Problem Rounds

May 18, 2006

Almost 4 year old male

with abdominal pain for 1 week
CC: abdominal pain x 1 week
HPI: Almost 4 year old Hispanic male was in usual state
of health until one week prior to admission when he
began to have abdominal pain. Patient began on 4/30/06
to complain of diffuse abdominal pain, greatest
immediately after eating. Patient’s abdomen also was
noted to swell and become taut. Mom would note relief
from pain after the patient had a bowel movement but
continued swelling. No fever, vomiting, or diarrhea.
Patient was having regular bowel movements (1/day,
brown, soft, NB but decreased in caliber-pinky size). No
history of constipation. No change in diet. No other sick
contacts or other family members with similar symptoms.
No recent URI or sore throat symptoms.
instructed parents to bring patient to WCH PER due to
abnormalities in his labs.
HPI Continued: Six days PTA, mom took patient to local clinic
as patient had an episode of vomiting (NB/NB, looked like food,
small amt) and continued abdominal pain. He had begun to have
decrease appetite and only wanted to lay down due to pain and
abdominal swelling. New symptom was the onset of headache
with abdominal pain. At the clinic, a hemacue was checked as
well as urine and mom was told everything was normal and the
abdominal pain was due to gas. The patient continued to have
abdominal pain and swelling – now drinking only milk and
orange juice with some fruit. Patient had another similar
episode of vomiting the following day and at noon on day of
admission.
Four days PTA, patient’s eyes became swollen and he was barely
able to open his eyes for 4 days, resolving one day PT coming to
the ER. No warmth or redness of the eyes, discharge or tearing,
and no itchiness.
HPI Continued: On day of admission, mom brought
patient to Outside Hospital due increased abdominal
pain, abdominal swelling and now swelling of BLE and R
cheek. Patient developed bilateral lower extremities
(describes as the top of his feet) swelling 2 days PTA and
had stopped walking at home- now spending the entire
day laying on the couch or bed. No redness, warmth or
color change associated with the swelling. On morning of
admission mom states patient awoke with swelling of his
right cheek and that both cheeks look red. Swelling does
not appear to be painful to the patient but mom notes he
would intermittently touch his right cheek- puzzled.
At Outside Hospital, mom states blood and urine were
obtained and then the ER physician instructed parents to
bring patient to WCH PER due to abnormalities in his
labs.
 Take a moment
to consider your differential diagnosis
ROS:
General: no fevers, no night sweats, no chills, no observed
weight loss
Neuro: + headache with abdominal pain
CVS: no chest pain
Pulm: no SOB or ↑WOB, + dry intermittent cough 1-2
days PTA
Abd: no diarrhea, normal BM 1/day, + vomiting x 3
Renal/GU: no dysuria, hematuria, polyuria
Derm: no rash or bruising

PMD: a clinic, no regular PMD

Birth Hx: FT, NSVD at Outside Hospital, +PNC, no
complications with pregnancy or labor. Spent one day in
the hospital, birth weight 9 lbs, 6 oz.
PMHx: none
PSurgHx: none
Hospitalizations: none
All: NKDA
Meds: Tylenol PRN headache/abdominal pain- given
twice

