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40 Cards in this Set
- Front
- Back
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Autosomal Recessive Polycystic Kidney Disease
Diagnosis: |
Made mostly by clinical criteria
Markedly enlarged echogenic kidneys at birth with poor corticomedullary differentiation Microcysts in linear radial pattern in cortex and medulla. Disease confined to collecting tubules Kidney biopsy usually not necessary Genetic testing for the PKHD1 gene usually not needed if clinical criteria are met -Hepatic findings Hepatic fibrosis Hepatomegaly Dilated intrahepatic (sometimes extrahepatic) bile ducts and mild echogenicity Liver function test generally normal -Lung Pulmonary hypoplasia Respiratory insufficiency -Renal function (i.e. BUN and creatinine) impaired -Hypertension treated with ACE inhibitors. May also require addition of nifedipine, hydralazine, and hydrochlorothiazide -Growth failure – may need growth hormone -Anemia – treated with oral iron and epoetin; may require PRBC transfusions -Nutritional problems |
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Autosomal Recessive Polycystic Kidney Disease
Prevention of Secondary Complications: |
Pneumococcal, H. influenza type B, and meningococcal vaccines
Palivizumab for RSV |
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Autosomal Recessive Polycystic Kidney Disease
Follow-up (Regular): |
BP
Renal function Hydration Nutrition Respiratory status Serum lytes, BUN, creatinine CBC |
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Agents to avoid
Autosomal Recessive Polycystic Kidney Disease |
Sympathomimetic drugs when hypertension is present
Known nephrotoxic agents including NSAIDs and aminoglycosides |
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1st step in hematuria
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1. Routine Urinalysis
-Dipstick Hemoglobin Myoglobin -Microscopy >5 RBC per hpf (40x) on spun urine WBC’s? Casts RBC morphology |
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“Red” Urine in diaper
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Neonates
Urate crystals Red/pink/orange stain in diaper Medications Other Chemicals |
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Red Urine
Positive dipstick, negative microscopy |
-Hemoglobinuria
Acute hemolysis DIC -Myoglobinuria Rhabdomyolysis Burns Myositis asphyxia |
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Red Urine
Positive blood on dipstick, positive red blood cells, absence of red blood cell casts |
Bleeding from a site past the renal tubules
Hematuria |
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Red Urine
Positive blood on dipstick, positivbe red blood cells, presence of red blood cell casts |
Glomerular disease
Nephritis |
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Glomerular vs Extraglomerular hematuria
table to draw |
Marshall ppt 1 slide 23
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***Causes of Gross Hematuria
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Urinary Tract Infection
Sickle cell trait/disease Trauma Strenuous exercise Hypercalcuria Urolithiasis Structural defects – obstructions, tumors |
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Causes of Microscopic Hematuria****
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IgA nephropathy
Benign familial hematuria Alport syndrome May progress to gross hematuria |
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Causes of Nephritis**
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Immune mediated inflammation
Post Streptococcal Acute Glomerulonephritis Inherited disease Alport syndrome -Vascular injury Acute tubular necrosis Cortical necrosis |
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Post Streptococcal Acute Glomerulonephritis (PSAGN)**
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Follows a strep infection, usually throat or impetigo
Usually 2-12 years boys > girls Social Crowding Poor hygiene Malnutrition Intestinal parasites |
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PSAGN Clinical Manifestations**
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Edema and cola- or tea-colored urine are most common presentation
Gross Hematuria (65%) Peripheral edema (75%) Hypertension (50%) Acute renal insufficiency |
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PSAGN Clinical Diagnosis**
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Hematuria
Proteinuria Edema Hypertension |
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Laboratory Evaluation for PSAGN**
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1. Urinalysis
Blood Protein Concentrated urine Casts -RBC -Granular 2. Metabolic panel to assess renal function (BUN, Cr) 3. Document strep infection Throat swab/culture ASO titer Streptozyme 4. Complement C3 – decreased C4 - normal |
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PSAGN Therapy
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-Sodium restriction
-Diuretics --------Furosemide -Antihypertensives --------Amlodipine --------ACE inhibitors |
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Prognosis of PSAGN**
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Very good
Hematuria and hypertension last several months Treat strep infection >95% recover completely |
