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C C Huang

Publications and source records attributed to C C Huang.

At least 19 recordsLinked to original sources
PubMed1992

X-linked recessive inheritance of dysgenesis of corpus callosum in a Chinese family.

This report describes a new syndrome of dysgenesis of corpus callosum with other anomalies, presenting as microcephaly, mental retardation, spasticity, and unusual facial appearance in 2 Chinese brothers and their maternal cousins. To date, there has not been any case reported in the Chinese population of this syndrome. All 4 patients in this report present with the same unusual face. Hydrocephalus and/or interhemispheric cyst were found among them. This syndrome is transmitted as an X-linked trait. The nosology is reviewed and discussed.

Agenesis of Corpus Callosum↗
PubMed1992

Sustained enhancement of NMDA receptor-mediated synaptic potential by isoproterenol in rat amygdalar slices.

The effect of isoproterenol (Iso) on synaptic transmission mediated by the N-methyl-D-aspartate (NMDA) receptors (EPSPNMDA) was investigated in slices of rat amygdala using intracellular recording techniques. EPSPNMDA was isolated pharmacologically by application of a solution containing the non-NMDA receptor antagonist 6-cyano-7-nitroquinoxaline-2,3-dione (CNQX, 10 microM) and GABAA receptor blocker bicuculline (20 microM). Superfusion of Iso (15 microM) produced a long-lasting enhancement of EPSPNMDA. Pretreatment the slices with propranolol (10 microM) completely prevented the effect of Iso confirming the mediation by beta-adrenergic receptors. These results provide the direct evidence for adrenergic modulation of excitatory amino acid neurotransmission in the vertebrate central nervous system.

6-Cyano-7-nitroquinoxaline-2,3-dione↗
PubMed1992

Triple-helix formation is compatible with an adjacent DNA-protein complex.

The effect of oligonucleotide-directed triple-helix formation on the binding of a protein to an immediately adjacent sequence has been examined. A double-stranded oligonucleotide was designed with a target site for the binding of a pyrimidine oligonucleotide located immediately adjacent to the recognition sequence for the herpes simplex virus type 1 (HSV-1) origin of replication binding protein, which is encoded by the UL9 gene of HSV-1. Since the optimal conditions for the binding of the UL9 protein and the pyrimidine oligonucleotide to the duplex DNA are markedly different, a pyrimidine oligonucleotide was designed to optimize binding affinity and specificity for the target duplex oligonucleotide. Consideration was given to length and sequence composition in an effort to maximize triple-strand formation under conditions amenable to the formation of the UL9-DNA complex. Using gel mobility shift assays, a trimolecular complex composed of duplex DNA bound to both a third oligonucleotide strand and the UL9 protein was detected, indicating that the UL9-DNA complex is compatible with the presence of a triple helix in the immediately adjacent sequences.

Animals↗
PubMed1992

Langerhans cells in human middle ear cholesteatomas.

Langerhans cells have been found in cholesteatomas for many years. It is believed that they are immunocompetent cells and have the same role in cell-mediated immunologic mechanisms in cholesteatoma as well as in skin. This study used the transmission electron microscope to observe the cellular characteristics of Langerhans cells and the apposition phenomenon of Langerhans cells with lymphocyte-like cells in human middle ear cholesteatomatous tissue. These findings are evidence for cell-mediated immune responses in middle ear cholesteatomas. In vitro Langerhans cells conditioned medium prepared from Lewis rat skin was used to show its effects on protein synthesis and the differentiation of basal cells. Since the cellular behaviour of basal cells is important in the development and pathogenesis of cholesteatoma, the present study shows that Langerhans cells may have some role in the clinical formation of a cholesteatoma. Since cells extracted from rat skin may have a different response from that of cells from human middle ear cholesteatoma, further investigations are necessary to compare the biological effects of both tissues.

Animals↗
PubMed1992

Molecular cloning and sequencing of the Mexico isolate of hepatitis E virus (HEV).

