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Query: UMLS:C0018801 (heart failure)
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A case of meningeal carcinomatosis associated with cerebral metastases from an adrenal neuroblastoma is described. The clinical picture was ushered-in by bilateral sciatic pain in a 50 years old female and was followed by rapidly progressive sensory-motor deficits of the arms and legs, leading to flaccid quadriplegia associated with paralysis of cranial nerves and episodes of mental confusion. Death occurred 4 months alter, in cardiac failure. At autopsy, a bilateral tumor of the adrenal glands was found. No metastases were detected anywhere except in the central nervous system. Histology identified the tumor as a neuroblastoma; meningeal carcinomatosis, radicular infiltration by tumor cells and parenchimal metastases were found in the central nervous system. Neuroblastoma is typically a tumor of childhood, only 13% of them being found in adult's according to Russell and Rubinstein. Meningeal metastases from adrenal neuroblastoma have not hitherto been reported in the literature. In our opinion, the most likely mode of spread of tumor cells to the central nervous system was hematogenous because of the presence of small multiple intraparenchimal metastases; however, possible spread through the perineural lymphatics, as proposed by others, cannot be excluded, due to the prominent localization of tumor cells at spinal roots level. The main differential diagnostic problems (paraneoplastic neuropathy (Wyburn-Mason) and infectious subacute or chronic meningitis) are discussed. The authors stress the emportance of complete cerebro-spinal fluid examination including a careful search for tumor cells.
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PMID:[Meningeal carcinomatosis: clinical and anatomical study of a case of suprarenal neuroblastoma (author's transl)]. 6

In order to determine the incidence and causes of death during the first 100 days after BMT (early deaths) in a pediatric population we have examined data reported in the AIEOP BMT Registry. Up to July 1990, data on 486 children who underwent allogeneic (180) or autologous (306) BMT were evaluable. The children had acute lymphoblastic leukemia (148 cases), acute non-lymphoblastic leukemia (127 cases), neuroblastoma (82 cases), chronic myelogenous leukemia (15 cases), aplastic anemia (nine cases), solid tumors, lymphoma, immunodeficiency or storage diseases. The overall survival is 55% for allogeneic HLA matched and 38% for autologous transplants at 5 years, 24% for HLA mismatched graft at 2 years. Out of the 486 children, 70 (14%) died during the first 100 days after BMT: 33/306 (11%) after autologous BMT, 24/150 (16%) after allogeneic matched BMT and 13/30 (43%) after mismatched BMT. Causes of early death were as follows: disease progression: 12 children (10/306 after autologous and 2/180 after allogeneic BMT); infection: 12 children (five after autologous and seven after allogeneic BMT); interstitial pneumonitis: 21 children (seven after autologous and 14 after allogeneic BMT); cardiac failure: five children (four after autologous BMT); veno-occlusive disease: eight children (three after autologous, five after allogeneic BMT); acute renal failure: three children (one after autologous and two after allogeneic BMT); multiple organ failure: two cases (one after autologous BMT); cerebral hemorrhage: three children (one after autologous BMT); hypertension: one child; acute GVHD: three children (12% of early deaths after allogeneic BMT).
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PMID:Early deaths in children after BMT. Bone Marrow Transplantation Group of the Italian Association for Pediatric Hematology and Oncology (AIEOP) and Gruppo Italiano Trapianto di Midollo Osseo (GITMO). 146 3

Ten patients in stages III or IV of neuroblastoma have been receiving treatment on the basis of a group study protocol that is supported by grant from the Ministry of Health and Welfare. Their chemotherapy regimen has consisted of a combination of cyclophosphamide, vincristine, THP-adriamycin, cisplatin, nimustine and dacarbazine. Surgery has been performed on all patients and in 7 patients their primary tumors were resected either as a delayed primary operation or a second look operation. Radiotherapy has been used for four patients at a total dose of 22-40 Gy. Six patients still survive (survival time: 5-27 months) with time spans that have ranged from 2-19 months of complete response. Major complications have been renal insufficiency, hearing loss, cardiac insufficiency, and bone marrow suppression, We focus mainly on the problems of this multidisciplinary treatment protocol.
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PMID:[Multidisciplinary treatment of advanced neuroblastoma--experience in treatment with the protocol of a group study supported by a grant from Ministry of Health and Welfare]. 313 59

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 liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma. The latter may present with intractable cardiac failure as a result of considerable shunting of blood. Acquired liver lesions often present in the newborn period or early infancy and this includes hepatitis and biliary atresia. The difficulties in the differentiation of the two lesions will be discussed together with the management of biliary atresia. As the child grows older, Reyes encephalopathy with microvesicular fat in the liver is not uncommon. The pathophysiology of Reyes encephalopathy as seen locally will be described. The choledochal cyst with direct (Caroli's disease) or indirect effect on the liver will be described. Problems of childhood portal hypertension as well as congenital hepatic fibrosis will be described. Hemosiderosis of the liver is chiefly seen in homozygous beta-thalassaemia patients who have been kept alive with repeated blood transfusions. Amoebic and pyogenic hepatitis, fatty liver due to protein malnutrition, biliary ascariasis, etc, which are common in tropical and subtropical countries are rarely seen now in Singapore children.
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PMID:Paediatric liver disorders in Singapore. 346 38

