• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.1
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• pp.109-117
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• 2021

Purpose: Ménétrier disease (MD) was first described in 1888, and 50 cases have been reported until now. We aimed to discuss the etiology, diagnostics, and management of MD in children. Methods: We searched for case reports published from 2014 till 2019 in English using PubMed. Articles were selected using subject headings and key words of interest to the topic. Interesting references of the included articles were also included. Results: The pathophysiology of MD is still uncertain. However, overexpression of transforming growth factor alpha with transformation of the gastric mucosa has been observed, which may be mediated by genetics and provoked by an infectious trigger. Clinically, MD is diagnosed by abdominal pain, vomiting, anorexia, and edema secondary to hypoalbuminemia. A gastroscopy with biopsy is the gold standard for the diagnosis of MD. In children, the disease is self-limiting and only requires supportive treatment. In general, children have a good prognosis and recover spontaneously within a few weeks. Conclusion: Few pediatric cases of MD have been described in recent years, and with all different etiology. Endoscopy with biopsy remains the golden standard for the diagnosis of MD, and in children, the disease is self-limiting.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.16 no.1
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• pp.10-16
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• 2013

The field of stem cell research has been rapidly expanding. Although the clinical usefulness of research remains to be ascertained through human trials, the use of stem cells as a therapeutic option for currently disabling diseases holds fascinating potential. Many pediatric gastrointestinal tract diseases have defect in enterocytes, enteric nervous system cells, smooth muscles, and interstitial cells of Cajal. Various kinds of therapeutic trials using stem cells could be applied to these diseases. This review article focuses on the recent achievements in stem cell applications for pediatric gastrointestinal tract diseases.

• Journal of Korean Neurosurgical Society
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• v.57 no.6
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• pp.432-435
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• 2015

Moyamoya disease is a unique cerebrovascular disorder characterized by idiopathic progressive stenosis at the terminal portion of the internal carotid artery (ICA) and fine vascular network. The aim of this review is to present the clinical application of quantitative digital subtraction angiography (QDSA) in pediatric moyamoya disease. Using conventional angiographic data and postprocessing software, QDSA provides time-contrast intensity curves and then displays the peak time ($T_{max}$) and area under the curve (AUC). These parameters of QDSA can be used as surrogate markers for the hemodynamic evaluation of disease severity and quantification of postoperative neovascularization in moyamoya disease.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.91-96
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• 1997

Polycystic kidney disease is defined as a heritable disorder with diffuse involvement of both kidneys without dysplasia other than cysts. The major clinical entities of autosomal recessive polycystic kidney disease and autosomal dominant polycystic kidney disease have a considerable overlap in clinical presentations and radiographic features in the pediatric population. We experienced three cases of autosomal recessive polycystic kidney disease of neonate who expired within 24 hours due to respiratory difficulty and the other case was detected by gross hematuria. So we report four cases with brief review of literatures.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.2
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• pp.139-142
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• 2016

The coexistence of Wilson disease with Alport syndrome has not previously been reported. The diagnosis of Wilson disease and its ongoing monitoring is challenging when associated with an underlying renal disease such as Alport syndrome. Proteinuria can lead to low ceruloplasmin since it is among serum proteins inappropriately filtered by the damaged glomerulus, and can also lead to increased urinary loss of heavy metals such as zinc and copper. Elevated transaminases may be attributed to dyslipidemia or drug induced hepatotoxicity. The accurate diagnosis of Wilson disease is essential for targeted therapy and improved prognosis. We describe a patient with a diagnosis of Alport syndrome who has had chronic elevation of transaminases eventually diagnosed with Wilson disease based on liver histology and genetics.