Hepatitis E virus (HEV) is the major causative agent of hepatitis E or what was formerly known as enterically transmitted non-A, non-B hepatitis. The disease has a worldwide distribution but occurs principally in developing countries in any of three forms: large epidemics, smaller outbreaks, or sporadic infections. Genetic variation of different HEV strains was previously noted and it will be important to determine the extent to which this variation may pose problems in the diagnosis and treatment of HEV infection. To analyze differences at the genetic level between HEV(Mexico; M) and the previously characterized HEV(Burma; B) and HEV(Pakistan; P) isolates, overlapping cDNAs were cloned from samples obtained from an infected human and an experimentally inoculated cynomolgus macaque. These cDNA clones, representing the nearly complete (7185-bp) genome of HEV(M), confirmed an expression strategy for the virus that involves the use of 3 forward open reading frames (ORFs). The HEV(M) strain has an overall 76 and 77% nucleic acid identity with the HEV(B) strain and HEV(P) strain, respectively; however, the degree of sequence variation was not uniform throughout the viral genome. A hypervariable region was identified in ORF1 that exhibited a 58 and 54% nucleic acid sequence and 13% amino acid similarity with the Burma strain and the Pakistan strain, respectively. A large number of the nucleotide differences occurred at the third codon position, with the deduced amino acid sequences similarity of 83, 93, and 87% between HEV(M) and HEV(B) isolates in ORF1, ORF2, and ORF3, respectively, and with 84, 93, and 87% amino acid identities between HEV(M) and HEV(P) isolates in ORF1, ORF2, and ORF3, respectively. The nucleotide sequences derived from the highly conserved regions of HEV genome will be useful in developing polymerase chain reaction-based tests to confirm the viral infection. Knowledge of the extent of the sequence variation encountered with HEV will not only aid in the future development of diagnostic and vaccine reagents but also further our understanding of how HEV strain variation might impact the pathological outcome of infection.

Amino Acid Sequence↗
PubMed1992

The clinical outcome of hepatitis C virus antibody-positive renal allograft recipients.

In order to investigate the prevalence of antibody to hepatitis C virus (anti-HCV) in renal transplant patients, the evolution of anti-HCV status, and clinical outcome in anti-HCV-positive renal allograft recipients, we tested the sera from 120 renal transplant patients for anti-HCV. Thirty-eight patients were hepatitis B surface antigen (HBsAg)-positive. Two patients were anti-delta-positive. A total of 79 patients (65.8%) had at least one serum positive for anti-HCV. Anti-HCV positivity decreased after transplantation for more than 5 years (65.5% at transplantation versus 37.9%, 78.3 +/- 13.4 months later). Among those with positive anti-HCV, the HBsAg-positive group had significantly higher incidence of chronic hepatitis (50% vs. 25.5%, P = 0.026) and liver cirrhosis (21.4% vs. 0%, P = 0.001) than HBsAg-negative group. Among the 82 HBsAg-negative patients, the prevalence of anti-HCV was significantly higher in those with chronic hepatitis than in those without (86.7% vs. 56.7%, P = 0.027). We conclude from this study: (1) anti-HCV positivity is quite prevalent in renal transplant patients; (2) coinfection of hepatitis B virus (HBV) and hepatitis C virus (HCV) may lead to aggressive liver disease and cirrhosis; HCV infection alone has a more benign clinical outcome; and (3) HCV infection is an important cause of posttransplant chronic hepatitis in HBsAg-negative patients.

Adult↗
PubMed1992

Effect of body iron stores on serum aluminum level in hemodialysis patients.

To evaluate the influence of body iron stores on the serum aluminum (Al) level, we studied the correlation between iron status (the serum ferritin, serum iron and transferrin saturation) and serum Al levels in 68 severely anemic hemodialysis patients. Among them, 36 underwent the desferrioxamine (DFO) mobilization test. These 68 patients were divided into three groups according to their serum ferritin level. The basal Al level in the patient group was 41.4 +/- 37.4 micrograms/l (control, 4.1 +/- 2.4 micrograms/l). The serum Al level after DFO infusion of the patient group was 111.1 +/- 86.8 micrograms/l. A significantly higher basal Al and peak Al level after DFO infusion were found in group 1 patients (serum ferritin less than 300 micrograms/l) when compared to group 2 (serum ferritin 300-1,000 micrograms/l) and group 3 (serum ferritin greater than 1,000 micrograms/l) patients. A significant negative correlation between serum ferritin and basal serum Al (r = -0.544, p = 0.0001), as well as peak serum Al after DFO infusion (r = -0.556, p = 0.0001), was noted. Similarly, a negative relationship between serum Al (both basal and peak) and either serum iron or transferrin saturation was noted. However, there was no correlation between the serum Al level and the dosage of aluminum hydroxide. In conclusion, serum ferritin, serum iron and transferrin saturation were inversely correlated with serum Al in our hemodialysis patients. Iron deficiency may probably increase Al accumulation in these patients.

