Lateral septum adenosine A2A receptors control stress-induced depressive-like behaviors via signal-ing to hypothalamus and habenula / 中国药理学与毒理学杂志

Depressive disorder ranks as a major bur-den of disease worldwide,yet the current antidepressant medications are limited by frequent non-responsiveness and significant side effects.The lateral septum(LS)is thought to control of depression,however,the cellular and circuit substrates are largely unknown.Here,we identified a subpopulation of LS GABAergic adenosine A2A receptors(A2AR)-positive neurons mediating depres-sive symptoms via direct projects to the lateral habenula(LHb)and the dorsomedial hypothalamus(DMH).Activa-tion of A2AR in the LS augmented the spiking frequency of A2AR-positive neurons leading to a decreased activation of surrounding neurons and the bi-directional manipula-tion of LS-A2AR activity demonstrated that LS-A2ARs are necessary and sufficient to trigger depressive pheno-types.Thus,the optogenetic modulation(stimulation or inhibition)of LS-A2AR-positive neuronal activity or LS-A2AR-positive neurons projection terminals to the LHb or DMH,phenocopied depressive behaviors.Moreover,A2AR are upregulated in the LS in two male mouse mod-els of repeated stress-induced depression.This identifica-tion that aberrantly increased A2AR signaling in the LS is a critical upstream regulator of repeated stress-induced depressive-like behaviors provides a neurophysiological and circuit-based justification of the antidepressant poten-tial of A2AR antagonists,prompting their clinical transla-tion.

Analysis of the expression levels of microRNA-205 and microRNA -24-3p in cervical cancer tissues and their correlation with survival prognosis / 中国医师进修杂志

Objective:To analyze the expression of microRNA(miR)-205 and miR-24-3p in cervical cancer tissues and their correlation with survival prognosis.Methods:A total of 82 patients with cervical cancer(cervical cancer group), 60 patients with uterine fibroids(uterine fibroids group) and 40 healthy women (healthy control group) treated in Hubei Hospital of Integrated Traditional Chinese and Western Medicine from March 2014 to March 2015 were enrolled. The expression levels of miR-205 and miR-24-3p in the tissues of the three groups were compared. The receiver operative characteristic (ROC) curve was used to analyze the diagnostic value and zero threshold of the two indicators for cervical cancer. Analyzed the 5-year survival status of cervical cancer patients at the upper and lower thresholds, and then further discuss the risk factors that affected the prognosis of cervical cancer.Results:The relative expression levels of miR-205 and miR-24-3p in the cervical cancer group were significantly higher than those in the uterine fibroids group and the healthy control group ( P<0.01). ROC curve analysis showed that the cut-off value was 1.13 and 1.35, the area under the curve was the biggest, and the sensitivity in diagnosis of cervical cancer were 83.87%, 84.38%, the specificity were 75.00%, 82.42%.The survival rate analysis based on these results showed that the 5-year survival rate of patients with miR-205≥1.13 was 69.05%, which was significantly lower than patients with miR-205<1.13 (87.50%) ( P<0.05); the 5-year survival rate of patients with miR-24-3p≥1.35 was 68.89%, which was significantly lower than that of patients with miR-24-3p<1.35 (89.19%)( P<0.05). The results of the Cox regression model showed that moderately and highly differentiated, FIGO stage Ⅱ, depth of invasion ≥1/2, combined lymph node metastasis, and high levels of miR-205 and miR-24-3p were risk factors affecting the prognosis of cervical cancer patients ( P<0.05). Conclusions:Serum miR-205 and miR-24-3p are at high levels in cervical cancer tissues, and are closely related to the prognosis of patients. They are expected to become auxiliary diagnosis and prognostic indicators for cervical cancer.

The effects of "rain classroom" wisdom teaching on nursing students′ self-learning and self-efficacy / 中国实用护理杂志

Objective:To explore the effects of "rain classroom" wisdom teaching on geriatric students′ autonomous learning ability and self-efficacy.Methods:From June to August, 2021, 61 nursing students who practiced in the department of gerontology of the Second Xiangya Hospital, Central South University were selected as research subjects, and divided into experimental group (30 students) and control group (31 students) by random number table method. In the experimental group, wisdom teaching based on "Rain classroom" was carried out at the same time as conventional teaching, while in the control group, conventional teaching was carried out, and the teaching effect was evaluated by the independent learning ability measurement scale and academic self-efficacy scale of nursing students.Results:The score of autonomous learning ability of nursing students in the experimental group was 68.55 ± 11.06, and that in the control group was 74.29 ± 9.16. The difference between the two groups was statistically significant ( t = -2.01, P<0.05). The academic self-efficacy scores of nursing students in experimental group and control group were 52.27 ± 11.56 and 52.64 ± 9.28 respectively. There was no significant difference between the two groups ( P>0.05). Conclusions:The wisdom teaching of "rain classroom" improves the independent learning ability of geriatric nursing students, and attaches importance to the cultivation of self-efficacy of nursing students, and the improvement of self-efficacy needs further research.

Recurrence factors for myelin oligodendrocyte glycoprotein antibody disease in children and the effect of recurrence prevention regimens / 中国当代儿科杂志

OBJECTIVE@#To study the clinical features and recurrence factors of myelin oligodendrocyte glycoprotein (MOG) antibody disease in children and the effect of recurrence prevention regimens.@*METHODS@#A retrospective analysis was performed on the medical data of 41 children with MOG antibody disease who were hospitalized in the Department of Pediatric Neurology, Xiangya Hospital of Central South University, from December 2014 to September 2020. According to the presence or absence of recurrence, they were divided into a monophasic course group (@*RESULTS@#For these 41 children, acute disseminated encephalomyelitis was the most common initial manifestation and was observed in 23 children (56%). Of the 41 children, 22 (54%) experienced recurrence, with 57 recurrence events in total, among which optic neuritis was the most common event (17/57, 30%). The proportion of children in the recurrence group who were treated with corticosteroids for less than 3 months in the acute phase was higher than that in the monophasic course group (64% @*CONCLUSIONS@#More than half of the children with MOG antibody disease may experience recurrence. Most children with recurrence are treated with corticosteroids for less than 3 months in the acute phase. Rituximab and azathioprine may reduce the risk of recurrence.

A school-aged boy with nephrotic syndrome with cough for one month and shortness of breath for half a month / 中国当代儿科杂志

A boy, aged 6 years and 11 months, was admitted due to nephrotic syndrome for 2 years, cough for 1 month, and shortness of breath for 15 days. The boy had a history of treatment with hormone and immunosuppressant. Chest CT after the onset of cough and shortness of breath showed diffuse ground-glass opacities in both lungs. Serum (1, 3)-beta-D glucan was tested positive, and the nucleic acid of cytomegalovirus was detected in respiratory secretions. After the anti-fungal and anti-viral treatment, the child improved temporarily but worsened again within a short period of time.

Mental health status of medical staff in the epidemic period of coronavirus disease 2019 / 中南大学学报(医学版)

OBJECTIVES@#To explore the psychological status of medical staff in the epidemic period of coronavirus disease 2019 (COVID-19), and to analyze its influential factors.@*METHODS@#A total of 373 medical staff from Xiangya Hospital and the Second Xiangya Hospital of Central South University were enrolled for this study. The General Sociological Data Questionnaire, Symptom Check-List 90 (SCL-90), and self-designed public opinion response questionnaire were used to assess general sociological data, mental health scores, and ability to respond to COVID-19 related public opinion information of medical staff. The mental health scores of medical staff with different general sociological data and public opinion information coping abilities were compared. Influential factors of mental health were analyzed.@*RESULTS@#The average score of 10 factors in SCL-90 of 373 medical staff was less than 2 points. 14.21% medical staff had one or more factor scores more than two points, including 11.26% with terror symptoms, 7.77% with compulsive symptoms, and 5.63% with anxiety. The main sources of COVID-19 information for medical staff included WeChat, microblog, Jinri toutiao, TV and radio. 66.22% medical staff regularly verified information about COVID-19 through official websites or formal channels. A great deal of COVID-19 information in WeChat could make medical staff nervous (34.05%), anxious (30.29%), and insecure (29.22%). 68.63% medical staff sometimes were worried about getting infected because they knew information about COVID-19. Different departments of medical staff, getting cough or having a fever recently, and the degree of fear of infection had an impact on the SCL-90 score of medical staff, the differences were all statistically significant (all <0.05). Stepwise regression analysis showed that the impact of COVID-19 information on their life in WeChat, getting cough or having a fever recently, insomnia-early caused by COVID-19 information in WeChat, different departments, and the degree of fear of infection COVID-19 were the influential factors for the mental health of medical staff (all <0.05).@*CONCLUSIONS@#During the epidemic of COVID-19, medical staff suffered from psychological problems to various degrees. It is necessary to establish a psychological assistance platform and guide the direction of public opinion correctly to promote the mental health of medical staff.

The comparison of hypertension prevalence among children based on different references of hypertension / 中华疾病控制杂志

Objective To compare the differences in the hypertension prevalence among children aged 7-12 in Guangzhou based on different references of hypertension. Methods A total of 7698 children aged 7-12 years old in Guangzhou were recruited by stratified cluster random sampling method. Demographic information such as gender and age was collected by questionnaire. Children’s height, weight, and blood pressure were objectively measured. There were five references for diagnosing children’s hypertension: Mi 2010, Mi 2017, Ma 2017, America 2004, and America 2017 reference. Results Based on the references above, the prevalence of hypertension for children aged 7-12 in Guangzhou reached a very high level. The prevalence of hypertension, high systolic blood pressure (SBP) and high diastolic blood pressure (DBP) based on Mi 2017 reference were all higher than those based on Mi 2010 reference, but both of them were distinctly higher than Ma 2017 reference. Compared with Mi 2010 reference, the agreement of diagnosis of high SBP and high DBP were both higher for Mi 2017 than those for Ma 2017. The agreement of high SBP was higher between Mi 2017 and America 2017 than that between Ma 2017 and America 2017 (Kappa: 0.846 vs. 0.727). Conclusion The prevalence of hypertension in children aged 7-12 in Guangzhou reachs a very high level. The prevalence of hypertension based on Mi 2017 reference is the highest and follows with America 2017 reference, and the agreement between them is excellent. Compared with Mi 2010 or America 2017 reference, the agreement for Ma 2017 is lower than that for Mi 2017 reference, respectively.

Effect of AMPK/mTOR/S6K1 pathways and the insulin-sensitizing effect for adiponectin in endometrial cancer cells / 中华妇产科杂志

Objective@#To explore effect of AMP-activated protein kinase (AMPK) /mammalian target of rapamycin (mTOR) /ribosomal protein S6 kinase-1 (S6K1) signaling pathways and the insulin-sensitizing effect by adiponectin in endometrial cancer HEC-1B cells.@*Methods@#The experiments were divided into 4 groups, adiponectin (Ad) group (HEC-1B cells treated with 20 μg/ml adiponectin for 30 minutes) , inhibitor group (HEC-1B cells treated with 10 μmol/L compound C for 30 minutes) , inhibitor+ Ad group (HEC-1B cells treated with 10 μmol/L compound C for 30 minutes following incubation with 20 μg/ml adiponectin for 30 minutes) , control group (only added the culture medium without serum DMEM) . (1) Real-time quantitative PCR and western blot analysis were used to detect the level of mRNA and protein of adiponectin receptor (AdipoR) 1 and AdipoR2. (2) Western blot analysis were used to detect phosphorylation of AMPK, mTOR, S6K1 or insulin receptor substrate 1 (IRS1) protein expression with stimulation in different concentrations of adiponectin (2.5, 5, 10, and 20 μg/ml) , or following incubation with insulin 50 nmol/L for 5 minutes; or treated with 20 μg/ml adiponectin for different times (15, 30, 45, and 60 minutes) , or following incubation with insulin 50 nmol/L for 5 minutes. (3) Cell counting kit-8 (CCK-8) assay was performed to investigate the cell proliferation, and transwell chamber assay was used to detect the cell migration in different groups.@*Results@#(1) The relative expression level of AdipoR1 mRNA and protein were higher than AdipoR2 in HEC-1B cell (8.50±0.09 to 1.00±0.00, and 0.91±0.03 to 0.69±0.03; P<0.05) . (2) The phosphorylation level of p-AMPK was significantly induced, and the phosphorylation level of p-mTOR and p-S6K1 proteins, and 20 μg/ml adiponectin at 30 minutes, AMPK protein had the highest level of activation. (3) Adiponectin induces increased tyrosine phosphorylation of IRS1 in a time-and concentration-dependent manner. (4) The proliferation inhibition ratio in Ad group (0.68±0.34) % was much more than that in inhibitor+Ad group (0.24±0.04) % (t=17.88, P<0.05) . The number of cell migration in Ad group (77±8) was much more than that in inhibitor+Ad group (132±13; t=-7.34, P<0.05) .@*Conclusions@#Adiponectin maybe inhibit proliferation and migration of endometrial cancer cells through AMPK/mTOR/S6K1 signal pathway. Adiponectin insensitizes insulin signaling may by regulating by the AMPK/S6K1/IRS1 pathway.

