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BackgroundPerforming total hip arthroplasty (THA) as early as possible is recommended for rapidly destructive coxarthrosis (RDC) as it causes pain that becomes progressively more severe. However, acetabular bone loss remains an issue in THA. Special devices, such as a Kerboull-type plate, may be used for acetabular bone defects, but the procedure is highly invasive and often the patients are elderly, further complicating matters. We retrospectively investigated the clinical and radiographic results of THA using conventional hydroxyapatite-coated cementless cup in RDC.MethodsA total of 32 patients (35 hips) with RDC were enrolled in the study with a minimum 10-year follow-up. All THAs were performed using conventional hydroxyapatite-coated cementless cup. All patients were evaluated clinically according to the Harris hip score (HHS). Acetabular bone deficiency was classified according to the American Academy of Orthopaedic Surgeons (AAOS) classification.ResultsEleven hips (31%) were AAOS type III, and none were type IV. Total HHS significantly improved from 36.5 to 79.4 (p < 0.01). Two cups exhibited loosening. The overall implant-associated survival rate after 10 years was 91.4%.ConclusionsClinical results of THA using conventional cementless implants for patients with RDC were acceptable. Thus, THA using conventional cementless implant is an effective and safe surgery for patients with RDC, minimizing surgical stress.  相似文献   
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Bohring–Opitz syndrome (BOS) is a rare disease with a number of characteristic features, including hypertelorism, prominent metopic suture, exophthalmos, cleft palate, abnormal posture, and developmental retardation. Here, we report a BOS patient presenting with lethal persistent pulmonary hypertension of the newborn (PPHN) and inspiratory respiratory failure. The female infant was treated with nitric oxide and vasodilator, which did not improve her condition. The inspiratory respiratory failure required management with deep sedation. She died on postnatal day 60 due to progressed heart failure. Whole exome sequencing revealed de novo mutation in the ASXL1 gene, c.1934dupG, p.Gly646TrpfsTer12.  相似文献   
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Although thrombotic thrombocytopenic purpura (TTP) is rare, early diagnosis and treatment are important for decreasing the mortality rate. Acquired vitamin B12 deficiency is frequently overlooked because of its rarity in developed countries, particularly in children and adolescents. The hematological changes in vitamin B12 deficiency present as megaloblastic anemia, increased lactate dehydrogenase, vasoconstriction, increased platelet aggregation, and abnormal activation of the coagulation followed by microangiopathy as well as neutropenia and thrombocytopenia. We report herein the case of a 15‐year‐old girl who had been neglected, which might have caused pseudo‐TTP through malnutrition, particularly vitamin B12 deficiency. When we encounter cases of TTP in children, clinicians must be aware of the possibility of malnutrition, particularly with vitamin B12 deficiency, even in developed countries, and investigate the cause of malnutrition including neglect.  相似文献   
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Self-care is gradually being recognized as important in the treatment of pollinosis, based up to now on data on airborne pollen. To determine the real-time numbers of airborne pollen would be more useful in self-care, however, so we studied the usefulness of the real-time pollen counter. Between Feb. 2, 2001, and Apr. 26, 2001, 4 types of airborne pollen i.e., Japanese cedar, Japanese cypress, black alder, and beech observed in Wakayama City were counted with a Durham pollen counter and a real-time pollen counter (Yamato Manufacturing Co. Ltd.). Correlation between the 2 pollen counters was r = 0.69 for Japanese cedar in March and r = 0.89 for Japanese cypress in April. A high correlation was observed between outcomes of the 2 pollen counters. The amount of pollen from black alder and beech was less than that from Japanese cedar and cypress. Unexpected peaks were observed not related to the pollen number is apparently due to snow. We have taken measure against snow, so we concluded that the real-time pollen counter was useful in counting the amount of airborne pollen over time.  相似文献   
