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A series of 106 cases of polycystic kidneys in adults is presented. The main clinical, exploratory and therapeutic data are analyzed. The average age of the patients at the time of the first clinical manifestation was 35 years; average age at the time of diagnosis was 43 years. The most common forms of presentation included renal colics, blood hypertension, noncolic lumbar pain, macroscopic hematuria, and polydipsia-polyuria. The most frequent symptoms were: abdominal pain of any type (73 patients), polydipsia-polyuria (66 patients), blood hypertension (61 patients), macroscopic hematuria (47 cases), episodes of urinary infection (41 cases), and passing of calculi (22 cases). Seventy-eight subjects had arterial high blood pressure; it was easily controlled in all except 14 cases. Proteinuria was slight in all except two cases. Values for hematocrit and hemoglobin remained high in relation to the degree of renal insufficiency. The mean value of hematocrit in patients with creatinine clearance below 10 ml/min was 30 percent. Renal function decreased gradually, from normal to a clearance of less than 10 ml/min over a period of 12 years on the average. Diagnosis was based mainly on abdominal physical examination and intravenous urography; 89 patients had palpable abdominal masses. Urography revealed typical images of polycystic kidney in every case. The following associated conditions were also discovered: liver cysts (17 cases among 57 liver scanning; bilateral ovarian cysts in one case; Cacci-Ricci's disease in one case; and cerebral arterial aneurysms in another patient. Treatment was conservative with the aim to control arterial blood pressure and urinary infection. Twenty-nine patients required saline replacement; peritoneal dialysis was practiced in two cases and permanent hemodialysis was prescribed for 15 individuals.
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PMID:[Polycystic kidneys in adults. A clinical study of 106 cases (author's transl)]. 52 27

Adult-onset osteomalacia with multiple renal tubular defects and generalized aminoaciduria is uncommon, and where familial it is characteristically an autosomal recessive disorder. This paper describes a kindred in which the syndrome has appeared in four successive generations, apparently inherited in a dominant manner, and possibly associated with diabetes mellitus. The proposita had hypophosphataemia, renal glycosuria, proteinuria and generalized aminoaciduria, and at the age of 22 developed symptoms of osteomalacia which responded to treatment with oral phosphate. Her father had been similarly affected: renal glycosuria was first noted when he was 24, and 12 years later he developed diabetes mellitus from which he died. One sister, aged 31, has renal glycosuria, aminoaciduria and hypophosphataemia without bone disease. In the three preceding generations at least seven other individuals had crippling bone disease and profound muscle weakness of early adult onset; in four, preterminal polydipsia was recorded, and others had renal glycosuria or diabetes mellitus. Three of the five children in the latest generation have slight proteinuria but not other detectable abnormality. The possible association between these renal tubular defects and diabetes mellitus is discussed.
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PMID:Hypophosphataemic osteomalacia and Fanconi syndrome of adult onset with dominant inheritance. Possible relationship with diabetes mellitus. 94 41

Interstitial nephritis secondary to analgesic ingestion is apparently an uncommon subject in pediatric literature. Two cases are reported in this article: case 1 is a girl followed for the last fifteen years when she had lipoid nephrosis which was treated initially with corticosteroids; she responded satisfactorily, but presented frequent relapses. After 8 years, she was given cyclophosphamide plus prednisone and lately, she responded and has remained well. Further on, her urinalysis showed specific gravity of 1,033 and no proteinuria. Five years ago, because of protracted headache due to psychological disturbance, she started to ingest a variety of analgesics in progressively increasing doses. For the last 2 years, abdominal pains, paleness, polydipsia and polyuria have been observed; at present, her blood pressure, serum chemistry, and urine sediment are normal, but there is a marked failure in the renal concentration capacity, as well as marked sodium urinary losses. A percutaneous renal biopsy showed tubulo-interstitial fibrosis and edema with normal glomeruli. Case 2 is a girl with rheumatoid arthritis which appeared 3 years ago; for over one year, the patient was given 15 mg/day prednisone plus 1.5 g. acetylsalycilic acid. She was admitted to the hospital because her osteoarticular problem did not improve. Her blood pressure, blood chemistries and urinary sediment were also normal. LE tests were negative. Renal concentrating capacity was reduced and the renal biopsy showed tubular atrophy; there was intestinal edema and mononuclear infiltration. Chronic interstitial nephritis, secondary to analgesics is supported in both cases; polyuria and a marked defect of renal concentrating capacity are the earliest and most characteristic features. Normal urinary sediment is a common finding leading to erroneous assessment of a lack of renal involvement. Pathological lesions are located in the interstice of the renal medulla and sometimes in the papilla. Early arrest of analgesic ingestion may stop and even reverse the renal lesion and the renal insufficiency.
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PMID:[Chronic interstitial nephritis caused by analgesics]. 113 12

