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)

There seems to be little doubt that FGS is a nonspecific lesion that represents one way in which the renal glomerulus responds to a variety of injuries. This is illustrated by the large number of diverse conditions with which the lesion is associated including various forms of glomerulonephritis, pyelonephritis, hereditary nephritis, and heroin usage. Nevertheless, there remains a relatively large isiopathic group which, though possibly heterogeneous, displays a number of characteristic clinical and pathologic features including the following: 1. Steroid-resistant nephrotic syndrome; 2. Hematuria and hypertension; 3. Normal serum complement; 4. Progressive renal insufficiency; 5. Typical pathologic lesion most common in or restricted to juxtamedullary cortex; 6. Absence of clearly defined evidence of immune complex deposition by immunofluorescent or electron microscopic studies; 7. Recurrence of the lesion following renal transplantation. The pathogenesis of these changes is unclear, the evidence for an immune complex mechanism meager, and the suggestion that the disease is mediated by a humoral mechanism remains to be explored. The potential recurrence of this lesion in the transplanted kidney affords a unique opportunity to study the disease early in its course by a variety of techniques that may help to clarify this still poorly understood entity.
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PMID:Focal glomerular sclerosis. 32 May 51

Nine hundred and forty children with hematuria were admitted to the nephrology service from 1958 to 1973. Percutaneous renal biopsies were performed in all of them. Thirty cases out of this group had recurrent hematuria and constitute the clinical material of this study. The clinical picture was: acute nephrotic syndrome in 19; monosymptomatic hematuria in 8, anaphylactoid purpura nephritis in 2, and hematuria associated with nephrotic syndrome in one patient. All patients with nephritic syndrome showed in their biopsies various types of glomerular lesions; most of the patients with monosymptomatic hematuria had normal glomeruli; at the light microscopy, the immunofluorescence was positive in some of them. Both patients with anaphylactoid nephritis showed diffuse endocapillary and focal extracapillary proliferation and in the only one with nephrotic syndrome, the hematuria was familial and the biopsy showed features of Alport's syndrome. Twenty-four patients who were followed for over two years showed no relationship between the age of onset, sex, initial significant proteinuria, hypertension, frequency of bouts of hematuria and the clinical evolution. At the end of the study, 7 patients had prolonged remission: the light microscopy showed normal glomeruli, endocapillary proliferation and endo and extracapillary proliferation with less than 30% of the glomeruli affected by "crescents". The remaining cases were still active and one of them with endo and extracapillary glomerulonephritis with more than 30% of the glomeruli affected by "crescents", developed chronic renal insufficiency. In conclusion, the prognosis of recurrent hematuria of glomerular origen is related with the type of glomerular lesions and constitutes an indication for renal biopsy. Renal specimens must be studied under light microscopy and immunofluorescence techniques; electromicroscopy is required when hematuria is present in more than one member of the family.
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PMID:[Recurrent hematuria of glomerular origin]. 127 66

We collected and evaluated the results of contrast sensitivity (CS) examination by means of Vistech chart with an arranged testing distance 208 and 420 cm covering spatial frequencies 1.15-27.25 cycles/degree (c/deg). Our test was comprised of normal population and patients with chronic renal insufficiency including the group of waiting patients, dialysed patients and dialysed patients after neuroretinopathy and also patients after kidney transplantation and those with Alport's syndrome. We gave our attention to the results of visual acuity (VA) and contrast sensitivity (CS) examination in patients after surgery for detached retina, aphakic patients and patients with artephakia. We examined and evaluated CS in patients with intraocular hypertension. All patients reached the VA values 6/9-6/6. 1. The results of examination of 100 healthy persons of different age displayed significant differences in age groups covering all spatial frequencies between the groups 21-50, 51-60 and 61-80 years whereas in sets of higher age we registered differences in the region of medial spatial frequencies only. These data served us to create control groups in the individual partial groups. 2. Patients with chronic renal insufficiency have CS significantly lowered. These examinations suggest that there is a certain relation between renal and retinal functions and that the dialyzation treatment is not able as yet to compensate fully all changes evoked by renal insufficiency. A clear tendency to normalize CS after renal transplantation is suggestive of a certain reversibility of these changes. This is valid for transplant patients with a clear lens. If opacity of the posterior cortex of the lens occurs after a long-time cortisone treatment, a substantial fall in the CS curve is registered in all spatial frequencies in spite of the VA being 6/9-6/6. Patients with neuroretinopathy have CS always significantly disturbed. These changes are reversible although this reversibility is not complete. The new way of dialyzation treatment secures a relatively rapid normalisation of pathological changes in the fundus and repair of subjective functions. At the same time we came to the conclusion that the prognostic outlooks of these patients have become distinctly better as far as their subjective visual functions are concerned. 3. Patients after surgery for detached retina displayed in all cases in the operated eye highly reduced CS in median and low spatial frequencies simultaneously with a statistically significantly lower threshold visual acuity and reduced slope of the acuity function in the diseased eye.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Contrast sensitivity in clinical practice. 130 11

