1/30. Focal segmental glomerulosclerosis associated with mitochondrial cytopathy.BACKGROUND: The nonspecific lesion of focal segmental glomerulosclerosis (FSGS) can occur as a primary disease or in a variety of secondary settings. In mitochondrial cytopathies (MCs), the phenotypic expression of the disease depends on the degree of cellular dysfunction, and this correlates with the proportion of abnormal mitochondrial dna in the cells and the dependence of tissues on oxidative metabolism. The most common renal manifestation in MCs is tubular dysfunction; little has been reported about glomerular diseases. methods: Cases of four adult patients with FSGS and MC are reported. Routine histology and mitochondrial dna analysis were carried out on renal biopsies. RESULTS: family history and clinical manifestations in the four patients with FSGS suggested a diagnosis of MC. An A3243G transition in the mitochondrial dna tRNA(leu(UUR)) was found in lymphocytes and kidney. Glomerular lesions of FSGS were associated with unusual hyaline lesions, which appeared to represent individual myocyte necrosis in afferent arterioles and small arteries. CONCLUSION: FSGS is a renal manifestation of MCs. The renal lesion can precede other manifestations of the genetic disease by many years. The striking arteriolar lesions in these cases may have resulted in glomerular hypertension and hyperperfusion, leading to secondary epithelial cell abnormalities and, ultimately, FSGS. However, primary epithelial cell dysfunction caused by mitochondrial defects could not be ruled out on morphological grounds. MCs should be considered in cases of so-called primary FSGS, particularly if there is a familial history of diabetes, neuromuscular disorders, or deafness.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/30. Super long-term surviving two renal grafts with severe arteriolosclerosis and glomerulosclerosis.Two long-term renal transplant survivors, for about 20 yr, with unusual histological features in the allograft kidney are reported. In both cases, marked hyalinosis was observed in the arterioles of the transplanted kidney, despite never having been administered cyclosporine or tacrolimus. The cause remains unknown at the present time, but we think that the changes could be multifactorial in origin, including due to aging of the graft, hypertension, hyperlipidemia and chronic rejection. We conclude that histological analysis of the allograft kidney must be performed in long-term renal transplant survivors, in order to understand the histological changes in the chronic phase after kidney transplantation and to predict the prognosis of the graft.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
3/30. Human T-cell lymphotropic virus-1-associated renal disease in Jamaican children.This report documents the clinicopathological features in two Jamaican children who presented with infective dermatitis, glomerulonephritis, renal failure and human T-cell lymphotropic virus (HTLV-1) seropositivity. Severe hypertension with hypertensive encephalopathy was the most impressive clinical feature. Histological findings from renal biopsy specimens in both cases revealed significant glomerulosclerosis with fibrosis, chronic inflammatory cell infiltrates in the interstitium, and arteriolar hypertensive changes. Membranoproliferative glomerulonephritis (MPGN) was demonstrable in case 1 and marked focal glomerulosclerosis in case 2. Case 1 developed end stage renal failure and died within 3 years of diagnosis. Case 2 remains hypertensive and in chronic renal failure. Although a causal relationship between HTLV-1 infection and renal disease cannot be proven by these two cases, it appears that renal involvement in children with HTLV-1 infection is severe, with the potential for chronic renal failure and malignant hypertension. HTLV-1 nephropathy should be suspected in children with infective dermatitis and renal disease.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
