Acta Pcediatr Scand 80: 482-485, 1991

CASE REPORT Protein Losing Enteropathy as a Manifestation of Henoch-Schonlein Purpura SHIMON REIF, ASHOK JAIN, JOSE SANTIAGO and THOMAS ROSS1 From the Department of Gastroenterology and Nutrition, Children's Hospital of Buffalo, Buffalo, New York, USA

ABSTRACT. Reif, S., Jain, A., Santiago, J. and Rossi, T. (Department of Gastroenterology and Nutrition, Children's Hospital of Buffalo, Buffalo, New York, USA). Protein losing enteropathy as a manifestation of Henoch-Schiinlein purpura. Ada Paediatr Scand 80: 482,1991. Gastrointestinal manifestations of Henoch-Schiinlein purpura (HSP) commonly include abdominal pain and gastrointestinal bleeding. Hypoproteinemia and edema could be related to renal involvement. We report a 14-year-old boy with classical features of HSP manifestated with edema due to severe intestinal protein loss, measured by elevated fecal alpha 1 antitrypsin secretion. The protein losing enteropathy subsided with corticosteroid therapy. Key words: protein bsing enteropathy, Henoch-Sch6n;nlcinpurpura, alpha I antitrypsin.

Henoch-Schonlein purpura is a systemic vasculitis of unknown etiology involving the small blood vessels ( 1 , 2). Classically it is associated with palpable purpura, arthritis, abdominal pain and renal manifestation (2, 3). The disease commonly involves the gastrointestinal tract, manifested most commonly by abdominal pain and gastrointestinal bleeding (4, 5). Intussusception, gross infarction and perforation are less common but known complications of HSP (6, 7). To our knowledge protein losing enteropathy has not been described in the pediatric literature. We report a patient who developed severe hypoalbuminemia and edema secondary to protein losing enteropathy in addition to the classic features of HSP. CASE REPORT The patient is a 14-year-old Caucasian who was previously healthy with the exception of left orchidectomy at the age of two days due to testicular torsion. Two months prior to this admission he had tonsillitis which was treated with oral penicillin. One week before his current admission he developed pain and swelling over his left knee and ankle which was followed two days later by a purpuric rash predominantly involving the legs and buttocks. He was admitted due to increase in the severity of his abdominal pain accompanied by nausea and vomiting, but with no diarrhea or melena. Physical examination revealed a well nourished boy with no distress. His temperature was 37"C, pulse rate 105 beatdmin and blood pressure 160/90. A coarse purpuric rash was noted on his lower limbs more on the dorsal side and also on his buttocks. There was mild peripheral pitting edema. No lymphadenopathy was noted. Lungs were clear and heart sounds were normal. The abdomen was soft with minimal diffuse tenderness, no mass or organomegaly was detected and bowel sounds were active. Left testis was not palpable. The peripheral blood count showed 15 900 leukocyte/mm3 with normal differential, 496 OOO platelets/mm3, hemoglobin level 137 g/l and hematocrit 0.41 Yo. Serum, glucose, BUN, creatinine, electrolytes and liver enzymes were within normal limits except for Ca 2.05 mmol/l. Serum total protein showed a value of 49 g/l with albumin 29 g/l. His PT and PTT were slightly prolonged. PT 15.7 sec (control 12.3) and PTT 29.5 (control 25.6).Erythrocyte sediment rate was 17. Serum IgA level was 3.98 g/l (normal value 0.81-2.32 g/l). C3 was 1.03 g/l and C4 0.2 g/l (within the normal range). His stool was heme positive.

Acta Paediatr Scand 80

Protein losing enteropathy in HSP 483

Fig. 1. The Upper GI series demonstrates areas of narrowing alternating with marked dilation in the second portion of the duodenum. Mucosal irregularities consistent with ulceration are seen as well. The proximal jejunal folds are thickened and irregular, consistent with bowel wall edema.

