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)

Of our 174 neuroblastoma patients, 11 were classified as Stage IV-S. The primary tumor is usually small and there is widespread disease in the liver, skin, bone marrow, or any combination of these. The prognosis is favorable and radiation and chemotherapy should be avoided if possible. In some of these children the rapidly enlarging liver may cause respiratory distress and vena caval compression. The creation of a ventral hernia by means of a Silastic patch is a useful, temporary procedure during the life-threatening period of rapid growth and increased intra-abdominal pressure. Closure is simple once the liver has returned to normal.
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PMID:Silastic abdominal patch for temporary hepatomegaly in Stage IV-S neuroblastoma. 12 71

From 1972 through 1974, 5 infants with severe respiratory distress secondary to mediastinal tumors were treated in the Division of Pediatric Surgery of The Johns Hopkins thospital; The mediastinal masses included 2 intramural bronchial cysts, a giant esophageal duplication, a benign teratoma, and a highly malignant neuroblastomamindividualized management was carried out successfully in all: total resection of the bronchial cysts and teratoma; partial resection of the duplication cyst with stripping of the remaining mucosa from the contiguous esophageal wall, thereby preserving esophageal integrity; and excision of involved chest wall and tumor combined with radiotherapy and chemotherapy for the neuroblastoma. This experience emphasizes the potential for lethal respiratory distress from mediastinal tumors in infants and supports the experience reported by others of serious consequences if resection is not performed. Primary physicians and thoracic surgeons must be aware of the lethal potential of such mediastinal tumors among the many other "surgical" causes of respiratory distress in neonates and infants.
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PMID:Life-threatening respiratory distress from mediastinal masses in infants. 112 61

A phase II trial using interleukin-2 (IL2) and lymphokine-activated killer (LAK) cells was carried out in an attempt to treat children with end-stage neuroblastoma. Fifteen patients (median age, 7 years) were enrolled in the study. Twelve were in relapse after massive chemotherapy and autologous bone marrow transplantation (ABMT), and three had a primary refractory disease after conventional chemotherapy. IL2 was administered as an 18 x 10(6) IU/m2/d continuous infusion. One course consisted of a double 5-day treatment period separated by a 6-day break. Cytapheresis to harvest LAK progenitor cells was performed during the rest period. After a 4-day in vitro culture, LAK cells were reinjected during the second cycle of therapy. A phenotypic and functional analysis of immunologic parameters was conducted along with the therapeutic protocol. Toxicity was significant with two toxic deaths (cardiotoxicity and respiratory distress). The reinfusion of large amounts of LAK cells was clearly involved in one case, but this particularly severe toxicity has to be related to the patient's status (ie, heavy pretreatment). No significant clinical response was seen. The immunologic monitoring showed phenotypic and functional modifications in these patients before initiation of treatment and an unexpected absence of evolution of these parameters during IL2 therapy. Although the origin of these immune dysfunctions is not clear, they could be involved in the failure of IL2 therapy. Future studies of IL2 therapy in neuroblastoma should be undertaken earlier in the course of the disease.
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PMID:Interleukin-2 and lymphokine-activated killer cells in 15 children with advanced metastatic neuroblastoma. 183 96

From 1970 to 1989, 121 children with mediastinal masses of various sorts were seen in the Department of Pathology, Royal Children's Hospital, Melbourne. The series is considered representative of the true incidence of these conditions in the state of Victoria, which had an average paediatric population during the time of this series of 900,000 children. The commonest cause of a mediastinal mass was NHL (36 cases). This was followed by HD (24 cases), then neuroblastoma and ganglioneuroma (16 and 9 cases respectively), duplication cysts (10 cases), teratomas (7 cases), neurofibroma (4 cases) and lymphangioma (3 cases). A great variety of rare conditions made up the remainder of the series and included mediastinal abscess, thymic cyst, pericardial cyst, accessory lobe of lung, plasma cell granuloma, fibromatosis, paravertebral Ewing's tumour, carcinoid tumour and neurofibrosarcoma. Presentation of the children with NHL was often acute with respiratory distress, while the child with HD was usually older and symptoms were more often systemic than local. The surgeon's role in diagnosis of these most frequently encountered mediastinal masses can be crucial and biopsy when indicated must be carried out with great care to produce material that is adequate for diagnosis and for the performance of cell marker studies and chromosome analysis. Neuroblastoma (NBL) and ganglioneuroma (GN) together were the third largest group. Children with neuroblastoma were usually young; 15 of the 18 cases were less than 2 years old. One-third of the infants with neuroblastoma presented with paraplegia and one-third with respiratory symptoms including wheeze, stridor and respiratory difficulty. Three children had Horner's syndrome. Prognosis of children with thoracic neuroblastoma is very good and contrasts with the poor outlook for those with abdominal neuroblastoma. Stage at presentation is probably the most important single prognostic variable. Ganglioneuroma presents at a later age than neuroblastoma and symptoms may be present for a long time or may be completely absent. Catecholamines, usually raised in neuroblastoma, are mostly normal in ganglioneuroma. Duplication cysts were the next most frequent group. Symptoms can often be acute and life threatening, although in three of our ten cases the cyst was an incidental finding on chest X-ray. However, only three of our patients had a normal respiratory examination. Teratomas were usually large and more often benign than malignant. Excision is the mandatory treatment and is usually curative. Although teratomas in young infants are often cellular and composed of many immature tissue types, their behaviour is benign.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Mediastinal masses in childhood: a review from a paediatric pathologist's point of view. 190 92

