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)

16 unselected patients with advanced neuroblastoma were given high-dose consolidation chemotherapy with vincristine, melphalan, etoposide and carboplatin over 5 h followed by autologous bone marrow rescue. 3 patients died from treatment-related toxicity, 2 from disease, 1 is alive with disease and 10 are alive and disease-free a median of 12.5 months (range 2-38 months) after bone marrow rescue. All had bone marrow toxicity, most mucositis and 6 had seizures. Renal failure was unexpectedly severe. In the last 3 patients, administration of carboplatin was delayed by 18 h in an attempt to reduce renal damage. The results show that this regimen produces significant morbidity and has a high mortality. Although the overall outcome is encouraging, too few patients have been studied to gauge its efficacy. Whether such aggressive consolidation is necessary in heavily pretreated children with neuroblastoma remains unknown.
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PMID:Toxicity of single-day high-dose vincristine, melphalan, etoposide and carboplatin consolidation with autologous bone marrow rescue in advanced neuroblastoma. 151 36

Fifty-one children, aged from 15 months to 13 years 5 months with metastatic neuroblastoma presenting sequentially at the participating institutions received four 3 to 4 weekly courses of high dose multiagent chemotherapy. High dose cisplatin (200 mg m-2) combined with etoposide (500 mg m-2), HIPE, was alternated with ifosfamide (9 g m-2), vincristine (1.5 mg m-2), and adriamycin (60 mg m-1), IVAd. Disease status was re-evaluated 3 to 4 weeks after the fourth course and the response classified according to the International Neuroblastoma Response Criteria (INRC). The overall response rate in evaluable patients was 55% and response rates by site were: bone marrow 67% (complete response 47%); bone scan 68%; primary tumour 61%, and urinary catecholamine metabolites (VMA/HVA) 95%. Serial 51Cr EDTA renal clearance studies showed a glomerular filtration rate (GFR) decline in 40% of patients but in only seven cases to below 50% of the pretreatment value. There was no instance of renal failure during induction, though two patients developed severe renal failure following 'megatherapy' given to consolidate remission. Serial audiometry showed a significant decline in hearing at frequencies above 2,000 Hz in 37% of children but at or below 2,000 Hz in only 17%. Neutropenia and thrombocytopenia were severe and intravenous antibiotics were required after 30% of courses. Each of two treatment-related deaths occurred during pancytopenia following courses of IVAd. Complete, or greater than 90%, removal of primary site tumour was possible in 70% of cases following this induction regimen and 75% of patients proceeded to elective megatherapy within a median time of 24 weeks after diagnosis. This short intensive induction programme is highly effective at achieving cytoreduction, enabling early surgery and early megatherapy procedures. It is, however, too early to draw firm conclusions about the impact of this approach to treatment on the cure rate.
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PMID:Short duration, high dose, alternating chemotherapy in metastatic neuroblastoma. (ENSG 3C induction regimen). The European Neuroblastoma Study Group. 238 51

Chromogranin A is a useful probe of neuroendocrine neoplasia in humans. Here we optimize a rapid, sensitive radioimmunoassay modification for detecting chromogranin A in humans and other species. The site of chromogranin A circulation is the acellular plasma; platelets contain no chromogranin A immunoreactivity. The immunoreactivity in plasma is stable to repeated freezing and thawing, prolonged incubation at 37 degrees C, and lyophilization. Venipuncture alone resulted in modest (+ 12%, P less than 0.03) increase in chromogranin A in plasma. Several classic neuroendocrine neoplasia-pheochromocytoma, carcinoid tumor, neuroblastoma, and (vasoactive intestinal polypeptide)oma-produce markedly increased chromogranin A in plasma. By contrast, subjects with malignant melanoma, renal cell carcinoma, and thymoma all had normal values for chromogranin A. Hypersecretion of human choriogonadotropin beta subunit from both malignant (choriocarcinoma) and normal (placenta) syncytiotrophoblast cells was unaccompanied by an increase in chromogranin A, a dissociation compatible with the lack of granular storage and release of syncytiotrophoblastic peptide hormones. Both hepatic and renal failure resulted in increased chromogranin A in plasma, with renal failure leading to concentrations otherwise seen only in neuroendocrine neoplasia. These observations refine the diagnostic specificity of chromogranin A in plasma.
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PMID:Rapid radioimmunoassay of circulating chromogranin A: in vitro stability, exploration of the neuroendocrine character of neoplasia, and assessment of the effects of organ failure. 254 34

