Clinical Manifestations and Amplitude-integrated Encephalogram in Neonates with Early-onset Epileptic Encephalopathy / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The patients with early-onset epileptic encephalopathy (EOEE) suffer from neurodevelopmental delay. The aim of this study was to analyze the clinical manifestations and amplitude-integrated encephalogram (aEEG) characteristics of infants with EOEE with onset within the neonatal period, to make early diagnosis to improve the prognosis.</p><p><b>METHODS</b>One-hundred and twenty-eight patients with neonatal seizure were enrolled and followed up till 1 year old. Sixty-six neonates evolved into EOEE were as the EOEE group, the other 62 were as the non-EOEE (nEOEE) group. Then we compared the clinical and aEEG characteristics between the two groups to analyze the manifestations in neonates with EOEE.</p><p><b>RESULTS</b>Compared to the nEOEE group, the incidence of daily seizure attacks, more than two types of convulsions, more than two antiepileptic drugs (AEDs) application, severely abnormal aEEG background, absence of cyclicity, and more than two seizures detection were significantly higher in the EOEE group (P < 0.05) (97% vs. 54.8%; 30.3% vs. 14.5%; 97.0% vs. 25.4%; 39.4% vs. 3.2%; 57.6% vs. 9.7%; and 56% vs. 3.2%, respectively). Severely abnormal background pattern (odds ratio [OR] = 0.081, 95% confidence interval [CI]: 0.009-0.729, P = 0.025) and more than two seizures detection by aEEG (OR = 0.158, 95% CI: 0.043-0.576, P = 0.005) were the independent risk factors for the evolvement into EOEE. The upper and lower margins of active sleep (AS) and quiet sleep (QS) were significantly higher in EOEE group than those of the control group (P < 0.05) (34.3 ± 13.6 vs. 21.3 ± 6.4; 9.9 ± 3.7 vs. 6.7 ± 2.2; 41.2 ± 15.1 vs. 30.4 ± 11.4; and 11.9 ± 4.4 vs. 9.4 ± 4.0; unit: μV, respectively). AS upper margin was demonstrated a higher diagnostic specificity and sensitivity for EOEE than another three parameters according to the receiver operating characteristic curves; the area under the curve was 0.827.</p><p><b>CONCLUSIONS</b>The clinical characteristics of the neonatal seizure which will evolve into EOEE were more than two AEDs application, high seizure frequency (daily attack), and more than two types of the seizure. Significant high voltage, severely abnormal background, absence of cyclicity, and more than two seizures detected on aEEG were the meaningful indicators to the prediction of EOEE.</p>

STXBP1 gene mutation in newborns with refractory seizures / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the relationship between STXBP1 gene mutations and refractory seizures with unknown causes in newborns.</p><p><b>METHODS</b>The coding region of STXBP1 gene was detected using direct Sanger sequencing in 11 newborns with refractory seizures of unknown causes.</p><p><b>RESULTS</b>STXBP1 gene mutation was found in 1 out of 11 patients. It was a missense mutation: c.1439C>T (p.P480L).</p><p><b>CONCLUSIONS</b>STXBP1 gene mutation can be found in neonatal refractory seizures of unknown causes, suggesting a new approach of further research of this disease.</p>

Combined methylmalonic aciduria and homocysteinemia with hydrocephalus as an early presentation: a case report / 中国当代儿科杂志

A case of combined methylmalonic aciduria and homocysteinemia presenting with hydrocephalus as an early manifestation was reported for its rarity to see and to discuss the relationship between metabolic diseases and hydrocephalus by literature review. The case was an infant with seizures and hydrocephalus as an early manifestation of the disease, combined with macrocyticanemia, development retardation and visual hearing function lesions. The EEG showed hypsarrhythmia and the MRI showed hydrocephalus. Plasma homocysteinemia level increased (143.06 umol/L) and urine methylmalonic aciduria was 1483 times beyond normal. Based on gene analysis results and increased methylmalonic aciduria and homocysteinemia levels, combined methylmalonic aciduria and homocysteinemia was confirmed, presenting CblC defect (gene mutations homozygous for c.609G>A). After treatment by venous injection of vitamin B12, oral folic acid and betaine, seizures were controlled and development was progressive with ventricle retraction. It was concluded that hydrocephalus can be the early presentation in children with combined methylmalonic aciduria and homocysteinemia. Doctors should carry out metabolic disease screening for patients with hydrocephalus, especially when the cause of hydrocephalus is uncertain.

