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Query: UMLS:C0018799 (heart disease)
34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The incidence of structural renal tract abnormalities in 109 children with documented congenital heart disease (CHD) was studied using real-time sonography. In these children, 11.9% had associated urologic anomalies, which included hydronephrosis, duplication, ectopia, agenesis, and dysplasia of kidneys. Children with associated extracardiac anomalies had a significantly higher incidence of renal tract anomalies (39.1%) compared to those with isolated CHD (4.7%). Therefore, sonographic screening of the renal tract should be performed routinely in patients with CHD, especially in those with multiple congenital defects.
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PMID:Sonographic screening for renal tract anomalies associated with congenital heart disease. 215 10

Dilated cardiomyopathy (DCM) is defined as a syndrome of dilated ventricles with gross impairment of ventricular systolic function. However, few reports on perioperative management of DCM were obtainable. This paper describes perioperative management of two patients with DCM. A 72-year-old man, whose DCM had been treated with medication, was planned for gastrectomy. His cardiac signs indicated NYHA class II. Cardiac function, evaluated prior to the surgery with echocardiography to determine an operative indication, turned out to be well compensated. Minimal cardiac derangements were anticipated perioperatively. The procedure was carried out under general anesthesia with neuroleptanalgesia utilizing butorphanol and vasoactive agents. No circulatory complications were observed throughout the surgery. A 66-year-old man, who had long-standing heart disease, hemiplegia and hydronephrosis due to ureteral stone, was planned for percutaneous nephrolithotomy. However, the planned surgery was withdrawn because he had DCM with minimal compensatory function and perioperative cardiac derangements were anticipated according to the categorized data classified with echocardiography. We conclude that preoperative assessment of cardiac function is essential to minimize perioperative cardiac derangements in patients with DCM.
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PMID:[Perioperative management of patients with dilated cardiomyopathy]. 273 70

From June 1980 to October 1985 we performed 22 ureteropyeloplasties on 20 infants and neonates with ureteropelvic junction obstruction. All patients were males less than 2 years old; 12 were less than 1-month-old. The diagnosis was suspected on the basis of maternal ultrasonography in 10 patients (50%), a palpable abdominal mass was the presenting symptom in 7 (35%), and obstruction was detected during evaluation of congenital heart disease in 3 (15%). This change in mode of presentation is expected to become even more pronounced as the use of fetal ultrasonography increases. All patients in whom the diagnosis was suspected antenatally underwent ultrasonography after birth to confirm the presence of hydronephrosis. All 22 obstructed kidneys were repaired by a dismembered technique under optical magnification. There were no operative mortalities. Of 21 postoperative intravenous pyelograms available, 3 demonstrated marked improvement, 17 showed improvement with residual hydronephrosis, and 1 showed deterioration. The methods used to diagnose, treat and evaluate ureteropelvic junction obstruction in 20 infants and neonates are presented. Given the existing clinical and experimental data we advocate early postnatal surgical correction of ureteropelvic junction obstruction to achieve a maximum recovery of renal function.
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PMID:Management of ureteropelvic junction obstruction in infants and neonates. 306 35

A male neonate with severe malformations, including facial dysmorphism, a short neck, postaxial-hexadactyly of the toes, congenital heart disease, hydronephrosis, imperforate anus and agenesis of corpus callosum, is described. His karyotype was 46,XYdel(2)(q12q14).
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PMID:De novo interstitial deletion of the long arm of chromosome 2 in a malformed newborn with a karyotype: 46,XY,del(2)(q12q14). 662 39

A female patient who had open heart surgery for cor triatriatum under hemodialysis, subsequent kidney transplantation and pregnancy is reported. We performed hemodialysis on the patient before, during and after heart surgery to control renal failure. Two years after heart surgery, she received a kidney graft from her mother. The kidney graft showed good function. She was treated with azathioprine and prednisone. Three years after renal transplantation she delivered a healthy male infant by elective Caesarean section at 37 weeks' gestation. Mother and infant did well following delivery. There was lack of hypertension, proteinuria, signs of graft rejection, and recurrence of heart failure during pregnancy. She showed serum creatinine level < 2 mg/dl, a prednisone of < 2 mg/kg/day. Elective Caesarean section has improved hydronephrosis due to the compression of the fetus. The aforementioned good criteria contributed to the successful pregnancy of the renal transplant patient in our experience. We believe early surgical intervention overcomes complicated heart disease even with endstage renal disease, and it gives a chance to receive renal transplantation and have a healthy child. To our knowledge, this is the first report that has described the successful management of open heart surgery under hemodialysis, subsequent renal transplantation and pregnancy in a female patient with chronic renal failure.
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PMID:A case of cor triatriatum with end-stage renal disease: successful management of open heart surgery under hemodialysis, subsequent renal transplantation and pregnancy. 848 11