4/30. proteinuria and focal segmental glomerulosclerosis in severely obese adolescents.OBJECTIVE: To describe the clinical and laboratory features of obesity associated proteinuria and focal segmental glomerulosclerosis. STUDY DESIGN: The patients were seen over a 12-year period at two large children's hospitals. Renal biopsies, performed for the diagnosis of unexplained heavy proteinuria and prepared for light, immunofluorescent, and electron microscopy, were read independently by two pediatric pathologists. blood pressure, body mass index, serum levels of creatinine, albumin, and cholesterol, and 24-hour urinary protein were measured. RESULTS: Seven African American adolescents were identified with obesity-associated proteinuria, which was characterized by severe obesity (120 /- 30 kg), markedly elevated body mass index (46 /- 11), mild hypertension (134/74 /- 10/18 mm Hg), slightly low to normal serum albumin levels (3.6 /- 0.2 g/dL), moderately elevated serum cholesterol levels (196 /- 60 mg/dL), and elevated 24-hour protein excretion (3.1 /- 1.3 g/dL). Calculated creatinine clearance was normal in 6 patients and decreased in one. Typical renal histologic features included glomerular hypertrophy, focal segmental glomerulosclerosis, increased mesangial matrix and cellularity, relative preservation of foot process morphology, and absence of evidence of inflammatory or immune-mediated pathogenesis. One patient showed a dramatic reduction in proteinuria in response to weight reduction. Three patients who were given angiotensin-converting enzyme inhibitors had reduced urinary protein losses from 2.9 g to 0.7 g per day. One patient developed end-stage renal disease. CONCLUSION: Obese adolescents should be monitored for proteinuria, which has distinct clinical and pathologic features and may be associated with significant renal sequelae. Such proteinuria may respond to weight reduction and/or treatment with angiotensin-converting enzyme inhibitors.- - - - - - - - - - ranking = 1.0110003783783keywords = hypertension, pressure (Clic here for more details about this article) |
5/30. Pseudomembranous candidosis in nephrotic syndrome: a case report.A 33-year-old male presented for evaluation of several large, recently discovered white oral lesions of unknown duration. Clinical examination revealed multiple white plaques on the soft palate, uvula, buccal mucosa, and tongue. These lesions could be wiped away, leaving an erythematous base. The lesions were asymptomatic, and the patient did not report difficulty in swallowing. The patient's medical history was noteworthy for several significant diagnoses within the previous 6 months: type 2 diabetes mellitus, mild systolic hypertension, gastroesophageal reflux disease, and adult idiopathic nephrotic syndrome, determined by kidney biopsy to be caused by focal segmental glomerulosclerosis. A provisional diagnosis of pseudomembraneous candidosis was made, and the patient responded to a 14-day course of clotrimazole, administered in 10-mg troches, five times a day. Management of nephrotic syndrome predisposes patients to recurrent fungal infections, and the disease has implications for the selection of systemic antifungal agents.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
6/30. Refractory hypertension and anemia in end-stage renal disease: an unusual manifestation of Kimura's disease.hypertension and anemia are common in uremic patients. This article describes a 35-year-old uremic Taiwanese who was admitted to our hospital with refractory hypertension and refractory anemia following chronic hemodialysis for more than two years. He was diagnosed with Kimura's disease finally. Refractory hypertension and refractory anemia were noted over two years before an enlarged inguinal lymph node was observed. The symptoms lead to the diagnosis of Kimura's disease. Unlike most cases, refractory hypertension and refractory anemia were first noted before the inguinal mass and eosinophilia were presented. The inflammatory parameters increased when the disease was active. Steroid treatment was conducted, and the symptoms including hypertension and anemia promptly decreased. To the authors' knowledge, this case is for first one in which Kimura's disease has induced refractory hypertension and anemia in an ESRD patient and in which these symptoms rapidly subsided following steroid treatment. The activity of Kimura's disease is closely related refractory hypertension, suggesting that inflammation may be involved in refractory hypertension and anemia in a dialytic patient with Kimura's disease.- - - - - - - - - - ranking = 11keywords = hypertension (Clic here for more details about this article) |
7/30. Focal segmental glomerulosclerosis in a patient with large bilateral asymptomatic adrenal myelolipomas.Adrenal myelolipomas are rare benign tumors, usually discovered by chance in patients with hypertension, obesity or various endocrine disorders. Focal segmental glomerulosclerosis (FSGS) can occur as a primary disease or in a variety of secondary settings. So far, no association between the two conditions has been described. We report a case of a woman admitted for nephrotic syndrome, in which a coexistence of FSGS and bilateral large adrenal myelolipomas was revealed.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
