1/409. Selective antibody deficiency to bacterial polysaccharide antigens in patients with netherton syndrome. Three patients with netherton syndrome, recurrent sinopulmonary infections, and humoral immune deficiency are described. Although quantitative serum immunoglobulin levels were generally normal, two patients had selective antibody deficiency to bacterial polysaccharide antigens, one associated with IgA-IgG-2 deficiency. A third patient had an antibody deficiency to protein antigens. This is the first report, to our knowledge, that describes antibody deficiency in patients with netherton syndrome. This finding demonstrates the importance of evaluating functional antibody responses to both protein and bacterial polysaccharide antigens and not relying on IgG subclass determination. ( info) |
2/409. Acute hemorrhagic leukoencephalitis in patients with acute myeloid leukemia in hematologic complete remission. The authors describe the cases of three patients affected by acute myeloid leukemia, in complete remission, who rapidly developed neurologic symptoms leading to death. Neither clinical characteristics, nor radiological or microbiological procedures, allowed an etiological diagnosis of the neurologic syndrome. Post-mortem examination of the brain showed both macroscopic and microscopic findings compatible with acute hemorrhagic leukoencephalitis. The difficulty in distinguishing this entity from other CNS disease-related complications (e.g. leukemia infiltration, drug toxicity, hemorrhages) should not lead to an underestimation of the true incidence of this complication. We believe that with more attention to the possibility of this complication there would probably be both a greater possibility of collecting clinical informations about the real impact of this dramatic disease and a stronger hope of finding the right treatment for it. ( info) |
3/409. Clinical and scientific importance of source control in abdominal infections: summary of a symposium. In May 1997, a panel of surgeon-investigators met to discuss the clinical importance and research implications of controlling the source of abdominal infections. It was concluded that source control is critical to therapeutic success and that antimicrobial therapy and other adjunctive interventions will fail if the source of infection is not controlled by resection, exteriorization or other means. The panelists presented different definitions of source control, depending on the scientific purpose of the definition. All participants agreed that failure to consider the adequacy of source control of infection has limited the value of most clinical trials of therapeutic anti-infective agents. Besides recognizing source control as an essential goal of patient care, the panelists emphasized the need for further investigative work to define, record and stratify the adequacy of source control in clinical trials of therapeutic agents for abdominal infections. ( info) |
4/409. Regression of cancer following surgery. Postsurgical tumor regressions are rare but well-recognized entities. The causes of such phenomena are unknown but probably are multiple. Regression of any tumor must ultimately come about by changes in the host-tumor interaction which suddenly becomes unfavorable for tumor growth. This is likely a manifestation of changes in the immunocompetence of the host. There are many variables influencing the host-tumor interaction, and the study of tumor immunology is stil in its infancy. It is through the exploration and investigation of basic mechanisms of the immunology of cancer that the most potentially fruitful associations between tumor growth or regression and surgical manipulations may be made and applied to the treatment of cancer patients. ( info) |
5/409. Significance of serum c-reactive protein elevation in patients with systemic lupus erythematosus. serum c-reactive protein (CRP) concentration was determined by a radial immunodiffusion method in serum samples collected over a mean period of 19 months from a group of 17 patients with systemic lupus erythematosus (SLE). Thirty-two episodes of CRP elevation were detected. Twenty of these were associated with active SLE, and 9 with proven or possible infection or bone fracture. In 3 cases no explanation for CRP elevation was readily apparent. There were 8 instances of onset or exacerbation of lupus activity without accompanying CRP elevation. These data indicate that CRP elevation in the course of SLE is frequently associated with activation of lupus, and that detection of such elevation does not differentiate between lupus activity and infection. ( info) |
