Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A relationship exists between tumours and malformations both generally and in particular combinations. This is also valid for minor errors of morphogenesis suggesting that embryonic tumours are an expression of aberrant intra-uterine morphogenesis. We speculated that these minor aberrations might also manifest in other morphological defects, especially in minor anomalies and malformations of the ribs. We reviewed chest roentgenographs of 1000 children with malignancies for rib anomalies and compared them to 200 patients with mainly infectious diseases. We found 242 rib anomalies in 218 children with tumours (21.8%) and 11 (5.5%) in children without malignancy. This difference was statistically highly significant (P less than 0.001). A high incidence of cervical ribs was found in neuroblastoma (33%), brain tumour (27.4%), leukaemia (26.8%), soft tissue sarcoma (24.5%), Wilms tumour (23.5%) and Ewing sarcoma (17.1%). Only neuroblastoma showed a high incidence of rib bifurcation (4.5%). The increased incidence of these mesenchymal defects in children with malignancies may be another clue for an altered morphogenesis in tumour origin. In neuroblastoma the rib anomaly may be another expression of neurocristopathy as proposed for the association of congenital heart disease and neuroblastoma.
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PMID:Association of rib anomalies and malignancy in childhood. 162 71

Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific well-known associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma.
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PMID:The association of congenital neuroblastoma and congenital heart disease. Is there a common embryologic basis? 292 25

Eight cases of nonimmunologic hydrops fetalis (HF) were seen at Cedars-Sinai Medical Center over a 3.5-year period. The etiology of the HF included 2 cases of fetal-maternal hemorrhage and 1 each of sacral teratoma, tachyarrhythmia, diaphragmatic hernia, neuroblastoma, and heart disease. For 1 infant, the cause was never found. These cases involved various diagnostic and therapeutic dilemmas. Only 4 were anticipated ant partum by ultrasound scanning. Two of the 8 fetuses died in utero, whereas 4 others died in the neonatal period. Earlier diagnosis and evaluation are likely to improve these outcomes.
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PMID:Nonimmunologic hydrops fetalis. 701 19

There have been 28 previously reported cases of neuroblastoma associated with congenital heart disease. Because many of these have been defects of the conotruncal region, it has been proposed that abnormal neural crest cell migration or maturation may be a factor that links these normally disparate pathologic conditions. Most neuroblastomas in these cases have been detected at autopsy or by radiologic studies conducted in the evaluation of the cardiac anomalies. Recently, we discovered an occult posterior mediastinal neuroblastoma in a patient undergoing a unifocalization procedure for tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals. The tumor was resected, and the patient has demonstrated no evidence of residual or metastatic neuroblastoma.
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PMID:Intraoperative discovery of neuroblastoma in an infant with pulmonary atresia. 943 87

This article summarizes the major PET studies which have been performed in pediatric patients to elucidate and characterize diseases and normal development. Issues special for the application of the technique in children, such as dosimetry, patient preparation, and image acquisition are discussed. Studies of central nervous system (CNS) development and pathology, including epilepsy, intraventricular hemorrhage, neonatal asphyxia, tumors, and effects on the CNS from treatment of other tumors are reviewed. These have contributed information fundamental to our understanding of CNS development and pathology. PET investigations into the pathophysiology of congenital heart disease have begun and hold great promise to aid our understanding of these conditions. The second major area in which PET has been applied is the study of non CNS neoplasms. Neuroblastoma has been investigated with tracers which explore basic biochemical features which characterize this tumor, as well as with tracers which explore biochemical events relatively specific for this malignancy. Other common and uncommon tumors of childhood are discussed. The PET technique has been shown useful for answering questions of clinical relevance for the management of these uncommon neoplasms. PET, using tracers that reflect basic metabolic processes, is likely to continue to aid our understanding of many pediatric diseases and may gain more widespread clinical acceptance as the technology continues to disseminate rapidly.
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PMID:PET applications in pediatrics. 954 19