8/30. Cardiac disease in children with primary glomerular disorders-role of focal segmental glomerulosclerosis.Anecdotal reports suggest a higher frequency of serious cardiac complications, particularly cardiomyopathy and congestive heart failure (CHF), in children with focal segmental glomerulosclerosis (FSGS). We report the occurrence of cardiac disease in children with FSGS compared with other glomerular causes of primary nephrotic syndrome (NS). A chart review was performed on all patients evaluated at the Schneider Children's Hospital between 1985 and 2003 with a diagnosis of membranoproliferative glomerulonephritis (MPGN), membranous nephropathy (MN), focal global glomerulosclerosis (FGGS), and FSGS. Clinical and demographic data were compiled, specifically whether or not the patient had clinically evident cardiac disease. The blood pressure (BP) and hematocrit in patients with FSGS and chronic renal failure (CRF) (glomerular filtration rate <30 ml/min per 1.73 m(2)) in the 3 months prior to the development of cardiac complications were compared with the values in FSGS patients with CRF but no cardiac complications, and in patients with the other causes of primary NS in whom CRF developed. There were 48 patients with FSGS, 22 with MPGN, 19 with MN, and 4 with FGGS. Cardiac disease occurred in 6 children (mean age 11 years), all with FSGS. Four of these patients were black and 5 were female. CHF occurred in all patients, cardiomyopathy in 4, and left ventricular hypertrophy in 5 patients. There was no significant difference in the BP and the hematocrit levels between the 6 patients with both FSGS and cardiac disease, 3 patients with FSGS and CRF but no cardiac disease, and the 5 patients with the other glomerulopathies in whom CRF occurred ( P>0.1). Our findings suggest that there is a clinical association between FSGS and cardiac disease in pediatric patients. We speculate that the immune mechanism responsible for the development of FSGS may also affect the heart.- - - - - - - - - - ranking = 0.35400854628339keywords = blood pressure, pressure (Clic here for more details about this article) |
9/30. Comparative renal histomorphometry: a case study of oligonephropathy of prematurity.Children born with very low birth weight have a decreased nephron number. Low nephron mass is associated with adult hypertension, proteinuria, and diabetes mellitus. The histomorphometry and radial glomerular count (RGC) of a total nephrectomy from a child with renal disease associated with extreme prematurity was compared with the kidney from a full-term age-matched child of normal gestation with chronic renal failure due to focal and segmental glomerulosclerosis (FSGS) and to a child without renal disease. Bowman's space area, mesangium and mesangial tuft area were determined in 50 glomeruli of each specimen by computer-assisted morphometry. RGC was 4 in the ex-preterm child, 8 in the patient with FSGS, and 9 in normal control. The patient with FSGS had larger glomerular area expressed as square micrometers (mum(2)) of Bowman's capsule, the mesangium and the mesangial tuft area measurements than the normal control and the child born preterm who subsequently developed renal failure had significantly larger Bowman's capsule and mesangium than the two controls. This case report begins to identify important pathologic findings of decreased nephron numbers and glomerulomegaly associated with preterm birth.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
10/30. peritoneal dialysis patient completes successful pregnancy.This patient was able to meet seven of the expected outcomes. Her weight and nutritional status improved after initiating CAPD, which she performed safely and effectively. She closely monitored her vital signs and appropriately notified the obstetrician when her blood pressure became elevated. She maintained proper fluid balance. The patient was successfully treated for exit site infection with antibiotics. She self-administered intraperitoneal antibiotics for peritonitis successfully. In spite of the infections, hypertension, and poor nutrition, the patient was able to complete her pregnancy to the 35th week and delivered a small, but healthy infant. Frequent monitoring and a team approach to S.B.'s well-being contributed greatly to her delivery of a viable infant. The education and training provided by the nephrology nurses was a key element in the successful management of the patient.- - - - - - - - - - ranking = 1.3540085462834keywords = hypertension, blood pressure, pressure (Clic here for more details about this article) |
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