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Biomedical subjects

C T Peng

Publications and source records attributed to C T Peng.

At least 73 records · Page 4Linked to original sources

Community-acquired Pseudomonas aeruginosa bacteremia and sepsis in previously healthy infants.

Pseudomonas aeruginosa bacteremia, or sepsis, often occurs in hospitals, affecting mainly children with underlying problems. However, it can also appear in communities, and affects infants and children without underlying diseases. We report eight cases of Pseudomonas aeruginosa bacteremia, or sepsis, in previously healthy infants over a three-year period. All patients were less than twelve months old and the majority presented with sepsis, diarrhea, ecthyma gangrenosum, and neutropenia. The infection route may have been the gastrointestinal tract. Concomitant gastrointestinal infections may have played a role in pathogenesis.

Bacteremia↗

An R248C mutation of FGFR3 leading to thanatophoric dysplasia type I.

Thanatophoric dysplasia (TD) is the most common form of lethal neonatal dwarfism with micromelic shortening of the limbs, macrocephaly, platyspondyly, and reduced thoracic cavity. R248C mutation in the extracellular domain of fibrobast growth factor receptor 3 (FGFR3) was common in TD type I. Two TD type I patients were examined for R248C mutation by use of restriction digestion and direct sequencing. The results showed that both patients carried R248C mutation. Because of the homogeneity of R248C mutation among different ethnic populations, all TD patients should be analysed using this PCR-based method presented in this work.

Humans↗

Chronic lung disease in extremely low birth weight infants: a two-year retrospective analysis.

To determine the incidence and classification of chronic lung disease (CLD) in extremely low birth weight (ELBW) infants, a 2-year retrospective analysis was performed. From January 1997 to December 1998, 117 infants weighing less than 1000 g were enrolled. The survival rate beyond 28 days was 60.7% (71/117). CLD was defined as a supplemental oxygen requirement at 28 days of age, with symptoms of persistent respiratory distress and chest radiograph showing characteristic appearance. In addition to the common finding of CLD, infants with bronchopulmonary dysplasia (BPD) had history of respiratory distress syndrome (RDS), infants with Wilson-Mikity syndrome (WMS) had no RDS but had early appearance of bubbly lung on chest x-ray, and infants with chronic pulmonary insufficiency of prematurity (CPIP) had only hazy appearance on chest x-ray. The incidence of CLD in infants who survived beyond 28 days was 50.7% (36/71). Among the 36 infants with CLD, 17 (47%) had BPD, 4 (11%) had WMS and 15 (42%) had CPIP. The median (min, max) days of mechanical ventilation were 45 (9, 112), 45.5 (45, 50) and 7.5 (0, 40) days in BPD, WMS and CPIP groups, respectively. The median (min, max) days of oxygen requirement were 73 (28, 120), 149 (70, 211) and 52.5 (38, 90) days, respectively. The infants still requiring oxygen at post-conceptional age of 36 weeks are significantly more in BPD (14 (82.4%)) and in WMS (4 (100%)) than in CPIP (3 (20%)). Two (1 BPD, 1 WMS) were discharged and received oxygen therapy at home. Four infants with BPD died of respiratory failure. CLD includes a wide range of conditions, from BPD or WMS with severe respiratory morbidity and mortality to no residual problems. Such information is important for design of appropriate strategies to prevent CLD.

Bronchopulmonary Dysplasia↗

3-hydroxy-3-methylglutaric aciduria presenting with Reye like syndrome: report of one case.

We report the case of a patient with 3-hydroxy-3-methylglutaric aciduria who presented with a repeat attack of Reye like syndrome clinically. Vomiting and somnolence, generalized tonic and clonic convulsions with hepatomegaly, hyperammonemia, liver function impairment, and mild metabolic acidosis were the presenting signs. 3-hydroxyisovaleric, 3-methylglutaric, 3-methylglutaconic and 3-hydroxy-3-methylglutaric acids were detected in the urine by gas chromatography-mass spectrometry. 3-methylglutarylcarnitine was also identified in the urine by fast atom bombardment and tandem mass spectrometry. Therefore, the possibility of metabolic disease should be considered in neonates and infants with repeat attacks of Reye like syndrome and a history of similarly affected siblings.