Previous studies have suggested a relationship between reproductive history, pregnancy and birth factors, and the risk of neuroblastoma. We conducted a case-control telephone interview study that included a total of 504 children under the age of 19 years with newly diagnosed neuroblastoma identified by two national collaborative clinical trials groups, the Children's Cancer Group and the Pediatric Oncology Group. A total of 504 controls, matched to cases on age, were identified by random digit dialing. Conditional logistic regression was used to estimate the matched odds ratio (OR) and 95% confidence interval (CI) with adjustment for household income, and maternal race and education. In addition, case subgroups defined by age at diagnosis, tumour MYCN oncogene amplification status, and stage were evaluated. A suggestive pattern of increased risk was seen for a greater number of prior pregnancies, history of previous miscarriages and induced abortions, with nearly a twofold increase in risk for two or more prior induced abortions (OR = 1.9, 95% CI [1.0,3.7]). No association was found for the following diseases or conditions during pregnancy: hepatitis, rubella, measles, mumps, chickenpox, mononucleosis, vaccinations, morning sickness, pre-eclampsia, bleeding, proteinuria, anaemia, urinary tract infections, heart disease, kidney disease, liver disease and diabetes. A weak association was found for hypertension during pregnancy. Several labour and delivery factors were related to an increased risk, including threatened miscarriage, anaesthetic during labour (specifically epidural) and caesarean delivery. We found associations between premature delivery (<33 weeks: OR = 1.9, 95% CI [0.7,4.8]), very low birthweight (<1500 g: OR = 2.6, 95% CI [0.7,10.3]) and risk of neuroblastoma. There was no consistent pattern of increased risk found for most factors within subgroups defined by age at diagnosis, stage or MYCN status.
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PMID:Association of pregnancy history and birth characteristics with neuroblastoma: a report from the Children's Cancer Group and the Pediatric Oncology Group. 1170 80

Five cases of sirenomelia presented with rare manifestations are discussed. Three neonates were born alive and died within 2 to 12 hours after birth. One case was the offspring of a triple in vitro fertilization pregnancy with history of early intrauterine death of one of the triplets. The main features included fusion of lower extremities (five of five), renal agenesis (three of five), polycystic renal dysplasia (two of five), anal atresia with large bowel hypoplasia (three of five), pulmonary hypoplasia (four of five), and single umbilical artery (five of five). Other features that have only rarely been associated with sirenomelia included concurrence of congenital heart disease and neuroblastoma, gallbladder agenesis, and upper extremity defects.
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PMID:Rare manifestations of sirenomelia syndrome: a report of five cases. 1547 30

