pediatric renal disease

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Pediatric Renal Disease Alyssa Brzenski

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Pediatric Renal Disease. Alyssa Brzenski. Case #1. - PowerPoint PPT Presentation

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Page 1: Pediatric Renal Disease

Pediatric Renal Disease

Alyssa Brzenski

Page 2: Pediatric Renal Disease

Case #1

• A 33 year old female G2P1 at 20 weeks presents for evaluation of fetal bladder distention which was found on routine prenatal ultrasound. There were no other abnormalities found. The mother wants to know what is the prognosis and what interventions she should undergo.

Page 3: Pediatric Renal Disease

Posterior Urethral Valve• 1:5,000 births• 1:2,500 prenatal

ultrasounds• Most common

obstructive uropathy

Page 4: Pediatric Renal Disease

Long term Side Effects

• Renal Scaring • Renal Failure• Decreased amniotic fluid• Pulmonary Hypoplasia

Page 5: Pediatric Renal Disease

Prenatal Ultrasound

Page 6: Pediatric Renal Disease

Theories of Etiologies

• Hypertrophy of the urethral ridge• Persistence of the urogenital membrane• Abnormal development of the wolffian or

mullerian duct• Fusion of the posterior urethral ridge

Page 7: Pediatric Renal Disease

Work-up- Voiding Cystourethrogram

Page 8: Pediatric Renal Disease

Antenatal Intervention

Page 9: Pediatric Renal Disease

Effectiveness of Antenatal Intervention on Survival

Page 10: Pediatric Renal Disease

Effectiveness of AnetnatalIntervention on renal function

Page 11: Pediatric Renal Disease
Page 12: Pediatric Renal Disease
Page 13: Pediatric Renal Disease

Outcomes

• Perinatal Mortality• Renal Function at 4-6 weeks• Serum Cr• Renal Ultrasound• Need for Dialysis/Transplant

• Renal Function at 12 months

Page 14: Pediatric Renal Disease

Prune Belly Syndrome• 1:40,000 births

• Weak abdominal muscles

• Weak cough

• Associated with• Orthopedic defects

(Congenital Hip Dislocation and Scoliosis)

• GI (malrotation and volvulus)

• Heart (TOF, VSD)• Trisomy 18 and 21

Page 15: Pediatric Renal Disease

Case 2

• 8 month old male presents to ED with fever of 102.8 and tachycardia. On initial work up a straight cath was performed which demonstrated a UTI. How should this child be evaluated?

Page 16: Pediatric Renal Disease

Vesicoureteral Reflux

• Present in 0.5-2% of children• May present with recurrent UTI or may be

asymptomatic• Most resolve without treatment

Page 17: Pediatric Renal Disease

Grading

Page 18: Pediatric Renal Disease

Prevalence in Siblings

Page 19: Pediatric Renal Disease

Prevalence with Hydronephrosis in Utero

Page 20: Pediatric Renal Disease

Current Management Guidelines

Page 21: Pediatric Renal Disease

Goals of Treatment

• Prevent recurrent UTI• Prevent Renal Damage/scarring• Minimize the morbidity of treatment and

followup

Page 22: Pediatric Renal Disease

Antibiotic Prophylaxis• Less than 1 years old• Febrile UTI- Antibiotic prophylaxis• Afebrile UTI with Grade III-V reflux- Antibiotic

Prophylaxis• Afebrile UTI with Grade I or II reflux- may offer

antibiotic prophylaxis

• Older than 1 years old• Febrile UTI- conservative management or antibiotic

prophylaxis• Recurrent UTI- start antibiotic treatment or if on

antibiotics surgical treatment

Page 23: Pediatric Renal Disease

Who needs surgery?

• Children with recurrent infections despite antibiotic prophylaxis

• Children who have developed renal scaring or poor renal function

• Severe reflux (Grade V or bilateral IV)• Mild to moderate reflux that persists as the

patient approaches puberty

Page 24: Pediatric Renal Disease

Deflux Injections• GA for cystoscopy

• Submucosal Injection of Deflux (dextranomer microspheres and hyaluronic acid)

• 80-90% success at first injection

Page 25: Pediatric Renal Disease

Ureteral Re-implant

• GA• Routine Monitors• Balanced anesthetic• Epidural or Caudal for post-op pain

management and to reduce post-op bladder spasm

Page 26: Pediatric Renal Disease

Case 4

• 5 year old, 15kg, female with chronic renal failure, secondary to polycystic kidney disease, is admitted for a kidney transplant. She is currently on peritoneal dialysis and was last dialyzed yesterday. She has limited exercize tolerance. Labs including potassium are all normal. ECHO was normal.

