Prevention of shaken baby syndrome: Never shake a baby

Shaken baby syndrome (SBS) represents injuries to the head, skeleton and eyes of a young child and is the leading cause of fatal or life-threatening child abuse. SBS is preventable. The dangers and consequences of shaking a baby are not well appreciated by the general public. Simple educational programs and community nursing support programs have been shown to be helpful. Inadequate physician training and knowledge in child maltreatment have also been identified as problems. This article outlines the evidence for interventions in the prevention of SBS and recommendations for health care providers and educators.     

In vivo proton magnetic resonance spectroscopy in phenylketonuria

In vivo nuclear magnetic resonance spectroscopy permits the non-invasive examination of metabolic characteristics of the human brain in a clinical environment. Methods to detect elevated phenylalanine (Phe) in patients with phenylketonuria (PKU) using difference spectroscopy and to estimate absolute brain Phe concentrations, [Phe]_brain, have been developed. In patients with classical PKU, [Phe]_brain typically varied between 0.14 and 0.78 mmol/l depending upon actual blood Phe concentrations, [Phe]_blood, between 0.47 and 2.30 mmol/l. Dynamic investigations can be used to extract information about Phe transport at the human blood-brain barrier, which may be described by a symmetric Michaelis-Menten model. Carrier saturation and competitive inhibition of the influx of other large neutral amino acids can be expected at blood levels usually found in PKU patients. In single cases of untreated, normal intelligent patients, abnormally low [Phe]_brain h 0.15 mmol/l were observed despite high stationary Phe levels ([Phe]_blood=1.15 - 0.10 mmol/l). Conclusion Significant variations in phenylalanine transport parameters in untreated, normal intelligent patients indicated that blood-brain barrier transport or intracerebral phenylalanine consumption are causative factors for the individual vulnerability to phenylketonuria.     

Minimally invasive management of a traumatic artery aneurysm resulting from shaken baby syndrome

Based on our review of the literature, we present the first use of coiling in an infant with a traumatic artery aneurysm that resulted from shaken baby syndrome. Computed tomography (CT) scans showed a skull fracture, hemorrhagic subdural collections, multiple parenchymal contusions, and intraventricular and subarachnoid hemorrhages in a 3-week-old infant who presented with lethargy, poor feeding, and seizure. These multiple injuries were consistent with shaken baby syndrome. After closed-head injury medical management, including subdural taps, the baby was discharged home. When increasing seizures and hydrocephalus developed 8 months later, CT angiographic scans showed a pseudoaneurysm of the anterior cerebral artery. We successfully occluded the aneurysm with pushable coils placed via a microcatheter and treated the obstructive hydrocephalus with endoscopic third ventriculostomy. We show that minimally invasive radiological and surgical techniques may be effective in managing the sequelae of trauma in children.     

Localized proton magnetic resonance spectroscopy of cerebral abnormalities in children with carbohydrate-deficient glycoprotein syndrome

Morphologic and metabolic abnormalities in six children aged 2-9 years with carbohydrate-deficient glycoprotein (CDG) syndrome were assessed by magnetic resonance imaging (MRI) and localized proton magnetic resonance spectroscopy (MRS). In all patients, MRI revealed pronounced cerebellar atrophy. Follow-up examinations in two patients suggested early onset and rapid progression in the first years of life. Further pathologies comprised Dandy-Walker malformation, atrophy of the pons, brain stem and olives, supratentorial frontotemporal cortical atrophy, slightly dilated ventricles and a small corpus callosum. Two patients presented with small cysts in the white matter. The prominent metabolic abnormality detected by proton MRS in five patients was a reduction in N-acetylaspartate in white matter by more than 20%, indicating loss of vital neuroaxonal tissue. Further findings in white matter were glutamine and 7-aminobutyrate increases by a factor of 2. One patient with type III CDG syndrome showed the most severe alterations of metabolite concentrations. CDG syndrome, cerebellar atrophy, magnetic resonance imaging, proton magnetic resonance spectroscopy     

