1/6. Primary aspergillosis of the larynx in a patient with Felty's syndrome.Herein we report the first case of primary aspergillosis of the larynx in a patient with Felty's syndrome. A 53-year-old man, a florist by profession, with a 12-year history of rheumatoid arthritis and on treatment with steroids, was admitted because of hoarseness, and intermittent fever of 2 weeks' duration. On admission, physical examination and laboratory data showed, among other findings, splenomegalia and neutropenia. At bone marrow examination, normal cellularity with mild dyserythropoiesis was observed. A fiberoptic laryngoscopy showed white plaques on both the true vocal cords. Both culture and microscopic examination of these lesions provided the diagnosis of invasive process by aspergillus flavus. A computed tomography of the middle ears, paranasal sinuses, and chest was normal. Thus, primary aspergillosis of the larynx and Felty's syndrome was diagnosed, and the patient was successfully treated with granulocyte colony-stimulating factor and systemic antifungal agents. Felty's syndrome, corticosteroid use, and occupational risk probably rendered our patient susceptible to Aspergillus infection.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/6. Deep soft tissue infections in the neutropenic pediatric oncology patient.PURPOSE: Necrotizing fasciitis and myonecrosis can be rapidly fatal without prompt and aggressive medical and surgical therapy. We reviewed our experience with necrotizing fasciitis and myonecrosis in neutropenic pediatric oncology patients to describe associated clinical characteristics and outline therapeutic interventions. patients AND methods: A retrospective chart review was performed for all cases of deep soft tissue infection found in neutropenic pediatric oncology patients during an 11-year period. RESULTS: Seven cases of necrotizing fasciitis and/or myonecrosis associated with chemotherapy-induced neutropenia were diagnosed during the study period. Deep soft tissue infection was diagnosed a median of 14 days after the initiation of chemotherapy. All of the patients presented with fever and pain, generally out of proportion to associated physical findings. Most patients (86%) also had tachycardia and subtle induration at the site of soft tissue infection. The pathogenic organism in four of seven patients originated in the gastrointestinal tract. patients were treated with antibiotics, surgical debridements, granulocyte colony-stimulating factor, and hyperbaric oxygen. Granulocyte transfusions were administered if there were no signs of neutrophil recovery. Five patients survived their deep soft tissue infection. CONCLUSIONS: The diagnosis of necrotizing fasciitis and/or myonecrosis should be considered in any neutropenic patient with fever, tachycardia, and localized pain out of proportion to the physical findings. Appropriate therapy includes broad-spectrum intravenous antibiotics and urgent surgical intervention. granulocyte colony-stimulating factor should be administered to all patients to enhance neutrophil recovery. Granulocyte transfusions should be considered if a prolonged period of neutropenia is anticipated.- - - - - - - - - - ranking = 2keywords = physical (Clic here for more details about this article) |
3/6. New observations in a child with angiofollicular lymph node hyperplasia (Castleman's disease) originated from the mesenteric root.Localized plasma cell type Castleman's disease (CD) is an unusual pathologic entity. It is frequently associated with clinical and laboratory characteristics and rarely occurs in children. Total surgical excision results in cure in all aspects. To make early diagnosis of mesenteric CD is not easy, especially for children. An 11-year-old Taiwanese boy was recently evaluated for anemia and delayed growth. His clinical findings included a syndrome of severe hypochromic microcytic anemia, neutropenia, thrombocytosis, hypoferremia, hypergammaglobulinemia, and growth failure. Radiological examinations (abdominal ultrasound, small intestinal series, and computerized tomography) identified hepatosplenomegaly, nephromegaly, and huge masses in the middle abdomen with precaval, celiac, and paraaortic lymph nodal enlargement. However, detailed physical examination failed to detect a mass. At laparotomy a double-fist-sized confluent mass was found arising from the mesenteric root. Most masses were discrete and were excised individually. The pathologic diagnosis was plasma-cell type angiofollicular lymph node hyperplasia (Castleman's disease). Seven weeks after surgery, he had an episode of acute hepatitis b. Postoperatively, he exhibited a dramatic growth spurt; the hemoglobin, red blood cell indices, serum iron, and immunoglobulins returned to normal in 2 months. neutropenia, which has not been previously related to mesenteric CD, was an unexpected finding in our case; however, it resolved spontaneously 3 months after the surgery, suggesting its causal relationship with the tumor.