Cognitive and psychosocial functioning of patients with congenital nephrogenic diabetes insipidus.

Cognitive and psychosocial functioning of patients with congenital nephrogenic diabetes insipidus.
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DOI:
10.1002/(sici)1096-8628(19960102)61:1
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发表时间:
1996-01
期刊:
American journal of medical genetics
影响因子:
--
通讯作者:
J. A. Hoekstra;A. F. van Lieburg;L. A. H. Monnens;G. M. Hulstijn-Dirkmaat;V. V. A. M. Knoers-V.
J. A. Hoekstra;A. F. van Lieburg;L. A. H. Monnens;G. M. Hulstijn-Dirkmaat;V. V. A. M. Knoers-V.
中科院分区:
其他
文献类型:
--
作者:
J. A. Hoekstra;A. F. van Lieburg;L. A. H. Monnens;G. M. Hulstijn-Dirkmaat;V. V. A. M. Knoers-V.

文献摘要

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相似文献

精神发育迟滞(MR)通常被认为是先天性肾性尿崩症(NDI)的主要并发症之一。然而,NDI 患者的心理测量研究很少且已经过时。在本研究中,17 名男性 NDI 患者接受了心理评估。 14 名患者的总智商在(n = 13)或高于(n = 1)正常范围内,1 名患者的智力得分在 -1 至 -2 标准差(S.D.)之间,2 名年轻患者的一般认知指数超过 2 S.D.。低于标准。 17 名患者中有 8 名符合注意力缺陷多动障碍标准,10 名患者中有 7 名的短期记忆得分较低。除了某一年龄组的治疗开始年龄与言语智商之间存在负相关外,测试表现与诊断时年龄或高钠血症之间没有发现任何关系。尽管可以对 MR 和 NDI 之间的关联做出多种解释,但目前这种疾病患者中 MR 的患病率似乎远低于文献中建议的水平。
Mental retardation (MR) is generally considered one of the main complications of congenital nephrogenic diabetes insipidus (NDI). However, psychometric studies of NDI patients are scarce and outdated. In the present study, 17 male NDI patients underwent psychological evaluation. Total intelligence quotient of 14 patients was within (n = 13) or above (n = 1) the normal range, 1 patient had an intelligence score between -1 and -2 standard deviations (S.D.) and 2 young patients had a general cognitive index more than 2 S.D. below the norm. Attention deficit hyperactivity disorder criteria were met by 8 out of 17 patients and scores on short-term memory were low in 7 out of 10. No relation between test performances and age at diagnosis or hypernatremia could be found, with the exception of a negative correlation between age at start of therapy and verbal IQ in one age group. Although several explanations for an association between MR and NDI can be postulated, it seems that the current prevalence of MR among patients with this disease is considerably lower than suggested in literature.