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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The extended physical examination of a patient includes measurement of blood pressure. In infancy and childhood the blood pressure measurement may be difficult due to an uncooperative and restless patient. In a 16-month-old girl apparently unmeasurable blood pressure was a hypertensive crisis with systolic blood pressure of more than 200 mmHg. The cause of the hypertension was found to be a nephroblastoma. In the case of rapidly progressive arterial hypertension in another 16-month-old girl with left ventricular dilatation and reduced function was a consequence of kidney dysplasia. Headache attacks lead to diagnosis of a subtotal coarctation of the aortic isthmus in a 17-year-old boy. Hypertensive crisis in infancy, childhood and adolescence is discussed based on these case reports. Special features of blood pressure measurement in the pediatric age group, pathogenesis of hypertensive crisis and the potential therapies are discussed incorporating a brief review of the literature.
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PMID:[Hypertensive crisis in infancy and childhood]. 1809 98

Renin plays a crucial role in the control of various physiological processes such as blood pressure and body fluid homeostasis. Here, we show that a splice variant of the Wilms' tumor protein lacking three amino acids WT1(-KTS) suppresses renin gene transcription. Using bioinformatics tools, we initially predicted that a WT1-binding site exists in a regulatory region about 12 kb upstream of the renin promoter; this was confirmed by reporter gene assays and gel shift experiments in heterologous cells. Co-expression of Wt1 and renin proteins was found in rat kidney sections, mouse kidney blood vessels, and a cell line derived from the juxtaglomerular apparatus that produces renin. Knockdown of WT1 protein by siRNA significantly increased the cellular renin mRNA content, while overexpression of WT1(-KTS) reduced renin gene expression in stable and transiently transfected cells. A mutant WT1(-KTS) protein found in Wilms' tumors failed to suppress renin gene reporter activity and endogenous renin expression. Our findings show that renin gene transcription is regulated by the WT1(-KTS) protein and this may explain findings in patients with WT1 gene mutations of increased plasma renin and hypertension.
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PMID:Wilms' tumor protein (-KTS) modulates renin gene transcription. 1867 Apr 5

The Wilms' tumor suppressor gene WT1 is an important regulator of development. Mutations in this gene have been associated with Wilms' tumor, Frasier syndrome, and Denys-Drash syndrome, as well as isolated glomerular disease. Here we report the case of a 4-month-old girl, who presented with end-stage renal disease, thrombopenia, anemia, and cardiac hypertrophy accompanied by severe hypertension. Histological analysis of kidney biopsies revealed a massive and diffuse nephroblastomatosis with a dramatic reduction in the number of glomeruli. Although no normal cortical nephrons could be detected, medullary organization was nearly normal. Sequence analysis demonstrated a heterozygous nonsense mutation in exon 9 of WT1, which leads to a truncation of the WT1 protein at the beginning of zinc finger 3. Given the requirement of WT1 for normal development of the kidney and heart, these data raise the hypothesis that the mutation identified was responsible for the severe phenotype observed in our patient.
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PMID:A novel Wilms' tumor 1 gene mutation in a child with severe renal dysfunction and persistent renal blastema. 1851 27

Wilms tumor (WT), also called nephroblastoma, is a solid, malignant renal mass that can sometimes grow so large it spreads outside the kidney and invades other structures. Most experts recommend complete tumor resection as a primary intervention. The clinical manifestations caused by the WT most significantly hypertension, can appear as a barrage of pathophysiological events to the nurse anesthetist. The case presented involves an 8-week-old infant who underwent a radical nephrectomy because of a WT. The occurrence, symptoms, pathophysiology, and intraoperative anesthetic management of WT are discussed.
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PMID:Anesthetic management of an infant undergoing radical nephrectomy for Wilms tumor: a case report. 1856 24

This study presents a case of juxtaglomerular cell tumor (JCT) in a 12-year-old girl with hypertension. Fine needle aspirate (FNA) cytology demonstrated a neoplasm with features of a papillary carcinoma, prompting a right radical nephrectomy. Histological examination revealed solid sheets of round epithelioid cells with eosinophilic granular cytoplasm, and distinct cell borders in a background of widespread hemorrhage. Electron microscopy revealed cytoplasmic renin granules. The differential diagnosis of a renal mass in a young patient with hypertension includes JCT, Wilm's tumor, and renal cell carcinoma, which may produce renin. The renin granules detected by electron microscopy are characteristic of JCT, and the diagnosis is confirmed by ultrastructural study. FNA cytology is not sensitive enough for the diagnosis of JCT and its results must be carefully interpreted.
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PMID:Juxtaglomerular cell tumor of the kidney: case report and differential diagnosis with emphasis on pathologic and cytopathologic features. 1909 17

