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)

We analyzed 137 cases of renal biopsies at the Department of Pathology from 2001 to 2007. The average age was 30.6 years (range, 13-72) for males and 32.9 years (range, 11-75) for females. The male to female ratio was 1.6: 1. The most common clinical syndromes for performing renal biopsy were nephrotic syndrome (81.6%) followed by asymptomatic urinary abnormalities (5.8%), nephritic syndrome (3.6%), hypertension (2.9%), chronic kidney disease (2.2%), acute renal failure (2.2%), cirrhosis (0.7%) and transplant rejection (0.7%). The major glomerular diseases in descending order of frequency were membranous glomerulonephritis (MGN; 42.3%), membranoproliferative glomerulonephritis (MPGN; 21.9%), minimal change disease (MCD; 10.2%), focal segmental glomerulosclerosis (FSGS; 8.0%), IgA nephropathy (2.9%), post infectious glomerulonephritis (GN) (2.2%), chronic GN (2.2%), tubulointerstitial nephritis (TIN; 1.5%), lupus nephritis (1.5%), focal proliferative GN (1.5%), Clq nephropathy (1.5%), amyloidosis (1.5%) and other minor form of glomerular diseases (2.8%). The pattern ofglomerulonephritis in our study is different from the reports of other developing countries. It could be due to various races and altered environmental condition. The information obtained from these results can be used as baseline data for making efficient research in Nepalese population in the future. The higher frequency of MGN and MPGN among Nepalese in comparison with other developing countries deserves further evaluation.
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PMID:Hisopathological spectrum of glomerular disease in nepal: a seven-year retrospective study. 1882 37

Nephrotic syndrome presenting in the first year of life is often challenging, with substantial risk of progression to end-stage renal disease (ESRD). Focal segmental glomerulosclerosis (FSGS) comprises up to 20% of biopsy-proven glomerular disease in children and adults. We report on a 9-month-old infant who presented with nephrotic syndrome, hypertension and progressive deterioration of renal function due to FSGS. As immunosuppressive agents are often unsuccessful in treating this condition, we adopted renoprotection as the mainstay treatment for this patient, through rigorous control of blood pressure and proteinuria with a multi-drug regimen including renin-angiotensin axis blockade. Initially, there was partial improvement, with a gradual decline in proteinuria and a concomitant rise in the glomerular filtration rate, before the disease eventually passed into complete clinical and laboratory remission. We speculate that infants with steroid-resistant nephrotic syndrome due to FSGS may benefit from tight control of hypertension, mainly though early blockade of the renin-angiotensin axis. We believe that its renoprotecive mechanism counteracts the deleterious effects of both hypertension and proteinuria, thereby not only preventing progressive renal disease, but even paving the way for a remission, as in our patient. To the best of our knowledge, this is the first report of an infant with nephrotic syndrome (NS) due to FSGS that passed into complete remission while the patient was on renoprotective measures including the use of angiotensin-converting enzyme inhibitors (ACEis).
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PMID:Complete remission of nephrotic syndrome in an infant with focal segmental glomerulosclerosis: is it renin-angiotensin blockade? 1885 98

Focal and segmental glomerulosclerosis (FSGS) is common and non-specific patterns of glomerular injury encountered in human renal biopsies. Cortico-resistant nephrotic syndrome is the main manifestation. We report epidemiological, clinical and pathological aspects of FSGS in Dakar. We report the results of a retrospective study about focal segmental glomerulosclerosis (FSGS) identified from 258 kidney biopsies performed in the medical clinic 1 of A. Le Dantec hospital from January 1993 to December 2003. FSG is found in 134 cases (52%), membranous glomerulonephritis in 32 cases (12,4%), minimal change disease in 20 cases (7.7%). Ninety eigths files were exploitable. FSGS has male gender predominance with a sex ratio of 3. Median age of patients is 28 years (15 and 79 years). Symptomatology is dominated by oedema in 86 cases (87,7%), hypertension in 12 cases (12.2%), hematuria in 5 cases (5.1%), nephrotic proteinuria in 65 cases (66,3%) and no nephrotic proteinuria in 33 cases (33.6%), renal failure in 25 cases (25%)and leucocyturia in 18 cases (18%). FSGS involving more than 50% of glomeruli is encountered in 41 cases (42%), severe interstitial fibrosis is associated in 26 cases. Different pathological aspects are: classical FSGS in 88 cases (88.7%), FSGS " collapsing" in 7 cases (7.1%), FSG "tip-lesion" in one case, FSGS associated to membranous glomerulosclerosis in 2 cases and to diabetic glomerulosclerosis in one case. FSGS is primitive in 88 cases (89,8%) and secondary in 10 cases (10.2%). FSGS is the most common primitive glomerulopathy in Dakar. Nephrotic syndrome is the main manifestation of this disease. Collapsing FSGS is not correlated with the HIV Infection.
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PMID:[Nephrotic syndrom with focal and segmental glomerulosclerosis in Dakar: epidemiological and clinicopathological characteristics (about 134 cases)]. 1910 17

