Hospitalization of children with acute immune thrombocytopenic purpura – is it necessary?

OBJECTIVE:

To identify a target group of children with acute immune thrombocytopenic purpura (ITP) that may not require hospitalization for management.

METHODS:

A retrospective chart review was conducted of all children admitted over a two-year period to a tertiary care paediatric hospital with the diagnosis of acute ITP. Patients were classified according to typical and atypical presentations. Typical patients were defined as those aged between one and 10 years, with no hepatomegaly or significant splenomegaly and who had typical laboratory features for ITP. Patients who did not meet these criteria were categorized as atypical. Outcome measures included length of stay (LOS) in hospital; frequency of bone marrow aspiration (BMA); type of treatment; incidence of intracranial hemorrhage (ICH) or severe bleeding; and admission and discharge platelet counts.

RESULTS:

There were 74 patients hospitalized for a mean of 3.6 days. No patients suffered an ICH or bleeding requiring transfusion. Patients with typical presentations (42) were compared with patients with atypical presentations (32) and were not significantly different for clinically important outcomes such as admission and discharge platelet counts, serious complications or type of therapy. Typical patients had significantly fewer BMAs than did atypical patients – 22 of 42 (52%) versus 25 of 32 (78%) (P=0.02), and a shorter LOS – 3.1 (±0.9) days versus 4.2 (±1.8) days (P=0.01).

CONCLUSIONS:

Children presenting with ITP have a low incidence of bleeding complications and many of these patients can be managed as outpatients. A multicentre study is needed to properly delineate a low risk group suited for outpatient medical management.     

骨髓间充质干细胞的分化潜能及临床应用价值

目的:观察人的骨髓间充质干细胞多分化潜能及在糖尿病治疗领域的价值。方法:实验于2005-07/2006-07在青岛大学医学院附属医院内分泌科完成。骨髓来源于非造血系统疾病的16岁儿童胸骨骨髓血(查体以排除造血系统疾病,结果显示为健康体质),经得家属同意。Percoll淋巴细胞分层液分离骨髓间充质干细胞,取第3代细胞,等密度接种于培养瓶中,经CD44抗体、CD45抗体、CD34抗体鉴定。取其第4代细胞,诱导其向脂肪细胞及神经细胞分化,利用碱性成纤维细胞生长因子预处理先获得巢蛋白阳性细胞,分别用两种方法诱导其向胰岛祖细胞的转化:化学物质诱导和共培养法诱导,免疫荧光检测胰岛祖细胞标志-胰十二指肠同源异型盒基因(蛋白的表达,电化学发光法检测其是否表达胰岛素)。胶原酶消化法获取胰腺间充质干细胞,鉴定,用添加碱性成纤维细胞生长因子的无血清低糖DMEM使其增殖。将胰腺间充质干细胞接种于底层已接种骨髓间充质干细胞的6孔板共培养,共培养法诱导骨髓间充质干细胞向胰岛祖细胞的初步转化。结果:成脂诱导及成神经诱导可获得油红O染色阳性细胞以及巢蛋白阳性细胞。化学法向胰岛祖细胞诱导后可检测到PDX-1免疫反应阳性细胞。共培养法诱导也可获得PDX-1免疫反应阳性细胞。新生儿胰腺具有巢蛋白、CK-19阳性的胰腺间充质干细胞,体外高糖诱导可形成胰岛样细胞团。结论:骨髓间充质干细胞在体外具有诱导分化为脂肪细胞、神经元样细胞及胰岛祖细胞的潜能。新生儿胰腺间充质干细胞向胰岛细胞分化过程中所分泌的一些物质对骨髓间充质干细胞向胰岛祖细胞的转化具有促进作用。     

Evaluation of atypical human immunodeficiency virus immunoblot reactivity in blood donors

