Increased perinatal intracranial pressure and prediction of early puberty in girls with myelomeningocele

An increased risk of developing precocious puberty has been reported in children with myelomeningocele. In order to evaluate this further and to study factors associated with early or precocious puberty the medical records of all girls with myelomeningocele, born from 1970 onwards (n = 64), who were admitted to our unit were reviewed. Early/precocious puberty (E/PP) was defined as breast development or pubic hair corresponding to Tanner stage 2 before the age of 9.2 years. In 32 out of 62 cases data were sufficient for evaluation of the timing of puberty. Twenty girls had E/PP and 12 girls normal timing of puberty. In the girls who had reached the age of 9.2 years the incidence of E/PP was at least 52%. Girls with E/PP had a higher incidence of hydrocephalus, were treated with intraventricular shunts more often, and had significantly higher frequency of increased intracranial pressure during the perinatal period (p < 0.05, p < 0.01, and p < 0.001, respectively). The group of girls developing E/PP was also more severely disabled with respect to motor and urological function and had more shunt revisions. In conclusion, E/PP in girls with myelomeningocele is strongly associated with increased intracranial pressure particularly during the perinatal period.     

Increased perinatal intracranial pressure and prediction of early puberty in girls with myelomeningocele.

An increased risk of developing precocious puberty has been reported in children with myelomeningocele. In order to evaluate this further and to study factors associated with early or precocious puberty the medical records of all girls with myelomeningocele, born from 1970 onwards (n = 64), who were admitted to our unit were reviewed. Early/precocious puberty (E/PP) was defined as breast development or pubic hair corresponding to Tanner stage 2 before the age of 9.2 years. In 32 out of 62 cases data were sufficient for evaluation of the timing of puberty. Twenty girls had E/PP and 12 girls normal timing of puberty. In the girls who had reached the age of 9.2 years the incidence of E/PP was at least 52%. Girls with E/PP had a higher incidence of hydrocephalus, were treated with intraventricular shunts more often, and had significantly higher frequency of increased intracranial pressure during the perinatal period (p < 0.05, p < 0.01, and p < 0.001, respectively). The group of girls developing E/PP was also more severely disabled with respect to motor and urological function and had more shunt revisions. In conclusion, E/PP in girls with myelomeningocele is strongly associated with increased intracranial pressure particularly during the perinatal period.     

Bladder-sphincter dysfunction in myelomeningocele

 Pediatric urodynamics taught us that detrusor-sphincter dyssynergia creates a bladder outlet obstruction in about 50% of any population of children with myelomeningocele. This functional obstruction causes renal damage due to obstructive uropathy, exactly the same way as a congenital anatomical urethral obstruction does. Pediatric urodynamics also taught us that in children with myelomeningocele pelvic floor activity and detrusor activity can be abnormal (hyperactive or inactive) completely independent from each other. These insights have changed the management of myelomeningocele. Children with overactivity of the pelvic floor can be singled out at infant age, and started on clean intermittent catherization, to prevent obstructive uropathy and preserve renal function. Children with detrusor overactivity can be singled out too at very early age, and treated with anticholinergics, to prevent irreversible structural damage to the detrusor and preserve normal bladder capacity and compliance. Received: 20 July 1999 / Accepted: 24 January 2001     