A 5-year-old boy is presented who exhibited the classical symptoms of the syndrome described by BARTTER et al. in 1962: growth retardation, hypokalemic alkalosis, hyperplasia of the juxtaglomerular apparatus with normotensive hyperreninism-hyperaldosteronism, polydipsia with impaired renal concentration ability resistant to pitressin. In addition, the patient showed the typical proximal tubular defects of the Fanconi syndrome: hyperphosphaturia with hypophosphatemic ricktts, generalized hyperaminoaciduria, proteinuria and glucosuria. In the cases of Bartter's syndrome described to date, no uniform pathogenetic mechanism could be identified. Proximal tubular salt wasting generally is assumed to be the primary defect. The studies carried out in this case support this hypothesis and indicate that the complete clinical picture of Bartter's syndrome may occur within the framework of multiple proximal tubular defects.
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PMID:[Bartter's syndrome and tubular functional disturbances]. 115 68

The association of nephronophthisis and tapeto-retional degeneration was described by both Senior and Loken in 1961, but prior to 1974 only 28 cases had been published. This report describes 8 new cases in 27 members of 5 families. The severe juvenile type produces blindness in infancy and death from renal failure before the age of ten. The adult type is characterized by later onset, slower progression of the renal disease and milder ocular manifestations. The eye disease may be congenital amaurosis of Leber type, pigmentary retinal degeneration or retinitis punctata albescens and the electroretinogram (ERG) is of value in the diagnosis of these varieties of hereditary tapeto-retinal degeneration. Renal involvement is often asymptomatic. Defective urinary concentration leading to polyuria and polydipsia is the earliest sign. Proteinuria is inconstant and urinary sediment is often normal. Two patients had aminoaciduria. The disease progresses inexorably to chronic renal failure. One patient has been successfully transplanted and two others are on chronic hemodialysis. Renal histological changes are those of nephronophthisis with tubulointerstitial lesions and multiple cysts. Senior-Loken syndrome appears to be transmitted by a single autosomal recessive pleotropic gene of variable expression. Degeneration of neuroepithelium and renal tubular epithelium, both tissues of ectodermal origin, may represent a genetically determined enzyme abnormality.
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PMID:Senior-Loken syndrome (nephronophthisis and tapeto-retinal degeneration): a study of 8 cases from 5 families. 124 84

A 45-year-old Turk had a year ago noticed a submandibular and a retroauricular node-like swelling, about 2 cm in diameter each, firm and freely mobile. During the preceding two months he had polydipsia and polyuria. Recently he developed a nephrotic syndrome with lower-leg oedema and proteinuria (14 g albumin in 24-hour urine). The concentrations of IgE (250 IE/ml) and IgA (745 mg/dl) were raised, and there was eosinophilia of 14%. Renal needle biopsy revealed glomerulonephritis with minimal proliferation. Excision of part of the nodular tumour revealed histologically the typical signs of Kimura's disease (eosinophilic follicular lymphadenitis of the skin; subcutaneous angiolymphoid hyperplasia with eosinophilia). During treatment with prednisolone, 20 mg daily by mouth, the clinical and biochemical findings regressed within two weeks. But eight weeks later, after dose reduction to 10 mg daily, the nephrotic syndrome recurred so that the dosage had to be increased again to 20 mg prednisolone daily. On this treatment the patient has now been symptom-free for six months. This case demonstrates the unusual association of Kimura's disease with minimally proliferative glomerulonephritis.
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PMID:[Kimura disease with minimally proliferative glomerulonephritis]. 204 61

