Poorer prognosis with ethylenediaminetetraacetic acid-dependent pseudothrombocytopenia: a single-center case-control study.

In ethylenediaminetetraacetic acid (EDTA)-dependent pseudothrombocytopenia (PTCP), automated platelet counts are lower than actual counts because of EDTA-induced aggregation. Factors contributing to the incidence of EDTA-PTCP are unknown, and no study has assessed the prognosis of EDTA-PTCP patients. This retrospective study assessed characteristics in EDTA-PTCP patients and matched controls to determine differences in prognosis. A retrospective case-control study was designed. From the University of Tokyo Hospital database, we identified patients diagnosed with EDTA-PTCP between 2009 and 2012, and performed 1:2 case:control matching for age and sex. A control group of sex- and age-matched patients was selected at random from the same database. We investigated differences in the frequency of complications, medication history, and blood transfusion history between the groups at the time of blood collection. Prognosis was evaluated using multivariate Cox regression analysis adjusting for age, sex, autoimmune disease, liver disease, and malignant tumor. We identified 104 EDTA-PTCP patients and 208 matched controls. The median age was 69.0 years (interquartile range: 54-76), with men comprising 51%. EDTA-PTCP patients had a higher frequency of malignant tumor and a lower frequency of hypertension and diabetes than controls. After adjustment for background factors, prognosis of EDTA-PTCP patients was significantly poorer than controls (hazard ratio, 11.8; 95% confidence intervals, 2.62-53.54). In conclusion, EDTA-PTCP patients had higher mortality, and EDTA-PTCP may need to be recognized as an indicator of worse prognosis

Successful Pregnancy and Lactation Outcome in a Patient With Gaucher Disease Receiving Enzyme Replacement Therapy, and the Subsequent Distribution and Excretion of Imiglucerase in Human Breast Milk

Background: Enzyme replacement therapy (ERT) with imiglucerase is a well-established, effective treatment for Gaucher disease. However, there have been no published reports regarding the excretion of imiglucerase into human breast milk and its effects on the nursing infant. Objective: This letter reports on the successful pregnancy and lactation of a patient with Gaucher disease receiving treatment with imiglucerase, and the subsequent distribution and excretion of imiglucerase in human breast milk. Methods: A 39-year-old Japanese female (height, 164 cm; weight, 55 kg) with Gaucher disease had 2 successful pregnancies and continued ERT through both. The study was conducted 6 months after the first delivery. She was administered a 1-hour infusion of imiglucerase 60 U/kg that coincided with her regular every-2-week regimen. Serum and breast-milk samples were obtained before and up to 24 hours after administration. Breast-milk samples were also obtained from 10 nursing mothers with galactorrhea as controls. Results: The preinfusion level of breast-milk beta-glucocerebrosidase was 0.008 nmol/h/mL. The peak of serum beta-glucocerebrosidase activity (0.119 nmol/h/mL) was obtained at the end of the 1-hour infusion period. Slightly increased enzymatic activity (0.016 nmol/h/mL) was observed in the first breast milk sampled after imiglucerase infusion. Conclusions: We report a case of successful pregnancy and breastfeeding in a Japanese patient with Gaucher disease. A small amount of imiglucerase was found to be excreted into human breast milk, but only in the first milk produced after infusion. (Clin Ther. 2010;32:2048-2052)ArticleCLINICAL THERAPEUTICS. 32(12):2048-2052 (2010)journal articl

A consortium study of Antarctic micrometeorites recovered from the Dome Fuji Station

Deposits in the water tank at the Dome Fuji Station were collected by the 37th Japanese Antarctic Research Expedition team in 1996. We recovered 233 micrometeorites from the deposits. A consortium study was started in late 1998 to investigate mineralogy, petrology, bulk chemistry, and isotopic compositions of the micrometeorites. This is the first case of an organized study of micrometeorites in Japan, in order to establish the methods to investigate micrometeorites routinely. Consortium results on mineralogy, petrology, minor and trace element compositions, isotopic compositions of noble gases of the micrometeorites are reported in this volume. We also found a sequence of mineralogical and compositional changes of micrometeorites experienced from frictional heating during atmospheric entry. INAA and ion probe studies are now in progress

