A model of quality assurance and quality improvement for post-graduate medical education in Europe.

BACKGROUND: The issue of quality assurance (QA) and quality improvement (QI), being the quality of medical education intimately related to the quality of the health care, is becoming of paramount importance worldwide. AIM: To describe a model of implementing a system for internal QA and QI within a post-graduate paediatric training programme based on the ISO 9001:2000 standard. METHODS: For the ISO 9001:2000 standard, the curriculum was managed as a series of interrelated processes and their level of function was monitored by ad hoc elaborated objective indicators. RESULTS: The training programme was fragmented in 19 interlinked processes, 15 related procedures and 24 working instructions. All these materials, along with the quality policy, the mission, the strategies and the values were made publicly available. Based on the measurable indicators developed to monitor some of the processes, areas of weakness of the system were objectively identified and consequently QI actions implemented. The appropriateness of all this allowed the programme to finally achieve an official ISO 9000:2001 certification. CONCLUSIONS: The application of the ISO 9001:2000 standard served to develop an internal QA and QI system and to meet most of the standards developed for QA in higher and medical education.

The cold pressor test for the pediatric population: refinement of procedures, development of norms, and study of psychological variables.

OBJECTIVE: The aims of the study were: (a) to propose focal refinements to the cold pressor test (CPT) for the pediatric population, contributing to the development of subjective and behavioral norms; (b) to analyze the influence of personal (gender, age, and skin-fold thickness), and psychological (anxiety, depression, internalization, and externalization) variables on pain perception and its correlation with room temperature. METHODS: After a phase of adaptation in a water bath (24-27 degrees C), the child immersed one arm in cold water (10 degrees C) and reported pain threshold and tolerance. RESULTS: The test was conducted on 141 healthy children. Pain tolerance was reached within the first minute by 50% of the children. Pain intensity affected the children's emotional status. Older children tolerated pain for longer. Gender, psychological, and environmental variables did not influence pain perception. CONCLUSION: This study provides methodological refinements to the CPT contributing to the development of norms for children.

Remifentanil for percutaneous intravenous central catheter placement in preterm infant: a randomized controlled trial.

BACKGROUND: There is limited evidence on the analgesic efficacy of opioids during percutaneous intravenous central catheter (PICC) insertion in preterm infants. AIM: To assess the analgesic and procedural efficacy of low-dose remifentanil infusion during PICC in preterm infants. METHODS: Fifty-four neonates [mean gestational age (+/-sd) 28 +/- 2 weeks; birth weight 1126 +/- 337 g] were randomly assigned to remifentanil infusion at 0.03 mcg.kg(-1).min(-1) (R) or placebo (C) in addition to 0.3 ml of 12% sucrose per os and non-nutritive sucking. RESULTS: Validated pain scales [Neonatal Infants Pain Scale (NIPS) and Premature Infants Pain Profile (PIPP)] administered at the baseline T0, skin preparation T1, needle insertion T2, and recovery T3, revealed differences in mean NIPS scores (C 5.3 +/- 1.3 vs R 4.2 +/- 1.4 at T1 and C 5.0 +/- 1.3 vs R 3.4 +/- 1.3 at T2) and PIPP scores (C 9.3 +/- 1.6 vs R 7.1 +/- 1.5 at T1 and C 8.6 +/- 1.7 vs R 6.1 +/- 1.4 at T2); P < 0.05. Cardiovascular and respiratory response, and body movements during PICC suggested better pain and distress control with remifentanil (P < 0.05), but the time to complete the maneuver and the number of attempts needed remained the same in the two groups. CONCLUSIONS: Low-dose remifentanil has a measurable, synergic analgesic effect in combination with 12% sucrose and non-nutritive sucking, but does not make PICC easier or quicker.

Method for the quantification of underivatized amino acids on dry blood spots from newborn screening by HPLC-ESI-MS/MS.

In our study we have developed an HPLC-ESI-MS/MS method for qualitative and quantitative analysis of underivatized amino acids on dry blood spots. The sensitive and specific instrumental performances permitted the chromatographic separation of 40 amino acids and their isomers within 10 min. The method has been set up for cases of suspected metabolic diseases revealed by newborn screening. What is new is that it is applied on the same blood spots used for newborn screening, instead of plasma, in order to avoid involvement of doctors, increased anxiety for parents, stress for patients for plasma collection, long time of waiting and further costs for analysis.

