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Premium member Presentation Transcript Approach to a child with Hematuria: Approach to a child with Hematuria Dinesh Dharel MD Pediatrics ResidentContents:: Contents: Definition Causes History Examination Case discussion Investigations ManagementDefination: Defination Microscopic Hematuria : five or more red blood cells per high-power field on microscopic evaluation of urinary sediment from two of three properly collected urinalysis specimens Gross H ematuria : generally recognized with the naked eye Red urine without hematuria occurs in Hemoglobinuria or Myoglobinuria ( Heme -positive urine without RBCs caused by the presence of either hemoglobin or myoglobin ) or other causesCauses of red or high-coloured urine:: Causes of red or high-coloured urine: Hematuria (RBC +nt) Heme +ve (No RBCs) Hemoglobinuria (Hemolysis) Myoglobinuria (Rhabdomyolysis – Skeletal muscle injury, viral myositis, Hypernatremia, hypophosphatemia, hypotension, DIC, Toxins, Prolonged seizures)Causes of red or high-coloured urine:: Causes of red or high-coloured urine: Heme – ve Drugs: Rifampin , Chloroquine , Deferoxamine , Ibuprofen, Iron sorbitol , Metronidazole , Nitrofurantoin , Phenazopyridine , Phenolphthalein, Phenothiazines , Salicylates , Sulfasalazine Dyes: Beetroot, Blackberries, Food colouring agents Metabolites: Homogentisic acid ( Alcaptonuria ), Melanin, Methemoglobin , Porphyrin , Tyrosinosis , Urates Highly concentrated urine Bile pigmentsPowerPoint Presentation: The prevalence of microscopic hematuria among school-aged children ranges from 0.4% to 2.1%. Screening urinalyses should be obtained during well child care visits at 5 yr and once during the 2nd decade of lifePathophysiology: : Pathophysiology: Structural disruption in the integrity of glomerular basement membrane caused by inflammatory or immunologic processes Toxic disruptions of the renal tubules Mechanical erosion of mucosal surfaces in the genitourinary tractCauses of Hematuria:: Causes of Hematuria: Glomerular hematuria Isolated renal disease Postinfectious GN (Post streptococcal) IgA nephropathy Alport syndrome Thin Glomerular Basement membrane disease Membranoproliferative GN Membranous nephropathy Focal segmental glomerulosclerosis Multisystem disease HSP nephritis SLE nephritis HUS Wegener granulomatosis Polyarteritis nodosa Goodpasture syndrome HIV nephropathy Sickle cell glomerulopathy Infective endocarditisPathophysiology of Hematuria in Glomerulonephritis: Pathophysiology of Hematuria in GlomerulonephritisB) Extraglomerular hematuria: B) Extraglomerular hematuriaB) Extraglomerular hematuria: B) Extraglomerular hematuria Upper Urinary tract Tubulointerstitial Pyelonephritis Interstitial nephritis ATN Papillary necrosis Nephrocalcinosis Anatomic Hydronephrosis Polycystic kidney disease Tumor ( Wilms , Rhabdomyosarcoma , angiomyolipoma ) Trauma Upper Urinary tract Vascular: Arterial/venous thrombosis Malformation (aneurysms, hemangioma ) Nutcracker syndrome Crystalluria : Calcium, Oxalate, Uric acid Hemoglobinopathies : Sickle cell trait/disease Medications : NSAIDs, anticoagulantsPowerPoint Presentation: Lower urinary tract Inflammation- infectious (Bacterial, viral, Tubercular) and non infectious Cystitis Urethritis Urolithiasis, Hypercalciuria, Hyperuricosuria Trauma, Foreign bodies, Parasites Coagulopathy Heavy exercise Munchausen syndrome / by proxyCauses of Gross Hematuria:: Causes of Gross H ematuria : Urinary tract infection Meatal stenosis Perineal irritation Trauma Urolithiasis / hypercalciuria Coagulopathy Tumor Glomerular IgA nephropathy Alport syndrome Thin glomerular basement membrane disease Post infectious glomerulonephritis HSP nephritis SLE nephritisD/D of Symptomatic and Asymptomatic Hematuria: D/D of Symptomatic and Asymptomatic Hematuria Symptomatic Renal symptoms Urinary tract infections Nephrolithiasis Urethrorrhagia Systemic symptoms Henoch-Schönlein purpura Tuberous sclerosis Asymptomatic Hypercalciuria Cystic disease Obstruction Vascular Arteriovenous malformation Thrombosis Trauma Tumor Hemoglobinopathies Coagulopathies Exercise-induced hematuria Benign familial hematuria (thin basement membrane) Glomerulonephritis (resolving) Acute