Induction of puberty in boys with delayed adolescence by methandrostenolone

Methandrostenolone administration at a daily dose of 0.03 mg/kg for 3 months was successful in inducing puberty in 9 boys (aged 14 6/12±6/12 years, m±SD) with delayed puberty and studied in the prepubertal stage. One year after initiation of treatment they reached a mid-pubertal stage (testicular volume m±SD 6±2 ml and pubic hair development Tanner stage 3–4). At the same time growth velocity accelerated from 5.3±1.5 to 8.5±3.4 cm/yr and bone age advanced from 10 9/12±9/12 to 13±6/12 years (m±SD).During treatment there was suppression of basal plasma LH and FSH (m±SD) from 1.3±0.3 to 0.5±0.2 mIU/ml (P<0.001) and from 1.4±0.8 to 0.8±0.3 mIU/ml (P<0.05) respectively, and of the LH response to LRH (50 mcg/m², i.v.) from 5.2±1.0 to 1.9±0.6 mIU/ml (P<0.001). After discontinuation of methandrostenolone there was a significant and prolonged elevation of the basal plasma LH (2.0±0.4 mIU/ml) and testosterone levels (from 24±7.7 to 175.6±67.5 ng/dl, P<0.01) and an enhanced LH response to LRH (8.3±2.4 mIU/ml, P<0.05), compared to the pre-treatment levels.Eleven prepubertal boys with constitutional short stature (aged 9 3/12±9/12 years, m±SD) maintained their prepubertal state one year following the same therapeutic regime with methandrostenolone. No significant changes in the basal plasma testosterone and gonadotropin levels, or the responses to LRH, were noted in this group.During treatment a significant increase in growth velocity was noted (from 4.1±1.7 to 9.7±3.0 cm/year, P<0.02), with a subsequent decrease to 5.4±2.9 cm/year (m±SD) which was not significantly different to the pre-treatment value. Bone age advanced from 6 3/12±1 before treatment to 8±1 6/12 years 12 months following methandrostenolone administration.It is concluded that methandrostenolone can induce puberty in boys with delayed puberty if administered in the prepubertal stage, but not in younger prepubertal boys with short stature. The concomitant changes in the basal plasma testosterone and gonadotropin levels, and their response to LRH stimulation, which were found in the boys with delayed puberty indicate that a certain degree of maturation of the hypothalamic pituitary gonadal axis is probably needed to permit induction of puberty by methandrostenolone. The effect of this drug is due in part to its androgenic potency and probably also to its modulation of negative feedback in the hypothalamic-pituitary-gonadal axis, causing a rebound phenomenon following brief suppression.Supported in part by the Harry C. Bernard Fund     

Alterations of intracranial pressure and cerebral blood flow velocity in healthy neonates and their implication in the origin of perinatal brain damage

Intracranial pressure and cerebral blood flow velocity were recorded in term healthy neonates during the first 3 days of life using non-invasive methods (LADD-fontanometry and cw-Doppler sonography). Intracranial pressure increased from 4.0±2.7 cm H₂O to 5.8±2.7 cm H₂O and maximal cerebral blood flow velocity in the anterior cerebral artery (ACA) increased from 33 cm/s to 58 cm/s as calculated from a Doppler shift of 0.63 to 1.10 kHz and vascular resistance decreased between the 1st and 3rd day of life. These alterations could not be demonstrated in the femoral artery. This is in accordance with other registrations obtained by different methods and under various conditions. They allow an explanation of some well known physiological phenomena like alterations of cranial volume and the structure of the bony skull in the first days of life. Furthermore, these physiological variations may have implications for the origin of cerebral damage during the perinatal period, especially of hypoxic-ischaemic encephalopathies.Abbreviations ACA anterior cerebral artery - CA carotid artery - FA femoral artery - IP intracranial pressure - MFV mean flow velocity - PI pulsatility index (Pourcelot) - REM rapid eye movements sleep state - TAM time-average-mean - V cranial volume     

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目的分析男性儿童同性性早熟的病因及临床特点。方法回顾性分析1988年1月至2009年4月中山大学附属第一医院收治的明确病因诊断的78例男性同性性早熟病例的病因及临床特点。结果中枢性性早熟(CPP)55例(70.51%),按构成比前三位病因为特发性性早熟、下丘脑错构瘤、先天性肾上腺皮质增生症(CAH)继发,其中下丘脑错构瘤患儿就诊年龄小、GnRHa激发试验后LH浓度最高,CAH患儿骨龄提前最多、HtSDSba负值最大;外周性性早熟(PPP)23例(29.49%),分泌HCG生殖细胞瘤和CAH为主要病因,CAH患儿由PPP转变为CPP的比例较大(5/9),尤其是初治年龄较大者更易发生。分泌HCG的生殖细胞瘤血和(或)脑脊液的β-HCG水平均升高。结论男性儿童性早熟以器质性病变引起多见,在诊治过程中应积极寻找病因。     

