Clinical Experiences and Selection of Accessory Devices for Pediatric Endoscopic Foreign Body Removal: A Retrospective Multicenter Study in Korea

Background@#Swallowing of foreign bodies (FBs) is the most common indication of therapeutic endoscopy in children. Endoscopic removal may be necessary depending on the type of FB, age of the child, and location of the FB. We attempted to analyze the characteristics of each device used for the endoscopic removal of FBs in children. @*Methods@#Medical records of the patient’s age, sex, weight, type, location, size, shape, type of device used for endoscopic removal, and endoscopic time were retrospectively collected. @*Results@#A total of 424 FB removal procedures were analyzed. The average age of the patients at the time of FB removal was 4.1 ± 3.7 years. Coins were the most common FBs (192, 45.3%). The most common locations of the FBs were the esophagus (45.7%) and the stomach (48.3%), respectively. For a total of 371 cases, forceps were used in 96 cases (25.9%) for esophageal FBs and in 25 cases for gastric FBs (6.7%), while nets were used in 250 cases (67.4%) for gastric FBs retrieval; the average durations of the procedures were 7.2 ± 7.4 minutes, 8.5 ± 7.2 minutes, and 5.7 ± 7.3 minutes, respectively (P = 0.003). The procedure time was significantly shorter, in the group of patients with low body weights, when nets were used than when forceps were used to remove gastric FBs (P = 0.001). @*Conclusion@#The endoscopic procedure duration, in low-weight children, was shorter when retrieval nets were used than that with forceps.

Epidemiological Correlation between Fecal Adenovirus Subgroups and Pediatric Intussusception in Korea

We investigated the adenoviral etiology and seasonal epidemic trends in intussusception and each adenoviral subgroup. Also we confirmed whether we can use the adenovirus data of Acute Infectious Agents Laboratory Surveillance Report (AIALSR) as an epidemic predictor of intussusception. Patients with intussusception (n = 126), < 5 years old, were enrolled and matched by age and sex with controls suffering acute gastroenteritis without intussusception (n = 106), all recruited at 8 centers. All fecal specimens were assayed for adenovirus, including subgroups A, B, C, E, and F, with reverse transcriptase-polymerase chain reaction (RT-PCR). Adenovirus was detected in 53 cases and 13 controls (P < 0.001). Nonenteric adenoviruses (NEAds) were detected in 51 cases and four controls (P < 0.001). We used Spearman's correlation analysis to analyze the incidence of intussusception and adenoviral epidemic trends, and compared them with fecal and respiratory adenoviral epidemic trends in the AIALSR. The trend of intussusception correlated with total NEAds (r = 0.635; P = 0.011), as did the fecal AIALSR adenovirus trends (r = 0.572; P = 0.026). Among the NEAd subgroups, subgroup C was dominant (P < 0.001), but subgroups B (P = 0.007) and E (P = 0.013) were also significant to intussusception. However, only subgroup C showed a significant epidemic correlation (r = 0.776; P = 0.001) with intussusception. Not respiratory but fecal AIALSR adenovirus trends correlated with the incidence of NEAds and intussusception. We suggest the possibility of using fecal AIALSR adenovirus data as an approximate epidemic predictor of intussusception.

Prevalence, Clinical Characteristics, and Management of Functional Constipation at Pediatric Gastroenterology Clinics

The purpose of this study was to investigate the prevalence, clinical characteristics, and management of functional constipation at pediatric gastroenterology clinics. A prospective survey using the Rome III criteria was distributed to a group of parents of children with a constipation history and its control group in May 2008. The mean prevalence of constipation was 6.4%, which was similar to those in other countries. Statistically significant variables for children without constipation were that more children had a body mass index of below the 10th percentile even though they received more mother's care and ate balanced meals compared to the constipation group. Meanwhile, the constipation group frequently showed a history of constipation in infancy, picky-eating, lack of exercise, and retentive posturing. When analyzed with the Rome III criteria, the children showed greater than 60% rate of hard stools, painful stools, a history of large fecal mass in rectum, and its disappearance of constipation symptoms after passing a large stool. Our study found different approaches amongst pediatric gastroenterologists like rectal examinations, disimpaction, or drug treatment. Several factors addressed in our study can provide better guidelines for clinicians treating constipation and its future research.