Corticosteroid immunosuppression has permitted the development of successful allotransplantation; however, corticosteroids are associated significant post-transplant complications. To circumvent these problems, we implemented a protocol of rapid discontinuation of corticosteroids in 19 consecutive pediatric primary kidney transplant recipients. Mean age at time of transplant was 13.4 (+/-4.5) yr, 52.6% were male, 63.2% underwent living donor transplantation. All patients were administered Thymoglobulin [anti-thymocyte globulin (rabbit)] as induction immunosuppression with a rapid tapering dose of corticosteroids (total of five daily doses), and maintained on mycophenolate mofetil and tacrolimus. Two patients had immediate recurrence of primary disease (FSGS), requiring further corticosteroid therapy. Otherwise, remaining 17 patients were maintained off corticosteroids, with excellent graft function; mean baseline eGFR of 112 mL/min/1.73 m(2) (+/-19) at 28 months (+/-14) post-transplantation. There was 100% patient and rejection-free graft survival at 27 months (range 5-58 months) post-transplantation; 47% underwent renal transplant biopsy secondary to acute rise in serum creatinine with or without worsening hypertension. All biopsies had no evidence of acute rejection; 62.5% had findings consistent with tacrolimus toxicity. Renal transplantation utilizing a rapid discontinuation of corticosteroid protocol in pediatric patients appears to be safe and effective, without increasing the risk of acute rejection or graft loss.
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PMID:Rapid discontinuation of corticosteroids in pediatric renal transplantation. 1941 17

Podocin is a critical component of the glomerular slit diaphragm, and genetic mutations lead to both familial and sporadic forms of steroid-resistant nephrotic syndrome. In mice, constitutive absence of podocin leads to rapidly progressive renal disease characterized by mesangiolysis and/or mesangial sclerosis and nephrotic syndrome. Using established Cre-loxP technology, we inactivated podocin in the adult mouse kidney in a podocyte-specific manner. Progressive loss of podocin in the glomerulus recapitulated albuminuria, hypercholesterolemia, hypertension, and renal failure seen in nephrotic syndrome in humans. Lesions of FSGS appeared after 4 wk, with subsequent development of diffuse glomerulosclerosis and tubulointerstitial damage. Interestingly, conditional inactivation of podocin at birth resulted in a gradient of glomerular lesions, including mesangial proliferation, demonstrating a developmental stage dependence of renal histologic patterns of injury. The development of significant albuminuria in this model occurred only after early and focal foot process effacement had progressed to diffuse involvement, with complete absence of podocin immunolabeling at the slit diaphragm. Finally, we identified novel potential mediators and perturbed molecular pathways, including cellular proliferation, in the course of progression of renal disease leading to glomerulosclerosis, using global gene expression profiling.
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PMID:Podocin inactivation in mature kidneys causes focal segmental glomerulosclerosis and nephrotic syndrome. 1976 89

Focal and segmental glomerulosclerosis (FSGS) is a major cause of idiopathic steroid-resistant nephrotic syndrome (SRNS) and end-stage renal disease (ESRD). In this retrospective study, we report on 34 pediatric patients with FSGS who were diagnosed and treated from 1992 to 2006. The mean age at onset was 6.3 + or - 4.3 years. All patients had nephrotic-range proteinuria. Microscopic hematuria was seen in three patients and hypertension was seen in 15 patients at presentation. All patients were treated with steroids (oral and/or methylprednisolone), while 23 patients received cytotoxic therapy in addition. The mean follow-up period was 8.6 + or - 3.3 years at the end of which, 59% of patients achieved complete or partial remission, 20.5% continued to have active renal disease while 20.5% of the patients developed CKD. Our study suggests that most of the patients with FSGS progress to renal insufficiency. Steroid therapy increases the chances of remission and preserves renal function in patients with sporadic primary FSGS.
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PMID:The long-term results of pediatric patients with primary focal and segmental glomerulosclerosis. 2006 99

