Hereditary Angioedema: A Case with Ascites Yet No Symptoms in the Family.

Abstract

An interesting case of hereditary angioedema in a 26-year-old female is reported, with a finding of transient effusion of fluid into the peritoneal cavity during the attacks. The patient suffered from recurrent abdominal pain for several years, but no family members had any similar symptoms. In spite of repeated hospital admissions and many examinations, accurate diagnosis was not made until the most recent admission. The recognition of hereditary angioedema as a cause of acute and/or recurrent abdominal pain may avoid useless invasive procedures and lead to adequate treatment in other similar cases.

Angiœdème : diagnostics différentiels

“…Mais si elles sont réalisées, elles montrent un oedème muqueux diffus. Les biopsies montrent un infiltrat inflammatoire minime non spécifique et un oedème de la lamina propria [21][22][23]. Il peut exister une ascite liée à l'extravasation de fluide dans la cavité péritonéale.…”

Hereditary Angioedema

“…Some individuals with biochemical findings consistent with HAE never experience an acute exacerbation of the disease. 79 We 80 described a patient with HAE whose 53-year-old father was asymptomatic despite having decreased C1-INH function (37%) in the setting of normal serum levels. Other patients do not manifest symptoms of the disease until as old as 70 years.…”

“…23 In 20% to 25% of patients with HAE, there is no family history of the disease. 16,49,50,79 Therefore, a positive family history of HAE is not a prerequisite for consideration of HAE in the differential diagnosis when typical symptoms are present.…”

“…Mild or moderate ascites, which resolves after the acute attack, may also be seen on ultrasound. 79 Patients typically are afebrile and have normal liver enzyme, bilirubin, amylase, and lipase levels during acute episodes. White blood cell counts may be normal or slightly elevated.…”

Chronic Abdominal Pain in Children

“…VAsCulAr DIseAses migraine abdominal (27;262-265;267;268;271;426;427) anaphylactic purpura (Henoch Schönlein disease) hypertensive crises (428) sickle cell crises nephrotic syndrome: hypovolemia mesenterial thrombosis, superior mesenteric artery thrombosis (429) ischemic disease (430;431) celiac artery compression syndrome (432)(433)(434) malrotation/non-fixation with midgut volvulus eXtrA-AbDomInAl DIseAse intrathoracic pathology vertebral osteomyelitis/discitis (435)(436)(437)(438)(439) epidural abscess intra/extraspinal tumor (42;440-443) vertebral arthritis with radiculitis psoas abscess abdominal epilepsy (272-275;444;445) arthritis (446) metAbolIC/enDoCrIne DIseAse porphyria (447) lead poisoning hereditary fructose intolerance hyperlipidemia (448;449) hyperparathyrioidism urea cycle disorders, e.g. ornithine transcarbamylase deficiency (450) Fabry disease (451) otHer rheumatic fever juvenile rheumatoid arthritis dermatomyositis (452) systemic lupus erythematosus polyarteritis nodosa c1-esterase inhibitor deficiency (453)(454)(455)(456)(457) hemophilia: bleeding in intestinal wall or retroperitoneal bleeding hemolytic anemias adverse reactions to drugs…”