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Approach to Pediatric Kidney Disease. Kamal Akl MD Professor of Pediatrics & Pediatric Nephrology July 2019. Objectives. To familiarize medical students with the pattern of Pediatric Kidney Disease in Jordan
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Approach to Pediatric Kidney Disease Kamal Akl MD Professor of Pediatrics & Pediatric Nephrology July 2019
Objectives • To familiarize medical students with the pattern of Pediatric Kidney Disease in Jordan • To approach children in a focused, geographically, and culturally oriented manner • Such approach will save time, money, and unnecessary investigations
Pediatric Kidney Disease in Jordan • Including influence of : • Cultural Habits • Religion • Economics
Approach to Pediatric Kidney Disease • Children are not small adults • Importance of the history and physical exam • Treat the Patient , not the lab • Developing Clinical sense(Sans Clinique)
Congenital Anomalies of the Kidney and Urinary Tract(CAKUT) • Kidneys • Pelvis • Ureters • Bladder • Urethra
CAKUT • Importance of Antenatal Diagnosis
Antenatal Diagnosis • Severity of Hydronephrosis based on AP pelvic diameter: • Severe > 10 mm in 2nd trimester • Severe > 15 mm in 3rd trimester
Antenatal Diagnosis • Society for fetal urology(SFU) hydronephrosis grading system: • Grade O – no dilatation • Grade 2- Urine fills intrarenal pelvis+/- major calyces • Grade 3- all calyces are visualized • Grade 4- Grade 3+ parenchymal thininng • Nguyen HT et al • J Pediatr Urol. 2010 Jun;6(3):212-31. doi: 10.1016/j.jpurol.2010.02.205. Epub 2010 Apr 15. • The Society for Fetal Urology consensus statement on the evaluation and management of antenatal hydronephrosis.
Antenatal Diagnosis • Red flags for significant pathology: • Oligohydramnios • Additional renal or extrarenal anomalies • Sinha A • Indian J Nephrol. 2013 Mar-Apr; 23(2): 83–97.
Antenatal Diagnosis • Patients with postnatal APD exceeding 10 mm and/or SFU grade 3-4 should be screened for upper or lower urinary tract obstruction and vesicoureteric reflux (VUR). • Sinha A et al Indian J Nephrol. 2013 Mar-Apr; 23(2): 83–97.
Acute Kidney Injury • Prerenal • Intrinsic Renal • Postrenal • Causes in Jordan
Chronic Kidney Disease(CKD) • Should know • CKD stages • Updated Shwartz Formula • Causes in Jordan
CKD Stages • I • II • III • IV • V
Updated Shwartz Formula [Height(cm)/serum creatinine(mg%] x 0.413
End Stage Renal Failure(ESRF) In Jordan • Most Common causes in Jordan • CAKUT: Reflux Nephropathy • Neurogenic Bladder • Posterior Urethral Valve • Heredofamilial • Glomerular Disease • Etc • Akl KF et al • Aetiology of Paediatric End-stage Renal Failure in Jordan: A Multicentre Study. West Indian Med J. 2015 May 11;65(2):263-266.
Urinary Tract Infection(UTI) • UTI in Jordan • E coli resistance • New AAP guidelines 2011
Not all Dysuria is UTI • Causes of Dysuria: • Infectious: • APN • Cystitis • Vaginitis • Balanitis
Not all Dysuria is UTI • Noninfectious: • Dysfunctional voiding • Labial adhesions • Crystalluria Meatal stenosis Chemical irritants Trauma
Vesicoureteral Reflux • Primary • Secondary
Asymptomatic urinary abnormalities • Hematuria • Proteinuria • Hematuria+ Proteinuria
Glomerular Disease • Primary • Secondary • Jordan
Should distinguish • Between - • Glomerular vs Non Glomerular Hematuria
Should distinguish • Between • Acute Nephritis vs Nephrotic syndrome
Classification of glomerular disease • May be based on serum complement • Presentation • Histopathology
Classification based on serum complement • Hypocomplementemic : • (1) Poststrepeptococcal acute GN • (2) Mesangiocapillary GN • (3) Lupus nephritis • (4) Shunt nephritis • Normocomplementemic : Others eg MCD,FSGS , IgA N , HSP nephritis
Classification according to clinical presentation • Asymptomatic urinary abnormalities • Nephritic syndrome • Rapidly progressive glomerulonephritis • Nephrotic syndrome • Chronic glomerulonephritis • Hypertension
Classification based on histopathology • Proliferative • Nonproliferative
Primary Glomerular Disease • IgA Nephropathy • Poststreptococcal Glomerulonephritis • Jordan
Primary Glomerular Disease • Poststreptococcal AGN
Secondary Glomerular Disease • Henoch Schonlein purpura • Systemic Lupus Erythematosis • Hemolytic Uremic Syndrome
Hereditary Nephropathy • Alport syndrome • Nail Patella syndrome • New syndromes in Jordan
Acute Interstitial Nephritis • Mostly under diagnosed • Causes AKI with minimal or no proteinuria
Nephrotic Syndrome • Minimal Change Disease • Focal Segmental Glomerulosclerosis(FSGS) • Mesangioproliferative Glomerulonephritis • Mesangiocapillary Glomerulonephritis • Membranous Glomerulopathy • Seconary causes
Nephrotic syndrome • Most common causes of Steroid resistance in Jordan • FSGS • MesProliferative GN
Not all Edema is secondary to Kidney Disease • Causes of Edema: • Increased Capillary Permeability • Decreased Capillary Oncotic Pressure • Increased Plasma Volume • Lymphatic Obstruction
Should distinguish • Between • Glomerular Disease vs Tubular Disease
Tubular Disorders • Approach to Metabolic acidosis • Calculate Serum anion Gap • Urine Anion Gap
Metabolic Acidosis • Normal Anion Gap( 8-16) • Increased Anion Gap
Normal AG Metabolic Acidosis • Diarrhea • RTA
RTA • Type 1 • Type 2 • Type 4
RTA Type 1 • Ser K: low • Urine pH during acidosis :high • Ser HCO3: 10-20 • Urine pCO2: <20 • Urine citrate:low • Urolithiasis
RTA Type 2 • Ser K:Low • Urine pH during acidosis: low • Serum HCO3: 16-18 • Urine pCO2: > 20 • Urine citrate: high • Fanconi syndrome: may be present
RTA Type 4 • Ser K: High • Urine pH during acidosis: low or high • Serum HCO3: 16-22 • Urine pCO2: >20 • Urine citrate: low
Increased anion Gap Metabolic Acidosis • Poisoning: Salicylate; Toluene; ethylene Glycol;Methanol • Ketoacidosis: Diabetic; starvation • Medications • Shock • Inborn errors of Metabolism; Methanol
Metabolic Alkalosis • Chloride Responsive(U cl < 15 mEq/L) • Chloride Resistant(U cl > 20 mEq/L)
Chloride Responsive Metabolic Alkalosis • Vomiting • Cystic Fibrosis • Pyloric Stenosis