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/6. Neonatal effects of breast cancer chemotherapy administered during pregnancy.A human fetus is most susceptible to teratogenic agents during the first trimester of pregnancy. cyclophosphamide and doxorubicin are pregnancy category D agents; however, potential benefits may warrant treatment with these agents during pregnancy under special circumstances. During her first trimester of pregnancy, a 37-year-old Caucasian woman was diagnosed with stage IIB infiltrating ductal carcinoma in situ (breast cancer) that was estrogen and progesterone receptor negative and human epidermal growth factor receptor-2 positive. The patient was treated with doxorubicin and cyclophosphamide in the second and third trimesters and delivered a premature baby boy at 31 weeks' gestation. The neonate was intubated on delivery because of respiratory distress and failure; however, no physical anomalies were observed. He had neutropenia and anemia, quite possibly as a result of his mother's chemotherapy 1 week before delivery. He was prophylactically treated for sepsis, but all cultures were negative. The infant grew and developed normally during his first year of life and remained in good health. An objective causality assessment revealed that it was probable that the infant's adverse events (prematurity, neutropenia, and anemia) were related to his mother's doxorubicin and cyclophosphamide therapy; however, these were the only adverse events potentially linked to in utero exposure to chemotherapy during the second and third trimesters. Due to the special considerations of both mother and infant, optimal treatment for patients with pregnancy-associated breast cancer requires the expert opinion of a multidisciplinary care team.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/6. Cyclic hematopoiesis: human cyclic neutropenia.Human cyclic neutropenia is a relatively rare disorder of unknown etiology. Study of patients and animals with the disorder has led to important information regarding the differentiation of blood cells and control mechanisms of hematopoietic regulation. It has a world-wide distribution, occurs in both sexes, and, in about one-fourth of the patients, a family history has been obtained. While usually benign, deaths from overwhelming infections occur. In addition to cycling of neutrophils, in the majority of cases the monocytes cycle and in about one-fifth of the cases eosinophils are elevated. In a small number of patients, cycling of platelets and reticulocytes occurs. Cycles of colony stimulating factor are present. Cycles of bone marrow cells are easily demonstrable. The recent transfer of human cyclic neutropenia following allogenic bone marrow grafting confirms the hypothesis that the disorder is of bone marrow origin. The following subjects are covered in this review article: A. Definition, history, and incidence; B. Etiology, geographic distribution, mode of transmission; C. Symptoms, physical signs, diagnosis, clinical course; D. Clinical laboratory studies; E. Experimental studies; F. prognosis; G. Treatment. It is felt that human cyclic neutropenia represents a heterogeneous group of disorders and that much remains to be learned about its cause(s).- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/6. A syndrome of facial dysmorphia, birth defects, myelodysplasia and immunodeficiency in three sibs of consanguineous parents.We report on three sibs (two females, one male) with a syndrome including physical and mental developmental delay, facial dysmorphia, an increased number of skin folds and recurrent infections. Pulmonary infections were especially severe and frequent, leading to bronchiectasis. steatorrhea was intermittent. A sweat test was normal. Congenital heart defect was present in two sibs and vesico-ureteral reflux was diagnosed in two sibs. neutropenia was the most obvious immunological defect. Myelodysplasia was noted. Acute myeloblastic leukemia developed in one sib. The healthy parents of the patients are first cousins. The consanguinity of the parents points-to an autosomal recessive mode of inheritance of this new syndrome but a mitochondrial disorder cannot be excluded.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |