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Biomedical subjects

L S Fu

Publications and source records attributed to L S Fu.

At least 19 recordsLinked to original sources

Mycobacterium avium complex-associated hemophagocytic syndrome in systemic lupus erythematosus patient: report of one case.

Hemophagocytic syndrome (HPS) in systemic lupus erythematosus(SLE) patients has not commonly been reported. In this case study, we report the first case of Mycobacterium avium complex (MAC)-associated hemophagocytic syndrome in a patient with systemic lupus erythematosus (SLE). This SLE patient, a 15-year-old girl, had been on a high dose of prednisolone (> 0.5mg/kg/day) for more than 3 years. She presented with a spiking fever, hepatosplenomegaly, pancytopenia, hyperferritinemia and adult respiratory distress syndrome. Bone marrow examination revealed hemophagocytosis as well as non-caseating granulomatosis. There was no indication of SLE fare-up. She responded poorly to initial treatment with methyl-prednisolone, intravenous immumoglobulin, etoposide, and drugs for Mycobacterium tuberculosis including rifampin, ethambutol, isoniazid and pyramide. However, gastric lavage culture revealed MAC. Following treatment with clarithromycin, ciprofloxacin and amikacin, her condition gradually improved and she was discharged 3 months after admission. In SLE patients with pancytopenia and hyperferritinemia, MAC-associated HPS should be considered in the differential diagnosis.

Adolescent↗

Psychiatric morbidity and post-traumatic symptoms among survivors in the early stage following the 1999 earthquake in Taiwan.

This study reports the clinical psychiatric presentations and post-traumatic symptoms among 525 survivors at Yu-Chyr District in Nantou County who sought psychiatric service in the first month following the devastating earthquake that struck the central area of Taiwan. All subjects received psychiatric interviews and assessments using the 12-item Chinese Health Questionnaire (CHQ-12) and a checklist for post-traumatic symptoms. The most common psychiatric symptoms reported were insomnia, palpitations, nervousness, and dizziness with headache. Eleven percent of the subjects reported having thought of death or having suicidal ideation. The mean score on the CHQ-12 was 6.43 (S.D.=2.89). The rate of probable psychiatric morbidity as defined by a CHQ-12 score > or =3 was 89.9%. Post-traumatic symptoms were very prevalent, particularly symptoms of re-experiencing the earthquake and hyper-arousal. Factors significantly associated with high psychiatric morbidity were being female, serious destruction of property and house, and personality characteristics of nervousness and obsessiveness. Findings of this study suggest that early psychiatric intervention, including pharmacological treatment for acute stress disorder, is indicated during the early stages following a disastrous earthquake.

Adult↗

Intra-abdominal actinomycosis with hepatic pseudotumor and xanthogranulomatous pyelonephritis in a 6-y-old boy.

We report the case of a 6-y-old boy with actinomycosis, presenting as xanthogranulomatous pyelonephritis (XGP), hepatic pseudotumor and abdominal abscess. Symptoms included intermittent fever, abdominal pain and significant weight loss. Hepatic and renal tumor masses were suspected on sonography and computerized tomography. XGP and actinomycosis were proven by pathology. The patient recovered well with antibiotic alone.

Abdominal Abscess↗

Clinical spectrum of Kawasaki disease in infants.

BACKGROUND: Kawasaki disease is a common acquired heart disease in children. Only a few reports have been published concerning Kawasaki disease in infants. This study was performed to assess the clinical spectrum of Kawasaki disease in infants. METHODS: Between January 1989 and December 1998, a total of 48 consecutive Kawasaki patients less than one year of age were enrolled and studied retrospectively. Coronary artery dilation was defined as the internal diameter of a coronary artery larger than 3 mm. All cases received 2 gm/Kg of intravenous immunoglobulin. We divided the patients into two groups; group I; coronary artery dilation (+) and group II; coronary artery dilation (-), and compared the clinical and laboratory data. RESULTS: Of 273 patients with Kawasaki disease, 48 (17.5%) were less than one year of age. Among these patients (< 1 year old), the median age was 7.8 +/- 2.8 months (range 2 months to 12 months), and the male to female ratio was 1.52:1. The incidence of atypical Kawasaki disease was 31.2% (compared with an incidence of atypical Kawasaki disease among patient more than one year of age of 7.5%; p < 0.001), and that of coronary artery dilation was 35.4%. Clinical manifestations included fever 100%, extremity change 91.6%, skin rash 89.6%, conjunctivitis 89.6%, oral mucosa change 89.6%, and cervical lymphadenopathy 0%. Laboratory data revealed white blood cell count: 15,403 +/- 6,282/mm3, hemoglobin: 10.1 +/- 1.0 gm/dl, neutrophil: 59.2 +/- 13.7%, lymphocytes: 30.6 +/- 13.1%, platelet count: 456,3000 +/- 216,4000/mm3, and C-reactive protein 8.2 +/- 5.6 mg/dl. Patients with coronary artery dilation had a longer duration of diagnosis, higher incidence of atypical presentation, lower incidence of conjunctivitis, lower incidence of skin rash, lower incidence of extremity change, and lower C-reactive protein. The predictive value of coronary artery dilation based on the combination of atypical presentation, duration of diagnosis, and C-reactive protein was 81.2%. CONCLUSIONS: Kawasaki disease in infants is associated with a high incidence of atypical presentation and increased risk of coronary artery dilation. We suggest that in an infant with insufficient diagnostic criteria for Kawasaki disease, care should be taken to avoid missing atypical Kawasaki disease. Echocardiography is an important tool for diagnosis of atypical Kawasaki disease.

C-Reactive Protein↗

Chronic granulomatous disease: a case report.

Chronic granulomatous disease (CGD) is a rare inherited disorder caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex of phagocytic leukocytes. The leukocytes of the CGD patients cannot produce adequate amount of superoxide and other oxygen metabolites which are toxic to microorganisms. As a result, the phagocytes fail to kill the ingested microorganisms, especially those with catalase activity. Typically, CGD patients suffer from recurrent pyogenic infections starting from the first year of life. We report a young boy who had experienced recurrent perianal abscess, osteomyelitis and bacterial enterocolitis. Flow cytometric analysis revealed defects in the neutrophil respiratory burst pathway and defined the carrier state of his mother and younger sister. He received antimicrobial prophylaxis at our out-patient clinics and remained well at present. We try to make clinical physician keep in mind the diagnosis of CGD by presenting this typical case. In the meantime, we review the recent literature regarding the advances in diagnosis and management of CGD.

Animals↗

Modulation of nigrostriatal dopaminergic transmission by antisense oligodeoxynucleotide against brain-derived neurotrophic factor.

Brain-derived neurotrophic factor (BDNF) promotes the differentiation and growth of developing dopamine (DA) neurons and supports the survival of mature DA cells in culture. However, the neurotrophic role of endogenous BDNF in the adult DA system in vivo has not been well established. To investigate the hypothesis that blockade of endogenous BDNF expression results in DA dysregulation, we used an 18-mer antisense oligodeoxynucleotide (ODN) targeted to the first ATG codon of the BDNF transcript. The biological activity of the antisense ODN was initially tested in vitro. In cultured dopaminergic MES 23.5 cells, antisense BDNF (20 microM) effectively reduced BDNF protein expression and cell survival. Furthermore, in primary embryonic mesencephalic cultures, antisense BDNF reduced the number of tyrosine hydroxylase positive neurons and inhibited [3H]DA uptake in a time- and dose-dependent manner. The specificity of the antisense molecule was confirmed by comparing its effects with those of a control ODN having the same base composition but in scrambled sequence. In rats, two days following an intranigral or intrastriatal injection of antisense BDNF (0.5 microg), we observed a two-fold and five-fold increase in nigral DA levels, respectively, but no change in striatal DA content. Seven days after an intrastriatal antisense BDNF injection, DA levels were elevated in the striatum apparently due to decreased DA turnover. These observations suggest that inhibition of endogenous BDNF expression tends to augment rather than inhibit nigrostriatal DA transmission. Thus, the biological effects of endogenous BDNF on the nigrostriatal DA system in the adult organism merits further investigation.

