Article 58(6); Jun 2015
Review Article
Haploidentical hematopoietic stem cell transplantation in children and adolescents with acquired severe aplastic anemia
Ho Joon Im, Kyung-Nam Koh, Jong Jin Seo
Clin Exp Pediatr. 2015;58(6):199-205.   Published online June 22, 2015

Severe aplastic anemia (SAA) is a life-threatening disorder for which allogeneic hematopoietic stem cell transplantation (HSCT) is the current available curative treatment. HSCT from matched sibling donors (MSDs) is the preferred therapy for children with acquired SAA. For patients who lack MSDs, immunosuppressive therapy (IST) is widely accepted as a first-line treatment before considering HCT from an unrelated donor (URD)....

Original Articles
Initial steroid regimen in idiopathic nephrotic syndrome can be shortened based on duration to first remission
Hee Sun Baek, Ki-Soo Park, Hee Gyung Kang, Cheol Woo Ko, Min Hyun Cho
Clin Exp Pediatr. 2015;58(6):206-210.   Published online June 22, 2015
Purpose

The use of a 12-week steroid regimen (long-term therapy, LT) for the first episode of idiopathic nephrotic syndrome (NS) reportedly induces a more sustained remission and lower relapse rate than previous regimens, including an 8-week steroid regimen (short-term therapy, ST). Here, we assessed the potential for selective application of 2 steroid regimens (LT vs. ST) based on the days to...

Hepatitis associated with Mycoplasma pneumoniae infection in Korean children: a prospective study
Kyu Won Kim, Jae Jin Sung, Hann Tchah, Eell Ryoo, Hye Kyung Cho, Yong Han Sun, Kang Ho Cho, Dong Woo Son, In Sang Jeon, Yun Mi Kim
Clin Exp Pediatr. 2015;58(6):211-217.   Published online June 22, 2015
Purpose

Mycoplasma pneumoniae (MP) infection is a major cause of respiratory infection in school-aged children. Extrapulmonary manifestations of MP infection are common, but liver involvement has been rarely reported. The aim of this study was to determine the clinical characteristics of MP-associated hepatitis.

Methods

This prospective study included 1,044 pediatric patients with MP infection diagnosed serologically with MP IgM at one medical center...

Cardiopulmonary function and scoliosis severity in idiopathic scoliosis children
Seokwon Huh, Lucy Yougmin Eun, Nam Kyun Kim, Jo Won Jung, Jae Young Choi, Hak Sun Kim
Clin Exp Pediatr. 2015;58(6):218-223.   Published online June 22, 2015
Purpose

Idiopathic scoliosis is a structural lateral curvature of the spine of unknown etiology. The relationship between degree of spine curvature and cardiopulmonary function has not yet been investigated. The purpose of this study was to determine the association between scoliosis and cardiopulmonary characteristics.

Methods

Ninety children who underwent preoperative pulmonary or cardiac evaluation at a single spine institution over 41 months were...

Thyroid dysfunction in very low birth weight preterm infants
Ji Hoon Lee, Sung Woo Kim, Ga Won Jeon, Jong Beom Sin
Clin Exp Pediatr. 2015;58(6):224-229.   Published online June 22, 2015
Purpose

Thyroid dysfunction is common in preterm infants. Congenital hypothyroidism causes neurodevelopmental impairment, which is preventable if properly treated. This study was conducted to describe the characteristics of thyroid dysfunction in very low birth weight infants (VLBWIs), evaluate risk factors of hypothyroidism, and suggest the reassessment of thyroid function with an initially normal thyroid-stimulating hormone (TSH) as part of a newborn...

Case Reports
A novel association between cerebral sinovenous thrombosis and nonketotic hyperglycinemia in a neonate
Sadık Yurttutan, Mehmet Yekta Oncel, Nursel Yurttutan, Halil Degirmencioglu, Nurdan Uras, Ugur Dilmen
Clin Exp Pediatr. 2015;58(6):230-233.   Published online June 22, 2015

Lethargy in newborns usually indicates central nervous system dysfunction, and many conditions such as cerebrovascular events, infections, and metabolic diseases should be considered in the differential diagnosis. Nonketotic hyperglycinemia is an autosomal recessive error of glycine metabolism, characterized by myoclonic jerks, hypotonia, hiccups, apnea, and progressive lethargy that may progress to encephalopathy or even death. Cerebral sinovenous thrombosis is a...

Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome
In Ah Jung, Won Kyoung Cho, Yeon Jin Jeon, Shin Hee Kim, Kyoung Soon Cho, So Hyun Park, Min Ho Jung, Byung-Kyu Suh
Clin Exp Pediatr. 2015;58(6):234-237.   Published online June 22, 2015

Hepatic glycogenosis in type 1 diabetes mellitus (DM) can be caused by poor glycemic control due to insulin deficiency, excessive insulin treatment for diabetic ketoacidosis, or excessive glucose administration to control hypoglycemia. Mauriac syndrome, which is characterized by hepatomegaly due to hepatic glycogenosis, growth retardation, delayed puberty, and Cushingoid features, is a rare diabetic complication. We report a case of...


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