Clinico-pathological Analysis of Kidney Diseases in Children: A Retrospective, Single Center Study Dr. Bassam Saeed Pediatric Nephrologist Surgical Kidney Hospital Damascus - Syria

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.

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.

Patients Characteristics Mean / Range10-15yrs5-10yrs2-5yrs<2 yrs Age 7.5yrs / 40days-15yrs FemalesMales Gender 35 patients / 37%60 patients / 63%

Geographic Distribution

Indications For Renal Biopsies

Indications For Renal Biopsies in Nephrotic Children (50)

Indications For Renal Biopsies in Hematuria Cases (20)

Indications For Renal Biopsies in Renal Insufficiency Cases (14)

Familial Cases (14)

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

Diagnosis

Histologic Diagnosis in Nephrotic Syndrome (51cases)

Clinico-pathologic Correlation in Nephrotic Syndrome

Clinico-pathologic Correlation in Minimal Change Nephrotic Syndrome

Clinico-pathologic Correlation in Focal & Segmental Glomerulosclerosis (12 cases)

Clinico-pathologic Correlation in Diffuse Mesangial Proliferative GN(10 Cases)

Clinico-pathologic Correlation in MPGN (9 Cases)

Clinico-pathologic Correlation in IgA Nephropathy (4 Cases)

Clinico-pathologic Correlation in Hematuria (20 cases)

Clinico-pathologic Correlation in Chronic Renal Insufficiency (14cases) Patient Number Diagnosis Patient Number Diagnosis 1FSGS2MesGN 1Alport2Interstitial Nephritis 1IgA nephropathy2Nephronophthisis 1Nephrocalcinosis2Chronic Rejection 2Acute Rejection

Clinico-pathologic Correlation in Nephritic Syndrome (9 cases)

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

IMMUNO HISTO CHEMISTRY IgA

Indications For IgA Immunostaining (29)

Indications & Results of IgA Immunostaining (5/29)

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

Indications & Results of IgM Immunostaining (11/16)

LM Diagnosis for Positive IgM Deposits Cases (11)

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.

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

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.

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…!

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.

B. Allouch 4.5 yrs IgA nephropathy

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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