Search PubMed⌕ Search

Biomedical subjects

C Y Lin

Publications and source records attributed to C Y Lin.

At least 541 records · Page 30Linked to original sources
PubMedDate not supplied

High-dose intravenous gamma-globulin therapy in Kawasaki disease.

The efficacy for reduction of coronary aneurysm in Kawasaki disease was studied from 1984 to 1988 in Taiwan. One hundred and six children with Kawasaki disease were treated by one of the following regimens: regimen I: aspirin and 130-200 mg/kg/day of intravenous gamma-globulin (group I = 7), regimen II: 201-400 mg/kg/day of intravenous gamma-globulin with aspirin (group II = 49) and regimen III: aspirin alone (group III = 43) and no treatment (group IV = 7). By using two-dimensional echocardiography and aortography, the coronary arterial aneurysms noted in group I, II, III and IV were 42.9%, 49.0%, 44.2% and 16.7% respectively within 4 weeks of the illness and were 28.6%, 18.4%, 16.4% and 16.7% respectively during the follow-up period of 11.4 +/- 8.2 months. The incidence of coronary aneurysm was reduced significantly (p less than 0.005) in patients with high-dose gamma-globulin therapy and with aspirin therapy alone. However, there was no difference between group II and III, probably due to delays in the time of start of prophylactic gamma-globulin therapy. There was also significant lower incidence of the giant coronary aneurysm in children with high dose gamma-globulin therapy and with aspirin therapy. (p less than 0.05) The incidences of giant aneurysm in groups I, II, III and IV were 28.6%, 2.0%, 4.7% and 14.3% respectively. These results suggest that even with delay in the time of start of prophylactic gamma-globulin therapy, it still can reduce the formation of giant coronary aneurysm.

Adolescent↗
PubMedDate not supplied

Neonatal lupus erythematosus: report of one case.

A female newborn of SLE mother developed transient typical discoid-like lupus skin lesions over her face soon after birth and had severe relapse with generalized spreading following an episode of upper respiratory tract infection at 50 days of age. Blood picture showed anemia, transient thrombocytopenia and high ESR. Cardiac echo disclosed small ASD with minimal TR. Both EKG and 24 hrs EKG monitor presented normal findings. Serological studies at the early relapse stage of this disease showed increased serum ANA, IgA and IgM level with normal IgG and decrease of C3 and C4. Both Ro(SSA) and La(SSB) antibody systems were positive in mother but only positive for La(SSB) antibody system in this baby. The alpha-anticardiolipin antibody was negative. We suggest that the Ro(SSA) and/or La(SSB) antibody systems may play a role in the pathogenesis of neonatal lupus erythematosus.

Adult↗
PubMedDate not supplied

Childhood nephrotic syndrome and heavy proteinuria in Taiwan. A retrospective clinicopathologic study.

From April 1981 to November 1987, 347 children with either nephrotic syndrome (NS; 262 cases, 75.5%) or heavy proteinuria (85 cases, 24.5%) were studied to determine the clinicopathologic manifestation of these diseases among Taiwanese children. All of the children were less than 18 years of age and all had undergone renal biopsy. IgM mesangial nephropathy (IgMN; 93 cases, 26.9%) and minimal change nephrotic syndrome (MCNS; 62 cases, 17.8%) are the most frequently found pathologic lesions. The clinical course of MCNS is always responsive to steroids and less relapsive. However, IgMN is characterized by its good initial response to steroids and frequent relapses. As for secondary glomerulonephritis, lupus nephritis has the first position and comprises 18.4% (64 cases) of all cases. Membranous nephropathy associated with hepatitis B antigenemia (HBVMN; 34 cases, 9.8%) has the second position. Most cases of membranous nephropathy in children in Taiwan are HBVMN. The age of peak incidence is around 2-7 years old. Most of them had frequent relapses of NS or persistent heavy proteinuria. Membranoproliferative glomerulonephritis (MPGN) accounts for only 1.2% (4 cases) of all cases, relatively lower than reported elsewhere. The high incidence of IgMN, HBVMN and low incidence of MPGN are probably due to geographic or racial differences in Taiwan with respect to those in other countries.