This paper reports late effects and health status of 198 children who had cancer or leukemia diagnosed under 2 years of age and their therapies electively withdrawn. This series (92 neuroblastoma [NBL], 57 Wilms' tumor [WT], 46 acute lymphoblastic leukemia [ALL], and 3 non-Hodgkin's lymphoma) was followed for 1-12 years after discontinuation of therapy. Thirty-three children were diagnosed before 1973, 92 between 1973 and 1977, and 73 after 1977 in 16 Italian Pediatric Oncology Centers. As of December 1983, 176 children were reported to be alive and without evidence of primary cancer by physicians responsible for their care. One child died from a second primary tumor, two from late recurrences of the primary cancer, and three from other causes; eight were alive with evidence of primary cancer; and eight were lost to follow-up. Kyphoscoliosis was found in 22 children and other musculoskeletal anomalies in 8. Neurological sequelae were observed in 8 out 35 children with ALL treated with radiotherapy (RT) and intrathecal methotrexate. All but one were in continuous complete remission when they developed seizures (three cases), leukoencephalopathy (three cases), or intracerebral calcifications (two cases). One child had cardiomyopathy and subsequently died from cardiac failure: he had received doxorubicin (400 mg/m2) and mediastinal RT (13 Gy) for NBL. Growth impairments were observed in children with NBL and WT.
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PMID:Health status of young children with cancer following discontinuation of therapy. 347 May 93

Unexpected sudden cardiac death among children with a history of Kawasaki disease has come to be reported in Japan. Death occurred between 2 months and 8 years after complete recovery from Kawasaki disease according to our study material. To study the lesions of Kawasaki disease sequelae we examined 61 cases of Kawasaki disease which came to autopsy. In 17 of these the deaths apparently to be due to sequelae of this disease, were characterized by cardiac insufficiency caused by ancient coronary aneurysm with organized thrombotic occlusion and superimposed acute ischemic myocardial degeneration and/or necrosis. The age of the lesions appears to correlate with the interval period between complete recovery from this disease and death. Six cases succumbed incidentally of other causes: one traffic accident, one hemophilus meningitis, one chronic myeloid leukemia, one neuroblastoma, one meningeal hemorrhage due to rupture of basilar arterial aneurysm, and one acute lymphatic leukemia. Even in these cases, definite sequelae of arteritis were detected. A surgically resected coronary aneurysm and a ventricular aneurysm were also examined. It was disclosed by mass physical check-up of school children that 0.1% had a history of Kawasaki disease, among which 5-6% showed cardiac and/or coronary abnormality including aneurysms. The high incidence of cardiac involvement in Kawasaki disease has been proved in this study and this kind of lesion has the possibility of resulting in unexpected cardiac death of children.
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PMID:Pathological study of sequelae of Kawasaki disease (MCLS). With special reference to the heart and coronary arterial lesions. 379 88

Rubidazone was administered to 24 children with advanced solid tumors or leukemia. The dose ranged from 80 to 150 mg/m2/IV daily to a total dose of 160 to 450 mg/m2/course. This course was repeated at intervals of approximately three weeks. Eighteen of 24 patients (75%) had received adriamycin and daunomycin as part of prior chemotherapy. The major toxic effects observed were myelosuppression, nausea, vomiting, mucositis, and skin rash. Four patients developed abnormal echocardiograms following the rubidazone therapy, 2 manifested clinical cardiac failure, of which one had anthracycline cardiomyopathic changes on autopsy. One of 7 adequately treated ALL patients achieved M2 marrow and improved peripheral counts for 3 weeks. One of the 2 neuroblastoma patients had subjective improvement of bone pain for 2 months. Rubidazone, in a previous heavily treated group of patients used in this study, had dosages of 360 and 450 mg/m2 which produced marrow hyperplasia to aplasia, with only minimal responses.
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PMID:Phase I trial of rubidazone (NSC 164011) in children with cancer. 726 27

The authors report on 3-year-old-girl with neuroblastoma complicated by severe hypertension and cardiac failure. She had cardiomegaly and pleural and pericardial effusions. Echocardiogram showed left ventricular hypertrophy and decrease of the left ventricular ejection fraction to 0.36 (normal > .40). Abdominal computed tomographic scan indicated a 7 x 7-cm tumor in the left suprarenal area. There was a marked increase in catecholamines and metabolites in her body fluids. After hypertension was controlled with doxazosin (a long-acting alpha 1 adrenergic blocker), her cardiac function gradually improved. A tumor was surgically removed and diagnosed as a poorly differentiated ganglioneuroblastoma. Preoperative differentiation between neuroblastoma and pheochromocytoma was not possible on the basis of catecholamine analysis or imaging studies including computed tomography scan and magnetic resonance imaging. It is important to control hypertension quickly in the patients with catecholamine-induced cardiomyopathy to facilitate surgical intervention for diagnosis and treatment.
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PMID:Severe hypertension and cardiac failure associated with neuroblastoma: a case report. 898 90

Three children with renal hypertension are described. Two had histories of neuroblastoma treated by surgical resection and chemotherapy. They both presented later with unilateral atrophic kidney and marked hypertension. Only the child with severe cardiac failure demonstrated high plasma brain natriuretic peptide (BNP) concentrations. The remaining patient had a history of chronic nephritis treated with continuous ambulatory peritoneal dialysis. She also had chronic hypertension and severe cardiac failure. This child demonstrated high plasma BNP levels. The endogenous secretion of BNP is not triggered by hypertension alone, even though exogenous BNP has the pharmacological effect of reducing renin activity.
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PMID:Plasma brain natriuretic peptides and renal hypertension. 1095 34


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