Our study aimed to obtain a comprehensive insight into the etiology of nephrotic syndrome in our patient population. We analyzed medical records of 290 patients with diagnosis of nephrotic syndrome as defined by International Study of Kidney Disease in Children (ISKDC), between January 1987 and December 2000, at the Sher-I-Kashmir Institute of Medical Sciences, Soura, Srinagar. Primary glomerular disease was found to be the most prevalent, accounting for 91.73% of all glomerular diseases. Among primary glomerular diseases, minimal change disease (MCD) was the most common histological lesion (43.79%). Most patients presented within 3 months duration (61.4%) and the most common symptom was puffiness of face (98.45%) followed by pedal edema (91%). Focal segmental glomerulosclerosis (FSGS) was the second most common lesion (16.89%) followed by membranous glomerulonephritis (GN) (13.4%) and membranoproliferative GN (11.72%). Amongst secondary glomerular diseases, diabetes mellitus was the most prevalent (4.48%), followed by lupus nephritis (3.1%). In conclusion, primary glomerular diseases constituted the most common group encountered and the prevalence of MCD was quite high with males, children and young adults. FSGS was associated with a high prevalence of end-stage renal disease (ESRD; 26.53%), hypertension (71.42%) and hematuria (81.63%).
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PMID:Etiological profile of nephrotic syndrome in Kashmir. 2036 13

Mapping by admixture linkage disequilibrium (LD) detected strong association between nonmuscle myosin heavy chain 9 gene (MYH9) variants on chromosome 22 and nondiabetic nephropathy in African Americans. MYH9-related variants were posited to be the probable, but not necessarily the definitive, causal variants as a result of impressive statistical evidence of association, renal expression, and a role in autosomal dominant MYH9 disorders characterized by progressive glomerulosclerosis (Epstein and Fechtner syndromes). Dense mapping within MYH9 revealed striking LD patterns and racial variation in risk allele frequencies, suggesting population genetic factors such as selection may be operative in this region. Genovese and colleagues examined large chromosomal regions adjacent to MYH9 using genome-wide association methods and non-HapMap single nucleotide polymorphisms identified in Yoruba from the 1000 Genomes project. Statistically stronger associations were detected between two independent sequence variants in the Apolipoprotein L1 gene (APOL1) and nondiabetic nephropathy in African Americans, with odds ratios of 10.5 in idiopathic FSGS and 7.3 in hypertension-attributed ESRD. These kidney disease risk variants likely rose to high frequency in Africa because they confer resistance to trypanosomal infection and protect from African sleeping sickness. Risk variants in MYH9 and APOL1 are in strong LD, and the genetic risk that was previously attributed to MYH9 may reside, in part or in whole, in APOL1, although more complex models of risk cannot be excluded. This association likely explains racial disparities in nondiabetic nephropathy as a result of the high prevalence of risk alleles in individuals of African ancestry.
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PMID:The apolipoprotein L1 (APOL1) gene and nondiabetic nephropathy in African Americans. 2068 34

Urinary tract infections (UTI) are common in childhood. Presence of pyuria and bacteriuria in an appropriately collected urine sample are diagnostic of UTI. The risk of UTI is increased with an underlying urological abnormality such as vesicoureteral reflux, constipation, and voiding dysfunction. Patients with acute pyelonephritis are at risk of renal scarring and subsequent complications such as hypertension, proteinuria with and without FSGS, pregnancy-related complications and even end-stage renal failure. The relevance and the sequence of the renal imaging following initial UTI, and the role of antimicrobial prophylaxis and surgical intervention are currently undergoing an intense debate. Prompt treatment of UTI and appropriate follow-up of those at increased risk of recurrence and/or renal scarring are important.
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PMID:Managing urinary tract infections. 2140 31

Focal segmental glomerulosclerosis (FSGS) is an important clinical problem as it leads to end-stage renal disease. Clinicians have long been able to treat patients with FSGS. Therefore, the demands the clinicians make on pathomorphologists, which include the diagnosis of FSGS at a possibly early stage, are justifiable. However, early diagnosis of FSGS is difficult. The analysis involved 150 cases of FSGS diagnosed between 2003 and 2008. These constitute 14.53% of renal biopsy material of that period. The test material comes from 138 adults and 12 children. The adult group mostly included patients with albuminuria (58 patients) and nephrotic syndrome (36 patients). Smaller groups included patients with albuminuria and hypertension, erythrocyturia and albuminuria, isolated erythrocyturia. The children group mostly included patients with the nephrotic syndrome. Individual patients suffered from isolated albuminuria and erythrocyturia. In both groups, FSGS NOS lesions prevailed. However, FSGS hilar and FSGS tip lesions, as well as completely sclerotized glomeruli were also present. Diverse symptoms of diseases may pose specific difficulties in clinical diagnosis. Similarly, determination of FSGS lesion type may be difficult due to simultaneous presence of different subtypes in the same punctate.
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PMID:Focal segmental glomerulosclerosis: a diagnostic problem. 2253 7


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