颅内出血早产儿生后四天内脑血流变化及围生因素分析

目的　探讨颅内出血早产儿生后 4d内脑血流动力学的变化及颅内出血与围生因素的关系。方法　应用经颅多谱勒超声 (TCD)检测 2 9例颅内出血早产儿脑血流速度、阻力指数的变化及脑血流速度与平均动脉血压的关系 ,分析临床围生相关因素 ,并与同期无颅内出血的 2 8例早产儿进行比较。结果　颅内出血组早产儿于生后 2 4、4 8、72h脑血流速度增快 ,出生 2 4h平均脑血流速度与平均动脉血压相关 ,且与出生窒息、胎龄、出生体重、高PaCO2 有关。结论　颅内出血早产儿于生后 2 4h内存在脑血流自主调节功能破坏 ,生后 3d脑血流存在过度灌注 ;且出生窒息 ;胎龄 <32周、出生体重 <15 0 0 g及PaCO2 >6 6 7kPa为颅内出血的高危因素。     

促性腺激素释放激素类似物治疗对中枢性性早熟和快速进展型青春期患儿成年终身高改善的Meta分析

目的 系统评价促性腺激素释放激素类似物（gonadotropin-releasing hormone analogue,GnRHa）治疗6岁以上中枢性性早熟（central precocious puberty,CPP）和快速进展型青春期（early and fast puberty,EFP）患儿的疗效。 方法 检索PubMed、MEDLINE、Embase、Cochrane Library、中国知网和万方数据库,收集GnRHa治疗CPP和EFP患儿的相关文献,应用Stata 12.0软件对文献资料进行Meta分析。 结果 共纳入10篇文献。总样本量为720例,其中GnRHa治疗组475例,对照组245例。Meta分析结果显示GnRHa治疗组成年终身高［加权均数差（weighted mean difference,WMD）=3.30,95%CI：2.49~4.12,P<0.001］、成年终身高标准差积分（WMD=0.51,95%CI：0.29~0.73,P<0.001）、身高获益（WMD=2.89,95%CI：2.17~3.60,P<0.001）均优于对照组。所有的研究均无严重不良事件报道。 结论 GnRHa治疗对于改善6岁以上CPP和EFP患儿的成年终身高安全有效。 引用格式:     

儿童颅内压监测研究进展

颅内高压是儿科较常见的急危重症,颅内压(intracranial pressure,ICP)监测能动态评估脑损伤患者的病情变化,计算脑灌注压,指导临床治疗.ICP监测有其局限性,不能及时反映大脑微血管功能障碍和细胞功能障碍,因此需要在ICP监测的基础上开展多模态监测(multimodality monitoring).ICP监测和多模态监测的信息整合有助于进一步理解脑损伤的病理生理机制,有助于对患者进行针对性个体化治疗.     

持续颅内压监测在创伤性脑损伤患儿中的应用

颅内压监测已经成为了创伤性脑损伤患儿监护的基本技术.随着医疗水平的发展,有关颅内压监测的研究已成为近年来神经重症监护的重要研究课题.该文就颅内压监测在创伤性脑损伤患儿中的应用作一综述.     

Auditory brainstem response in neonates with asphyxia and intracranial haemorrhage

Auditory brainstem response (ABR) was used to assess possible brainstem damage in 76 neonates with asphyxia and intracranial haemorrhage (ICH). Fifty-eight neonates had ICH, 52 had neonatal asphyxia and 34 of these patients had both. Eighty-nine percent of the patients with neonatal asphyxia showed some abnormal patterns in response, the major one being an increase in the threshold of wave V. In the ICH group, abnormal patterns were observed in 62.5%, among whom the prolongation of the I–V interpeak latency (IPL) and of wave V latency was seen more frequently than the increase of threshold of wave V. In the case of neonatal asphyxia associated with ICH, both the prolongation of the latency and the increase of threshold were observed equally. These abnormalities of ABR were associated with worsening clinical condition and conversely normalized gradually following the improvement of the underlying disease. Especially the I–V IPL, wave V latency and the threshold of wave V could serve as indicators of the treatment.Abbreviations ABR auditory brainstem response - ICH intracranial haemorrhage - IPI interpeak latency - NICU neonatal intensive care unit - AFD appropriate-for-date - SFD small-for-date - SDH subdural haemorrhage     

