Treatment With Octreotide in Patients With Lymphangioleiomyomatosis

Launched by NATIONAL HEART, LUNG, AND BLOOD INSTITUTE (NHLBI) · Jun 9, 2000

Keywords

ClinConnect Summary

Lymphangioleiomyomatosis (LAM), a disease primarily of women of child-bearing age, is characterized by cystic lung disease and abdominal tumors (e.g., angiomyolipomas). Within the LAM patient population is a subset of patients who develop chylous ascites, chylous pleural effusions, chyluria, peripheral lymphedema, and/or lymphangioleiomyomas. Lymphangioleiomyomas are believed to result from a proliferation of abnormal smooth muscle cells within the lymphatic system, which appears to obstruct fluid outflow, leading to fluid accumulation and an increase in size. The lymphangioleiomyomas may o...

Gender

FEMALE

Eligibility criteria

• * INCLUSION CRITERIA:
• Patients enrolled in the lymphangioleiomyomatosis natural history protocol who have symptoms associated with one of the following:
• • 1. lymphangioleiomyomas
• • 2. chylous pleural effusions
• • 3. peripheral lymph-edema
• • 4. chyloptysis
• • 5. protein-losing enteropathy
• • 6. chyluria
• • Patients will be included in this protocol if symptoms are attributed to the above processes. Patients with malabsorption disorders, diabetes, hypo/hyperthyroidism, or other endocrine-related disorders will be included if justified clinically based on severity of symptoms.
• EXCLUSION CRITERIA:
• • 1. Hypersensitivity to somatostatin, octreotide or its analogues
• • 2. Patients with hepatitis B, hepatitis C, or other clinically significant liver diseases
• • 3. Transplant patients
• • 4. Pregnant women or women who are beast-feeding
• • 5. Patient or another responsible party is unable to give the subcutaneous injection
• • 6. Patient unwilling to be followed per the guidelines set forth
• • 7. Patients with decreased renal function (creatinine greater than 1.5)
• • 8. Patients with HIV infection
• • 9. Immunosuppressed patients