Da Vinci robot assistance surgical coordination for the resection of azygous vein aneurysm: a case report / 中国实用护理杂志

Objective Summary of a patient who was diagnosed as an azygous vein aneurysm during Da Vinci robotic surgery,which continue to complete surgical resection of varicose veins by Da Vinci robot assistance. Methods After the patient is diagnosed as the azygos vein aneurysm during the operation, the emergency plan is started immediately, and the nursing cooperation is determined according to the operative coordination of the main problems during the operation. Results With the close cooperation of the medical team, an azygous vein aneurysm with a size of approximately 5 cm×4 cm×3 cm was successfully removed with the Da Vinci robot assistance, no complications occurred after surgery, and good recovery occurred, the patient was discharged on the fourth postoperative day. Conclusions It is essential to make corresponding emergency plan and exercise for Da Vinci robot operation , and to start contingency plan immediately when needed, which is an important guarantee to continue to complete misdiagnosis surgery under the aid of Da Vinci robot.

Diagnosis and treatment of 3-hydroxyisobutyryl-CoA hydrolase deficiency: a case report and literature review / 中国当代儿科杂志

A case of 3-hydroxyisobutyryl-CoA hydrolase deficiency was reported, and its clinical features, gene mutation characteristics, and diagnosis and treatment were analyzed with reference to related literature. The patient aged 1 year and 6 months had developmental regression and paroxysmal dystonia after pyrexia and diarrhea, and head MRI showed symmetrical lesions in the bilateral basal ganglia. No pathogenic mutation was found in the full-length detection of mitochondrial genome. Nuclear gene detection of mitochondrial-related diseases found new compound heterozygous mutations in the HIBCH gene, i.e., c.439-2A>G and c.958A>G (p.K320E), which were inherited from his father and mother, respectively. The boy was given cocktail therapy, dietary valine restriction, and symptomatic treatment. After 2 weeks of treatment, there were improvements in dystonia and motor and intellectual development.

Fever, generalized pain, and multiple pulmonary nodules in a school-aged boy / 中国当代儿科杂志

A 9-year-old boy was admitted to Xiangya Hospital due to pain after trauma in the left lower limb for 5 days and fever with generalized pain for 2 days. The results of X-ray of the left lower limb were normal. Pulmonary computed tomography (CT) showed multiple pulmonary nodules in both lungs. Adrenal CT showed marked enlargement of the left adrenal gland. The patient also experienced generalized herpes and intermittent delirium and had a blood pressure up to 155/93 mm Hg. He was transferred to our hospital with a suspected diagnosis of pheochromocytoma. On admission, the patient had a blood pressure of 86/44 mm Hg, sporadic maculopapule and herpes, touch-evoked pain, exposure of superficial veins, white pus coating on the right side of the tongue, and tension in the abdominal muscle. No skin damage was observed in the left lower limb, and the patient was forced to be in the extending position and experienced significant swelling below the knees. Laboratory examination showed a reduction in platelet count, hypoproteinemia, a significant increase in creatase, a C-reactive protein level of 348 mg/L, and a procalcitonin level of >100 ng/mL. Thoracoabdominal and pelvic CT showed multiple patchy and nodular lesions in both lungs, which had an undetermined nature, as well as an enlarged spleen. The tests of puncture fluid from the left knee joint and the periosteum of the left tibia, blood culture, and bone marrow culture all showed methicillin-resistant Staphylococcus aureus. The patient was given anti-shock treatment, anti-infective therapy with vancomycin, debridement and continuous irrigation/drainage of osteomyelitis lesions in the left tibia, but the patient still experienced recurrent shivering and severe fever and increased subcutaneous and pulmonary nodules. Linezolid was added on day 8 after admission, and the patient's body temperature returned to normal on day 24 after admission. Subcutaneous and pulmonary nodules were gradually reduced and disappeared. The patient was treated for 2 months and then evaluated as cured.

A New Hasubanan Alkaloid from Stephania hernandifolia / 中草药·英文版

Objective: To study the chemical constituents of Stephania hernandifolia. Methods: Various chromatographic techniques were used to isolate the constituents, and the structures were elucidated on the basis of HR-EI-MS, 1D-NMR and 2D-NMR spectral analyses. Results: A hasubanan alkaloid, hernsubanine D (1) was isolated from the whole plants of S. hernandifolia. The compound was screened for the cytotoxic activity against two human cancer cell lines in vitro. Conclusion: Compound 1 is a new compound without cytotoxicity against A549 and K562 cells. © 2014 Tianjin Press of Chinese Herbal Medicines.

