Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 34-month-old girl with stage IV neuroblastoma who developed hives when parenteral nutrition (PN) containing amino acids, dextrose, electrolytes, minerals, vitamins, and trace elements was infused. Administration of diphenhydramine resulted in disappearance of the rash. Infusion of the PN solution without intravenous fat emulsion produced a similar rash with itching. The pediatric multiple vitamin (PMV) preparation was removed from the PN formula and the formula was infused without incident. The patient was maintained on PN and an oral vitamin supplement with no further complaints. Inadvertent administration of a PN solution containing PMV resulted in a recurrence of hives. Absence of any adverse reactions when the PMV preparation was removed from the PN solution and an allergic reaction when the multivitamin was added to the PN solution support the possibility that the allergic reaction was related to the infusion of the multiple vitamin preparation.
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PMID:Case report of an allergic reaction to parenteral nutrition in a pediatric patient. 213 46

During a 7 year period, 16 episodes of VM-26 (teniposide) hypersensitivity occurred in our Department of Pediatrics. Eight of these (50%) were observed in neuroblastoma patients, of whom a total of 22 children had been treated with VM-26. The predominant signs were facial edema, flushing, urticaria, bronchospasm, tachycardia, and hypotension. All children with hypersensitivity recovered, but four of them were critically ill. No risk factors were found. In order to elucidate the mechanism of the hypersensitivity episode further, and to identify a possible allergen, histamine release from basophil leukocytes was performed by use of a glass microfiber method. Blood samples from nine cases reacting to VM-26, eight controls (children exposed to VM-26 without any hypersensitivity reactions), and 12 healthy children without previous exposure were challenged with VM-26 alone and with its vehicle, cremaphor. In all samples, it was found that VM-26 degranulated basophils, whereas no histamine release was seen after challenge with cremaphor. The reaction was dose-dependent, and not IgE-mediated, since IgE depletion of the cells did not abolish histamine release after VM-26 challenge.
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PMID:VM-26 (teniposide)-induced hypersensitivity and degranulation of basophils in children. 246 73

The murine IgG3 monoclonal antibody (MoAb) 3F8, specific for the ganglioside GD2, activates human complement, is active in antibody-dependent cell-mediated cytotoxicity (ADCC), and can target specifically to human neuroblastoma in patients with metastatic disease. In a phase I study, 3F8 was administered intravenously (IV) to 17 patients with metastatic GD2 positive neuroblastoma or malignant melanoma at doses of 5, 20, 50, and 100 mg/m2. Serum 3F8 levels achieved were proportional to the dose of 3F8 infused. However, serum antimouse antibody levels did not increase with the amount of 3F8 administered. Toxicities included pain, hypertension, urticaria, and complement depletion. All acute side effects were controllable with symptomatic therapy. No long-term side effects were detected in patients observed for more than 14 months. None of the 17 patients received any antitumor therapy postantibody treatment. Antitumor responses occurred in seven of 17 patients. These ranged from complete clinical remissions to mixed responses. The murine monoclonal antibody (MoAb) 3F8 has clinical utility for the diagnosis and therapy of neuroblastoma and melanoma.
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PMID:Ganglioside GD2 specific monoclonal antibody 3F8: a phase I study in patients with neuroblastoma and malignant melanoma. 154 29

Cefotiam (CTM) was evaluated for its safety and efficacy in children. Twenty-six patients were treated with 40 to 200 mg/kg per day of CTM by intravenous administrations. The diagnosis of the patients were acute pharyngitis (2), acute bronchitis (1), pneumonia (4), empyema (2), urinary tract infection (2), typhoid fever (1), acute enterocolitis (2), partially-treated purulent meningitis (1), and suspected septicemia in neuroblastoma (1); and the remaining ten patients were considered to have nonbacterial infections. The pathogens recovered were Streptococcus pyogenes (1), Streptococcus pneumoniae (1), Staphylococcus aureus (4), Haemophilus influenzae (4), Escherichia coli (1), enteropathogenic Escherichia coli (1), Salmonella typhi (1), and Campylobacter jejuni (1). All but two patients of bacterial infections were cured after the CTM therapy, and the rate of efficacy was 87.5%. Diarrhea (3), urticaria (1), transient elevation of GOT and GPT (1), and transient eosinophilia (3) were found to be associated with the CTM therapy. However, no severe adverse reactions were encountered. Half life of the serum CTM level was 0.93 +/- 0.13 hours, and excretion into the urine was rapid. CSF concentration obtained 1 hour after an intravenous injection of 21 mg/kg of CTM in a case with inflamed meninges was 1.5 mcg/ml, and the CSF/serum ratio was 9.0%. From these data, CTM appears to be a safe and effective antibiotic when used in children with susceptible bacterial infections.
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PMID:[Clinical evaluation of cefotiam therapy in children (author's transl)]. 627 Apr 13