Female↗

Treatment results of the TPOG-NHL92 protocols for childhood non-Hodgkin's lymphomas in Taiwan: a report from the Taiwan Pediatric Oncology Group (TPOG)

A nation-wide chemotherapeutic trial for childhood non-Hodgkin's lymphoma (NHL) was conducted by the Taiwan Pediatric Oncology Group (TPOG). Four TPOG-NHL92 protocols based on stage and histology were activated in 1992: TPOG-92LD (treatment duration: 8 months) was used for localized (stages I/II) NHL with any histology, 92LB (2 years), 92SNC (5 months), and 92LC (1 year) for advanced (stages III/IV) lymphoblastic (LB), small non-cleaved cell (SNC), and large cell (LC) lymphoma, respectively. From January 1992 through June 1998, 200 children with newly diagnosed NHL from 13 member hospitals of TPOG were enrolled. There were 140 boys and 60 girls. Their ages at diagnosis ranged from 2.4 months to 18.3 years with a median of 8.2 years. There were 54 (27.3%) patients with LB, 94 (47.5%) with SNC including B-cell acute lymphoblastic leukemia (B-ALL), and 50 (25.2%) with LC. Stages I, II, III, and IV (including B-ALL) of the disease comprised 5%, 10%, 43%, and 42% of cases, respectively. There were 176 patients eligible for evaluation of treatment results. The remission rate of induction was 82.4%, induction failed in 22 (12.5%) patients, and nine patients died during induction. As of August 31, 1999, 26 patients relapsed, six died during remission, one patient developed secondary acute myelomonocytic leukemia, and 105 patients remained in continuous remission with a median remission duration of 49 months. The event-free survival (EFS) at 7 years was 63.5%, 61.5% and 65% for LB, SNC, and LC groups (P = 0.8298). The 7-year EFS for stages I/II, III, and IV of the disease was 73%, 68.9%, and 50.3% (P = 0.0212), respectively. We concluded that following the strategy of stratification of therapy, only disease stages had prognostic significance in this study. More efforts are needed to improve our treatment results.

Adolescent↗

Risk factors of pulmonary hemorrhage in very-low-birth-weight infants: a two-year retrospective study.

Pulmonary hemorrhage is a serious complications in very-low-birth-weight (VLBW) infants with respiratory distress syndrome (RDS). We undertook a 2-year retrospective study to investigate the predisposing factors and the incidence of pulmonary hemorrhage in VLBW infants. From January 1997 through December 1998, twenty infants were diagnosed with massive pulmonary hemorrhage (MPH) according to the following criteria: active bleeding from the endotracheal tube, acute drop in hematocrit (> or = 10%), and the development of multilobar infiltration on chest radiograph. The mean gestational age was 26.9 +/- 2.5 weeks, the mean birth weight was 909 +/- 290 g. Twenty historic controls with similar gestational age and birth weight were retrospectively identified during the study period. The incidence of MPH in VLBW infants was 5.9%(20/340). A lack of prenatal corticosteroid administration, surfactant replacement therapy for RDS, and a patent ductus arteriosus (PDA) with cardiovascular dysfunction requiring dopamine support were the significantly predisposing factors of MPH in the acute stage (< or = 7th day of life). To avoid MPH and decrease mortality and morbidity in the acute stage, prenatal corticosteroid administration, evaluation of the necessity of surfactant therapy, and early recognition and aggressive treatment of hemodynamically significant PDA were necessary.

Adrenal Cortex Hormones↗

Bacterial infection in patients with transfusion-dependent beta-thalassemia in central Taiwan.

