Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.15.1 (ACE)
 18,300 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 30-year-old female developed symptoms consistent with Heerfordt's syndrome (complete type) and was effectively treated with steroid. However, she developed hepatosplenomegaly, bloody pleural effusion, and ascites one year after discontinuation of steroid therapy. The symptoms were considered to be due to sarcoidosis since serum ACE level, and ACE level and OKT4/8 ratio in pleural and peritoneal effusions were significantly elevated, and readministration of steroid normalized these changes. Bloody pleural and peritoneal effusions are very rare complications of sarcoidosis.
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PMID:[A case of sarcoidosis presenting with Heerfordt's syndrome, associated with hepatosplenomegaly, pleural effusion, and ascites]. 140 89

Sarcoidosis, a systemic granulomatous disease of unknown cause, may involve any body organ. The most common manifestation in the head-neck area is lymph node sarcoidosis or, alternatively, Heerfordt's syndrome (with involvement of lacrimal and parotid gland and possible facial palsy). Far less common is a isolated laryngeal sarcoidosis. The typical histopathological appearance proves the diagnosis. However, obtaining representative and sufficient biopsy material in some cases may carry risks for the patients or might technically be difficult. In these cases, the clinician can fall back on two other, noninvasive diagnostic procedures: ACE level activity determination in blood serum and 67gallium citrate scintigraphy. Elevated ACE levels in combination with a positive 67gallium scintigraphy have a specificity (combined) of 99%. As a valuable side effect, this procedure sensitively may discover additional disease in the body and also serves well as a therapy-control examination. The presented case report shows clearly that these clinical methods, if combined, are sufficient for diagnosis of Heerfordt's syndrome/sarcoidosis; no additional histopathological examination is required.
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PMID:[Clinical diagnosis of salivary gland sarcoidosis (Heerfordt syndrome)]. 1099 73

A 29-year-old woman with symmetrical bilateral swelling of the parotid, submandibular, and lacrimal glands--also called Mikulicz's symptoms--showed skin rash and pedal erythema. Serum IgG4 was within the normal range but serum ACE and lysozome were elevated. Incisional biopsy of the parotid glands and histopathological examination indicated sarcoidosis. This was complicated by uveitis and a low-grade fever. No facial nerve palsy was observed. Based on these findings, the definitive diagnosis was Mikulicz's syndrome associated with sarcoidosis. An alternative diagnosis was incomplete Heerfordt syndrome. The administration of steroid hormones, caused parotid, submandibular, and lacrimal glands swelling to disappear.
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PMID:[A case of Mikulicz's syndrome associated with sarcoidosis]. 1919 46

A 50-year-old male patient demonstrated an existing left proptosis for several weeks. The patient was suffering from physical exhaustion and had lost considerable weight. Furthermore, we observed greatly enlarged parotid and submandibular glands on both sides. MRI of the neck showed multiple, sharply circumscribed lesions in the major salivary glands and both lacrimal glands as well as in the orbit. Initially we suspected Heerfordt's syndrome, a manifestation of sarcoidosis, but laboratory diagnosis could not reveal a pathological erythrocyte sedimentation rate or an increased ACE titer. After exploratory excision from the right submandibular gland, histological examination revealed Castleman's disease. Therefore, we initiated an immunomodulatory therapy with interleukin-6 receptor antagonists.Castleman's disease is one of the very rare, benign, lymphoproliferative processes that have a tendency to turn malignant. Isolated findings of Castleman's disease should be completely resected. There are no clear treatment strategies for multiple localizations of Castleman's disease. The approaches range from systemic glucocorticoid therapy with chemotherapy to immunomodulatory treatment. In contrast to isolated findings, the prognosis for multicentric occurrence is unfavorable.
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PMID:[Castleman's disease : a rare differential diagnosis for Heerfordt's syndrome]. 2253 80