1.inical and radiological features of the late-onset methylmalonic aciduria: a review of three cases
Zhaoxia NG ; Wei ZHANG ; Yanling YANG ; Yun YUAN
Chinese Journal of Neurology 2000;0(04):-
jective To study the clinical and radiological features of the patients with late-onset methylmalonic aciduria ( MMA). Methods Two men and one woman with MMA were screened and confirmed by urinary organic acid analysis with gas chromatograph/mass spectrometer (GC/MS) at their 26, 18 and 34 years old, respectively. Their clinical features, laboratory findings, radiological manifestations, treatment and outcome were reviewed. Results The clinical features of 3 patients were varied with neurological abnormalities. Case 1 had periodic enuresis for 16 months, progressive dementia and movement disorder for 3 months. Physical examination showed an apparent cognitive decline with psychiatric symptoms. Dysarthria, bilateral weakness and pyramidal signs, rigidity and mild tremor of limbs were observed. Case 2 had a progressive memory deterioration, learning difficulty, walking unstably and decreased vision when he was 13 years old, and a general seizure at an age of 16 years. Diffused abnormalities of EEG and rnild renal defects were found in the above two patients. General white matter hyperintensity and cerebral atrophy on T2-weighted MR images were evident. Additionally, these two patients had hyperhomocystinemia and carnitine deficiency. Case 3 had complained about walking unstably and fatigue over 2 months. Reduced facial movements, deep sensation loss and muscle weakness in lower extremities, marked rigidity and diminished tendon reflexes were detected. Megaloblastic anemia and cobalamin deficiency were found. MRJ performed revealed bilateral symmetric areas having high density involving the globi pallidi, posterior limbs of internal capsule and the cerebral peduncles. Remarkable elevations of urinary methylmalonic and methylcitric acid were confirmed in all 3 patients. After vitamin B12 supplementation, significant improvement was observed. Conclusion Three cases with vitamin BI2 responsive type of late-onset MMA were reported. Two patients were combined with hyperhomocystinemia and the other had only isolated MMA. There might remain prominent differences among MMA subgroups in clinical presentations and neuroradiologic findings. VitaminB12 might be very effective to improve the prognosis of the patients. MMA should be considered as a differential diagnosis for the etiological investigation of adult cerebral metabolic or degenerative diseases.
2.The manual MGIT system for the detection of M tuberculosis in respiratory specimens: an experience in the University Malaya Medical Centre
Fadzilah Mohd Nor ; Kee Peng Ng ; Yun Fong Ngeow
The Malaysian Journal of Pathology 2009;31(2):93-97
A prospective study was conducted on 510 respiratory specimens for the presence of M. tuberculosis
detected by direct acid-fast bacilli (AFB) smear examination, culture in the Manual Mycobacteria
Growth Indicator Tube (BBL MGIT, Becton-Dickinson) and culture on Lowenstein-Jensen (LJ)
medium. From positive BBL MGIT tubes, Ziehl-Neelsen and Gram stains were performed and
subcultures were put up on LJ medium. A total of 101 (19.8%) specimens were positive by the BBL
MGIT, 60 (11.8%) by primary LJ medium culture, 31 (6.1%) by direct smear examination and 29
(5.7%) by all three methods. Using primary LJ culture as the gold standard, the sensitivity and
specifi city of the BBL MGIT were 90% and 89.6% respectively but the sensitivity of AFB smear
microscopy was only 48.3%. About half (51.1%) of the BBL MGIT false positives were due to
contamination by non-AFB bacteria. The remaining false positives comprised specimens that were
AFB microscopy positive but LJ culture negative. Of the AFB isolates obtained on LJ primary
and sub-cultures, almost all (93.3%) were identifi ed as Mycobacterium tuberculosis complex. The
mean time-to-detection was signifi cantly shorter (p<0.0001) for the BBL MGIT than for LJ culture.
