ABSTRACT
A case of ARC syndrome was found in a newborn male with persistent cholestasis. He showed renal dysfunction, failure to thrive, and ichthyosis as well as arthrogryposis. The patient who had a dead sibling with similar symptoms has not been described previously in our country.
Subject(s)
Humans , Infant, Newborn , Male , Arthrogryposis , Cholestasis , Failure to Thrive , Ichthyosis , Jaundice, Obstructive , SiblingsABSTRACT
PURPOSE: Kawasaki disease is the most common cause of systemic vasculitis in children less than 5 years of age. Recent immunohistochemistry findings suggest that many vascular growth factors play a role in the formation of the coronary artery lesions. Active remodeling of the coronary artery lesions in Kawasaki disease continues in the form of intimal proliferation and neoangiogenesis for several years after the onset of the disease. Intravenous immunoglobulin (IVIG) and corticosteroid have been used in the treatment of Kawasaki disease but the exact mechanism is not clear. We have investigated that IVIG and corticosteroid inhibited vascular endothelial growth factor (VEGF)- induced tube formation of endothelial cells in vitro on Matrigel assay. METHODS: Human umbilical vein endothelial cells (HUVECs) were cultured and seeded on Matrigel coated 24 well plates in medium with or without the following agents: VEGF, VEGF plus IVIG, VEGF plus VEGF antibody, VEGF plus methylprednisolone, VEGF, IVIG plus methylprednisolone for 18 hours. The total length of tube structures in each photograph was quantified. RESULTS: IVIG significantly inhibited the proliferation of HUVECs. The inhibitory effect of IVIG was also reversible. In the meantime, VEGF induced the differentiation of HUVECs into capillary like structures on Matrigel, which was inhibited by VEGF antibody in a dose-dependent manner. Interestingly, IVIG and methylprednisolone inhibited VEGF-induced tube formation of HUVECs. IVIG was more effective in inhibition than methylprednisolone alone. CONCLUSION: We revealed that VEGF induced the differentiation of HUVECs and this effect was inhibited by IVIG and methylprednisolone.
Subject(s)
Child , Humans , Capillaries , Coronary Vessels , Endothelial Cells , Human Umbilical Vein Endothelial Cells , Immunoglobulins , Immunoglobulins, Intravenous , Immunohistochemistry , Intercellular Signaling Peptides and Proteins , Methylprednisolone , Mucocutaneous Lymph Node Syndrome , Systemic Vasculitis , Vascular Endothelial Growth Factor AABSTRACT
The heart disease during gestation complicates approximately 0.5-1.5% of pregnancies. The common cause of heart disease during gestation is acquired rheumatic valvular lesions and congenital heart defects. In contrast, infective endocarditis during pregnancy or the puerperium is quite rare, with fewer than 100 cases reported over the past 50 years. We present a case of bacterial endocarditis complicated by severe tricuspid valvular insufficiency and associated septic pulmonary emboli. Therapy consisted of cesarean section at 32 weeks gestation followed by tricuspid valvular replacement, removal of vegetation and primary closure of congenital ventricular septal defect.
Subject(s)
Female , Pregnancy , Cesarean Section , Endocarditis , Endocarditis, Bacterial , Heart Defects, Congenital , Heart Diseases , Heart Septal Defects, Ventricular , Postpartum PeriodABSTRACT
Suppurative sialadenitis is rare in the neonate and usually involves the parotid glands. Isolated suppurative submandibular sialadenitis in the neonatal population is extremely rare. There are only 11 cases found in the literature of suppurative submandibular sialadenitis occurring as an isolated lesion. We describe a case of isolated submandibular sialadenitis progressing to submandibular abscess requiring incision and drainage in a term neonate. Pus culture yielded methicillin-resistant Staphylococcus aureus (MRSA). A brief review of literature is included.