosts when neutropenia is long-standing.- - - - - - - - - - ranking = 0.14285714285714keywords = infection (Clic here for more details about this article) |
10/137. Penicillin-resistant aerococcus viridans bacteremia associated with granulocytopenia.aerococcus viridans, a catalase-negative gram-positive coccus rarely causing bacteremia, was isolated from blood cultures of a 52-yr-old man under the granulocytopenic condition. The isolate showed the typical characteristics of A. viridans, i.e., tetrad arrangements in gram stain, positive pyrrolidonyl aminopeptidase (PYR) and negative leucine aminopeptidase (LAP) reactions, and no growth at 45 degrees C.The isolate was revealed to be highly resistant to penicillin, erythromycin, clindamycin, and ceftriaxone, although most strains of A. viridans isolated from the previously reported patients were susceptible to penicillin and other commonly used antibiotics. Even though A. viridans is rarely associated with human infections, it could be a potential causative agent of bacteremia, especially in immunocompromised patients.- - - - - - - - - - ranking = 0.14285714285714keywords = infection (Clic here for more details about this article) |
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