Most patients with X-linked dominant chondrodysplasia punctata (X-DCDP) have whorled, thick, or spiky adherent hyperkeratosis on their whole body. Histopathologically, there are large keratotic plugs of hair follicles where calcium deposits. This is the characteristic finding in this disease. We performed histopathologic examination of skin specimens from two newborn babies with X-DCDP. The desquamated thick scales from the hyperkeratotic lesions had calcium in the center of the keratotic plugs. Histopathologic examination of thick scales is easy and helpful in diagnosing X-DCDP.
BACKGROUND Cross‐matched platelet (cross‐matched PLT) transfusion is effective for immune‐mediated platelet transfusion refractoriness (PTR), but is more costly and time‐consuming for physical cross‐match than using standard PLT units. Recent studies have reported the utility of human leucocyte antigens (HLA) virtual cross‐matched PLT (HLA‐matched PLT) that is defined as HLA‐A/B matched or no antibody against donor‐specific antigen. Here, we evaluated the effect of HLA‐matched PLTs for PTR in post hematopoietic stem cell transplant (HSCT) recipients. STUDY DESIGN AND METHODS Our study included a total of 241 PLTs in 16 patients who underwent HSCT at Okayama University Hospital between 2010 and 2017, receiving either HLA‐matched or cross‐matched PLTs. We calculated the 24‐hour corrected count increments (CCI‐24) to evaluate the effect of PLTs. A CCI‐24 ≥ 4500 was considered to be a successful transfusion. RESULTS We analyzed 139 cross‐matched PLTs and 102 HLA‐matched PLTs. In the immune‐mediated PTR, the rate of successful transfusion was 60.5% for cross‐matched PLT and 63.4% for HLA‐matched PLT (p = 0.825). On the other hand, the median CCI‐24 for cross‐matched PLT transfusions and HLA‐matched PLT transfusions were 1856 and 5824 (p < 0.001), with a success rate of 28.1 and 54.1% in cases with non‐immune‐mediated PTR, respectively (p = 0.001). CONCLUSION The effectiveness of HLA‐matched PLT is not inferior to cross‐matched PLT. This result indicates that physical cross‐match can be omitted in post HSCT PTR.
Studies examining risk factors associated with vasovagal reactions (VVRs) during autologous blood donations, especially in younger subjects, have been limited. The aim of the present study was to define risk factors for VVRs during preoperative autologous blood donation in patients, including those younger than 18 years old. We retrospectively analyzed 4,192 autologous, preoperative blood donations between 2007 and 2015 at Okayama University Hospital. Eighty-seven (2.08%) of the patients experienced VVRs. VVRs occurred approximately three times as often in patients 0-17 years old (16/320, 5.0%) than in patients 18 years and older (71/3,872, 1.8%). In particular, VVRs occurred more frequently in those 10-13 years old, and decreased with older age (P = 0.006). In a univariate analysis, younger age, lower body mass index, lower systolic blood pressure, lower body weight, lower total blood volume, female gender, first-time collection, and higher heart rate were associated with a higher incidence of VVRs. In a multivariate analysis, lower systolic blood pressure (P < 0.001), higher heart rate (P = 0.007), and first-time collection (P = 0.015), remained independent predictors of VVRs. These results emphasize the need for careful attention during blood collection. Keywords: Autologous blood donation, vasovagal reactions 3 IntroducitonAutologous blood donation is widely used for preoperative blood preparation to reduce exposure to allogeneic blood [1]. Autologous and allogeneic blood donors occasionally experience adverse reactions during or after blood withdrawal, such as weakness, pallor, nausea, sweating, and fainting [2,3]. These symptoms are generally called "vasovagal reactions" (VVRs) [2,4]. However, the pathophysiology of VVRs is not yet completely understood [5]. Although risk factors for VVRs have been analyzed mainly in healthy allogeneic donors or peripheral blood stem cell donors [2,4,[6][7][8], reports describing patients undergoing autologous collection have been limited. In particular, there are few data concerning patients younger than 16-18 years old. In the present study, we analyzed the risk factors for VVRs during autologous donation in patients, including those younger than 18 years old, at a single institution. Materials and methodsWe retrospectively collected medical information from 4,192 patients (groups I + II) who underwent autologous Patients were screened for vital signs, height, body weight, and complete blood count. Enrolled patients underwent whole blood phlebotomy in a designated room: blood volumes did not exceed 400 mL, or [body weight]/50 × 400 mL if the patient's body weight was less than 50 kg. All collections were conducted by a physician and nurse from the Department of Transfusion Medicine at Okayama University Hospital. An 18-gauge needle was inserted into an antecubital vein or a large forearm vein of each patient, and autologous blood was collected using a negative pressure blood drawing device (Hemoquick; Terumo, Tokyo, Japan). After phlebotomy, the patients were adm...
Most patients with X-linked dominant chondrodysplasia punctata (X-DCDP) have whorled, thick, or spiky adherent hyperkeratosis on their whole body. Histopathologically, there are large keratotic plugs of hair follicles where calcium deposits. This is the characteristic finding in this disease. We performed histopathologic examination of skin specimens from two newborn babies with X-DCDP. The desquamated thick scales from the hyperkeratotic lesions had calcium in the center of the keratotic plugs. Histopathologic examination of thick scales is easy and helpful in diagnosing X-DCDP.
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