Urinalysis was positive for microscopic hematuria without any casts. There was no proteinuria. Upper GI series revealed thickening folds, edema and ulcerations limited to the duodenal and jejunal mucosa (Fig. I). During hospitalization the severity of the edema was increased and also a significant amount of ascitic fluid was demonstrated by abdominal sonogram. His serum albumin decreased to a level of 18 g/l necessitating albumin infusion. Fecal excretion of alpha 1 antitrypsin was found to be highly elevated up to 139 mg/g dry stool (normal value c 2 mg/g dry stool). Intravenous prednisolone 20 mg bid was started. which resulted in improvement of his clinical manifestation. Serum albumin increased gradually to 35 g/1 and fecal alpha 1 antitrypsin decreased to 31.36 mg/g after 2 weeks. The patient was discharged on oral prednisone 40 mg/day which was tapered without any relapse. His fecal alpha 1 antitrypsin diminished to normal value within 2 months.

DISCUSSION Protein losing gastroenterology (PLE) is characterized by hypoalbuminemia due to excessive loss of plasma proteins into the gut. The excessive transmucosal efflux of plasma proteins is postulated to be caused by a variety of pathological mechanisms. Mucosal and cell damage and increased permeability to proteins occurs in such entities as in celiac disease (8) or severe gastroenteritis with mucosal injury (9, 10). PLE is also associated with conditions in which mucosal inflammation, erosion and ulceration occur, e.g. Crohn’s disease, ulcerative colitis (1 1). Lymph flow obstruction is another main mechanism for PLE as seen in congenital lymphangiectasia (12).

484 S. Reget al.

Acta Paediatr Scand 80

Edema with severe hypoalbuminemia was one of the main manifestations of the classic presentation of Henoch-Schonlein purpura in our patient. However, there was no evidence of nephrosis or liver dysfunction. The excessive fecal protein loss which was evident by repetitive high fecal alpha 1 antitrypsin, was the sole cause of the hypoalbuminemia in this patient. Random fecal alpha 1 antitrypsin is now accepted as a reliable test for protein loosing enteropathy (1 3, 14). The gastrointestinal tract involvement in HSP is due to vasculitis with submucosal hemorrhage infarction and ulceration (15). Although rare manifestations such as pancreatitis (16) and hydrops of gallbladder (1 7) were described, PLE was reported only once and only in adults (18). However, the serum albumin level never decreased below 25 g/l in that report, also no edema or ascites were noted. Moreover, even the authors of that report stated: “The techniques used in this study may not provide an accurate quantitative measurement of gastrointestinal protein loss.” We speculate that two different mechanisms may be responsible for the protein loss in HSP. Mucosal erosion, hemorrhage and ulceration which was evident as “thumb printing” and spiculations on upper GI series was one mechanism of the PLE. Secondly, increased vascular permeability to the plasma proteins may cause the protein loss into the gut. Increased capillary permeability was found in patients with collagen vascular disease such as systemic lupus erythematosus and polyarteritis nodosa who were having unexplained edema without nephrosis (19). It was suggested that increased capillary permeability can be the cause of PLE in these patients (20). It is well known that the gastrointestinal symptoms of HSP such as abdominal pain or melena respond to corticosteroids (2 1). Whether severe manifestation such as intussusception or perforation can be prevented by steroids is controversial. However, our patient demonstrated good response to steroid treatment with immediate decrease of the intestinal protein loss. REFERENCES 1. Silber DL. Henoch-Schonlein syndrome. Pediatr Clin North Am 1972; 19: 1061-70. 2. Allen DM, Diamond LK, Howel DA. Anaphylactoid purpura in children (SchonleinHenoch syndrome). Am J Dis Child 1960; 99: 833-54. 3. Sterky G, ThilCn A. A study on the onset and prognosis of acute vascular purpura (The Henoch-Schonlein Syndrome) in children. Acta Pediatr Scand 1960; 49: 217-29. 4. Glasier CM, Siege1 MJ, McAllister WH. Henoch-Schonlein syndrome in children. Gastrointestinal manifestation. AJR 1981; 136: 1081-85. 5. Bryn BR, Fitzgerald JF, Northway JD. Unusual manifestation of Henoch-Schonlein syndrome. Am J Dis Child 1976; 130 1335-37. 6. Goldbloom RB, Drummond KN. Anaphylactoid purpura with massive gastrointestinal hemorrhage and glomerulonephritis. Am J Dis Child 1968; 116: 97-102. 7. Martinex-Frontanilla LA, Northway JD. Surgical complications in Henoch-Schonlein syndrome. J Pediatr Surg 1984; 19: 434-36. 8. Rubini ME, Sheehy TW, Meroney WH, Lour0 J. Exudative enteropathy. I. Observations in tropical sprue. J Lab Clin Med 1961; 58: 892-901. 9. Schreiber DS, Blacklow NR, Trier JS. The mucosal lesion of the proximal small intestine in acute infectious non-bacterial gastroenteritis. N Engl J Med 1973; 288: 1318-23. 10. Sherman P, Liebman WM. Apparent protein-loosing enteropathy associated with giardiasis. Am J Dis Child 1980; 134: 893-94. 11. Beeken WL, Busch HJ,Sylvester DL. Intestinal protein loss in Crohn’s disease. Gastroenterology 1972; 62: 207-1 5. 12. Sorenson RU, Halpin TC, Abramowsky CR, Hornick DL, Miller KM, Naylor P, Incefy GS. Intestinal lymphangiectasia and thymic hypoplasia. Clin Exp Immunol 1985; 59: 2 17-26.