Neurologic outcome in children after extracorporeal membrane oxygenation: prognostic value of diagnostic tests.

This report presents the long-term (36 months) neurologic outcome in 12 neonates and 9 children who survived after extracorporeal membrane oxygenation and attempts to identify its prognostic indicators through a prospective study in the pediatric intensive care unit of a university hospital. Outcome assessment, neurodevelopmental tests, electroencephalogram, auditory evoked potentials, visual evoked potentials, and somatosensory evoked potentials, cerebral sonography, or cerebral tomography were evaluated at the end of bypass and at 6, 12, 24, and 36 months after extracorporeal membrane oxygenation. "Before extracorporeal membrane oxygenation" variables (oxygenation index, pH, oxygen arterial partial pressure) and "during extracorporeal membrane oxygenation" variables (pH, oxygen arterial partial pressure, duration of bypass, clotting activated time, electroencephalogram) were also analyzed. A negative neurologic outcome (Glasgow Outcome Score different from "good recovery" or neurodevelopmental score less than 70) 12 months after extracorporeal membrane oxygenation was documented in 8.3% of neonates and in 30% of children who survived. There was no further change in subsequent evaluations (24 and 36 months follow-up). The most abnormal electroencephalogram during extracorporeal membrane oxygenation, the first electroencephalogram, neuroimaging score, and somatosensory evoked potentials after extracorporeal membrane oxygenation treatment were associated with negative neurologic outcome. The study documented that neonates and children treated with extracorporeal membrane oxygenation require long-term follow-up; electroencephalogram, neuroimaging score, and somatosensory evoked potentials have prognostic value for abnormal neurologic outcome.

Systolic and diastolic performance late after the Fontan procedure for a single ventricle and comparison of those undergoing operation at <12 months of age and at >12 months of age.

To evaluate whether surgical history can influence systolic and diastolic properties of a functional single left ventricle after Fontan operation, we echocardiographically investigated 31 patients (mean age 93.7 months; range 21 to 276); 21 patients were >12 months of age (group A) and 10 were <12 months of age (group B) at the time of the Fontan and/or cavopulmonary procedure. In group A we found persistent abnormalities of left ventricular mass index (95.9 vs 64.1 g/m(2), p <0.05) at long-term follow-up, whereas group B had normal left ventricular mass (61.9 vs 64.1 g/m2, p = NS). In contrast, a diastolic pattern characterized by augmented late diastolic filling was present in both patient groups regardless of age at operation and length of follow-up (E/A in group A 1.3 +/- 0.4, E/A in group B 1.6 +/- 1.5, E/A in controls 1.7 +/- 0.6; A vs B, p = NS; A vs controls, p <0.05). We concluded that patients with a single left ventricle who undergo an unloading procedure performed within the first year of life have complete normalization of left ventricular mass, although a diastolic filling pattern suggestive of augmented compliance persists, regardless of the age at operation.

Evaluating pain induced by venipuncture in pediatric patients with developmental delay.

OBJECTIVES: Little attention has been paid to the assessment of pain in children with developmental delay. The aim of this study was to explore several methods for assessing pain during venipuncture in this population of children, using classic and modified scales to evaluate the children's response to simplified tools. METHODS: Sixteen children with mild or moderate developmental delay were evaluated using three standard self-rating scales (Visual Analog Scale [VAS], Eland Scale, and Faces Scale) and three modified methods (Cube Test, Modified Eland Scale, and Modified Faces Scale), recording subjective self-ratings and behavioral expressions of pain during a venipuncture procedure, apart from the initial fear. The children's pain and reaction time were assessed by an outside observer, while their pain and fear were also evaluated by the parents. RESULTS: The VAS was used without difficulty by all the children and revealed a good consistency with the Cube Test. The parents' and neutral observer's indirect pain assessment was also consistent with the child's evaluations. The Eland Scale proved difficult to use, especially for Down's syndrome children, while its modified version was easier. Results emerging from the original and modified Faces Scales were inconsistent. Frightened children attributed higher pain scores, demonstrating that negative emotions exacerbate the experience of pain in developmentally delayed children. The patients showed a limited capacity for verbal and behavioral expression in reaction to the painful stimulus (especially the Down's cases). DISCUSSION: These findings support the conviction that even developmentally delayed children can use self-rating methods effectively. This sector demands further, more extensive study, including the development of simplified tools, to ensure an adequate pain assessment and optimal antalgic approach to this particular pediatric population.

Metabolomics: moving towards personalized medicine.

