Racial Differences in Paroxysmal Nocturnal Hemoglobinuria ...
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![Page 1: Red urine Hemoglobinuria Myoglobinuria Drugs (rifampicin),food Inborn errors of metabolism(porphyria) Urate crystals Hematuria Do dipstick,microscopy (>5cells/ul.](https://reader036.fdocuments.net/reader036/viewer/2022062309/56649ee75503460f94bf8640/html5/thumbnails/1.jpg)
Red urine
Hemoglobinuria Myoglobinuria Drugs (rifampicin),food Inborn errors of metabolism(porphyria) Urate crystals Hematuria Do dipstick,microscopy (>5cells/ul in
fresh uncentrifuged urine, >3 cells/HPF in centrifuged sediment.
![Page 2: Red urine Hemoglobinuria Myoglobinuria Drugs (rifampicin),food Inborn errors of metabolism(porphyria) Urate crystals Hematuria Do dipstick,microscopy (>5cells/ul.](https://reader036.fdocuments.net/reader036/viewer/2022062309/56649ee75503460f94bf8640/html5/thumbnails/2.jpg)
Red if fresh(bladder), or brown color as Hb converted to acid haematin by urinary acids
Early hematuria:urethral cause Terminal hematuria:bladder cause Microscopy:dysmprhic RBC,RBC
cast:glomerular bleeding Prevelance of .5-1.6% in school children
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RBC casts
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RBC casts
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History
Fever,urinary symptoms….infection Colicky loin pain,lower tract
symptoms…..renal calculus Rash,arthritis…HSP,SLE Preceding URTI…..PSGN,IgA nephropathy Coagulopathy,trauma,tumor,exercise FH of hematuria,deafness,renal failure…
Alport O/E:HTN,kidneys,genitalia,rash
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Investigations
Urine protein/creat ratio EUC,albumin ASOT,C3,C4,ANA FBC,clotting studies Urine calcium/creat ratio U/S ,XRAY,CT Urine analysis on parents IgA,cystoscopy Renal biobsy
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Macroscopic hematuria
Most common cause is infection,then perineal irritation,trauma
Viral infections,adenovirus 11,12 may cause hemorrhagic cystitis
Exercise induced hematuria not associated with renal disease.
Recurrent gross hematuria as IgA nephropathy,Alport,nut cracker (thin,loin pain,compression of renal vein bet aorta,SMA
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Causes of hematuria
1.Glomerular causes Familial benign hematuria GN:primary as postinfectious,MPGN,IgA
nephropathy secondary GN as SLE,HSP. HUS,interstital nephritis,renal vein
thrombosis,cystic renal disease Alport
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Non glomerular causes UTI Hypercalcuria,renal calculi Trauma,exercise Coagulpathy as sickle Vascular malformations Nut cracker syndrome Menarche Malignancy as nephroblastoma of the
kidney or bladder tumors
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Persistent MA without proteinuria : 1.Beningn familial hematuria 2.idiopathic hypercalcuria 3.IgA nephropathy 4.Alport syndrome
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Persistent MA without proteinuria : 1.Beningn familial hematuria 2.idiopathic hypercalcuria 3.IgA nephropathy 4.Alport syndrome
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Alport Syndrome
80% XL,20% AR
Renal failure,sensorineural deafness higher frequencies,ocular changes(anterior lenticonus,retinal changes
Present as micro and rarely macroscopic hematauria with URTI
Proteinura,HTN later age
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Diagnosis by EM:Thinning of GBM,split and duplicated lamina densa,basket weave
Males progress to ESRD,deafness by 30y
ACEI may delay progression to ESRD
Defiency of α5 of type 4 collagen
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Benign Familial Hematuria(TBMN)
AD inheritance Present as microscopic
hematuria,no proteinuria or renal failure
EM:thinning of GBM Follow up for proteinuria,HTN
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GBM in Alport syndrome
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GBM in Alport syndrome
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IgA nephropathy
Recurrent macroscopic hematuria,loin pain 1-2 days following URTI,last < 3 days.
Persistent microscopic hematuria ±proteinuria
Nephritic,nephrotic syndrome rare
Present second decade,more in males
![Page 24: Red urine Hemoglobinuria Myoglobinuria Drugs (rifampicin),food Inborn errors of metabolism(porphyria) Urate crystals Hematuria Do dipstick,microscopy (>5cells/ul.](https://reader036.fdocuments.net/reader036/viewer/2022062309/56649ee75503460f94bf8640/html5/thumbnails/24.jpg)
Familial cases reported IgA high in 35-50% Diagnosis:LM:focal or diffuse mesangial cell
proliferation,expansion of mesangial matrix IM:IgA,C3 deposits Prognosis for children better than adults Young children without macroscopic
hematuria have the best long term outcome
![Page 25: Red urine Hemoglobinuria Myoglobinuria Drugs (rifampicin),food Inborn errors of metabolism(porphyria) Urate crystals Hematuria Do dipstick,microscopy (>5cells/ul.](https://reader036.fdocuments.net/reader036/viewer/2022062309/56649ee75503460f94bf8640/html5/thumbnails/25.jpg)
Heavy proteinuria is a risk factor for progression to ESKD.
Progression to ESRD is slow(25% need dialysis in 20y)
Children with nephrotic syndrome are treated with steriods
ACEI are used to delay progression,decrease proteinuria
Fish oil
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IF showing mesangial depostis
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Normal glomerulus
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IgA deposits
![Page 29: Red urine Hemoglobinuria Myoglobinuria Drugs (rifampicin),food Inborn errors of metabolism(porphyria) Urate crystals Hematuria Do dipstick,microscopy (>5cells/ul.](https://reader036.fdocuments.net/reader036/viewer/2022062309/56649ee75503460f94bf8640/html5/thumbnails/29.jpg)
Thin glomerular basement membrane disease
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PROTEINURIA
DIPSTICK values .trace (.15g/l),+1 (.3g/l),+2 (1g/l),+3 (3g/l),+4
(20g/l)
False postive when urine concentarted,alkaline urine
Children excrete 150 mg/day of protein or 60-240 mg/m2/day
40 % of normal urinary protein is tissue rather than plasma as tamm-horsfall (a gp secreted in TALH )
Almost of the flitered smaller proteinare absorbed in the proximal CT.
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Protein-creatinine ratio to estimate protein excretion
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Urine protein/creat ratio correlates with measurement of protein in 24 hour collection.
Most normal subjects have a ratio below.02 mg/umole (.2g/g) in above 2y,.05 in children 6-24 m
Micoalbuminuria ranges 30-300 mg albumin/g creat
B2-microglobulin in urine indicates proximal tubular injury
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Classification 1-Functional:seen in
stress,fever,cold,exercise,CHF
2-Idiopathic transient or intermittent
3-postural:in adolesents,males,less than 2g/day,proteinuria in upright position but not when recumbent
Decrease in amount with age Has a good outcome
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4-pathologic:increase permeability of GBM,loss of anions(nephrotic,GN)
Loss of nephron mass:single kid,cystic disease from hyperfilteration
secretory,overflow:UTI,in newborns as tam