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13. Thomas DW, Sinatra FR, Merritt RJ. Random fecal alpha-1 antitrypsin concentration in children with gastrointestinal disease. Gastroenterology 1981; 80: 776-82. 14. Maguzzu G, Jacono G, Pasquale GD. Reliability and usefulness of random fecal alpha 1 antitrypsin concentration: further simplification of the method. J Pediatr Gastroenterol Nutr 1985; 4: 402-17. 15. Tomomasa T, Hsu JY, Itch K,Kurume T. Endoscopic findings in pediatric patients with Henoch-Schonlein Purpura and gastrointestinal symptoms. J Ped Gastroenterol Nutr 1987; 6: 725-29. 16. Garner JAM. Acute pancreatitis as a complication of anaphylactoid (Henoch-Schbnlein) purpura. Arch Dis Child 1977; 52: 971-72. 17. McCrindle BW, Wood RA, Nussbaum AR. Henoch-Schonlein syndrome. Unusual manifestation with hydrops of the gallbladder. Clin Pediatr 1988; 27: 254-56. 18. Jones NF, Creamer B, Gimlette TMD. Hypoproteinemia in anaphylactoid purpura. Br Med J 1966; 2: 1166-68. 19. Wood ML, Foulds IS, French MA. Protein loosing enteropathy due to systemic lupus erythematosus. Gut 1984; 25: 1013-15. 20. Marks J, Birkett DA, Shuster S. Capillary permeability in patients with collagen vascular disease. Br Med J 1972; 1: 782-84. 2 1. Rosenblum ND, Winter HS.Steroid effects on the course of abdominal pain in children with Henoch-Schonlein purpura. Pediatrics 1987; 79: 1018-21.

Submitted Feb. 6, 1989. Accepted March 20, 1990 (A. J.) State University of New York at Buffalo Children’s Hospital of Buffalo Department of Gastroenterology and Nutrition 219 Bryant Street Buffalo, NY 14222 USA

Protein losing enteropathy as a manifestation of Henoch-Schönlein purpura.

Gastrointestinal manifestations of Henoch-Schönlein purpura (HSP) commonly include abdominal pain and gastrointestinal bleeding. Hypoproteinemia and e...
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