62 children (20 girls and 42 boys, ranging in age between 3 and 15 years), presenting with acute hypocomplementemic glomerulonephritis or morphologically confirmed endotheliomesangial glomerulonephritis, were admitted to the University Children's Hospital, Berne from 1970 to 1991. The annual incidence of cases of acute hypocomplementemic glomerulonephritis was stable during the study period. The site of the antecedent infection was the throat in 26 patients, upper respiratory tract in 15, the skin in 9, and unknown in 10. The latent period ranged from 0.5 to 3.5 weeks. 41 patients developed hypertension and 17 renal failure. Hypertensive complications were observed in 6 patients and remitted completely in 5 cases. A nephrotic syndrome (edema, proteinuria of 40 mg/[m2.h], albuminemia < 25 g/l) was observed in 11 patients. Microscopic hematuria persisted in many patients for one year or more. Proteinuria remitted in all but one patient, who was found to have Alport syndrome. This study shows the stable frequency of hypocomplementemic glomerulonephritis since 1970, its good prognosis, and the importance of the measurement of C3-complementemia in children presenting with acute glomerulonephritis.
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PMID:[Glomerulonephritis with transient C3 hypoclompimentemia and endotheliomesangial glomerulonephritis in childhood. A long-term experience]. 144 87

Persistent microscopic haematuria, especially when present in young individuals and associated with proteinuria, is generally of glomerular origin. Important causes include glomerulonephritis and Alport's syndrome. A new type of benign familial haematuria has now been reported characterised by little or no proteinuria, no deafness or hypertension and no deterioration of renal function. Examination by electron microscopy of renal biopsy material shows thinning or attenuation of the glomerular basement membrane. A family with thin basement membrane nephropathy is reported to illustrate this newly recognised entity. This disorder should be considered in any patient with persistent microscopic haematuria. Pathologists who assess renal biopsy material should pay greater attention to an ultrastructural morphometric analysis of the glomerular basement membranes of at least two glomeruli.
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PMID:Familial haematuria due to thin basement membrane nephropathy. 237 Oct 4

A retrospective multicentre study of 341 children with persistent/recurrent, isolated haematuria is described. The haematuria was isolated for at least 6 months at the beginning of observation. The duration of follow-up was 2-5 years in 201, 5-10 years in 119, 10-15 years in 19, and over 15 years in 2 cases. Of these patients 47.8% became symptom-free. In 18.4% the haematuria remained isolated; in 13.8% it was combined with proteinuria over 250 mg/day more than 2 years later. The occurrence of associated proteinuria increased progressively with time. It was 8.6% between the 3rd and 5th years, and 37.0% after the 5th year. Renal biopsy was performed because of the symptoms of glomerular disease in 47 cases at an average time of 12 months following the appearance of proteinuria. Proteinuria appeared after a 2-5, 5-10, 10-15 and more than 15 years follow-up period in 16, 23, 6, and 2 patients respectively; 14 of them had Alport's nephropathy. The percentage of more serious azotaemia was 1.7 (creatinine clearance: 10-50 ml/min per 1.73 m2) and 0.3 (creatinine clearance: less than 10 ml/min per 1.73 m2). Mortality was 0.58%. Most of the patients who developed severe azotaemia had persistent microscopic haematuria at the beginning. The prevalence of hypertension was only 1.2%. The time of its appearance was above 5 years in 2 and below 5 years in 2 cases. All these patients had chronic glomerulonephritis. The haematuria was associated with hypercalciuria in 19.9%. In 14.3% of the overall group of patients urolithiasis developed 2-15 years after onset. All of these had hypercalciuria.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Long-term follow-up of patients with persistent/recurrent, isolated haematuria: a Hungarian multicentre study. 270