The prenatal diagnosis of abdominal mass poses the problem of its origin. Renal tumors are rarer than neuroblastoma but they are most often congenital mesoblastic nephroma. The congenital mesoblastic nephroma has a good forecast in spite of a sonographic impressive aspect. MRI can help to locate tumor but cannot tell difference between the different kinds of renal tumor. Prenatal forecast is especially linked with hydramnios and hydrops fetalis. Histolological study of the tumor is important for the prognosis. Two morphological subtypes are currently distinguished: the classic type with a good forecast and the atypical or cellular type. Distant metastases have been related only to the cellular form but especially in infants aged more than 3 months and never in the newborns. The diagnosis of the tumor does not change the mode of delivery except in case of an important volume. Complications are searched during the first days of life: hypertension, hypercalcemia, vomiting, hyperreninemia. Radical nephrectomy is performed after the end of the first week. In case of a classic form, the healing is always obtained. In case of cellular form, distant metastases are searched. In any rate, the follow-up is recommended until the end of the growth.
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PMID:[Management of antenatal fetal abdominal tumors. Clues for the diagnosis of a congenital mesoblastic nephroma]. 1938 47

Moderate to severe pulmonary embolism (PE) can cause pulmonary arterial hypertension and right ventricular (RV) heart damage. Previous studies from our laboratory indicate that the basal outflow tract of the RV is injured and has acute inflammation followed by tissue remodeling while the apex appears normal. The present studies examine transcription responses to chronic PE in RV apex and outflow tracts using DNA microarrays to identify transcription responses by region. Changes predominated in the RV outflow tract (8,575 genes showed >/=1.5-fold expression change). Gene ontology and KEGG analyses indicated a significant decrease in genes involved in cellular respiration and energy metabolism and increases in inflammatory cell adhesion molecules and extracellular matrix proteins. Signal pathways for wound healing such as fibroblast growth factor, collagen synthesis, and CCN proteins (named for the first three members of the family: cysteine-rich protein 61, connective tissue growth factor, and nephroblastoma overexpressed gene) were strongly upregulated. In comparison, few genes (422) showed significant change in the RV apex tissue. Apex-selective genes included two genes affecting metabolism and a stretch-sensitive transcription factor (ankyrin repeat domain 1). We conclude that the RV outflow tract is subject to strong proinflammatory and profibrotic remodeling transcriptional responses in chronic PE. Severe loss of genes involved in cellular respiration is consistent with previous histology indicating a shift in cell types present within the outflow tract tissue away from highly energy-dependant cardiomyocytes to less metabolically active cells during remodeling. The apex region of the RV had few compensating adaptations.
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PMID:Transcriptional changes in right ventricular tissues are enriched in the outflow tract compared with the apex during chronic pulmonary embolism in rats. 1960 18

Metanephric stromal tumor (MST) is a recently recognized benign renal stromal tumor. MST is thought to be part of a spectrum of benign metanephric renal lesions, which also includes the epithelial lesion metanephric adenoma and the mixed stromal-epithelial lesion metanephric adenofibroma. Metanephric lesions may represent hyperdifferentiated counterparts to Wilms' tumor (WT). MST characteristically shows renovascular angiodysplasia and juxtaglomerular (JG) cell hyperplasia. This is remarkably similar to the renal pathology described in neurofibromatosis-1 (NF-1) syndrome, a condition which is also associated with WT. Here, we report the first case of MST arising in a patient with NF-1. The patient presented with hypertension, and the MST was associated with florid angiodysplasia and JG cell hyperplasia. This case tightens the link between NF-1, WT, and MST.
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PMID:Metanephric stromal tumor arising in a patient with neurofibromatosis type 1 syndrome. 1987 83

The diagnosis of Frasier syndrome is based on the association of male pseudohermaphroditism (as a result of gonadal dysgenesis), with steroid-resistant nephrotic syndrome due to focal and segmental glomerular sclerosis (FSGS), which progresses to end-stage renal failure (ESRF) during adolescence or adulthood. Frasier syndrome results from mutations in the Wilms' tumour suppressor gene WT1, which is responsible for alterations in male genital development and podocyte dysfunction. We describe the case of a 7-year-old girl who was referred to the paediatric emergency department with ESRF. Haemodialysis was started immediately because of severe hypertension and hyperkalaemia. In view of the fact that our patient had a past medical history of pseudohermaphroditism, we suspected that the acute presentation in ESRF may be related to a new diagnosis of Frasier syndrome. Our hypothesis was confirmed on examination of the medical records. There had been no medical follow-up for several years and, in particular, no renal imaging or functional assessment had ever been performed. This lack of surveillance explains why our patient presented with ESRF much earlier in this disease than expected and subsequently had to undergo kidney transplantation at a very young age.
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PMID:Frasier syndrome, a potential cause of end-stage renal failure in childhood. 1992 Dec 79

Wilms Tumor (WT) is a very rare malignancy in adults representing 1% of all renal neoplasms. It is however the most common renal tumor of children and adult patients are treated like pediatric cases. Bilateral tumors occur in 5% of adult cases. The typical presenting features are asymptomatic abdominal mass (most common), hematuria, pain, fever, and hypertension. As clinical presentation of WT is similar to that of renal cell carcinoma (RCC), it tends to be an unsuspected pathological diagnosis in most cases. The diagnosis of the tumor needs positive sonographic and computed tomography (CT) findings with histopathological confirmation. Prognosis of adult WT is relatively poor and resistant to chemotherapy. We present a case of wilms tumor in a 68 years old male patient with right sided non tender abdominal mass and occasional flank pain. The patient was normotensive but hematuric and radiological findings suggested right renal mass with enlarged lymph node and histopathological analysis revealed nephroblastoma associated with lymph node metastases.
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PMID:Wilms tumor in adult. 2039 30


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