ABSTRACT
The association of biliary atresia (BA) and idiopathic thrombocytopenic purpura (ITP) is extremely rare, with only 2 cases being reported in the literature. This report describes the very rare case of a 1-year-old boy with BA complicated with ITP after cholangitis and the successful steroid treatment of ITP. We review the literature on this type of relationship between BA and ITP and discuss the clinical features of this complication. Furthermore, the possible cause of the onset of ITP complicated with BA was explored in this report.
Subject(s)
Biliary Atresia/complications , Biliary Atresia/surgery , Purpura, Thrombocytopenic, Idiopathic/complications , Purpura, Thrombocytopenic, Idiopathic/drug therapy , Biliary Atresia/diagnosis , Cholangitis/complications , Cholangitis/diagnosis , Cholangitis/surgery , Diagnosis, Differential , Glucocorticoids/therapeutic use , Humans , Infant , Liver Transplantation , Male , Prednisolone/therapeutic use , Purpura, Thrombocytopenic, Idiopathic/diagnosisABSTRACT
Postoperative portal and splenic vein thrombosis (PSVT) is a rare but potentially fatal complication after abdominal surgery. We present 2 infants with PSVT after splenectomy and total colectomy, respectively, and also provide a review of the literature. We conclude that clinicians should consider that PSVT might occur after splenectomy for a huge splenomegaly or after a total colectomy for ulcerative colitis.
Subject(s)
Colectomy/adverse effects , Portal Vein , Splenectomy/adverse effects , Splenic Vein , Venous Thrombosis/etiology , Adolescent , Colitis, Ulcerative/physiopathology , Colitis, Ulcerative/surgery , Humans , Male , Portal Vein/diagnostic imaging , Primary Myelofibrosis/surgery , Risk Factors , Splenic Vein/diagnostic imaging , Tomography, X-Ray Computed , Venous Thrombosis/diagnostic imaging , Venous Thrombosis/epidemiology , Venous Thrombosis/physiopathologyABSTRACT
This report describes the use of superparamagnetic iron oxide-enhanced magnetic resonance imaging to diagnose fibronodular hyperplasia of the liver in a 12-year-old girl.
Subject(s)
Contrast Media , Electron Spin Resonance Spectroscopy/methods , Focal Nodular Hyperplasia/diagnosis , Iron , Magnetic Resonance Imaging/methods , Oxides , Abdominal Pain/etiology , Algorithms , Anemia, Iron-Deficiency/complications , Case Management , Child , Contrast Media/pharmacokinetics , Dextrans , Diagnosis, Differential , Female , Ferrosoferric Oxide , Focal Nodular Hyperplasia/complications , Focal Nodular Hyperplasia/diagnostic imaging , Focal Nodular Hyperplasia/pathology , Focal Nodular Hyperplasia/surgery , Hepatectomy/methods , Humans , Iron/pharmacokinetics , Liver Neoplasms/diagnosis , Magnetite Nanoparticles , Mononuclear Phagocyte System/metabolism , Oxides/pharmacokinetics , Phagocytosis , UltrasonographyABSTRACT
Wiskott-Aldrich syndrome (WAS) is caused by defects in the WAS protein (WASP) gene on the X chromosome. We previously reported that flow cytometric analysis of intracellular WASP expression (FCM-WASP) was useful in the diagnosis of WAS in patients and carriers. In this study, we applied FCM-WASP to evaluate the mixed chimera (MC) status of 12 WAS patients who underwent hematopoietic stem cell transplantation (HST). After HST, donor- and recipient-derived peripheral blood mononuclear cells (PBMCs) could be distinguished easily with this method, since the donor cells were WASP(bright), whereas the defective recipient cells were WASP(dim). Furthermore, with use of 2-color FCM-WASP, the MC status could be characterized by cell lineage. Six of the 12 patients with WAS were found to have MC status after HST, whereas others had complete chimera status. MC status was observed in every cell lineage examined. However, among PBMCs, recipient cells were most commonly observed in the monocyte population. Finally, to investigate the naive/memory status of donor and recipient T cells in these patients, 3-color FCM-WASP using anti-CD45RA or CD45RO was performed. We found that, in contrast to WASP(bright) T cells, most WASP(dim) T cells remained naive (CD45RA(+)/RO(-)) more than a year after HST. No imbalance in the ratio of naive to memory T cells was observed in WAS patients before HST. We conclude that FCM-WASP is a potentially useful method for clinical follow-up of WAS patients who have undergone HST. Our findings may also have important implications for the role of WASP during hematopoietic development.
