Morning Report

Lu Lu Waterhouse, PGY-2 March 28, 2012

3mo M w/ FTT, vomiting, hematochezia

3 day PTA: blood specks in every stool, still eating well

PCP called Dr. Jackson who recommended Elecare formula for possible milk protein intolerance and mom to start dairy-free diet KUB done at OSH reportedly normal

NBNB emesis with every feed Pt would only take 1-2oz of Elecare per feed. Parents tried mixing Elecare with breastmilk and then switched back to breastmilk 1 day PTA with improvement in emesis, but pt still taking only 1-2oz Also with looser than usual stools; blood specks resolved Afebrile Abdomen was not hard or distended Parents took him to PCPs office who found he had lost weight, arranged for direct admit to PCMC for further work up.

Past Medical History

Ex-35 week preemie, spent 2 months in PCMC NICU Gastroschisis w/ jejunal atresia, prenatally diagnosed

Silo placed and Jejunostomy done on 1st day of life Staged abdomen closure completed at 1 week old Reanastomosis done at 1 month old, ileocecal valve removed

NEC at 2 weeks old (bloody stool)

Medically treated

Enterococcus faecium and CoNS central line infection at 2wks old h/o anemia requiring multiple transfusions Discharged 2/10/12 on PO feeds of breastmilk fortified to 22kcal/oz with microlipids

Had been eating very well (4oz q3) Gaining weight: 8lb on NICU discharge, 8lb 14oz one week prior to admission

MEDS: PCP had started Zantac 3 days ago ALLERGIES: NKDA IMMUNIZATIONS: UTD FHx: no known h/o GI disorders, metabolic or genetic disorders, autoimmune disorders SHx: Lives with mom and dad. He is first child. Does not attend daycare. No recent travel. No one else sick in family. ROS: hematochezia, now seems resolved; NBNB emesis; decreased PO; loose stools PHYSICAL EXAM:

Wt 3.4kg = 7lb 7oz (<0%ile), Ht 55.5cm (0%ile), OFC 37.5cm GEN: Pale, cachectic appearing, but alert and in NAD HEENT: Head appears large compared to body, AF open, slightly depressed, MMM but lips dry. O/P clear. Sclera non-icteric. ABD: Soft, NT/ND, +BS. Liver edge 1cm below RCM, no masses. Well healed surgical scars. EXT: Thin, slightly mottled, but warm. No edema or cyanosis. SKIN: Pale, thin skin that is slightly loose. No rashes or jaundice.

LABS & IMAGING

CMP: Na 125, K 4.9, Cl 95, bicarb 12, BUN 30, Cr 0.41, gluc 84, Ca 10.0, prot 7.3, alb 4.3, bili 1.1, alk phos 248, ALT 62, AST 70

Anion Gap = ___

CBC: WBC 18.1 (16%N, 47%L, 10%M, 25%E), Hct 36.2, Plt 397 CRP <0.5, ESR 0

U/A: SG 1.025, pH 5.5, trace protein. 1 WBC, 0 RBC, no bacteria, 2+ urate crystals, 16/lpf hyaline casts Stool guaiac: positive Stool culture and EHEC pending
KUB: Diffuse mild bowel wall thickening, with scattered air-fluid levels that may represent underlying gastroenteritis. Additionally, there is an unusual collection of mottled linear lucency in the left lower quadrant; pneumatosis is a consideration.

DDx for 3mo w/ significant NICU history w/ FTT, vomiting, hematachezia and metabolic acidosis

GI:

ID:

Food protein-induced proctitis/colitis, enteropathy, and enterocolitis of infancy NEC Meckels diverticulum Anal fissure Intussusception Vascular malformation Eosinophilic esophagitis or gastroenteritis GER Dehydration

Viral gastroenteritis Bacterial gastroenteritis Sepsis

RENAL:

RTA type 4 (hyperkalemia is cardinal feature) RTA (proximal) type 2 (low bicarb is cardinal feature)

METABOLIC ACIDOSIS:

MUDPILES

NAT

Food protein-induced proctitis/proctocolitis, enteropathy, and enterocolitis of infancy

Terms like milk protein intolerance and cows milk sensitivity are no longer recommended. Classification of immunologic reactions

IgE mediated
Immediate hypersensitivity (anaphylaxis, hives) Oral allergy syndrome

Non IgE mediated

Food protein-induced proctitis/proctocolitis Food protein-induced enteropathy Food protein-induced enterocolitis syndrome (FPIES)

Mixed
Eosinophilic esophagitis Eosinophilic gastroenteritis

Food protein-induced proctitis/proctocolitis

Vast majority of affected infants are breastfed Inflammatory response limited to rectum and distal sigmoid colon Infants are well appearing with blood-tinged stools Breastfed:

Completely eliminate all dairy, including butter, casein, and whey May need to eliminate soy, then egg if not improved Replace with protein hydrolysate formula
Alimentum, Nutramigen LIPIL, Pregestimil

Formula fed:

5-10% may need amino acid based formula

Elecare Infant, Neocate Infant, Nutramigen AA

Response: visible bleeding decreases within 72hrs Prognosis: almost all can tolerate cows milk and soy by 1yo (can start reintroducing at 6mo)

Food protein-induced enteropathy

Infants have FTT, malabsorption, anemia, diarrhea, vomiting, hypoproteinemia (symptoms overlap with other enteropathies like Celiacs) Usually occurs if infant is fed intact cows milk <9mo or following gastroenteritis in response to other foods (eggs, rice, poultry, fish, shellfish) Diagnosis: endoscopy with biopsy in small bowel patchy villous atrophy Management: strict elimination of offending protein Prognosis: generally resolves spontaneously after 2yo

FPIES (or DPIES)

Infants usually sicker, have bloody diarrhea, similar symptoms with enteropathy (but affects SB and colon)

DDx can include sepsis, metabolic disorders surgical abdomen, anaphylaxis Poor weight gain, dehydration, hypoalbuminemia, leukocytosis Very rare in breastfed infants

Cows milk and soy are most common proteins, but almost any protein can trigger FPIES Diagnosis: endoscopy with biopsy crypt abscesses in colon Management:

Strict maternal diet elimination of causal protein if breastfed Protein hydrolysate formula or AA based formula Reintroduce after FTT has achieved full catch-up growth and no earlier than 6mo (re-challenge cautiously)

Prognosis: most are able to tolerate causal protein 1-2yrs after diagnosis. Some may have prolonged sensitivity or develop IgEmediated allergy.

Take Home Points

RAST (in vitro immunoassays) and skin prick testing are not recommended because disorders not IgE mediated (but can have eosinophilia) Food protein-induced proctitis/proctocolitis is most common Strict elimination, whether through mothers diet if breastfed or formula change is appropriate management

Almost all infants grow out of protein intolerance, generally can start reintroducing around 6mo