Page 27: Pediatric Renal Disease

Causes of Pediatric Renal Failure

From NAPRTCS Annual Report. 2010. Accessed March 25, 2013 at www.naprtcs.org

Page 28: Pediatric Renal Disease

Causes of Pediatric Renal Failure by Age

Page 29: Pediatric Renal Disease

Pediatric Chronic Renal Disease

• CV- HTN, LV thickening, CHF, Volume Overload

• Pulm-Volume Overload• GI- Delayed gastric emptying• Heme- anemia, dysfunctional platelets• Endo/Metabolic- Hyperkalemia,

hypercalcemia• Growth- Delayed growth

Page 30: Pediatric Renal Disease

Polycystic Kidney Disease

• Autosomal Dominant- 90% of cases• Typically presents in adulthood with

macrocysts

• Autosomal Recessive- 10% of cases• Presents in-utero screening or in early in

infancy• Microcysts of the collecting tubules

Page 31: Pediatric Renal Disease

Autosomal Recessive Polycystic Kidney Disease

• Affects • Kidneys- 30% progress to ESRD by 1st decade

with 58% needing a renal transplant by adulthood

• Liver- 50% will develop hepatic fibrosis with seqelae of portal hypertension

• May have pulmonary hypoplasia from decreased urine production in-utero

Page 32: Pediatric Renal Disease

Surgical Approach

Page 33: Pediatric Renal Disease

Anesthetic Considerations

• Intravenous Induction• Routine Monitors, CVP +/- Aline• Balanced Anesthetic• Epidural for Post-op Pain Control

Page 34: Pediatric Renal Disease

Anesthetic Considerations

• On release of the renal artery clamp, have CVP of 10-15 with blood pressure at baseline or 10% higher (may need pRBC or dopamine)

• Adult kidney in a small child or infant will require a significant portion of total blood flow, leading to potential hypotension (volume load prior)

• Small infants or very sick children should remain intubated, but most children can be extubated in the OR

Page 35: Pediatric Renal Disease

When should we remove a kidney?

• Nephrectomy before transplant due to:• Large proteinuria• Refractory Hypertension• Recurrent UTI or urosepsis• Urolithiasis• Polyuria

Page 36: Pediatric Renal Disease

Sources• Williams G, Fletcher J, Alexander S, Craig J. Vesicoureteral Reflex. Journal of American Society of Nephrology. May 2008; 19:

849-62.

• Peters C, et al. Summary of the AUA Guideline on Managemnt of Primary Vesicoureteral Reflux in Children. The Journal of Urology. Sept 2010; 184:1134-44.

• Bogaert G, Slabbaert K. Vesicoureteral Reflux. European Urology Supplements. April 2012; 11: 16-24.

• Skoog S, et al. Pediatric Vesicoureteral Reflux Guidelines Panel Summary Report: Clinical Practice Guidelines for Screening Children with Vesicoureteral Reflux and Neonates/Infants with Prenatal Hydronephrosis. The Journal of Urology.Sept 2010; 184: 1145-51.

• Holmes N, Harrison M, Baskin C. Fetal Surgery for Posterior Urethral Valves: Long-Term Postnatal Outcomes. Pediatrics. 2001; 108: 1-7.

• Casella D, Tomaszewski J, Ost M. Posterior Urethral Valves: Renal Failure and Prenatal Treatment. Internation Nephrology. 2012; 1-4.

• Uejima T. Anesthetic Management of the Pediatric Patient Undergoing Solid Organ Transplantation. Anesthesiology Clinics of North America. 2004; 22: 809-23.

• Sharbaf F, et al. Native Nephrectomy prior to Pediatric Kidney Transplant: Biological and Clinical Aspects. Pediatric Nephrology. 2012; 27: 1179-88.

• Dell K. The Spectrum of Polycystic Kidney Disease in Children. Adv Chronic Kidney Disease. 2011; 18: 339-47.