Diet: Well balance diet. Fast food once/3 weeks. + candy-

lollipops and chocolate.
24oz of milk per day, 16 oz juice, some water. No
vitamins
FH: mom- 24, father 25, mom works at fast food
restaurant, father works as a plumber. There are two
children- patient 31/2 year old male and 2 year old sister.
No family history of asthma, DM, cancer, HTN, CAD,
MI/strokes, GI, kidney or rheumatologic disease
SH: Lives with parents in 2 bedroom house.
No pets, alcohol, tobacco, drugs, violence, guns.
Patient in preschool- no known illnesses/outbreaks,
enjoys school, likes to sing his ABCs.
No sick contacts. No recent travel or foreign visitors, no
exotic foods.
DevHx: walk at 8 months, first words “mama” at 10
months. Bilingual, speech fully understandable. Dress
himself, rides a tricycle with helmet.
Admission PE:
Temp 97.8F BP 93/61 HR 105 RR 20 Pain 0/10
Wt 22kg (>95th%) Ht- 109 cm (>95th%) BMI- 18.5 (>95th%)
Gen: well-developed, well-nourished male, smiling, cooperative, NAD
HEENT: NCAT, PERRL, EOM intact, sclera clear, no periorbital edema, TM
clear bilateral, nares turbinates non-erythematous, + dried discharge, no
polyps, MMM, good dentition, no oral/mucosal lesions, OP clear, tonsils 2+ no
exudates, no erythema, neck supple, FROM, no palpable thyroid, + facial
symmetry, fullness to R lower cheek, redness to both cheeks, NT
Lymph: no LAD- cervical, posterior, axillary or inguinal
CV: RRR, normal S1 and S2, no m/r/g
Pulm: CTA B/L, slightly decreased BS bilateral bases, dullness to percussion
BL bases, no w/r/r, no retractions
Abd: mildly distended, positive BS, NT, no visible fluid wave, no HSM, no
masses
GU: Tanner 1 male, uncircumcised, testes down B/L, mild edema scrotum/fat
pad, NT
Ext: no c/c. No edema on B/L feet, no sacral edema, no other swelling of any
extremities. 2+ distal pulse. CR~ 2 seconds
Neuro: CN II-XII intact, strength 5/5, DTR 2+ bilaterally, normal gait
Skin: no petechiae/purpura, no ecchymoses, no rash, no jaundice or pallor
ER course:
Arrived at 1316
Vitals: T- 97 (ax), BP 97/57, HR 119 RR 24
pain 0/10
Cc: abdominal pain x 1 week and mom notes increasing
abdominal girth
Was seen at OSH and referred with lab results:
albumin 1.3, radiology report of small ascites and BL small
pleural effusions, hemoconcentrated H/H, ua- trace protein