For the former, positive results were available within 14 days for both AFB smear-positive and
AFB smear-negative specimens. On the average, positive results were obtained 1.8 days earlier
for direct AFB smear-positive samples than for AFB smear-negative samples. On the other hand,
positive growth on LJ medium appeared after at least 33 days of incubation. These fi ndings suggest
that the BBL MGIT system will be a suitable alternative to LJ culture for the routine diagnosis
of pulmonary tuberculosis, but a combination of liquid and solid cultures is still required for the
highest diagnostic accuracy.
3.Tacrolimus therapy in refractory lupus nephritis: a prospective study in a single center
Yun-yun FEI ; Qing-jun WU ; Wen ZHANG ; Dong XU ; Meng-tao LI ; Xuan NG ZHA ; Yan ZHAO ; Xiao-feng ZENG ; Feng-chur NG ZHA
Chinese Journal of Rheumatology 2012;16(1):9-12
ObjectiveThe purpose of this study was to assess the efficacy,safety and optimal dose of tacrolimus monotherapy in patients with refractory lupus nephritis(LN) who were resistant to cyclophosphamide(CYC).MethodsA total of 14 LN patients (2 men and 12 women) with persistent proteinuria who were resistant to CYC treatment more than 8 g for half a year were enrolled.Tacrolimus was initiated at 2 mg/d (patient weight<60 kg) or 3 mg/d(patient weight≥60 kg) which was administered in two divided doses.Prospective data on daily proteinuria,serum album level and serologic lupus activity were collected and followed for 6 months.ANOVA and Pearson correlation analysis were used for statistical analysis.Results Mean age at baseline was(30±9) years.Mean urinary protein decreased significantly from(6.2±5.1) g at baseline to (1.1±0.9) g at 6 months (F=16.21,P<0.01).Mean serum album level increased significantly from (27.9±9.7) g/L at baseline to(37.8±2.2) g/L at 6 months(F=16.71,P<0.01 ).Complete or partial response was observed in 86% of patients receiving tacrolimus therapy.The effective dosage in this study was 0.03-0.06mg·kg-1·d-1 of the patients who had complete response or partial response to tacrolimus.The tacrolimus level in partially and completely responding patients was less than 3 ng/ml.There was no significant difference among blood tacrolimus levels of complete,partial,and no response patients [(1.6-±0.4),(2.0±0.6) and (22±1.1) ng/nl],respectively).No definite correlation was found between efficacy and tacrolimus level.Tacrolimus was well tolerated at current dose,besides one with new onset hypertension and one with alopecia.ConclusionOur results suggest that tacrolimus at low dosage and serum level is potentially effective and safe for the treatment of patients with LN and persistent proteinuria resistant to CYC.The optimal dosage of tacrolimus for LN may be 0.03-0.06 mg·kg-1·d-1.
4.The effect of fluoride and arsenic pollution on bone metabolism in exposed population
Qi-bing, ZENG ; Yun, LIU ; Ai-hua, ZHANG ; Feng, HONG ; Jun NG YA ; Xian, YU
Chinese Journal of Endemiology 2011;30(4):393-395
Objective To explore the effect of fluoride and arsenic pollution on bone metabolism in exposed population. Methods One hundred and fifty-two fluoride and arsenic exposed people were selected from Jiaole village, Yuzhang town, Xingron county, Guizhou province in 2006, and 59 not exposed people from Daguoduo village 13 km away from Jiaole village were selected as control. Urinary fluorine(UF), urinary arsenic (UAs), urinary hydroxyproline (UHYP), cross-linked N-telopeptides of type I collagen (UNTX) and bone strength index(STI) were detected. Results The main effect of fluoride on UHYP and UNTX were statistically significant (F = 9.785, 4.225, P < 0.01 ), but was not significant on STI(F = 0.183, P > 0.05). The main effect of arsenic on UNTX was statistically significant (F = 2.660, P < 0.05 ), but was not significant on UHYP and STI(F = 2.012, 0.183,all P > 0.05). The interaction between fluoride and arsenic on UNTX was statistically significant (F= 2.429, P <0.01), but was not significant on UHYP and STI(F= 1.218, 1.001, all P> 0.05). Conclusions Fluoride exposure can affect the metabolism of collagen and bone resorption, and Arsenic exposure main affect bone resorption, fluoride and arsenic co-exposure have more significant effect on bone resorption. UNTX may be used as biological biomarker of bone metabolism for population co-exposed to fluoride and arsenic in health monitoring.