Association of SCORAD Index and Aeroallergen Sensitization on Urinary Leukotriene E4 in Children with Atopic Dermatitis / 소아알레르기및호흡기학회지

PURPOSE: Atopic dermatitis (AD) is a genetically determined, chronic relapsing skin disease. The pathogenesis of AD is complex and the course is unpredictable. Atopy is an important risk factor for the development of AD. Cysteinyl leukotrienes (Cys-LTs) were implicated in the pathophysiology of allergic diseases, and are being targeted for their diagnosis and treatments. Early detection of tissue inflammation of target organ is important to enable early prevention and management of allergic diseases. The aim of our study is to evaluate the differences in urinary leukotrienes E4 (LTE4) levels, according to AD symptom score and aeroallergen sensitization in children with AD by using noninvasive techniques. METHODS: We recruited 46 children with AD, using predetermined criteria. Clinical features of AD were evaluated by a physician, using scoring atopic dermatitis (SCORAD) index. Aeroallergen sensitization was measured by using a skin prick test and UniCap. Urine samples were also collected on day of the 1st and 2nd visits, and were analyzed for LTE4 with an enzyme-linked immunoassay kit. RESULTS: SCORAD indeces of children with AD were correlated with urinary LTE4 levels. Total immunoglobulin E (IgE) and eosinophil counts also had significant correlation with urinary LTE4 levels. Especially, aeroallergen sensitization of atopic AD significantly correlated with urinary LTE4 of these patients. CONCLUSION: Urinary LTE4 levels significantly correlated with serum total IgE and number of sensitized aeroallergen in children with AD. Clinical features of AD evaluated with SCORAD index related with urinary LTE4 level. Urinary LTE4 might be a valuable, noninvasive marker for different pathogenesis of AD.

Authorship / 대한소아소화기영양학회지

Authors are actual contributors on article's academic and publication procedure. The contributions on publication process include the establishment of conception and design, the collection, analysis and interpretation of data, drafting or correction of article and final approval of the paper. Recently, the relationship between researchers becomes more complex because of more numbers of researchers and more co-works with various specialized research fields than before. Authorship is the deciding of roles between authors. It is very important on performing scholarly work in terms of academic and publication ethics. Inappropriate authorship encompasses gift, honorary, ghost, swapping and disguised authorship. Inappropriate authorship often lead legal problem as well as ethic problem or conflict of interest. Therefore obvious establishment of authorship is ethically and legally important to researchers.

Authorship / 대한소아소화기영양학회지

Authors are actual contributors on article's academic and publication procedure. The contributions on publication process include the establishment of conception and design, the collection, analysis and interpretation of data, drafting or correction of article and final approval of the paper. Recently, the relationship between researchers becomes more complex because of more numbers of researchers and more co-works with various specialized research fields than before. Authorship is the deciding of roles between authors. It is very important on performing scholarly work in terms of academic and publication ethics. Inappropriate authorship encompasses gift, honorary, ghost, swapping and disguised authorship. Inappropriate authorship often lead legal problem as well as ethic problem or conflict of interest. Therefore obvious establishment of authorship is ethically and legally important to researchers.

Gastro-Esophageal Reflux Disease in Healthy Older Children and Adolescents / 대한소아소화기영양학회지

Gastro-esophageal reflux disease (GERD) in otherwise healthy older children and adolescents is commonly encountered in pediatric clinics and poses a complex treatment problem involving changes of diets and lifestyle. After an initial history taking and a physical examination, typical symptoms of GERD in older children and adolescenct are initially treated with the trials of acid suppressants. With an increase of severe cases, more and more GERD children have been evaluated with endoscopy, which helps to delineate an erosive esophagitis from a non-erosive reflux disease as they are presumed to have different pathogenesis. For the pediatric patients without a significant underlying disease, a reflux esophagitis can be treated adequately with acid suppressants. Recently, the rapid increase of children who are taking anti-reflux medication has brought up a serious alarm among pediatricians. Some at risk pediatric patients with recurrent and/or chronic GERD have been linked to adulthood GERD. In this paper, pediatric GERD with and without erosive esophagitis was reviewed along with treatment options and issues specifically for the otherwise healthy older children and adolescents in the primary clinics or the secondary hospitals.