Relationship between degree of white matter damage and EEG changes in premature infants early after birth / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the relationship between the degree of white matter damage and changes in brain function in premature infants early after birth according to amplitude-integrated electroencephalogram (aEEG) and raw EEG (with burst-suppression patterns).</p><p><b>METHODS</b>Thirty-eight premature infants of less than 32 weeks' gestational age and with white matter damage, including 20 cases of mild white matter damage and 18 cases of severe white matter damage, were included in the study. Forty-two premature infants without white matter damage were selected as a control group. After birth, they were examined using aEEG and brain ultrasound once a week until four weeks after birth or a corrected gestational age of 32 weeks. The white matter damage and control groups were compared in terms of aEEG patterns and amplitudes and burst suppression ratio (BSR) on EEG.</p><p><b>RESULTS</b>The white matter damage and control groups had highly discontinuous patterns and had no complete sleep cycles. The lower amplitude was significantly smaller in the severe white matter damage subgroup than in the mild white matter damage subgroup and control group. There was alternating burst-suppression activity on the raw EEG in the white matter damage and control groups; and the severe white matter damage subgroup had a significantly longer suppression time and a significantly higher BSR on EEG compared with the mild white matter damage subgroup and control group.</p><p><b>CONCLUSIONS</b>Brain function monitoring should be performed in premature infants with white matter damage early after birth so as to detect cases of severe white matter damage in time.</p>

Measurement of brain regional oxygen saturation in neonates in China: a multicenter randomized clinical trial / 中华儿科杂志

<p><b>OBJECTIVES</b>To understand the value of measuring neonatal cerebral regional oxygen saturation (rSO2) using near infrared spectroscopy (NIRS) in assessing cerebral oxygenation, to establish the normal range of neonatal cerebral rSO2 and to collect data of the changes of cerebral rSO2 under certain disease status.</p><p><b>METHODS</b>Nine large hospitals participated in the multicenter randomized clinical trial from Jan 2007 to Apr 2008. Using the NIRS human tissue oximeter (TSAH-100) independently developed in China, the cerebral rSO2 of 223 normal full-term and 95 otherwise healthy preterm neonates without any special disease, was detected at 1, 2 and 3 days after birth, respectively. The cerebral rSO2 of 102 neonates with diseases which may affect the cerebral oxygenation, was also detected during the severe phases. The pulse oxygen saturation (SpO2) measured at the finger tip, and also the arterial oxygen saturation (SaO2) measured by blood gas analysis, which could indicate the oxygen supply of the whole body, were obtained simultaneously. The correlations among cerebral rSO2, pulse SpO2 and arterial SaO2 were analyzed.</p><p><b>RESULTS</b>(1) The cerebral rSO2 of the normal full-term neonates was (62+/-2)%. Cerebral hypoxia was defined as rSO2 lower than 58%. The cerebral rSO2 of the normal full-terms was steady at 1, 2 and 3 days after birth respectively, without any significant differences among them (F=0.610, P>0.05). The cerebral rSO2 of the neonates with diseases was (55+/-7)%, which was significantly lower than that of the normal full-term neonates (t=15.492, P<0.05). (2) The cerebral rSO2 was positively correlated with the SpO2 (r=0.74, P<0.01) and the SaO2 (r=0.71, P<0.01). (3) Under some special diseases, the changes of cerebral rSO2 was asynchronous with those of the SpO2: (1) For 18 cases under severe cerebral damages or under relatively low hemoglobin concentration, the cerebral rSO2 was significantly low (50%-58%), but the SpO2 was still normal (above 90%). (2) During the recovery of some critically ill neonates, the increase of cerebral rSO2 was lagged as compared with that of pulse SpO2. Especially, during the severe phases of 6 cases with multi-organ failure, the SpO2 and the cerebral rSO2 were both significantly low (55%-80% for SpO2, and 44%-50% for cerebral rSO2); when the diseases were alleviated, although the SpO2 recovered to above 85%, the cerebral rSO2 was still significantly low (around 50%). (3) In 3 cases, during the severe phases of serious hypoxic-ischemic encephalopathy (HIE), the cerebral rSO2 significantly increased to 70%-72%, which was significantly higher than the normal value (62%).</p><p><b>CONCLUSIONS</b>The range of cerebral rSO2 of the normal full-term neonates was (62+/-2)%. Cerebral oxygenation can be externally indicated by the rSO2 noninvasively and continuously measured by NIRS, which was positively correlated with traditional pulse SpO2 and arterial SaO2. In some special diseases, the rSO2 measured by NIRS can be helpful for clinical diagnoses and treatments.</p>

