Biomedical subjects

C S Chi

Publications and source records attributed to C S Chi.

At least 127 records · Page 7Linked to original sources

PubMedDate not supplied

Guillain-Barré syndrome in children: a cooperative study in Taiwan.

Seventy-two children with Guillain-Barré syndrome (GBS), diagnosed at 11 major teaching hospitals in Taiwan during the period 1986-1990, were studied retrospectively. There were 44 males and 28 females ranging in age from 7 months to 15 years. Preceding events could be traced in 61 patients (85%), including antecedent infection in 59 patients and previous vaccination in 2. As well as the consistent pictures of progressive weakness and generalized hyporeflexia, there were sensory complaints (26%), cranial nerve lesions (46%), respiratory failure (14%) and autonomic dysfunction (25%). Motor symptoms reached a maximum within 20 days in 88% of the patients, with the plateau lasting less than 2 weeks in 75%, and became stable within 3 months in 76%. Overall outcome showed complete recovery in 73% of the patients within 6 months after onset. Four (5.6%) had recurrence, and there was no mortality. The present study revealed that the annual incidence of GBS in Taiwan can be estimated roughly as 0.66 per 100,000 and that the course of childhood GBS is relatively benign.

Adolescent↗

PubMedDate not supplied

Oral glucose lactate stimulation test in mitochondrial disease.

We selected 23 patients with neurologic diseases, including 15 boys and 8 girls from 1 month to 10 years of age, who were divided into mitochondrial and nonmitochondrial disease groups. All patients were required to fast and rest for at least 4-10 hours before the test. Glucose was administered orally using a 50% glucose:water solution at a dose of 1.75 mg/kg. Blood samples then were drawn from a retained heparinized venous line at 0, 30, 60, 120, and 180 min and tested for lactate and glucose levels. Of the mitochondrial disease group, 10 of 11 patients had an upward sloping curve of lactate metabolism; the one who had a flat line response was a patient who suffered from a free-fatty-acid metabolic defect presenting with a recurrent Reye-like syndrome. There was a relatively flat line response in the nonmitochondrial disease group except in a patient with alternating hemiplegia whose symptoms responded well to flunarizine therapy. A significant increase in blood lactate content at 60 min after glucose loading occurred in the mitochondrial disease group, in contrast to that of the nonmitochondrial group. Sensitivity and specificity of a 5 mg/dl increase in blood lactate concentration at 60 min was 72.7% vs. 91.7% Moreover, all 4 patients whose blood lactate increased by 13 mg/dl at 60 min were in the mitochondrial disease group.

Biopsy↗

PubMedDate not supplied

The preliminary result of the training course of pediatric advanced life support.

To improve the preparedness of health care providers in pediatric resuscitation and to evaluate the effectiveness of the Pediatric Advanced Life Support course, we followed the standard guidelines of American Heart Association to conduct the first 10-hour course of Pediatric Advanced Life Support course in our hospital and designed this study. A total of 160 doctors and nurses were enrolled in the providers course held on Jan. 11, 1998. A pretest, posttest and survey questionnaire were given to each participant. One hundred and twenty-nine completed these tests. The average scores were 73.1 for the pretest and 86.8 for the posttest. The average scores on the pretest and posttest among doctors (n = 57) and nurses (n = 72) are 72.6, 86.7 and 73.5, 86.8, respectively (p < 0.001). In the participants with pretest score of less than 80 (n = 92), the mean values of pretest and posttest scores were 69.0 and 86.4. The participants with pretest scores higher than or equal to 80 (n = 37) had pretest and posttest mean values of 83.3 and 87.7. The participants with less background knowledge (pretest < 80) had better increment scores after this course (p < 0.05). The mean posttest scores in those working in private clinics and having 20 years of working experience are 74.0 and 72.0 (lower than the criteria for successful completion of written examination). We conclude that this provider course did increase the knowledge and skill pertaining to pediatric resuscitation personnel, particularly in participants with less background knowledge. The participants from private clinics or with working experience more than 20 years need a repeatedly educational PALS training course.

Adolescent↗

PubMedDate not supplied

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↗

PubMedDate not supplied

Clinical experience of ketogenic diet on children with refractory epilepsy.