5.Case Of Significant Weight Loss And Dysphagia " Due To A Curse"
Farhad F Vasanwala ; Dr. Shu-Yun Tan ; Rukshini Puvanendran ; Beng-Yeong Ng
ASEAN Journal of Psychiatry 2012;13(1):1-4
Objective: This case illustrates how a patient with medically unexplained symptoms was “cured” using symbolic healing rituals of Christianity and traditional Malay
black magic. Method: We report a case of a 49-year-old lady who presented with unexplainable weight loss and dysphagia despite extensive outpatient and inpatient
medical investigations. She later attributed these symptoms to a “curse” by a Boyanese man with whom she had disagreements. After catharsis with a Roman
Catholic priest and cleansing with a Bomoh (Malay witch doctor), the patient’s health improved. Results: We believe this patient had a conversion disorder due to
recent multiple stressors in her life and she attributed her symptoms to the “curse” inflicted to her. The symbolic healing rituals by the Catholic priest and Bomoh
“cured” her of her illness which concurred with the patient’s own beliefs for her illness. Conclusion: This article illustrates the importance of the physician being
familiar with various local traditional beliefs, and how the interplay between various different religions and customs can come together to treat medically unexplained
symptoms in a country like Singapore.
6.Aging Male Symptoms Scale (Ams) For Health-Related Quality Of Life In Aging Men: Translation And Adaptation In Malay
Hui Meng Tan ; Wah Yun Low ; Seng Fah Tong ; Jamaiyah Haniff ; Geeta Appannah ; Verna K.M. Lee ; Ee Ming Khoo ; Chirk Jenn Ng ; Christopher Chee Kong Ho
Malaysian Journal of Public Health Medicine 2015;15(2):17-23
The Aging Male Symptoms Scale (AMS) measures health-related quality of life in aging men. The objective of this paper is to describe the translation and validation of the AMS into Bahasa Melayu (BM). The original English version of the AMS was translated into BM by 2 translators to produce BM1 and BM2, and subsequently harmonized to produce BM3. Two other independent translators, blinded to the English version, back-translated BM3 to yield E2 and E3. All versions (BM1, BM2, BM3, E2, E3) were compared with the English version. The BM pre-final version was produced, and pre-tested in 8 participants. Proportion Agreement, Weighted Kappa, Spearman Rank Correlation Coefficient, and verbatim responses were used. The English and the BM versions showed excellent equivalence (weighted Kappa and Spearman Rank Coefficients, ranged from 0.72 to 1.00, and Proportion Agreement values ranged from 75.0% to 100%). In conclusion, the BM version of the AMS was successfully translated and adapted.
7.Treatment of clavicular fractures using intramedullary nailing or K-wire versus plating fixation: a meta-analysis.
Ke-xue ZHANG ; Jing-xin ZHAO ; Zhe ZHAO ; Li-cheng NG ; Xiu-yun SU ; An-hua LONG ; Zhi MAO ; Jin-hui ZHANG ; Li-hai ZHANG ; Pei-fu TANG
China Journal of Orthopaedics and Traumatology 2015;28(5):454-461
OBJECTIVETFo compare the efficacy and complications rate of intramedullary (IM) nailing or K-wire versus plating fixation for clavicular fractures.