Adolescent↗
PubMedDate not supplied

Persistent histological and immunological abnormalities in congenital syphilitic glomerulonephritis after disappearance of proteinuria.

A 40-day-old male infant suffered from generalized anasarca, proteinuria and hematuria. Serologic study revealed marked elevation of antibody titer against Treponema pallidum, the same serologic finding was also noted in both his parents. Two weeks of treatment with penicillin was given. The edema subsided 7 days later, and proteinuria and hematuria disappeared 20 days after the initiation of penicillin treatment. At the age of 60 days, all the clinical symptoms and signs vanished; urinalysis and renal function were all within normal limits and renal biopsy was performed. Pathologic study revealed membranous glomerulonephritis with deposition of IgG, IgM and complement C3, C1q, C4 and treponema antigen in the subepithelial area of the glomerular basement membrane. These phenomena suggest that treponemal antigen-antibody complexes were deposited in the glomeruli and activated the classic pathway of complements, leading to an immune complex nephritis. Immunologic study revealed that the patient had high circulating helper T cells (CD4 cells), low level of suppressor T cells (Leu2+15+ cells) with high CD4/CD8 ratio. It was accompanied by detectable circulating immune complex and high titer of T. pallidum hemagglutinin antibody titer. This change suggests that abnormal immune regulation may play an important role in the development of syphilitic glomerulonephritis.

Glomerulonephritis↗
PubMedDate not supplied

Purpura nephritis in Chinese children from northern Taiwan.

From Jan. 1976 to Feb. 1989, 101 pediatric patients were diagnosed as Henoch-Schönlein purpura in Veterans General Hospital Taipei, 35 (35%) of whom were found to have purpura nephritis by both urinalysis and renal biopsy. All of them have been followed up for more than 4 months to 8 years. During this period, 7 (20%) of patients progressed to renal insufficiency or chronic renal failure, while 20 cases (57%) recovered from the renal disease completely, and the remaining 8 (23%) had urinary abnormalities till the last follow-up. The patients who had older onset age, who had acute nephritic syndrome, nephrotic syndrome as their initial renal manifestations or who had poor grading in renal pathology, had a relatively poor prognosis. Although most cases recovered completely from renal disease, a substantial minority of cases will have long-term renal morbidity. It is mandatory to combine clinical data, biopsy findings and close follow-up data in the management of pediatric purpura nephritis. The disease cannot be viewed as a benign disease in Chinese children.

Age Factors↗
PubMedDate not supplied

Type I primary hyperoxaluria associated with type I renal tubular acidosis.

An 8-year-old boy who had suffered from recurrent stone formation since the age of 4 years, was admitted as an emergency due to anuria for a half day on November 20, 1986. Kidney-ureter-bladder film showed that the urethra was obstructed by a stone, and emergent cystoscopy was performed to remove it. He is the product of consanguinous marriage, his parents being first cousins. There was no family history of renal stone. Laboratory investigations showed hypokalemic, hyperchloremic metabolic acidosis. The ammonium chloride loading test revealed inability to acidify the urine and a markedly decreased excretion of titrable hydrogen ion and ammonium ion in the urine. These results indicate that this is a case of Type I renal tubular acidosis. His 24-hour urinary excretion of oxalate and glyoxylate were also markedly increased. There were no underlying causes leading to the development of secondary hyperoxaluria. These results also establish the diagnosis of Type I primary hyperoxaluria. The patient then received regimens of Polycitra 1ml/kg/day and Vitamin B6 50mg/day for 4 months. However, urinary stone developed again in this patient 4 months later. To our knowledge, Type I primary hyperoxaluria in association with Type I renal tubular acidosis has not been previously reported.

