Protein-Losing Hypertrophic Gastropathy:Another Cause of Gastric Wall Thickeningin Children

Vıctor Pineda, MD,1 Goya Enriquez, MD,1 Francisco Castello, MD,2 Celestino Aso, MD1

1Department of Radiology, Vall d’Hebron Hospital, Passeig De la Vall d’Hebron 119-129, 08035 Barcelona, Spain2Department of Pediatrics, Vall d’Hebron Hospital, Passeig De la Vall d’Hebron 119-129, 08035 Barcelona, Spain

Received 10 March 2003; accepted 14 August 2003

ABSTRACT: Protein-losing hypertrophic gastropathy(PLHG) is an uncommon, self-limited disease inyoung children that can produce generalized edema,hypoproteinemia, and nonspecific prodromic symp-toms. Recently, Helicobacter pylori and cytomegalo-virus infections have been reported to be associatedwith this condition. We present the case of an 18-month-old boy with PLHG and positive detection ofH. pylori on fecal culture. Abdominal sonographydemonstrated gastric wall thickening with preserva-tion of the mucosal layers. Sonographic findings areuseful for suggesting a diagnosis of PLHG and is alsoan effective, noninvasive technique for follow-up ofchildren with this condition. ª 2003 Wiley Periodicals,Inc. J Clin Ultrasound 32:47–49, 2004; Published on-line in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/jcu.10219

Keywords: hypertrophic gastropathy; Helicobacterpylori; ultrasonography

Transient protein-losing hypertrophic gastrop-athy (PLHG) occurs uncommonly in children.

The cause of this condition is unknown, but it hasbeen associated with the presence of infectiousagents (eg, Helicobacter pylori, cytomegalovirus),allergies, and autoimmune processes. ChildhoodPLHG has been labeled infantile Menetrier’s dis-ease, although several characteristics of this condi-tion are different from those of Menetrier’s diseasein adults. In children, PLHG is usually benign andself-limited.1,2 We present the case of a young child

in whom PLHG was diagnosed by his clinical andsonographic findings.

CASE REPORT

An 18-month-old boy was admitted to our hospitalfor the sudden onset of generalized edema. Twoweeks earlier, he had experienced an episode ofbronchitis that was treated with bronchodila-tors and corticosteroids. On hospitalization, thepatient was observed to be in poor overall condi-tion and had a loss of appetite. Physical examina-tion confirmed the presence of generalized edema,particularly in the lower extremities.

Laboratory testing revealed low concentrationsof total serum protein (39 g/l) and albumin (20 g/l).The concentrations of immunoglobulinMwere high(336 mg/dl), whereas those of immunoglobulins A(62.5 mg/dl) and G (421 mg/dl) were normal. Inaddition, he had high concentrations of ammonia(122 mmol/l). In the absence of proteinuria, a renalcause was ruled out. The results of fecal evaluationsshowed protein loss and the presence of H. pyloriantigen.

Abdominal sonography, performed with aSequoia (Acuson, Mountain View, CA) ultrasoundscanner equipped with a 5–8-MHz linear-arraytransducer, demonstratedmoderate, diffuse muco-sal thickening (6 mm) of the gastric body withpreservation of the wall layers (Figure 1).

The patient was given supportive therapy withan intravenous albumin infusion. His edemaimproved within 3 days and was completelyresolved at 1 week. Laboratory testing performed20 days later showed normalized concentrationsof serum protein (65 g/l), albumin (43 g/l), and

Case Report

Correspondence to:V.Pineda,HospitalVall d’Hebron,Department

of Radiology, Passeig Vall d’Hebron 119–129, 08035, Barcelona,

Spain

ª 2003Wiley Periodicals, Inc.

VOL. 32, NO. 1, JANUARY 2004 47

ammonia (18 mmol/l). Sonographic follow-up 1month after the patient’s initial evaluationshowed an almost normal gastric wall thickness(3.5 mm) (Figure 2).

DISCUSSION

PLHG is an uncommon cause of enteral proteinloss in children. The condition is characterized bygastric wall thickening and loss of protein throughthe digestive tract. PLHG generally occurs duringthe first years of life and is more common in malethan in female children.1 Clinical findings typi-cally include edema and occasionally ascites andpleural effusion. Nonspecific prodromal symp-toms, such as vomiting, diarrhea, and abdominalpain, are present in many instances. Affected chil-dren often have decreased serum protein andalbumin concentrations, and peripheral hypereo-sinophilia is a frequent finding.2 The ammoniemiaalso noted in our patient may have been related tothe fact that H. pylori produces urease.