Adolescent↗
PubMed1992

The prevalence of hepatitis C virus antibodies in patients treated with continuous ambulatory peritoneal dialysis.

OBJECTIVE: To assess the prevalence of anti-HCV (hepatitis C virus antibodies) in continuous ambulatory peritoneal dialysis (CAPD) patients and staff members. DESIGN: Fifty-nine serum samples were collected and tested by an enzyme immunoassay for anti-HCV. Records and past history of blood transfusion and hemodialysis were reviewed. Results were analyzed using the Fisher's exact test. SETTING: A medical college-operated teaching hospital. PARTICIPANTS: Fifty-two patients and 7 staff members from the Chang Gung Memorial Hospital CAPD unit were studied. RESULTS: Anti-HCV prevalence in patients and staff were 15.4% and 0%. A history of maintenance hemodialysis was associated with a higher prevalence of anti-HCV (33.3% versus 5.9% without hemodialysis, p = 0.015). The prevalence of anti-HCV did not increase with longer CAPD duration. Among those with an episode of hepatitis, higher prevalence of anti-HCV was observed (57.1% versus 8.9% without hepatitis, p = 0.0073). CONCLUSION: HCV is an important agent of hepatitis in CAPD patients. The risk of HCV infection among CAPD staff members is negligible. CAPD offers better control of HCV infection among patients with end-stage renal failure.

Blood Transfusion↗
PubMed1992

Measurement of separate glomerular filtration rate by means of 99mTc-DTPA.

We detected the separate glomerular filtration rate (SGFR) with 99mTc-DTPA in patients on the nephrology ward since 1987. There were 40 patients, 19 males and 21 females. Their age ranged from 15 to 79 years old, with a mean +/- 1SD of 44 +/- 17 years old. They all received a SGFR examination and one or two creatinine clearance (Ccr) examinations during hospitalization. The Ccr values ranged from 4.6 to 141.5ml/min, and the mean +/- 1SD was 68.1 +/- 37.9ml/min. The total GFR value by the SGFR method was 1 to 142ml/min, with a mean of 78.9 +/- 39.4ml/min. The correlation coefficient of Ccr and SGFR was r = 0.81 (p = 0.0001). All 16 patients who had a SGFR less than 40% of the total GFR had specific clinical problems in that particular kidney. We concluded that 99mTc-DTPA renography was a correct and rapid method to check GFR, and may be another choice in addition to the traditional Ccr measurement. It was also a useful method for the diagnosis of disease in one kidney.

Adolescent↗
PubMed1992

Metformin-induced lactic acidosis: report of a case.

Lactic acidosis associated with diabetic patients receiving metformin therapy is rare but may cause significant morbidity and mortality. In nearly all reported cases of metformin-associated lactic acidosis, contraindications to its use were noted, especially renal insufficiency. We describe a 59-year-old diabetic man treated with metformin for more than three years. During the third year of use, he experienced progressive renal function impairment, and during the final month of use, he became azotemic. He was maintained on continuous ambulatory peritoneal dialysis. Several days prior to admission, he suffered from epigastralgia, nausea and vomiting, followed by progressive dyspnea which was Kussmaul in nature. Profound hypotension developed and he sank progressively into a coma. Wide-anion gap metabolic acidosis without ketonemia was detected. His blood lactate level was elevated and metformin-induced lactic acidosis was substantiated. An elevated plasma metformin level of greater than 50 mg/mL was determined later by high-performance chromatography. Rigorous treatment including bicarbonate therapy, bicarbonate hemodialysis and vasoactive agents as well as supportive measures were provided. With a return of pH to normal, the hypotension resolved and his consciousness level slowly improved. Our patient survived this disastrous event, but some neurologic sequelae remained. In order to avoid this life-threatening metabolic disturbance, patients with any contraindications should not be prescribed metformin.

