1/765. 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.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
2/765. cytomegalovirus associated neonatal pneumonia and Wilson-Mikity syndrome: a causal relationship?lung injury caused by intrauterine inflammation has recently been strongly implicated in the pathogenesis of Wilson-Mikity syndrome (WMS). This article supports this theory by suggesting a causative role of intrauterine cytomegalovirus (CMV) infection for the development of WMS. A male premature infant, born at 33 weeks of gestational age, developed chronic lung disease compatible with WMS and diagnostic evaluation was positive for CMV infection. High-resolution computed tomography scan and lung histology revealed typical features of WMS in association with signs of interstitial pneumonia. CMV was found in urine, breastmilk, bronchoalveolar lavage material and lung tissue from open lung biopsy. Follow-up after treatment with ganciclovir and steroids showed resolving lung disease at the age of 6, 10 and 16 months, with lung function signs of mild obstruction. Assuming that a chance coexistence of cytomegalovirus pneumonia and Wilson-Mikity syndrome is rather unlikely, it is possible that intrauterine cytomegalovirus infection caused a pattern of lung injury consistent with Wilson-Mikity syndrome. Further cases of Wilson-Mikity syndrome should be investigated as to a possible role of congenital infection.- - - - - - - - - - ranking = 4keywords = infection (Clic here for more details about this article) |
3/765. Congenital hydranencephalic-hydrocephalic syndrome associated with mitochondrial dysfunction.We report the case of a 3-year-old girl, the only child of a nonconsanguineous couple without relevant antecedents, who was born with hydranencephalic-hydrocephalic syndrome diagnosed by ultrasonography at gestation week 28, and who was treated during the neonatal period by implantation of a ventriculoperitoneal shunt. She showed severe mental retardation, and died at age 4 years following an acute respiratory infection. Due to persistently high lactic acid levels in blood, muscle and skin biopsies were taken. Analysis of muscle biopsies revealed microscopic and ultrastructural alterations typical of mitochondrial disorders, and low levels of complexes III and IV of the mitochondrial respiratory chain. The enzymes of the pyruvate dehydrogenase complex showed normal activities in cultured skin fibroblasts. These findings raise the possibility that at least some cases of congenital hydranencephalic-hydrocephalic syndrome may be due to alterations in the mitochondrial respiratory chain.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
4/765. Recurrent anterior uveitis associated with streptococcal pharyngitis in a patient with a history of poststreptococcal syndrome.PURPOSE: To provide additional evidence that anterior uveitis can be a manifestation of poststreptococcal syndrome. METHOD: A case report providing follow-up information on a previously described patient. RESULTS: An adolescent girl in whom anterior uveitis was the only manifestation of poststreptococcal syndrome subsequently developed recurrent anterior uveitis after another episode of streptococcal pharyngitis. CONCLUSION: Anterior uveitis can recur in a manner similar to other manifestations of poststreptococcal syndrome after reinfection with group A streptococci.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
5/765. The Ask-Upmark kidney: a form of ascending pyelonephritis?The case is presented of a young girl with recurrent urinary tract infection and vesico-ureteric reflux who developed a small scarred kidney and subsequently, hypertension. Pathologically, the renal changes were compatible with those of an Ask-Upmark kidney. The pathogenesis of the Ask-Upmark kidney is discussed. It is postulated that the lesion is not necessarily of congenital origin but may well be related to infection and intrarenal reflux, it is concluded that long-term follow-up of a young patient with a scarred kidney is indicated.- - - - - - - - - - ranking = 2keywords = infection (Clic here for more details about this article) |
6/765. prenatal diagnosis of congenital varicella syndrome and detection of varicella-zoster virus in the fetus: a case report.Varicella syndrome (VS) specific malformations were sonographically seen at 22 weeks and 3 days of gestation. Fetal infection was demonstrated by detection of varicella-zoster virus (VZV) dna in fetal blood and amniotic fluid by polymerase chain reaction (PCR). Following therapeutic abortion, fetal infection was confirmed by detection of VZV dna in several fetal tissues and placenta, and by histopathological findings like miliary calcified necroses in fetal organs.- - - - - - - - - - ranking = 2keywords = infection (Clic here for more details about this article) |
7/765. Enteroviral meningoencephalitis as a complication of X-linked hyper IgM syndrome.We describe 5 children from 2 families with mutations in the cd40 ligand (CD40L) gene leading to absent expression of CD40L on activated CD4 cells. All subjects presented with interstitial pneumonia with low serum IgG and normal serum IgM. One child had normal and one child had elevated serum IgA. Four had confirmed pneumocystis carinii pneumonia. In spite of intravenous immunoglobulin treatment yielding therapeutic serum immunoglobulin levels, 3 children had enteroviral encephalitis. When assessed by flow cytometry, the 3 surviving affected male children had absent CD40L expression on activated CD4( ) T cells. The affected children from both families were shown to have the same single nucleotide insertion (codon 131) resulting in frameshift and early termination within exon 4 (extracellular domain). This observation demonstrates that persistent enteroviral infection is not only observed in X-linked agammaglobulinemia but may also occur in patients with X-linked hyper IgM syndrome.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
8/765. The spectrum of reactive hemophagocytic syndrome in systemic lupus erythematosus.We address the relationship between reactive hemophagocytic syndrome (RHS), systemic lupus erythematosus (SLE) activity, and treatment in 4 female patients with SLE. Febrile pancytopenia was related to cytologically proven RHS in all patients. Followup was 45 /-7 months from RHS onset. No causal infection could be identified. Outcome could be classified as: (1) RHS onset during a SLE flare and complete efficacy of high dose steroids; (2) death despite therapy for concomitant severe RHS and active SLE; (3) severe RHS in inactive SLE under immunosuppressants, with remission after steroid tapering and cyclophosphamide withdrawal. Three patients were treated with intravenous IgG. We conclude that (1) when SLE is active, RHS should be considered a specific manifestation and treated with steroids; (2) RHS occurring in otherwise inactive SLE might be related to iatrogenic immunosuppression; (3) intravenous IgG treatment might be indicated in both situations.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
9/765. A new syndrome of spondyloepimetaphyseal dysplasia, eczema and hypogammaglobulinaemia.We describe a female infant with a combination of very short stature, severe eczema and igg deficiency causing recurrent infections in infancy. The radiological features of this condition are presented in the neonatal period, at the age of 5 months and at 2 years and 6 months. We propose that this condition is a previously undescribed type of spondyloepimetaphyseal dysplasia.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
10/765. Polyarthralgia-arthritis syndrome induced by low doses of rifabutin.We describe 2 cases of polyarthralgia-arthritis syndrome induced by rifabutin, an effective treatment for infections of mycobacterium avium intracellulare complex. This syndrome has been reported with doses higher than 1 g per day when rifabutin is given in monotherapy. But our cases were treated with low doses, 300-450 mg per day, in combination with clarithromycin. The plasma concentration of rifabutin has been shown to be increased by clarithromycin, suggesting that co-prescription of clarithromycin could lead to development of rifabutin induced polyarthralgia-arthritis syndrome.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
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