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Clinico-pathological Analysis of Kidney Diseases in Children: A Retrospective, Single Center Study Dr. Bassam Saeed Pediatric Nephrologist Surgical Kidney Hospital Damascus - Syria
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Aim of The Study ► Assessing the correlation of renal histopathological findings with clinical diagnosis in order to achieve a useful data on when and how a renal biopsy could be a useful tool for the management of a child with kidney disease. ► Recognizing the pattern of kidney diseases in our pediatric population.
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Patients And Methods ● A retrospective study ● Duration: 3 years (between January 2003 and December 2005) ● Surgical Kidney Hospital (a tertiary care center in Damascus) ● A total of 95 native and allograft renal biopsies performed on children who presented to the pediatric nephrology department were reviewed and categorized. ● All Biopsies had been evaluated by the same histopathologists using LM. ● IgA, IgM, and IgG immunohistochemical staining were performed in some selected cases.
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Patients Characteristics Mean / Range10-15yrs5-10yrs2-5yrs<2 yrs Age 7.5yrs / 40days-15yrs 2936219 FemalesMales Gender 35 patients / 37%60 patients / 63%
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Geographic Distribution
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Indications For Renal Biopsies
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Indications For Renal Biopsies in Nephrotic Children (50)
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Indications For Renal Biopsies in Hematuria Cases (20)
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Indications For Renal Biopsies in Renal Insufficiency Cases (14)
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Familial Cases (14)
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Associated Extra-Renal Disorders Patient N.DisordersPatient N.Disorders 1 Asthma 6 Failure to thrive 1 Diabetes mellitus 3 Hepatitis C 1 Eczema 3 Hearing loss 1 Hypothyroidism 2 Delayed psych- motor development 1 Tuberculosis 2 Mediterranean fever 1 Rickets 2 Chondrdysplasia 2 HSP
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Diagnosis
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Histologic Diagnosis in Nephrotic Syndrome (51cases)
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Clinico-pathologic Correlation in Nephrotic Syndrome
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Clinico-pathologic Correlation in Minimal Change Nephrotic Syndrome
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Clinico-pathologic Correlation in Focal & Segmental Glomerulosclerosis (12 cases)
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Clinico-pathologic Correlation in Diffuse Mesangial Proliferative GN(10 Cases)
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Clinico-pathologic Correlation in MPGN (9 Cases)
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Clinico-pathologic Correlation in IgA Nephropathy (4 Cases)
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Clinico-pathologic Correlation in Hematuria (20 cases)
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Clinico-pathologic Correlation in Chronic Renal Insufficiency (14cases) Patient Number Diagnosis Patient Number Diagnosis 1FSGS2MesGN 1Alport2Interstitial Nephritis 1IgA nephropathy2Nephronophthisis 1Nephrocalcinosis2Chronic Rejection 2Acute Rejection
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Clinico-pathologic Correlation in Nephritic Syndrome (9 cases)
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Clinical Presentation Patient Number Histopathologic Diagnosis Nephritic Syndrome5Post-Infectious GN Nephrotic S {SD(1) SR(1) late onset(1)} 3Membranous GN Nephrotic Syndrome (early onset) 2CNS Finnish type Renal Insufficiency {acute(1) Chronic (1)} 2Interstitial Nephritis Chronic Renal Insufficiency (familial) 2Nephronophthisis Hematuria (1) - Familial CRI (1)2Alport syndrome Nephritic Syndrome1Cresentic GN Chronic Renal Insufficiency1Nephrocalcinosis Clinico-pathologic Correlation in Variant Pathologies
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IMMUNO HISTO CHEMISTRY IgA
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Indications For IgA Immunostaining (29)
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Indications & Results of IgA Immunostaining (5/29)
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Positive IgA Deposits Cases (5) HSP One case IgA Nephropathy 4 cases Diagnosis 5 cases 12Diffuse Mesangial proliferation 02Focal Mesangial Proliferation 02Positive IgM deposits 01Familial Cases 01Renal Insufficiency
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Indications & Results of IgM Immunostaining (11/16)
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LM Diagnosis for Positive IgM Deposits Cases (11)
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Results ►Nephrotic syndrome constitutes the main indication (53%) to perform a renal biopsy in our series; followed by hematuria (21%) and renal insufficiency(15%) ► FRNS and/or SDND was the leading indication to perform a renal biopsy in nephrotic children (46%), followed by SRNS in 28%. ► 17% of our patients had a family history of similar renal disorders ► 27% of our patients had associated extra-renal disorders ► MCD was the most frequent histopathological diagnosis (28 cases) followed by FSGS in 12 cases, and Mesangial GN in 10 cases.
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Nephrotic Syndrome ► MCD is responsible for 55% of all cases ► FSGS is responsible for 20% of all cases ► Mesangial GN is responsible for 10% of all cases. ► Membranous GN, MPGN, and CNS Finnish-type: each is responsible for 5% of all cases. ► MCD was the most common diagnosis in all NS sub-groups except in SRNS group where FSGS was the most common one in 6 cases (43%), while MCD was seen in 2 cases (14%). ► 60% of FSGS cases were steroid-resistant
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MesGN & MPGN ► Mesangial GN was seen in a different setting of clinical presentation (Hematuria, Nephrotic syndrome, renal insufficiency) with no clear impact on the therapeutic plan for these patients ► Membrano-proliferative GN: was mostly seen in nephritic patients or in hematuric cases and less frequently in nephrotic patients.
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Hematuria ► With available facilities, Renal Biopsy enables us to establish the diagnosis in 60% of cases. ► 8 out of 20 hematuria cases (40%) had a normal biopsy and no diagnosis knowing that 6 of these 8 patients had gross hematuria ► In such circumstances, one could ask why to do such an invasive procedure if it may not lead to the diagnosis…!
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Immunostaining ► Immunohistochemical staining for IgA, IgM, and IgG have performed for some selected cases and not as routine due to its cost, therefore we cannot draw a conclusion. ► IgA deposits were detected in 5 out of 29 tested patients; thus, this method enables us to establish the diagnosis of IgA nephropathy in 4 patients. ► IgM deposits were present in 11 out of 16 tested patients (almost two thirds); moreover, these 11 patients had a wide different clinical presentation. So one could wonder about its usefulness.
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B. Allouch 4.5 yrs IgA nephropathy
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Conclusion T his study provides an important data on the pattern of pediatric renal diseases in our center and highlights the usefulness of histologic findings in guiding the therapeutic plan. M ore facilities should be available to achieve more accurate and comprehensive histologic findings with a better impact on the clinical practice.
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Thank you
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