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Hemolytic Uremic Syndrome/HUS
Characteristic triad |
1. Microangiopathic hemolytic anemia
2. Thrombocytopenia 3. Renal Injury (insufficiency or failure) |
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Hemolytic Uremic Syndrome/HUS
Pathophysiology |
-Verotoxins
Shiga-like toxin from E. coli 0157:H7 Outbreaks from contaminated meat,fruit,vegtables and water HUS results in 5-25% of affected children Any bacteria which makes verotoxins can cause HUS |
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HUS
Clinical Findings |
-Gastroenteritis
-One week later Weakness Lethargy Irritability Pallor Edema Petechia Dehydration Seizures (20%) |
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HUS
Work up |
-Complete Blood Count
Leukocytosis Thrombocytopenia -Urinalysis Hematuria Proteinuria Casts -Coombs test negative -Stool culture |
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HUS Treatment
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-Supportive
Volume repletion/control Dialysis may be necessary Red blood cell transfusion as necessary Avoid antibiotics Avoid antidiarrheals Platelets infused only for bleeding |
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Pediatric Urinary Tract Infection
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-Urine and Urinary tract normally sterile
-Urinary tract infection includes Cystitis Pyelonephritis – infection of parenchyma, calices or pelvis Renal abscess – intrarenal or parenchymal -Febrile vs Afebrile |
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-Urinary tract infection includes
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Cystitis
Pyelonephritis – infection of parenchyma, calices or pelvis Renal abscess – intrarenal or parenchymal |
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Bugs of UTI
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-E. coli – 90% of 1st infections
P-pili adheres to various receptor on epithelium -Klebsiella -Enterococcus -Pseudomonas -S. saprophyticus Staph,chlamydia,Ecoli - acute urethral syndrome |
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Host Factors of UTI
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Short urethra in females
Colonized forskin of uncircumsized males Sexual intercourse Diaphragms Urine and stool holding Vesicoureteral reflux (VUR) Anatomic abnormalities |
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UTI epidemiology
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5% of girls by age 11 years
1% of boys by age 11 years 30% of girls over lifetime 1% of boys over lifetime |
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Clinical Symptoms of UTI
neonates |
Failure to thrive
Feeding problems Hyperbilirubinemia (indirect vs direct) |
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Clinical Symptoms of UTI
1 month to 2 years |
Feeding problems
Failure to thrive Diarrhea Vomiting Fever |
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Clinical Symptoms of UTI
> 2 years |
Typical symptoms of older child/adult
Urgency Dysuria Frequency Abdominal pain Fever |
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Diagnosis of UTI
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Requires urine culture
Suprapubic aspiration is gold standard Clean catch: >100,000 cfu/ml Catheter: >10,000 cfu/ml Suprapubic tap: >1,000 cfu/ml Don’t use a bag!!!!!!! Urine culture should be obtained in all children who have dysuria, frequency, hematuria, recent onset of enuresis, and those <3 years old with unexplained fever -Urinalysis Pyuria >10 WBC/mm3 Leukocyte esterase – only 50% sensitive for pyuria Nitrates – only 30% sensitive for colony count >100,000 Bacteria |
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UTI Complications
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Relapse
Bacteremia Nephronia Abscess |
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Outpatient Therapy of UTI
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Amoxicillin
TMP/SMX Nitrofurantoin Treat for 10 days |
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Inpatient Therapy of UTI
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<1 year
Vomiting Questionable compliance Cocci- Ampicillin Gm - Rod- gentamicin TMP/SMX Ceftriaxone |
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Additional Workup for UTI
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-Renal ultrasound
-Voiding Cystourethrogram (VCUG) Vesicoureteral reflux Ureteral duplication -Radionuclide scans Controversial Identifies scarring Scarring doesn’t appear for at least 4 months |
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Vesicoureteral Reflux
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-Can result in scarring of the kidney
Scarring can lead to hypertension and ESRD -Grade 1-3 resolve at 13% per year for 1st 5 years Then 3.5% per year afterward -Grade 4 & 5 resolve at rate of 5% per year |
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VUR Therapy
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-UTI prophylaxis
TMP/SMX Nitrofurantoin -Treat constipation -Ureter Reimplantation Surgery |
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UTI Prophylaxis and Prevention
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Antibiotic prophylaxis
TMP/SMX Nitrofurantoin Treat constipation Ureter reimplantation therapy |
