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Focal Segmental Glomerulosclerosis

FSGS


Presentation

  • Patrickharrington@doctors.org.uk Abstract The authors present the case of a patient who presented to the nephrology department of a district general hospital with end-stage renal failure.[ncbi.nlm.nih.gov]
  • This case also signifies that chylous ascites may be a rare presenting feature of nephrotic syndrome and hence this aspect should be considered while in diagnostic dilemma regarding such a clinical presentation.[ncbi.nlm.nih.gov]
  • The clinical picture in those who present with nephrotic syndrome is almost indistinguishable from those with minimal change nephrotic syndrome (MCNS).[accessmedicine.mhmedical.com]
  • We present the first report of immunoglobulin A nephropathy (IgAN) and associated focal segmental glomerulosclerosis in a patient with asymptomatic low grade B-cell lymphoma.[anzsnasm.com]
  • CASE PRESENTATION: A 53-year-old Caucasian man, previously healthy, with no history of hypertension, alcohol use or smoking presented with rapid weight gain, massive peripheral oedema, and hypertension.[ncbi.nlm.nih.gov]
Weight Gain
  • He was mildly overweight (BMI 26.9 kg/m2) due to an increased weight gain (10 kg) over the past year.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: A 53-year-old Caucasian man, previously healthy, with no history of hypertension, alcohol use or smoking presented with rapid weight gain, massive peripheral oedema, and hypertension.[ncbi.nlm.nih.gov]
  • When symptoms are present the most common include: Proteinuria – Large amounts of protein “spilling” into the urine Edema – Swelling in parts of the body, most noticeable around the eyes, hands and feet, and abdomen which causes sudden weight gain.[raredr.com]
  • Symptoms In children and some adults, FSGS presents as a nephrotic syndrome, which is characterized by edema (associated with weight gain), hypoalbuminemia (low serum albumin, a protein in the blood), hyperlipidemia and hypertension (high blood pressure[checkrare.com]
  • Over time and as the disease progresses the following symptoms may occur: Foamy urine (from excess protein in the urine) Poor appetite Swelling of the hands, feet, legs, abdomen and around the eyes Pain from swelling Weight gain 03 Diagnosis Several tests[curascriptsd.com]
Pleural Effusion
  • Furthermore, an accumulation of pleural effusion was confirmed by chest X-ray.[ncbi.nlm.nih.gov]
  • Pleural effusion and ascites may be present; pericardial effusions are rare. Gross edema may predispose patients to ulcerations and infections in dependent areas, such as the lower extremity.[physio-pedia.com]
  • Pleural effusion (fluid in the lung cavity) and ascites (fluid in the abdomen) may occur. Pericardial effusions are rare. Rarely, patients experience severe renal failure.[naturallivingcenter.net]
Saddle Nose
  • External anomalies included ocular hypertelorism, saddle nose, elongated philtrum, tent-like lips, and low-set auricles. Mental retardation was evident. The karyotype was 46,XX,del(6) (p.22.1-p22.3) with an interstitial deletion.[ncbi.nlm.nih.gov]
Pneumonia
  • Opportunistic infections with rituximab have not been studied extensively, but recent studies suggest an increased risk for Pneumocystis jirovecii pneumonia (PJP) after rituximab therapy.[ncbi.nlm.nih.gov]
Abdominal Pain
  • Abdominal pain, a common finding in children, may be a sign of peritonitis. Rarely, xanthomas may be evident in association with severe hyperlipidemia.[physio-pedia.com]
  • At 12 months, he started to experience abdominal pain, diarrhoea, vomiting and increased amylase.[revistanefrologia.com]
  • pain because of cyst enlargement and hemorrhage and hematuria “dragging sensation” - also seen in ovarian masses Chronic renal failure in 50% of patients by age 60 HTN accelerates the process M F, african americans Increased incidence of kidney stones[sites.google.com]
Hypertension
  • We report an unusual association of PRES and malignant hypertension secondary to focal segmental glomerulosclerosis in a young woman, presenting with sudden loss of vision and seizures.[ncbi.nlm.nih.gov]
  • A renal biopsy revealed he suffered from focal segmental glomerulosclerosis (FSGS), possibly due to obesity and hypertension, not CVID - associated MPGN. CONCLUSION: This is the first case report of FSGS in a CVID patient.[ncbi.nlm.nih.gov]
  • The type with nodular lesions is also called Kimmelstiel-Wilson syndrome . focal segmental glomerulosclerosis focal sclerosing lesions of the renal glomeruli, with proteinuria, hematuria, hypertension, and the nephrotic syndrome ; it may be idiopathic[medical-dictionary.thefreedictionary.com]
  • CASE PRESENTATION: A 53-year-old Caucasian man, previously healthy, with no history of hypertension, alcohol use or smoking presented with rapid weight gain, massive peripheral oedema, and hypertension.[ncbi.nlm.nih.gov]
  • Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) are medications known to reduce proteinuria and are effective even in people without hypertension.[davita.com]
Yellow Nails
  • Intestinal lymphangiectasia in a background of chylous ascites and without any lymphatic obstruction has been reported in association with yellow nail syndrome, which is a rare clinical occurrence in itself.[ncbi.nlm.nih.gov]
Kidney Failure
  • If kidney failure does occur, treatment options are available.[davita.com]
  • In other cases kidney failure can develop which may give tiredness or sickness if kidney failure is very advanced. What causes FSGS? Basically doctors do not know fully.[kidney.org.uk]
  • Over time, the damage to the kidney may cause kidney failure. FSGS is only one of many causes of kidney failure or end-stage renal disease (ESRD). Most people affected by FSGS do NOT have a family history of kidney disease.[dmpi.duke.edu]
  • The goal of treatment is to control the symptoms of nephrotic syndrome and prevent chronic kidney failure.[medlineplus.gov]