Acidosis, Renal Tubular↗
PubMedDate not supplied

T cell subsets in glomerulonephritis.

Abnormal lymphocyte function has been postulated to have a pathogenetic role in nephrotic syndrome. In an attempt to investigate the pathogenetic role of lymphocyte subsets in human glomerular disease, we studied 110 children suffering from nephritis during the acute nephrotic phase or nephritis without steroid treatment, 4 weeks later after steroid treatment, in remission and relapse. These patients included minimal change nephrotic syndrome (MCNS) 15 cases, focal segmental glomerular sclerosis (FGS) 6 cases, mesangial cell proliferative nephropathy (MesPGN) 42 cases, membranoproliferative glomerulonephritis (MPGN) 2 cases, hepatitis B surface antigenemia associated with membranous nephropathy (HBVMN) 10 cases, IgA mesangial nephropathy (IgAN) without nephrotic syndrome 7 cases, poststreptococcal glomerulonephritis (PSGN) 24 cases and chronic glomerulonephritis (CGN) 4 cases. There was no significant difference in the total lymphocyte count of each different pathological group of nephritis except that lymphopenia was noted in the CGN patients. When the lymphocyte phenotypic profile was examined, OKT8 cells were significantly increased in the MesPGN patients and both OKT4 and OKT8 cells were significantly increased in HBVMN. Comparison of MCNS and MesPGN during the acute nephrotic phase showed the OKT4/OKT8 ratio decreased significantly in MesPGN. Four weeks after steroid treatment, OKT4 cells decreased both in MCNS and MesPGN being pronounced in MCNS. In the remission stage with steroid treatment the OKT4/OKT8 ratio decreased in MCNS and was mildly elevated in MesPGN. In relapse, the OKT4/OKT8 ratio was the same as it was during the onset of nephrotic phase. MCNS cases were steroid responsive whereas in MesPGN there were frequent relapses or partial steroid response.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMedDate not supplied

A chemotactic inhibitory factor in the minimal change nephrotic syndrome.

This study attempts to investigate the role of neutrophil in the increased incidence of infection in nephrotic syndrome. We investigated the function of neutrophils in the various categories of nephritis and the relationship to the response of steroid therapy in the nephrotic syndrome. We used peripheral blood for the study of chemotaxis in 62 children suffering from nephritis in the acute phase and in remission. These patients included minimal change nephrotic syndrome (9 cases), focal segmental glomerular sclerosis (3 cases), mesangial cell proliferative nephropathy (23 cases), Hepatitis B antigenemia associated membranous glomerulonephropathy (4 cases), poststreptococcal glomerulonephritis (20 cases) and chronic glomerulonephritis (3 cases). The chemotactic index was significantly increased in normal range in the remission stage. The molecular weight of the chemotactic inhibitory factor was estimated to be about 89,000 using Sephacryl S-200 column. All cases of mesangial proliferative nephropathy had nephrotic syndrome with frequent relapses or steroid resistance. These results suggest that the chemotactic index may serve as an important parameter between steroid responsive and non-responsive nephrotic syndrome.

Adolescent↗
PubMedDate not supplied

Peritonitis in children being treated with continuous ambulatory peritoneal dialysis. CAPD Team.