Abdominal girth measured: 65.4 cm

ER PE:
Gen: asleep, arousable, smiling, in NAD
HEENT: NCAT, PERRL, EOM intact, no periorbital edema,
TM clear B/L, dried discharge noted on B/L nares, MMM, OP
clear, no exudates, neck supple, no LAD
Pulm: CTABL, decreased BS at bilateral bases, + dullness to
percussion at bases
CV: tachycardic, RR, no m/r/g
Abd: soft, NT, slight distension, + hyperactive BS, no HSM
GU: Tanner I male, uncircumscribed, testes descended BL, slight
scrotal edema
Back: no deformities
Ext: no c/c/e, pulses 2+ cap refill<2 secs
Neuro: CN II-XII grossly intact
Skin: erythematous cheeks, no other rashes
ER Plan:
admission- fluid management
diagnosis-
abdominal pain
hypoalbuminemia
R/O nephrotic syndrome vs protein losing gastroenteropathy,
R/O spontaneous bacterial peritonitis
labs- CBC, TPN panel, PT/PTT, blood culture, C3/C4, urine dip,
micro and culture, repeat CXR, KUB
Admission Plan- spoke with Renal and GI Attendings
Neuro- monitor pain- abdominal, activity bed rest
CVS- HDS
Pulm- CXR, SORA, watch for S/S respiratory distress, O2 sat
Q4
FEN/GI- regular diet, no fluid restriction
Renal- strict I/O, maintain balance
ID- watch fever- SBP, f/u urine and blood cultures
Heme- H/H hemoconcentrated- 3rd spacing
Labs:
total IgA, anti-endomysial IgA and anti-tranglutaminase IgA
upper GI w/contrast- increase gastric folds
CMV and h.pylori
Studies
CXR- reassess pleural effusions
KUB
Upper GI
 Take a moment
to consider your differential diagnosis
Labs
Labs Continued
Labs Continued
 CXR: Expiratory film, Left pleural effusion,
interstitial prominence suggestive of interstitial edema
“KUB: nobg pattern”
 Take a moment
to consider your differential diagnosis
Upper GI Scout
Film
Upper GI
Continued
Upper GI
Continued
Upper GI
Continued
Upper GI
Continued
Upper GI
Continued
 Take a moment
to consider your differential diagnosis
Hospital Course
5/9/06 HD#1
GI Consult: acute onset ↓ albumin with absence of proteinuria- most
likely protein losing enteropathy- infantile Menetrier’s disease
(associated with CMV) Celiac less likely, onset of malrotation
possible.
UGI: thickened folds consistent with Menetrier’s disease
Progress Note: ↓appetite- jello, milk, UOP 1.5 cc/hr, started high
protein diet
5/10/06 HD#2
Progress Note: headache, + intermittent cough, ↑ PO- liquid, now
solids, nutrition teaching, started on zinc 1 mg/kg (due low alk phos),
UOP 1.2 cc/hr
Labs: ↓ alb- 1.6 (1.8)
Hospital Course
5/11/06 HD#3
Progress Note: ↑ abd pain. FROC- ↑ abdominal girth and scrotum
size. Abdominal girth- 67 cm (65 cm) PE-2+ tibial edema, dullness
abdomen, distended, scrotal edema. Later oxygen sat 93-94%,
ordered CXR, started oxygen 0.5L- 98% sats. PE- crackles B/L.
Increased 1L then down 0.5L. Labs- ↓ alb- 1.5 (1.6)
5/12/06 HD#4
GI Consult: PE ascites, scrotal edema, consider single trial albumin
(1gm/kg) and lasix (1 mg/kg). likely to only be transient benefit but
may be useful in improving mucosal function and subsequent
increase in protein absorption.
Progress Note: UOP 0.9 cc/hr, PE- distended, dullness, scrotal
edema, 1+ pitting edema sacral, continue oxygen, albumin/lasix
Resident On Call: ↑ abdominal girth- 70cm, comfortable, 96-98%
0.5L NC, pedal and scrotal edema
Labs: ↑ alb- 1.7 (1.5)
Hospital Course
5/13/06 HD#5
Progress Note: UOP 4.2 cc/hr, decrease to 67 cm, frustrations
ID Consult: CMV positive, rec- gancicyclovir x 7days
Labs: ↑ alb- 2.0 (1.7)
5/14/06 HD#5
Progress Note: no pain/swelling, girth 65 cm, no scrotal edema, wean
to RA
5/15/06 HD#6
Progress Note: ↑ appetite and activity, girth-66 cm, RA, cough, rales
on exam, UOP 3.4 cc/hr
Labs: ↑ alb- 2.2 (2.0)
5/16/06 HD#7
Progress Note: Good enery, regular appetite, walking, girth 62 cm
Labs: ↑ alb- 2.4 (2.5)
 Menetrier’s Disease
Hyperplastic gastropathy is a rare condition characterized by
giant gastric folds associated with epithelial hyperplasia. Two
clinical syndromes have been identified: Ménétrier’s disease and
a variant of it referred to as hyperplastic, hypersecretory
gastropathy, and Zollinger-Ellison syndrome.
Ménétrier’s disease is typically associated with protein-losing
gastropathy and with hypochlorhydria, whereas the hyperplastic,
hypersecretory variant is associated with increased or normal
acid secretion and parietal and chief cell hyperplasia, with or
without excessive gastric protein loss.
Other more common conditions can also cause enlarged gastric
folds, including gastric neoplasm (lymphoma, carcinoma),
granulomatous gastritides, gastric varices, infectious gastritis
(particularly H. pylori and CMV), and eosinophilic gastritis.
Resolution of H. pylori hypertrophic lymphocytic gastritis with
antibiotics may result in reversal of excess protein loss.
Taken From Feldman: Sleisenger & Fordtran's Gastrointestinal and Liver Disease, 7th ed.
 Examples of Hyperplastic gastropathy with giant gastric folds

Ménétrier’s disease

Zollinger-Ellison syndrome

Taken From Feldman: Sleisenger & Fordtran's Gastrointestinal and Liver Disease, 7th ed.
 In one series, 31 patients with large gastric folds of
uncertain etiology underwent full-thickness gastric mucosal
biopsies. Six patients showed the features of Ménétrier’s disease,
1 showed the features of hyperplastic, hypersecretory
gastropathy, and 6 showed features of Zollinger-Ellison
syndrome.The remaining 18 (the majority) had peptic ulcer
disease. (Komorowski R, Caya J: Hyperplastic gastropathy clinicopathologic correlation. Am J Surg Pathol 15:577, 1991.)
The enlarged gastric folds in Ménétrier’s disease are due
to foveolar cell hyperplasia, edema, and variable degrees of
inflammation. Patients may present with weight loss, epigastric
pain, vomiting, anorexia, dyspepsia, hematemesis, and positive
fecal occult blood tests. The mechanism responsible for the low
gastric acid secretion is unclear, but it could be related to
transforming growth factor-α (TGF-α) or its closely related
peptide, epidermal growth factor (EGF).