In many fields of medicine there is a growing interest in characterizing diseases at molecular level with a view to developing an individually tailored therapeutic approach. Metabolomics is a novel area that promises to contribute significantly to the characterization of various disease phenotypes and to the identification of personal metabolic features that can predict response to therapies. Based on analytical platforms such as mass spectrometry or NMR-based spectroscopy, the metabolomic approach enables a comprehensive overview of the metabolites, leading to the characterization of the metabolic fingerprint of a given sample. These metabolic fingerprints can then be used to distinguish between different disease phenotypes and to predict a drug's effectiveness and/or toxicity.Several studies published in the last few years applied the metabolomic approach in the field of pediatric medicine. Being a highly informative technique that can be used on samples collected non-invasively (e.g. urine or exhaled breath condensate), metabolomics has appeal for the study of pediatric diseases. Here we present and discuss the pediatric clinical studies that have taken the metabolomic approach.

Rapid diagnostic testing procedures for lysosomal storage disorders: alpha-glucosidase and beta-galactosidase assays on dried blood spots.

BACKGROUND: Lysosomal storage disorders (LSDs) are pathologies caused by the deficit of lysosomal enzymes; late diagnosis may render therapeutic programs less effective. As early, pre-symptomatic detection could change the natural history of the disease, we are setting up rapid microassays using dried blood spots (DBS) on filter paper. Here we report alpha-glucosidase and beta-galactosidase assays. METHODS: Enzymatic activities were evaluated on DBS from five different groups of subjects including healthy controls and patients affected with an LSD. A 260-day monitoring of DBS preservation at five different temperatures and a comparison of the enzymatic activities measured in DBS obtained from a single (sDBS) or a double (dDBS) blood drop were performed as well. RESULTS: Both assays could clearly distinguish the affected patients from the other subjects analyzed. Storage of DBS at 4 degrees C and below allowed a longer preservation of the enzymatic activities. No significant differences were detected between sDBS and dDBS. CONCLUSIONS: DBS can be used for non-invasive, easy, inexpensive lysosomal enzyme assays. Reliability of assays on DBS needs to be checked using a control enzyme such as beta-galactosidase. DBS can be still reliably analyzed even if generated incidentally by two overlapped drops.

High transduction efficiency of human amniotic fluid stem cells mediated by adenovirus vectors.

In the last few years some studies have shown the possibility of deriving progenitors with various potential from the amniotic fluid. Amniocentesis is a widely accepted method for prenatal diagnosis; it is associated with low risk both for the mother and the fetus and overcomes the ethical problems commonly associated to other sources. Recently we have described that amniotic fluid stem (AFS) cells, for their ability to differentiate to various lineages, could represent a good candidate for therapeutic applications. For gene therapy purposes human AFS (hAFS) cells should be genetically modified with a therapeutic gene and delivered systematically or injected directly into the tissue of interest. The aim of this study was to investigate the feasibility of transducing hAFS cells with adenoviral vectors and to determine whether transduced stem cells retain the ability to differentiate into different lineages. Herein, we showed that hAFS cells could be efficiently infected by first generation adenovirus vectors. In addition, we demonstrated that infection and expression of two different marker genes, LacZ and EGFP, have no effect on cells phenotype and differentiation potential. In particular, on undifferentiated status, hAFS cells continued to express both the transgenes and stemness cell markers OCT4 and SSEA4. When cultured under mesenchymal conditions, infected cells could still differentiate into osteocytes and adipocytes expressing lineage specific genes. These preliminary findings suggest that adenovirus may be useful to engineer populations of pluripotent stem cells, which may be used in a wide range of gene therapy treatments.

Low exhaled nitric oxide in school-age children with bronchopulmonary dysplasia and airflow limitation.