An eight-year retrospective study was performed to determine the incidence of peritonitis in a pediatric continuous ambulatory peritoneal dialysis (CAPD) population of 24 children, half of whom were boys and half, girls. All suffered from end stage renal disease (ESRD). When these children, aged 2 through 17 years (mean: 10.7 +/- 3.8), were examined, the incidence of peritonitis was one episode every 15.2 patient-months. Microbiologic evaluation showed that 76.4% of the 34 episodes were culture positive, with Staphylococci species (coagulase negative staphylococci 32.4%, Staphylococcus aureus 14.7%) causing most cases especially early in dialysis. Half the patients presented with a triad of symptoms (fever, abdominal pain and cloudy dialysate), with cloudy dialysate was the major presentation (88%). Peritonitis was treated with intraperitoneal administration of cefacin and/or netromycin when suspected, and 52.9% of the episodes needed hospitalization. Except for two patients who died of complications (sepsis, acute pancreatitis), all episodes of peritonitis were cured; in four episodes it was necessary to remove a catheter, and two of those cases came from fungal peritonitis. Peritonitis rates differed among disconnect systems. The manual spike had peritonitis rate of one episode per 4.6 patient-months which was higher than the O-set (one episode/22.2 patient-months), UV-XD and Y-set disconnect systems. Therefore, the major causes of peritonitis arose from contamination provoked by the technical aspect of the procedure. Nutrition status was stable in these patients. Serum albumin and total protein were adequate in all patients without relation to episode of peritonitis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMedDate not supplied

Effects of fluid retention on the measurement of body composition using bioelectric impedance.

Body composition measurements using bioelectrical impedance analysis (BIA) were performed on 50 uremic patients immediately before and after hemodialysis (HD) therapy, on 10 uremic patients under continuous ambulatory peritoneal dialysis (CAPD) therapy before and after the fluid was drained out of the peritoneal cavity and on 3 cirrhotic patients before and after abdominal paracentesis. Thirty-two pairs of measurements were performed on the CAPD patients and 3 pairs on the cirrhotic patients. Significant increases in both resistance and reactance were noted after HD. However, resistance and reactance before and after peritoneal fluid had been drained were not significantly different. In HD, body fluid loss as estimated by BIA (LBIA) was higher than that from the body weight method (LBW). The relationship between the two is described by the equation LBW = 0.841LBIA + 0.588. The total body fat estimated before HD was significantly lower than after HD, and it was significantly higher when measured with peritoneal fluid retention than with fluid drained. Generalized edema caused an increase in resistance and reactance, while local peritoneal fluid retention did not cause any significant changes. When total body fluid is measured by the BIA method, it is often overestimated in the case of generalized edema and underestimated in case of peritoneal fluid retention. Therefore, total body fat is underestimated by BIA in generalized edema before HD and overestimated in peritoneal fluid retention.

Adolescent↗
PubMedDate not supplied

Plasma levels of atrial natriuretic factor in normal children and patients treated by hemodialysis and continuous ambulatory peritoneal dialysis.

In an attempt to establish the reference data of plasma levels of atrial natriuretic factor (ANF) in different age groups and to evaluate plasma ANF changes before and after hemodialysis (HD) and during continuous ambulatory peritoneal dialysis (CAPD), fifty normal healthy children and 20 children with end-stage renal disease (ESRD) were included. Ten ESRD children received HD and 15 received CAPD therapy. Five cases received HD in the earlier period and then CAPD. The results showed that in normal children, at the age of under one month, there were higher plasma ANF levels. As age increased, plasma ANF levels progressively decreased. During the ESRD before dialysis, there was a significantly increased plasma ANF level (434.1 +/- 80.1 vs 17.5 +/- 2.3 pg/ml, P < 0.0001). Both HD and CAPD reduced the plasma ANF of patients to a lower degree, but the result was still higher than that in normal controls. There were significantly decreased plasma ANF level in the patients with CAPD therapy than those with HD (71.8 +/- 13.7 vs 270.3 +/- 51.9 pg/ml, P < 0.0001). The change of ANF levels was positively correlated with the body weight change and creatinine change after HD.

Adolescent↗
PubMedDate not supplied

Serum and urinary interleukin-6 (IL-6) levels as predicting factors of Kawasaki disease activity.