Animals↗

Glutathione-related enzyme activities in human fetal adrenal, liver, and kidney.

AIM: To understand the capacity of fetal adrenal to catalyze reaction metabolites. METHODS: Subcellular fractions were prepared by differential centrifugation in fetal adrenal and liver. Glutathione (GSH)-transferase, reductase, and peroxidase were measured. RESULTS: The mean values (mumol.min-1/g protein) of GSH-transferase activities in adrenal microsome (112 +/- 34), mitochondria (62 +/- 35), and cytosol (191 +/- 89) were 373%, 270%, and 167%, respectively, higher than those in the corresponding fractions of fetal liver. Adrenal microsomal GSH-transferase was positively correlated with adrenal microsomal P-450 (r = 0.821, P < 0.01), and with adrenal microsomal aminopyrine N-demethylase (r = 0.829, P < 0.01). The GSH reductase contents (mumol.min-1/g protein) in adrenal mitochondria (24 +/- 14), and in S9 (36 +/- 15) were almost 5 times higher, compared with that in liver. Selenium-dependent GSH peroxidase was present in all the adrenal. CONCLUSION: Fetal adrenal, with greater capacities than those of liver in detoxifying reaction, may act as a drug-metabolizing organ during development.

Adrenal Glands↗

[Effect of ageing on hepatic biotransformation function and microsomal membrane fluidity in rats].

Microsomes were isolated from livers of male Sprague-Dawley rats at 3-4, 14 and 24 months of age for each group for the determination of monooxygenase components and drug metabolism activities. Some GSH-related enzyme activities in microsomes and cytosol were also measured. DPH (1,6-diphenyl-1,3,5-hexatriene) was used to determine the microsomal membrane lipid region fluidity. Microsomal cytochrome P-450 content and NADPH-cytochrome c reductase activities remained unchanged in old rats, but there were significantly decreases in cytochrome P-450 dependent aminopyrine N-demethylase and aniline hydroxylase activities. Glutathione S-transferase (GST) in cytosol and microsomes and glutathione peroxidase (GSH-Px) in cytosol were also decreased in old rats. Simultaneously, microsomal membrane fluidity of 24-month old rats decreased (measured at 25 degrees C and 37 degrees C), accompanied by an elevation of cholesterol/phospholipid ratio. Results suggest that there might be some relationship between the lipid environment and membrane fluidity changes and the decreases of hepatic biotransformation function.

Aging↗

Class IV lupus nephritis associated with Graves' disease.

Graves' disease was initially diagnosed in an 11-year-old Chinese boy in March 1989. After regular propylthiouracil (PTU) and thyroxine, he achieved a euthyroid state. Heavy proteinuria with class IV lupus glomerulonephritis, anemia, arthralgia, low serum complement and anti-dsDNA (+) appeared 15 months later. Thyrotoxicosis also relapsed at this time. His condition fitted the diagnostic criteria of systemic lupus erythematosus. His antimicrosomal antibody titer was 1:1,600 (+) thyroid-stimulating hormone receptor antibody level was strongly positive, and the titer of antiinsulin antibody was high as well. These clinical, laboratory and histological findings indicate that class IV lupus nephritis may be associated with Graves' disease.

Carbimazole↗

[Studies on correlation between liver drug metabolizing enzyme activities and microsomal membrane fluidity in phenobarbital treated rats].

By the use of ANS(1-anilinonaphthalene-8-sulfonate) and DPH(1, 6-diphenyl-1, 3, 5-hexatriene) as fluorescent probes, correlation between liver microsomal membrane fluidity and drug metabolizing enzyme activity has been studied in rats. phenobarbital(PB) ip treatment caused an increase in P-450 content, cytochrome C reductase.aminopyrine N-demethylase(AMD) and glutathione S-transferase(GST) activities by 78, 66, 270 and 52%, respectively. However, there was a simultaneous decrease in microsomal membrane fluorescent intensity and microviscosity, i.e. an increase in membrane fluidity. There is a positive linear correlation between microsomal membrane fluidity and cytochrome C reductase and AMD activities (r = 0.798, r = 0.781, respectively, P less than 0.05). This result suggests that there may be some relationship between microsomal membrane fluidity and drug-metabolizing enzymatic activities in PB-treated rats.

