TABLE 1:

The PBS RUBACE Severity Scoring Rubric

EAGLE-BARRETT SYNDROME PBS TRIAD ("RUBAC") MAX SCORE: 16					EXTRA-GENITOURINARY MANIFESTATIONS ("E") MAX SCORE: 15
No subcategory points are awarded for normal anatomy with absent pathology					No subcategory points are awarded for normal anatomy with absent pathology
Renal Max Score: 6	Ureteral Max Score: 3	Bladder/Outlet Max Score: 3	Abdominal Wall Max Score: 2	Cryptorchidism Max Score: 2	Neurologic Max Score: 3	Cardiac Max Score: 3	Gastrointestinal Max Score: 3	Musculoskeletal Max Score: 3	Respiratory Max Score: 3
1 pt: G1-G2: Structural damage (dilation, dysplasia, scarring) with preserved GFR ≥ 60	1 pt: Low grade or absent VUR, or distal ureter 7-10mm	1 pt: urotherapy required to empty bladder, or bladder size is 100-200% of normal	1 pt: Can do "sit-up" exercise before or after abdominoplasty, musculature mostly intact, minimal laxity, or mild/nonconvincing prune appearance	1 pt: History of or current unilateral or palpable undescended testicle(s), or patient is female		1 pt: congenital septal defects (PDA, ASD, VSD) which are spontaneously resolved	1 pt: mild to moderate constipation, managed with diet or laxatives	1 pt: mild facies, abnormally shaped fingers or toes (no loss of function), or rib flaring	1 pt: minimal respiratory support at birth, some difficulty coughing, mild intermittant asthma, or mild reactive airway disease
2 pts: G3a-b: Kidney damage with decreased GFR 30-59	2 pts: Persistent high grade VUR (no surgery), s/p lower tract ureteral reconstruction, or distal ureter 1-2 cm	2 pts: Urethral-catheterization or pharm required to empty, bladder size >200% normal, or patient takes antibiotic prophylaxis for recurrent UTI	2 pts: Severe abdominal laxity with obvious thinning of abdominal wall, classic prune appearance, or s/p abdominoplasty + cannot do a "sit-up"	2 pts: History of or current bilateral nonpalpable testes		2 pts: small septal defects which are not spontaneously resolved, minimal L to R shunting, or PDA requiring surgical management	2 pts: MACE/cecostomy required, or malrotation s/p Ladd's	2 pts: scoliosis, hip dysplasia, club or rockerbottom foot, genu valgum, pectus excavatum or carinum	2 pts: persistent/moderate asthma, frequent respiratory tract infections (≥3/year), or ≥2 hospitalizations for pneumonia
3 pts: G4: Severe loss of kidney function with GFR 15-29					PBS-PLUS OR CONGENITAL/GENETIC SYNDROME OR ASSOCIATION
4 pts: Renal failure with GFR <15, or on dialysis or s/p renal transplant	3 pts: Persistent high grade VUR despite surgery, distal ureter 2+ cm girth, or current diverting ureterostomy or nephrostomy	3 pts: current surgical diversion (e.g. s/p tube, vesicostomy, APV), urethral atresia, or megalourethra requiring repair			3 pts: seizures, tethered cord, spina bifida, hearing loss, intellectual delay, autism, or severe neurologic condition not otherwise specified	3 pts: severe congenital or cyanotic heart disease-Tetralogy of Fallot, left sided obstructive lesions, reversed shunt, CHF or severe cardiologic conditions not otherwise specified	3 pts: required surgical bowel diversion (e.g. imperforate anus, anal atresia), gastrointestinal malignancy (e.g. hepatoblastoma), or severe GI condition not otherwise specified	3 pts: arthrogryposis, muscular dystrophy, or severe musculoskeletal malformation not otherwise specified	3 pts: ventilator-dependent > 1 week, tracheostomy, history of pneumothorax, or severe asthma
Additional Points for Young Age: if patient is <2 years, add +2 pt and if patient is <13 years, add +1 pt
TOTAL TRIAD RUBAC SCORE:____________					TOTAL E SCORE:____________
TOTAL TRIAD RUBAC SCORE____________ + TOTAL E SCORE____________ = TOTAL RUBACE SCORE____________