Multicenter follow-up report of 147 premature infants with brain injuries from 6 hospitals in China / 中国当代儿科杂志

<p><b>OBJECTIVE</b>Sponsored by the Subspecialty Group of Neonatology of Pediatric Society, China Medical Association, more than 10 large-scale hospitals participated in the near two-year multicenter investigation for Brain Injuries in Premature Infants in China. The present study presents the follow-up results of 147 premature infants with brain injuries from 6 Third Class A Level hospitals.</p><p><b>METHODS</b>All premature infants with intraventricular hemorrhage (IVH) or periventricular leukomalacia (PVL) diagnosed in the early neonatal period in the 6 hospitals were followed-up between January 2005 and August 2006. Based on the synthetic results of physical development, examination of nervous system, intelligence tests and cranial ultrasound, the premature infants with brain injuries were classified as normal development, marginal development and retarded development.</p><p><b>RESULTS</b>One hundred and forty-seven premature infants with brain injuries from the 6 hospitals consisted of 141 cases of IVH and 36 cases of PVL (30 cases having IVH and PVL). Based on the synthetic follow-up results, 51.4% of premature infants with brain injuries were generally assessed as normal development, 38.4% as marginal development and 10.7% as retarded development. Among them, delayed growth in head circumference, height and weight was 13.4%; the occurrence frequency of cerebral paralysis (CP) was 7.1% in PVL grade I, 28.6% in PVL grade II and 100% in PVL grade III; 12.7% showed retarded development of intelligence; and 30% presented post-injurious changes on cranial sonography.</p><p><b>CONCLUSIONS</b>The data of the multicenter follow-up can basically reflect the short-term prognosis of premature infants with brain injuries in major big cities of China. About 10% of them have retarded physical, motor-and mental developments. The long-term regular follow-up study is expected for more premature infants with brain injuries, and behavioral sequelae of brain injuries which may occur in peri-school age and adolescence should be paid particularly close attention.</p>

Effect of hyperbaric oxygenation on the differentiation of implanted human neural stem cells into neurons in vivo / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the effect of hyperbaric oxygenation (HBO) on the differentiation of the implanted human neural stem cells (hNSCs) into neurons in neonatal rats following hypoxic-ischemic brain damage (HIBD).</p><p><b>METHODS</b>HIBD model was prepared by ligation of the left common carotid artery, followed by 8% hypoxia exposure in 7-day-old Sprague-Dawley rat pups. Three days later, the rats received implantation of hNSCs into the left cerebral ventricles. Then the survived rats were randomly divided into two groups: transplantation alone and transplantation+HBO (n=8 each). HBO treatment was administered (1.8 ATA, 1 hr once daily for 10 days) in the transplantation+HBO group 1 hr after hNSCs transplantation. Brains were removed 10 days after transplantation. Frozen coronal sections were prepared for immunofluorescence analysis to detect the neural differentiation of the transplanted cells in the cerebral cortex and hippocampus.</p><p><b>RESULTS</b>Differentiated neurons of implanted cells distributed mainly in the cortex and the hippocampus of the injured side. There was no difference in the number of neurons in the cortex between the two groups, while the number of neurons in the hippocampus significantly increased in the transplantation+HBO group compared with that in the transplantation alone group (231.4+15.1 vs 162.6+5.6; P<0.05).</p><p><b>CONCLUSIONS</b>HBO treatment may promote the differentiation of implanted hNSCs into neurons in the hippocampus of neonatal rats following HIBD.</p>