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A newborn hearing screening was conducted on 319 neonatal intensive care unit (NICU) and 1200 well-born nursery (WBN) infants. Mean NICU birth weight was 1997 g and mean gestational age 34 weeks. Auditory brainstem response was studied in screening in NICU infants. Otoacoustic emission (OAE) or automated auditory brainstem response (AABR) was used in primary WBN screening. All infants not bilaterally passing hearing screening before discharge were recalled for outpatient retesting. If the outcome was still failure, ABR screening was conducted. The prevalence of infants diagnosed with hearing loss was 1.1% (16 of 1519). Of these 16, 75% were from the NICU and 75% were at risk for hearing loss. Mean age at hearing loss identification was 12.9 weeks in the WBN and mean age at hearing aid fitting was 16.1 weeks. Mean age at hearing aid fitting was lower for WBN than for NICU infants. Hearing loss identification and hearing aid fitting are thus feasible in NICU and WBN infants in universal newborn hearing screening.  相似文献   
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In Japan, the number of households who have pets has gradually increased, together with the number of people who have pet allergies. Many reports exist on pollen and mite allergy, but few on pet allergy. We conducted an epidemic study in 531 first-year junior high school students in Wakayama prefecture in 1999. Questionnaires covered allergy and measurement of total IgE antibody using CAP system (Pharmacia Co. Ltd.) and specific IgE antibody using MAST26 system (Hitachi chemical Co. Ltd.). Of 306 students having pets, 11 were allergic to dogs, 11 to cats, 8 to both, and 1 to rabbits. Clinical symptoms were various. No differences in symptoms were observed among allergens. Serum total IgE tended to increase in students who suffered from pet allergies. Positive rates of specific IgE antibodies were high in mites and Japanese cedar pollen (36.7% for mites and 37.0% for Japanese cedar pollen), and also in dogs and cats (15.4% for dogs and 18.2% for cats). Specific dog and cat IgE antibodies increased significantly (p = 0.033 for dog and for p < 0.0001 cat), but no significant correlation was found between the positive specific IgE antibody and history of pet keeping.  相似文献   
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Insects and other arthropods have an effective innate immune system that can clear infections with bacteria and other microorganisms. Despite this ability, one group of bacteria, the spiroplasmas, survive unharmed within the haemolymph of a wide range of arthropod hosts. We investigated the interaction between one member of this clade, a relative of Spiroplasma poulsonii, and the immune system of its Drosophila host. Expression of antimicrobial genes in spiroplasma-infected flies did not differ from wild-type controls either in the naturally infected state, nor after septic shock. We therefore concluded that spiroplasma infection did not induce an immune response in its host, but that this absence of response was unlikely to be because the bacterium inhibited response. Further experiments revealed immune reactions induced ectopically did reduce parasite titre. We therefore conclude that this bacterium has a novel form of interaction with its host, being hidden from the host immune system, but potentially suppressible by it.  相似文献   
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We previously reported that chronic Ultraviolet-B (UVB) irradiation causes wrinkle formation, decreases skin elasticity, and damages/curls dermal elastic fibers. Those UVB-induced wrinkles can be improved by treatment with retinoic acid or with a CO2 laser which results in a recovery of skin elasticity and a repair of elastic fiber linearity. We showed further that topical application of N-phenetyl-leucyl-tryptophane, an agent that specifically inhibits fibroblast-derived elastase, immediately after UVB irradiation inhibited UVB-induced wrinkle formation, maintained skin elasticity, and inhibited changes in the three-dimensional structure of dermal elastic fibers in a dose-dependent manner. In this study, the effects of an extract of Sanguisorba officinalis L., which also inhibits fibroblast-derived elastase, was evaluated for possible inhibition of UVB induced wrinkle formation, maintenance of skin elasticity, and prevention of damage to the 3-dimensional structure of dermal elastic fibers. Hind limb skins of 3-week-old Sprague-Dawley rats were irradiated with UVB at a suberythemal dose 3 times a week for 6 weeks. Simultaneously, an extract of Sanguisorba officinalis L. (at 0.2% (v/v) or 1% (v/v)) was topically applied 5 times per week immediately following each UVB irradiation and 1 d later. The extract of Sanguisorba officinalis L. inhibited wrinkle formation, maintained skin elasticity, and inhibited the decrease of dermal elastic fiber linearity in the rat hind limb skin in a dose-dependent manner. We have confirmed that the inhibition of elastase activity in fibroblasts immediately after UVB irradiation using an extract of Sanguisorba officinalis L. prevents chronic photodamage following UVB irradiation.  相似文献   
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