postinfectious nephritis IgA nephropathy Henoch-Schönlein purpuraCauses of Hematuria in the Newborn:: Causes of Hematuria in the Newborn: Renal vein thrombosis (Asphyxia, dehydration, shock) Renal artery thrombosis Autosomal recessive polycystic kidney disease Obstructive uropathy Urinary tract infection Bleeding and clotting disorders Trauma, bladder catheterization Cortical necrosis (Hypoxic/ischemic perinatal insult) Nephrocalcinosis (Frusemide in premature)History:: History: Age: Wilms tumor usu. 2-5yrs, PSGN usu. 5-12yrs Sex: UTI F>>M in >1-2yrs, SLE nephritis F>>M, X-linked form of Alport syndrome M>F Race: Idiopathic hypercalciuria common in whites, Sickle cell disease is more common in blacksColour of urine:: Colour of urine: Colour Causes Dark yellow Normal concentrated urine Dark brown or black Bile pigments Homogentisic acid, melanin, tyrosinosis, methemoglobinuria Cola coloured Glomerular hematuria Red or pink urine Extraglomerular hematuria, Hemoglobin, Myoglobin, Porphyrins , Chloroquine , Deferoxamine , Beets, blackberries, Rifampin, Red dyes in food, UratesD/D of high coloured urine:: D/D of high coloured urine: All that glitters (red) is not gold (blood).History:: History: Characteristics of urine: Amount of urine: Reduced in AGN, ARF Clots in urine: Extraglomerular Frequency, Dysuria, recent enuresis : UTI Frothy urine: Suggests Proteinuria seen in Glomerular diseases Timing: Initial stream – from urethra (Urethrorrhagia – spotting in underwear); Terminal (with suprapubic pain, disturbance of micturition) – from bladderHistory:: History: Associated Symptoms: Fever: Infections, SLE, AGN Facial puffiness, Oedema of legs, weight gain, Shortness of breath: Acute Glomerulonephritis Hypertension (Headache, visual changes, epistaxis, seizures): AGN, ARF Abdominal pain: Urolithiasis (Loin to groin), UTI, clots, Nutcracker syndrome Painless: Glomerular Abdominal mass: Hydronephrosis, PKD, Wilm’s tumour Joint pain (HSP, SLE) Rashes (HSP, SLE, PAN) Neurologic – SLE, HUS (seizures, irritability) Jaundice: Hemolysis, Obstructive jaundiceHistory:: History: H/o exercise, menstruation, recent bladder catheterization or passage of a calculus Recent upper respiratory (1-2wks back), skin infection (3-6 wks): PSGN or GI infection: HUS, HSP nephritis Gross hematuria precipitated by URI: Alport syndrome, IgA Nephropathy, TGBMD H/o bleeding from other sites: Bleeding disorders, Hemoptysis in Good Pasture syndrome H/o Trauma, abdominal surgery, Child abuse (Social factors - Munchausen), crush injury H/o ingestion of drugs (ATT – Rifampicin, Ibuprofen, Chloroquine, Metronidazole, Iron), i.v. contrast agents (Toxic nephropathy, RVT)History:: History: H/o Vision or hearing defects: Alport syndrome Family h/o: Hereditary glomerular diseases (Alport syndrome, Thin glomerular Basement Membrane Disease, IgA Nephropathy), Urolithiasis, Hypercalciuria, Sickle cell disease/trait; H/o consanguinity or affected siblings in ARPKD, Metabolic disordersExamination:: Examination: Vitals: BP: in AGN, PKD Temperature Oedema: in AGN Pallor: Bleeding disorders, HUS, SLE, CRF JVP: Raised in CHF Anthropometry Malformations: VATER (vertebral body anomalies, anal atresia, tracheo-oesophageal fistula, renal dysplasia); Hemihypertrophy Neurocutaneous syndromes: Tuberous sclerosis (Angiomyolipoma), von Hippel-Lindau syndrome (Renal cysts, RCCa), Zellweger syndrome (Dysmorphic features, cataract, renal cysts)Examination:: Examination: Skin lesions (HSP, SLE, PAN), Bruises (Trauma) Per abdomen: Mass Kidney: Hydronephrosis (Urinary tract obstruction), Wilms tumour; B/L in ARPKD, B/L RVT, hydronephrosis Bladder palpable: Distal obstruction Tenderness: HSP Abnormal external genitalia e.g. ambiguous genitalia in WAGR syndrome (Wilms, aniridia, genital anomalies, mental retardation), Wilms tumour – hypospadias, cryptorchidism, Genital trauma, Foreign bodiesExamination:: Examination: Signs of Congestive cardiac failure, HTNsive encephalopathy: AGN Joint swelling, tenderness: HSP, SLE Ophthalmologic: Alport syndrome (Anterior lenticonus, macular flecks, recurrent corneal erosions), Aniridia (Wilms) Hearing assessment: Alport (B/L SNHL) Systemic examinationInvestigations:: Investigations: Detail history