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

Background: The incidence of type 1 diabetes (T1D) is increasing rapidly worldwide, predominantly in younger individuals. We developed a checklist of all symptoms of T1D reported in the literature and compared the completeness of the recording of symptoms at initial presentation before and after the checklist was adopted.

Methods: We retrospectively reviewed the records of patients newly diagnosed with T1D from January 1, 1979 through September 30, 2006 to assess the presenting features and test the usefulness of a symptom checklist in evaluating the history on presentation. The checklist was incorporated into the records as of October 1, 1994.

Results: Of the 304 patients identified, 130 (43%) had checklists in the charts. There were 146 (48%) boys, 98 (32%) who were diagnosed under the age of 6 years, and 198 (65%) presented with diabetic ketoacidosis (DKA). Records with a checklist noted diabetic symptoms that were subtle and easily ignored more often than records without the checklist. As compared with those diagnosed at an older age, patients diagnosed at < or = 6 years were more likely to be male, have DKA and a shorter symptom duration, and report more episodes of preceding viral infection and dyspnea. Patients with DKA also had a shorter symptom duration.

Conclusions: A diabetic symptom checklist was helpful in identifying clinical diabetic symptoms and signs which were otherwise easily ignored. Younger children were more likely to have a shorter symptom duration and a higher incidence of DKA.

Although allogeneic stem cell transplantation can cure patients with hematologic malignancies and nonmalignant disorders, limiting factors such as lack of suitable donors and graft-versus-host disease (GVHD) toxicity have led to the exploration of umbilical cord blood (UCB) as an alternative source of hematopoietic stem cells. The unique immunologic properties of UCB likely contribute to a decreased risk of GVHD. Thus, UCB represents a highly convenient hematopoietic stem cells (HSC) source that may significantly expand the HSC donor pool. This overview examines the advantages and disadvantages of UCB as a donor source and outlines the current status of UCB transplantation (UCBT), with emphasis on pediatric practice. Strategies to improve kinetics of hematopoietic recovery after UCB grafting in children and adults are warranted.

Background: The aim of this retrospective study was to assess neonatal outcomes of pregnant women with myasthenia gravis (MG).

Methods: Pregnant women with MG who were treated in our hospital over an 8-year period were enrolled. Data relating to the course of the mother's MG (including the anti-acetylcholine receptor antibody (anti-AchR) titer and drug dosage), delivery mode, delivery course, puerperium period, and neonatal outcomes were obtained from the medical records.

Results: Twelve women with MG had 13 pregnancies in our hospital from January 1997 to December 2005. None of the mothers needed intensive care. Two patients delivered vaginally, and ten delivered by cesarean section. Fourteen infants were born at an average gestational age of 37.2 +/- 2.0 weeks; their average birth weight was 2838.6 +/- 724.2 g. Two neonates (14.2%) had a congenital anomaly. Transitory neonatal myasthenia gravis (NMG) was diagnosed in one infant (7.1%).

Conclusions: In our study, MG exacerbations occurred in 38.5% of the patients. Postpartum, all MG patients experienced deterioration except one patient who deteriorated in the first trimester with a concomitant upper airway tract infection. Only 1 of the 14 neonates developed transient NMG; the incidence of transient NMG was lower than that previously reported. There was no correlation between the occurrence of NMG and the maternal anti-AChR titer. The cesarean section rate was approximately 33%; this high rate of elective cesarean sections in these MG patients could have prevented the occurrence of some of the complications related to vaginal delivery.

Background: The present study aimed to elucidate the clinical characteristics and outcomes of three types of occipital epileptic syndromes: early-onset and late-onset childhood epilepsy with occipital paroxysm (ECEOP and LCEOP) and symptomatic occipital epilepsy (SOLE).

Methods: We retrospectively reviewed the medical records of 54 children (18 ECEOP, 10 LCEOP, and 26 SOLE) and compared the clinical features, EEG findings, treatments, and outcomes among these patients.

Results: Nocturnal seizures occurred in 55% of the patients with ECEOP, whereas of those with LCEOP and SOLE, 80% and 61% had diurnal seizures, respectively (P = 0.04). Status epilepticus was more common in the ECEOP group, less in LCEOP. Autonomic auras were more common in the SOLE group than in the other two groups. Secondary generalized seizure was often found in the SOLE group (P = 0.03). EEG findings in 72% of the ECEOP group, 60% of the LCEOP group, and 19.2% in the SOLE group were changeable and became to be normal after 5 years of follow-up. Slow EEG background activity was more evident in the SOLE group than in the other two groups. The prognoses of these groups of patients were different. The ECEOP group had the best, and the SOLE group had the worst.

Conclusions: These findings suggest that some clinical features of these three syndromes differ from each other, which may provide clinicians a basis for determining the appropriate diagnosis in children with one of these childhood occipital epileptic syndromes.

Kimura's disease (KD) is a rare, chronic inflammatory disease, and the etiology is not yet to be determined. The disease is usually found in young Asian men but rarely in Western population. Here, we report an 8-year-old boy with the initial presentation of right upper eyelid swelling. Laboratory tests disclosed peripheral eosinophilia and elevated IgE level. KD with lacrimal gland involvement was diagnosed. Surgical excision was performed several times and steroid was also used, however the disease recurred. Thereafter, steroid, cyclosporine-A and azathioprine were prescribed. The tumor mass resolved and the laboratory data returned to normal level.

Background: Poor health outcomes among neonates born to aborigines has been reported in many countries. This study was aimed to examine the nationwide characteristics of live births, adverse birth outcomes, and age-specific mortality among neonates born to non-aboriginal and aboriginal parents in Taiwan.

Methods: All neonates born alive during the period of 2000 to 2003 in Taiwan were included. The adverse birth outcomes including low birth weight, preterm, and small-for-gestational-age births, and age-specific mortality were obtained. Logistic regression analysis was used to estimate odds ratios for parental ethnicity in relation to birth outcomes, while Cox's proportional hazards regression models were used to estimate hazard ratios for parental ethnicity in relation to age-specific infant deaths.

Results: A total of 947,317 live births were included that consisted of 9,381 born to aboriginal mothers, 6,429 born to aboriginal fathers, and 15,354 born to aboriginal parents. There was a gradual increase in the risk of having a baby with low birth weight, preterm, or small for gestational age born to the four parental aboriginal ethnicity groups: non-aboriginal parents, aboriginal mother only, aboriginal father only, and aboriginal parents. Similar trends were also found for early neonatal, neonatal, and infant mortalities after stratification of residential areas. The neonates born to both aboriginal parents with residence in rural or mountain areas were at highest risk of adverse birth outcomes and age-specific mortality.

Conclusions: Our results demonstrated that aboriginality and residential area are important risk factors for adverse perinatal and infant outcomes.

We report a girl with acute lymphoblastic leukemia who developed severe conjugated hyperbilirubinemia (total bilirubin 34.32 mg/dl, direct bilirubin 26.94 mg/dl) following gram-negative (Escherichia coli ) sepsis. Despite broad-spectrum antibiotics, her hyperbilirubinemia progressed. Her mental status deteriorated and steroid therapy (hydrocortisone 8 mg/kg/d) was initiated. Her condition then recovered rapidly. Based on our findings, we suggest that steroid may help to treat progressive conjugated hyperbilirubinemia due to gram-negative sepsis.