[Sacrococcygeal chordoma. Apropos of 1 case--review of the literature].
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Biomedical subjects
Publications and source records attributed to R Slim.
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The authors report a case of multilocular cyst of the kidney in an adult. The diagnosis was made, despite the fact that hydatid disease was endemic in the area, on the basis of the finding at urography of a renal mass with the density of water, the finding at echotomography of a multiloculated mass of mixed nature and finally the absence of any blood supply at arteriography. Excision of the multilocular mass left in place a normal kidney with a good result confirmed after 18 months' follow-up. This case is used as a basis to describe the differential diagnosis in the context of the results of special investigations: I.V.U., echotomography, CT scan and arteriography. Diagnoses which must be eliminated are: renal adenocarcinoma in its necrosed form, solitary renal cyst, angiomyolipoma, nephroblastoma and, in endemic areas obviously, hydatid cyst, in particular in its multivesicular form. Despite the multiplicity of investigations, the diagnosis is often uncertain until the time of surgery. Treatment is excision of the abnormal mass with preservation of healthy parenchyma.
This report concerns two pheochromocytomas of renal vasculary pedicle. Diagnosis is made on clinical features and urinary vanylmandelic acid excretion rate. Localisation is made by arteriography. In one case, there is a renal artery compression, and in the other the tumor is vascularised by lombar artery with hypoplasic ipsilateral kidney. These two cases stress the problem of the respective responsibility of both pheochromocytoma and renovascular disease in hypertension genesis. Beside the clinical and biological context pre-operatively, the sulpiride and propranolol tests may have a discrimination value. Excision of the tumor afforded complete relief of symptoms, the two patients remained normotensive three years later.
Eleven cases of hypertension secondary to a renal tumour secreting renin have been described in the literature between 1967 and 1978. The authors here report another case which presented with severe hypertension and a cerebrovascular accident in a 30-year-old woman. Intravenous urography and renal arteriography revealed a tumour of the upper pole of the left kidney. Estimation of renin levels in the renal veins demonstrated hypersecretion on the side of the tumour. Tumourectomy was followed by normalisation of blood pressure levels. Histological examination confirmed a diagnosis of a tumour of the juxtaglomerular apparatus.
Eleven cases of hypertension secondary to a renal tumour secreting renin have been described in the literature between 1967 and 1978. We report here another case which presented with severe hypertension and a cerebro-vascular accident in a 30 year old woman. Intravenous urography and renal arteriography revealed a tumour of the upper pole of the left kidney. Measurement of renin levels in the renal veins demonstrated hypersecretion on the side of the tumour. Tumorectomy was followed by normalisation of blood pressure levels. Histopathological examination confirmed the diagnosis of a tumour of the juxta-glomerular apparatus.
Eleven cases of hypertension due to a renin secreting renal tumor have been reported since 1967. Here we are dealing with another case which appeared as hypertension and cerebro vascular accident in a thirty - year old woman an I.V. Pyélogram and a renal angiogram showed a tumor in the upper pole of the left kidney. Renal veins renin dosage showed hypersecretion on the side of the tumor. The tumorectomy was followed by a normalization of blood pressure. The microscopic study confirmed the diagnosis of renin secreting juxta-glomerular cell tumor.
A case of phaeochromocytoma which presented with hypertension and severe psychiatric disturbances including three attempts at suicide. These psychiatric problems regressed completely following removal of the tumour. Although inconstant, the combination of hypertension and psychiatric problems should always lead to studies for the possible presence of a phaeochromocytoma. The mechanism of these disturbances is not clearly understood but either a direct action of catecholamines on cerebral tissue or changes secondary to blood pressure variations are suggested.
Hydatidiform mole is a benign trophoblastic neoplasia characterized by an abnormal development of the embryo and proliferation of placental villi. Using microsatellite markers amplified by the polymerase chain reaction, we have performed a genetic study on eight independent molar tissues occurring in two sisters. Karyotype and genotype data demonstrate a diploid and biparental constitution in seven of the analyzed moles suggesting a common mechanism underlying the etiology of the various molar pregnancies in this family. The data reported here suggest that complete and partial hydatidiform moles are not always separate entities and that women with familial recurrent hydatidiform moles are homozygous for an autosomal recessive mutation.