Correlation of brain hypoxia at different degrees with brain function and brain damage investigated using near infrared spectroscopy / 中华儿科杂志

<p><b>OBJECTIVE</b>To study correlation of brain hypoxia of different degrees with brain function and damage.</p><p><b>METHODS</b>The brain regional oxygen saturation (rSO2) was determined by using a non-invasive near infrared spectroscopy (NIRS) technique in 15 piglets; the piglets were subjected to inhale 3% - 11% oxygen-nitrogen mixed gas through mechanical ventilation for 30 min. The piglets were divided into groups according to the level of brain rSO2 (i.e. < 30%, 30% - 35%, 35% - 40%, and 40% - 50%), and the data were compared with those of the control group (rSO2 > 60%). Changes of brain function were detected through amplitude and frequency of EEG waves and signal complexity. The piglets were sacrificed via decapitation 72 h after brain damage, and then histopathological and ultrastructural examinations were performed on cerebral cortex and hippocampal CA1 area.</p><p><b>RESULTS</b>In the group with rSO2 > 40%, the mean arterial pressure (MAP) after hypoxia was (56 +/- 0.00) mm Hg (1 mm Hg = 0.133 kPa), the blood lactic acid (LA) was (2.3 +/- 1.2) mmol/L, the EEG findings were within normal range, and there was no change in brain tissue ultrastructure. In the group with brain rSO2 = 30% approximately 40%, the MAP was (73 +/- 8) mm Hg, the LA was (8.2 +/- 3.9) mmol/L, the EEG waves showed decreased amplitude, frequency and complexity, but restored to some extent after hypoxia. The brain tissue ultrastructure showed damages to the cerebral cortex and neuron mitochondria at hippocampal CA1 area. In the group with brain rSO2 < 30%, the MAP was (35 +/- 0) mm Hg, the LA was (12 +/- 2) mmol/L, the EEG showed decreased amplitude, frequency, and complexity of signals compared with those of the normal control group, and was difficult to restore after hypoxia in some of the piglets; the brain tissue ultrastructure appeared to be similar to the changes seen with high-degree swollen cerebral cortex and neuron mitochondria at hippocampal CA1 area.</p><p><b>CONCLUSION</b>Different degrees of hypoxia had different influence on brain function and brain damage. The lower the brain rSO2, the more severe the damages to the brain and its function. The rSO2 of brain tissues detected with noninvasive NIRS can reflect brain injury and its severity during cerebral anoxia.</p>

Evaluation of early cognitive ability of infants born preterm by near-infrared spectroscopy / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To compare the differences in cerebral oxygenation responses between the infants born preterm and full-term infants and to evaluate the early cognitive ability of infants born preterm.</p><p><b>METHODS</b>Cerebral oxygenation after light stimulation was detected by near infrared spectroscopy (NIRS) in preterm infants at 3 or 6 months corrected gestational age (GA). The results were compared with those of age-matched infants born at term.</p><p><b>RESULTS</b>The start and peak response time of cerebral oxygenation occurring after light stimulation in preterm infants at 3 months corrected GA was 17.2 +/- 5.2 and 38.4 +/- 9.6 seconds respectively, which were significantly longer than in age-matched term infants (13.1 +/- 2.7 and 28.9 +/- 5.0 seconds respectively) (P < 0.05). The maximum response value of hemoglobin, oxyhemoglobin and regional oxygen saturation of the preterm infants at 3 months corrected GA was (1.2 +/- 0.5)%, (1.5 +/- 0.6)%, and (1.3 +/- 0.4)% respectively , which were significantly lower than that of the term infants [(2.3 +/- 0.3)%, (2.8 +/- 0.3)% and (2.4 +/- 0.5)% respectively] (P < 0.05). Cerebral oxygenation responses to light stimulation in preterm infants examined at 6 months corrected GA were not significantly different from age-matched term infants.</p><p><b>CONCLUSIONS</b>Cerebral oxygenation responses to light stimulation in infants born preterm at 3 months corrected GA are not as good as age-matched term infants, but were close to the level of age-matched term infants at 6 months corrected GA. This suggests that the early cognitive ability of preterm infants before 3 months corrected GA might fall behind age-matched term infants.</p>

Relationship between neonatal polycythemia and brain damage / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinical manifestations, imaging characteristics as well as prognosis of neonatal polycythemia complicated with brain damage.</p><p><b>METHODS</b>One hundred and sixteen in-patients with neonatal polycythemia admitted to our hospital during January 2003 to October 2005 were analyzed. Their clinical manifestations were observed. Craniocerebral ultrasonic examination (2D, 3D), CT and MRI were employed to dynamically observe the craniocerebral imaging variances as well as the cerebral hemodynamic variations and near infrared spectroscopy (NIR) was adopted to test the cerebral oxygenation. Twenty-two cases with moderate or severe disease were followed up for 3 to 12 months.</p><p><b>RESULTS</b>Out of the 116 polycythemic neonates, 53 cases had brain damages, of whom 31 had mild, 14 had moderate, and 8 had severe damages. Cranial imaging alterations were mostly ischemic injuries of various areas of different severity. The severity of brain damage was closely related to the duration of polycythemia, oxygen saturation of cerebral tissues as well as cerebral hemodynamic abnormalities. Brain injury was likely to occur in those whose polycythemia persisted for more than three days. The regional saturation of oxygen (rSO(2)) in mild brain injury cases was found to be 52.1%, while it was 47.1% in moderate and severe brain injury cases. Compared to the 58% as found in non-brain injury cases, the variance was found to be statistically significant (F = 104.466, P < 0.01). Among the cases with brain injury, cerebral hemodynamics displayed a slowdown in the blood flow velocity in the cerebral anterior artery and medium artery during the systolic phase and/or the diastolic phase. The abnormality ratio was closely related to the severity of brain injury. Through the chi(2) test the variance was proved to be statistically highly significant (chi(2) = 18.889, P < 0.01), however it was not correlated with the increase of the initial levels of hemoglobin (Hb) and hematocrit (HCT) (P > 0.05). During the follow up, neurological developmental abnormalities of various severity were found to exist in the cases with moderate (5/12) and severe disease (7/8), while cerebral palsy or epilepsy was not yet found.</p><p><b>CONCLUSION</b>Neonatal polycythemia might tend to bring about a reduction in the perfusion of cerebral blood flow and damaged cerebral oxygenation metabolism which in turn might lead to cerebral ischemic injury, which in some of the moderate and severe cases might lead to long-term neurological complications. Imaging investigations especially craniocerebral ultrasonic examination could be the practical means for the early diagnosis and evaluation of prognosis.</p>