9 patients with stage IV neuroblastoma were treated with 19 courses of human/mouse chimeric monoclonal antiganglioside GD2 antibody ch14.18 at dose levels of 30, 40 and 50 mg/m2/day for 5 days per course. The maximum tolerated dose (MTD) per injection was 50 mg/m2/day. 7 patients received more than one course of treatment, and none revealed any human anti-mouse antibody (HAMA) response. Clinical side-effects of patients treated with ch14.18 were abdominal and joint pains, pruritus and urticaria. One patient presented with a transient pupillatonia, while 2 others showed a unilateral atrophy of the optical nerve that was probably attributable to prior therapies. A complete remission was seen in 2 patients, partial remission in 2 patients, a minor response in 1 patient and stable disease in 1 patient. 3 patients showed tumour progression. Thus, our results indicate that treatment with chimeric MAb ch14.18 can elicit some complete and partial tumour responses in neuroblastoma patients.
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PMID:A phase I study of human/mouse chimeric antiganglioside GD2 antibody ch14.18 in patients with neuroblastoma. 771 35

3F8 is an IgG3 murine monoclonal antibody directed against the ganglioside GD2. In a phase II study, 3F8 was administered i.v. to 16 patients (pts) who had stage 4 neuroblastoma. Response was seen in bony lesions (2 of 7 pts) and marrow (3 of 8 pts). Acute toxicities of pain, fever, urticaria, hypertension, hypotension and anaphylactoid reactions were self-limited and manageable. Three pts are long-term survivors between 79-130+ months after 3F8 treatment without additional systemic therapy and no delayed neurological complications. The potential benefits of 3F8 when added to chemoradio-therapy warrant further investigation.
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PMID:3F8 monoclonal antibody treatment of patients with stage 4 neuroblastoma: a phase II study. 959 90

Familial cold autoinflammatory syndrome (FCAS, MIM 120100), commonly known as familial cold urticaria (FCU), is an autosomal-dominant systemic inflammatory disease characterized by intermittent episodes of rash, arthralgia, fever and conjunctivitis after generalized exposure to cold. FCAS was previously mapped to a 10-cM region on chromosome 1q44 (refs. 5,6). Muckle-Wells syndrome (MWS; MIM 191900), which also maps to chromosome 1q44, is an autosomal-dominant periodic fever syndrome with a similar phenotype except that symptoms are not precipitated by cold exposure and that sensorineural hearing loss is frequently also present. To identify the genes for FCAS and MWS, we screened exons in the 1q44 region for mutations by direct sequencing of genomic DNA from affected individuals and controls. This resulted in the identification of four distinct mutations in a gene that segregated with the disorder in three families with FCAS and one family with MWS. This gene, called CIAS1, is expressed in peripheral blood leukocytes and encodes a protein with a pyrin domain, a nucleotide-binding site (NBS, NACHT subfamily) domain and a leucine-rich repeat (LRR) motif region, suggesting a role in the regulation of inflammation and apoptosis.
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PMID:Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle-Wells syndrome. 1168 85

The PYRIN domain is a recently identified protein-protein interaction domain that is found at the N terminus of several proteins thought to function in apoptotic and inflammatory signaling pathways. We report here that PYPAF1 (PYRIN-containing Apaf1-like protein 1) is a novel PYRIN-containing signaling protein that belongs to the nucleotide-binding site/leucine-rich repeat (NBS/LRR) family of signaling proteins. The expression of PYPAF1 is highly restricted to immune cells, and its gene maps to chromosome 1q44, a locus that is associated with the rare inflammatory diseases Muckle-Wells syndrome and familial cold urticaria. To identify downstream signaling partners of PYPAF1, we performed a mammalian two-hybrid screen and identified ASC as a PYRIN-containing protein that interacts selectively with the PYRIN domain of PYPAF1. When expressed in cells, ASC recruits PYPAF1 to distinct cytoplasmic loci and induces the activation of NF-kappaB. Furthermore, coexpression of PYPAF1 with ASC results in a potent synergistic activation of NF-kappaB. These findings suggest that PYPAF1 and ASC function as upstream activators of NF-kappaB signaling.
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PMID:PYPAF1, a PYRIN-containing Apaf1-like protein that assembles with ASC and regulates activation of NF-kappa B. 1178 56