• Clinical and Experimental Pediatrics
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• v.59 no.3
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• pp.145-148
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• 2016

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially fatal condition characterized by skin rash, fever, eosinophilia, and multiorgan involvement. Various drugs may be associated with this syndrome including carbamazepine, allopurinol, and sulfasalazine. Renal involvement in DRESS syndrome most commonly presents as acute kidney injury due to interstitial nephritis. An 11-year-old boy was referred to the Children's Hospital of Pusan National University because of persistent fever, rash, abdominal distension, generalized edema, lymphadenopathy, and eosinophilia. He previously received vancomycin and ceftriaxone for 10 days at another hospital. He developed acute kidney injury with nephrotic range proteinuria and hypocomplementemia. A subsequent renal biopsy indicated the presence of acute tubular necrosis (ATN) and late exudative phase of postinfectious glomerulonephritis (PIGN). Systemic symptoms and renal function improved with corticosteroid therapy after the discontinuation of vancomycin. Here, we describe a biopsy-proven case of severe ATN that manifested as a part of vancomycin-induced DRESS syndrome with coincident PIGN. It is important for clinicians to be aware of this syndrome due to its severity and potentially fatal nature.

• Clinical and Experimental Pediatrics
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• v.56 no.7
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• pp.282-285
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• 2013

Purpose: Recent studies have established the association between hypotonic fluids administration and hospital-acquired hyponatremia in children, and have contended that hypotonic fluids be removed from routine practice. To assess current intravenous fluid prescription practices among Korean pediatric residents and to call for updated clinical practice education Methods: A survey-based analysis was carried out. Pediatric residents at six university hospitals in Korea completed a survey consisting of four questions. Each question supposed a unique scenario in which the respondents were to prescribe either a hypotonic or an isotonic fluid for the patient. Results: Ninety-one responses were collected and analyzed. In three of the four scenarios, a significant majority prescribed the hypotonic fluids (98.9%, 85.7%, and 69.2%, respectively). Notably, 69.2% of the respondents selected the hypotonic fluids for postoperative management. Almost all (96.7%) selected the isotonic fluids for hydration therapy. Conclusion: In the given scenarios, the majority of Korean pediatric residents would prescribe a hypotonic fluid, except for initial hydration. The current state of pediatric fluid management, notably, heightens the risk of hospital-acquired hyponatremia. Updated clinical practice education on intravenous fluid prescription, therefore, is urgently required.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.120-125
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• 2020

Gorham-Stout syndrome is a rare bone disorder characterized by progressive massive osteolysis and proliferation of vascular and lymphatic vessels. A 15-year-old boy was initially diagnosed with Gorham-Stout at the age of 8 years based on clinical and radiological findings. Following diagnosis, he was treated with pamidronate, interferon alfa, propranolol, oral corticosteroids, and sirolimus. He developed proteinuria at the age of 15 and progressed into the nephrotic range 2 years later. A renal biopsy revealed focal segmental glomerulosclerosis, not otherwise specified variant. The sequential increase in proteinuria associated with medications suggested that the focal segmental glomerulosclerosis may be caused by pamidronate and sirolimus, but cannot completely rule out the possibility of kidney involvement of GSS itself.

• Journal of Korean Neurosurgical Society
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• v.62 no.2
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• pp.225-231
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• 2019

Objective : Epidural haematoma (EDH) most commonly occurs in the supratentorial area, particularly in the temporal region, of the brain. Posterior fossa epidural haematoma (PFEDH) is less frequently observed, accounting for only 1.2% to 12.9% of all EDH cases. Because of the non-specific symptoms and the potential for rapid and fatal deterioration in children, an early computed tomography (CT) scanning is necessary for all suspicious cases. The aim of the present study was to share the experience of 48 cases and review the literature concerning PFEDH. Methods : A retrospective analysis was conducted for 48 paediatric cases diagnosed with PFEDH and admitted to Yuying Children's Hospital of Wenzhou Medical University from January 2010 to August 2015. The clinical features and outcomes were analyzed and compared with previous literature. Results : Seventeen patients were surgically treated in this series and 31 patients received non-operative treatment. The outcomes were good in 46 patients, evaluated using the Glasgow outcome score (GOS), while mild disability was observed in one patient, and only one case showed severe disability. There were no cases of mortality in this series. Conclusion : Posterior fossa epidural haematoma is relatively rare compared with supratentorial epidural haematoma. Early and serial CT scans should be performed for all suspicious cases. The criteria for the surgical treatment of paediatric patients with PFEDH were concluded. The overall prognosis was excellent in paediatric patients.

