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Biomedical subjects

A Storari

Publications and source records attributed to A Storari.

15 recordsLinked to original sources

Familial interstitial nephropathy without hyperuricemia.

Progressive hereditary nephropathy is described in 6 members of a single family. Renal biopsies, performed in 3 patients, revealed tubular atrophy, interstitial fibrosis, and lymphomonocytic infiltration associated with severe vascular lesions. These features were disproportionately serious when related to age, arterial pressure, and renal function. Similar familial nephropathy has been reported in the literature, generally in association with gout or asymptomatic hyperuricemia. The patients described here had normal blood concentrations of uric acid. It is proposed that the members of the present group of patients are suffering from the same interstitial nephropathy as that described in the literature and that the hyperuricemia found by other investigators is coincidental and does not play a pathogenetic role.

Adult↗

Clinical and radiological features of bone disease in long-term (15 or more years) hemodialysis patients.

Fifteen patients on regular dialytic treatment for more than 15 years were given X-rays of the skull, spine, shoulders, wrists, pelvis and knees with the purpose of studying the principal skeletal and articular alterations due or not due to the uraemic status. Serum calcium, phosphorus, parathyroid hormone, alkaline phosphatase and basal aluminium were recorded. Osteopenia was evident in all the patients. Ten of whom (67%) showed alterations due to hyperparathyroidism. Nine patients presented the marks of dialysis spondyloarthropathy; in 14/15 cases geodes were present in the wrists, humeral heads or hip-joints; in ten patients there were multiple amyloid lesions. Two patients with serum basal aluminum above 100 micrograms/L showed the typical radiographic marks of osteomalacia. The majority of the long-term survivors showed multifactorial osteo-articular alterations resulting mainly from the combination of hyperparathyroidism and dialysis-related amyloidosis. The less frequent joint alterations were represented by arthrosis, enthesopathy and chondrocalcinosis. Disability and decreased articular mobility resulted in being mainly due to amyloid osteo-arthropathy.

Adult↗

Histological features of non-A, non-B hepatitis in hemodialysis patients.

In 12 hemodialysis (HD) patients with persistently raised serum alanine amino-transferase concentrations, 6 of which with anti-HCV antibodies, a liver biopsy was performed. The histological examination showed chronic persistent hepatitis or less significant changes in 11 patients and mild chronic active hepatitis in only 1. Non-A, non-B hepatitis seems to demonstrate in HD patients a low tendency to induce an active and progressive liver disease.

Aged↗

[Radiology of osteoarticular changes in patients undergoing periodic hemodialysis for more than 15 years].

Osteoarticular complications, which are characterized by osseous pain, pathologic fractures, and decreased articular mobility, represent one of the major problems affecting long-term (over 15 years) hemodialysis patients. These changes seem to have a multifactorial etiology; they include osteomalacia, secondary hyperparathyroidism, and dialysis-related amyloidosis. Ten patients (5 males and 5 females, mean age 55 +/- 7 years) on long-term (over 15 years) hemodialysis were submitted to X-ray examinations of the skull, spine, shoulders, wrists, pelvis, and knees. Serum calcium, phosphorous, parathyroid hormone, alkaline phosphatase, and basal aluminium levels were also calculated. Osteopenia was demonstrated in all patients. Seven of them had alterations due to hyperparathyroidism. Six patients exhibited signs related to dialysis spondyloarthropathy; in 9 cases amyloid lesions, geodes, and erosions were present in wrists, humeral heads, or hips. One patient exhibited osteomalacic changes. Most long-term dialysis patients presented multifactorial osteoarticular changes due to hyperparathyroidism, osteomalacia, and dialysis-related amyloidosis. Clinical symptoms and decreased articular mobility appeared to be due mainly to amyloid osteoarthropathy.

Adult↗

Henoch-Schönlein purpura after Salmonella hirschfeldii infection.

The authors present a case of Henoch-Schönlein purpura in a young soldier (19 years old) which they consider important for its etiology and the length of its oligoanuric phase. The syndrome followed a Salmonella hirschfeldii infection, and a protracted oligoanuric phase was followed by nephrotic syndrome and selective glomerular proteinuria which lasted for 1 year. The young man recovered after the eradication of the Salmonella. It seems possible that there was an overall anomalous regulation of the 'lymphoid system of the mucosa', perhaps dependent on a genetic predisposition.

Adult↗

Radiological features of dialysis amyloid spondyloarthropathy.

Nine patients undergoing regular dialytic treatment (RDT) for more than 60 months (mean 125 +/- 33 months) showed clinical and radiological evidence of non-infective destructive spondyloarthropathy (DSA). The cervical spine was the skeletal segment most affected (100% of cases). Three patients were found also to be suffering from discal and bone alterations of the dorsal column, and in two other patients the vertebral bodies L4-L5 were changed. Typical radiological pictures showed a narrowing of intervertebral spaces with the destruction or sclerosis of adjacent subchondral bones, erosions of vertebral body plates and cavitations. CT studies of the altered spines confirmed discal lesions and osteolytic areas with bone condensation at each level. Ligamentous lesions resulting in severe disorders of spinal statics were discovered during autopsy of three patients. Histological study of disc and peridiscal ligaments indicated the presence of large amyloid deposits containing beta-2-microglobulin (B2-m). It is possible that the minor biocompatibility of the cuprophan membrane of dialyzers is the most significant factor responsible for the hyperproduction of B2-m and thus of the osteo-articular deposition of a new type of amyloidosis.

Aged↗

Use of 3'HVR genomic probe for presymptomatic diagnosis of adult polycystic kidney disease in northern Italy: comparison of DNA analysis and renal ultrasonographic data.

A highly polymorphic DNA probe (3'HVR) with genetic linkage to the locus of autosomal dominant polycystic kidney disease was used for screening. Families with subjects at risk were from the Po river delta region (Northern Italy), where the disease accounts for 24% of the demands for dialysis. 3'HVR alleles were investigated in white blood cell DNA from 142 members of 18 families. The genomic marker was found informative in 88% of cases. Two recombinations between the marker and the disease locus were observed in 79 meioses. In 42 of the subjects at risk the results of DNA analysis and renal ultrasonography were compared. In 36 subjects the tests confirmed each other (18 were positive). In the other six subjects (all under 20 years of age and four under 10) only DNA analysis could diagnose the inheritance of cystic disease in the absence of demonstrable cysts. The findings indicate that in the population of the Po river delta the presymptomatic detection of adult polycystic kidney disease by 3'HVR linkage analysis is feasible in 88% of cases with approximately 95% reliability.

Adolescent↗

[Behavior and significance of phalangeal bone changes in uremic patients under periodic hemodialysis treatment].

Bone damage in hand phalanges has been evaluated with reference to age and duration of hemodialysis (on the basis of 248 radiological observations), in 93 cases with chronic renal failure (age: 20-59 years). These patients were on regular dialytic treatment (RDT) from 1 to 138 months. 72% of the patients underwent several periodic (annual) controls using the mammographic technique. The radiologic evaluations have been arranged into groups according to age. Skeletal damage was more evident when RDT was prolonged. Bone damage increases with age in the first 48 months; afterwards, on the contrary, bone changes were more evident in middle aged patients. At the beginning of RDT, acroosteolysis was the most important change always present. Both subperiosteal and intracortical resorption are more evident increasing age and duration of RDT. Radiological changes give a clear picture of the possible histo-morphologic pattern that characterizes uremic osteodystrophy.

Adult↗