• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.2
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• pp.134-140
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• 2018

Chylomicron retention disease, also known as Anderson's disease, is a rare hereditary hypocholesterolemic disorder, recessive inherited, characterized by nonspecific symptoms as abdominal distension, steatorrhea, and vomiting associated with failure to thrive. We describe a patient with failure to thrive, chronic diarrhea and steatorrhea who the diagnosis of chylomicron retention disease was established after several months of disease progression. The genetic study confirmed a homozygosity mutation in SAR1B gene, identifying a mutation never previous described [c.83_84delTG(p.Leu28Argfs*7)]. With this case report the authors aim to highlight for this very rare cause of failure to thrive and for the importance of an attempting diagnosis, in order to start adequate management with low fat diet supplemented with fat-soluble vitamins, reverting the state of malnutrition and avoiding possible irreversible and desvantating complications.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.3
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• pp.310-310
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• 2020

• Journal of Korean Clinical Nursing Research
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• v.19 no.2
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• pp.195-207
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• 2013

Purpose: This study was conducted to describe the relationship of parents' stress, coping, and partnership between nurse and parents whose children were hospitalized in the Pediatric Intensive Care Unit. Methods: A descriptive correlation study design was used. The participants were 81 parents whose children were hospitalized at S-hospital located in Seoul from October 18 to November 27, 2012. This study used the 'Parental Stress Scale: Pediatric Intensive Care Unit,' the coping scale, and 'Pediatric Nurse Parents Partnership Scale, PNPPS'. The data were analyzed using t-test, ANOVA and pearson correlation. Results: The parents of children hospitalized in pediatric intensive care unit were experiencing high level of stress ($3.22{\pm}0.82$). There was a significant positive correlation between parents' coping and nurse parents partnership (p<.001), however there was no relationship between parents' stress and coping, and parents' stress and nurse parents partnership. Conclusion: Nurse-parents partnership had significant relationship with parents' coping In the care of children hospitalized in pediatric intensive care unit. The results of this study provided a foundation to recognize importance of nurse parents partnership and to develop intervention program for nurses and parents to improve their partnership.

• Child Health Nursing Research
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• v.7 no.4
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• pp.461-473
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• 2001

The purpose of this study was to identify nursing interventions performed by pediatric care unit nurses. For data collection this study used the taxonomy of Nursing Intervention Classification (NIC : 486 nursing intervention) which was modified by McCloskey & Bulecheck(2000). The 419 nursing interventions were selected by panel group, which consist of pediatric clinical experts and nursing scholars. The data were collected 104 nurses of pediatric care unit. There were 158 nursing interventions identified as being used at least monthly 50％ or more of the nurses. The 32 nursing interventions were used at least daily, indication a set of core interventions unique to pediatric care unit practice. The most frequently used nursing interventions were 'Medication administration: intravenous' & 'Medication administration: oral'. The 27 nursing interventions were rarely performed by 90％ or more of the nurses. The rarely used interventions were 'Ostomy care' & 'Rectal prolaps management'. The domain received the highest mean score was Health System, followed by Physiolocal: complex, Physiolocal: Basic, Safty, Community, Family, Behavior domain. These findings will help in building of a standardized language for the pediatric care units and enhance the quality of nursing care.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.8
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• pp.3351-3354
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• 2015

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric age group. All patients with RMS regardless of their initial stage or group receive combination chemotherapy as 'standard therapy' consisting of vincristine, actinomycin-D and cyclophosphamide. Actinomycin-D was not readily available in Turkey at one time. Carboplatin was used instead in order to prevent delays in treatment. The aim of this report is to present the results of patients with rhabdomyosarcoma receiving carboplatin or actinomycin-D therapy. Materials and Methods: Twenty four patients with rhabdomyosarcoma treated between December 2000 and June 2011 were included in this retrospective study. The patients were treated according to International Rhabdomyosarcoma Study Group guidelines. Eleven patients were treated with actinomycin-D and 13 with carboplatin ($250mg/m^2/dose$ for 2 days). The two groups were then compared in terms of 2- and 5-year overall survival (OS) and hematological and non-hematological toxicities. Results: Age, sex, stage and the mean duration of follow-up were similar in both groups (p>0.05). Two- and five-year OS levels were 68.2% in the carboplatin group and 78.0% and 40.0%, respectively, in the actinomycin-D group. There was no statistical difference in the number of febrile episodes (p=0.86) and no other hematological and non-hematological adverse effects were recorded in both groups. Conclusions: The findings show that carboplatin can be used as an alternative drug in the primary treatment of rhabdomyosarcoma in the event that actinomycin-D is unavailable or not tolerated.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.11
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• pp.4549-4553
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• 2014

