Postoperative cardiac homograft involvement in Erdheim-Chester disease.

[erdheim-chester disease]

Erdheim-Chester disease (ECD ) is a rare multisystem disorder which is known to affect the skin , lungs , bone , pituitary gland , retroperitoneum and cardiovascular system . The case is described of a patient with ECD who had previously undergone a Ross procedure for presumed endocarditis involving the aortic valve and aortic root . The patient subsequently developed arthralgias , abdominal pain (requiring an exploratory laparotomy ) and polydipsia . Furthermore , he developed progressive , symptomatic stenosis of the pulmonic homograft . A reoperative replacement of the homograft was required . The clinically suspected diagnosis of ECD was confirmed by a pathologic analysis of the explanted pulmonary homograft , and also (retrospectively ) of previously resected mesenteric tissue . It is postulated that the patient may have developed ECD as a result of an immunologic reaction to the homograft tissue used for the Ross procedure .

Diseases
Validation

Diseases presenting "abdominal pain" symptom

22q11.2 deletion syndrome

adrenal incidentaloma

alpha-thalassemia

benign recurrent intrahepatic cholestasis

carcinoma of the gallbladder

child syndrome

cholangiocarcinoma

congenital diaphragmatic hernia

cushing syndrome

cutaneous mastocytosis

cystinuria

dedifferentiated liposarcoma

erdheim-chester disease

erythropoietic protoporphyria

fabry disease

familial hypocalciuric hypercalcemia

familial mediterranean fever

focal myositis

liposarcoma

lymphangioleiomyomatosis

malignant atrophic papulosis

pleomorphic liposarcoma

primary effusion lymphoma

primary hyperoxaluria type 1

proteus syndrome

scrub typhus

sneddon syndrome

systemic capillary leak syndrome

typhoid

von hippel-lindau disease

well-differentiated liposarcoma

This symptom has already been validated