• Clinical and Experimental Pediatrics
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• v.55 no.7
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• pp.254-258
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• 2012

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed an abnormally dilated thoracic and abdominal aorta. The infant was initially treated with prednisolone, followed by commissuroplasty of the aortic valve but neither approach ameliorated the heart failure. The patient was eventually treated with a mechanical aortic valve replacement surgery at the age of 12 months, and her condition stabilized. Although unusual, this case indicates that the diagnosis of Takayasu arteritis should be considered in children with unexplained systemic symptoms, aortic valve regurgitation, and heart failure. Because severe aortic regurgitation may be a fatal complication of Takayasu arteritis, early aortic valve replacement surgery should be considered, even in very young children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.3
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• pp.214-217
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• 2018

Inflammatory bowel disease (IBD) is a well-recognized risk factor for thrombotic events in adults but data on children are scarce. In the great majority of adult patients, thrombotic events are usually deep vein thrombosis and pulmonary embolism. Other sites such as jugular veins are extremely rare. We present a case of Lemierre syndrome in an adolescent girl with active ulcerative colitis and discuss possible risk factors. This is the first reported case of severe Lemierre syndrome with thrombus extension to cranial veins in a patient with ulcerative colitis. Early recognition of Lemierre syndrome in patients who present with rapidly worsening symptoms of neck pain, fever and signs of pharyngitis is imperative because it increases a chance of favorable prognosis. It is important for pediatricians treating IBD patients not to underestimate possible thrombotic events in children with IBD. Recognition of additional risk factors is crucial for prompt diagnosis and adequate treatment.

• Journal of Korean Public Health Nursing
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• v.29 no.3
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• pp.515-527
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• 2015

Purpose: This study was to the experiences hospital school programs health impaired children as perceived by mothers. Methods: The participants were 9 mothers of children with health impairment who had in hospital school program. ata were collected through in-depth interview using semi-structured questionnaire. The interviews were analyzed by content analysis. Results: domains and 13 categories were extracted from the analysis. The 5 domains related to the experience hospital school program were emotion, therapeutic course, interpersonal relationship, school reentry, and needs for hospital school management. Conclusion: The results of this study indicate that participation hospital school programs not only educational support but it is also helpful for physical, psychosocial adjustment of health impaired children and could be used develop effective hospital school programs to improve the adaption of children with health impairment.

• Childhood Kidney Diseases
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• v.21 no.1
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• pp.35-39
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• 2017

Azathioprine is commonly used as immunosuppressive therapy for various inflammatory diseases including chronic glomerulonephritis. Myelosuppression is a common side effect of azathioprine, resulting in the need for dose reduction. However, severe pancytopenia or alopecia is not often encountered. Here, we report a case of severe myelosuppression, and alopecia totalis that occurred after azathioprine treatment in a patient with IgA nephropathy. A 10-year-old boy with IgA nephropathy was treated with oral deflazacort and later with azathioprine. After 4 weeks, the patient complained of hair loss, and despite a dose reduction in azathioprine, he developed bone marrow suppression and alopecia totalis in two weeks. The blood indices and alopecia of the patient had returned to normal after azathioprine withdrawal and 3 consecutive doses of granulocyte colony-stimulating factor. We suggest that physicians remain vigilant to the side effects of azathioprine. Unusual hair loss after azathioprine treatment might suggest a defect in the metabolism of the drug, warranting the discontinuation of azathioprine to prevent more severe side effects.

• Child Health Nursing Research
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• v.11 no.3
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• pp.273-281
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• 2005

Purposes: The purposes of this study were to evaluate parent satisfaction with inpatient hospital services in children's hospitals and to identify variables related to parent satisfaction. Method: A descriptive correlation study was conducted. Parents of 165 children who were inpatients in two children's hospitals participated in the study. Data were collected using structured questionnaire at the time of discharge. For statistical analysis, t-test, ANOVA, and Pearson correlation analysis were used. Results: The highest parent satisfaction domain was nursing service, and the lowest parent satisfaction domain was hospital service and accommodations. Parents were less likely to be satisfied with hospital facilities, equipment, noise and cleaning and less likely to be satisfied with the lack of information they received and with the lack of communication with health care professionals. Parents with longer length of stay and with older children reported higher satisfaction than their counterparts. Moreover, parent satisfaction was related to their intention to revisit and related to intention to recommend this surveyed hospital over others. Conclusions: Efforts to improve parent satisfaction in children's hospital service and accommodation are needed to improve the quality of health care. Communication by health care professionals with parents and a partnership between parents and health care professionals are necessary to improve quality of care.

• Journal of Genetic Medicine
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• v.10 no.1
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• pp.47-51
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• 2013

Loeys-Dietz syndrome (LDS) is an autosomal dominant disorder caused by heterozygous mutations in the genes encoding transforming growth factor-${\beta}$ receptor type 1 or 2. It is typically characterized by a triad of hypertelorism, cleft palate or bifid uvula, and arterial tortuosity with aneurysm or dissection. Characteristic vascular abnormalities such as tortuosity, aneurysms, dissections, and stenosis are the most severe complications of LDS and can occur in the neurovascular system. We report a 5-year-old boy who presented with headaches and neurovascular abnormalities and was diagnosed with LDS with a novel mutation of the TGFBR1 gene. It is the first Korean report of neurovascular abnormalities in LDS.