ABSTRACT
This is the first description in which the diagnosis of vanishing bile duct syndrome (VBDS) preceded the diagnosis of Hodgkin disease (HD) by several months, and for which patients received modifications to modern MOPP-ABV chemotherapy with successful clinical remission. VBDS is an uncommon form of liver disease manifested by severe cholestasis and progressive liver failure. We report 2 cases of stage IIIB pediatric HD and VBDS. Because VBDS is progressive and the only curative treatment is liver transplant, it is imperative to recognize that children with VBDS may also have concurrent HD.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cholestasis/diagnosis , Hodgkin Disease/diagnosis , Bleomycin/therapeutic use , Child , Cholestasis/drug therapy , Combined Modality Therapy , Doxorubicin/therapeutic use , Hodgkin Disease/drug therapy , Humans , Male , Mechlorethamine/therapeutic use , Prednisone/therapeutic use , Procarbazine/therapeutic use , Radiotherapy Dosage , Remission Induction , Syndrome , Treatment Outcome , Vinblastine/therapeutic use , Vincristine/therapeutic useABSTRACT
OBJECTIVE: This study was undertaken to define the spectrum, activity, and spatial distribution of antimicrobial peptides in vernix caseosa and amniotic fluid in the absence of clinical chorioamnionitis. STUDY DESIGN: Characterization of innate immune proteins in vernix and amniotic fluid obtained from pregnancies with gestational ages greater than 37 weeks by Western analysis, immunohistochemistry, and antimicrobial growth inhibition assay. RESULTS: Lysozyme, lactoferrin, human neutrophil peptides 1-3, and secretory leukocyte protease inhibitor were identified by Western analysis in vernix suspensions (n = 25) and amniotic fluid samples (n = 10). Three other important antimicrobial proteins, human beta defensin-2, lactoperoxidase, and LL-37 were not detected. Amniotic fluid and soluble extracts of vernix exhibited muramidase (lysozyme) activity, and there was selective efficacy in inhibiting growth of common perinatal pathogens. Antimicrobial peptides were concentrated in discrete, organized, acellular "granules" embedded in the vernix lipid matrix. CONCLUSION: In the absence of chorioamnionitis, vernix and amniotic fluid contain an organized pool of antimicrobial peptides with a defined spectrum of bioactivity against common bacterial and fungal pathogens.