Antarctic micrometeorites collected at the Dome Fuji Station

Antarctic micrometeorites (AMMs) were found among the precipitated fine particles recovered from a water tank in the Dome Fuji Station. These AMMs had been contained in the recent fallen snow around the station. Initial processing of the precipitated particles revealed that they were dominated by natural and artificial terrestrial materials, thus a series of processes were developed to separate AMMs from terrestrial particles. The recovery rate of AMMs by the processes was approximately 45% in weight, which was determined from a weight ratio of recovered/accreted AMMs. The micro-morphology and major-element concentration of the recovered AMMs were characterized. They appear to have been heated upon atmospheric entry to varying temperatures and can be classified into two major types based on the degree of heating : (1) fine-grained, irregular-shaped, partial-melted micrometeorites with chondritic composition, and (2) total-melted spherical micrometeorites with chondritic composition except for volatile elements. A digital catalog for the AMMs identified in this study was established on the web site [URL : http : //dust. cc. gakushuin. ac. jp/], in which optical characteristics, high-resolution images, and chemical compositions of individual AMMs are presented. The AMMs listed in the catalog are the first Japanese collection of extraterrestrial dust. The criterion and techniques developed for the selection and initial analysis of AMMs are applicable for the dust samples that are being collected by the 39th Japanese Antarctic Research Expedition team

General characterization of Antarctic micrometeorites collected by the 39th Japanese Antarctic Research Expedition: Consortium studies of JARE AMMs (III)

From November 1998 to January 1999,the 39th Japanese Antarctic Research Expedition (JARE-39) undertook Japanese first large-scale collection of Antarctic micrometeorites (AMMs), with sizes larger than 10μm, at the Meteorite Ice Field around the Yamato Mountains in Antarctica (at three different locations, for a total of 24 collection sites). The number of collected AMMs larger than 40μm is estimated to be about 5000. Here we present the general characterization (i.e., micro-morphology and surface chemical composition using SEM/EDS) of ∿810 AMMs chosen from 5 of the 24 sites. Additionally, the mineral composition of 61 out of 810 AMMs was determined by Synchrotron X-ray radiation. Preliminary results on mineralogical and chemical compositions show similarities with that of previous studies, even though a pronounced alteration of some AMMs is noticed. A correlation is found between the Mg/Si ratio at the sample\u27s surfaces of unmelted AMMs and the age of snow/ice in which the AMMs are embedded

INCREASING AND DECREASING PHASES OF FERRITIN AND HEMOSIDERIN IRON DETERMINED BY SERUM FERRITIN KINETICS

2013-08We attempted to clarify the mechanism of the storage iron metabolism. A new program of serum ferritin kinetics was applied for studying the increasing and decreasing phases of ferritin and hemosiderin iron in iron addition and removal in patients with a normal level of iron stores or iron overload. The change of ferritin iron in response to iron addition and removal was rapid in the initial stage, but it was slow later. In contrast, the change of hemosiderin iron was slow in the initial stage, but it became rapid later. These changes of ferritin and hemosiderin iron suggest that the turnover of ferritin iron is preferential to that of hemosiderin iron, and that the initially existed ferritin iron is gradually replaced by the ferritin iron recovered by taking iron from hemosiderin in iron mobilization. The crossing of the increasing curves of ferritin and hemosiderin iron in iron addition indicates a switching of the principal storage iron from ferritin to hemosiderin. The crossing point shifted toward a higher storage iron level in the increase of iron deposition. Iron storing capacity can be increased not only by the transformation of ferritin into hemosiderin, but also by the expansion of cell space as seen by hepatomegaly in hereditary hemochromatosis. The amounts of hemosiderin iron exceeded ferritin iron in all 10 patients with chronic hepatitis C even though they had normal storage iron levels. This suggests it is difficult to store iron in the form of ferritin in chronic hepatitis C.departmental bulletin pape