Breakfast and Health in Adolescents / 대한소아소화기영양학회지

Breakfast is an important meal for adolescents to supply adequate nutrition for growth and maintenance and to prevent chronic disease. Nevertheless, the rate of skipping breakfast, which decreased 6 years ago, has gradually increased again in recent years in Korea. Manystudies show that skipping breakfast increases the risk for obesity, amenorrhea, and poor academic performance. The recommended breakfast for adolescents is a low glycemic load (GL) diet, fiber-rich family breakfast. The hunger and lazy feeling after breakfast are enhanced by a high GL and low fiber diet, which leads to additional intake of calories. A family breakfast can help to maintain a healthy life-style and reduce the risk of chronic diseases such as metabolic syndrome, obesity, type 2 diabetes, cancer, or cardiovascular disease.

Nonpharmacologic Pain Relief with Oral 25% Dextrose or/and Pacifier for Newborn Infants

PURPOSE: The goal of this study was to compare the efficacy of oral 25% dextrose treatment or/and pacifier for analgesia in healthy newborns during intramuscular injection of a hepatitis B vaccine. METHODS: A prospective, randomized, partially blinded, clinical trial was performed in 132 healthy newborns. They were assigned randomly to 4 treatment groups: control group (2 mL distilled water), dextrose group (2 mL 25% dextrose), pacifier group, dextrose+ pacifier group (pacifier coating with 25% dextrose) during intramuscular injection of hepatitis B vaccine. For all groups, Neonatal Infant Pain Scale (NIPS), Neonatal Facial Coding System (NFCS), Premature Infant Pain Profile (PIPP) scores were evaluated before the injection, during the injection, and at 2 minutes after the injection. Pain scores were compared among the 4 groups. RESULTS: Maternal and neonatal characteristics were similar among the 4 groups. 25% dextrose treatment led to lower NIPS pain scores during injection (6.4+/-0.9 vs. 5.5+/-1.7, P=0.01) and after injection (1.6+/-2.0 vs. 0.6+/-0.9, P=0.01) and NFCS pain scores after injection (1.5+/-2.3 vs. 0.7+/-0.8, P=0.04) than control group. The number of neonates who feel the pain (indication of scores: NIPS> or =4, NFCS> or =3) decreased (9 (23.1%) vs. 0 (0%), P=0.04 via NIPS, 7 (17.9%) vs. 0 (0%), P=0.02 via NFCS). However, all treatment groups did not decreased PIPP scores, compared with the control group. CONCLUSION: Oral 25% dextrose is effective than distilled water or using pacifier with or without 25% dextrose in reducing pain during intramuscular injection of hepatitis B vaccinations. Further study based on this preliminary study need about nonpharmacologic management of pain in newborns.

Current feeding practices and maternal nutritional knowledge on complementary feeding in Korea / 소아과

PURPOSE: To evaluate current feeding practices and maternal nutritional knowledge on complementary feeding. METHODS: Mothers of babies aged 9-15 months who visited pediatric clinics of 14 general hospitals between September and December 2008 were asked to fill questionnaires. Data from 1,078 questionnaires were analyzed. RESULTS: Complementary food was introduced at 4-7 months in 89% of babies. Home-made rice gruel was the first complementary food in 93% cases. Spoons were used for initial feeding in 97% cases. At 6-7 months, <50% of babies were fed meat (beef, 43%). Less than 12-month-old babies were fed salty foods such as salted laver (35%) or bean-paste soup (51%) and cow's milk (11%). The following were the maternal sources of information on complementary feeding: books/magazines (58%), friends (30%), internet web sites (29%), relatives (14%), and hospitals (4%). Compared to the 1993 survey, the incidence of complementary food introduction before 4 months (0.4% vs. 21%) and initial use of commercial food (7% vs. 39%) had decreased. Moreover, spoons were increasingly used for initial feeding (97% vs. 57%). The average maternal nutritional knowledge score was 7.5/10. Less percentage of mothers agreed with the following suggestions: bottle formula weaning before 15-18 months (68%), no commercial baby drinks as complementary food (67%), considering formula (or cow's milk) better than soy milk (65%), and feeding minced meat from 6-7 months (57%). CONCLUSION: Complementary feeding practices have considerably improved since the last decade. Pediatricians should advise timely introduction of appropriate complementary foods and monitor diverse information sources on complementary feeding.