From January 1988 to Autumn 1991, 60 patients suffering from Kawasaki disease (KD) were recruited in this study. Their ages ranged from 4 months to 5 years. Diagnosis was based on the criteria revised in 1984 by the KD Research Committee in Japan. Of these, 12 cases developed coronary aneurysms. First, blood samples from 60 KD patients were taken on admission before aspirin and/or intravenous immunoglobulin (IVIG) treatment. Convalescent blood samples were taken 3 months after onset of disease. The control group included (1) 10 cases of viral infection with skin rash and fever (aged 5 months to 5 years) and (2) 10 age and sex matched normal children admitted for elective pediatric surgery such as inguinal hernia. Second, urinary samples were collected from 32 cases during the acute phase of KD. Of these, 10 cases had pyuria and/or proteinuria. The results showed that the serum IL-6 levels from KD patients during the first week of acute phase were significantly increased while undetectable in the convalescent sera and controls. There was also a statistical difference between the with and without coronary aneurysm groups during the first week (336.8 +/- 95.1 vs 125.5 +/- 56.5 pg/ml, P < 0.001). Urinary IL-6 levels were significantly elevated in KD patients with pyuria and/or proteinuria (156.6 +/- 77.7 pg/mg Cr) and undetectable in the group without pyuria and proteinuria and controls during the first week. There was no difference between with and without coronary aneurysm. These results suggest that serum IL-6 level is a useful factor for predicting formation of coronary aneurysm even within one week after onset of disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Biomarkers↗
PubMedDate not supplied

Molecular study on the infantile form of Pompe disease in Chinese in Taiwan.

Glycogen-storage disease type II, Pompe disease, is caused by the deficiency of acid alpha-D-glucosidase in lysosome. Previously we found that acid alpha-D-glucosidase did exist in the skin fibroblasts and there was also no difference of mRNA in quantity and size of Chinese infantile type Pompe disease patients in Taiwan. However, functional assay of the acid alpha-D-glucosidase of these patients showed its enzyme function to be defective. In the present study, first we identified a substitution site in four Chinese infantile patients with Pompe disease which is a cytidine to adenosine (C1935-->A) transversion at 5' end of exon 14 causing substitution of glutamic acid for aspartic acid at position 645 of the acid alpha-D-glucosidase. This substitution was introduced in wild-type cDNA and expressed in COS-1 cells. The Asp-645-->Glu substitution resulted in significant reduction of acid alpha-D-glucosidase activity. Second, according to the screening data in 25 Chinese Pompe disease patients using digestion of RT-PCR amplified specific fragment with Aat II, the restriction fragment length analysis showed that patients presented the 861 bp band and the normal individuals presented the 728 bp and 133 bp polymorphic bands. We found that the frequency of mutant allele is 0.8 in infantile patients with Chinese Pompe disease and 0 in normal individuals. These results therefore indicate that Asp-645-->Glu mutation results in infantile form of Pompe disease as the major cause in Chinese patients in Taiwan.

Alleles↗
PubMedDate not supplied

Early detection of neonatal adrenal hemorrhage by ultrasonography.

To investigate clinical features and course of neonatal adrenal hemorrhage (NAH) from July 1992 to August 1993, fifteen babies suffering from NAH born at Veterans General Hospital-Taipei were included. Portable ultrasound scanner was used for initial screening. The frequency of NAH was approximately 0.35% which is lower than other reports. The distribution of their birth weight was 3 cases lower than 3000 gm, 3 cases over 4000 gm and 9 cases within 3000 to 4000 gm. Only one was premature and another one post-term. Four of them had a history of either prenatal or perinatal asphyxia. Only one patient had a palpable abdominal mass. On the sonographic findings, eleven had bleeding in the right-side, two in the left-side and two in both sides. Only one had adrenal calcification. During their early life, no patient developed adrenal cortical insufficiency or secondary profuse bleeding. Two patients had pathologic jaundice. Two patients had severe anemia. After conservative treatment, none of them developed any severe complication. In conclusion, with the aid of renal sonoscreening in the newborn, we can discover NAH early and give adequate treatment at once. Severe complications also can be prevented.

Adrenal Gland Diseases↗
PubMedDate not supplied

A study of the relationship between IgG subclass/IgM and idiopathic nephrotic syndrome.

