It is a challenge in diagnosis of hemophagocytic lymphohistocytosis (HLH).Rapid diagnosis is the key to make decisions in clinical management of HLH.The timely and reliable diagnosis of HLH can be assisted by laboratory tests including nature killer (NK) cell activity, sCD25, hemophagocytosis, ferritin, surface CD107a, and genetic testing.Here is introduced lab assays and evaluate the clinical applications in HLH.The aim is to raise awareness of the values of laboratory evaluations in diagnosing HLH.

Objective To explore the relationship between the ischemic stroke subtypes and its risk factors and leukoaraiosis(LA).Methods The severity of LA was classified into LA1,LA2 and LA3 in 213 patients with ischemic stroke combined LA.The relationship between LA degree and the ischemic stroke subtypes(transient ischemic attack,lacunar cerebral infarction,atherothrombotic and cardioembolic cerebral infarction) and its risk factors(age,sex,hypertension,diabetes and coronary artery disease,etc.)were analyzed.Results In the 213 patients with ischemic stroke combined LA,the age of the cases with LA2 and LA3 were significantly higher than the cases with LA1(all P

Methylmalonic acidemia(MMA) is one of the most common diseases of autosomal recessive inherited organic acid metabolic disorder,which can cause multiple organ involvement.It can be found in infants or even in the neonatal period and be manifested as feeding difficulty,repeated vomiting,convulsions,abnormal muscle tension,mental retardation,hemolytic uremic syndrome(HUS),etc.In which HUS caused by MMA is better and better known by pediatricians when treating renal diseases while the diagnosis rate is increasing year by year.This article reviews the epidemiology,etiology,pathogenesis,clinical manifestations and treatment of HUS associated with MMA.

Objective To study the effect of methotrexate on the neointimal formation after arterial balloon injury. Methods Male rabbits were randomized into four groups: MTX 0.5 mg/kg per week, MTX 1.5 mg/kg per week, MTX 5 mg/kg per week and the control. Drugs were administered by intramuscular injection. Rabbit carotid arteries were harvested 2 and 4 weeks after injury. Results Histologically, the intimal areas were reduced significantly in MTX treated animals compared with the controls. The VSMC proliferation in injured vessels was identified by immunostaining for proliferating cell nuclear antigen (PCNA). In comparison, PCNA-positive cells in both intima and media were significantly reduced by treatment of MTX. But MTX did not enhance reendothelialization in the injured carotid arteries as determined by Evans blue stain.Conclusion Low dosage of MTX could attenuate neointimal formation after arterial injury by inhibiting VSMC proliferation.

Objective To evaluate the therapeutic efficacy of congenital muscular torticollis (CMT) by two different methods. Methods A total of 86 patients with congenital muscular torticollis in recent 7 years were involved in this study, including 69 cases under 12 years old and 17 cases over 12. Two different operation methods, incision of inferior extremity of sternocleidomastoid muscle and incision of amphi-sternocleidomastoid muscle with post-operative traction, were alternatively chosen according to the ages and deformed degrees of the patients. Results 72 patients were followed-up for 1 to 7 years (averaged 4.5 years). Based on the effect of function and cosmetology in the group of 57 cases under 12 years old, there were 52 cases (91.2 %) excellent, 5 cases (8.8 %) good and no case with side-effects. Meanwhile, in the group of 15 cases over 12 years old, there were 11 cases (73.3 %) excellent, 4 cases (26.7 %) good and no case with side-effects. The effect was satisfactory. Conclusion Different patients with congenital muscular torticollis should be treated with different therapeutic methods.

