8 research outputs found

    ブラダー・ウイリー ショウコウグン ニオケル セイチョウ ホルモン リョウホウ ト ソクワンショウ トノ カンケイ

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    成長ホルモン(GH)療法がプラダー・ウイリー症候群(PWS)の低身長に対して適応され5年が経過し,様々な効果が報告されてきている.しかし一方で, GH治療が側弯症を誘発または増悪させる可能性が問題になってきている.今回我々は,獨協医科大学越谷病院小児科でフォロー中のPWS患者72名(男性46名,女性26名,年齢1歳から49歳)を対象にGH治療と側弯症の関係を検討した.対象の72名中, GH療法を受けた者は41名,受けなかった者は31名であった. 72名中33名(45.8%)にコブ角10度以上の側弯症を認めた.側弯症は, GH療法を受けた41名中20名(48.8%)に, GH療法を受けなかった31名中13名(41.9%)に認められ,両群間に統計学的有意差は認められなかった. GH療法を受けた患者中,側弯症あり群となし群間でのGH療法開始後1年目の身長増加の比較では,それぞれ8.59±1.92cmと10.70±2.54cmであった.側弯なし群のGH療法開始後1年目の身長は側弯あり群より有意に増加していた.したがって,統計学上では身長の加速は側弯症を悪化させていなかった. GH開始年齢と側弯症発症との関係では,側弯症なし群でGH療法開始年齢が有意に低かった(p=0.029). GH療法を受けている群で,側弯症を認めた20名のコブ角の経過は,変動なし10名,増悪6名,増悪軽快1名,軽快3名であった. PWS患者においてGH療法は身長増加をもたらすが側弯症増悪因子になっていないと考えられた.Growth hormone (GH) therapy for Prader-Willi syndrome (PWS) has started worldwide and various favorable effects have been reported. But the possibility of progression of scoliosis is being new problems for GH treatment. In this study we analyzed 72 patients who are followed at our hospital (46 males and 26 females, aged from 1 year old to 49 years old, 41 patients with GH use and 31 without GH). We studied 1. The overall frequency of scoliosis in PWS, 2. The frequency of scoliosis with and without GH therapy, 3. The two factors (i.e. height velocity of the first year of GH therapy and the starting age of GH) which may be related to the progression of scoliosis, and 4. The prognosis of the scoliosis. The results are ; 1. Out of 72 patients, 33 patients (45.8%) showed scoliosis more than 10 degree of Cobb angle. 2. Out of 41 patients with GH therapy 20 patients (48.8 %) had scoliosis and out of 31 patients without GH therapy 13 patients (41.9 %) showed scoliosis. No statistical difference was detected between two groups. 3. Height velocities of the first year of treatment were 8.59±1.92 cm and 10.70±2.54 cm with and without scoliosis, respectively (p<0.001). GH therapy was started earlier in patients without scoliosis than with scoliosis (p<0.0001). 4. Out of 20 patients with scoliosis and GH use, the degree of scoliosis progressed in six patients, improved in three, and fluctuated in one during the GH treatment. Our results show that there is a high frequency of scoliosis in PWS with and without GH treatment, Increased height velocity by GH does not develop scoliosis, and early start of GH is not an exacerbating factor of scoliosis. Many patients showed progression of scoliosis with age irrespective of the use of GH and at least some patients improved their scoliosis in degree by GH

    Lower Hb at the initiation of dialysis does not adversely affect 1-year mortality rate

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    Abstract Background The management of renal anemia in the pre-dialysis period has been remarkably improved by long-acting erythropoiesis-stimulating agents (ESA). However, many incident dialysis patients cannot achieve target hemoglobin (Hb) levels (> 10 g/dL) and sometimes require blood transfusions. Anemia at the time of dialysis initiation is reportedly correlated with cardiomegaly and early cardiovascular events. Here, we investigated whether this V-shaped depression in Hb level at dialysis initiation adversely affects short-term prognosis. Methods The medical charts of 166 patients who underwent initial dialysis were retrospectively reviewed for Hb level, ESA treatment status, dry weight (DW), cardiothoracic rate (CTR), and brain natriuretic peptide (BNP) level at dialysis initiation and 1 year later. Patients were subdivided into three groups according to the tertile of Hb levels. The risk of mortality within 1 year after initiation was analyzed using multivariable-adjusted Cox proportional hazard model. Result Mean Hb level at initiation was 8.6 ± 1.3 g/dL despite the administration of sufficient ESA. After initiation, Hb levels rapidly increased and the Hb time course showed a V-shape with the bottom at initiation. Hb level, CTR, and log BNP showed a significant negative correlation. The Hb level and CTR 1 year after initiation did not correlate with Hb levels at initiation. Lower Hb levels at initiation as a V-shaped depression do not adversely affect 1-year mortality rate by multivariable-adjusted Cox proportional hazard model. Conclusion Hb level around dialysis initiation showed a V-shaped depression despite ESA use. Our findings suggest that the V-shaped Hb depression at initiation does not affect short-term prognosis

    Electron transfer and decay processes of highly charged iodine ions

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    In the present experimental work we have investigated multi-electron transfer processes in Iq+ (q = 10, 15, 20 and 25) + Ne, Ar, Kr and Xe collisions at 1.5q keV energy. Using the coincidence technique between charge-selected projectile and recoil ions, the branching ratios between Auger and radiative decay channels have been measured in decay processes of multiply excited states formed by multi-electron transfer collisions. By combining these ratios with the measured absolute cross sections for total and single electron transfer processes, the partial cross sections for various charge changing processes have been determined. It has been shown that, in all the multi-electron transfer processes investigated, the Auger decays are far dominant over the radiative decay processes and the branching ratios are clearly characterized by the average principal quantum number <n> of the initial excited states of projectile ions, estimated from the extended classical-over-barrier-model (ECBM). We could express the branching ratios in high Rydberg states formed in multi-electron transfer processes by using the decay probability of one Auger electron emission
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