Renal amyloidosis was diagnosed in 14 young Chinese Shar Pei dogs, all of which were related. Clinical signs were those of renal failure and included vomiting, anorexia, lethargy, polydipsia, polyuria, weight loss, and dehydration. Some dogs had a history of intermittent fever and joint swelling. Laboratory findings also were compatible with renal failure and included azotemia, hyperphosphatemia, low total CO2 content in serum, isosthenuria, proteinuria, and hypercholesterolemia. All dogs had medullary deposition of amyloid, and 9 of 14 (64%) had glomerular involvement. The remaining renal lesions were typical of end-stage renal disease. In some dogs, amyloid deposits were found in other tissues (eg, liver, spleen, stomach, small intestine, myocardium, lymph node, prostate gland, thyroid gland, and pancreas). Amyloid deposits were sensitive to potassium permanganate oxidation, suggesting the presence of amyloid protein AA.
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PMID:Familial renal amyloidosis in Chinese Shar Pei dogs. 221 Dec 93

The medical records of 59 dogs with renal amyloidosis were reviewed. Most dogs with amyloidosis were greater than 6 years old, and females were affected more often than males. Beagles, Collies, and Walker Hounds were at increased risk, whereas German Shepherd Dogs and mixed-breed dogs were at decreased risk. Common historical findings were anorexia, polyuria, polydipsia, lethargy, vomiting, and weight loss. Common laboratory findings were leukocytosis, lymphopenia, nonregenerative anemia, hypercholesterolemia, azotemia, hyperphosphatemia, metabolic acidosis, isosthenuria, cylindruria, and proteinuria. Proteinuria was moderate to severe in most dogs, as assessed by qualitative determination of urine protein concentration, urine protein/urine creatinine ratio, and 24-hour urine protein excretion. Conservative medical management was of little value, and survival ranged from 3 to 20 months in 12 dogs for which this information was available. Moderate to severe diffuse global glomerular amyloidosis was detected in all dogs. Medullary amyloid deposition was multifocal and less severe, but was evident in most dogs. Secondary tubulointerstitial and glomerular lesions were mild or absent in most dogs. Thromboembolism was identified in approximately 14% of affected dogs, underlying inflammatory disease in 37%, and neoplasia in 20%. Laboratory indicators of renal function correlated poorly with histologic lesions, with the exception of glomerular amyloid deposition and "chronic renal disease" index with endogenous creatinine clearance.
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PMID:Clinicopathologic findings in dogs with renal amyloidosis: 59 cases (1976-1986). 276 63

Chronic renal failure was diagnosed in 15 Bull terrier dogs. The dogs ranged in age from one to 8 years. History and clinical findings typically included lethargy, anorexia, polyuria, polydipsia and weight loss. Affected dogs were azotaemic, had elevated serum phosphate and cholesterol, and proteinuria was apparent in all dogs tested (13/13). The concentration of urine was consistently in the nil to minimally concentrated range (specific gravities 1.011-1.017). In those dogs necropsied, both kidneys were approximately two-thirds normal size, tough in consistency, with a pale cortex and a finely nodular capsular surface. Histologically, there was marked nephron loss, diffuse interstitial fibrosis and focal dense radial fibrosis which was especially evident in the renal medulla. Tubular dilation was widespread with focal mineralisation of tubular epithelium and adjacent basement membranes. Glomeruli were often shrunken and segmentally fibrotic. Some Bowman's spaces were extremely dilated. Many less severely affected glomeruli had thickened basement membranes.
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PMID:Chronic renal disease in bull terriers. 277 60

Familial juvenile nephronophthisis (FJN) is a frequent cause of chronic renal failure in children and adolescents. Typically it presents after 6 years of age through adolescence, but may become apparent in early childhood. The clinical presentation is insidious, and the early symptoms of polyuria and polydipsia are often overlooked in the presence of a relatively normal urinalysis and in the absence of proteinuria, azotemia, and hypertension. Thus most patients are not diagnosed until after the onset of renal failure. These children are excellent candidates for properly selected transplantation.
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PMID:Familial juvenile nephronophthisis. A review and differential diagnosis. 351 Jul 94


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