Bronchopulmonary dysplasia (BPD), the chronic lung disease of prematurity, may be associated with long-term airflow limitation. Survivors of BPD may develop asthma-like symptoms in childhood, with a variable response to beta(2)-agonists. However, the pathologic pathways underlying these respiratory manifestations are still unknown. The aim of this study was to measure exhaled nitric oxide (FE(NO)) and lung function in a group of 31 school-age survivors of BPD. They showed variable degrees of airflow obstruction (mean FEV(1) 77.8 +/- 2.3% predicted) unresponsive to beta(2)-agonists in 72% of the subjects. Their FE(NO) values (geometric mean [95% confidence interval]: 7.7 [+/- 1.1] ppb) were significantly lower than in a group of healthy matched control subjects born at term (10.7 [+/- 1.1] ppb, p < 0.05) and a group of preterm children without BPD (9.9 [+/- 1.1] ppb, p < 0.05). The children with BPD were also compared with a group of 31 patients with asthma with a comparable airflow limitation (FEV(1) 80.2 +/- 2.1% predicted) and showed FE(NO) values four times lower than in those with asthma (24.9 [+/- 1.2] ppb, p < 0.001). In conclusion, unlike children with asthma, school-age survivors of BPD have airflow limitation associated with low FE(NO) values and lack of reversibility to beta(2)-agonists, probably as a result of mechanisms related to early life structural changes in the airways.

Office spirometry in primary care pediatrics: a pilot study.

OBJECTIVE: The aim of this study was to investigate the validity of office spirometry in primary care pediatric practices. METHODS: Ten primary care pediatricians undertook a spirometry training program that was led by 2 pediatric pulmonologists from the Pediatric Department of the University of Padova. After the pediatricians' training, children with asthma or persistent cough underwent a spirometric test in the pediatrician's office and at a pulmonary function (PF) laboratory, in the same day in random order. Both spirometric tests were performed with a portable turbine flow sensor spirometer. We assessed the quality of the spirometric tests and compared a range of PF parameters obtained in the pediatricians' offices and in the PF laboratory according to the Bland and Altman method. RESULTS: A total of 109 children (mean age: 10.4 years; range: 6-15) were included in the study. Eighty-five (78%) of the spirometric tests that were performed in the pediatricians' offices met all of the acceptability and reproducibility criteria. The 24 unacceptable test results were attributable largely to a slow start and failure to satisfy end-of-test criteria. Only the 85 acceptable spirometric tests were considered for analysis. The agreement between the spirometric tests that were performed in the pediatrician's office and in the PF laboratory was good for the key parameters (forced vital capacity, forced expiratory volume in 1 second, and forced expiratory flow between 25% and 75%). The repeatability coefficient was 0.26 L for forced expiratory volume in 1 second (83 of 85 values fall within this range), 0.30 L for forced vital capacity (81 values fall within this range), and 0.58 L/s for forced expiratory flow between 25% and 75% (82 values fall within this range). In 79% of cases, the primary care pediatricians interpreted the spirometric tests correctly. CONCLUSIONS: It seems justifiable to perform spirometry in pediatric primary care, but an integrated approach involving both the primary care pediatrician and certified pediatric respiratory medicine centers is recommended because effective training and quality assurance are vital prerequisites for successful spirometry.

Exhaled breath condensate cysteinyl leukotrienes are increased in children with exercise-induced bronchoconstriction.

BACKGROUND: It is recognized that airway inflammation has a central role in the pathogenesis of asthma, but how it relates to exercise-induced bronchoconstriction (EIB) is not completely understood. OBJECTIVE: The aim of our study was to investigate the relationship between EIB and baseline concentrations of cysteinyl leukotrienes (Cys-LTs) and other inflammatory markers in exhaled breath condensate (EBC). METHODS: EBC was collected, and the fraction of exhaled nitric oxide (FE NO ) was measured in a group of 19 asthmatic children, after which they performed a treadmill exercise test. Fourteen healthy children were enrolled as control subjects. RESULTS: The asthmatic children were divided into the EIB group (decrease in FEV 1 , > or =12%) and the non-EIB group. The EBC was analyzed for the presence of Cys-LTs, leukotriene B 4 , and ammonia. Asthmatic patients with EIB (mean FEV 1 decrease, 23% +/- 3%) had higher Cys-LT concentrations than either asthmatic patients without EIB or control subjects (42.2 pg/mL [median] vs 11.7 pg/mL and 5.8 pg/mL; P < .05 and P < .001, respectively). Ammonia concentrations were lower in both the EIB and non-EIB groups than in control subjects (253.2 microM and 334.6 microM vs 798.4 microM; P < .01 and P < .05, respectively). No difference in EBC leukotriene B 4 levels was found among the 3 groups. Both asthmatic groups had higher FE NO levels than control subjects ( P < .001). EBC Cys-LT ( P < .01; r = 0.7) and FE NO ( P < .05; r = 0.5) values both correlated significantly with the postexercise FEV 1 decrease. CONCLUSION: this study shows that EBC Cys-LT values are higher in asthmatic children with EIB and correlate with the decrease in FEV 1 after exercise. These findings suggest that the pathways of both Cys-LT and nitric oxide are involved in the pathogenesis of EIB.

