1/18. Idiopathic multicentric osteolysis with nephropathy.Idiopathic multicentric osteolysis is a rare syndrome that manifests with progressive loss of carpal and tarsal bones in childhood. Affected children have arthritic-like episodes, followed by progressive deformities, radiographic osteolytic changes, and variable degrees of disability. A rare form of this disease (type III, sporadic) is associated with serious nephropathy. We present the first reported case of type III idiopathic multicentric osteolysis in a Chinese woman. The patient, a 34-year-old woman with normal mental development and no family history of bone or kidney disease, presented with a 4-day history of nausea and vomiting. She had shortening and swelling of the hands, which had occurred in childhood and persisted at the time of admission. X-ray studies showed disappearance of the carpal bones, and multiple osseous erosions of the tarsal bones. hypertension, severe azotemia, and metabolic acidosis were also noted. Advanced renal disease was documented after a series of investigations, including renal biopsy. She is now dialysis-dependent. This case illustrates the importance of early diagnosis and management of idiopathic multicentric osteolysis with nephropathy.- - - - - - - - - - ranking = 1keywords = azotemia (Clic here for more details about this article) |
2/18. Renal sarcoidosis with superimposed postinfectious glomerulonephritis presenting as acute renal failure.We describe two patients with sarcoidosis with lesions of granulomatous interstitial nephritis (GIN) and postinfectious glomerulonephritis (GN). Both patients presented with heavy proteinuria, hematuria, and renal failure. Renal histology in both showed GIN and glomerular changes of proliferative GN with hump-like subepithelial deposits by electron microscopy of postinfectious GN. Antecedent history of pneumonia was present in one, and ASO titer was elevated in the other. The proteinuria and azotemia improved in both with steroid therapy. Reports of "postinfectious" or diffuse proliferative GN in patients with sarcoidosis are rare. The authors are unaware of reports of concomitant sarcoid GIN and postinfectious GN. Although acute renal insufficiency or failure can occur with GIN or other more common renal lesions primary glomerular disease should be considered in patients with sarcoidosis who present with renal dysfunction. This is a US government work. There are no restrictions on its use.- - - - - - - - - - ranking = 1keywords = azotemia (Clic here for more details about this article) |
3/18. New syndrome involving the visual, auditory, respiratory, gastrointestinal, and renal systems.A previously undescribed fatal multisystem syndrome involving the eyes, ears, lungs, intestines, and kidneys occurred in sibs. They both presented during early childhood with cataracts, otitis media, intestinal malabsorption, chronic respiratory infection, and failure to thrive. Later, they developed recurrent pneumonia (one was shown to have immotile bronchial cilia) and progressive azotemia leading to end-stage renal disease (ESRD) by late childhood. Both died of overwhelming infection (sepsis, meningitis). An autosomal recessive mode of inheritance is proposed since the normal parents were distant cousins, and 4 other sibs were normal.- - - - - - - - - - ranking = 1keywords = azotemia (Clic here for more details about this article) |
4/18. Familial juvenile nephronophthisis in two siblings--histological findings at an early stage.We present two female siblings with familial juvenile nephronophthisis (FJN) which was diagnosed at the early stage of renal failure. Diagnosis was made during the investigation of anemia in case 1 and by a subsequent family survey in case 2. Most patients with FJN are not identified until the terminal stage of renal failure and such cases have rarely been reported in japan. Case 2 had a reduction in the maximum urinary concentration ability but no azotemia, and among the FJN patients reported in japan so far she has the least advanced renal disease. Histological examination of the renal biopsy in case 1 showed typical findings of FJN, such as thickening and lamination of the tubular basement membrane (TBM), interstitial fibrosis, and round cell infiltration of the interstitium. In case 2, renal biopsy revealed an irregular marked thickening of the TBM with trivial interstitial changes and a normal glomerular appearance. The histology of these two cases suggests that the TBM may be the primary site affected in FJN.- - - - - - - - - - ranking = 1keywords = azotemia (Clic here for more details about this article) |
5/18. Pulmonary and renal toxicity of intravenous immunoglobulin.Three patients treated with intravenous immunoglobulin developed respiratory difficulty and decreased renal function. Previously reported adverse reactions to this class of drugs have included pulmonary toxicity but not, to the best of our knowledge, renal toxicity. Renal dysfunction was mild in one patient but severe in two patients, one of whom required temporary hemodialysis. In all three patients, renal function returned to the pretreatment level after stopping the drug. urinalysis and urine sodium concentration at the onset of renal failure were consistent with pre renal azotemia although renal biopsy performed later in one patient showed acute tubular necrosis. The exact mechanism of toxicity is unknown, but the fact that all three cases occurred with a particular immunoglobulin preparation would suggest that a contaminant, possibly aggregated IgG might be responsible.- - - - - - - - - - ranking = 1keywords = azotemia (Clic here for more details about this article) |