The microorganisms, outcome of infections and the risk factors were evaluated in 39 patients with beta-thalassemia who received frequent blood transfusions. Among these patients, thirteen developed 22 episodes of infections, and bacteremia accounted for 72.7% (16/22) of all infections. Three patients developed meningitis, two patients had liver abscesses, three patients had soft tissue infections, one patient had a urinary tract infection and one patient had lobar pneumonia. Interestingly, a large proportion of the patients were infected by Gram-negative bacteria. Patients who were implanted with intravascular catheters were most susceptible to bacterial infection (1.70 episodes/patient) (P = 0.0069). So were patients with ferritin levels over 2,000 ng/mL (1.18 episodes/patient) (P = 0.028). The frequency of bacterial infections in patients with splenectomies (1.08 episode/patient) was also significantly higher than that of the average patient (P = 0.025). In conclusion, three major risk factors for bacterial infection were identified in this group of patients: intravascular catheterization, high serum ferritin levels (> or = 2,000 ng/mL) and splenectomy. The infection rate of these patients (0.45 episode/100 patient-year) is about 20-fold higher than that of general pediatric patients (0.023 episode/100 patient-year).

Adolescent↗

Pitfalls of PCR-based genotyping in patients with 21-hydroxylase deficiency.

Mutation analysis of CYP21A2 gene was performed in seven patients with congenital adrenal hyperplasia (CAH) by combining differential long template polymerase chain reaction (PCR) amplification and amplified created restriction site (ACRS) methods. All mutations were identified, including five alleles of deletions, three alleles of splicing (IVS2-12[C/A] > G), four alleles of Ile172Asn, and two alleles of Arg356Trp. During the course of genetic analysis of CYP21A2, we found that misgenotyping of CAH by PCR-based method is possible if both alleles of a CAH patient were deletion mutations and at least one of them carried a CYP21A1P-CYP21A2 fusion gene. We also found a patient's mother was misgenotyping as IVS2-12[C/A] > G homozygous due to "allele dropout" in the PCR amplification process. We present in this article evidences of mis-genotyping by PCR-based amplification method. Due to the pitfalls observed in this study, we recommend that more methods, including microsatellite linkage analysis and direct sequencing, should be performed with direct amplification of known mutations in prenatal diagnosis of CAH to avoid misdiagnosis.

Adrenal Hyperplasia, Congenital↗

Persistent pulmonary hypertension of the newborn: echocardiographic assessment.

Twenty seven newborn infants with persistent hypoxemia in the first 3 days after birth were enrolled for hemodynamic assessment using echocardiography. Measurements included pulmonary arterial pressure (peak velocity of tricuspid regurgitation (TR), patent ductus arteriosus (PDA) flow pattern, interatrial shunting flow pattern and pulmonary flow velocity ratio (the time to peak velocity/right ventricle ejection time ratio (TPV/RVET)) and left ventricular ejection fraction. The estimated systolic pulmonary arterial pressure and the systemic arterial pressure determined via an indwelling arterial line were recorded at the time of echocardiographic examination, and pulmonary arterial pressure/systemic arterial pressure was calculated. Nineteen infants (70.4%) had a TR sufficient to estimate systolic pulmonary arterial pressure. The median value of pulmonary arterial pressure/systemic arterial pressure was 1.02 (range, 0.68 to 1.78). Twenty two infants (81.5%) had a PDA and flow patterns indicating pulmonary arterial pressure above or approaching systemic arterial pressure. All infants had a foramen ovale and flow patterns were bi-directional or pure right-to-left. TPV/RVET had a wide range of values (0.23 to 0.55), and only 44.5% of infants had high pulmonary arterial pressure as reflected by low TPV/RVET ratio. Eleven infants (40.7%) had an ejection fraction below the normal range. Results of 17 survivors were compared with 8 deceased infants (2 infants of birth weight less than 1000 gm were excluded who died of massive pulmonary hemorrhage). There were no significant differences for any parameter of pulmonary arterial pressure, but ejection fraction was significantly lower in deceased infants. This study has demonstrated that it is possible to evaluate pulmonary arterial pressure noninvasively by using echocardiography in most newborn infants with clinical evidence of persistent pulmonary hypertension of the newborn (PPHN). Ejection fraction is an echocardiographic parameter which can significantly predict mortality.

Echocardiography, Doppler↗

Glucose-insulin infusion for the early treatment of non-oliguric hyperkalemia in extremely-low-birth-weight infants.