围产因素对早期早产儿淋巴细胞亚群演变趋势的影响

目的 了解早期早产儿淋巴细胞亚群变化趋势及围产因素的影响。方法 流式细胞术检测61例早期早产儿生后第1、7、14、28 天以及其中17 例早期早产儿出生后6 个月的淋巴细胞亚群水平。分析围产期因素的影响。结果 早期早产儿出生时除自然杀伤（NK）细胞外各亚群淋巴细胞绝对计数均处于最低水平,1 周时迅速升高,6 个月升至正常;NK 细胞至6 个月方增高。与胎龄P P 0.05）;第7、14 天的B 细胞绝对计数以宫内感染组较高（P P 结论 早期早产儿出生时免疫细胞数量不足,以后逐渐增高,6 个月左右达正常。母亲产前使用激素以及出生胎龄、宫内感染、UU 定植等多种围产因素对早期早儿淋巴细胞亚群水平有较长时间的影响。     

Tap-water enema for children with myelomeningocele and neurogenic bowel dysfunction

AIM: To evaluate the outcome of transrectal irrigation (TRI) using clean tap water without salt in children with myelomeningocele and neurogenic bowel problems. METHODS: 40 children (21 boys and 19 girls; aged 10 mo to 11 y) with myelomeningocele and neurogenic bowel dysfunction were treated with TRI given by a stoma cone irrigation set daily or every second day. A questionnaire on the effects on faecal incontinence, constipation and self-management was completed by the parents, 4 mo-8 y (median 1.5 y) after start. Effects on rectal volume, anal sphincter pressure and plasma sodium were evaluated before and after the start of irrigation. RESULTS: At follow-up, 35 children remained on TRI, four had received appendicostomy, while one defecated normally. For all children but five (35/40; 85%) the procedure worked satisfactorily, but a majority found the procedure very time consuming and only one child was able to perform it independently. All children were free of constipation; most (35/40) were also anal continent. Rectal volume and anal sphincter pressure improved, while plasma sodium values remained within the normal range. CONCLUSION: Transrectal irrigation with tap water is a safe method to resolve constipation and faecal incontinence in children with myelomeningocele and neurogenic bowel dysfunction, but it does not help children to independence at the toilet.     

Testosterone esters advance skeletal maturation more than growth in short boys with chronic renal failure and delayed puberty

Four young males with chronic renal failure and absent or stagnant puberty were treated with testosterone esters. Endocrine evaluation before therapy showed low plasma follicle stimulating hormone (FSH) levels and relatively high luteinizing hormone (LH). Following therapy skeletal maturation accelerated more than growth velocity, resulting in a lower predicted adult height. In three patients osteoporosis increased or rickets developed. Testosterone therapy was effective in developing sex characteristics, but endogenous pubertal development was not stimulated. Growth velocity was increased, but the effect on growth was more than outweighed by bone age acceleration.     

Evaluation of bone mineral density after ileocystoplasty in children with and without myelomeningocele