6/409. Pocket infection complicating inadvertent transarterial permanent pacing. Successful percutaneous explantation. This report describes a patient admitted for the treatment of a pocket infection occurring 5 months after a dual chamber pacemaker implantation. The ventricular lead had been inadvertently placed into the left ventricle through the arterial system. After careful examination using transesophageal echocardiography and left heart angiogram, successful percutaneous extraction was performed without complication. ( info) |
7/409. A heterozygous mutation of beta-actin associated with neutrophil dysfunction and recurrent infection. A human disorder caused by mutation in nonmuscle actin has not been reported. We report here a variant of nonmuscle actin in a female patient with recurrent infections, photosensitivity, and mental retardation. She also had abnormalities in neutrophil chemotaxis, superoxide production, and membrane potential response. Two-dimensional PAGE analysis of proteins from neutrophils and other cell types from this patient demonstrated a unique protein spot migrating at 42 kDa with pI shifted slightly to neutral relative to normal beta- and gamma-actin. digestion peptide mapping and Western blotting showed this spot to be an abnormal actin. A full-length cDNA library was constructed by using mRNA from patient's cells and cDNA encoding the mutant beta-actin molecule was identified by an in vitro translation method. Sequencing of the clones demonstrated a G-1174 to A substitution, predicting a glutamic acid-364 to lysine substitution in beta-actin and eliminating a HinfI DNase restriction site found in normal beta-actin sequence. By HinfI digestion and by sequencing, the mutation in one allele of patient's genomic dna was confirmed. Though no defect in cell-free polymerization of actin was detected, this defect lies in a domain important for binding to profilin and other actin-regulatory molecules. In fact, the mutant actin bound to profilin less efficiently than normal actin did. Heterozygous expression of mutant beta-actin in neutrophils and other cells of this patient may act in a dominant-negative fashion to adversely affect cellular activities dependent on the function of nonmuscle actin. ( info) |
8/409. Infected ovarian cyst in a homozygous beta-thalassemic patient. An infected ovarian cyst in a thalassemic patient is rarely reported. We describe the case of a 22-year old woman with splenectomized homozygous beta-thalassemia who developed high fever and was diagnosed as having an infected ovarian cyst. The mechanisms which beta-thalassemia might predispose to infection and considered to be immunocompromized are discussed. She was given an intravenous antibiotic regimen and the infected ovarian cyst was removed. The difficulties in the diagnosis of an infected ovarian cyst is because of its rarity and the paucity of information on it in the literature. Therefore, the triad of ovarian cyst, immunocompromized host, and signs of infection with failure to identify any other source of infection should raise the suspicion of an infected ovarian cyst. ( info) |
9/409. rett syndrome: photographic evidence of rapid regression. rett syndrome is known to occur in females, around the second year, with loss of hand use, onset of stereotypes and acquired microcephaly. Such regression is often very rapid, but this has never been documented. In one of our patients, photographs taken at different times clearly demonstrate the rapid progression of first symptoms. Moreover, in the present case, the occurrence of a febrile illness, which preceded the onset of the neurological picture, support the hypothesis that environmental factors may trigger the onset of rett syndrome in genetically predisposed subjects. ( info) |
| Using a computer database, we conducted a retrospective review of all ankle fractures treated at our institution from March 1985 to October 1996. Twenty-one patients with diabetes mellitus and isolated ankle fractures that were treated operatively met all inclusion criteria. Seven had insulin-dependent diabetes, and 14 had non-insulin-dependent diabetes. A randomly selected control group of 46 patients without diabetes who also underwent operative treatment of ankle fractures during this same time period were matched for age, sex, and fracture severity. The complication rate was 43% with 13 complications in nine patients with diabetes. There were seven (15.5%) complications in the control group. Complications in the diabetic group included seven infections (five deep, two superficial) and three losses of fixation. The complications were more severe in our diabetic population, requiring seven additional procedures including two below-knee amputations; a third patient refused an amputation. No additional procedures were required in our control group. All complications in our control group resolved with treatment. The relative risk for postoperative complications in patients with diabetes who sustained ankle fractures that were treated operatively was 2.76 times greater than the control group's (95% confidence interval, 1.57-3.97). ( info) |