and comprehensive examination → Aids in diagnosis and directs the course of investigations Steps: Cause of the colour change: Urinalysis for RBC, hemoglobin/myoglobin or other causes Localization of bleeding site: Glomerular or Extraglomerular (Upper or lower urinary tract) R/o infection R/o Structural anomalies Renal function R/o Systemic diseasesInvestigations:: Investigations: Urine dipstick test: Based on the peroxidase -like activity of hemoglobin (a reagent strip impregnated with orthotolodine -peroxide and enhanced with 6-methoxyquinolone is used) It can detect trace amounts ( 0.02 to 0.03 mg/ dL ) of hemoglobin and myoglobin . Can detect 5-10 intact RBC per mm 3 of unspun urine False + ve : Urine pH >9, H 2 O 2 False – ve : High ascorbic acid, formalin Also for urine albuminInvestigations:: Investigations: Glomerular 1. Brown, cola coloured or smoky 2. RBC casts 3. Proteinuria 2+ or more 4. Deformed urinary RBCs Non - glomerular 1. Bright red, pink 2.Terminal hematuria/ Passage of clots 3. Proteinuria of < 2+ 4. Normal morphology of RBCs Urine microscopy : Presence of RBCs and casts (> 5 RBCs per HPF) in centrifuged urineInvestigations:: Investigations: Urine microscopy: Albumin: Glomerular cause Pus cells, WBC casts: Urinary tract infection, AGN, Eosinophils in Interstitial nephritis Crystals (Oxalates), bacteria, protozoa ( Schistosoma hematobium ova) Confirmation by dipstick and sediment examination is necessary to differentiate between true hematunia , pigmenturia , and interfening compounds such as beets or aniline dyes.For Asymptomatic, isolated microscopic hematuria: : For Asymptomatic, isolated microscopic hematuria: Isolated microscopic hematuria Repeat urinalysis weekly x 2 (No exercise x 48hrs) F/U Urine R/M with examination Test parents for hematuria Benign Familial Hematuria Family h/o calculi + Hearing test, + USG, + X-Ray KUB Urine Ca/Cr ratio Yearly Urine R/M , examination, BP Negative Positive Yes Normal Persistent hematuria No NoPowerPoint Presentation: Microscopic hematuria with proteinuria Oedema, Hypertension Rpt. Urine R/M in 1 wk CBC, RFT, Alb, Chol, ASO, dsDNA titre, Renal USG, C3, C4 F/U as indicated Yes Yes No -ve +ve Persistent hematuria/ proteinuria/ oedema/ HTN Refer to paediatric nephrologist AbnInvestigations:: Investigations: Urine C/S RFT : Blood urea nitrogen/serum creatinine, Na/K ( ↓Na in AGN, ↑K in ARF) Complete blood counts ( CBC ): Hb - ↓ in bleeding, HUS, SLE, CRF; Abnormal TC, DC in infections, HUS, ↓ in SLE; Platelet counts and Coagulation studies: (history suggestive of bleeding disorder, HUS), Sickle cell (Hemoglobin electrophoresis) PBS: Microangiopathic hemolytic anemia ESR, CRP - Infections 24 hr urinary protein, Spot urinary protein: Creatinine ratio , Serum albumin and cholesterol if associated proteinuria (Nephrotic syndrome) Urine calcium : Hypercalciuria is a relatively common finding in children. 24-hour urinary calcium (>4 mg/kg/d), or Spot urine calcium-creatinine ratio >0.21Investigations:: Investigations: Serologic testing : ASO titer, Anti-DNase B Low serum C 3 are seen in postinfectious glomerulonephritis, SLE nephritis, Bacterial endocarditis and membranoproliferative glomerulonephritis. ANA, anti dsDNA (SLE) ANCA, Anti-GBM Ab Throat swab C/SInvestigations:: Investigations: Imaging Studies Renal and bladder sonography: Urinary tract anomalies, such as hydronephrosis, hydroureter, nephrocalcinosis, tumor, and urolithiasis, Renal parenchymal disease X-Ray KUB: calculi Doppler study of renal vessels and IVC: Renal vein thrombosis Intravenous urography Spiral CT scan - Urolithiasis, Wilms tumor and polycystic kidney disease, Renal trauma Micturating cystourethrograms - Urethral and bladder abnormalities (eg, cystitis), in recurrent UTI to r/o VUR, anomalies Radionuclide studies – Renal function and perfusion Angiogram Chest X-Ray (Pulmonary oedema, CHF)Investigations:: Investigations: Investigations for recurrent/multiple Urolithiasis : Serum Calcium, Phosphorus, Uric acid, electrolytes, Alkaline phosphatase, Creatinine Urine: C/S, Ca:Cr ratio; 24 hr Urinary Calcium, Creatinine, Phosphorus, oxalate, uric acid; Spot test for cystine (Kit LC , Filler G , Pike J , Leonard MP . Can Urol Assoc J. 2008 