A retrospective multicentre study of 341 children with persistent/recurrent, isolated haematuria is described. The haematuria was isolated for at least half a year in the beginning of observation. 47.8% of the patients became symptom-free. In 18.4% the haematuria remained isolated, in 13.8% it was combined with greater than 250 mg/day proteinuria greater than 2 years later. The occurrence of associated proteinuria was 8.6% between the 3rd to fifth years, and 37.0% after the 5th years. 14 cases had Alport's nephropathy. The percentage of more serious azotaemia was 1.7 (Ccreat: 10-50 ml/min/1.73 m2) and 0.3 (Ccreat: less than 10 ml/min/1.73 m2). Mortality was 0.58%, rate of hypertension 1.2%. Most of the patients who developed severe azotaemia, had persistent microscopic haematuria in the beginning. The haematuria was associated with hypercalciuria in 19.9%. In 14.3% of the overall group of patients urolithiasis developed 2-15 years after onset. All of them had hypercalciuria. Our findings suggest that symptoms of isolated haematuria may last for a long-term period and need systematic control. When proteinuria and/or hypertension associates to haematuria a worse prognosis can be expected.
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PMID:[Long-term follow up of chronic recurrent isolated hematuria]. 274 57

The authors present a review of the most commonly observed ophthalmological troubles in 244 cases of children affected by cranio-facio stenoses and hospitalized for a first examination. The fundus examination shows that nearly all types of cranio-facio stenoses can be complicated by intracranial hypertension with potential damage on the optic nerve. The hyper and hypotelorisms, particularly frequent, carry along aesthetic prejudice and effect on the ocular motility. In the most severe forms of the diseases (Alport), pseudo-exophthalmy by retrusion of the face can justify preventive surgical procedures in emergency. Squint is frequent (36.5%). All types can be found. Finally, the authors certify the frequency and the seriousness of ocular repercussion, even in so called benign types of cranio-stenoses, such as plagiocephaly, trigonocephaly, and scaphocephaly.
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PMID:[Ophthalmologic complications of craniofacial stenoses. Apropos of 244 cases]. 358 28

A long term follow up study of 100 children referred with recurrent haematuria for at least one year to two regional paediatric nephrology units is described. The mean duration of follow up was 8.2 years. An adequate renal biopsy was obtained in 96 and eight cases of Alport's syndrome and 10 of IgA nephropathy were diagnosed (20% and 26% respectively of the biopsies examined by electron microscopy and immunofluorescence). Five patients developed end stage renal failure and six hypertension requiring treatment, with the occurrence of these complications increasing progressively with increasing duration of follow up (1% at five years compared with 12% at 10 years). Adverse prognostic features were persistence of microscopic haematuria, proteinuria at presentation, and appreciable changes on renal biopsy. Eighty four patients had first degree relatives tested for haematuria; 30% of these families had another affected member. With long term follow up recurrent haematuria is associated with considerable morbidity and potential mortality.
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PMID:Long term prognosis of recurrent haematuria. 401 46

Previous reviews of hematuria in children and adolescents have included patients with proteinuria and other renal functional abnormalities such as hypertension and reduced GFR. We report the clinico-pathological correlations in 76 pediatric patients, aged 3 to 19 years, who underwent a renal biopsy because of isolated hematuria during the 10-year period, 1972 to 1981. All specimens were examined by light and electron microscopy and immunofluorescence techniques. The overall prevalence of abnormal renal histology was 56%. The vast majority (41 of 43) of the abnormal biopsy specimens could be classified into four distinct histological categories: (1) Alport syndrome (N = 9); (2) IgA nephropathy (N = 8); (3) thinning of the glomerular basement membrane (N = 17); (4) vascular C3 staining (N = 7). The children were divided into three clinical subgroups (1) isolated microscopic hematuria ( IMH ), N = 42; (2) IMH plus a family history of hematuria in a first degree relative, N = 15; and (3) IMH plus at least one episode of gross hematuria, N = 19. A significant graded increase in the likelihood of obtaining an abnormal renal biopsy was demonstrated (X2 = 10, P less than 0.007) from groups one to three. Sex, age at onset, or duration of hematuria were not associated with an increased proportion of histopathologic abnormalities. These findings indicate that the yield of a renal biopsy in children with isolated hematuria can be predicted accurately from specific clinical characteristics.
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PMID:Isolated hematuria in children: indications for a renal biopsy. 672 31


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