Aminopyrine N-Demethylase↗

Estimating frequency of disease findings from combined hospital databases: a UMLS project.

Merging data from the Salt Lake VA hospital database and the LDS hospital HELP system into a UMLS sponsored unified patient database has demonstrated that distribution of variables within a disease is hospital independent. Although disease prevalence is clearly not the same among hospitals, analysis of data within a disease group across hospitals can be done using such a merged database. This unified patient database would allow study of unusual diseases not possible using data from a single institution.

Databases, Factual↗

Infants of twin pregnancies with one twin demise in the uterus: a retrospective study.

Many papers have reported that twin pregnancies with one twin demise in the uterus can cause complications in the surviving twin. We retrospectively reviewed charts from 1988 to 1997 at our hospital and found 17 of 302 twin deliveries with one twin intrauterine fetal death. The incidence of intrauterine death of a single twin was 5.7%. Six of the surviving twin (35.3%) had renal function impairment. One died due to acute renal failure. The other five patients recovered normal serum creatinine level. Five patients (29.4%) had abnormal brain imaging findings. One had multicystic encephalomalacia and microcephaly. Three of the five patients were complicated with cerebral palsy. After dividing the patients into complicated (n = 8, 47.1%) and non-complicated (n = 9, 52.9%) groups, we found that the patients with complication had lower Apgar score at 5 minutes and those with monochorionic diamniotic placenta had higher incidences of complication (62.5% vs 12.5%). We conclude that twin pregnancies with one fetal demise in the uterus do result in a higher incidence of complication in the surviving twin, especially if placentation type is monochorionic diamniotic. In managing the surviving twin of the monozygous twin pregnancies with one fetal demise in the uterus, we must be careful to monitor these patients. If complication was suspected, we should arrange the brain and kidney ultrasonography and manage these patients appropriately to decrease the complication risk.

Delivery, Obstetric↗

Levamisole in steroid-sensitive nephrotic syndrome children with steroid-dependency and/or frequent relapses.

The study is to evaluate the effect of levamisole on steroid-sensitive nephrotic syndrome (SSNS) children with multiple relapses, from October 1996 through December 1998, we prospectively recruited 27 SSNS children. Eight children had minimal change nephrotic syndrome and nine had mesangial nephropathy proven by renal biopsy. The other 10 children had normal renal function and no secondary cause of nephrotic syndrome (NS). Nine were frequent relapsers (FR), (> 4 attack per year), nine were steroid-dependent (SD), and another nine were both FR and SD. The dosage of levamisole given was 2 to 3 mg/kg daily or every other day, depending on the patients' response. All were followed regularly at outpatient department. Follow-up items included a routine urinalysis every month, complete blood count (CBC) every 3 months and BUN/Cr, AST/ALT every 6 months. After 6 to 24 (mean 12.2) months of follow-up, the frequency of relapse (FoR) decreased (5.74 +/- 3.24 vs 1.91 +/- 2.0/year p < 0.05). Seven (26%) had no relapse at all. Nine (33.3%) had less than 1/3 the FoR; four (14.8%) had 1/3 to 1/2 the FoR; and seven (26%) still had more than 1/2 FoR as before levamisole. The oral prednisolone dosage also decreased (0.62 +/- 0.42 vs 0.21 +/- 0.35 mg/kg/day, p < 0.05). The levamisole response was independent of the age of NS onset, the interval between NS onset and initiation of levamisole, previous number of relapse, the FoR, and previous use of cytotoxic drug. There were 7 episodes of leukopenia, which returned to normal after discontinuing levamisole for 1 to 2 weeks in 4 patients. Two (7.5%) had reversible leukopenia for more than 4 weeks. No abnormal BUN/Cr, or ALT/AST levels were noted during follow-up. Levamisole is an effective and safe drug for children who have SSNS with FR and/or SD. Two thirds of patients obtain satisfactory control. The dosage can be 2 to 3 mg/kg daily or every other day. The most common side effect is transient leukopenia.

Adolescent↗

Limited cutaneous systemic sclerosis: report of one case.