• Pediatric Infection and Vaccine
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• v.29 no.2
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• pp.61-69
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• 2022

Coronavirus disease 2019 (COVID-19) has been a global pandemic for over 2 years. During the Omicron (B.1.1.529) variant-predominant period in South Korea, confirmed cases among children and adolescents surged. This review found that, although younger children may be less susceptible to COVID-19 than adolescents, more research is needed on the role of children and adolescents in the disease's spread. Detailed epidemiological information about the transmissibility of the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) strain in children and adolescents is currently scarce, and more research is needed on the role of children and adolescents in disease's spread. There may be a difference in the proportion of cases with severe disease requiring hospitalization depending on the dominant mutant strain; however, COVID-19 generally presents with a mild-to-moderate course in children aged 5-11 years old.

• Clinical and Experimental Pediatrics
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• v.49 no.11
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• pp.1152-1157
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• 2006

Inherited metabolic disease is rare disorders that show symptoms mainly in pediatric age and early treatment is important for preventing complications of the disease. Recent development in molecular and biochemical techniques help clinicians with proper diagnosis of patients, however, many of the disease still remain lack of effective therapeutic strategies. Better understanding on biochemical and molecular basis of pathogenesis of the disease combined with advanced medical care would provide new sight on the disease that can also improve the quality of life and long-term prognosis of patients. Traditionally, there are several modalities in the treatment of metabolic diseases depend on the biochemical basis of the disease such as diet restriction, removing or blocking the production of toxic metabolites, and stimulating residual enzyme activity. The inherited metabolic disease is not familiar for many clinicians because the diagnosis is troublesome, treatment is complicated and prognosis may not as good as expected in other diseases. Recently, new therapeutic regimens have been introduced that can significantly improve the medical care of patients with metabolic disease. Enzyme replacement therapy has showed promising efficacy for lysosomal storage disease, bone marrow transplantation is effective in some disease and gene therapy has been trying for different diseases. The new trials for treatment of the disease will give us promising insight on the disease and most clinicians should have more interest in medical progress of the metabolic disease.

• Journal of the Korean Data and Information Science Society
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• v.27 no.5
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• pp.1225-1239
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• 2016

We investigate pediatric tumor incidence data collected by the Florida Association for Pediatric Tumor program using various models commonly used in disease mapping analysis. Particularly, we consider Poisson normal models with various conditional autoregressive structure for spatial dependence, a zero-in ated component to capture excess zero counts and a spatio-temporal model to capture spatial and temporal dependence, together. We found that intrinsic conditional autoregressive model provides the smallest Deviance Information Criterion (DIC) among the models when only spatial dependence is considered. On the other hand, adding an autoregressive structure over time decreases DIC over the model without time dependence component. We adopt weighted ranks squared error loss to identify high risk regions which provides similar results with other researchers who have worked on the same data set (e.g. Zhang et al., 2014; Wang and Rodriguez, 2014). Our results, thus, provide additional statistical support on those identied high risk regions discovered by the other researchers.

• Childhood Kidney Diseases
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• v.28 no.1
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• pp.1-7
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• 2024

Antenatally diagnosed urinary tract dilatation (UTD), previously referred to as antenatal hydronephrosis, is the most commonly detected abnormality by prenatal ultrasonography. Several grading systems have been developed for the classification of antenatal UTD using prenatal and postnatal ultrasonography. UTD comprises a wide variety of congenital abnormalities of the kidney and urinary tract ranging from transient UTD to more significant abnormalities such as vesicoureteral reflux, ureteropelvic junction obstruction, ureterocele, ureterovesical junction obstruction, posterior urethral valves, and non-refluxing megaureter. Optimizing the evaluation of antenatally detected UTD is essential to recognize children with important disorders while avoiding excessive investigations. Conservative approach with close follow-up is increasingly accepted as an appropriate treatment option for patients with asymptomatic vesicoureteral reflux and ureteropelvic junction obstruction in recent years. However, predicting permanent kidney damage in an unselected group of children with antenatal UTD is still challenging. The management and follow-up of children with UTD should be individualized based on recommendations from a pediatric nephrologist, a pediatric urologist, or both. Future research directed at predicting long-term outcomes of children diagnosed with UTD from mild findings to severe disease is needed to refine management for those at higher risk of kidney disease progression.

• Clinical and Experimental Pediatrics
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• v.64 no.4
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• pp.139-140
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• 2021