The Nutritional Assessment in Children: Understanding of Anthropometric Assessment and Biochemical Indexes in Children / 대한소아소화기영양학회지

Nutritional assessment is based on anthropometric, clinical, dietary and biochemical data. There is a lack of studies about the propriety of biochemical indexes for the nutritional assessment in children despite biochemical data in pediatric population are different from them in adult's in many respects. Serum albumin is useful index to evaluate the severity of malnutrition. Hemoglobin and hematocrit tend to decrease in malnutrition on account of defect of iron metabolism and to increase in metabolic syndrome on account of enhancement of erythropoiesis. But, unlike adult, total lymphocyte count is not so useful biochemical indexes in children. We should consider pediatric characteristic when interpret biochemical indexes for nutritional assessment in children, and nutritional status in children should be assessed comprehensively with anthropometric, clinical, dietary and biochemical data.

The effectiveness of biochemical indexes for evaluating the nutrition states of children / 소아과

PURPOSE: This study has been conducted to analyze whether the biochemical nutrition indexes might be useful and effective for evaluating the nutrition states of children. METHODS: We evaluated 269 children, aged 3-9 years old, who had visited Gangneung Asan Hospital for elective surgery from January 2006 to December 2007, and examined their anthropometric and preoperative laboratory data with retrospective analysis. The children were classified into underweight, normal weight, overweight, and obese groups according to body mass index (BMI). The biochemical nutrition indexes (total lymphocyte count (TLC), hemoglobin, hematocrit, serum albumin, cholesterol, et al) of each group were then analyzed statistically. RESULTS: None of the groups showed statistically significant differences in TLC. Serum albumin decreased significantly in the underweight group. Red blood cell (RBC) count, hemoglobin, hematocrit, and serum total cholesterol in the obese group were higher than in the normal weight group. None of the groups showed statistically significant increase in mean corpuscular volume or mean corpuscular hemoglobin, and it seems that the increase of hemoglobin and RBC count in the overweight and obese groups is due to the enhancement of erythropoiesis rather than iron metabolism. However, in females, almost all nutrition indexes except albumin were statistically significantly poor. CONCLUSION: Serum albumin, total cholesterol, RBC count, hemoglobin, and hematocrit were useful as nutrition indexes. However, except for albumin, these indexes were significantly poor for females. More control studies are needed to confirm the effectiveness of biochemical indexes for evaluating the nutritional state of children.

Variation of parameters according to cardiac cycle length, evaluated by TDI in children / 소아과

PURPOSE: This study aimed to determine the variation in parameters according to cardiac cycle length (CL; time interval between the QRS peaks on ECG) in children by using the conventional pulsed Doppler and tissue Doppler imaging echocardiography. METHODS: Eighteen children with an anatomically normal heart were enrolled for the study. All children were examined by conventional and pulsed Doppler echocardiography at Gangneung Asan hospital between July 2006 and June 2007. We measured the CLs, mitral inflow velocities (E,A) and tissue Doppler imaging (TDI) parameters (s', e', a') from apical 4-chamber view. The TDI parameters were measured at the lateral (Lat) and septal (Sep) part of the mitral valve. All parameters were measured at 6 to 18 consecutive beats from each child. We then evaluated the linear correlation between CL and each parameter. RESULTS: The mean age was 3.6+/-0.5 years (M:F=8:10). There were significantly negative linear correlations between CL and A, Lat s', Lat a', Sep s', Sep e', Sep a' (P<0.01). There were significantly positive linear correlations between CL and E/A, Lat e'/a', Sep e'/a' (P<0.01). However, the E and Lat e' were not correlated with CL (P=0.229 and 0.221, respectively). CONCLUSION: This study showed that the values of the left ventricular functional parameters were changed according to CL. From our results, it is imperative to carefully examine beat-to-beat variations in children.