Acidosis, Lactic↗
PubMed1992

Clinical analysis of 206 cases of kidney transplantation.

From July 1981 to May 1991, 206 kidney transplantations were performed at the Chang Gung Memorial Hospital. There were 762 patients on our waiting list for transplantation at that time. Patient follow-up care was undertaken, alternately, by urologists and nephrologists. The average follow-up period was 4.0 +/- 2.8 years. Patient data were registered in the UCLA (University of California at Los Angeles) International Kidney Transplant Registry. Eighty patients received living-related transplants, 126 received cadaveric transplants. Twenty-four per cent of transplant recipients were carriers of HBsAg. Their survival rates were equal to those of non-carriers up to five years postoperatively, although they were prone to episodes of hepatitis. Nineteen kidneys were from HBsAg carriers with recipient survival rates, five years postoperatively, not significantly different from those who received kidneys from non-carriers. However, there was one case of seroconversion from HBsAg negative to HBsAg positive status. There were 19 deaths among the recipients, the major cause of death being infection (57.9%). Eight grafts were lost due to medical noncompliance. Malignant lymphomas were noted in three cases who are now alive and well. Three cases of hepatomas were noted, but unfortunately none of them survived. The transplant recipients were found to enjoy a better quality of life after undergoing psychiatric evaluation. The one-year patient survival rate was 97.4% and 96.2% for living-related and cadaveric transplants, respectively. The one-year graft survival rate was 96.0% and 88.5% for living-related and cadaveric transplants, respectively. Kidney transplantation is a well-accepted method of treatment for end-stage renal disease.

Adult↗
PubMed1992

Wilson's disease: clinical analysis of 71 cases and comparison with previous Chinese series.

We analyzed 71 patients (45 males and 26 females) with Wilson's disease (WD) who were seen at our hospital from 1979 through 1990. The mean age at onset was 18.1 +/- 6.5 years, with 17.0 +/- 6.6 years for males and 20.2 +/- 5.7 years for females. The mean age at the time of diagnosis was 21.0 +/- 6.3 years. Hepatic WD was the most frequent mode of presentation in childhood with a mean age of 15.5 +/- 6.0 years, while neurologic WD tended to occur in adolescence with a mean age of 21.0 +/- 8.9 years. The ages of onset were 12.5 +/- 0.5 years for renal WD and 25.3 +/- 2.4 years for psychiatric WD. The common initial symptoms were neurologic and hepatobiliary. In addition, hematologic and renal disorders were also common during evaluation. The neurologic findings at the time of diagnosis were tremors (66.2%), dysarthria (56.3%), gait disturbances (46.5%), dystonia (42.3%) and decreased facial expressions (40.8%). Less frequent but notable neurologic presentations were psychosis (11.3%), epileptic seizures (5.6%) and hypokalemic periodic paralysis (1.4%). When compared with two previous large Chinese series, the present data show a male preponderance, an earlier age of onset for males and higher incidences of hepatic, hematologic and renal involvement. The possible reasons for the discrepancies between the present study and previous Chinese series are discussed.

Adolescent↗
PubMed1992

Resolution of cerebral white matter lesions following long-term penicillamine therapy for Wilson's disease: report of a case.

Although lenticular gray matter lesions in Wilson's disease (WD) may resolve following long-term decoppering therapy, response of cerebral white matter lesions to such a treatment has not been reported. A patient with WD developed dystonia of the left hand and focal seizures involving the left upper limb with occasional generalization. CT disclosed a low density area in the right frontal white matter. Initiation of penicillamine therapy resulted in worsening of clinical manifestations, further extension of the right frontal lesion, and development of a new left parietal lesion. However, after five years of continued penicillamine therapy, clinical improvements were noted, including disappearance of the left parietal lesion and almost complete resolution of the right frontal lesion. The present case suggests that cerebral white matter lesions in WD may also respond to long-term chelating therapy.

