Histidinaemia: a benign metabolic disorder

Histidinaemia is a relatively common inherited metabolic disorder with an incidence similar to phenylketonuria. This paper reports the long term outcome of patients diagnosed by newborn screening in the north west of England. Between 1966 and 1990, 108 infants were diagnosed as having histidinaemia by a regional neonatal screening programme (incidence 1:11,083). A further five children were detected following diagnosis in a sibling. Of the 113, nine were lost to follow up. Infants diagnosed before 1981 (n = 47) were placed on a low histidine diet (225 mg/kg/d) for an average period of 21 months (SD 4.5). All patients were reviewed regularly, Griffiths developmental quotients (DQ) were assessed at 2 and 4 years, and WISC-R intelligence quotients (IQ) at 8, 12, and 18 years. IQ data were converted to standard deviation scores (IQ SDS) to account for increasing IQ norms with time. Neither DQ nor IQ correlated with plasma histidine at diagnosis or with the mean plasma histidine throughout life. Growth was normal in all patients. There was no apparent benefit from a low histidine diet in early childhood. In contrast to other studies, there was no excess of clinical symptoms. On the basis of these findings, histidinaemia is a benign metabolic disorder that does not require treatment.     

肺靶向米托蒽醌明胶微球的研究

采用二步法制备米托蒽醌明胶微球,球径范围为5.1～25.0μm的占总数87.36%,体外释药与原药相比t1/2延长4倍,DTA曲线上的特征吸热峰为133℃,经37℃,RH75%考察3月,几乎无变化。经小鼠体内分布试验表明具有明显的肺靶向性,靶向效率增加3～35倍,肺中药代动力学行为可用一室开放模型描述,平均滞留时间延长10h。     

Lingual thyroid gland: sonographic appearance

Thyroid dysgenesis is often manifested by a lingual or sublingual location of the thyroid gland. If radionuclide studies are not readily available or are contraindicated, ultrasound (US) may be used to locate the gland. The US appearance of a lingual thyroid gland and the role of US in the evaluation of suspected thyroid dysgenesis are described.     

HPLC测定益肤霜中红霉素和地塞米松的含量

采用ＨＰＬＣ测定益肤霜中红霉素和地塞米松的含量，控制制剂的质量。色谱条件：固定相为Ｋｒｏｍａｓｉｌ Ｃ１８柱；流动相为乙腈－０．２ｍｏｌ／Ｌ醋酸铵－水，流速０．８ｍｌ／ｍｉｎ；检测波长２１５ｎｍ。以ｃ对峰面积Ａ作直线回归，红霉素在１．５－２４ｇ／Ｌ地塞米松在２０－３００ｍｇ／Ｌ的范围内，其浓度与峰面积呈直线关系。红霉素回收率为９９．６９％；地塞米松的回收率为１００．２８％。本法操作简便，结果准确，     

Quality assessment and assurance in diagnostic imaging. Theoretical and practical considerations and a quality audit of the excretory urogram

Concepts necessary to an understanding of the basics of quality assurance audits are presented. Included are specific examples that bridged theory and practice by applying the protocol to a real-life diagnostic imaging situation. This method meets the present requirements of the Joint Commission of the Accrediation of Hospitals.     

Secondary hematological malignancies after breast cancer chemotherapy

According to several reports, the 10 year incidence of secondary acute myelogenous leukemia (AML) or myelodysplastic syndrome (MDS) after systemic chemotherapy is approximately 1.5%. The cumulative risk increases by 0.25 - 1% for the first 8 years after treatment. We have reported only 6 cases of hematological malignancies (0.3%) after breast cancer chemotherapy in our institute. We detected 2 cases of secondary AML and 1 case of MDS, 19, 52 and 12 months, respectively, after systemic chemotherapy for breast cancer. Published data on the occurrence of secondary hematological malignancies other than AML or MDS in this setting are scarce. We encountered diffuse large B-cell lymphoma, angioimmunoblastic lymphoma and mantle cell lymphoma as secondary hematological malignancies after systemic chemotherapy for breast cancer.