Effect of different dentin cleaning agents on the bond strength of self-adhesive resin cement to dentin / 华西口腔医学杂志

<p><b>OBJECTIVE</b>This study aims to evaluate the bond strength of a self-adhesive resin cement to dentin by ethylene diamine tetraacetic acid (EDTA) and NaClO.</p><p><b>METHODS</b>Twenty-seven freshly extracted non-carious human premolars were prepared to expose the buccal dentin and randomly divided into three groups: control group (A group), EDTA group (B group) and NaClO group (C group). All teeth were bonded to dentin using a self-adhesive resin cement after the teeth in the A group were processed with distilled water. The B and C group were processed with 3%EDTA and 1%NaClO, respectively. After 24 hours at 37 °C water, the shear bond strengths of the twenty-four specimens were measured. All statistical analysis was performed using SPSS 17.0 software package. Each fractured specimen was examined under dental microscope. Three new specimens were cut, and the morphologies of the cement-dentin interface were observed under scanning electron microscope (SEM).</p><p><b>RESULTS</b>The shear bond strength in the A group, B group and C group was (8.55±0.63), (8.47±0.56) and (12.97± 0.59) MPa, respectively. The difference between A group and B group was no statistically significant (P>0.05), whereas the difference between C group and B group (or A group) was statistically significant (P<0.05). SEM observation of the cement-dentin interface in the C group showed good adaptation, but resin tags were not observed. The other two groups showed poor bonding interface. Most of the fractured adhesive dentin surfaces exhibited cohesive failure in the A group and B group. All the fractured adhesive dentin surfaces exhibited cohesive failure in the C group.</p><p><b>CONCLUSION</b>1% NaClO can increase the bond strength of self-adhesive resin cement to dentin, but 3%EDTA has no effect.</p>

A complicated case study: Hennekam syndrome / 中国当代儿科杂志

Hennekam syndrome (HS) is a rare autosomal recessive syndrome characterized by defective lymphatic development. A 34-month-old boy with HS and who had unexplained developmental retardation and hypoalbuminemia as main clinical manifestations is reported here. He had a history of generalized edema and poor feeding. He was not thriving well. He manifested as facial anomalies (hypertelorism, flat nasal bridge and flat face), fracture of teeth, and superficial lymph nodes enlargement. He had low serum total protein, low serum albumin, and low serum immunoglobulin levels. Duodenal bulb biopsy revealed lymphangiectasia. Color Doppler ultrasound, magnetic resonance imaging and CT scan showed multi-site lymphangioma, and HS was thus confirmed. Mutations in CCBE1 and FAT4 have been found responsible for the syndrome in a part of patients. Diagnosis of the disease depends on the familial history, clinical signs, pathological findings and genetic tests.

A new bufadienolide with cytotoxic activity from the Chinese traditional drug Ch'an Su / 中国天然药物

AIM@#To study the bufadienolides in the Chinese traditional drug "Ch'an Su" and their cytotoxic activity.@*METHOD@#Various chromatographic techniques were used to isolate the constituents, and their structures were elucidated through physical and spectroscopic data.@*RESULTS@#Twenty compounds were isolated, and eighteen were evaluated in vitro for their cytotoxic activity against A-549 and K-562 cells.@*CONCLUSION@#Compound 1 (bufalin 3β-acrylic ester) was a new bufadienolide and exhibited the most potent activity against the two tumor cell lines with IC50 values of 7.16 and 6.83 nmol · L(-1). The relationships between structure and activity are discussed.

Hernsubanine E, a new hasubanan alkaloid from Stephania hernandifolia / 中国中药杂志

A new hasubanan alkaloid, hernsubanine E (1), as well as two known compounds p-hydroxybenzaldehyde (2) and (-)-syringaresinol (3) have been isolated from the whole plants of Stephania hernandifolia by various column chromatographic methods. Their structures were identified by physicochemical properties and spectral analyses. Compounds 2 and 3 were isolated from the genus of Stephania for the first time.