OBJECTIVES: We mapped a locus for autosomal recessive molar pregnancies with biparental genomic contribution to chromosome 19q13.4 between D19S924 and D19S890. This 5-Mb region is homologous to proximal mouse chromosome 7 and contains a cluster of Krüppel-type zinc finger genes, including the human homologue of the mouse imprinted genes: the paternally expressed gene 3 (PEG3) and the maternally expressed Zim1 genes. We analyzed the PEG3 gene for mutations in women with familial recurrent hydatidiform moles and to determine its imprinting status in humans. METHODS: We used database searches and screened cDNA libraries to find the complete genomic structure of PEG3. Polymerase chain reaction (PCR) amplification and direct sequencing of coding exons and flanking introns were performed on genomic DNA from the affected women. Allele-specific methylation and expression were studied by methylation-sensitive Southern analysis of a 5' located CpG island and by reverse-transcription PCR of total lymphoblast-derived RNA of normal individuals who were informative for two expressed polymorphisms. RESULTS: We did not detect any mutations in the coding region of PEG3 in the affected women. We observed allele-specific methylation of the CpG island and expression from the paternal allele in two independent informative pedigrees. CONCLUSION: Consistent with the findings in the mouse, the human PEG3 gene is expressed from the paternal allele. Our data support that PEG3 is not mutated in women with familial recurrent hydatidiform moles, although mutations in the regulatory regions that might affect imprinting or transcriptional level of the gene could not be evaluated.
Rupture of hepatocellular carcinoma is a severe complication that occurs in about 10% of patients. It may occur as a terminal event in patients with advanced disease or it may be the first presentation in a healthy individual. Various treatment options have been proposed, which include conservative treatment, transarterial embolization and operative hemostasis or liver resection. We report intraperitoneal hemorrhage and hypovolemia in two patients with spontaneous rupture of an hepatocellular carcinoma treated successfully by transarterial hepatic embolization. On follow-up, these patients died 7 and 8 months after this treatment respectively.
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5-fluorouracil (5-FU), a fluoropyrimidine antimetabolite, is widely used in the treatment of cancers of the digestive tract and breast. The clinical cardiotoxicity of 5-FU was first reported in 1975. Adverse cardiac effects include coronary disorders, heart failure and sudden death of suspected cardiac origin. Six new cases are reported, including 5 cases of angina and one of heart failure. The patients, 4 males and 2 females, were 26 to 71 years of age (mean: 56.2). They had no medical history of heart failure, myocardial ischemia or electrocardiographic anomalies prior to 5-FU treatment. Three patients had hypertension of whom one had had type-II diabetes mellitus for the past 20 years. Clinical symptoms included chest pain in 4 patients and heart failure in one, whereas the last patient had ECG changes with no associated clinical symptoms. Clinical symptoms of angina totally disappeared after the cessation of 5-FU administration, but heart failure was alleviated only after the introduction of digitalis, a converting-enzyme inhibitor and a diuretic. It has been estimated that 1.6% of patients treated with 5-FU develop adverse cardiac effects. Patients at greater risk are those with a history of ischemic cardiac disease, thoracic radiotherapy or high-dose 5-FU therapy. The mechanism involved is not clearly elucidated. Spasms of the coronary arteries or toxic inflammation of the myocardium have been suspected. These 6 new cases confirm the potential for cardiotoxicity of 5-FU and the need for careful cardiac monitoring of treated patients.
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BACKGROUND: Mycotic aneurysms are classic complications of infective endocarditis. Their diagnosis can be difficult when they are located in deep vessels. CASE REPORT: A 8 year-old boy was admitted for psoitis, tenderness of the left iliac fossa and a palpable mass. He was treated with antibiotics for 2 months for infective endocarditis, but vomiting and fever began after 3 weeks of therapy. Blood cultures showed Staphylococcus epidermidis. Ultrasonography of the mass showed findings compatible with an abscess but sampling of its contents showed blood. Immediate Doppler echography showed that the mass was an aneurysm of the left iliac artery. This diagnosis was confirmed by CT scan and aortography. The aneurysm was excised and a satisfactory repair was made using a Gore-tex graft. CONCLUSION: Although this mass had no clinical vascular characteristics, its origin should have been recognized because of the history of infective endocarditis and a better analysis of the ultrasonographic findings.