Jennifer Leechik, RN, BSN, C(Neph)C Renal Nurse Clinician BC Children’s Hospital March 20, 2013 Outline the etiology of chronic kidney disease in the
pediatric population
Review common congenital and acquired disorders that
lead to chronic kidney disease in the pediatric population
Discuss diagnostic tools and treatments for pediatric
patients
Focus on concerns in the pediatric population
Identify the different support systems in the pediatric
population 2/3 of pediatric CKD is caused by acquired GN and congenital anomalies.
About 15% of pediatric CKD is caused by rare hereditary diseases.
Although diabetes and hypertension may begin in childhood, these disorders cause CKD about 5% of the time. Obstructive uropathy 19% Aplastic/hypoplastic/dysplastic kidney 18
Hereditary diseases 15
Focal segmental glomerulosclerosis 14
Chronic GN 4
Unknown 8
TOTAL 75%
All other disease categories <4% each
*North American Pediatric Renal Transplant Cooperative Study Glomerulonephritis/Vasculitis Focal segmental glomerulosclerosis (FSGS) Chronic GN, unclassifiable Hemolytic Uremic Syndrome (HUS) Idiopathic rapidly progressive GN (RPGN) Membranoproliferative GN (MPGN), Type I MPGN, Type II Lupus nephritis Henoch Schonlein purpura (HSP) IgA nephritis Membranous GN Other GN (Wegener’s, ANCA vasculitis)
Commonly presented with Nephrotic Syndrome (NS) - proteinuria, hypoproteinemia, edema, hyperlipidemia
Focal segmental glomerulosclerosis (FSGS) Membranoproliferative glomerulonephritis (MPGN) Type 1&2 Membranous Nephropathy - not usually with NS but with nephritis
IgA Nephritis/ Nephropathy (Berger’s Disease) Lupus Nephritis
Presentation with NS or proteinuria that is unresponsive or resistant to steroid therapy
More likely to have hematuria and/or hypertension
Less common cause of NS than minimal change disease in children < 10 years
50% reach CKD within 10 years
If ESRD – 30% recurrence in initial transplant patients & >90% in subsequent transplants Presentation with NS, proteinuria or hematuria/nephritis that is controlled with steroid therapy
Low serum C3 in >70%
Type I progresses slowly to CKD in 10-20 years
Type II often progresses rapidly
Steroids: oral prednisone, 3 days of IV methylprednisolone
Cytotoxic agents: cyclophosphamide, cyclosporine, mycofenilate mofetile (MMF)
ACE Inhibitors/ARB Blockers: for chronic proteinuria
Control hypertension and edema: fluid & sodium restriction
Control anemia, renal osteodystrophy
Commonly occurs in asian, african american and hispanic girls
80% of children with SLE have some kind of renal impairment
Renal biopsy is needed for diagnosis and prognosis
Focal (class III) or diffuse proliferative (class IV) nephritis & rarely membranous GN (class V) may progress to CKD
Aggressively treating class III and IV with steroids or cytotoxic drugs is usually effective.
