Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi最新文献

Background: Scrub typhus, a potentially fatal rickettsial infection, is endemic in western Pacific Rims including Taiwan. Most reports have been concerned about mainly adult patients, whereas serologic surveys suggested that as many as one-half of cases of scrub typhus might be in children.

Methods: We conducted a retrospective study of childhood scrub typhus in our hospital from January 1997 to December 2006. Scrub typhus was diagnosed on the basis of serology tests or polymerase chain reaction (PCR) examination in fifteen children.

Results: Fever and chigger bite history were presented in all 15 cases, and eschar lesion was identified in 12 patients (80%). Nine (60%) patients had headache and six (40%) complained of abdominal pain. Three patients (20%) had meningoencephalitis, and two (13%) hemophagocytic syndrome were confirmed by bone marrow biopsy. One patient died of progressive acute respiratory distress syndrome (ARDS) and pulmonary hemorrhage. Myocarditis was revealed in autopsy. All surviving patient responded well to antibiotic therapy, and the average duration to defervescence after treatment was 1.8 days.

Conclusions: We conclude that scrub typhus should be taken into consideration among patients of acute systemic febrile illness, especially those with suspected exposure history and typical skin lesion. Although most patients responded well to treatment, severe complications such as meningoencephalitis, interstitial pneumonia, acute respiratory distress syndrome, hemophagocytic syndrome, and myocarditis might lead to morbidity and mortality.

Background: Acute lung injury, such as meconium aspiration syndrome in neonates, may present with exacerbated ventilation and perfusion abnormalities. This can impair the efficacy of intravenous antibiotic therapy in treating pulmonary infection. Intrapulmonary administration via perfluorochemical as a vehicle may adequately deliver drugs, such as the poorly pulmonary-penetrative antibiotic vancomycin, to affect lung regions while maintaining gas exchange and non-toxic serum level.

Methods: Twelve newborn piglets were injured with intra-tracheally installed human meconium, and randomly grouped into intravenous group (intravenous injection with vancomycin 15 mg/kg, followed by conventional gas ventilation) or intrapulmonary group (intrapulmonary instilled with vancomycin 15 mg/kg and perfluorochemical emulsion, followed by partial liquid ventilation). Blood samples were obtained to check vancomycin serum concentrations (0-240 mins). Lung tissues were tested for vancomycin contents.

Results: Intrapulmonary group animals had significantly lower vancomycin serum levels than the intravenous group, but vancomycin contents (198.9 +/- 72.5 microg/g dry tissue) in lung tissues was significantly higher than in the intravenous group (134.9 +/- 39.1 microg/g dry tissue) (p < 0.05). Gas exchange and lung compliance in the intrapulmonary group were also significantly better than in the intravenous group.

Conclusions: Perfluorochemical is a good vehicle to deliver vancomycin and maintains gas exchange in severe meconium-injured lungs.

Harlequin ichthyosis (HI) is the most devastating form of skin disorder, which is inherited as autosomal recessive trait related to consanguineous marriage. Although prenatal examination has become scheduled and convenient throughout Taiwan, an unexpected case of HI in a male premature infant born at 32 weeks of gestation was presented. The parents were healthy, neither relatives nor having history of congenital abnormality. We report our management and the massive impact left on both parents. We believe this is an extremely rare case in Taiwan.

Inflammatory myofibroblastic tumor (IMT) is a very rare benign tumor composed of myofibroblastic spindle cells of uncertain etiology, which can occur at any age and affect any organ system. More and more cases of IMT in children have been described in pediatric literature in recent years. However, this tumor occurring intraabdominally in children has rarely been reported in Taiwan. Here we present a 1-year-9-month-old boy who had fever and abdominal pain only for 2 days, symptoms mimicking acute abdomen. After imaging study, a huge tumor nearly 10 cm in diameter was incidentally found over the right abdomen with unknown origin and nature. After surgical removal of the tumor, IMT was confirmed by the pathological findings. It is very difficult to make an accurate preoperative diagnosis on this tumor according to past experience, so the role of pathological diagnosis with immunohistochemical study becomes important. This case illustrates that IMT should be considered as a possible cause of intra-abdominal mass in children who have fever of unknown origin.

Background: Our aim was to establish reference data and linear regression equations for lumbar bone mineral density (BMD) in normal Taiwanese children. Several influencing factors of lumbar BMD were investigated.

Methods: Two hundred fifty-seven healthy children were recruited from schools, 136 boys and 121 girls, aged 4-18 years were enrolled on a voluntary basis with written consent. Their height, weight, blood pressure, puberty stage, bone age and lumbar BMD (L2-4) by dual energy x-ray absorptiometry (DEXA) were measured. Data were analyzed using Pearson correlation and stepwise regression tests.