Taken From Feldman: Sleisenger & Fordtran's Gastrointestinal and Liver Disease, 7th ed.
 Ménétrier’s disease may be associated with hypertrophic
lymphocytic gastritis and a carcinoid-like syndrome due to
increased gastric mucosal production of prostaglandin E2
concentrations. Ménétrier’s disease may be self-limited and
completely resolve in patients younger than 10 years of age and
when it occurs in the postpartum period. The cause of
Ménétrier’s disease of childhood may be infection with CMV and
activation of TGF-α. The risk of developing carcinoma in association with
Ménétrier’s disease is an open question, and some authors question whether
there is a significant risk. Of the 200 cases of Ménétrier’s disease reported in
the literature, 30 (15%) have been associated with carcinoma.
The mucosa of patients with Ménétrier’s disease
demonstrates irregular hypertrophic folds that involve the entire
gastric body. The mucosa also demonstrates a swollen, spongy
appearance subdivided by creases creating a picture similar to
cerebral convolutions. A polypoid variant of Ménétrier’s disease
has been described that resembles multiple hyperplastic gastric
polyps.
Taken From Feldman: Sleisenger & Fordtran's Gastrointestinal and Liver Disease, 7th ed.
Gastric resections from patients with Ménétrier’s disease
typically show large polypoid gastric folds or large cerebriform
gastric folds with antral sparing.

Total gastrectomy specimen

in a patient with Ménétrier’s disease
(right, body, with hyperplastic mucosa
and cerebriform rugal folds;
left, antrum, with relative sparing)

Taken From Feldman: Sleisenger & Fordtran's Gastrointestinal and Liver Disease, 7th ed.
In the absence of a gastrectomy, a full-thickness gastric mucosal
biopsy is required to adequately assess the gastric histology in
patients with hyperplastic gastropathy. The predominant
microscopic feature of Ménétrier’s disease and hyperplastic,
hypersecretory gastropathy is foveolar hyperplasia with cystic
dilation.

Histology of
Ménétrier’s disease
showing enlarged
folds with foveolar
hyperplasia, cystically
dilated glands, and
minimal gastritis

The parietal and chief cells may be decreased and replaced by

mucous glands. Inflammation in hyperplastic gastropathies is
variable and may be absent.
Taken From Feldman: Sleisenger & Fordtran's Gastrointestinal and Liver Disease, 7th ed.
 Ideal treatment of hyperplastic gastropathy is unclear,
because the condition is rare and controlled trials are lacking. H.
pylori infection should be treated, if present, and the entire
syndrome may resolve. Symptoms may improve with
antisecretory agents (histamine2 [H2 ] receptor antagonists,
anticholinergic agents, proton pump inhibitors), especially if the
patient has Zollinger-Ellison syndrome or normogastrinemic
hyperplastic, hypersecretory gastropathy. It has been suggested
that H2 blockers and anticholinergics reduce gastric protein loss
by strengthening intercellular tight junctions. Some patients with
Ménétrier’s disease have responded to corticosteroids,
octreotide, antifibrinolytic agents, or monoclonal antibody
against the EGF receptor. Partial or total gastric resection is
reserved for severe complications, such as refractory or
recurrent bleeding, obstruction, severe hypoproteinemia, or
cancer development.
Taken From Feldman: Sleisenger & Fordtran's Gastrointestinal and Liver Disease, 7th ed.
 Eosinophilic gastroenteritis is a disease characterized by tissue
eosinophilia that can involve any layer or layers of the gut wall.
Kaijser is credited with the first description of eosinophilic gastroenteritis in
1937.

A definite diagnosis must fulfill the following criteria:

(1) the presence of GI symptoms
(2) eosinophilic infiltration of one or more areas of GI tract on biopsy
(3) absence of eosinophilic involvement of multiple organs outside GI tract
(4) absence of parasitic infestation.

Peripheral blood eosinophilia is absent in at least 20% of patients and should

not be considered a diagnostic criterion. Furthermore, food intolerance or
allergy is not required for the diagnosis, because many patients have no
objective evidence of these problems.
The disease is rare; although increasing numbers of cases have been reported
in the medical literature, the incidence is difficult to estimate, because some
patients are probably undiagnosed and surely unreported. Patients typically
present in the third through fifth decades of life, but the disease can affect any
age group. An equal gender distribution or a slight male preponderance has
been reported.
Taken From Feldman: Sleisenger & Fordtran's Gastrointestinal and Liver Disease, 7th ed.