Thirteen children with refractory epilepsy received a ketogenic diet (medium chain triglyceride oil diet) as an alternative therapy since September 1997. Their seizure patterns included (1) generalized tonic-clonic seizures, (2) myoclonic seizures, (3) generalized tonic + atonic seizures, (4) complex partial seizures, (5) generalized clonic + atonic + myoclonic seizures, (6) head nodding + myoclonic + gelastic seizures, and (7) generalized tonic-clonic + myoclonic + atonic seizures. Major concerns emphasized on the efficacy and side effects of the diet. Clinical observation one month after the diet revealed that 53.8% of the patients had a > 75% reduction in seizure frequency and 76.9% of the patients had a > 50% reduction in seizure frequency. Six patients had some degrees of improvement in cognitive function and/ or school performances. The most common side effects were body weight loss (n = 6) and diarrhea (n = 5). Others included bad temper (n = 1), abdominal cramps (n = 2), nausea (n = 2), bad body smell (n = 1), and renal stones (n = 1). Even after discontinuation of the diet, 61.5% of patients still had a > 50% reduction in seizure frequency. We concluded that the ketogenic diet deserves a trial in children with refractory epilepsy.

Adolescent↗

PubMedDate not supplied

Individualized developmental care in the newborn intensive care unit.

With recent advances on newborn intensive care, the survival rate of very low birth weight preterm infants has been improved. The consequently new morbidities such as learning disabilities, behavioral problems, lower mean IQ and motor clumsiness have become more frequent. Neonatal individualized developmental care seeks to read the behavior of premature infants, and pace our procedures and caregiving according to individual infant's cues. Studies have shown that with implementation of developmental care, the time spent on ventilator and hospitalization was decreased, and infants started oral feeding earlier. The short and long-term neurobehavioral outcomes were improved. Developmental care focuses not only on changing the environment to provide a calming and soothing environment for the infants and family, but also provides a chance for all the staff members to read the behavior of the infants, think and reflect before doing something to the infants.

Developing Countries↗

PubMedDate not supplied

Nosocomial infection in a neonatal intensive care unit--from a viewpoint of national health insurance.

In order to survey both the epidemiology of nosocomial infection in our neonatal intensive care unit (NICU) and the changing face of nosocomial infection after the introduction of National Health Insurance (NHI) in Taiwan, we retrospectively reviewed the nosocomial infections which occurred in our NICU from March 1, 1991, to February 28, 1999. We also compared the nosocomial infections from the viewpoint of NHI. The mean rate of nosocomial infections in our NICU during these 8 years was 13.6%, and it had significantly increased after the NHI plan was implemented (from 7.9% to 19.0%). The most common type of nosocomial infection was blood stream infection (53.8%, 120 of 223 infections). Coagulase-negative Staphylococci and fungi were the two most common pathogens of nosocomial blood stream infection in our NICU, accounting for 28.1% and 24.2% of the infections, respectively. The survival rate of very low birth weight (VLBW) infants increased from 76.3% to 78.4% after the implementation of NHI. However, the nosocomial infection rate of the VLBW infants had markedly increased from 22.6% to 41.9%. The total number of hospitalization days of the patients with nosocomial infection was significantly greater than that of those without nosocomial infections (p < 0.05), and the patients with the lower birth weights had longer hospital stays. The risk factors for nosocomial infection including invasive procedures, multiple and empirical antibiotics, and extremely low birth weight premature infants, remained the same, but the prevalence of nosocomial infection in the neonatal intensive care unit has changed markedly since the NHI plan began. Further investigation to determine strategies for preventing nosocomial infection in very low birth weight infants is warranted.

Cross Infection↗

PubMedDate not supplied

Barrett's esophagus in a child with de Lange syndrome: report of one case.

Barrett's esophagus, a premalignant condition, is recognized as stratified squamous epithelium of the esophagus substituted by columnar epithelium. The risk factors for development of Barrett's esophagus include frequent gastroesophageal reflux, esophageal stricture, male sex and mental retardation, but there is no report of Barrett's esophagus in children with de Lange syndrome. We report a 7-year-old boy who was diagnosed as de Lange syndrome shortly after birth and had gastroesophageal reflux since early infancy. Upper gastrointestinal endoscopic examination revealed a cauliflower-like mass and a pink-red velvety mass over the lower third of the esophagus. Biopsy showed goblet cells metaplasia, confirming Barrett's esophagus. We suggest surveillance of Barrett's esophagus could be done ahead of schedule in children with long-standing gastroesophageal reflux or with de Lange syndrome.

Barrett Esophagus↗

PubMedDate not supplied

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↗

PubMedDate not supplied

The follow-up on the training course of pediatric advanced life support.