In order to investigate the status of non-oliguric hyperkalemia and to evaluate glucose-insulin infusion treatment among extremely-low-birth-weight (ELBW) infants, 161 infants weighting less than 1000 gm at birth were enrolled for this study. They were divided into two groups: a hyperkalemic group and a non-hyperkalemic group. Hyperkalemia was defined here as a serum potassium level of greater than 6 mEq/L in a non-hemolyzed arterial blood sample. A glucose-insulin infusion was administered to the patients when hyperkalemia was detected in them during the first few days after birth. The infusion was discontinued when the serum potassium levels had been less than 6 mEq/L and stabilized for 6 hours. The incidence of non-oliguric hyperkalemia among ELBW infants in this study was 58% (93/161). The mean gestational age of neonates was 25.7 +/- 1.8 weeks (hyperkalemic) and 26.6 +/- 1.7 weeks (non-hyperkalemic). The mean rate of increases in serum potassium levels was 0.32 +/- 0.29 mEq/L/hr (hyperkalemic) and 0.13 +/- 0.12 mEq/L/hr (non-hyperkalemic). The incidence of severe intraventricular hemorrhage (IVH) was 19% (18/93) (hyperkalemic) and 4.4% (3/68) (non-hyperkalemic). The incidence of cardiac arrhythmia was 12% (11/93) (hyperkalemic) and 0% (non-hyperkalemic) respectively. Neonates with fewer weeks of gestation at birth and faster increases in serum potassium levels were associated with a more prominent tendency toward hyperkalemia. Hyperkalemia markedly increases the risk of severe IVH and arrhythmia for ELBW infants. A higher glucose infusion rate should be maintained to prevent hypoglycemia following insulin treatment.

Case-Control Studies↗

Niemann-Pick disease type B with ocular involvement: report of a case.

Niemann-Pick disease (NPD) type B was diagnosed clinically and enzymatically in a 5-year-old girl presenting with failure to thrive, hepatosplenomegaly, diffuse interstitial infiltration of both lungs on chest roentgenograms, cherry red spot, and foam cells in the bone marrow aspirate. Intelligence and neurological examination were normal, sphingomyelinase activity was severely deficient in cultured skin fibroblasts. We present the first case of NPD type B in Taiwan and relevant literatures were reviewed.

Child, Preschool↗

[Degenerative changes in neutrophils as an indicator of neonatal sepsis].

Detection of the degenerative changes of neutrophils (D.C.N.) including vacuolization and toxic granulation in peripheral blood smear can be of value in identifying neonates with infection. A prospective study of 264 neonates who required septic work-ups in the first month of life was conducted. Thirty neonates were proved to have sepsis subsequently. The accuracy of the degree of the D.C.N. to predict neonatal sepsis was assessed. Vacuolization and/or toxic granulation in neutrophils varied from 0% to over 50%/100 neutrophils scanned. The higher the degree of D.C.N. the greater was the like-hood of sepsis. The positive predictive accuracy and specificity increased with the degree of D.C.N. from 24.7% to 100% and 70% to 100% respectively, but the sensitivity decreased accordingly. When D.C.N. was less than 10% the chance of sepsis was less than 5%. Most of newborns dying of proven sepsis had higher percentage of D.C.N.. These findings suggest that degree of D.C.N. may be associated with the outcome of the patients. This simple test can be performed easily in all hospital; it does not require special laboratory facilities and provide a valuable adjunct in early detection of severity and outcome of the neonates with sepsis.

Cytoplasmic Granules↗

[Beta-thalassemia major complicated with Vibrio vulnificus septicemia: report of one case].

Vibrio vulnificus is a halophilic Vibrio that has been isolated repeatedly from sea-water and shellfish during the warm months of the year. It's a virulent pathogen for men and is frequently associated with overwhelming infections including sepsis, gangrene of extremities and high mortality rate. We encountered a 13-year-old boy who had a history of beta-thalassemia major with secondary hemochromatosis, suffering from vomiting, diarrhea, fever and hypotension. Physical examination revealed that ecchymosis, bullae and ulceration were noted over the left leg. Vibrio vulnificus was isolated from the blood. Initially, the patient did not respond to the appropriate antibiotics treatment, subsequently surgical debridement was performed. After that, the patient recovered gradually, and discharged home after 17 days of admission. In conclusion, when patients present with sepsis and/or characteristic skin lesion-hemorrhagic bullae, particularly those with thalassemia major, hemochromatosis or underlying liver disease and a history of marine exposure, clinicians should be alerted to this potentially fatal infection and should commence appropriate assessment and treatment immediately.

