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Primary Care Dec 2020Nephrotic syndrome is one cause of end-stage kidney disease. Because edema is a common presenting feature and hypertension and dyslipidemia are often present in... (Review)
Review
Nephrotic syndrome is one cause of end-stage kidney disease. Because edema is a common presenting feature and hypertension and dyslipidemia are often present in nephrotic syndrome, it is important for the primary care physician to suspect this entity. Common causes in adults include diabetic nephropathy, focal segmental glomerulosclerosis, and membranous nephropathy. In adults, many primary causes are due to an underlying disease. A cause of the nephrotic syndrome should be established with serologic workup and renal consultation. Renal biopsy is necessary in those with an unknown cause to or classify disease. Treatment focuses on symptoms, complications, and the primary cause.
Topics: Age Factors; Biomarkers; Blood Pressure; Dyslipidemias; Edema; Humans; Nephrotic Syndrome; Primary Health Care; Racial Groups; Referral and Consultation; Sex Factors
PubMed: 33121631
DOI: 10.1016/j.pop.2020.08.002 -
Pediatric Clinics of North America Feb 2019Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia, and hyperlipidemia. Minimal change disease, the most common cause in childhood, generally... (Review)
Review
Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia, and hyperlipidemia. Minimal change disease, the most common cause in childhood, generally responds to corticosteroids, although most patients experience disease relapses. Focal segmental glomerulosclerosis is usually resistant to corticosteroids and carries a significant risk of kidney failure, necessitating renal transplantation. Nephrotic syndrome may also be secondary to gene mutations and systemic diseases such as lupus. Clinical evaluation involves distinguishing primary and secondary causes and monitoring for disease complications, including blood clots and serious infections such as spontaneous bacterial peritonitis. Immunosuppressive medications are used to prevent relapses and treat corticosteroid-resistant disease.
Topics: Adrenal Cortex Hormones; Child; Diagnosis, Differential; Humans; Nephrotic Syndrome; Risk Factors
PubMed: 30454752
DOI: 10.1016/j.pcl.2018.08.006 -
The Medical Clinics of North America Jul 2023Nephrotic syndrome (NS) is a key clinical entity for the internist to recognize and understand. A wide range of infectious, metabolic, malignant, and autoimmune... (Review)
Review
Nephrotic syndrome (NS) is a key clinical entity for the internist to recognize and understand. A wide range of infectious, metabolic, malignant, and autoimmune processes drive nephrosis, leading to a syndrome defined by proteinuria, edema, and hypoalbuminemia. NS occurs due to increased permeability to proteins at the level of the glomerulus, which allows for passage of albumin and other proteins into the urine. Proteinuria leads to a cascade of clinical complications characterized by fluid accumulation, kidney inflammation, and dysregulation of coagulation and immunity. In this article, the authors review the clinically important etiologies of NS that should inform an initial clinical evaluation.
Topics: Humans; Nephrotic Syndrome; Proteinuria; Edema
PubMed: 37258010
DOI: 10.1016/j.mcna.2023.03.006 -
Paediatrics and International Child... Nov 2017Nephrotic syndrome is defined by nephrotic-range proteinuria (≥40 mg/m/hour or urine protein/creatinine ratio ≥200 mg/mL or 3+ protein on urine dipstick),... (Review)
Review
Nephrotic syndrome is defined by nephrotic-range proteinuria (≥40 mg/m/hour or urine protein/creatinine ratio ≥200 mg/mL or 3+ protein on urine dipstick), hypoalbuminaemia (<25 g/L) and oedema. This review focuses on the classification, epidemiology, pathophysiology, management strategies and prognosis of idiopathic nephrotic syndrome of childhood, and includes a brief overview of the congenital forms.
Topics: Child; Disease Management; Humans; Infant; Nephrotic Syndrome; Prognosis
PubMed: 28914167
DOI: 10.1080/20469047.2017.1374003 -
BioMed Research International 2018
Topics: Animals; Humans; Nephrotic Syndrome
PubMed: 29670902
DOI: 10.1155/2018/6215946 -
Journal of Perinatology : Official... Dec 2021Congenital nephrotic syndrome (CNS), a challenging form of nephrotic syndrome, is characterized by massive proteinuria, hypoalbuminemia, and edema. Extensive leakage of... (Review)
Review
Congenital nephrotic syndrome (CNS), a challenging form of nephrotic syndrome, is characterized by massive proteinuria, hypoalbuminemia, and edema. Extensive leakage of plasma proteins is the main feature of CNS. Patients can be diagnosed in utero or during the first few weeks of life, usually before three months. The etiology of CNS can be related to either genetic or nongenetic etiologies. Pathogenic variants in NPHS1, NPHS2, LAMB2, WT1, and PLCE1 genes have been implicated in this disease. The clinical course is complicated by significant edema, infections, thrombosis, hypothyroidism, failure to thrive, and others. Obtaining vascular access, frequent intravenous albumin infusions, diuretic use, infection prevention, and nutritional support are the mainstay management during their first month of life. The best therapy for these patients is kidney transplantation. CNS diagnosis and treatment continue to be a challenge for clinicians. This review increases the awareness about the pathogenesis, diagnosis, and management of CNS patients.
