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Diagnosis and Primary Care Management of Focal Segmental Glomerulosclerosis in Children

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Diagnosis and Primary Care Management of Focal Segmental Glomerulosclerosis in Children 1.5 CONTACTCOCONOONNTATACACACT HOURSHOUROURRS 1.5 CONTACT HOURS Monkey Business Images / Thinkstock Diagnosis and primary care management of focal segmental glomerulosclerosis in children Abstract: Focal segmental glomerulosclerosis (FSGS) is a pattern of kidney damage that can occur in individuals at any age, including children. Pediatric patients with FSGS require medication monitoring, growth, and psychological health. This article discusses the NP’s role in the clinical presentation, diagnostic workup, and treatment of FSGS in pediatric patients. By Angela Y. Wong, MS, CPNP-PC and Rita Marie John, DNP, EdD, CPNP-PC, FAANP P, a 10-year-old girl, told her parents several This article discusses the pathophysiology, epide- times, “I feel puffy,” before the family sought miology, clinical presentation, and treatment options J medical attention. This complaint could in- for FSGS. In addition, the article elucidates the role dicate a myriad of issues ranging from sudden weight of the NP in managing this disease when it occurs gain to simple abdominal gas. For this child, “puffy” during childhood. depicted her progressive edema—the only overt symp- tom of her diagnosis of focal segmental glomerulo- ■ Overview sclerosis (FSGS). Although some children with FSGS FSGS is a pattern of kidney damage involving scarring may only present with asymptomatic proteinuria at of glomeruli in the kidney. This pattern of glomerulo- a routine physical, FSGS can affect individuals of all sclerosis is focal and segmental, meaning not all glom- ages, and is a common cause of end-stage renal disease eruli are affected and only parts of the glomeruli are (ESRD) in children.1 damaged, respectively.2 FSGS is the most common Keywords: end-stage renal disease, focal segmental glomerulosclerosis, kidney transplant, nephrotic syndrome, pediatrics 28 The Nurse Practitioner • Vol. 43, No. 9 www.tnpj.com Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved. Diagnosis and primary care management of focal segmental glomerulosclerosis in children glomerular condition that leads to ESRD and the ■ Pathophysiology third most common cause of ESRD in children; ap- Disease pathway. FSGS is a glomerular disease char- proximately 50% to 70% of pediatric patients with acterized by injury at the glomerular epithelial cell FSGS progress to ESRD and require dialysis or kidney (podocytes) and sclerosis in parts of some glomeruli.9 transplant.3 Podocyte injury results in effacement of the podocyte FSGS recurs in 30% to 40% of children who receive foot processes, leading to impaired function of the kidney transplants.4 This high rate of recurrence fur- glomerular fi ltration barrier and causes FSGS’s defi n- ther emphasizes the severe burden of stress this con- ing feature of proteinuria.10 This structural change to dition places on pediatric patients and their families, the kidney podocytes is progressive and irreversible providing a strong incentive for additional research in FSGS. into the direct mechanism behind FSGS. ■ Etiology of disease ■ Epidemiology Circulating factors. In patients with primary FSGS, it FSGS has an estimated worldwide incidence rate of is believed that circulating factors (most likely cyto- seven cases per million persons per year and is respon- kines or similar molecules) play a role in the initial sible for 20% of pediatric nephrotic syndromes.1 The disease process.8 These circulating factors are thought rate of occurrence of FSGS in patients with nephrotic to induce the effacement of glomerular podocytes syndrome is approximately 10% in children under age and remain in the body even after a kidney transplant. 6 years, 20% in adolescents, and 20% to 25% in adults.5 The factors then have the potential to cause podocyte Over 50% of patients will reach ESRD within 8 years, damage and FSGS to reoccur in the transplanted requiring dialysis or kidney transplant. kidneys. This theory of circulating molecules as the There are differences in the prevalence of FSGS stimulant of podocyte injury may explain the high among various ethnic groups. For example, the an- rate of reoccurrence of FSGS immediately after kid- nual incidence of FSGS in Black Americans is 1.6 ney transplants.8 per 100,000, whereas in White Americans it is 0.3 per 100,000.6 The risk of developing advanced kidney Black Americans have a 7.5% risk of progressing to ESRD, whereas disease is two to seven times greater for White Americans have a signifi- individuals carrying APOL1 alleles. cantly lower risk of 2%.6 The gene apolipoprotein L1 (APOL1) is much more frequent in Black Americans Genetics. Over 20 gene mutations are associated than in White Americans and