Also known as “Hamburger Disease”
Triad: Hemolytic anemia
Uremia Thrombocytopenia
Presentation with bloody diarrheal episodes, abdominal
cramps, irritability, lethargy, fever and vomiting
Peak age of onset: 1 – 2 years old
Acute dialysis: 30% Chronic dialysis: ~5%
E.Coli 0157 bacteria invades the GI tract and releases toxins that enter the bloodstream causing hemolysis of RBCs and
platelets (cells become irregular and ‘sticky’)
The damaged cells ‘clog’ the nephrons decreasing perfusion to the kidneys, decreasing u/o, causing electrolyte
imbalances, increasing BP, etc. – Acute renal failure
High risk of stroke, seizures, respiratory and cardiac issues
No cure; only symptomatic management
Vasculitis disorder that involves inflammation of the blood vessels
Presents with: petechial and purpuric rash mainly to lower extremities Abdominal pain N/V; anorexia Arthralgias to lower joints with soft tissue edema
Treatment: ◦ adequate hydration, or fluid intake ◦ careful attention to nutrition ◦ pain control with medications such as acetaminophen ◦ glucocorticoids (to control inflammation)
Onset <20 yrs of age
Aplasia/hypoplasia/dysplasia 44% Obstructive uropathy 40 Reflux nephropathy 12 Prune Belly Syndrome 4
Total 100%
Aplasia: The absence of a kidney Usually unilateral If bilateral, associated with other defects such hypoplastic lungs, oligohydramnios
Hypoplasia: Significantly small sized kidney with less nephrons; no dysplasia Unilateral or bilateral Usually develops hypertension
Kidney malformation with differentiation of the metanephric tissue
Unilateral or bilateral
Usually small in size
Maturation abnormality in the glomeruli and tubules
More common than aplasia and hypoplasia
Posterior Urethral Valves (PUV): ◦ Only in boys ◦ Valves situated at the distal portion of the prostatic urethra i.e. Proximal urethra causing obstruction in urine flow. ◦ Can be diagnosed antenatally during mother’s routine US that shows enlarged bladder, bilateral hydronephrosis, oligohydramnios. ◦ After birth, baby may have multiple UTIs and ‘dribbling’ ◦ Repair with ablation
Ureteropelvic junction (UPJ) obstruction Uretovesical junction (UVJ) obstruction ◦ Less common
Most common in caucasian girls Unilateral or bilateral Presents with multiple UTI Antibiotic prophylaxis may preserve function May develop CKD/ESRD as child grows older Familial – screen siblings Diagnosis by VCUG Renal U/S without (grades 1-2) or with (grades 3-5) hydronephrosis Also known as Eagle Barrett Syndrome Mostly in males Triad of symptoms: ◦ Complete or lack of abdominal muscle; wrinkled skin ◦ Undescended testicles ◦ Urinary tract abnormalities: Large ureter, large bladder, accumulation and backflow of urine Polycystic kidney disease 21% Medullary cystic/juvenile nephronophthisis 19 Congenital nephrotic syndrome 19 Familial nephritis (Alport) 16 Cystinosis 14 Denys-Drash/Fraser syndrome 4.5 Oxalosis (primary hyperoxaluria) 4 Sickle cell nephropathy 2 Other <1
Autosomal Recessive (“Infantile” PKD) enlarged kidneys found in fetus or at birth Tubular dilatation with small cysts in collecting ducts Associated with congenital hepatic fibrosis (scarring of the liver) – may need liver transplant ESRD usually before age 10
Autosomal Dominant (“Adult” PKD) Identifiable by multiple cysts on renal US in fetus, newborn or child, but usually diagnosed in adolescent or adult with hypertension Tubular dilatation with cysts throughout the nephron Cysts of liver, pancreas; Berry aneurysm of brain; diverticulosis ESRD usually after age 30
Medullary cystic disease Autosomal dominant Usually diagnosed in adolescence or adulthood
Juvenile nephronophthisis Autosomal recessive Presents with polyuria, polydipsia, anemia, weakness, &/or growth failure in child <10y/o Often associated with other anomalies (skeletal, opthalmologic,developmental delay) Autosomal Recessive Finnish type: passed down through families Presents before 6 months of age Abnormal form of nephrin (protein) found in the kidneys Presents with cough, decrease u/o, foamy urine, failure to thrive, poor appetite, edema, hypertension Routine U/A shows large protein and fat.
Treatment: ◦ Antibiotics to control infections ◦ Blood pressure meds: ACE inhibitors ◦ Albumin infusion (usually with diuretics) ◦ NSAIDS to slow protein buildup in the urine ◦ Possible nephrectomy and dialysis
Prognosis: Can lead to death by 5 years of age but many die within their first year. Inherited disorder involving the basement membranes of the kidney, cochlea and the eye.