The Effect of Treating Head Lice on Residual Lindane Concentrations in Children / 대한피부과학회지

BACKGROUND: Organochloride pesticides are prohibited around the world due to their toxicity and environmental persistence. However, lindane, one organochloride pesticide, is still widely used to treat head lice and scabies in Korea. OBJECTIVE: For evaluation of the effect of lindane usage and blood lindane concentration, we conduct an evaluation of blood lindane concentrations among children who were treated for head lice. METHODS: The total study population of 80 was divided into rural and urban areas. Age, sex, parent's farming, sea food consumption and head lice treatment information were gathered using a structured questionnaire. Blood lindane concentrations were analyzed by a solid phase extraction(SPE) method. Quantitative analysis was done by GC/ECD. Non-parametric analysis was done for comparing blood lindane concentrations between the head lice treated group and the non treatment group. After a log transformation, regression analysis was done for controlling confounding variables. RESULTS: The median age of the study population was 11. The study population involved 38 (47.5%) male and 42 (52.5%) female children. Twenty (25.6%) children were infested by head lice. The blood concentration of lindane was higher in the head licetreatment group [N.D (N.D-78.9)] than in the non-treated group [N.D (N.D-43.4), P < 0.01]. The results were the same after regression analysis controlling for confounding variables. CONCLUSION: Lindane has toxicity to the endocrine system and nervous system and persists in the environment and in the human body. Therefore, careful usage of lindane shampoo is needed to treat head lice.

The Effect of Treating Head Lice on Residual Lindane Concentrations in Children / 대한피부과학회지

BACKGROUND: Organochloride pesticides are prohibited around the world due to their toxicity and environmental persistence. However, lindane, one organochloride pesticide, is still widely used to treat head lice and scabies in Korea. OBJECTIVE: For evaluation of the effect of lindane usage and blood lindane concentration, we conduct an evaluation of blood lindane concentrations among children who were treated for head lice. METHODS: The total study population of 80 was divided into rural and urban areas. Age, sex, parent's farming, sea food consumption and head lice treatment information were gathered using a structured questionnaire. Blood lindane concentrations were analyzed by a solid phase extraction(SPE) method. Quantitative analysis was done by GC/ECD. Non-parametric analysis was done for comparing blood lindane concentrations between the head lice treated group and the non treatment group. After a log transformation, regression analysis was done for controlling confounding variables. RESULTS: The median age of the study population was 11. The study population involved 38 (47.5%) male and 42 (52.5%) female children. Twenty (25.6%) children were infested by head lice. The blood concentration of lindane was higher in the head licetreatment group [N.D (N.D-78.9)] than in the non-treated group [N.D (N.D-43.4), P < 0.01]. The results were the same after regression analysis controlling for confounding variables. CONCLUSION: Lindane has toxicity to the endocrine system and nervous system and persists in the environment and in the human body. Therefore, careful usage of lindane shampoo is needed to treat head lice.

Comparison of Neonatal Medical Terminology between South Korea and North Korea / 대한주산의학회잡지

OBJECTIVE: The purpose of this study is to analyze the similarities and differences in neonatal medical terminology between South Korea and North Korea. METHODS: This study was carried out by searching North Korean neonatal terminologies used in textbooks published in North Korea and comparing those terms with the South Korean terms used in textbooks in South Korea. RESULTS: The North Korean terms are different from the South Korean terms in that North Korean terms adopt more from the native language. There are also some differences in the inscription of foreign language, spelling and spacing. But, there are few terms that cannot be capable of communication. CONCLUSION: We could identify the major differences between South Korean and North Korean neonatal medical terms. We should take effort to establish unified terms by establishing more communication between both sides.