Background: Infection is a serious cause of mortality in febrile neutropenia of pediatric cancer patients. Recently, monotherapy has replaced the combination therapy in empirical treatment of febrile neutropenia. Since there has been no reported trial comparing the efficacy of meropenem and piperacillin-tazobactam (PIP/TAZ) monotherapies, the present retrospective study was conducted to compare safety and efficacy in febrile neutropenic children with cancer. Materials and Methods: Charts of febrile, neutropenic children hospitalized at our center between March 2008 and April 2011 for hemato-oncological malignancies were reviewed. Patients received PIP/TAZ 360 mg/kg/day or meropenem 60 mg/kg/day intravenously in three divided doses. Duration of fever and neutropenia, absolute neutrophil count, modification, and success rate were compared between the two groups. Resolution of fever without antibiotic change was defined as success and resolution of fever with antibiotic change or death of a patient was defined as failure. Modification was defined as changing the empirical antimicrobial agent during a febrile episode. Results: Two hundred eighty four febrile neutropenic episodes were documented in 136 patients with a median age of 5 years. In 198 episodes meropenem and in 86 episodes PIP/TAZ were used. Duration of fever and neutropenia, neutrophil count, sex, and primary disease were not different between two groups. Success rates and modification rate between two groups showed no significant differences (p>0.05). Overall success rate in the meropenem and PIP/TAZ groups were 92.4% and 91.9% respectively. No serious adverse effects occurred in either of the groups. Conclusions: Meropenem and PIP/TAZ monotherapy are equally safe and effective in the initial treatment of febrile neutropenia in children with cancer.

• Journal of Cardiovascular Imaging
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• v.30 no.1
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• pp.92-94
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• 2022

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.3
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• pp.218-227
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• 2022

Purpose: To analyze the characteristics of pediatric inflammatory bowel disease (IBD) over the past three decades in Argentina and determine if there are differences between the first two decades and the past decade. Methods: We conducted a retrospective multicenter analytical study in children with IBD between 0 and 18 years of age diagnosed between 1987 and 2017 in three tertiary health centers in Argentina. The evaluation included clinical characterization, endoscopy, histology, and imaging data together with therapeutic strategies. The patients were divided into two groups: Group 1, diagnosed between 1987 and 2007, and Group 2, diagnosed between 2008 and 2017. Results: Of the 756 patients included, 409 (54%) had ulcerative colitis (UC), 250 (33%) had Crohn's disease (CD), and 97 (13%) had IBD-unclassified (IBD-U). The positive family history was 3.8%, which was more frequent among children under two years of age (6.7%). There were no significant differences in clinical presentation and extraintestinal manifestations between periods, with hepatic manifestations being the most frequent. In the last decade, we found an upward trend in CD, a downward trend in UC/IBD-U, even after adjustment for socioeconomic status, and a decrease of 50% in surgical treatments coinciding with the advent of biological therapy. Conclusion: This is the first multicenter cohort study in a Latin American country to describe clinical, endoscopic, and therapeutic data across the past 30-year period. Although CD was responsible for the overall increase in incidence, UC was still prevalent in this region.

• Clinical and Experimental Pediatrics
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• v.63 no.5
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• pp.184-188
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• 2020

Background: Many critically ill patients require transfer to a higher-level hospital for complex medical care. Despite the publication of the American Academy of Pediatrics guidelines for pediatric interhospital transportation services and the establishment of many pediatric transport programs, adverse events during pediatric transport still occur. Purpose: To determine the incidence of adverse events occurring during pediatric transport and explore their complications and risk factors. Methods: This prospective observational study explored the adverse events that occurred during the interhospital transport of all pediatric patients referred to the pediatric intensive care unit of Ramathibodi Hospital between March 2016 and June 2017. Results: There were 122 pediatric transports to the unit. Adverse events occurred in 25 cases (22%). Physiologic deterioration occurred in 15 patients (60%). Most issues (11 events) involved circulatory problems causing patient hypotension and poor tissue perfusion requiring fluid resuscitation or inotropic administration on arrival at the unit. Respiratory complications were the second most common cause (4 events). Equipment-related adverse events occurred in 5 patients (20%). The common causes were accidental extubation and endotracheal tube displacement. Five patients had both physiologic deterioration and equipment-related adverse events. Regarding transport personnel, the group without complications more often had a physician escort than the group with complications (92% vs. 76%; relative risk, 2.4; P=0.028). Conclusion: The incidence of adverse events occurring during the transport of critically ill pediatric patients was 22%. Most events involved physiological deterioration. Escort personnel maybe the key to preventing and appropriately monitoring complications occurring during transport.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.2
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• pp.244-244
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• 2021