Objective To monitor the function of infection on myelination in white matter damage,neonatal Wistar rats of postnatal day 2 (P2) and postnatal day 7 (P7) were injected intraperitoneally with the same doses of lipopolysaccharides (LPS),and 2',3 '-cyclic nucleotide phosphodiesterase (CNPase) and myelin basic protein (MBP) were labeled in immature oligodendrocytes and mature oligodendrocytes.To investigate the function of tumor necrosis factor(TNF)-α according to test the change of TNF-α expression in the brain.Methods Ninty-six neonatal Wistar rats were randomly divided into four groups (each group 24 rats):group A:LPS (5.0 mg/kg) was injected intraperitoneally on P2 ; group B:LPS (5.0 mg/kg) was injected intraperitoneally on P7 ;group C1 and C2 were control groups in which equal amount of normal saline was injected intraperitoneally on P2 or P7.The expression of CNPase at 24 h after injection and MBP at P14 in brain tissue of each group were measured by immunohistochemistry and express of TNF-α mRNA at 4 h after the injection was measured by RT-PCR.Results Punctate hemorrhage in the corpus callosum,external capsule and intraventricular hemorrhage were seen in group A.Periventricular leukomalacia appeared in the corpus callosum and glial cells hyperplasia could be seen periventricular in P14 rat brains,but not found in the group B and any of the saline-injected rat brains.Compared with group C1 and C2 respectively,CNPase-positive cells showed obvious decrease in the area of white matter in periventricular in group A(106.93 ± 2.62 vs 113.67 ± 2.69,P ＜ 0.01) and group B (96.37 ± 1.82 vs 101.65 ± 2.01,P ＜ 0.01).Following LPS treatment in group A,the protein expression of MBP in neonatal brain decreased evidently compared with group C1 at P14 (128.21 ± 2.99 v s 134.81 ± 2.98,P ＜ 0.01),while no significant change was found between group B and group C2(134.77 ±3.68 vs 134.81 ±2.98,P ＞0.05).After 4h of the LPS treatment,the level of TNF-α mRNA was greatly increased in group A,it was significantly higher than that in group B (1.79 ± 0.04 vs 1.18 ± 0.04,P ＜ 0.01).Conclusion Intraperitoneal injection of LPS to the development neonatal rats can lead to dysmyelination and white matter damage.The expression of TNF-oα mRNA increased significantly in these immature neonatal rats,while only myelination delay occurred in those of mature neonatal rats without dysmyelination.

AIM:To establish a HPLC fingerprint of Stellera chamaejasme L. from Inner Mongolia Region. METHODS: The RP-HPLC method was used with Akzonobel Kromasil C_ 18 (250 mm?4.6 mm, 5 ?m) the acetonitrile-0.5% phosphoric acid (gradient elution) was used as mobile phase, analytic time was 60 min, and detective wavelength was at 297 nm, the column temperature of 15℃ were adopted. RESULTS: The HPLC fingerprint of Stellera chamaejasme L. set up showed that 14 peaks were co-possessing in different sources. The results of method validation met technical standard of fingerprint, the similarities of Stellera chamaejasme L. were 0.9 to 1.0. CONCLUSION: The method is stable and reliable with a good reproducibility and provides a reference standard for the quality control of Stellera chamaejasme L. from Inner Mongolia Region.

Humans ; Integrative Medicine ; Medicine, Chinese Traditional ; methods

Objective To study a correlativity of amplitude of low frequency fluctuation change of restingstate brain activity and aphasia quotient in aphasia patients after stroke and investigate recovery and mechanism of the aphasia.Methods Adopting amplitude of low-frequency fluctuation (ALFF) in blood oxygenation level dependent functional MRI(BOLD-fMRI) and Siemens version 3.0T MR Scanner was used to obtain 12 aphasia patients and 20 normal volunteers of fMRI data.The fMRI data were processed with the software of DPARSF and analyzed by ALFF,and group analysis was performed with two sample t-test by REST software to obtain increased and decreased ALFF map.Brain regions,in which,ALFF of aphasia group was lower than that of normal control group,were done correlation analysis with aphasia quotient.Results As compared with those in normal subjects,the regions showing decreased ALFF in aphasia patients were distributed in left middle temporal gyrus,left medial prefrontal gyrus,right cerebellum,which were positively correlated with aphasia quotient (r Roi1 =0.48,r Roi2 =0.36,r Roi3 =0.28,respectively);while the regions showing increased ALFF covered left occipital lobe,precentral gyrus,insula,right precuneus.Conclusion As compared with those in normal subjects,the regions positively correlated with aphasia quotient show decreased ALFF in aphasia patients,which are one of the mechanisms of aphasia.The regions increased ALFF in aphasia patients may participate in language function of recovery.

Objectives To explore the clinical manifestations, treatment and prognosis of a case of blindness caused by nephrotic syndrome with cerebral venous sinus thrombosis (CVST). Methods The clinical manifestations, diagnosis and treatment of a case of NS with CVST were analyzed. The latest domestic and foreign reseach progresses in treatment for CVST in children were reviewed. Results Epilepsy suddenly appeared with diplopia, binocular vision loss and blindness in anticoagulant therapy for the child with NS. Brain magnetic resonance venography (MRV) suggested CVST. MRV reexam-ined showed that the intracranial thrombosis was completely dissolved after urokinase thrombolysis for one month followed by ineffective heparin anticoagulation. At present, international standards of anticoagulant therapy have been adopted in the treatment for CVST patients. Coagulation function (e.g.APTT) and international standardization ratio were monitored in order to prevent bleeding. Conclusions It is better to perform neural imaging examination early in suspected CVST patients. Anti-coagulation and thrombolytic therapy should be given immediately once the risk of bleeding was excluded and used for 3-6 months.