We report a case of systemic sclerosis, limited type, in a 5 year old boy in Taiwan. He presented with erythema, tight skin over his face and digits, subcutaneous calcification, telangiectasis, as well as Raynaud's phenomenon. The laboratory data showed weakly positive antinuclear antibody (ANA), and Topoisomerase 1 antibody (anti-Scl-70) titers. However his esophagogram, CO diffusion capacity, as well as cardiac and renal function were all normal. He suffered from the skin change for 2 to 3 years before visiting our pediatric clinic when he was 7 years old. We gave him Dipyridamole, D-penicillamine, Colchicine, and Nifedipine. There was some remission of the scleroderma and Raynaud's phenomenon after our treatment.

Child, Preschool↗

Newborns of Chinese mother with systemic lupus erythematosus (SLE).

From January, 1979 to December, 1990, 105 pregnancies of the 87 mothers with systemic lupus erythematosus (SLE) were studied. There were 15 (14.29%) fetal losses. Among the 90 livebirths, 23 (25.5%) were moderately premature; and 1 (1.1%), was extremely premature. All but 2 (2.2%) had an Apgar score more than 7 at 1 minute. There was no neonatal death. Significantly lower birth body weights were noted compared with the matched control group (p = 0.0001). Birth body length and head circumference were not different. Only 1 of the 13 newborns who had been small for gestational age at birth had body weight and length less than the 3rd percentile during follow-up. Three (3.3%) newborns presented as congenital complete atrioventricular block (CCAVB). Their mothers were all positive for SSA (Ro) antibody. One of them obligatorily needed pacemaker implantation. ECG abnormalities including multiple ventricular premature contractions, wandering atrial pacemaker, sinus arrhythmia, and first degree A-V block were detected in another six newborns. Congenital cardiac defect with secundum type atrial septal defect was noted in two newborns (2.2%). Among the 59 mothers who had been tested for SSA antibody, 29 (49.1%) were positive. The incidence of complete A-V block was significantly higher in newborns of mothers with SSA antibody (p < 0.001). On the contrary, the frequency of fetal loss has higher in newborns of mothers without SSA antibody (p = 0.0043).

Female↗

Researcher's Workbench.

The Researcher's Workbench, developed at the University of Utah College of Nursing, is a computer toolkit for nurse researchers. Workbench includes a set of software tools, most of which are commercially available, to support each stage of the research process. The tools reside on a Macintosh II desk-top computer (Apple Computer, Cupertino, CA) that houses processors to run both Macintosh and MS-DOS (Microsoft Corporation) software. The Workbench is available to all College of Nursing faculty, research associates, and graduate students. This article describes the purpose, methods, design, and implementation of the Workbench. The article assumes familiarity with personal computer terminology.

Computers↗

Niemann-Pick disease type C presenting as neonatal hepatitis: report of one case.

A three-month old Chinese male infant was a victim of neonatal hepatitis presenting with prolonged jaundice, poor body weight gain, progressive hepatosplenomegaly and extremely elevated serum alpha-fetoprotein level. Niemann-Pick disease (NPD) type C was confirmed by autopsy, which revealed sphingolmyelin deposition in multiple visceral organs, and normal sphingomyelinase activity in liver. This is the first case of NPD type C in Taiwan. In idiopathic neonatal hepatitis with hepatosplenomegaly here, NPD type C must be taken into consideration.

Hepatitis↗

Intravesical ureterocele in children.

Eighteen intravesical ureteroceles associated with single collecting system were found in 16 children over past 6 years. Hydronephrosis of varying severity, with hydroureter, was present in 10 patients. The ipsilateral kidney was nonfunctioning in 5 patients and normal in 1. The ureterocele was easily detected by ultrasound and usually revealed as a cystic mass in the bladder. On intravenous urogram the ureterocele usually showed a positive cobra-head dilatation. However, it may exhibit a negative filling defect when the function of the associated kidney is impaired. In this series, 9 patients were asymptomatic, and the condition was detected incidentally by ultrasound. 7 patients received surgery. The remaining 9 patients were followed up at an Outpatient Clinic.

Adolescent↗