METHODSPubmed, Embase, Cochrane Library databases, CNKI, VIP and Wangfang databases were searched to find all randomized or quasi-randomized controlled trials of clavicle fractures using plating versus IM nailing or K-wire. The methodologic quality of the studies was assessed. After independent study selection by 2 authors ,data were collected and extracted independently. Outcomes of postoperative shoulder functional measurement, the efficacy and information of the operation and complications rate were meta-analyzed using RevMan 5 software.
RESULTSNine hundreds and seventy-six patients in 10 randomized controlled trials (RCTs) and 3 quasi-RCTs were involved in the meta-analysis,of which 5 studies compared the K-wire and the plating fixations and 8 studies compared the IM nailing and the plating fixations. The overall odds ratio(OR) (with 95% CI) of the operation efficacy for K-wire versus the plating was 3.79 (1.93, 7.46). The overall weighted mean difference (with 95% CI) of Constant Shoulder score for plating versus IM fixation was -1.39 (-3.43, 0.65) in 6 studies. The overall OR of the plating versus IM nailing was 9.34(2.70, 32.32) for the overall major complications in 5 studies and 5.04 (1.52,16.77) for the revision rate in 5 studies.
CONCLUSIONThe current limited evidences suggested that the IM fixation could reduce the incidences of the overall major complications and the revision surgery, while the post-operative efficacy of the plating was superior to the K-wire. More high quality RCTs are still needed in the future.
Bone Nails ; Bone Wires ; Clavicle ; injuries ; surgery ; Fracture Fixation, Intramedullary ; instrumentation ; methods ; Fractures, Bone ; surgery ; Humans ; Randomized Controlled Trials as Topic
8.Multiple soft tissue defects of hand repaired by muliplefoliated tissue flap only pedicled by descending branch of lateral circumflex femoral artery
Wen-ya ZHANG ; Hui-guo WU ; Yu-xiang HU ; Dong-ning SONG ; Ya-fei HU ; Ke-luo NG JIA ; Ze-ang PAN ; Yun-feng WANG ; Bin-hui LI
Chinese Journal of Microsurgery 2011;34(4):280-282
ObjectiveTo introduction of perforator flaps,muscle flaps pedicled by descending branch of lateral circumflex femoral artery,method and their clinical application that multiple soft tissue defects of hand are repaire by muliplefoliated tissue flap only branch lateral circumflex femoral artery.MethodsFifteen patients with multiple soft tissue defects of hand were repaired muliplefoliated tissue flap only pedicled bydescending branch lateral circumflex femoral artery.At first,the anterolateral thigh perforator flap was designed and harvested according to the soft tissue defects of hand, then the descending branch lateral circumflex femoral artery was dissected at the same time the segmented perforator flap,fascia lata flap,rectus femoris muscle flap, vastus lateralis muscle flap, vastus intermedius muscle flap and distal spatium intermusculare flap were harvested in need according to distance among soft tissue defects.The muliplefoliated tissue flap was harvested only pedicled by descending branch lateral circumflex femoral artery, at last muscle flaps and fascia lata flaps were covered by skin graft, so the multiple soft tissue defects of hand were repaired in one time.ResultsNo vascular crisis happened. All skin grafts survived well, the contour of all repaired soft tissue defects was good and protective feeling was recovered by skin grafts of all flaps. All cases were got follow-up and the range was from 6 to 20 months(the average was 8.7 months).Wound of donor site healed well, muscle strength of quadriceps and motion of knee were normal. Three cases were excellent,nine cases were well and 3 cases were good, according to upper extremity function evaluation criteria of Chinese Medical Society for the Surgery of the Hand, the rate of good was 80 percent.ConclusionMultiple soft tissue defects of hand can be repaired by muliplefoliated flap only pedicled by descending branch of lateral circumflex femoral artery. Its advantages included reduction of operation time and treatment, good recovery of hand contour and function. It is a good method to repair multiple soft tissue defects of hand.