Knowledge gained by pediatric residents after neonatal resuscitation program courses.

BACKGROUND: The efficacy of the Neonatal Resuscitation Program (NRP) courses was previously evaluated, demonstrating good retention of knowledge in the participants. However, there is a lack of information regarding the participants' performance in relation to the different steps of neonatal resuscitation. We aimed to assess the knowledge gained and retained by pediatric residents who participated in a NRP course in relation to the different steps. METHODS: An 80-item questionnaire derived from the standard test contained in the American Heart Association and American Academy of Pediatrics Neonatal Resuscitation Manual was given to 25 pediatric residents before, immediately after and 6 months after the course. RESULTS: The percentages of correct answers significantly improved from before (37.6 +/- 3.1%) to immediately after the course (94.1 +/- 0.9%) (P < 0.001). The percentages at the 6 months follow-up test (62.7 +/- 2.2%) significantly decreased from posttest (P < 0.001), but remained significantly higher with respect to pretest performance (P < 0.001). The percentages of correct answers were different among the four neonatal resuscitation steps during the entire study (pretest, posttest and follow-up test). CONCLUSIONS: The knowledge gained by pediatric residents participating in the NRP course was very high, but was only partially retained over time. In particular, it was different among the four steps of neonatal resuscitation suggesting further studies on teaching resuscitation.

CXCR3/CXCL10 expression in the synovium of children with juvenile idiopathic arthritis.

The accumulation of T cells in the synovial membrane is the crucial step in the pathophysiology of the inflammatory processes characterizing juvenile idiopathic arthritis (JIA). In this study, we evaluated the expression and the pathogenetic role in oligoarticular JIA of a CXC chemokine involved in the directional migration of activated T cells, i.e. IFNgamma-inducible protein 10 (CXCL10) and its receptor, CXCR3. Immunochemistry with an antihuman CXCL10 showed that synovial macrophages, epithelial cells, and endothelial cells bear the chemokine. By flow cytometry and immunochemistry, it has been shown that CXCR3 is expressed at high density by virtually all T lymphocytes isolated from synovial fluid (SF) and infiltrating the synovial membrane. Particularly strongly stained CXCR3+ T cells can be observed close to the luminal space and in the perivascular area. Furthermore, densitometric analysis has revealed that the mRNA levels for CXCR3 are significantly higher in JIA patients than in controls. T cells purified from SF exhibit a definite migratory capability in response to CXCL10. Furthermore, SF exerts significant chemotactic activity on the CXCR3+ T-cell line, and this activity is inhibited by the addition of an anti-CXCL10 neutralizing antibody. Taken together, these data suggest that CXCR3/CXCL10 interactions are involved in the pathophysiology of JIA-associated inflammatory processes, regulating both the activation of T cells and their recruitment into the inflamed synovium.

Cystic fibrosis carriers have higher neonatal immunoreactive trypsinogen values than non-carriers.

Following cystic fibrosis (CF) neonatal screening implementation, a high frequency of heterozygotes has been reported among neonates with elevated immunoreactive trypsinogen (IRT) and normal sweat chloride levels. We studied the relationship between normal IRT values and CF heterozygosity: 10,000 neonates were screened for CF by IRT measurement and tested for 40 CF mutations; the 294 carriers detected were coupled with newborns negative to the same genetic testing, and the two groups' IRT levels compared. Heterozygotes had higher IRT levels than their controls (mean 35.32 vs. 27.58 microg/L, P<0.001). Even within normal trypsinogen range, the probability of being a CF carrier increases with neonatal IRT concentration.

Gender-sensitive association of CFTR gene mutations and 5T allele emerging from a large survey on infertility.