氢质子磁共振波谱在小儿惊厥中的应用进展

惊厥是儿科常见急症,其发生的机制、发作部位、脑损伤的程度、治疗效果及预后评估一直是人们所关注的问题.近年来,氧质子磁共振波谱的问世,为临床提供了一种新的测定脑中与能量生成有关的各种代谢物质浓度,概观脑部特定区域的神经生化状态的非创伤性技术.目前已广泛应用于神经系统疾病.它在小儿惊厥性疾病的应用中,从生化代谢水平上,对了解和研究惊厥后脑内组织代谢的变化,为惊厥的早期诊断、治疗效果及预后评估等提供了一种新的手段.     

Use of localized proton nuclear magnetic resonance spectroscopy in Canavan's disease

Canavan's disease is characterized by megalencephaly, leukodystrophy and early motor and mental retardation. On computerized tomography and magnetic resonance imaging, severe changes compatible with white matter disease due to demyelination is observed. It has been demonstrated that urinary N-acetylaspartate levels are increased because of a deficiency of aspartoacylase (N-acyl-L-aspartate aminohydrolase) in these patients. In this study, with the use of proton nuclear magnetic resonance spectroscopy, we were able to demonstrate elevated levels of N-acetylaspartate compared to choline and creatine in the frontal region white matter of three patients. The in vivo measurement of N-acetylaspartate, choline and creatine in the brain by magnetic resonance spectroscopy offers an additional noninvasive diagnostic test for establishing the diagnosis of Canavan's disease.     

In vivo proton magnetic resonance spectroscopy assessment for muscle metabolism in neuromuscular diseases

Muscle metabolites were obtained by in vivo proton magnetic resonance spectroscopy of 3 patients with Duchenne muscular dystrophy (DMD), 6 patients with spinal muscular atrophy (SMA), and 10 normal volunteers. Patients with DMD and SMA had lower trimethyl amide (TMA)/water and TMA/total creatine (tCr) ratios but normal tCr/water ratios.     

氢质子磁共振波谱在原发性癫癎中的应用研究

目的：应用氢质子磁共振波谱(proton magnetic resonance spectroscopy, 'H-MRS)检测癫癎患儿的脑组织生化代谢物,探讨'H-MRS在原发性癫癎中的临床应用价值及意义。方法：对33例原发性癫癎患儿（癫癎组,其中14例有热性惊厥史）和6例正常儿童（对照组）进行常规头颅MRI和颞叶海马区的'H-MRS检查。检测N-乙酰天门冬氨酸(NAA)、肌酸(Cr)、胆碱(Cho)和乳酸(Lac)的信号强度,计算并比较NAA/（Cho+Cr）和Lac/Cr的比值。结果：头颅MRI检查：除1例癫癎患儿表现为髓鞘发育不良外其余所有研究对象均无异常。'H-MRS检测：NAA/（Cho+Cr）比值在癫癎组为0.64±0.07,低于对照组(0.73±0.05)（P＜0.01）;同时伴有热性惊厥史的癫癎患儿为0.61±0.07,低于不伴有热性惊厥史的癫癎患儿（0.66±0.06）（P＜0.05）。癫癎组Lac/Cr比值与对照组差异无统计学意义。结论：'H-MRS作为一项无创的检查,能更敏感地发现癫癎患儿的早期脑损伤,为癫癎的诊断和评估预后提供客观依据。［中国当代儿科杂志,2010,12（6）：425-428］     

Cerebral proton magnetic resonance spectroscopy of neonates after extracorporeal membrane oxygenation

During venoarterial extracorporeal membrane oxygenation the right carotid artery is ligated in a hypoxic neonate. The aim of the present study was to compare the morphology and metabolism of the left and right basal ganglia in 10 neonates after extracorporeal membrane oxygenation, using proton magnetic resonance imaging and spectroscopy. Data could be obtained in 9 neonates. No significant metabolic differences were found between either the left or right basal ganglia, despite a small right-sided thalamic infarct in one child. Metabolism was normal in all cases. All the infants showed symmetrical neurodevelopment.