1) X-linked (80%) • Gene mutation • High sensory, progressive, sensorineural deafness • Lens abnormalities of the eyes • ESRD progression in mostly males by age of 20
2) Autosomal recessive (15%) • Deafness and ESRD for males and females by age of 30
3) Autosomal dominant (~5%) • Deafness and ESRD early in life • Associated thrombocytopenia and platelet abnormalities
Metabolic disease
Autosomal recessive
Lysosomal storage disorder caused by defective
transport of the amino acid cystine out of lysosomes that crystallizes damaging the tissues of kidneys and other organs. Fanconi Syndrome: proximal tubular wasting of amino
acids, glucose, bicarb, phosphate, calcium, magnesium, uric acid, organic acids, low molecular wgt proteins, sodium, potassium and water Corneal ulcerations, retinal blindness, severe
photophobia Severe growth failure; treated with GH
Most common malignancy of the urinary tract in children Abnormal proliferation of metanephric blastema that develops nephrons 90% present before the age of 7 Peak onset at age 2-4 years old Treatment: Chemotherapy and/or radioactive therapy Resection of the tumor to preserve tissue > 85% survival rate with combination therapy Can lead to ESRD Need to be at least in remission for 1 year for kidney transplantation
Specific therapy for specific disease
Gene analysis
Sibling/family screening
Prenatal diagnosis
?Gene therapy in future
Interstitial nephritis (IN) Idiopathic Can occur after streptococcal infection Drug related acute IN (methicillin, ampicillin, penicillin, sulfonamides, NSAIDS) Chronic pyelonephritis Associated with reflux nephropathy, chronic inflammation Not chronic urinary tract infection Presentation Mild proteinuria with few other urine abnormalities Hematuria in drug related cases Acute or chronic renal failure Blood work (urea, creatinine, electrolytes, hematology) Urine testing (U/A, C&S, 24hr) Ultrasound Cystoscopy, VCUG Renal biopsy
Medication dosing are specific and ordered by the weight of the child. ◦ Oral: pills, capsules, liquid ◦ Intravenous, CVC ◦ NG/GT ◦ Intraperitoneal Most renal medications come in different formulation
Need to consider decreasing dose of medication depending on kidney function. Imperative for medication education for adolescents and families (especially for transplantation) ◦ Name and describe the drug ◦ What is it for? ◦ When to take it? ◦ How to take it? ◦ Complications from it? Importance of immunizations prior to transplantation Transplantation Pre-emptive whenever possible Donor needs to be >2years old Recipient needs to be at least 10 Kg Living Related Donor preferred Workup may take up to 2-3 months Medical priority on the waiting list until the age of 19 Children have better overall outcomes
Peritoneal Dialysis Mostly CCPD, but able to do IPD and CAPD Dwell volumes ranging from 60cc-2500cc Use single and double cuff coiled catheter depending on child’s size First choice (if able) More gentle to body Immature veins and arteries Preserve vascular accesses for future
Hemodialysis Extracorporeal circuit volumes of 60-200ml 3-4 times a week for 2-4 hour runs CVC: permanent double lumen catheters AVF: 15 gauge needles, older adolescents
End of Life/Palliative Care Ethics Review Parents request
1) Growth and Development Monthly height and weight; head circumference <2 years old Nutritional supplements to maintain growth (formulas, protein powders, caloric supplements, oils) Growth hormone started if growth falling under the 3rd percentile NG/GT feeds if needed Possible use of IDPN
Protein recommendations for 0-6 month old (example):
◦ Non- CKD: 1.5g/Kg/d ◦ Stage 3 CKD : 1.5 - 2.1g/Kg/d (100-140% DRI) ◦ Stage 4-5 CKD: 1.5 – 1.8g/Kg/d (100-120% DRI)
◦ HD: 1.6g/Kg/d (100% DRI + losses) ◦ PD : 1.8g/Kg/d (100% DRI + losses)
2) Bone Disease Prevent osteodystrophy, rickets Monitor iPTH monthly Bone Age and X-ray of hands and wrists done yearly Monitor dietary phosphate intake
3)Transitioning to Adulthood Transition Clinic following formalized pathway starts at 12-13 years of age Youth Health involved Physical development ◦ Body Image: smaller stature, weight gain/loss, acne, catheters, etc.
Cognitive development ◦ Missing school, lack of concentration, lack of motivation, etc.
“Chronically ill child syndrome” Adherence Communication
Support System
Multidisciplinary Team: Community: Nephrologists Family Members Nurses Pediatricians Social Workers Respite Nurses Dieticians Peers (i.e. camp) Pharmacists
Psychologists Child Life Specialists School Teachers Volunteers