Aug;2(4):381-6. Forty-one percent (28/69) of children with urolithiasis who underwent metabolic investigation had an abnormality.)Investigations:: Investigations: Renal biopsy: Relative indications - Significant proteinuria (3+ or more) or nephrotic syndrome + Recurrent persistent hematuria (Microscopic > 2yrs) Abnormal renal function, Persistent HTN Hematuria, Proteinuria, diminished renal function, low C3 level persist beyond 2 mo of onset of AGN Absence of evidence of streptococcal infection Serologic abnormalities (abnormal ANA or dsDNA levels) A family history of end stage renal disease or evidence of Chronic renal disease in patientPowerPoint Presentation: Dx Histology Clinical Lab IgA Nephro-pathy IgA deposition in the mesangium , glomerular sclerosis, proliferative changes, crescents in severe cases Gross, intermittent, painless hematuria No specific changes, although increased serum IgA levels observed in some patients HSP Same as IgA nephropathy Purpura , joint pains, abdominal pain, hematuria , etc No specific laboratory data SLE Mild glomerulitis , proliferative changes, immune complex deposition, crescents, immunoglobulin deposition Hematuria, proteinuria, hypertension, joint pains, rashes, etc Abnormal C3, C4, ANA, dsDNA , anemia, thrombocytopenia, etcPowerPoint Presentation: Dx Histology Clinical Lab Alport Syndrome Some thinning of basement membranes, "basket weave" changes in the glomerular basement membrane on electron microscopy Sensorineural hearing loss, corneal abnormalities, hematuria , renal failure No specific changes Thin Basement Membrane disease Average glomerular basement membranes thin (reported to be 100-200 nm) Persistent microscopic or gross hematuria, significant family history No specific changes Mesangio-proliferative GN Glomerular lobulations, thickening of the mesangial matrix and glomerular basement membranes, crescents, etc Hematuria, proteinuria, hypertension C3 levels may be lowInvestigations:: Investigations: Cystourethroscopy : Terminal hematuria , disturbances of micturition , suprapubic pain (Only if strong suspicion of bladder ulceration, tumours ) Screening of first degree relatives in persistent hematuria Investigations for other causes of red urine: Methemoglobin level, Plasma Tyrosine, Urine homogentisic acid, UroporphyrinsManagement:: Management: According to cause: Reassurance and F/U Treat cystitis, pyelonephritis, AGN: Antibiotics Supportive treatment: Diuretics, Fluid and salt restriction, Antihypertensives Monitoring – BP, I/O, weight, Urine R/M Treat Hyperkalemia, ARF, CHF, acidosis, fluid overload, HTN and its complications ACE inhibitors useful in proteinuria Immunosuppressive therapy: Depending on cause (Steroids, cyclophosphamide)Management:: Management: Idiopathic Hypercalciuria: Hydrochlorothiazide, Potassium citrate, Sodium restriction Calculi: Plenty of water ESRD: Dialysis, Renal transplantation Correct thrombocytopenia, anemia, coagulation factor deficiency Renal vein thrombosis: Anticoagulant therapy or thrombectomy may be needed Surgical correction: Calculi, PUJ obstruction, Posterior urethral valves, Wilms tumourCola/brown urine? Proteinuria>30mg/dL? RBC cast? acute nephritic syndrome?: Cola/brown urine? Proteinuria>30mg/dL? RBC cast? acute nephritic syndrome? Glomerular hematuria CBC with differential Electrolytes, ca BUN/creatinine Serum protein/albumin Cholesterol C3/C4 ASO/Anti DNase B ANA Antineutrophil antibody throat/skin culture if indicated 24 hour urine total protein creatinine clearance Extraglomerular hematuria Step 1 Urine culture Step 2 Urine calcium/creatinine Sickle prep Renal/bladder USG Step 3 Urine analysis, siblings, parents Serum electrolytes, Cr,Ca 24hr urine Ca, creatinine, uric acid, oxalate( if crystauria, nephrolithiasis Cystogram, +/- renal scan if hydronephrosis/pyelocallictasis NO YESReferences:: References: Nelson Textbook of Paediatrics , 18 th Ed Paediatric Nephrology and Urology, Kaplan, Meyers, 1 st Ed O.P. Ghai Essential pediatrics, 6 th Ed Pediatric Nephrology, Shrivastava , Bagga , 4 th Ed Various WebsitesPowerPoint Presentation: THANK YOU You do not have the permission to view this presentation. In order to view it, please contact the author of the presentation.