is associated with a high- with changes to the podocyte structure that compro- er risk of developing FSGS.4 The risk of developing mises podocyte integrity and functionality.4 Specifi - advanced kidney disease is two to seven times greater cally, these genes encode products that alter cellular for individuals carrying APOL1 alleles.6 components and disturb cell energetics.11 Mutations causing FSGS are more common in younger patients. Classifi cation of FSGS A study of 125 Spanish patients found that muta- FSGS was initially classifi ed into two categories: pri- tions were identifi ed in 100% of cases with congenital mary and secondary. The primary, or idiopathic, onset, 57% of cases with infantile onset, 24% with type arises from unknown etiology and accounts for early childhood onset, 36% with late childhood on- 80% of FSGS cases.7 Secondary FSGS has identifi- set, and only 14% with adult onset.12 This knowledge able underlying causes including genetic, adaptive, of genetic mutations may also provide information virus-associated, or drug-related etiologies.4 Recently, regarding the likelihood of responsiveness to specifi c Rosenberg and Kopp proposed that FSGS be reclas- treatments or the likelihood of reoccurrence of disease sified into six clinical forms: primary (idiopathic), after kidney transplant. adaptive, high-penetrance genetic, viral-mediated, Histological subtypes. FSGS is further classifi ed into drug-associated, and genetic APOL1 types (see Types fi ve histological subtypes based on the Columbia Clas- and causes of FSGS).8 sifi cation published in the seminal paper by D’Agati www.tnpj.com The Nurse Practitioner • September 2018 29 Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved. Diagnosis and primary care management of focal segmental glomerulosclerosis in children and colleagues.13 These FSGS subtypes are based on the dysfunction, children may present with full ne- morphology of the specifi c glomerular lesions under phrotic syndrome, most obviously manifesting as the microscope. Specifi c histological subtype can play a severe progressive edema.14 In children, nephrotic signifi cant role in the treatment and overall prognosis syndrome is described as proteinuria (greater than of FSGS (see Histological subtypes of FSGS). 1 g/m2 urine protein per day), hypoalbuminemia (less than 2.5 g/dL serum albumin), hypercholester- ■ Clinical presentation olemia (greater than 200 mg/dL total cholesterol), The most common presenting symptom of FSGS in and edema.10 Edema is present in almost half of children is asymptomatic proteinuria during rou- patients at the time of disease onset and can be tine physical exam.2 In cases of extreme nephron categorized as mild or moderate (involving the feet, Types and causes of FSGS8-10,34 Types Causes Primary (idiopathic) FSGS Specifi c cause unknown; likely cytokine acting as a circulating factor in the blood Adaptive FSGS • Mismatch between metabolic input and glomerular capacity • Podocyte stress with genetic susceptibility • Excessive workload on nephron due to: – Obesity – Reduced renal mass • Ischemia secondary to hypertension, renal artery stenosis High-penetrance genetic FSGS • Podocyte slit diaphragm – NPHS1 (nephrin), NPHS2 (podocin) – CD2-associated protein (CD2AP) • Actin cytoskeleton – ACTN4 (alpha-actinin 4) – Myosin IIA – Myosin 1E (MYO1E) • Lysosomes – Scavenger receptor class B member 2 (SCARB2)/Lysosomal integral membrane protein-2 (LIMP-2) • Mitochondria – Coenzyme Q2 (COQ2) – tRNA(Leu) – Coenzyme Q6 (COQ6) • Cellular nucleus – Wilms’ tumor 1 (WT1) Viral-mediated FSGS • Direct viral infection of the podocyte receptors: – HIV – Parvovirus B19 – Cytomegalovirus – Epstein-Barr virus Drug-associated FSGS • Damage of podocytes and kidney tubules by: – Heroin – Bisphosphonate – Calcineurin inhibitors (post renal allograft) – Interferon – Lithium – Androgen use – Chronic use of nephrotoxic drugs 30 The Nurse Practitioner • Vol. 43, No. 9 www.tnpj.com Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved. Diagnosis and primary care management of focal segmental glomerulosclerosis in children Histological subtypes of FSGS4,10,20 Variants Lesion histology Associations Not otherwise • Foot-process effacement is variable • Most common variant specifi ed (NOS) • Does not meet defi ning criteria for other variants • Other variants can evolve into NOS overtime Perihilar • Foot-process effacement relatively mild and focal • More commonly associated with adaptive (secondary) FSGS Cellular • Foot-process effacement usually severe • Least common variant • Possibly represents early stage of sclerotic lesions Tip • Foot-process effacement usually severe • Most favorable prognosis, with high rate of response to corticosteroid therapy and low risk of progression Collapsing • Foot-process effacement usually severe • Worst prognosis, with poor response to • Severe tubular injury
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