Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 32-week estimated gestational age female infant had elevated systolic blood pressure at birth and a palpable left abdominal mass. Radiologic evaluation revealed a left upper pole renal mass. The renin level was significantly elevated. Left nephrectomy confirmed a diagnosis of congenital mesoblastic nephroma. The postoperative renin level normalized along with the blood pressure. Histologic analysis identified renin production from either the mesoblastic nephroma or secondarily from compression or ischemia. Mesoblastic nephroma should be considered as part of the differential diagnosis for hypertension in the young. The renin concentration and a renal ultrasound scan should be obtained as part of the evaluation.
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PMID:Hyperreninemia and congenital mesoblastic nephroma: case report and review of the literature. 1079 10

The differential diagnosis of hematuria with or without proteinuria is extensive, and isolated hematuria is a common problem in children and adolescents. Extensive evaluation is often necessary for the child presenting with macroscopic plus microscopic hematuria including nonglomerular and glomerular etiologies, while children with only isolated microscopic hematuria can generally be followed after baseline evaluation to rule out infection, hypercalciuria, familial hematuria, sickle cell disease, post-streptococcal glomerulonephritis (GN), and structural abnormalities (cysts, stones, obstruction, Wilms tumor). Children with the combination of hematuria and proteinuria require rapid systematic evaluation, generally including renal biopsy, except in cases where post-streptococcal GN can be clearly documented. Post-streptococcal GN occurs 7-21 days after a streptococcal infection, is associated with an acute fall in C3 levels with return to normal by approximately 8 weeks, rarely causes acute renal failure, and in children has a pattern of gradual resolution of hypertension, hematuria, and proteinuria over a course of 6-12 months.
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PMID:Asymptomatic hematuria in childhood: a practical approach to evaluation. 1079 63

A retrospective analysis of 77 children treated between 1974 and 1996 was undertaken to evaluate morbidity and the evolution of therapy. A Wilms' tumor (WT) was present in 73 children. 74% of patients (pats.) with WT survived (54 of 73 pats.). Histological specimens of 67 patients were re-evaluated, including 4 children with non-WT histology. Among patients with Wilms' tumors (WT), nephroblastoma (NB) of intermediate risk predominated (73%; 46 of 63 pats.). Low-risk tumors occurred in 5 of 63 children (8%; mesoblastic nephroma 3, cystic partially diff. NB 1, completely necrotic NB 1). High-risk WT were diagnosed in 12 of 63 patients (19%) (NB with anaplasia 10, clear cell sarcoma 1, malignant rhabdoid tumor 1). Nephrogenic rests were present in 14 cases. We observed 3 children of school age with renal carcinoma and one patient with an intrarenal neuroblastoma. WT histology was the most important factor determining prognosis (p = 0.018). The risk for relapses was 2.6-fold higher in patients with high-risk WT compared to the standard risk group. Stages were re-evaluated according to SIOP 93-01. Comparing relapse-free survival of stages I, II and III, respectively, there was a reduced survival rate for stage III (p=0.019). According to the SIOP/GPOH protocol in 1989, the regimen was switched from primary surgery to preoperative chemotherapy without biopsy in 1989 (11 pats.). Compared to earlier years, survival improved (n.s.). In 3 patients preoperative diagnosis by means of imaging failed. During preoperative chemotherapy a venous occlusive disease of the liver occurred in 2 patients. Preoperative chemotherapy led to an impressive tumor shrinkage in the majority of patients. 2 patients of the preoperative group died (focal anaplastic NB). Long-term morbidity was analysed in 49 patients and included radiation-induced scoliosis (35), chest-wall deformity (3), congestive cardiomyopathy after relapse (1) and arterial hypertension (2). Over the years there was a trend to reduce frequency and dose of irradiation. Prognosis of WT is excellent but unfavorable histology (high risk) predicts a poor prognosis. In our experience, reduction of tumor volume due to preoperative chemotherapy facilitates tumor removal by surgery and may prevent tumor spillage and the deleterious effects of radiation in young children. Surgery without delay is necessary if the diagnosis is unclear or the tumor fails to respond to preoperative chemotherapy.
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PMID:A 23-year experience with malignant renal tumors in infancy and childhood. 1137 Oct 43

One aspect of concern for survivors of Wilms' tumour has been the late outcome in terms of renal function. Previous studies have documented low glomerular filtration rate and high blood pressure in some patients. Furthermore, disorders in tubular function (especially urinary concentration defects) have been suggested but not confirmed in small studies. The aim of this study was to determine the prevalence and nature of subclinical and overt glomerular, proximal and distal renal tubular toxicity in a population based cohort of survivors of Wilms' tumour. Forty patients (24 female) with a median age of 4.3 years (3 months-11.8 years) at diagnosis were studied. Median follow-up was 8.8 (range 0.06-27.5) years. Glomerular filtration rate was measured by (51)Cr-EDTA plasma clearance, proximal tubular function by electrolyte fractional excretions, urine excretion of low molecular weight proteins (retinol-binding protein) and renal tubular enzymes (alanine aminopeptidase; N-acetylglucosaminidase) and distal tubular function by the osmolality of the first two urines of the day on 3 consecutive days. Renal size (ultrasound) and blood pressure were also measured. Mean (range) glomerular filtration rate was 100 (61-150) ml min(-1) 1.73 m(-2). Nine were below the reference range for healthy individuals with two kidneys. Most serum electrolyte concentrations (sodium, potassium, chloride, calcium, magnesium and phosphate) fell within the normal range for age, as did the fractional excretions. The values that fell outside the normal range were only marginally abnormal. Subclinical measures of tubular toxicity (retinal-binding protein, alanine aminopeptidase, N-acetylglucosaminidase) were abnormal in only four patients. Thirty-seven patients achieved maximal urine osmolalities > or =800 mOsm kg(-1), but three failed to achieve this value even after DDAVP administration. Two patients had evidence of increased urinary albumin excretion. Compensatory renal hypertrophy was seen in all but two patients, but blood pressure was within normal limits in all patients. Current and past treatment for Wilms' tumour does not have any clinically important nephrotoxic effect in the majority of patients. This finding will enable paediatric oncologists to reassure patients and parents that treatment for Wilms' tumour rarely causes long-term renal impairment.
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PMID:Nephrotoxicity in survivors of Wilms' tumours in the North of England. 1240 47