Conclusion: Ligation of the right carotid artery for venoarterial extracorporeal membrane oxygenation did not produce persistent changes in brain metabolism in the basal ganglia in this small group of patients.     

Localised proton magnetic resonance spectroscopy of the brain after perinatal hypoxia: a preliminary report

Objectives. Perinatal hypoxic ischaemic injury is a significant cause of neurodevelopmental impairment. The aim of this study was to evaluate localised proton magnetic resonance spectroscopy (¹H-MRS) after birth asphyxia. Materials and methods. Thirty newborn infants suspected of having perinatal asphyxia (Apgar score < 3) were studied. The mean gestational age was 37 weeks, mean age at the MR examination was 18 days and mean weight was 2.9 kg. A 1.5-T unit was used for imaging and spectroscopy. None of the babies had mechanically assisted ventilation. No sedation was used. Axial T1-weighted and T2-weighted images were obtained. ¹H-MRS was recorded in a single voxel, localised in white matter, using a STEAM sequence. Results. Image quality was good in 25 of 30 babies. ¹H-MRS was performed in 19 of 30 subjects, with adequate quality in 16. Choline, creatine/phosphocreatine and N-acetylaspartate peaks and peak-area ratios were analysed. Lactate was detected in four infants. The N-acetylaspartate/choline ratio was lower in infants with an impaired neurological outcome, but the difference was not statistically significant. Conclusions. This study suggests that ¹H-MRS may be useful for assessing cerebral metabolism in the neonate. A raised lactate level and decreased N-acetylaspartate/choline ratio may be predictive of a poor outcome. However, in our experience this method is limited by the difficulty in performing the examination during the first hours after birth in critically ill babies, the problems related to use of a monovoxel sequence, the dispersion of the ratios and the lack of determination of the absolute concentration of the metabolites. Received: 30 March 1998 Accepted: 24 September 1998     

Proton magnetic resonance spectroscopy reflects metabolic decompensation in maple syrup urine disease

Using localized proton magnetic resonance spectroscopy (¹H-MRS), accumulation of branchedchain amino acids (BCAA) and their corresponding 2-oxo acids (BCOA) could be non-invasively demonstrated in the brain of a 9-year-old girl suffering from classical maple syrup urine disease. During acute metabolic decompensation, the compounds caused a signal at a chemical shift of 0.9 ppm which was assigned by in vitro experiments. The brain tissue concentration of the sum of BCAA and BCOA could be estimated as 0.9 mmol/l. Localized¹H-MRS of the brain appears to be suitable for examining patients suffering from maple syrup urine disease in different metabolic states.     

Analysis of fetal and neonatal urine using proton nuclear magnetic resonance spectroscopy.

AIM--To use high field proton nuclear magnetic resonance spectroscopy (1H NMR) to characterise the low molecular weight metabolite composition of neonatal and fetal urine in relation to gestational age and perinatal outcome. METHODS--The first urine passed by two neonatal groups, six full term and five preterm infants with normal renal function, was analysed by 1H NMR and compared with fetal urine from 14 cases with obstructive uropathy. RESULTS--The mean ratios of taurine, myo-inositol, and trimethylamine-N-oxide (TMAO) to creatinine were 4.3, 10.1, and 14.1 times higher, respectively, in the preterm group when compared with those of the full term group. Fetal obstructive uropathy was characterised by glycosuria, amino and organic aciduria, regardless of gestational age (13-30 weeks). CONCLUSIONS--Samples of the first urine passed--that is, urine produced in fetal life--by normal preterm infants are useful controls for cases of obstructive uropathy detected in the third trimester. 1H NMR will become a clinically useful tool for monitoring renal development and abnormalities in utero.