Adult↗
PubMed1992

[Continuous arteriovenous hemodialysis in critical patients with acute renal failure].

Continuous arteriovenous hemodialysis (CAVHD) offers a modified therapeutic approach for the patient with acute renal failure. The system is modified fron the CAVH write out method by adding two pumps to control the flow rate of the dialysis solution and to reduce the nursing load. The blood flow through the dialyzer is dependent on the net blood pressure gradient. Peritoneal dialysis or bicarbonate dialysate is infused through the dialysate ports utilizing both diffuse and convective transport for an average blood flow rate of 0.9 L/hour. The two pumps control the dialysate inflow and outflow rates. Ten patients with complications and acute renal failure were treated with CAVHD for periods ranging from 10 to 154 hours. Average urea clearance was 9.39 mL/min. Average creatinine clearance was 9.12 mL/min, and in stable patients, the mean BUN was maintained at 60 mg/dL and the mean serum creatinine level was 4.6 mg/dL. The average ultrafiltration rate obtained was 100 mL/hr and was adjusted for the body fluid condition. most patients tolerated CAVHD without further hemodynamic instability and did not develop serious complications. In conclusion, CAVHD is a safe and technically simple procedure, which is particularly suitable for hemodynamically unstable patients requiring fluid removal.

Acute Kidney Injury↗
PubMed1992

Hypothalamic amenorrhea in a case of mitochondrial encephalomyopathy.

A 26-year-old female with myoclonus epilepsy associated with ragged-red fibers is reported. Clinically, she had myoclonus epilepsy, cerebellar ataxia, a bilateral neurosensory type hearing loss, retinitis pigmentosa and short stature. She also presented with primary amenorrhea and poor development of secondary sexual characteristics. Endocrinologic studies revealed that hypothalamic dysfunction was the most plausible cause of her primary amenorrhea. Magnetic resonance imaging showed marked dilatation of the third ventricle indicating thalamic and hypothalamic degeneration. We conclude that hypothalamic dysfunction may be one of the characteristic features of mitochondrial encephalomyopathies.

Adult↗
PubMed1992

Assessment of the association of HLA-DR 3/4 heterozygotes with diabetes mellitus and non-diabetic diseases.

To evaluate the disease association with HLA-DR 3/4 heterozygotes, 1,074 subjects, who had been analyzed consecutively for HLA-DR antigens for organ transplantation or to study the disease association with HLA from June 1984 to June 1986, were enrolled in this study. Of these subjects, 278 had diabetes, 168 were healthy controls or donors, and 628 had other diseases. Of the 1,074 subjects, 35 subjects (3.2%) were DR 3/4 heterozygotes and 1,039 subjects (96.7%) were non-DR 3/4 heterozygotes. Among the 35 DR 3/4 positive subjects, 23 were diabetic (65.7%), two were healthy donors (5.7%), 10 had other diseases (28.5%) such as recurrent abortion (n = 3), hepatoma (n = 2), Graves' disease (n = 1), idiopathic hypoparathyroidism (n = 1), IgA nephropathy (n = 1), uveitis (n = 1) and gout (n = 1). Among the 23 DR 3/4 positive diabetics, 19 (82.6%) had insulin-dependent diabetes mellitus (IDDM), three (13.0%) had non-insulin-dependent diabetes mellitus (NIDDM), and one (4.3%) had maturity onset diabetes of the young (MODY). When these DR 3/4 positive diabetics were compared with the other disease and control/donor groups, significant increases in the relative risk were seen for IDDM patients (RR = 32.61, 43.80, respectively, p < 0.001). No significant association could be seen for NIDDM and MODY patients. In those non-diabetic patients positive for DR 3/4, there was no significant association with DR 3/4 heterozygotes. These findings suggest that: 1) DR 3/4 positive subjects are highly associated with IDDM; and 2) there is no significant association of DR 3/4 with NIDDM, MODY and other non-diabetic diseases.

Adult↗