Workup

  • She had been diagnosed with streak ovaries during diagnostic workup for infertility. Mutational analysis identified the heterozygous mutation c.1372C T (p.Arg458*) in WT1 and the heterozygous non-neutral polymorphism c.868G A (p.Arg229Gln) in NPHS2.[ncbi.nlm.nih.gov]
  • An extensive diagnostic workup revealed POEMS syndrome. Serum concentrations of vascular endothelial growth factor (VEGF) were highly elevated, consistent with POEMS syndrome.[ncbi.nlm.nih.gov]
  • As workup for proteinuria, a renal biopsy sample was obtained. EM showed multiple crystalline depositions in renal tubular cells and podocytes. Focal segmental glomerulosclerosis with crystalloid podocytopathy was diagnosed.[ncbi.nlm.nih.gov]
  • Figure 2: Pre-transplant workup and management of patients with FSGS [3].[medcraveonline.com]
Glycosuria
  • Hematuria, evidence of tubular dysfunction such as glycosuria, hypertension, and mild azotemia, may be present in 15–30% of patients with new-onset nephrotic syndrome and these features may increase the clinical suspicion that a patient has FSGS.[accessmedicine.mhmedical.com]
Treponema Pallidum
  • KEYWORDS: Sexually transmitted infections; Treponema pallidum; doxycycline; focal segmental glomerulosclerosis; glomerulonephritis; late; nephrotic; proteinuria; renal; syphilis[ncbi.nlm.nih.gov]
Pleural Effusion
  • Furthermore, an accumulation of pleural effusion was confirmed by chest X-ray.[ncbi.nlm.nih.gov]
  • Pleural effusion and ascites may be present; pericardial effusions are rare. Gross edema may predispose patients to ulcerations and infections in dependent areas, such as the lower extremity.[physio-pedia.com]
  • Pleural effusion (fluid in the lung cavity) and ascites (fluid in the abdomen) may occur. Pericardial effusions are rare. Rarely, patients experience severe renal failure.[naturallivingcenter.net]

Treatment

  • In resistant cases, addition of extracorporeal treatment options or treatment with alkylating agents may be considered.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: In kidney transplantation recipients with recurrent FSGS, rituximab therapy may be a recommended treatment for cases that have failed either the initial treatment or weaning from PE.[ncbi.nlm.nih.gov]
  • Treatment can also be guided by the results of the biopsy. Treatment: Some treatment is used to control the symptoms associated with the disease as well as slow down the progression of the disease.[glomerularcenter.org]
  • […] with rituximab when compared with before treatment (p The systematic review found that 72 out of 73 people with MCD had a response to rituximab treatment, and 6 out of 7 participants with FSGS achieved at least partial remission following treatment with[nice.org.uk]
  • Study limitations included small sample size and heterogeneity in age and comparative treatments across the studies. CONCLUSIONS: Cyclosporin-based treatments provided a significantly better partial remission rate as compared with other therapies.[ncbi.nlm.nih.gov]