In summary, carcinoma is the most frequent cancer that metastasizes to the skin; lung cancer in men and breast cancer in women. Clinically distinctive patterns of cutaneous metastasis of epithelial origin include alopecia neoplastica, pulsatile nodules, Sister Mary Joseph's nodules, morpheaform, and cellulitis-like lesions. Biopsying these lesions reveals adenocarcinoma, squamous cell carcinoma, or anaplastic carcinoma. The type of histologic pattern seen can be a clue to the organ of origin giving rise to the cutaneous metastasis. Skin that is damaged allows for circulating malignant cells, often of epithelial or leukemic origin, to lodge and proliferate locally (inflammatory oncotaxis). The commonest form of leukemia to affect the skin of elderly males is chronic lymphocytic leukemia. However, when leukemia involves the mucous membranes, acute myeloid leukemia (acute monocytic and acute myelomonocytic leukemia) is the most likely diagnosis. When papules, nodules, or plaques develop on the head, neck, or torso in a middle-aged male accompanied by lymphadenopathy, there must be a high index of suspicion that these lesions are metastatic lymphomatous deposits. Definitive histologic diagnosis on a skin biopsy specimen is difficult. In this situation, it is best to rely on histologic patterns seen in lymphoid tissue along with cellular marker studies. An elderly patient having bone pain, anemia, elevated blood calcium level, and renal failure along with purplish or skin-colored nodules and plaques on the trunk has a good chance of having multiple myeloma. Biopsying these lesions is most certain to reveal atypical plasma cells, and blood immunoelectrophoresis will demonstrate characteristic monoclonal gammopathy. There are two malignancies seen in children under 3 years of age that often times affect the skin in a characteristic fashion. Letterer-Siwe disease, which is distinguished from other histocytic disorders by its cell of origin, the Langerhans cell, clinically shows maculopapular and erosive lesions distributed in a seborrheic pattern. Neuroblastoma derived from cells of the neural crest demonstrates clinically widespread bluish papulonodules. Kaposi's sarcoma, a multifocal vascular malignancy, has a wide spectrum of clinical expression. Those patients who are immunocompromised secondary to concomitant disease or immunosuppressive therapy are more susceptible to a disseminated fulminant course accompanied by opportunistic infection. In conclusion, although specific signs of internal malignancy are less common than nonspecific ones, they are just as important; if the clinician managing the cancer patient is familiar with these clues to internal disease, proper patient management will ensue.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Specific cutaneous manifestations of internal malignancy. 307 47

A 3,900 g full-term male newborn had to be operated on for suspected intraabdominal bleeding due to birth injury. Intraoperatively, a tennis-ball sized, ruptured tumor between right kidney and liver was found to be the source hemorrhage. The tumor was excised in toto. The newborn died 25 hours after the end of operation from protracted shock and renal failure. Post mortem examination did not reveal further malformations or metastases. Histological diagnosis: congenital neuroblastoma of the adrenal gland. Improved antenatal ultrasound diagnosis followed by cesarian section could help to improve the prognosis in such cases in future.
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PMID:[A case of ruptured congenital neuroblastoma caused by birth injury]. 388 96

We have reviewed the clinical course of 30 pediatric bone marrow transplant (BMT) recipients requiring dialysis for acute renal failure early after BMT. Patients requiring dialysis were not significantly different from the general pediatric BMT population except for: (1) a greater proportion of neuroblastoma patients in the dialysis group, and (2) fewer autologous and more unrelated BMT donors in the dialysis group. Twenty-three patients (77%) died without recovering renal function 1-72 days (mean 12 days) after dialysis was begun. Sepsis was the most commonly cited cause of renal failure and death in these patients. Seven patients (23%) recovered sufficient renal function to stop dialysis; all long-term survivors were in this group. Factors at the onset of dialysis associated with persistent renal failure were weight gain of > or = 10% of baseline body weight, requirement of three or more drugs for blood pressure support and hyperbilirubinemia. Although acute renal failure requiring dialysis is an ominous development following BMT, recovery of renal function is possible with aggressive supportive care.
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PMID:Outcome of dialysis for acute renal failure in pediatric bone marrow transplant patients. 805 14