Blood donors reactive by enzyme-linked immunosorbent assay for antibody to the human immunodeficiency virus (HIV) who showed atypical patterns of viral core protein reactivity on Western blot were monitored for several months. Characterization of their antibodies was performed by 1) use of recombinant HIV proteins; 2) determination of cross-reactivity to HTLV-I, HTLV-II, and HTLV-IV: 3) assessment of immune status; and 4) identification of potentially interfering autoantibodies. Nineteen of 20 donors maintained the same HIV antibody reactivity throughout the follow-up period; the other donor became fully antibody-positive. Eighteen of 20 donors' sera showed clear reactivity with HIV recombinant core proteins. Ten of 19 donor samples demonstrated cross-reactivity to HTLV-IV; 3 of these 10 also cross-reacted with HTLV-I. The immune status of all donors was normal, although the medical histories and HLA antibody screens suggested possible autoimmune reactivity in 9 of 18 donors. During follow-up interviews, three donors reported possible risk factors for HIV infection that had not been acknowledged at the time of blood donation. We conclude that exclusion of donors with these atypical serologic test results is warranted while further studies to determine significance are being conducted.     

Human erythrocyte protein 4.2 deficiency associated with hemolytic anemia and a homozygous 40glutamic acid-->lysine substitution in the cytoplasmic domain of band 3 (band 3Montefiore)

Red blood cell (RBC) protein 4.2 deficiency is often associated with a moderate nonimmune hemolytic anemia, splenomegaly, and osmotically fragile RBCs resembling, but not identical to, hereditary spherocytosis (HS). In the Japanese type of protein 4.2 deficiency (protein 4.2Nippon), the anemia is associated with a point mutation in the protein 4.2 cDNA. In this report, we describe a patient with moderate and apparently episodic nonimmune hemolytic anemia with splenomegaly, spherocytosis, osmotically fragile RBCs, reduced whole cell deformability, and abnormally dense cells. Sodium dodecyl sulfate- polyacrylamide gel electrophoresis analysis of the proposita's RBC membrane proteins showed an 88% deficiency of protein 4.2 and a 30% deficiency of glyceraldehyde-3-phosphate dehydrogenase (band 6). Structural and molecular analyses of the proposita's protein 4.2 were normal. In contrast, limited tryptic digestion of the proposita's band 3 showed a homozygous abnormality in the cytoplasmic domain. Analysis of the pedigree disclosed six members who were heterozygotes for the band 3 structural abnormality and one member who was a normal homozygote. Direct sequence analysis of the abnormal band 3 tryptic peptide suggested that the structural abnormality resided at or near residue 40. Sequence analysis of the proposita's band 3 cDNA showed a 232G-->A mutation resulting in a 40glutamic acid-->lysine substitution (band 3Montefiore). Allele-specific oligonucleotide hybridization was used to probe for the mutation in the pedigree, showing that the proposita was homozygous, and the pedigree members who were heterozygous for the band 3 structural abnormality were also heterozygous for the band 3Montefiore mutation. The band 3Montefiore mutation was absent in 26 chromosomes from race-matched controls and in one pedigree member who did not express the band 3 structural abnormality. In coincidence with splenectomy, the proposita's anemia was largely corrected along with the disappearance of most spherocytes and considerable improvements of RBC osmotic fragility, whole cell deformability, and cell density. We conclude that this hereditary hemolytic anemia is associated with the homozygous state for band 3Montefiore (40glutamic acid-->lysine) and a decreased RBC membrane content of protein 4.2. We speculate that band 3 structural abnormalities can result in defective interactions with protein 4.2 and band 6, and in particular, that the region of band 3 containing 40glutamic acid is involved directly or indirectly in interactions with these proteins.     

Thymic cysts in mediastinal Hodgkin disease

Three cases of proved thymic cysts associated with mediastinal Hodgkin disease are presented. Two illustrate regression of lymphoma with chemotherapy but persistence of thymic cysts. The third case demonstrates a thymic cyst in untreated Hodgkin disease. These cases suggest that such cysts are probably neither coincidental with nor a consequence of therapy but are probably related to initial thymic involvement by Hodgkin disease.     

Noncardiogenic pulmonary edema caused by decompression sickness: rapid resolution following hyperbaric therapy

Noncardiogenic pulmonary edema is a recognized but uncommon manifestation of type 2 decompression sickness. It typically occurs within 6 hours of a dive. Because the adult respiratory distress syndrome in this setting is believed to be due to microbubbles in the pulmonary vasculature, recompression in a hyperbaric chamber has been recommended as a form of therapy. A patient developed noncardiogenic pulmonary edema following a seawater dive to 75 feet. There was complete radiologic and clinical resolution within 5 hours of hyperbaric therapy.