C1q nephropathy in the pediatric population: pathology and pathogenesis

Wenderfer, Scott E.; Swinford, Rita D.; Braun, Michael C.

doi:10.1007/s00467-009-1429-x

Cited by 23 publications

(20 citation statements)

References 66 publications

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“…Although originally described as a form of FSGS, the histological spectrum for C1q nephropathy (Figure 8) could range from MCD to mild mesangial proliferation to FSGS (35). The clinical manifestations include nephrotic range proteinuria with or without hematuria, with hypertension and renal insufficiency in a subset of patients (36, 37). Serology is usually negative, and serum C3 and C4 levels are normal.…”

Section: Other Histological Variants Of Fsgsmentioning

confidence: 99%

Pathology of Podocytopathies Causing Nephrotic Syndrome in Children

Ranganathan

2016

Front. Pediatr.

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Nephrotic syndrome (NS) in children includes a diverse group of diseases that range from genetic diseases without any immunological defects to causes that are primarily due to immunological effects. Recent advances in molecular and genomic studies have resulted in a plethora of genetic defects that have been localized to the podocyte, the basic structure that is instrumental in normal filtration process. Although the disease can manifest from birth and into adulthood, the primary focus of this review would be to describe the novel genes and pathology of primary podocyte defects that cause NS in children. This review will restrict itself to the pathology of congenital NS, minimal change disease (MCD), and its variants and focal segmental glomerulosclerosis (FSGS). The two major types of congenital NS are Finnish type characterized by dilated sausage shaped tubules morphologically and diffuse mesangial sclerosis characterized by glomerulosclerosis. MCD has usually normal appearing biopsy features on light microscopy and needs electron microscopy for diagnosis, whereas FSGS in contrast has classic segmental sclerosing lesions identified in different portions of the glomeruli and tubular atrophy. This review summarizes the pathological characteristics of these conditions and also delves into the various genetic defects that have been described as the cause of these primary podocytopathies. Other secondary causes of NS in children, such as membranoproliferative and membranous glomerulonephritis, will not be covered in this review.

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Section: Other Histological Variants Of Fsgsmentioning

confidence: 99%

Pathology of Podocytopathies Causing Nephrotic Syndrome in Children

Ranganathan

2016

Front. Pediatr.

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“…The global incidence of C1qN in the biopsied pediatric population ranges from 1.9% to 6.6% [5]. The incidence in children biopsied for SRNS is 16% [6].…”

Section: Discussionmentioning

confidence: 99%

“…Multiple mechanisms have been suggested for C1q deposition in mesangial cells: 1) the passage of plasma proteins in the glomerulus leads to non-specific entrapment of these immunoglobulins in the mesangium and the C1q then binds to Fc receptors of these trapped immunoglobulins; 2) immune complex formation with C1q; 3) C1q directly binds to receptors on the mesangial cells; and 4) increased synthesis of C1q by the macrophages triggered by inflammatory cytokines [5,13].…”

Section: Discussionmentioning

confidence: 99%

“…Analysis of the published literature on C1qN treatment in pediatric patients showed that 96% of patients were treated with corticosteroids, of which 66% had partial response, 30% were steroid-resistant and 14% progressed to endstage kidney disease (ESKD) [5]. Patients with nephrotic syndrome and MCD histology responded well to treatment and had good prognosis, while patients with FSGS morphology showed poor outcome, with almost 30% progressing to ESKD.…”

Section: Discussionmentioning

confidence: 99%

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The Heterogeneity and Controversy of C1q Nephropathy: A Report of Two Cases and Review of the Literature

Husain

2014

World J Nephrol Urol

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C1q is an uncommon, controversial and under-recognized entity. There is disagreement regarding whether it is an established disease or just part of the spectrum of minimal change disease and focal segmental glomerulosclerosis. C1q nephropathy is diagnosed solely by kidney biopsy. We report two cases of C1q nephropathy, one in a 39-year-old man and the other in an 8-year-old boy. Our two cases highlight the variable nature of this disease. In this article, we present our cases, review the criteria for diagnosis and highlight the heterogeneous nature of this disease in terms of clinical presentation, renal biopsy and variable outcomes. We also discuss the postulated etiopathogenetic mechanisms and note the reported associations.

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“…Besides some case reports and small adult case series, there had been eight exclusively pediatric case series of C1qN. [9][10][11][12][13][14][15][16][17] Major clinicopathological parameters of those pediatric case series are summarized in Table II.…”

Section: Patientmentioning

confidence: 99%

C1q nephropathy among children with nephrotic syndrome: Ten-year experience from a pediatric nephrology unit

Shahin¹,

Khader²,

Qattan³

et al. 2018

Turk J Pediatr

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Abu-Shahin N, Al-Khader A, Qattan D, Akl K. C1q nephropathy among children with nephrotic syndrome: Ten-year experience from a pediatric nephrology unit. Turk J Pediatr 2018; 60: 14-21. C1q nephropathy (C1qN) is a rare glomerulopathy mostly seen in children, and presents with nephrotic syndrome (NS). Diagnosis depends on immunoflourescence or immunohistochemical C1q mesangial deposition, excluding other immune-mediated diseases. We retrospectively investigated C1qN incidence, clinicopathological features, and outcome among pediatric NS in our institution.Clinical data, microscopic slides and corresponding tissue blocks of pediatric renal biopsies were retrieved. According to diagnostic criteria for C1qN, 53 pediatric NS renal biopsies were selected for Anti-C1qA IHC stain microscopic examination. Clinicopathological features and follow up data were recorded. C1qN incidence was 9.4% among pediatric NS biopsies. Mesangial proliferation was the most common histopathological pattern. Steroid dependency with frequent relapses was the most frequent outcome, with a second line immunosuppressant added, yet without impact on progression. Small sample size hinders coherent conclusions; nevertheless, it indicates that C1qN is a rare cause of pediatric NS. C1qN may require second line immunosupressants more often than non-C1q NS.

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C1q nephropathy in the pediatric population: pathology and pathogenesis

Cited by 23 publications

References 66 publications

Pathology of Podocytopathies Causing Nephrotic Syndrome in Children

Pathology of Podocytopathies Causing Nephrotic Syndrome in Children

The Heterogeneity and Controversy of C1q Nephropathy: A Report of Two Cases and Review of the Literature

C1q nephropathy among children with nephrotic syndrome: Ten-year experience from a pediatric nephrology unit

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