Social support and negative emotion in parents of children with congenital heart disease before operation / 中南大学学报(医学版)

Objective:To explore the relationship between social support and negative emotion in parents of children with congenital heart disease before the operation. Methods:Social support rating scale (SSRS), self-rating anxiety scale (SAS), and Center for epidemiological survey-depression scale (CES-D) were used to assess the association between social support and anxiety and depression in 226 parents. Results:The mean SSRS score was signiifcantly lower than the normal. There was signiifcant difference in social support of parents in monthly family earning and knowledge about surgery. Parents with monthly family income≤1000 yuan had lower scores than the other groups. Parents who knew nothing about the surgery approach had lower scores. Social support was significantly correlated to negative emotion (anxiety and depression). Social support could predict anxiety and depression. Conclusion:Social support is widely and significantly correlated to negative emotion in parents of children with congenital heart disease before the surgery.

Video-assisted thoracoscopic sympathictomy plus bypass fiber resection for patients with primary palmar hyperhidrosis / 中南大学学报(医学版)

OBJECTIVE@#To evaluate the efficiency of video-assisted thoracoscopic surgery for primary palm hyperhidrosis by T4 sympathicotomy plus bypass fiber resection, and to describe our experience regarding the prevention of related postoperative complications.@*METHODS@#Clinical data for 32 cases of primary palmar hyperhidrosis in patients who underwent bilateral video-assisted thoracoscopic sympathictomy plus bypass fiber resection from October 2008 to June 2011 were analyzed retrospectively.@*RESULTS@#The operation was performed successfully on all patients, and their palmar hyperhidrosis was completely alleviated after operation. No severe, surgery-related, postoperative complications occurred.@*CONCLUSION@#Video-assisted thoracoscopic sympathictomy is an effective, safe and minimally invasive procedure for primary palmar hyperhidrosis.

Experimental study of local administration of silver nanoparticles for the treatment of pyogenic spondylitis / 中南大学学报(医学版)

OBJECTIVE@#To establish a proper animal model of pyogenic spondylitis, to evaluate the efficacy of silver nanoparticles for treatment of spinal pyogenic infections, and to explore the distribution of the particles in the body of the animals.@*METHODS@#Staphylococcus aureus was inoculated into intervertebral discs of rabbits to establish a spinal pyogenic infection model. These rabbits were divided into Group A [0.1 mg/(kg.d)], Group B [1 mg/(kg.d)], and a Control group. Groups A and B were injected with different doses of silver nanoparticles solution daily at locally infected side. Two weeks later, bacterial cultures, radiographic outcomes, histopathology were analyzed. Atomic absorption spectrometry was utilized to measure silver contents in some vital organs of the rabbits to detect the distribution and accumulation of silver particles.@*RESULTS@#Staphylococcus aureus (100 CFU/mL), induced 100% pyogenic spondylitis. 1 mg/kg dose of silver nanoparticles solution could effectively inhibit the occurrence of spinal pyogenic infection with the effective rate of 75%. While the effect of 0.1 mg/kg dose of silver nanoparticles solution was less obvious, the efficiency was only 25%. Significant numbers of silver nanoparticles were observed to accumulate in the animal. In the 1 mg/kg group silver was deposited mainly in the spinal cord, liver, kidneys, spleen, and testicles, while in the 0.1 mg/kg group it accumulated only in the spinal cord.@*CONCLUSION@#Local administration of silver nanoparticles can effectively prevent and treat pyogenic spondylitis; the particles accumulate in the body commensurate with the administered drug concentration.

Succinic semialdehyde dehydrogenase deficiency / 中国当代儿科杂志

Succinic semialdehyde dehydrogenase (SSADH) deficiency is a rare autosomal recessive disorder. This paper reports three cases of SSADH deficiency in infants. The infants developed the symptoms including developmental delay, intellectual disability, hypotonia, hyporeflexia and seizures. The electroencephalogram (EEG) showed background slowing and focal spike discharges in all of 3 patients. Head magnetic resonance imaging (MRI) demonstrated abnormalities in 2 patients, including basal ganglia damage and increased T2-weighted signal in bilateral cerebral peduncles. Urinary organic acid analysis with gas chromatography-mass spectrometry (GC-MS) revealed increased levels of 4-hydroxybutyrate (GHB) in 3 patients. SSADH deficiency was definitely diagnosed based on the clinical manifestations and the results of urinary organic acid analysis in the 3 children. It was concluded that early urine organic acid analysis is essential for children presenting with mental retardation, neuropsychiatric disturbance or epilepsy of unknown etiology.