We evaluated the bone mineral density (BMD) after ileal augmentation cystoplasty in a group of children with and without myelomeningocele. Between 1996 and 2003, eight patients with neurogenic bladder and seven patients with non-neurogenic bladder underwent augmentation ileocystoplasty. Preoperative and postoperative serum creatinine and electrolytes were measured. All patients underwent clinical evaluation, supine height measurement, blood gas analysis, and BMD measurement using a dual energy X-ray absorptiometry (DEXA) postoperatively. BMD was measured at L1–L4 and femoral neck, and compared to age- and sex-matched population. Follow-up time was calculated from the day of surgery to the day of DEXA performance. A total of eight boys and seven girls with the mean age of 10.2±4.1 years were evaluated with respect to BMD measurement. Mean age was 9.8 years in neurogenic group and 10.5 in non-neurogenic group. Mean follow-up was 728 and 616 days in neurogenic and non-neurogenic groups, respectively. There were no significant differences between ages, follow-up times, preoperative and postoperative creatinine levels, pH and bicarbonate values, and supine heights. Mean BMD at L1–L4 was 55.7% in neurogenic group and 83.8% in non-neurogenic group. There was a statistically significant difference between the two groups (P=0.02). Mean BMD at femoral neck was 72% in neurogenic group and 86.2% in non-neurogenic group. The difference was also statistically significant (P=0.028). After augmentation ileocystoplasty, the BMD in early postoperative period of patients with myelomeningocele is lower than the patients with non-neurogenic neurogenic bladder, which have the same clinical characteristics except the neurologic pathology. In the light of our findings and the reported literature data as well, we may claim that BMD decrease after augmentation ileocystoplasty depends more on the underlying neurologic pathology and its locomotor consequences rather than the enterocystoplasty itself.     

脑干听觉诱发电位对重症手足口病神经系统损害的早期诊断价值

目的 探讨脑干听觉诱发电位（brainstem auditory evoked potential,BAEP）对手足口病重症病例神经系统损害的早期诊断价值.方法 本研究通过对297例手足口病患儿进行BAEP检测,之后按照严重程度分级及年龄进行分组比较,手足口病普通病例、年龄＜3岁为A组（n=80）,重症病例重型、年龄＜3岁为B组（n=94）,普通病例、年龄≥3岁为C组（n=38）,重症病例重型、年龄≥3岁为D组（n=85）.回顾性分析各组脑电图（electroencephalogram,EEG）、头部MRI的检测结果,并与BAEP结果进行比较.结果 （1）B组BAEP异常率（18/94,19.15％）高于A组（3/80,3.75％）,D组BAEP异常率（13/85,15.29％）高于C组（1/38,2.63％）,差异有统计学意义（P＜0.05）.（2）B组EEG异常率偏低（2/94,2.13％）,A组无一例EEG异常.（3）D组EEG异常率（49/85,57.65％）高于C组（6/38,15.79％） （P ＜0.05）,且高于D组BAEP异常率.（4）B组MRI异常率（9/94,9.57％）高于A组（1/80,1.25％）（P＜0.05）,D组MRI异常率（9/85,10.59％）高于C组（0）.（5）全部手足口病重症病例重型患儿的BAEP异常率（31/179,17.32％）高于MRI异常率（18/179,10.06％） （P＜0.05）.结论 BAEP检测可为手足口病重症病例神经系统损害的早期诊断提供客观依据.     

Cardiac enzyme levels in myocardial dysfunction in newborns with perinatal asphyxia

Objective  To study the usefulness of cardiac enzymes in evaluating myocardial damage in perinatal asphyxia. Methods  Thirty term babies with perinatal asphyxia and without any congenital malformations were selected as cases. They were compared with thirty healthy term babies without asphyxia. Myocardial dysfunction was evaluated using clinical, electrocardiography, echocardiography and cardiac enzymes i.e, troponin-T and CK-MB levels. Results  Among the 30 cases 23 had evidence of myocardial involvement while one baby in the control group had ECG evidence compatible with cardiac involvement. Cardiac enzymes were significantly increased in babies with perinatal asphyxia. The mean level of C-troponin-T among cases and controls were 0.22±0.28 and 0.003±0.018 while CK-MB levels were 121±77.4 IU/L and 28.8 ± 20.2 IU/L respectively. C-troponin-T had higher sensitivity and specificity compared to CK-MB levels. Moreover, C troponin-T levels correlated well with severity and outcome in babies with perinatal asphyxia. Conclusion  C-Tropopnin assay is useful in evaluating the severity of myocardial damage and outcome in perinatal asphyxia.