A 60-year-old man presented with sudden palpitations in September 1993, and was admitted to hospital with a diagnosis of atrial fibrillation and heart failure. The patient was suspected of having collagen disease, because of a positive test for antinuclear antibodies and a high sedimentation rate. He was referred to lou hospital. Chest radiographic findings were suggestive of cardiomegaly and cardiac murmurs were audible, which indicated the presence of heart disease. A chest Ct scan revealed a lesion surrounding the intramediastinal large vessels and the heart from the level of the confluence of the left brachiocephalic vein and the superior vena cava. Suspicion of a mediastinal tumor led the patient to be admitted to the respiratory department. Percutaneous needle biopsy with a Trucut needle revealed non-specific chronic inflammation. An abdominal CT scan showed that the lesion surrounding the descending aorta traversed the diaphragm, reached the renal pelvis along both renal arteries, and caused narrowing of the ureter and left hydronephrosis. Based on these findings, retroperitoneal fibrosis was diagnosed. Treatment with steroids caused the lesion to shrink.
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PMID:[Retroperitoneal fibrosis with involvement up to the mediastinal space]. 877 75

We have treated seven children with multicystic dysplastic kidney during the last 15 years. Three of then presented contralateral upper urinary tract anomalies. Four cases were diagnosed during prenatal period and three during the first term of life. We did analytic kidney function evaluation, ultrasound examination, voiding cystourethrography, radionuclide scan, gammagraphy, excretory urography and urinary cultures in all cases. We have found a vesicoureteral reflux with a bifid pelvis associated in the same child and two other cases with nonobstructive hydronephrosis. The child with vesicoureteral reflux presented also ipsilateral anomalies like a bladder diverticulum and an incomplete urethral duplication. One child with hydronephrosis opposite to the multicystic dysplastic kidney, also presented a bowel malrotation and a congenital cardiopathy, as extra-urologic associated anomalies.
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PMID:[Multicystic renal dysplasia: changes in the contralateral urinary tract]. 892 84

A 70-year-old man with metastatic liver tumors showed carcinoid syndrome with clinical symptoms of facial flushing, palpitation, dyspnea, and an itching sensation. Regurgitation of the tricuspid and aortic valves was observed by echocardiography. An elevated serum level of serotonin and a high urine excretion of serotonin metabolites were confirmed. Autopsy confirmed a serotonin-immunoreactive 1 cm ileal carcinoid tumor with metastasis to the liver, bone and peritoneum. The tumor cells were argyrophilic but not argentaffin, and showed erythrophagocytosis in the primary lesion. All the four heart valves and bilateral atrial endocardium showed fibromyxoid thickening, indicating the association of carcinoid heart disease. Desmoplastic reaction with deposition of sulfated acid mucopolysaccharides was also observed in the retroperitoneal space (secondary retroperitoneal fibrosis). Stenosis of the bilateral ureters and inferior mesenteric artery provoked hydronephrosis and lethal ischemic necrosis of the left-sided colon, respectively. Rarity of functioning ileal carcinoid tumor and pathogenesis of systemic fibroplasia are discussed.
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PMID:Ileal carcinoid tumor complicating carcinoid heart disease and secondary retroperitoneal fibrosis. 1084 30

The Marshall-Smith syndrome (MSS) is rare congenital disorder, characterized by a triad of orofacial dysmorphism, failure to thrive, and accelerated osseous maturation. An 8-year-old boy with MSS associated with congenital glaucoma, corneal erosion, laryngomalacia, glossoptosis, choanal stenoses, bilateral peripheral hearing defect, ventriculomegaly, congenital heart disease (atrial septal defect), chronic pulmonary disease, and scoliosis was reported. A tracheostomy with endotracheal tube insertion was performed for his difficult airway. He has longer survival time than those MSS patients hitherto reported. High-resolution chromosome banding and extensive metabolic investigation did not detect any abnormality, except for low blood levels of zinc and thyroxine. Besides, brain atrophy with hypoplastic cerebellum and brainstem, and bilateral hydronephrosis with hydroureter were detected by image studies.
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PMID:Long-term follow-up of Marshall-Smith syndrome: report of one case. 1562 71

We report a case of the Antley-Bixler syndrome in an 11-year-old girl. She presented with bilateral proximal femoral focal deficiency, right clubfoot, left radiohumeral synostosis, bilateral ear hypoplasia, cleft palate, tongue tie, missing teeth, congenital heart disease, a pelvic kidney with hydronephrosis, and mental retardation. Proximal femoral focal deficiency has never been reported before as a manifestation of Antley-Bixler syndrome. Her mother was exposed to radiation during an intravenous urogram done in the first trimester of pregnancy. Exposure to radiation has not been implicated as a cause of Antley-Bixler syndrome.
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PMID:Proximal femoral focal deficiency as a manifestation of Antley-Bixler syndrome: a case report. 1742 25


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