Between January 1982 and December 1987, of 283 children with neuroblastoma treated in the Pediatric Department of Institut Gustave Roussy, 34 were stage IV-S. Two groups were distinguished according to the initial clinical presentation: 16/34 patients had life-threatening symptoms and needed immediate treatment, and 18/34 had no life-threatening symptoms. Hepatic irradiation was used in 12/16 patients of the first group with respiratory distress. Chemotherapy was used in three patients. Of these 16 patients, three patients died of progressive disease (one patient died of a reason not related to the tumor). Twelve are alive in first complete remission and one in second complete remission. Of the 18 patients of the second group, eight had spontaneous complete remission, and one of them relapsed thereafter and died despite treatment. Ten patients needed treatment, radiotherapy, and/or chemotherapy depending on the site of disease progression. Two of them died of the disease. The overall disease-free survival for these 34 patients is 75% at 90 months postdiagnosis. Using this therapeutic strategy, it was possible to avoid any treatment of metastases in 25% of the patients.
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PMID:Treatment of stage IV-S neuroblastoma: a study of 34 cases treated between 1982 and 1987. 196 Nov 34

Phrenic nerve palsy (PNP) is seen in infants and young children usually resulting from operative trauma or birth injury. Spontaneous recovery usually occurs, but occasionally surgical plication is necessary. Twenty-three cases of PNP over a 10-year period were managed surgically. Patient ages ranged from 1 day to 30 months (median, 4 months), 18 were male and five female. Cause was operative trauma in 18 (17 cardiac surgery, one neuroblastoma), birth trauma in two, and idiopathic in three. The right side was involved in 14, the left in eight, and both in one. Indications for plication were inability to wean from the ventilator (group 1, 16 patients), recurrent pneumonia (group 2, four patients), and respiratory distress (group 3, three patients). The 16 patients in group 1 were intubated for a median of 18.5 days from onset of PNP to plication. Postoperatively, three had continuing congestive heart failure (one died at 16 days of age, one was still chronically ventilated at 22 months, one was extubated at nine days); the other 13 were extubated at a median of two days postoperatively. All the patients in groups 2 and 3 were extubated within two days of surgery. Twelve plications were transthoracic and 11 were transabdominal. Postoperative complications included pneumonia (2), wound infection (1), pneumothorax (2), and mucous plug with pulmonary collapse (1). One patient died of cardiac failure at 16 days. One patient in group 3 developed recurrent respiratory distress 4 months postoperatively; he had a recurrent elevated hemidiaphragm requiring a second plication.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Plication of the diaphragm for infants and young children with phrenic nerve palsy. 317 45

The clinical presentation, diagnosis and treatment of a series of 18 cases of neuroblastoma is reviewed. About half the patients were below the age of 2 years. Fever, loss of weight and appetite appeared to be the common manifestations, underscoring the vague and non-specific symptomatology of such a grave childhood disorder. Unusual presentations included paraplegia, chronic diarrhoea, lower limb edema and respiratory distress. Management consisted of radical surgery with adjunctive chemotherapy where indicated. It is emphasised that in resectable lesions every attempt must be made to remove tumour involved tissue as thoroughly as possible. With this approach 50% of patients followed up to 4 years have survived. Comparison of characteristics of survivors against non-survivors showed that the only significant difference was in the state of the disease--survivors having generally earlier state tumours.
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PMID:Experiences in the treatment of neuroblastoma. 319 Jan 52

A rare case of congenital neuroblastoma of the neck associated with complete Horner syndrome and respiratory distress is described. The case was successfully treated by total excision secondary to chemotherapy. Horner syndrome was of value for early diagnosis of this lesion.
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PMID:Congenital cervical neuroblastoma associated with Horner syndrome. 324 95

To document the incidence and severity of symptomatic hepatomegaly in patients with Stage IV-S neuroblastoma, we reviewed the charts of children with neuroblastoma seen at Children's Hospital of Pittsburgh between 1951 and 1985. Sixteen met the criteria for IV-S disease, and 11 of these (69%) had massive hepatomegaly. Five children had symptoms referable to their liver size including respiratory distress, gastroesophageal reflux, or decreased urine output. Liver function appeared to be normal or only mildly abnormal in the 9 patients where data were available. Because therapy was so variable, it was not possible to correlate treatment regimen with outcome. However, 3 symptomatic patients who received less than or equal to 600 rad without chemotherapy had prompt subjective responses. Follow-up was available on 10 children 6 months-18 years (median 18 years) from diagnosis. Eight were alive with resolved or resolving hepatomegaly. High dose (greater than or equal to 3,300 rad) radiation-related side effects included multiple rib chondromas, chest- and pelvic-wall hypoplasia in one patient, and radiation nephritis with hepatic fibrosis resulting in death of a second patient. Our results support prior recommendations that for symptomatic hepatomegaly, low doses of radiation be considered.
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PMID:Results of therapy in stage IV-S neuroblastoma with massive hepatomegaly. 362 25

Five cases of neonatal thoracic neuroblastoma were either clinically asymptomatic or presented as uncharacteristic respiratory distress. Chest radiography demonstrated a spectrum of abnormalities varying from a lobulated, apical thoracic mass with or without evidence of expansion, to a spindle-shaped paraspinal soft tissue widening, or a homogeneously dense hemithorax. In the newborn a neoplasm in the posterior mediastinum most likely represents a neuroblastoma, either primary or metastatic. In a high percentage of cases the primary tumour as well as possible skeletal metastases have an increased uptake of radiopharmaceuticals. Therefore a bone scan should be the next step for evaluation of the neoplasm. Supplementary sonographic screening of the retroperitoneum and lesser pelvis is easily performed and may contribute significant information. Urography is hardly mandatory any longer. Metrizamide CT myelography, highly recommended in infants and children, may be omitted in the neonate with no neurologic deficit. Urinary excretion of catecholamine metabolites is usually elevated and must always be determined.
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PMID:Thoracic neuroblastoma of the neonate. 405 May 25


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