Uremic encephalopathy is a complication of renal failure that reflects stresses exerted by as yet poorly defined uremic toxins. All cells respond to stresses by undergoing the "heat shock" response. Although urea kinetics and creatinine concentration are routinely used to assess dialysis adequacy, the roles of urea and creatinine as uremic toxins remain controversial. To investigate their potential roles in uremic encephalopathy, cultured human neuroblastoma cells (SK-N-SH) were exposed to 0.5 to 14 mg/dL creatinine, or to 20 to 200 mg/dL urea, or to mannitol, NaCl, or glycerol at equivalent osmolalities for 30 min to 48 h, and the induction of Hsp72 (heat shock) protein was used as a marker of cell stress. Although creatinine failed to elicit a heat shock response, urea in clinically relevant concentrations (40 to 200 mg/dL) induced it at 30 min. The response peaked at 10 h and returned to zero by 48 h. Cells exposed to equivalent osmolalities of mannitol, NaCl, or glycerol failed to exhibit this response. Protein extracts from cells exposed to urea showed significant carbamylation that increased as a function of time. These results demonstrate: (1) that urea is neurotoxic in vitro and that creatinine is not: (2) that the insult urea causes is not simply the result of hypertonicity; but rather (3) that urea, via breakdown to cyanate and ammonium ions, may cause cell stress because of its ability to cause carbamylation of cellular proteins. The cells attenuation of the heat shock response after 10 h of exposure to urea suggests that they can adapt to the presence of urea or carbamylation. This may explain, in part, why the same degree of azotemia causes fewer neurological symptoms in patients with chronic as opposed to acute renal failure.
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PMID:Urea induces the heat shock response in human neuroblastoma cells. 878 97

Neuropeptide Y (NPY) is a peptide hormone that is expressed, stored, and released in sympathetic neurones together with noradrenaline. Elevated plasma concentrations of NPY have been reported in patients with neural crest tumors (neuroblastoma, pheochromocytoma) and following exercise. We studied plasma concentrations of NPY in children and adults with chronic and terminal renal failure and compared them with those in healthy controls. Neuropeptide Y was significantly higher in children and adolescents receiving peritoneal dialysis (5.3 +/- 2.8 pmol/L; n = 11 [mean +/- SD]) or hemodialysis (5.4 +/- 2.1 pmol/L; n = 14) than in healthy children (2.3 +/- 0.9 pmol/L; n = 19) or pediatric patients with impaired renal function who are not receiving dialysis (2.7 +/- 0.6 pmol/L; n = 8; mean glomerular filtration rate, 41 mL/min x 1.73 m2). There was a small but insignificant negative correlation between glomerular filtration rate and NPY concentrations in children with impaired renal function (r = 0.49; P = 0.25). In healthy adults, NPY concentration was similar to that in healthy children (1.8 +/- 1.0 pmol/L; n = 13), and it was significantly elevated in adults receiving hemodialysis (5.9 +/- 1.7 pmol/L; n = 16). No significant changes in NPY concentrations were found before and after hemodialysis in pediatric or adult patients. We conclude that plasma concentrations of NPY are elevated in patients with chronic renal failure who are receiving either peritoneal or hemodialysis, but not in patients with moderately impaired renal function. Whether elevated NPY concentration indicates increased sympathetic activity or is caused by reduced NPY clearance remains to be shown.
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PMID:Elevated plasma concentrations of neuropeptide Y in children and adults with chronic and terminal renal failure. 921 97

Leukemia, aplastic anemia, sickle cell anemia, hemophilia, lymphoma, neuroblastoma, rhabdomyosarcoma, renal failure, cystic fibrosis, scoliosis, diabetes, and asthma. These are only a few of the many chronic diseases or handicapping conditions confronting children today. Little attention has been paid to children's emotional reactions to illness and hospitalization, important facets of the healing process. In the first part of this paper, children's characteristic emotional reactions to hospitalization are discussed within a developmental framework. Next, some of the emotional reactions elicited in hospital staff and parents by seriously ill children are discussed. It is hoped that an elucidation of the types of feelings hospitalized children experience, and the kinds of emotional responses they elicit in adult caretakers, can lead to more sensitive and effective child care-giving in hospital settings.
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PMID:Coping with feelings: seriously ill children, their families, and hospital staff. 1026 27

A prospective study was performed on 20 bacteriologically proven pediatric cases of severe shigellosis admitted to the Department of Pediatrics, Chulalongkorn Hospital during March 1989 to March 1990. Fourteen patients were male and six were female. Shigella B was found in 85% and Shigella D in 15% of cases. The major indications for admission were convulsions and dehydration. Fifteen per cent of cases had underlying malignancies and 42.1% had malnutrition. Most patients had a peak of fever between 39.5 and 40.5 degrees C, serum sodium between 128-144 mEq/l. Mild acidosis was detected in 45% and moderate acidosis in 30% of cases. There were no statistical differences in peak of fever and serum sodium between patients who had convulsion and who did not. Shigellemia was found in one case who also had underlying neuroblastoma. One patient died due to necrotizing enterocolitis, septic shock and renal failure. Most of the organisms found resisted to ampicillin and trimethoprim-sulfamethoxazole (TMP-SMX). However, TMP-SMX was prescribed in most immunocompetent patients and they recovered well. All of three patients with underlying malignancy responded well to ceftriaxone.
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PMID:Severe shigellosis in childhood. 1043 56


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