Although PLHG shares some of the character-istics of Menetrier’s disease in adults, the clinicalprocess of PLHG is quite different. In children,the onset of PLHG is sudden, and edema occursmore commonly than it does in adults affected byMenetrier’s disease. PLHG also has a benign, self-limited course in children, whereas Menetrier’sdisease is a chronic condition in adults.

The cause of PLHG is unknown. Various stud-ies have indicated a multifactorial origin, and ahereditary predisposition has been established insome cases.3 Numerous investigators have foundassociations between PLHG and cytomegalo-virus4,5 and H. pylori6,7 infection. In our patient’scase, the results of fecal analysis were positive forthe presence of H. pylori antigen. H. pylori infec-tion has also been reported to be associated withseveral conditions involving gastric wall changes.7

Among these conditions, gastric mucosa-asso-ciated lymphoid tissue lymphoma has receivedparticular attention in the medical literature.

In contrast with other proliferative processessuch as lymphoma, PLHG is associated with pres-ervation of the various gastric wall layers.8 Insevere cases of PLHG, a barium study can demon-strate contour changes (thumbprinting) mainly inthe gastric fundus and body; the gastric antrummay be spared. Sonography is useful for demon-strating gastric wall thickening, and because it isa noninvasive, radiation-free technique, it is themethod of choice for follow-up of patients withPLHG. Resolution of the gastric wall thickeningwithin a few weeks is a key feature in establishingthe diagnosis of PLHG and in ruling out the pos-sibility of other entities, such as gastric lymphan-giectasia, mucosa-associated lymphoid tissue lym-phoma, and eosinophilic gastroenteritis.

When biopsy is performed in patients withPLHG, macroscopic evaluation reveals the pres-

FIGURE 1. Transverse sonogram obtained on the patient’s admission

shows thickening of the gastric wall (arrows) and well-defined layers.

FIGURE 2. Transverse sonogram obtained 3 weeks later shows that

the gastric wall (arrows) has returned to a normal thickness.

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48 JOURNAL OF CLINICAL ULTRASOUND

ence of hypertrophic gastric folds, and histo-pathologic examination shows massive foveolarhyperplasia with cystic dilatation of the glandsand markedly increased mucosal thickness. Inour patient, the typical course and fast resolutionof the disease, together with clinical and sono-graphic findings, were considered sufficient forestablishing the diagnosis of PLHG without theneed for biopsy.

The findings in this case indicate that PLHGshould be kept in mind as an unusual cause ofgastric wall thickening in young children. Theuse of sonography is helpful in both the diagnosisand follow-up of this condition.

REFERENCES

1. Chouraqui JP, Roy CC, Brochu P, et al. Menetrier’sdisease in children: report of a patient and review ofsixteen other cases. Gastroenterology 1981;80(5 pt 1):1042.

2. Kraut JR, Powell R, Hruby MA, et al. Menetrier’sdisease in childhood: report of two cases and a

review of the literature. J Pediatr Surg 1981;16:707.

3. Larsen B, Tarp U, Kristensen E. Familial gianthypertrophic gastritis (Menetrier’s disease). Gut1987;28:1517.

4. Coad NA, Shah KJ. Menetrier’s disease in child-hood associated with cytomegalovirus infection: acase report and review of the literature. Br J Radiol1986;59:615.

5. Occena RO, Taylor SF, Robinson CC, et al. Associa-tion of cytomegalovirus with Menetrier’s disease inchildhood: report of two new cases with a review ofliterature. J Pediatr Gastroenterol Nutr 1993;17:217.

6. Badov D, Lambert JR, Finlay M, et al. Helicobacterpylori as a pathogenic factor in Menetrier’s disease.Am J Gastroenterol 1998;93:1976.

7. Riddell RH. Pathobiology of Helicobacter pyloriinfection in children. Can J Gastroenterol 1999;13:599.

8. Park MS, Kim KW, Yu JS, et al. Radiographic find-ings of primary B-cell lymphoma of the stomach:low-grade versus high-grade malignancy in relationto the mucosa-associated lymphoid tissue concept.AJR Am J Roentgenol 2002;179:1297.

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