Topics: Humans; Infant; Intracellular Signaling Peptides and Proteins; Membrane Proteins; Mutation; Nephrotic Syndrome
PubMed: 34983935
DOI: 10.1038/s41372-021-01279-0 -
Handbook of Clinical Neurology 2014Nephrotic syndrome refers to excessive proteinuria, with associated hypoalbuminemia, edema, and hyperlipidemia. A diverse spectrum of disorders has been associated with... (Review)
Review
Nephrotic syndrome refers to excessive proteinuria, with associated hypoalbuminemia, edema, and hyperlipidemia. A diverse spectrum of disorders has been associated with nephrotic syndrome and related neurologic complications, although the relative infrequency of these cases limits conclusive associations. Neurologic manifestations of nephrotic syndrome may result from hypoproteinemia, hypercoagulability, hyperlipidemia, hypertension, amyloid deposition, hormonal changes, or electrolyte disorders. Neurologic diagnosis hinges on prompt recognition of this syndrome and rational therapeutic strategies are aimed at the underlying systemic disorder.
Topics: Humans; Nephrotic Syndrome; Nervous System Diseases
PubMed: 24365309
DOI: 10.1016/B978-0-7020-4086-3.00026-6 -
Pediatrics in Review Feb 2022Nephrotic syndrome (NS) encompasses a variety of disease processes leading to heavy proteinuria and edema. Minimal change disease (MCD) remains the most common primary...
Nephrotic syndrome (NS) encompasses a variety of disease processes leading to heavy proteinuria and edema. Minimal change disease (MCD) remains the most common primary cause of NS, as well as the most responsive to pharmacologic treatment with often minimal to no chronic kidney disease. Other causes of NS include focal segmental glomerulosclerosis, which follows MCD, and secondary causes, including extrarenal or systemic diseases, infections, and drugs. Although initial diagnosis relies on clinical findings as well as urine and blood chemistries, renal biopsy and genetic testing are important diagnostic tools, especially when considering non-MCD NS. Moreover, biomarkers in urine and serum have become important areas for research in this disease. NS progression and prognosis are variable and depend on etiology, with corticosteroids being the mainstay of treatment. Other alternative therapies found to be successful in inducing and maintaining remission include calcineurin inhibitors and rituximab. Disease course can range from recurrent disease relapse with or without acute kidney injury to end-stage renal disease in some cases. Given the complex pathogenesis of NS, which remains incompletely understood, complications are numerous and diverse and include infections, electrolyte abnormalities, acute kidney injury, and thrombosis. Pediatricians must be aware of the presentation, complications, and overall long-term implications of NS and its treatment.
Topics: Adrenal Cortex Hormones; Glomerulosclerosis, Focal Segmental; Humans; Nephrotic Syndrome; Proteinuria; Rituximab
PubMed: 35102405
DOI: 10.1542/pir.2020-001230 -
Pediatric Clinics of North America Dec 2022Nephrotic syndrome in children is mostly idiopathic in origin. About 90% of patients respond to corticosteroids; 80-90% have at least one relapse and 3-10% become... (Review)
Review
Nephrotic syndrome in children is mostly idiopathic in origin. About 90% of patients respond to corticosteroids; 80-90% have at least one relapse and 3-10% become corticosteroid resistant after the initial response. A kidney biopsy is seldom indicated for diagnosis except in patients with atypical presentation or corticosteroid resistance. For those in remission, the risk of relapse is reduced by the administration of daily low dose corticosteroids for 5-7 days at the onset of an upper respiratory infection. Some patients may continue having relapses through adult life. Many country-specific practice guidelines have been published, which are very similar with clinically insignificant differences.
Topics: Child; Humans; Nephrotic Syndrome
PubMed: 36880923
DOI: 10.1016/j.pcl.2022.08.002 -
American Family Physician Nov 2009Nephrotic syndrome may be caused by primary (idiopathic) renal disease or by a variety of secondary causes. Patients present with marked edema, proteinuria,... (Review)
Review
Nephrotic syndrome may be caused by primary (idiopathic) renal disease or by a variety of secondary causes. Patients present with marked edema, proteinuria, hypoalbuminemia, and often hyperlipidemia. In adults, diabetes mellitus is the most common secondary cause, and focal segmental glomerulosclerosis and membranous nephropathy are the most common primary causes. Venous thromboembolism is a possible complication; acute renal failure and serious bacterial infection are also possible, but much less common. There are no established guidelines on the diagnostic workup or management of nephrotic syndrome. Imaging studies are generally not needed, and blood tests should be used selectively to diagnose specific disorders rather than for a broad or unguided workup. Renal biopsy may be useful in some cases to confirm an underlying disease or to identify idiopathic disease that is more likely to respond to corticosteroids. Treatment of most patients should include fluid and sodium restriction, oral or intravenous diuretics, and angiotensin-converting enzyme inhibitors. Some adults with nephrotic syndrome may benefit from corticosteroid treatment, although research data are limited. Intravenous albumin, prophylactic antibiotics, and prophylactic anticoagulation are not currently recommended.
Topics: Adult; Diagnosis, Differential; Humans; Nephrotic Syndrome
PubMed: 19904897
DOI: No ID Found