Adolescent↗

Molecular diagnosis of Gaucher disease type II.

Gaucher disease is a rare autosomal recessive lysosomal storage disorder in Chinese. A mutation at nucleotide 1448C (T-to-C) of the glucocerebrosidase gene is described in type 2 Gaucher disease. This mutation creates a Bcn I site in a polymerase chain reaction (PCR) amplified fragment. This technique was applied to a Chinese infant with type 2 Gaucher disease, and homozygosity for 1448C mutation was proved. This new finding can make prenatal diagnosis and carrier detection possible in Chinese population with type 2 Gaucher disease.

Base Sequence↗

Typhlitis in acute childhood leukemia.

Typhlitis is a necrotizing inflammation of the cecum usually found in acute leukemia patients on chemotherapy in the setting of agranulocytosis. We describe five cases of typhlitis, characterized by fever, abdominal pain, abdominal tenderness and watery diarrhea, occurring during periods of neutropenia. In 4 cases, sonography showed thickening of the cecum wall. Ultrasound appears to offer an easy noninvasive method of diagnosing this potential lethal disease. Four patients were treated successfully with broad-spectrum antibiotics and bowel rest. There was 1 death resulting from septic complication.

Acute Disease↗

Fetal alcohol syndrome with Arnold-Chiari malformation: report of one case.

We report a rare case of fetal alcohol syndrome (FAS) with Arnold-Chiari malformation. The new-born had the typical facial abnormalities associated with FAS and the mother had a history of heavy drinking. CNS malformation, which is rarely seen in FAS, was also presented. The relationship between the developmental defects and maternal alcohol drinking are discussed together with a review of the literature.

Abnormalities, Drug-Induced↗

Effect of erythromycin on feeding intolerance in very low birth weight infants: a preliminary observation.

To investigate the effect of erythromycin on feeding intolerance in very low birth weight infants, from February 1997 to December 1997 twenty infants weighing less than 1500 g, with prolonged intolerance of enteral feeding, were enrolled in this study. The protocol for erythromycin treatment was: a loading dose of 30 mg/kg/day, divided into three portions given every eight hours intravenously for 1 hour over a three day period; then a maintenance dose of 3-5 mg/kg intravenously for one hour once a day was given until full feeding was well established. The assessment of erythromycin effect was the daily net orogastric balance (volume of orogastric tube feeding minus volume of orogastric aspirates). The mean gestational age was 27.1 +/- 2.0 weeks (mean +/- SD) and the mean birth weight was 1025 +/- 196 g. The mean age when erythromycin started was 19.5 +/- 14 days; the mean days after the initiation of erythromycin when orogastric tube feeding could be started and full feeding established were 2.4 +/- 1.1 days and 15.1 +/- 2.2 days, respectively. At the beginning of erythromycin treatment, the net balance of tube aspirates was -4.8 +/- 4.1 ml. The net balance rose significantly to 30.6 +/- 15.3 ml, 92.6 +/- 25.4 ml and 125.3 +/- 18.1 ml at 7, 14 and 21 days after erythromycin treatment, respectively. In conclusion, erythromycin treatment is a safe method to improve intolerance of enteral feeding in very low birth weight infants. It is suggested that the effect of erythromycin on gastrointestinal motility in these infants should be further investigated in the context of a randomized, controlled trial before widespread clinical implementation of this treatment.

Anti-Bacterial Agents↗

Sister chromatid exchange in Chinese newborn infants treated with phototherapy for more than five days.

The frequency of sister chromatid exchange (SCE) was determined in newborn infants given phototherapy for more than five days, then determined again one year later. There were 8 healthy newborn infants and 15 icterus infants; 8 of these were treated with phototherapy for more than 5 days and the other 7 were not. In the follow-up study, 6 treated infants were evaluated. The results revealed that there was an increase in mean SCE frequency of peripheral lymphocytes after phototherapy, which reversed itself one year after treatment discontinuation. Prolonged continuous phototherapy may be responsible for the increase observed in SCEs in Chinese newborn.

Female↗