Human infertility in relation to mutations affecting the cystic fibrosis transmembrane regulator (CFTR) gene has been investigated by different authors. The role of additional variants, such as the possible forms of the thymidine allele (5T, 7T and 9T) of the acceptor splice site of intron 8, has in some instances been considered. However, a large-scale analysis of the CFTR gene and number of thymidine residues, alone and in combination, in the two sexes had not yet been addressed. This was the aim of this study. Two groups were compared, a control group of 20,532 subjects being screened for perspective reproduction, and the patient group represented by 1854 idiopathically infertile cases. Analyses involved PCR-based CFTR mutations assessment, reverse dot-blot IVS8-T polymorphism analyses, denaturing gradient gel electrophoresis (DGGE) and DNA sequencing. The expected 5T increase in infertile men was predominantly owing to the 5/9 genotypic class. The intrinsic rate of 5T fluctuated only slightly among groups, but some gender-related differences arose when comparing their association. Infertile men showed a significantly enriched 5T + CFTR mutation co-presence, distributed in the 5/9 and 5/7 classes. In contrast, females, from both the control and the infertile groups, showed a trend towards a pronounced reduction of such association. The statistical significance of the difference between expected and observed double occurrence of 5T + CFTR traits in women suggests, in line with other reports in the literature, a possible survival-hampering effect. Moreover, regardless of the 5T status, CFTR mutations appear not to be involved in female infertility. These results underline the importance of (i) assessing large sample populations and (ii) considering separately the two genders, whose genotypically opposite correlations with these phenomena may otherwise tend to mask each other.

Reduction of GAG storage in MPS II mouse model following implantation of encapsulated recombinant myoblasts.

BACKGROUND: Hunter syndrome, mucopolysaccharidosis type II (MPS II), is a X-linked inherited disorder caused by the deficiency of the enzyme iduronate-2-sulfatase (IDS), involved in the lysosomal catabolism of the glycosaminoglycans (GAG) dermatan and heparan sulfate. Such a deficiency leads to the intracellular accumulation of undegraded GAG and eventually to a progressive severe clinical pattern. Many attempts have been made in the last two to three decades to identify possible therapeutic strategies for the disorder, including gene therapy and somatic cell therapy. METHODS: In this study we evaluated the intraperitoneal implantation of allogeneic myoblasts over-expressing IDS, enclosed in alginate microcapsules, in the MPS II mouse model. Animals were monitored for 8 weeks post-implantation, during which plasma and tissue IDS levels, as well as tissue and urinary GAG contents, were measured. RESULTS AND CONCLUSIONS: Induced enzyme activity occurred both in the plasma and in the different tissues analyzed. A significant decrease in urinary undegraded GAG between the fourth and the sixth week of treatment was observed. Moreover, a biochemical reduction of GAG deposits was measured 8 weeks after treatment in the liver and kidney, on average 30 and 38%, respectively, while in the spleen GAG levels were almost normalized. Finally, the therapeutic effect was confirmed by histolochemical examination of the same tissues. Such effects were obtained following implantation of about 1.5 x 10(6) recombinant cells/animal. Taken together, these results represent a clear evidence of the therapeutic efficacy of this strategy in the MPS II mouse model, and encourage further evaluation of this approach for potential treatment of human beings.

Flow limitation in infants with bronchopulmonary dysplasia and respiratory function at school age.

Bronchopulmonary dysplasia is associated with abnormalities in lung function during infancy, yet many infants recover with no respiratory problems in the long term. We therefore did a longitudinal study of pulmonary function in 18 children with moderate to severe bronchopulmonary dysplasia. Forced expiratory volume in 1 s (FEV1) and forced mid-expiratory flow (FEF25-75) at school age were lower than normal in 15 of 18 children, and both showed a significant positive correlation with the maximal flow at functional residual capacity (Vmax(FRC)) at 24 months of age (r=0.68 and 0.85, respectively). Our results suggest that assessment of respiratory function during infancy can help to identify children with bronchopulmonary dysplasia at risk of incomplete recovery of respiratory function during childhood.

Acute surgical abdomen as presenting manifestation of Kawasaki disease.

Ten children (4.6%) among a cohort of 219 with Kawasaki disease (KD) had their onset with severe abdominal complaints. Incomplete KD presentation at the time of acute abdomen was present in nine of 10 patients. Acute abdominal pain and distension, vomiting, hepatomegaly, and jaundice were the most common symptoms at onset. Hematemesis was present in one; toxic shock syndrome requiring care in the intensive care unit occurred in four. Five patients had laparotomy, three had percutaneous transhepatic biliary drainage, and one had a gastrointestinal endoscopy. Postoperative diagnosis was gallbladder hydrops with cholestasis in five, paralytic ileus in three, appendicular vasculitis in one, and hemorrhagic duodenitis in one. All patients completely recovered, but 50% developed coronary aneurysms despite early intravenous gammaglobulin treatment. Acute surgical abdomen can be the presenting manifestation of KD. In older children with fever, rash, and acute abdominal pain or hematemesis, KD should be considered in the differential diagnosis.