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Case Report
Acute treatment of hyperammonemia by continuous renal replacement therapy in a newborn patient with ornithine transcarbamylase deficiency
Hyo Jeong Kim, Se Jin Park, Kook In Park, Jin Sung Lee, Ho Sun Eun, Ji Hong Kim, Jae Il Shin
Clin Exp Pediatr. 2011;54(10):425-428.   Published online October 31, 2011

Ornithine transcarbamylase (OTC) deficiency is well known as the most common inherited disorder of the urea cycle, and 1 of the most common causes of hyperammonemia in newborns. We experienced a case of a 3-day-old boy with OTC deficiency who appeared healthy in the first 2 days of life but developed lethargy and seizure soon afterwards. His serum ammonia level...

Macroamylasemia in a 4-year-old girl with abdominal pain
Jeong Hee Ko, Dae Hyoung Lee
Clin Exp Pediatr. 2009;52(11):1283-1285.   Published online November 15, 2009
Macroamylasemia is a benign condition characterized by abnormally large-sized serum amylase; it has been reported to occur in 1–2% of the population. In macroamylasemia, a macromolecular complex consisting of amylase linked to immunoglobulins circulates in the plasma and usually causes hyperamylasemia with low or normal amylasuria. Macroamylasemia is extremely rare in children. We report a case of a 4-year-old girl...
Original Article
Characterization of Molecular Defects in Korean Families with Inherited Ornithine Transcarbamylase Deficiency and Their Genotype-Phenotype Correlations
Han-Wook Yoo
Clin Exp Pediatr. 1999;42(7):900-910.   Published online July 15, 1999
Purpose : This study was undertaken to characterize molecular defects in Korean families with ornithine transcarbamylase(OTC) deficiency, correlate it with phenotype using in vitro expression study, and utilize it for making prenatal molecular diagnosis. Methods : To investigate molecular lesions resulting in OTC deficiency in 15 unrelated Korean families, the OTC genes of probands were amplified exon by exon and analyzed...
Case Report
A Case of Ornithine Transcarbamylase Deficiency Successfully Treated with Protein Restriction and Living Related Liver Transplantation
Bong Seong Kim, Kyung Mo Kim, Han-Wook Yoo, Sung Gyu Lee
Clin Exp Pediatr. 1999;42(6):868-873.   Published online June 15, 1999
Ornithine transcarbamylase deficiency(OTCD), the most common inborn error of the urea cycle, is inherited in X-linked manner. In affected hemizygote males, OTCD manifests hyperammonemic coma that often leads to death during the newborn period. Our patient was at high risk for inborn error of urea cycle metabolism, since his two elder brothers died a few days after birth due to...
Case of Chronic Pancreatitis Complicated Pancreatic Ascites and Pleural Effusion.
Gye Ja Lee, Yong Aee Chun, Hey Sun Lee, Yong Mi Hong, Young Min Ahn
Clin Exp Pediatr. 1987;30(1):108-113.   Published online January 31, 1987
A case of pancreatic ascites and pleural effusion as complication of chronic pancreatitis in the twelve-year-old boy was reported. He was admitted to the pediatric ward with chief complaints of abdominal pain and abdominal distension. He has been apparently well until one month prior to admission, when he began to feel intermittent abdominal pain and dyspnea and his abdomen was progressively distended. Serosanguinous fluid was...
Original Article
A Study on Four Serum Enzymes in Acute Leukemia in Children.
In Joon Seol, Seung Jae Yang, Kyoo Hwan Rhee, Hahng Lee, Chong Moo Park, Jae Kyung Koh
Clin Exp Pediatr. 1986;29(2):130-142.   Published online February 28, 1986
In order to find out whether some of serum enzyme levels could be used as markers for acute leukemia in children, activities of amylase, alkaline phosphatase, 5 -nucleotidase and ribonuclease (RNase) and positive rates of these enzymes as markers for acute leukemia in children were determined in serum of patients with acute lymphocytic leukemia (ALL) and acute non-lymphocytic leukemia(ANLL). Since the...
Decrease Serum Amylase Activity in Acutely Starvated Children with Epidemic Enephalitis.
Kyung Yong Huh, Chull Shon
Clin Exp Pediatr. 1984;27(12):1185-1191.   Published online December 31, 1984
To evaluate the effects of acute starvation on blood constituents, serum amylase activity and levels of serum urea nitrogen (BUN) and protein fractions were measured in 336 normal children and 60 children acutely starved as a result of encsphalitis. The mean starvation period was 4 days. