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

OBJECTIVETo observe the effect of gastric dynamics by transcutaneous electrical acupoint stimulation (TEAS) combined general anesthesia when controlled hypotension dropped to 60% of the mean arterial prenssure (MAP) baseline, and to provide experimental evidence for organ protection in clinical controlled hypotension.

METHODSEighteen male beagles were randomly divided into three groups, the general anesthesia group (blank), the general anesthesia induced controlled hypotension group (control), and the general anesthesia combined TEAS induced controlled hypotension group (experiment), 6 in each group. Controlled hypotension was performed in the latter two groups with isoflurane inhalation and intravenous injection of sodium nitroprusside (SNP). The mean arterial pressure (MAP) was lowered to 60% of the MAP baseline and kept for 60 min. Controlled hypotension was not performed in Beagles of the control group. For Beagles in the experiment group, TEAS [2/100 Hz, (4 ± 1) mA] was applied to bilateral Hegu (LI4), Quchi (LI11), Zusanli (ST36), and Sanyinjiao (SP6) from stable physiological conditions to the end of maintaining stages. Changes of EGG frequencies and EGG amplitudes were monitored. Serum levels of gastrin (GAS) and motilin (MTL) were also detected at corresponding time points during and after experiment.

RESULTSAs for the pressure control effect of TEAS combined general anesthesia in the controlled hypotension, during the process of controlled hypotension (T1-T4), MAP levels of two controlled pressure groups remained relatively stable, and were kept at 60% of the MAP baseline. When the blood pressure dropped to the target low MAP and maintained at 60 min (T1-T4), EGG amplitudes of Beagles in all the three groups showed decreasing tendency. But it was more obviously lower than its basic level in the control group (P <0.05), while it was not obviously decreased in the experiment group (P < 0.05). EGG frequencies of Beagles in all the three groups showed no obvious change during this stage. By the end of the MAP rising stage (T8), the EGG amplitude of the experimental group was significantly higher than that of the control group and the blank group (P < 0.05), while it didn' t show any obvious increase in the control group. During this period, EGG frequencies of the two controlled hypotension groups decreased more than those of the blank group. Two h after rising blood pressure (at T9), EGG amplitudes and frequencies in the two controlled hypotension groups basically restored to their respective baselines and levels of the blank group at T9. At 2 h (T9) after controlled hypotension, serum levels of GAS and MTL were lower than those of basic levels in the two controlled hypotension groups (P <0.05). However, serum levels of GAS and MTL had an increasing trend in the two controlled hypotension groups at 24-72 h (T10-T12). Besides, the increasing speed and amplitude was better in experiment group than in the control group at T10-T12. However, there was no statistical difference between the two groups (P > 0.05). At 72 h (T12) serum levels of GAS and MTL had basically restored to their basic levels in the two controlled hypotension groups and that of the blank control group.

CONCLUSIONEGG amplitudes could be effectively improved in TEAS combined general anesthesia for controlled hypotension at 60% of the MAP baseline, the recovery of the serum GAS level accelerated, gastric power improved and stomach protected.

Acupuncture Points ; Anesthesia ; methods ; Anesthesia, General ; Animals ; Arteries ; Dogs ; Gastrins ; Hypotension, Controlled ; Male ; Motilin ; Nitroprusside ; Transcutaneous Electric Nerve Stimulation

At the present time, a kind of monitoring technology assuring highly effectual anesthesia is urgently required in the clinical practice. Electroencephalogram (EEG) assumes a dominant position in the current research of the depth detection of anesthesia. In this paper, the monitoring technique of the depth detection of anesthesia by bispectral index (BIS) is systematically showed. The bispectral index is a compound parameter which is composed of time domain, frequency domain, and high order spectral subparameters of the electroencephalograph. This nonlinear compound calculation method is worth research and is of great significance to the development of a new monitor of closed-loop control of anesthesia.
Anesthesia ; methods ; Electroencephalography ; methods ; Monitoring, Intraoperative ; methods

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.

Objective To explore the expression of c-Fos protein and character of mossy fiber sprouting(MFS) in hippocampus of rat with febrile seizures(FS).Methods Thirty-six 21-day-old male Sprague-Dawley rats were randomly divided into FS group,febrile control(FG) group and normal control(NG) group.FS was established by hyperthermal bath.Immune histochemistry and(Timm′s) staining were used to examine the expression of c-Fos protein in CA1 region and MFS in CA3 region of hippocampus.Results Excessive expression of c-Fos protein presented in the hippocampal CA1 region of FS group.The surface area percentage of c-Fos protein of FS group[(2.26?0.23)%] was higher than that of FG group[(1.08?0.19)%] and NG group[(0.71?0.14)%],there were significant difference between FS group and the other two groups(?~2=10.48 P

Objective To study the plasma folate concentrations in the third trimester of pregnant women and newborn babies so as to assess the association between them.Methods Pregnant women in Yuanshi and Laoting counties in Hebei province from May to June in 2009 were recruited with related information collected at enrollment.Those pregnant women being enrolled were followed up until delivery.Maternal blood was collected before delivery,and cord blood was collected after the expulsion of the placenta.Data from 437 pairs of women and newborns were analyzed.Plasma folate concentration was measured by Microbiological assay,with maternal plasma folate concentration ＜6.8 nmol/L defined as folate deficiency.Neonatal plasma folate concentration below 10％ was defined as relative deficiency.Student t-test and ANOVA were used to compare the plasma folate concentrations between the groups and x2 test was used to compare the situation of folate deficiency.In order to assess the association between maternal and newborn folate levels,logistic regression analysis was used to estimate the odds ratio of the neonatal plasma folate relative deficiency between the maternal folate deficient and normal groups after adjusting factors as age,BMI,region,career and education.Linear regression was used to test the trend by quintiles of maternal plasma folate concentration.Pearson' s test was used to test the relationship between the ratio of neonatal and maternal plasma folate level and the level of maternal plasma folate.Results The geometric mean of maternal plasma folate concentration was 8.0(95％CI:7.6-8.5) nmol/L and the deficiency was 29.3％,but in newborn babies,they were 24.0(95％CI:23.1-25.0) nmol/L and 0.9％ respectively.The plasma folate level in newborn babies was 3.0 times as high as in maternal (t=32.519,P＜0.01 )but the neonatal plasma folate deficiency status was higher than in matemal ( x2=137.2,P＜0.01 ).When compared with the normal plasma folate level group,the risk on neonatal plasma folate relative deficiency in the maternal folate deficiency group was significantly higher aiter adjusted for confounders (OR=1.96,95％CI:1.02-3.80).The neonatal plasma folate level significantly increased along with the maternal plasma folate level (Ptrend＜0.05).The ratio of neonatal and maternal plasma folate level was significantly inversely correlated with the maternal folate level (r=-0.810,P＜0.001 ).Conclusion Folate status in newborns was much better than in their mothers',in the northern rural areas of China.The maternal folate status was positively correlated with their offspring' s.Active placental transport for folate was significantly increasing when the maternal plasma folate level decreased.

Objective To investigate the intelligence standard for diagnose the sub-cretin children and children with mental retardation of socio-cultural type.Methods The full intelligence quotient(IQ),verbal intelligence quotient(VIQ)and performance intelligence quotient(PIQ)was tested by Wechsler scale(C-WISC)for mild iodine deficiency disordem children,children living in abnormal socio-cultural condition and normal children aged 7～14 years old in Qinba mountain area.The test results had been compared between the groups.Results There were no significant difference between psychomotor functioning well children and children living normal sociocuhural condition in VIQ,PIQ and full IQ(89.24±18.44 vs 90.75±17.58,87.58±15.78 vs 88.95±15.56,87.42±17.84 vs 89.02±17.18,t=1.14,1.19 and 1.24,respectively,all P＞O.05).PIQ and full IQ were significantly lower in mild iodine deficiency disorders children than in children with abnormal socio-cultural background (65.81±10.22 vs 72.33±13.23,62.42±12.31 vs 68.13±14.54,t=3.26,2.55,P＜0.01 or＜0.05,respectively).But the VIQ was not significantly different between these two groups.The average difference of VIQ and PIQ among mild iodine deficiency disorders children wag-0.32 without significant difierence(t=0.28,P＞0.05),however it was-2.91 among children under abnormal socio-cultural condition with significant difierenee(t=-3.59,P＜0.01).Conclusions IQ for iodine deficiency disorders children is characterized by that VIQ is damaged in parallel with PIQ,while that in children under abnormal soeio-cuhural condition is marked by that VIQ is retarded more severely than PIQ,which ean be used as an intelligence standard for differentiating the sub-cretin children from children wjth socio-cuhural mental retardation.

OBJECTIVETo study the clinicopathological features, immnophenotype and differential diagnosis of low-grade fibromyxoid sarcoma (LGFMS).

METHODSThe clinical, radiological and pathological features of 9 cases of LGFMS were reviewed.

RESULTSThe patients consisted of six males and three females with ages ranging from 11 to 65 years (mean 31.4 years). Clinically, most cases presented as slowly growing painless masses located in the extremities, trunk and neck. Two cases had a history of a rapid recent enlargement. Three cases presented with recurrent diseases after incomplete resection. Ultrasound showed heterogeneous signal. Precontrast CT and T1-weighted MRI showed a nodular mass of low to isodensity, while contrast-enhanced CT and T2-weighted MRI demonstrated contrasting zonal areas of hypo/hyperintensity. The tumors measured 3 to 16 cm (mean 7.7 cm), with a fibrous to myxoid appearance on cut surface. Histologically, they were composed of alternating collagenous and myxoid areas. A transition between the two areas could be identified. Giant rosette-like structures were observed in 3 cases. The center of the giant rosettes was composed of eosinophilic collagen, which showed centrifugal in arrangement. There was also gradual transition between the giant rosettes and the fibromyxoid component. On high magnification, the tumor was composed of ovoid to spindle-shaped cells with hyperchromatic nuclei. Nuclear atypia was inconspicuous. The tumor cells were arranged mostly in interlacing fascicles or whorls. Vessels were not prominent and necrosis was absent. Immunohistochemically, the tumor cells showed unique staining for vimentin, consistent with a fibroblastic differentiation.

CONCLUSIONSLGFMS is a distinctive low grade fibroblastic sarcoma of young adults. Recognizing the characteristics of the rare entity may help to avoid misdiagnosis. Wide local excision is recommended to avoid local recurrences.

Adolescent ; Adult ; Aged ; Child ; Diagnosis, Differential ; Extremities ; pathology ; Female ; Fibrosarcoma ; diagnosis ; metabolism ; pathology ; surgery ; Head and Neck Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Myxosarcoma ; pathology ; Soft Tissue Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo investigate the intervention effect of transcutaneous electrical acupoint sitmulation (TEAS) on heart under limiting level of controlled hypotension.

METHODSEighteen male Beagles were randomly divided into a general anesthesia group, a control group and an experimental group, 6 dogs in each group. All animals were applied with general anesthesia, and the animals of the control group and the experimental group were administered with combination of isoflurane and sodium nitroprusside (SNP) for controlled hypotension to 30% mean arterial pressure (30% MAP) of base line for 60 min. In the experimental group, TEAS (2 Hz/100 Hz, 6-8 mA) was applied to "Hegu" (LI4), "Zusanli" (ST 36), "Sanyinjiao" (SP 6) and "Quchi" (LI 11) from the beginning of physiological conditions stability to the end of maintained low MAP for 60 min. Creatine kinase isoenzymes MB (CK-MB) and lactate dehydrogenase (LDH) activity were detected at the base line and 2 h, 24 h, 48 h, 72 h after operation. Total superoxide dismutase (T-SOD) and malondialdehyde (MDA) in myocardium were observed at 72 h after operation.

RESULTSThe peak of LDH arrived at 24 h after operation and then restored in 3 groups. Compared with basal level, the activity of LDH in controlled group at 72 h after operation was significantly increased (P < 0.05), and in the other two groups no obvious change (P > 0.05). The activity of CK-MB arrived to peak from 2 h to 24 h after operation, and then came back gradually in 3 groups. Compared with postoperative 72 h of the same group, the control group at 48 h significantly increased (P < 0.05), while the other two groups had no statistically significant difference (P > 0.05). The content of MDA at 72 h after operation in controlled group was higher significantly than that in the general anesthesia group and the experimental group (both P < 0.05).

CONCLUSIONTEAS combined with anesthesia for controlled hypotension can elevate myocardial effect of anti-oxide free radical, reduce the postoperative release of LDH and CK-MB, so as to produce myocardial protection.

Acupuncture Points ; Anesthesia, General ; Animals ; Apoptosis ; Dogs ; Heart ; physiopathology ; Humans ; Hypotension, Controlled ; Male ; Malondialdehyde ; metabolism ; Myocardium ; cytology ; metabolism ; Oxidative Stress ; Transcutaneous Electric Nerve Stimulation

OBJECTIVETo study into the genetic polymorphism of DC-SIGN and DC-SIGNR's exon 4 in Chinese hepatitis C patients and its relationship with HCV infection susceptibility.

METHODSPatients with hepatitis C (n=300, group A) and healthy subjects (n=520, group B) were genotyped and analysed for the repeat sequence of polymorphism of DC-SIGN and DC-SIGNR's exon 4 using PCR and DNA sequencing.

RESULTSThe distribution of genotypes and alleles in DC-SIGN's exon 4 in the two groups did not differ significantly (P > 0.05). The difference of allele frequency in DC-SIGNR's exon 4 between the two groups was also not significant (P > 0.05). However, 9/5 genotype distribution frequency of DC-SIGNR's exon 4 in patients with hepatitis C was significantly higher than that in the healthy subjects (P < 0.05).

CONCLUSIONThere is no significant correlation between the genetic polymorphism of DC-SIGN's exon 4 and HCV infection susceptibility. 9/5 genotype distribution frequency of DC-SIGNR's exon 4 in patients with hepatitis C is significantly higher and may be associated with HCV infection susceptibility.

Adolescent ; Adult ; Aged ; Asian Continental Ancestry Group ; genetics ; Blood Donors ; Case-Control Studies ; Cell Adhesion Molecules ; genetics ; Child ; Exons ; Female ; Genetic Predisposition to Disease ; Genotype ; Hepatitis C, Chronic ; ethnology ; genetics ; Humans ; Lectins, C-Type ; genetics ; Male ; Middle Aged ; Polymorphism, Genetic ; Receptors, Cell Surface ; genetics ; Young Adult