Two parts are included in this article. First, to improve the training course in pediatric advanced life support (PALS), we held a 2-day PALS training course on March 28 and 29, 1999. A pretest, posttest, and survey questionnaire were given to each participant. Of the 143 participating in the 2-day PALS course, 137 completed the study. The mean pretest score was 70.2 (SD = 11.5) and the mean posttest score was 86.9 (SD = 9.1). There was significant improvement noted after the 2-day PALS training course (p < 0.001). The 2-day PALS training course resulted in greater improvement in scores compared with the previous 1-day PALS training course in some certain groups of participants. The number of participants complaining that they were too rushed to get the best learning result declined from 71 of 106 (66.3%) taking the 1-day training course to 43 of 137 (31.4%) taking the 2-day training course. Second, a same written test was given to the 129 participants who had finished a 1-day PALS training course one year previously to evaluate the participants' retention of knowledge after 1 year. Of 82 responders who took the 1-year follow up test, 75 were from the medical center. The score of the follow-up test for these 75 participants showed a significant decline when compared with the posttest, except for those receiving another training course of advanced life support during the follow-up year. We conclude that the 2-day PALS training course is better than the 1-day PALS training course. Those who participate in the pediatric critical or emergent care had better receive the 2-day PALS training course. The participants receiving the 1-day PALS training showed a significant decline in their knowledge after 1 year, and they should reinforce their knowledge in the following years.

Child↗

PubMedDate not supplied

Congenital complete heart block.

Congenital complete heart blocks (CCHB) are mostly related to the neonatal lupus syndrome. The purpose of this paper was to assess the clinical spectrum of CCHB in our hospital. Nine patients were retrospectively enrolled between 1994 and 1999. The birth history, electrocardiography, 24-hour Holter monitoring, pacemaker insertion and its complications, maternal disease, and maternal and infant autoantibody levels were studied. All nine cases were diagnosed prenatally. Hydrops fetalis was noted in five (55.6%). Six cases were live births and the other three were terminated. No anatomical heart defects were noted. Initial electrocardiography revealed the atrial rates ranged from 150 to 166 beats per minute. The minimal ventricular rates ranged from 46 to 80 beats per minute. VVI mode pacemakers were inserted through xyphoid approach in all live-birth infants. Complications were noted in three of them (50%). Antinuclear antibody and anti-SSA/Ro antibody were positive in all 8 mothers (100%). The anti-SSB/La antibody was positive in 6 of the eight mothers (75%). Five infants tested positive for anti-SSA/Ro antibody. None of the infants tested positive for anti-SSB/La antibody. In conclusion, all CCHBs in our series were associated with maternal autoantibodies. Because of high complication rate of permanent pacemaker insertion during the neonatal period, it should be restricted in certain conditions.

Antibodies, Antinuclear↗

PubMedDate not supplied

Zellweger syndrome: report of one case.

Zellweger syndrome is a fatal autosomal-recessive hereditary disease characterized by the absence of peroxisomes in liver and kidneys. The absence of peroxisomes results in impairment of many metabolic pathways, especially beta-oxidation of very long chain fatty acids (VLCFAs). We report a case of a three-month-old male infant with facial dysmorphism, hypotonia, psychomotor retardation, and hepatomegaly. He had an elder brother with the same facial features and hypotonia who died of hepatic failure at four months of age. Biochemical studies revealed elevation of blood pipecolic acid and VLCFAs, compatible with peroxisomal disorder. Electron microscopy of liver biopsy revealed absence of peroxisomes. Zellweger syndrome was diagnosed. Because this syndrome is usually fatal in early life, genetic counseling and prenatal diagnosis are crucial.

Biopsy↗

PubMedDate not supplied

Breastfeeding knowledge and attitudes of health professionals and students.

Thirty-one physicians, 108 nurses, 105 medical students, and 126 student nurses practicing in the same medical center received a questionnaire to evaluate their knowledge and attitudes about breastfeeding. The mean knowledge score of the 139 staff member was 49.4 +/- 10.8 (The highest possible score was 73); that of the medical students was 31.2 +/- 8.9, and that of the nurse students was 39.5 +/- 7.6. Thirteen percent of the medical students vs. 68.6% of the student nurses reported that they had received breastfeeding education in school. All of the respondents had a positive attitude toward breastfeeding. Occupation and in-service education for breastfeeding could increase the knowledge score. Having been breastfed during infancy could increase both the knowledge score and positive attitude score. We suggest that breastfeeding be integrated into the curricula of both medical and nursing schools. Health professionals should receive in-service education. The mechanism of lactation, management of breast problems and infant problems, contraindications for breastfeeding, the properties of human milk and the benefits of breastfeeding for the infant should all be included.

Adult↗

PubMedDate not supplied

Precocious puberty associated with growth hormone deficiency in a patient with craniopharyngioma: report of one case.