In an attempt to elucidate the relationship between serum IgG subclass/IgM ratio and prognosis in different types of idiopathic nephrotic syndrome (INS), 46 cases of treatment responders and 23 cases of either frequent relapse or non-responders were studied. All patients had received renal biopsy. Serum IgG subclass/IgM ratios were compared between the acute nephrotic phase and remission. The association between therapeutic effect and the parameters of INS were also investigated. There were 24 cases of minimal change nephrotic syndrome (MCNS), 32 cases of IgM nephropathy (IgMN) and 13 cases of focal segmental glomerular sclerosis (FSGS). All cases of MCNS were steroid-responders. Seventeen cases of IgMN experienced frequent relapses. The other 15 cases were responsive to the treatment. 6 cases of FSGS had poor response to any therapy; 7 cases were responsive to either triple therapy or cyclosporine A plus prednisolone. Both IgGI/IgM and IgG2/IgM were correlated with serum albumin and cholesterol levels at the acute nephrotic phase (p < 0.05). Longitudinal change of the immunologic parameters in patients with INS revealed significantly elevated serum IgM level decreased serum IgG level, IgG/IgM, IgG1/IgM, IgG2/IgM, IgG3/IgM, IgG4/IgM and IgG/IgE ratio during acute nephrotic phase. Correlation between therapy responses showed that, for serum IgG1/IgM ratio > 3.0, most of the cases were treatment-responders. For IgG1/IgM ratio > 1.0, all the cases were frequent relapsers or non-responders. These results suggest the immunologic changes in patients of INS may be caused by immune regulatory abnormality. Serum IgG1/IgM ratio may serve as one of the therapeutic and prognostic guides, especially if the patient refuses renal biopsy.

Child↗
PubMedDate not supplied

Neonatal thyroid function is unaffected by single treatment with different preparations of povidone-iodine on a wide skin surface.

Povidone-iodine (PV-I) are commonly used for disinfection in hospitals. Previous investigators had proved that iodine can be absorbed from the skin. In an attempt to determine the effect of single treatment of various PV-I preparations on thyroid function in fullterm newborns, serial measurements of serum thyroxine (T4) and Thyroid stimulating hormone (TSH) were done in 48 neonates treated with one dose antiseptics (tincture PV-I, aqueous PV-I, tincture PV-I followed with 75% alcohol and no treatment in control group). The results revealed no significant change of thyroid function in any study group. In conclusion, PV-I with different preparations, aqueous or tincture, did not significantly influence neonatal thyroid function if they were used to a fullterm neonate only once and even to a wide skin surface.

Anti-Infective Agents, Local↗
PubMedDate not supplied

DiGeorge syndrome with microdeletion of chromosome 22q11.2: report of one case.

DiGeorge syndrome (DGS) is a congenital anomaly involving developmental defects of the third and fourth pharyngeal pouches. Thymic aplasia or hypoplasia, parathyroid aplasia or hypoplasia, cardiac malformations, and dysmorphic facies are characteristics features. We present a case which had thymic aplasia, hypocalcemia, facial dysmorphism (hypertelorism, low set ears, cleft of soft palate, fish-like mouth and micrognathia) and congenital heart disease (ventricular septal defect, perimembranous type). The T-cell immunologic functions as a percentage of T-cell and phytohemagglutinin stimulation test were within normal range matched with age. Molecular study showed microdeletion of chromosome 22q11.2 by genotype analysis, but chromosome study of high-resolution cytogenetic analysis by G-banding technique was normal. To our knowledge, about 90% of DiGeorge syndrome patients show chromosome abnormalities, most involving chromosome 22 (monosomy of 22q11.2). In the past, most cases were proven by high-resolution cytogenetic analysis or fluorescence in situ hybridization(FISH). We report a case of DGS in Taiwan with microdeletion of chromosome 22q11.2 detected by genotype analysis.

Chromosome Deletion↗