6/18. Renal involvement in patients with thrombotic thrombocytopenic purpura.A retrospective review of the records of 15 patients with thrombotic thrombocytopenic purpura (TTP) was performed to determine the spectrum of renal involvement that occurs in this disease. All cases exhibited some evidence of renal involvement, the most common manifestation of which was an abnormal urinalysis. Twelve cases (80%) had some degree of elevation of the serum urea nitrogen (SUN) or creatinine level at some time during the course of their disease. Renal involvement could be categorized into three types depending on the severity of TTP. In those cases presenting as an acute devastating illness, renal insufficiency, when present, was severe and a dominant component of the disease. In those that pursued a more protracted course, with subsequent acute exacerbations of TTP, renal insufficiency was variable and less severe than in the first group. In those presenting with a mild form of TTP, renal involvement consisted primarily of an abnormal urine sediment and azotemia that corrected rapidly following fluid replacement. A review of the literature beginning with 1966, when renal disease was established as part of the features that characterize TTP, provided 216 cases in whom sufficient data on renal involvement were recorded. Of the 168 cases where urinalysis was reported, hematuria was noted in 78% of the cases, proteinuria in 75, pyuria in 31, and cylindriuria in 24%. Of the 181 cases where the admission SUN was reported, it was higher than 20 mg/dl in 69% and greater than 60 mg/dl in 17% of the cases. The level of SUN was a significant determinant of the final prognosis of these patients.- - - - - - - - - - ranking = 1keywords = azotemia (Clic here for more details about this article) |
7/18. Transient paradoxical renal vasoconstriction following cardiac operation. Treatment with volume depletion.Following cardiac operation complicated by inferior myocardial injury, a patient developed normal cardiac output congestive heart failure associated with severe renal vasoconstriction, oliguria and azotemia. The patient's renal dysfunction responded to volume depletion with hemofiltration. These paradoxical renal responses to volume changes may be caused by transiently altered cardiac volume receptor thresholds or afferent signals resulting in cardiorenal dysfunction.- - - - - - - - - - ranking = 1keywords = azotemia (Clic here for more details about this article) |
8/18. Muscular, renal, and metabolic complications of acute arterial occlusions: myonephropathic-metabolic syndrome.Acute arterial occlusions of the extremities may result, in approximately 7.5% of cases, in a severe and complex metabolic syndrome which often leads to loss of limb and life. The manifestations of this syndrome are divided into two stages: (1) the ischemic or devascularization phase, and (2) the revascularization phase. The ischemic phase includes severe clinical manifestations, of which the rigidity of the limb ("rigor mortis") is an outstanding sign, as are nephropathic-metabolic changes (oliguria, acidosis, myoglobinuria, azotemia, hyperkalemia). Their identification and correction at this phase may minimize their impact on the revascularization syndrome. The clinical and metabolic manifestations during the latter phase are more severe and may determine the outcome of the viability of the limb and the survival of the patient. amputation rates are quite high (40% to 50%) and mortality rates range between 30% and 80%. The ischemic rhabdomyolysis appears to be the initiating event which leads to the biochemical and metabolic alterations that dominate the prognosis as to limb and life. The guiding principles of the management in these severe ischemic cases consist of early revascularization with emphasis on concurrent fasciotomy, alkalinization of the patient, reestablishment of acid-base balance, hemodialysis for renal shutdown, and often early amputation for better control of the metabolic omplications.- - - - - - - - - - ranking = 1keywords = azotemia (Clic here for more details about this article) |
9/18. Nephronophthisis with massive proteinuria.A 23-year-old male whose uncle died of nephronophthisis, and whose pathology is also discussed, presented with 5 g of protein in a 24-hour urine collection. Nephrogenic diabetes insipidus and salt wasting were present in addition to azotemia. Characterization of the proteinuria, including elevated alpha globulins by electrophoresis and markedly elevated urinary beta-microglobulins by radioimmunoassay (49.55 mg/L) indicated predominantly tubular proteinuria. A percutaneous renal biopsy showed normal glomeruli, interstitial inflammation and fibrosis, and tubular atrophy. Electron microscopy revealed notable alterations of the tubular basement membrane.- - - - - - - - - - ranking = 1keywords = azotemia (Clic here for more details about this article) |
10/18. captopril. Long-term treatment of hypertension in a preterm infant and in older children.Five children with high-renin hypertension unresponsive to conventional medications were effectively treated with the angiotensin i-converting enzyme inhibitor, captopril, for six to 26 months. age groups included children 6 to 13 years old and a 33-week gestation preterm infant with hypertension related to umbilical artery catheterization. Significant adverse reactions were limited to transient renal insufficiency in the preterm infant and increased azotemia in an older child with chronic renal failure.- - - - - - - - - - ranking = 1keywords = azotemia (Clic here for more details about this article) |
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