Prognosis

  • Indigenous people had a female predominance and are over-represented in relation to their population size, however, were not associated with poorer prognosis.[ncbi.nlm.nih.gov]
  • On the basis of this experience, we suggest the significance of the detection of urinary podocytes for determining renal prognosis in FSGS following renal allograft.[ncbi.nlm.nih.gov]
  • Prognosis is extremely variable and depends on the amount of proteinuria, the level of plasma creatinine, the morphological subtype, the degree of renal fibrosis, and the response to therapy.[bestpractice.bmj.com]
  • It strongly suggested that RA patients with the abnormal kidney functions should be routinely screened for FSGS to guide the therapy, achieve both RA and FSGS remission, determine a prognosis, and avoid end-stage renal lesion.[ncbi.nlm.nih.gov]
  • The clinical course and prognosis is heterogeneous in children. The aim of this study was to analyze treatment and outcome of children with FSGS.[ncbi.nlm.nih.gov]

Etiology

  • Two frameworks for the classification of FSGS have been described: etiologic and morphologic. The etiologic classification is distinguished among genetic, adaptive, virus-associated, drug-induced, and idiopathic types.[ncbi.nlm.nih.gov]
  • Admixture mapping recently identified the nonmuscle myosin heavy chain 9 gene (MYH9) as a susceptibility factor strongly associated with several nondiabetic etiologies of end-stage renal disease in African Americans, less strongly with diabetes-associated[ncbi.nlm.nih.gov]
  • Here we report two families with multiple members who have collapsing FSGS with no clear associated secondary etiology.[ncbi.nlm.nih.gov]
  • Identifying the etiology of FSGS guides selection of therapy and provides prognostic insight.[ncbi.nlm.nih.gov]
  • Failing to find a possible etiology may lead to misdiagnosis. CASE PRESENTATION: A young man presented to the emergency room with a 4-day history of cough, neck swelling and sore throat.[ncbi.nlm.nih.gov]

Epidemiology

  • Author information 1 Department of Nephrology and Dialysis, Hyogo Prefectural Amagasaki General Medical Center, Japan. 2 Department of Healthcare Epidemiology, Graduate School of Medicine and Public Health, Kyoto University, Japan.[ncbi.nlm.nih.gov]
  • Nevertheless, the available data suggest that large epidemiological case-control studies to examine the association between NPHS2 variants and nephrotic syndrome are warranted.[ncbi.nlm.nih.gov]
  • Homogeneous epidemiologic, pathogenetic, clinical or histological data are not available. Recent research on the pathogenetic role of mesangial IgM has, however, renewed interest in IgMN and naturally the controversies.[ncbi.nlm.nih.gov]
  • Epidemiology, major drivers, restraints, and opportunities have been covered to provide an exhaustive picture of the market.[businesswire.com]
  • Epidemiology FSGS has a high prevalence among African Americans. It is 2 to 3 times more frequent in this population in comparison to Caucasians. FSGS is the lesion found in 35% of the cases in adults who present with nephrotic syndrome.[news-medical.net]
Sex distribution
Age distribution

Pathophysiology

  • There is emerging evidence showing that perturbed lipid metabolism plays a role in the pathophysiology of FSGS.[ncbi.nlm.nih.gov]
  • To our knowledge, our report is the first to show evidence of a possible connection between BS and FSGS and discusses the pathophysiologic mechanism that explains this link.
.[ncbi.nlm.nih.gov]
  • Areas covered: We searched for investigational drugs agents that target different pathophysiological pathways using the key words 'FSGS' and 'podocyte' in American and European clinical trial registers (clinicaltrials.gov; clinicaltrialsregister.eu).[ncbi.nlm.nih.gov]
  • The primary pathophysiologic process in FSGS is an injury inherent within or directed to podocytes.[emedicine.medscape.com]

Prevention

  • This drug prevents the virus from copying its genetic code, which prevents the virus from replicating.[naturallivingcenter.net]
  • Genetics Lab, IRCCS Istituto Auxologico Italiano, Milan, Italy. 4 Department of Medical Biotechnology and Translational Medicine, Università degli Studi di Milano, Milan, Italy. 5 Molecular Bases of Genetic Risk and Genetic Testing Unit, Department of Preventive[ncbi.nlm.nih.gov]
  • It is important to have a high clinical suspicion for this uncommon condition in an appropriate clinical setting, because a timely intervention can prevent long-term complications. 2016 BMJ Publishing Group Ltd.[ncbi.nlm.nih.gov]
  • Rituximab partially prevented SMPDL-3b and ASMase down-regulation that was observed in podocytes treated with the sera of patients with recurrent FSGS.[stm.sciencemag.org]
  • Early glomerulopathy is rare in myeloproliferative neoplasm, and aggressive follow-up is required to prevent progression of kidney disease. BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved.[ncbi.nlm.nih.gov]

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