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

Aberrations in the metabolic pathways of catecholamines in patients with neural crest tumors result in characteristic urinary excretion patterns of their catabolites. Tumors such as pheochromocytoma, neuroblastoma and ganglioneuroma usually defy clinical diagnosis because of their rarity, small size, intraabdominal position and clinical symptoms similar to those of essential hypertension. Quantitative determination of catecholamine metabolites such as vanillylmandelic acid (VMA) and 3-methoxy-4-hydroxyphenylethyleneglycol (MHPG) offers possibilities for reliable confirmation of diagnosis. However, previous techniques for the assessment of catabolite levels suffered from inadequate sensitivity, reproducibility or specificity, which seriously diminished their usefulness as biochemical determinants in the prognosis of these life-threatening tumors. Reported in this paper is the analysis of urinary levels of VMA and MHPG using reversed-phase high-performance liquid chromatography with electrochemical and sectrophotometric detection. We present the excretion patterns showing these metabolites in 15 control subjects, 15 patients with pheochromocytoma and 5 patients with neuroblastoma.
 ...
PMID:Diagnosis of neural crest tumors by reversed-phase high-performance liquid chromatographic determination of urinary catecholamine metabolites. 54 40

1. We investigated the usefulness of neuropeptide Y as a plasma marker for phaeochromocytoma, ganglioneuroblastoma and neuroblastoma using a simple and highly sensitive r.i.a. for human neuropeptide Y. 2. Plasma immunoreactive neuropeptide Y concentrations were measured without extraction in plasma samples (100 microliters) from patients with various diseases. 3. The plasma immunoreactive neuropeptide Y concentration in patients with phaeochromocytoma (172.3 +/- 132.4 pmol/l, mean +/- SD, n = 23) was significantly higher than that in healthy adult subjects (40.1 +/- 10.1 pmol/l, n = 40, P < 0.0001). The plasma immunoreactive neuropeptide Y concentrations in patients with ganglioneuroblastoma (590.7 +/- 563.6 pmol/l, n = 6) and patients with neuroblastoma (566.9 +/- 524.4 pmol/l, n = 15) were significantly higher than those in control children (1-9 years old, 82.2 +/- 39.9 pmol/l, n = 72, P < 0.0001). 4. The plasma immunoreactive neuropeptide Y concentration in patients with essential hypertension (34.0 +/- 3.7 pmol/l, n = 18) was within the normal range, but in patients with chronic renal failure undergoing maintenance haemodialysis (192.1 +/- 68.0 pmol/l, n = 25) and in non-dialysed patients with chronic renal failure (85.1 +/- 23.1 pmol/l, n = 7) it was significantly higher than that in healthy adult subjects (P < 0.0001). 5. Eighty-seven per cent of the patients with phaeochromocytoma, 67% of the patients with ganglioneuroblastoma and 80% of the patients with neuroblastoma showed plasma immunoreactive neuropeptide Y concentrations higher than the upper limits in the control subjects [62 pmol/l (adult) and 160 pmol/l (children)].(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Neuropeptide Y as a plasma marker for phaeochromocytoma, ganglioneuroblastoma and neuroblastoma. 132 37

Aromatic-L-aminoacid (dopa) decarboxylase (ALAAD) was determined in human plasma by its ability to form dopamine from the substrate 3,4-dihydroxyphenylalanine in the presence of pyridoxal-5-phosphate as cofactor. Dopamine formed was quantitated by high performance liquid chromatography with electrochemical detection. A preincubation step of plasma with the cofactor and dithioerythritol was necessary to obtain optimal reaction conditions. The assay method showed good linearity and reproducibility. The inhibition pattern of the therapeutically used peripheral dopa decarboxylase inhibitors, carbidopa and benserazide, was studied and appeared to be dependent on whether the inhibitor was added before or after the preincubation step. Mean levels in 40 control subjects, in 40 patients with essential hypertension and in 15 patients with phaeochromocytoma, were 34.6 (SD 12.1), 28.5 (SD 10.9) and 34.7 (SD 18.4) mU/l respectively. In the patients with essential hypertension the enzyme level decreased with age (p less than 0.05). Very high levels were found in plasma of two patients with metastatic phaeochromocytoma and in two patients with untreated neuroblastoma, but not in two patients with neuroblastoma after chemotherapy. The method described can be used for measuring uninhibited ALAAD activity in patients treated with benserazide, as well as for measuring total, i.e. the sum of inhibited and uninhibited, ALAAD activity in patients treated with carbidopa.
 ...
PMID:Determination of aromatic-L-amino acid decarboxylase in human plasma. 376 7

The term "Western diseases" refers to those conditions that are rare or absent in underdeveloped areas of the Third World and increase in frequency with adoptions of Western customs. In adults, they include such common conditions as coronary artery disease, essential hypertension, appendicitis, cholesterol gall stones, and colon cancer. The best examples of Western diseases in the pediatric population are asthma, allergies, appendicitis, and inflammatory bowel disease. Limited data from sub-Saharan Africa suggest other pediatric surgical conditions may fall into this category, including hypertrophic pyloric stenosis, gastroesophageal reflux, perirectal abscess, anal fissure, gastroschesis, and neuroblastoma. Existing theories for the origins of Western diseases have postulated a role for decreased dietary fiber, improved hygiene, fetal programming, and a protective effect of tropical enteropathy. How these factors might relate to the rise of appendicitis, inflammatory bowel disease, and possibly other common pediatric surgical diseases in industrialized societies remains poorly understood. Further research is needed to better define geographical differences in common pediatric surgical conditions and to investigate how genetic and environmental factors interact to modify risk of disease. Understanding the molecular mechanisms that give rise to Western diseases could lead to new therapeutic and prevention strategies for some of the most common pediatric surgical conditions in industrialized countries.
 ...
PMID:Western diseases: current concepts and implications for pediatric surgery research and practice. 1808 4