Despite diverse initiating insults, glomerulosclerosis and tubulointerstitial fibrosis are pathological features common to most forms of progressive renal disease. Control of systemic hypertension and blockade of the renin-angiotensin system ameliorate the rate of progression of chronic renal disease; however they generally fail to completely arrest the scarring process. While the chain of events leading to scarring are still being defined, TGF-beta is a cytokine that plays a pivotal role in the pathogenesis of glomerulosclerosis and tubulointerstitial fibrosis [1]. Given the pleiotropic effects of TGF-beta, significant attention has focused on the potential of its downstream mediators as therapeutic targets. Connective tissue growth factor (CTGF) is a member of the CCN gene family, which includes CyR61 (cysteine rich 61), Nov (Nephroblastoma overexpressed) and the WISP family (for review see [2,3,4]). These immediate-early genes coordinate complex biologic processes during differentiation and tissue repair [5]. Increased expression of CTGF has been detected in experimental and human renal fibrosis where it correlates with glomerulosclerosis and the degree of tubulointerstitial fibrosis [6]. In these settings CTGF expression is regulated at least in part by TGF-beta. This review details the biology of CTGF with specific reference to its potential as a therapeutic target in renal fibrosis.
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PMID:Connective tissue growth factor: an attractive therapeutic target in fibrotic renal disease. 1254 Feb 64

Denys-Drash syndrome is a rare disorder consisting of pseudohermaphrodism, Wilms' tumor and nephropathy. We describe here a boy with severe hypospadias and undescended testes, who presented with end-stage renal failure at the age of 1 year and 8 months when he was referred to our hospital. Emergency hemodialysis was performed because of oliguria, edema and severe hypertension, and then peritoneal dialysis was started. The findings of the renal biopsy showed diffuse mesangial sclerosis, consistent with the characteristic change in Denys-Drash syndrome. The analysis of WT1 gene revealed a G-to-A point mutation at 1,186 resulting in a change from Asp to Asn at 396 in exon 9. Since he had no urine output and his kidneys were not functional and in addition, patients with this mutation have been reported to have a high risk of Wilms' tumor, bilateral nephrectomy was performed. The removed kidneys showed no malignancies. Since Denys-Drash syndrome is frequently associated with Wilms' tumor, renal biopsy and gene analysis should be performed on male patients with gonadal anomaly, such as hypospadias and/or undescended testes, and proteinuria.
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PMID:[A case of Denys-Drash syndrome with prophylactic bilateral nephrectomy]. 1268 Mar 20

We present the case of a 74-year-old Caucasian male with an expansive process of the left kidney. No clinical and laboratory signs of polycytemia or hypertension were present. Microscopically, the spherical main tumor mass was composed of small basophilic cells arranged in longitudinal branching tubules typical of metanephric adenoma (MA). Another component, different from MA, was formed by a tumor in a papillary and tubopapillary arrangement. This component was diagnostic of grade 3 papillary renal cell carcinoma. The third component, which was merging with the papillary one, was composed of sarcomatoid, spindle cell carcinoma with prominent nuclear polymorphism and a high number of mitotic figures, including atypical mitoses. The sarcomatoid component filled the entire cortico-medullary space and infiltrated the surrounding non-neoplastic renal tissue, including the renal pelvis. Areas of necroses, hemorrhages, and mitotic figures were frequent. No structures of Wilm's tumor were seen in our case. One year after the excision, the patient is without recurrence and metastasis. The existence of the above mentioned tumor supports the hypothesis that metanephric adenomas and papillary renal cell carcinomas are interrelated lesions.
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PMID:Metanephric adenoma and papillary carcinoma with sarcomatoid dedifferentiation of kidney. A case report. 1462 Dec

In the present article, we report, for the first time, a prenatal diagnosis of a congenital mesoblastic nephroma in combination with a post-partum hyperreninemia with hypertension. A newborn was delivered at 35 weeks gestation who had an intrauterine diagnosis of a renal mass as early as 32 weeks gestational age by ultrasound examination. Tumor nephrectomy was performed on day 11 after delivery when an increase in hypertension was observed in the newborn.
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PMID:Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. 1571 57

Congenital mesoblastic nephroma is the most common renal mass in the newborn period and can present with atypical findings. Certain associated conditions such as hypercalcaemia, hypertension and reninism have been described. We report a cellular variant of congenital mesoblastic nephroma with hypercalcaemia and contralateral medullary nephrocalcinosis.
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PMID:Cellular congenital mesoblastic nephroma with contralateral medullary nephrocalcinosis. 1512 9

Hypertension is commonly associated with Wilms tumor, but hypertension secondary to renin-secreting Wilms tumor is uncommon. We present an infant with severe hypertension and renin hypersecretion, which was resistant to multiple antihypertensive agents. Blood pressure was eventually controlled with a combination of captopril and losartan.
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PMID:Treatment of Wilms tumor-related hypertension with losartan and captopril. 1513 26


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