9.Change s of haemagglutination inhibitionan tibody level within one month after in fluenza A (H1N 1) vaccination
Qing-hua CHEN ; Guo-ming ZHANG ; Yan LI ; Fang-jun LI ; Qiu-feng TU ; Ping YUAN ; Fu WANG ; Qi-you XIAO ; Hua-qing WANG ; Yun-tao NG ZHA
Chinese Journal of Microbiology and Immunology 2013;(10):744-749
Objective To find the changes of haemagglutination inhibition ( HI ) antibody level against A/California/07/2009 (H1N1) within one month after pandemic A/H1N1 influenza vaccine (A/H1N1InfV) vaccination, and to provide data for drawing up immunization protocols against novel influenza . Methods The HI antibodies against A/California/07/2009 (H1N1) in sera from the inoculated subjects were tested by HI test .The geometric mean titer ( GMT) , geometric mean increase ( GMI) , seroconversion (SC) rate, seroprotection (SP) rate of HI antibodies were compared among the sera collected on day 3, 7, 14, 30 post vaccination .Results 961 participants were injected with A/H1N1InfV.In subjects aged 3 to 11 years, the antibody level peaked on day 14 post vaccination, but neither on day 14 nor on day 30, the lower bound of the two -sided 95%CI for the SP rate could fulfill the criteria of the FDA for influenza vac-cine.In subjects aged 12 to 60 years, the antibody level peaked on day 14 post vaccination and the SC rate , SP rate and GMI fulfilled the criteria of the European Medicines Agency ( EMEA) and the FDA for influenza vaccine. In subjects aged more than 60 years, the antibody level peaked on day 30 post vaccination , and the SC rate, SP rate and GMI on day 30 fulfilled the criteria of the EMEA and the FDA .Conclusion One dose A/H1N1InfV vaccination was able to induce enough protection on day 14 for subjects aged 12 to 60 years, on day 30 for subjects aged more than 60 years;however , for subjects aged 3 to 11 years who were antibody-negative at baseline , the lower bound of the two-sided 95%CI for the SP rate on day 14 and day 30 couldn′t fulfill the criteria of the FDA for influenza vaccine .
10.Management of erectile dysfunction: barriers faced by general practitioners.
Wah-Yun LOW ; Chirk-Jenn NG ; Ngiap-Chuan TAN ; Wan-Yuen CHOO ; Hui-Meng TAN
Asian Journal of Andrology 2004;6(2):99-104
AIMTo explore the barriers faced by general practitioners (GPs) in the management of patients with erectile dysfunction (ED).
METHODSThis was a qualitative analysis of focus group discussions and in-depth interviews involving 28 Malaysian GPs.
RESULTSGPs' perception of ED being not a serious condition was a major determinant of their prescribing practice. Doctor's age (younger), gender (female), short consultation time and lack of experience were cited as barriers. The GPs' prescribing habits were heavily influenced by the feedback from the first few patients under treatment, the uncertainty of etiology of ED without proper assessment and the profit margin with bulk purchase. Other barriers include Patients' coexisting medical conditions, older age, lower socio-economic status, unrealistic expectations and inappropriate use of the anti-impotence drugs. Cardiovascular side effects and cost were two most important drug barriers.
CONCLUSIONThe factors influencing the management of ED among the general practitioners were multiple and complex. An adequate understanding of how these factors (doctors, patients and drugs) interact can assist in the formulation and implementation of strategies that encourage GPs to identify and manage ED patients.
Adult ; Age Factors ; Drug Costs ; Drug Prescriptions ; Erectile Dysfunction ; epidemiology ; psychology ; therapy ; Focus Groups ; Humans ; Malaysia ; epidemiology ; Male ; Middle Aged ; Phosphodiesterase Inhibitors ; adverse effects ; economics ; therapeutic use ; Physicians, Family ; Piperazines ; adverse effects ; economics ; therapeutic use ; Purines ; Referral and Consultation ; Sex Factors ; Sildenafil Citrate ; Socioeconomic Factors ; Sulfones