Isosexual precocious puberty in girls is not uncommon, but its association with craniopharyngioma and growth hormone deficiency is rarely reported. We present a patient with this combination. An 8-year-old girl developed breasts and then had menarche at 7 9/12 years old. Growth hormone deficiency was suspected due to inappropriate height and growth velocity in association with idiopathic precocity and a poor predicted adult height of 138.8 cm. Growth hormone deficiency was confirmed by clonidine and insulin stimulation tests. Intracranial lesion was suspected due to precocity associated with GH deficiency. MRI of the sella's region revealed a 1 cm mass in the hypothalamus. After surgical resection, pathology of the tumor disclosed a craniopharyngioma which has rarely been reported to cause precocious puberty. The precocious puberty regressed after surgery. Growth hormone deficiency persisted and GH therapy was given to improve growth. The growth rate of patients with both growth hormone deficiency and precocious puberty may be maintained within the normal prepubertal range by the effect of sex steroid. We suggest that in patients with central type precocity in association with an inappropriate growth status, physicians should investigate the underlying intracranial lesion, and the possibility of growth hormone deficiency.

Biopsy, Needle↗

PubMedDate not supplied

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↗

PubMedDate not supplied

Growth response and final height in growth hormone-deficient patients treated with biosynthetic growth hormone.

Twenty-three patients with growth hormone deficiency (GHD) followed until their final heights (FH) were reported by retrospective review. Seven patients had spontaneous puberty (group 1) and sixteen required induction of puberty (group 2). Their heights prior to growth hormone (GH) therapy were -3.7 +/- 1.4 and -2.8 +/- 1.4 SDS in men and women respectively. The mean ages at initiation of GH therapy were 17.2 +/- 3.3 in men and 13.0 +/- 1.7 years in women, with a growth velocity < 4 cm/year and a mean bone age (BA) of 12.6 +/- 0.7 years (men) and 9.8 +/- 1.2 years (women). The dose of GH was 0.27-0.83 IU/kg/week, with a total duration of 2.5 +/- 1.0 years. Their consecutive annual mean growth velocities on GH therapy were: 10.6 cm/year, 8.0 cm/year, 6.1 cm/year, 5.1 cm/year and 4.6 cm/year respectively. They reached a mean final height of 167.1 +/- 5.3 cm (-0.4 +/- 1.0 SDS) in men and 157.9 +/- 3.5 cm (-0.1 +/- 0.7 SDS) in women, which is slightly taller than their target height. Demographic factors related to growth response and final heights were analyzed. We conclude that GH therapy is very effective in linear growth promotion. Their final height was correlated with initial height SDS, target height SDS, predicted adult height SDS according to the bone age at the start of GH therapy and height SDS at the onset of puberty.

Adolescent↗

PubMedDate not supplied

Transient neonatal hypoparathyroidism: report of four cases.

Neonatal hypocalcemia (NH) is common in the neonatal period. Its cause falls into one of two clinical categories, early NH occurs in first 24-48 hours of life; late NH is observed at the end of the first week of life. NH due to congenital hypoparathyroidism, either permanent or transient, is rare. They both present with hypocalcemia, low levels of intact parathyroid hormones, and hyperphosphatemia. In this paper we report on four cases of neonatal hypocalcemia due to transient hypoparathyroidism. They are all full-term infants with normal birth weights, carried by nondiabetic mothers. The age of onset was 6 days old to 17 days old, there were three male and one female. Seizure was the major symptom except for case 2, who had a high pitch crying, irritability and opisthotonus. Laboratory data revealed calcium: 4.7 to 6.3 mg/dl, phosphorus: 6.8 to 9.2 mg/dl, and magnesium: 1.2 to 2.8 mg/dl. The intact parathyroid hormone levels were abnormally low in two cases (<13 pg/ml and 5.7 pg/ml), yet only subnormal in the other two (25.2 pg/ml and 22.2 pg/ml). Further studies on these four babies showed no evidence of Di George syndrome. Interestingly, two patients' mothers were found to have hyperparathvroidism. In conclusion, in case of neonatal hypocalcemia, measurements of calcium, phosphorus, and intact-PTH in neonates are required to recognize hypoparathyroidism. Pediatricians should always check maternal parathyroid status to rule out maternal hyperparathyroidism.

Female↗

PubMedDate not supplied

Stiff-Baby--an unusual manifestation of cytoplasmic body myopathy: report of one case.

A 2-month-old male baby was admitted to our hospital with episodic cyanosis and respiratory failure which required mechanical ventilation. He was found to have upper limb flexion rigidity and poor weight gain since one month old. Progressive muscle stiffness over the abdomen, chest wall, back and four limbs were also noted. He could not be weaned from the ventilator smoothly due to recurrent CO2 retention. Laboratory tests revealed a high serum creatine kinase level. Cytoplasmic body myopathy was confirmed by muscle biopsy. The unusual initial presentations of generalized stiffness and early onset of respiratory failure were quite different from those of patients reported in the literature, who had floppiness, muscular atrophy and weakness. Prednisolone and Vigabatrin were given and the patient showed slight improvement in muscle stiffness and spontaneous movement.

Humans↗