Assessment of the Effectiveness of Biofeedback Therapyin Children with Pelvic Floor Dyssynergia / 대한소아소화기영양학회지

PURPOSE: Recently well-developed anorectal function tests have revealed that there is an obvious pelvic floor dyssynergia (PFD) pattern in pediatric patients with constipation, as well as in adult's. The use of biofeedback therapy (BT) has been widely implemented in adult PFD patients; however, this approach has only rarely been considered for pediatric PFD patients. Therefore, we assessed the effectiveness of BT in children with PFD. METHODS: We studied 70 children with PFD, who were referred to the department of pediatrics at the Asan Medical Center for the management of soiling or chronic constipation from September 2002 to February 2005. Diagnosis of PFD and assessment of the efficacy of BT for PFD treatment were carried out along with several ano-rectal function tests (cine-defecography, ano-rectal manometry, balloon expulsion test and intra-anal EMG); in addition, a questionnaire was administered. The BT based intra-anal EMG was performed. A follow-up telephone interview was performed more than 6 months later. RESULTS: Most of the symptoms and results of the ano-rectal function tests were statistically improved after BT. In comparisons between the BT and control groups (BT refusal group due to poor compliance), the symptoms were statistically improved at follow-up. Therefore, for the short- term improvement of symptoms, BT was better than conservative therapy alone. The negative feelings associated with ano-rectal function testing and BT were directly associated with failure or success of therapy. CONCLUSION: Pediatric patients with constipation or soiling that presented with an obvious PFD pattern showed that BT was a useful therapeutic tool for rapid improvement of symptoms.

A Case of Multiple Gastric Mucosa Lacerations in Near Drowning

The causes of complicated upper gastrointestinal bleeding in near-drowning have been known to be stress-induced gastritis, gastric ulcer, Mallory-Weiss syndrome, or Boerhaave syndrome. However, gastric mucosa laceration has often been observed in a drowned body's autopsy. Gastric fundus is the most common site of gastric mucosa lacerations, and the number of lacerations is almost always less than three. Reflex gasping in drowning leads to passive vigorous vomiting, the mechanical force of which causes gastric mucosa laceration. Gastric mucosa laceration is an important forensic clue in autopsy because it means that the victim was drowned alive or was abandoned after being murdered. However, gastric mucosa laceration has rarely been observed in a drowning survivor. We report a neardrowning case of a 9-year-old boy, who had multiple gastric mucosa lacerations treated by endoscopic hemostatic procedure. In our case, we suspect abdominal blunt trauma also to be another cause of gastric mucosa laceration because of uncommon site of the laceration and the large number of lesions. We also present a literature review.

The difference between the two methods for myocardial performance index in children / 소아과

PURPOSE: The object of this study was to determine the difference between two methods for myocardial performance index(MPI) in children, using the conventional and pulsed Doppler echocardiography. METHODS: A total of 27 children with anatomically normal hearts were enrolled for the study. all were examined by conventional and pulsed Doppler echocardiography at Gangneung Asan Hospital between December, 2005 and February, 2006. First, we measured the time interval(a1) between the mitral inflows from apical 4-chamber view, and the ejection time(ET1) from apical 5-chamber view. And then, we calculated MPI1, isovolumic contraction time(ICT1) and isovolumic relaxation time (IRT1). Secondly, we measured ICT2, ET2 and IRT2 from apical 5-chamber view with a Dopper signal placed at just below junction between mitral and aortic valve at the same cardiac cycle. And then, we calculated MPI2. We compared MPI1 to MPI2. All MPIs were calculated by using the formula, MPI=(ICT+IRT)/ET. RESULTS: The mean age was 5.7+/-2.2 years old(M:F=15:12). The MPI2 was higher than MPI1: 0.277+/-0.083 vs. 0.428+/-0.081(MPI1 vs MPI2, P=0.000). Also, the ICT2 was higher than ICT1: 56+/-15 msec vs 97+/-18 msec(ICT1 vs ICT2, P=0.000) and the IRT2 was higher than IRT1: 42+/-8 msec vs 53+/-9 msec(IRT1 vs IRT2, P=0.000). But, the ET2 was lower than ET1: 260+/-16 msec vs 254+/-14 msec (ET1 vs ET2, P=0.01). There was, as well, positive linear correlation between MPI1 and MPI2. CONCLUSION: This study showed that there is a difference between MPI1 and MPI2 in connection with estimating methods. However, the two MPIs had a positive linear correlation. Judging from our results, the MPI of the new method might be a useful index of venticular global function in children.

Hemophagocytic Syndrome Presenting as Severe Acute Hepatitis / 대한소아소화기영양학회지

PURPOSE: Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis. METHODS: Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively. RESULTS: Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11). CONCLUSION: HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.