Chylothorax and chylopericardium are rare conditions occurring in infants and children. Both may be traumatic or nontraumatic in origin. We reviewed our experiences with the management of sixteen pediatric cases (10 males, 6 females; 11-days to 14-years old) of chylothorax and chylopericardium from 1997 to 2003. There were fifteen cases of chylothorax (9 left, 2 right, 4 bilateral) and two cases of chylopericardium (1 isolated and 1 associated with chylothorax), and their incidences of occurrence after cardiothoracic surgery were 0.89% and 0.12%, respectively. Of the fifteen cases occurring after cardiothoracic surgery, thirteen patients had corrective or palliative surgery for complex congenital heart disease, and two patients had removal of thymolipoma and neuroblastoma, respectively. Included also in this review was an 11-day old preterm infant with hydrops fetalis and congenital heart disease who developed chylothorax. Characteristics of chylous effusion included a presence of whitish opaque fluid in the pleural cavity and the pericardial cavity, having a triglyceride content ranging from 59 to 1689 mg/dl which was higher than a plasma triglyceride, a protein content of 2.4 to 7.4 g/dl, and a presence of lymphocyte predominance. The average latent period for diagnosis of chylothorax or chylopericardium was 13 days (range 3-30). All patients were treated primarily with nutritional modification using medium-chain triglycerides (MCT) instead of long-chain triglycerides in their diet; and only a few cases needed bowel rest with total parenteral nutrition (TPN). Twelve patients completely responded to a MCT-rich diet; two cases resolved after switching to TPN and another case needed surgery for ligation of lymphatic vessels around the thymus gland. The mean duration of lymph drainage was 12.1 days (range 3-29) and the average length of time of continued conservative treatment (MCT-rich diet and TPN) was 29.8 days (range 18-47). Fourteen patients (87.25%) had good outcome, i.e. resolution from chylothorax or chylopericardium and return to normal diet. Two of the fourteen patients developed severe infections; one was diagnosed with suspected bacterial endocarditis and the other had candidemia. However, both responded well to antibacterial and antifungal drugs, respectively. One case succeeded after surgery. A case that had low compliance with dietary recommendations and required repeated placement of drainage devices died due to infection with enterococcal septicemia. Early and good compliance with MCT-rich diet is essential for achieving a favorable outcome in the management of chylothorax and chylopericardium in children.
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PMID:Management of chylothorax and chylopericardium in pediatric patients: experiences at Siriraj Hospital, Bangkok. 1592 38

We report the fourth example of a patient with germline partial trisomy of 2p21-pter and congenital neuroblastoma. The male infant had a dysmorphic facial expression and presented with congenital heart disease, supernumerary nipples, hypospadias, shawl scrotum, hemilateral persistent hyperplastic primary vitreous, and neuroblastoma. His germline karyotype of 46,XY,der(8)t(2;8)(p21;p23.2) was inherited from a maternal-balanced translocation, which indicates that the proto-oncogene MYCN region of 2p24.3 is tripicated in germline cells. A cytogenetic study of the biopsied tumor cells did not show MYCN amplification, but the DNA index was 2.4 and histologic fluorescent in situ hybridization analysis indicated somatic mutation with near-pentaploidy of the tumor cells. This could be an alternative mechanism of MYCN activation in the process of the tumorigenesis of neuroblastoma.
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PMID:Congenital neuroblastoma in a patient with partial trisomy of 2p. 1679 7

The possibility of links between psychosocial factors and cancer incidence and progression has generated considerable scientific and public interest. Tachykinins, including substance P, neurokinin A and B, hemokinin-1 and endokinins, are a family of neuropeptides, acting through three types of transmembrane G-protein coupled receptors denoted NK1, NK2 and NK3. Besides their role as neurotransmitters in peripheral and central nervous system, tachykinins and their receptors are also expressed in several non neuronal cells contributing to the fine connections between nervous systems and peripheral organ system such as respiratory, cardiovascular, immune, endocrine, gastrointestinal and genitourinary. Being so much involved in regulating physiological functions, they, of course, can concur to pathological conditions including cancer. Tachykinins can act on different steps of carcinogenesis. Tumors expressing NK receptors, such as astrocytoma, glioma, neuroblastoma, pancreatic cancer and melanoma, can misuse tachykinin-induced signaling, operating in normal cells, to promote proliferation and survival of cancer cells and to release cytokines and soluble mediators favoring tumor growth. In neuroblastoma, breast and prostate carcinomas tachykinins facilitate tumor metastatic infiltration in the bone marrow. In neuroendocrine carcinoma, tachykinins are responsible of symptoms associated with these pathologies including flushing, diarrhea, wheezing and right heart disease. In addition, regardless tumor histology, tachykinins may favor cancer incidence and metastatic progression by influencing blood flux and neovascularization in tumor formation as well as inducing immunosuppression mediated by neurogenic inflammation due to stress or surgery. However, the precise involvement of tachykinins in cancer pathologies and the potentiality to become effective pharmacological drug targets remain to be fully defined.
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PMID:Tachykinins and their receptors in human malignancies. 1691 32


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