Results: All measurements increased with age. Prior to age 8, there was no gender difference. Parameters such as height, weight, and bone age (BA) in girls surpassed boys between ages 8-13 without statistical significance (p> or =0.05). This was reversed subsequently after age 14 in height (p<0.05). BMD difference had the same trend but was not statistically significant either. The influencing power of puberty stage and bone age over BMD was almost equal to or higher than that of height and weight. All the other factors correlated with BMD to variable powers. Multiple linear regression equations for boys and girls were formulated.

Conclusions: BMD reference data is provided and can be used to monitor childhood pathological conditions. However, BMD in those with abnormal bone age or pubertal development could need modifications to ensure accuracy.

Background: Sedation of children is always necessary for magnetic resonance imaging (MRI). General anesthesia may be recommended for infants and small children to protect the airway or in children who have previously failed sedation. Our practice has been to use intermittent administration of small doses of propofol.

Methods: We retrospectively reviewed the anesthesia records of all children who underwent sedation for MRI between March 2004 and March 2007.

Results: A total of 120 children underwent sedation for MRI. Few adverse events (respiratory 3%, movement 8%) and no complications occurred during sedation. Almost all of the procedures (99%) were completed successfully.

Conclusions: Our experience demonstrates that intermittent administration of small doses of propofol by experienced personnel for MRI can be used safely and effectively in pediatric patients.

Background: Antiepileptic drugs have been shown to be associated with a lowering of bone mineral density in childhood and adolescence, which are critical periods of skeletal mineralization. A lower peak bone mass attained at the end of adolescence is associated with greater involutional osteoporosis and risk for fracture in the elderly. Our purpose was to evaluate the effects of carbamazepine and valproate monotherapy on bone mineral density in children in Taiwan.

Methods: From November 1995 to April 2005, forty-two children with uncomplicated epilepsy, who were treated with either carbamazepine (n=21) or valproate (n=21) monotherapy for more than 6 months, were enrolled in this study. All subjects were 5 to 18 years of age, seizure-free for 5 months or more, with normal daily activity, and normal diet. Lumbar bone mineral density of L1 to L4 was measured by dual-energy X-ray absorptiometry.

Results: The mean serum levels of carbamazepine and valproate were 5.12 +/- 2.15 mcg/ml and 49.61 +/- 20.84 mcg/ml, respectively. Treatment durations were 37.05 +/- 31.11 months and 22.86 +/- 18.84 months, respectively. The serum levels of calcium and phosphate in both groups were within therapeutic range. The serum level of alkaline phosphatase was significantly higher in the carbamazepine group (264.71 +/- 66.91, U/L) than in the valproate group (179.48 +/- 79.37, U/L). Three patients (140%) had bone mineral density Z-score of -2.0 or lower in the carbamazepine-treated group, but none in the valproate-treated group (p=0.232). Comparing the Z-score in carbamazapine- and valproate-monotherapy children, 7 (33%) had Z-score of -1.5 or lower in the carbamazepine-treated group, and none in the valporate-treated group had Z-score of -1.5 or lower (p=0.009). Four (57%) patients in the 7 carbamazepine-treated children with Z-score of -1.5 or lower had serum drug level lower than therapeutic range.

Conclusions: Children receiving carbarmazepine monotherapy had increased frequency of lower bone density than children receiving valproate monotherapy.

Background: Cri-du-chat syndrome is a genetic disorder associated with various sized deletions of the short arm of chromosome 5. There are typical physical features, but individual phenotypes vary considerably.

Methods: The records of 23 patients with cri-du-chat syndrome admitted to Mackay Memorial Hospital from June 1984 to February 2006 were retrospectively reviewed. Data abstracted from the records included abnormal facial features and physical findings, results of echocardiography, bronchoscopy, auditory evoked potential, visual evoked potential, brain ultrasonography, and karyotype. We examined the various clinical phenotypes to see if there was an association with specific karyotypes.

Results: Among congenital heart conditions, atrial septal defect (8/15, 53%) was the most common, followed by ventricular septal defect (4/15, 26%), tricuspid regurgitation (4/15, 26%) and patent ductus arteriosus (3/15, 20%). Laryngomalacia was the most frequent airway problem (8/23, 34%) and strabismus the commonest visual disorder (1/23). A high percentage of patients had impaired hearing (5/23, 21%). There was no clear relationship found between deletion size and major clinical manifestations in this study.

Conclusions: Karyotype is not a reliable indicator of specific organ involvement in cri-du-chat syndrome. However, karyotyping is still useful, particularly if parental translocation are found to be involved. It helps for prenatal diagnosis of next pregnancy.