Evaluation of relationship between early brain response and neurodevelopment in newborns by using near infrared spectroscopy / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the relationship between early brain response to extrinsic stimulation and neurodevelopment in preterm infants, assess the brain function of preterm infants in the early stage, and thereby to provide objective evidence for the degree of neurodevelopment in preterm infants and to evaluate prognosis.</p><p><b>METHODS</b>Using near infrared spectroscopy (NIRS), the brain response to sound stimulation of 90 preterm infants at different gestational age was observed and compared with the result obtained from 20 full term infants. The neonatal behavioral neurological assessment (NBNA) was performed at corrected age of 40 weeks, and the infants were followed up for 2 years. The effect of gestational age and brain damage on preterm infants, the relation between early brain response in preterm infants and their neurodevelopment was evaluated.</p><p><b>RESULTS</b>All the preterm infants responded to different degrees to auditory stimulation after birth. The time to beginning to react and the time to appearance of the peak reaction were attained after auditory stimulation and the time to beginning to resume when the auditory stimulation was stopped was (278 +/- 94) s, (446 +/- 67) s and (199 +/- 52) s, respectively, which were significantly longer than those observed in the full term infants (107 +/- 30) s, (264 +/- 51) s and (131 +/- 46) s, respectively. The maximum reactions of hemoglobin, oxyhemoglobin and regional oxygen saturation in the infants after gestational age 32 weeks was (0.3 +/- 0.3)%, (0.7 +/- 0.5)% and (0.3 +/- 0.3)%, respectively, which were significantly lower than those in the full term infants (1.7 +/- 0.7)%, (1.7 +/- 0.8)% and (1.6 +/- 0.7)%, respectively. When the brain response of preterm infants was compared with that in infants without brain damage, the speed of the reaction was slow, the maximum reaction was low. The brain response in preterm infant was correlated with NBNA at corrected age of 40 weeks. It was found during the following-up that abnormal neurodevelopment was associated with poor brain reaction.</p><p><b>CONCLUSIONS</b>NIRS can be used to evaluate brain response of infants. Preterm infants display brain response to auditory stimulation. Early brain response is correlated with neurodevelopment.</p>

Clinical Study on Infantile Congenital and Non-Congenital Cytomegalovirus Infection / 实用儿科临床杂志

Objective To learn the damage on infants and effect of treatment of congenital cytomegalovirus(CMV) infection and non-congenital cytomegalovirus infection which included perinatal infection and postnatal infection.Methods According to the diagnosis standard of cytomegalovirus infection in China,46 infants diagnosed CMV infection were retrospectively reviewed.In this research,CMV infection was diagnosed if serum CMV-IgM or CMV-pp65 in polymorphous leukocytes was positive.According to beginning time of CMV infection symptom,46 cases were divided into 2 groups: congenital infection and non-congenital infection,clinical features of 2 groups were compared.Ganciclovir was given to the infants with infantile hepatic syndrome of 2 groups,and compared liver transaminases and bilirulin with itself after treatment,side-effect was observed.Infants of 2 groups were followed up,and prognosis was compared.Results In 46 infants,21 were enrolled congenital infection group,25 were non-congenital infection group.CMV-IgM was positive in 38 cases of 46 patients,CMV-pp65 was positive in 19 cases of 43 patients,both CMVIgM and CMV-pp65 were positive in 11 cases.More congenital malformation were found in congenital group than non-congenital group,there was significant difference between 2 groups((P

Diagnosis and prognosis of neonatal cerebral infarction / 中华儿科杂志

<p><b>OBJECTIVE</b>To analyze the relationship between clinical characteristics and prognosis of neonatal cerebral infarction and to draw attention to the disease to improve the long-term outcome through early diagnosis and intervention.</p><p><b>METHODS</b>The clinical characteristics of 6 confirmed cases were summarized. Perinatal conditions and other factors were analyzed for possible causes of the disease. The survived patients were followed-up for 6-8 months.</p><p><b>RESULTS</b>The authors diagnosed 6 cases of neonatal cerebral infarction in one year, which accounted for 0.6% (6/969) of all the in-patients in the same time period. Among them 3 cases were confirmed as cerebrovascular malformations by magnetic resonance angiography (MRA), In 1 case the infarction was due to severe bilateral intraventricular hemorrhage, and in another case the disease was related to comprehensive factors such as prematurity, maternal pregnancy induced hypertension and respiratory failure secondary to bronchopulmonary dysplasia (BPD), and in 1 case the cause was undetermined. Four out of the 6 patients presented with varied forms of convulsions, which became the second leading cause for all the neonatal convulsive events (20%). None of the patients had localized neurological signs in the early course except for abnormal muscular tone of some extent. Cerebral ultrasound scanning in 5 out of 6 cases showed positive results. The diffusion-weighted magnetic resonance imaging (DW-MRI) was highly valuable for early confirmative diagnosis. Only one case was found normal within one year of follow-up and all the other 5 cases had unfavorable prognoses of varied severity.</p><p><b>CONCLUSION</b>Neonatal cerebral infarction is not a rare condition and should be considered as one of the important causes for neonatal convulsion. Imaging study is the main technique for diagnosis. The prognoses were poor for those cases for whom early diagnosis and treatment can not be made or those with widespread cerebral lesions.</p>

