Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The study involves 10 children with primary immunologic deficiency of the humoral and cellular immunity diagnosed on the basis of the clinical symptoms of the disease and immunologic investigation. In 7 children, out of whome three were infants, suffering of recurrent respiratory tract infections, examinations revealed either absence or deficiency of the IgA in the serum and saliva. 2 children presented a classical picture of the Wiskott-Aldrich's syndrome followed by eczema, recurrent infections and trombocytopenia. Having studied the immunologic status in these two children in vitro and in vivo, the authors established deficiency in the humoral and cellular immunity. In an infant aged three months, with diagnosed histiocytosis X after histologic examination of the skin, the authors had examined the function of the T and B cells after which it was concluded that it was a case of rare form of the combined primary immunodeficiency.
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PMID:[Congenital deficiency of humoral and cellular immunity]. 30 54

Accumulated lines of evidence indicate that inactivated probiotics could have beneficial effects similar to those of live probiotics. Two strains of disrupted, cobalt-enriched, lactic acid bacteria (Lactobacillus acidophilus and Lactobacillus casei) and a disrupted fungal mycelium (Scytalidium acidophilum) were spray-mixed onto a mash basal feed, in 2 concentrations, prior to pelleting. The effects of these probiotics on production performance and immune response in broiler chickens were investigated. The production parameters, including BW, feed intake (FI), BW gain (BWG), and feed conversion ratio (FCR), were monitored weekly during a 6-wk trial. The immune response was evaluated by immunizing the birds with the antigen keyhole limpet hemocyanin (KLH) followed by a serological assay to measure blood IgA and IgG titers. Some of the production parameters were significantly improved by low L. casei (LCL; for BW and BWG), high L. acidophilus (LAH; for BW and BWG), and high fungal (FH; for BW, BWG, and FI) in comparison with the nonadditive control (NC-). However, these 3 treatments (LCL, LAH, and FH) did not enhance the measured immune responses. Instead, the titers of serum KLH-specific IgA in high L. casei (LCH) and low L. acidophilus (LAL) were significantly higher than those of NC-, 10 d after immunization. None of the probiotic treatments increased the titer of KLH-specific IgG in blood. Our results indicate that disrupted and cobalt-enriched L. acidophilus or L. casei was able to enhance production performance of broiler chickens. The fungal mycelium, S. acidophilum, when used at a high concentration, also demonstrated its potential for the first time to be used as a probiotic. In addition, the optimal concentration for administering probiotics is strain dependent. A higher dose does not always result in a better performance.
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PMID:Effects of Lactobacilli and an acidophilic fungus on the production performance and immune responses in broiler chickens. 1514 37

Tfifha M, Kamoun T, Mama N, Mestiri S, Hassayoun S, Zouari N, Jemni H, Abroug S. Childhood sclerosing cholangitis associations in a Tunisian tertiary care hospital: a many-faceted disease. Turk J Pediatr 2019; 61: 905-914. Sclerosing cholangitis (SC) is a liver disorder affecting children and adults, causing chronic cholestasis and secondary biliary cirrhosis. The purpose of this study was to present different associated diseases to SC in a Tunisian tertiary care hospital. Six patients were identified with SC associated with other diseases, four males and two females. The first symptom was liver enlargement in all cases with abnormal liver biochemistry. A moderate increase in AST and ALT levels was registered in all cases with moderate cholestasis in 4 patients. Three of them presented an auto-immune condition. Two patients were diagnosed with auto-immune hepatitis prior to SC and Crohn disease in only one patient. One developed linear IgA bullous dermatosis. Three patients were diagnosed with Multisystemic Langerhans Cell Histiocytosis (LCH). The primary site of LCH was the liver associated secondary to insipidus diabetes (one case), mastoiditis (two cases) and chest localization (one case). The outcome of those patients was variable with poor prognosis especially for SC secondary to LCH. No patient underwent liver transplantation. SC is a rare disorder with variable clinical presentations. To our knowledge, this is the first report of this condition in Tunisian and North African children. Diagnosis and treatment of SC and its associations remains a challenge, especially because there is still no effective medical therapy aimed at preventing disease progression. Pediatric liver transplantation is the only life-extending therapeutic alternative for patients with end-stage liver failure. Liver transplantation has not been performed on young children in our country.
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PMID:Childhood sclerosing cholangitis associations in a Tunisian tertiary care hospital: a many-faceted disease. 3213 85

Many pediatric skin conditions can be safely monitored with minimal intervention, but certain skin conditions are emergent and require immediate attention and proper assessment of the neonate, infant, or child. We review the following pediatric dermatology emergencies so that clinicians can detect and accurately diagnose these conditions to avoid delayed treatment and considerable morbidity and mortality if missed: staphylococcal scalded skin syndrome (SSSS), impetigo, eczema herpeticum (EH), Langerhans cell histiocytosis (LCH), infantile hemangioma (IH), and IgA vasculitis.
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PMID:Pediatric dermatology emergencies. 3235 37