Incidence of Hypoglycemia in Infants of Diabetic Mothers and Its Relationship with Brain Injury / 实用儿科临床杂志

Objective To explore the relationship between the incidence of hypoglycemia in infants of diabetic mothers and brain injury.Methods The incidence of 86 infants of diabetic mothers combined with hypoglycemia as well as the relationship time of persistent hypoglycemia of infants were studied.And the association of the incidence and degree of brain injury with the time of persistent hypoglycemia,complication of other diseases and symptomatic hypoglycemia was also investigated.Results Seventy-five cases of temporary hypoglycemia(87.2%),and 11 cases of frequent hypolycemia(12.8%)were observed in the study.In the group of unsatisfactory maternal blood glucose control cases,the incidence of frequent hypoglycemia was 19.4%;in the group of satisfactory maternal blood glucose control cases,the incidence of frequent hypoglycemia was 8%.The overall incidence of the brain injury and the incidence of severe brain injury in the group of frequent hypoglycemic cases were higher than those in the group of temporary hypoglycemic cases.The incidence of brain injury in cases complicated with other diseases(77.4%) and in those with clinical symptoms(81.2%) were significantly higher than those in without other diseases(48.5%) and clinical symptoms(57.4%)(Pa

Case of Infant with Congenital Dermal Sinus Complicating Multiple Intramedullary Spinal Abscess / 实用儿科临床杂志

Objective To improve the recognition of intramedullary spinal abscess by a case of congenital dermal sinus with intramedullary spinal abscess and reduco the incidence of congenital dermal sinus with intramedullary spinal abscess.Methods Clinical,laboratory data and image of a confirmed case about one infant of congenital dermal sinus with multiple intramedullary spinal abscess were investigated,the related literature was reviewed.Results In this case,when the infant with congential dermal sinus had infection,he failed to gain antibiotic therapy, timely surgical treatment,his infection had diffused, and multiple intramedullary spinal abscess flared up.Conclusions Intramedullary spinal abscess is a rare disease.If treatment is delayed, the prognosis is poor and the mortality rate is high.MRI is the ideal investigation for diagnosis.Intramedullary spinal abscess can happen subsequent to congenital dermal sinus with infection, and cause neurological sequela. So an infant with congenital dermal sinus should be offered to avoid complication caused by infection.

Clinical Characteristics and Follow-up on 6 Cases of Newborn Incontin entia Pigmenti / 实用儿科临床杂志

Objective To explore the clinical features,diagnosi s and prognosis of incontinentia pigmenti.Methods Analyzing and summarizing the clinical characteristic, diagnosis and prognosis of neonatal incontinentia pigmenti in 6 neonatal infants that were hospita- lized in our department during the period from January 1 998 to December 2003 were studied,and some relevant literature were reviewed. Results 1.Three of 6 infants were male which was unusual;2.Four infants had typical skin lesions at birth and 1 case at 6 days old.Four cases had typical 3 stages o f skin lesions including the erythematous and vesicular inflammatory stage,verr ucous lesions and hyperkeratosis stage,macular hyperpigmentation stage,but the re was overlap;3.Four infants were complicated by central nervous system involv ement (two cases presented mental retardation,2 infants were temporary damage). Two cases were complicated by ocular manifestations ( one case had optical nerve atrophy and blind in left eye,the other had severe bilateral retinal lesions); 4.On specific examination 5 infants were diagnosed by skin biopsy.Gene analysis was made in 1 case,but we didn′t find the mutations of NEMO. Conclusions Incontinentia pigmenti is a rare X-linked dominant multisystem disease.It may be misdiagnosed in the initial stages.Except typical clinical features,skin biops y and gene analysis are main evidence for diagnosis.Early detection and interven tion are important for prognosis. J Appl Clin Pediatr,2005,20(2):123-125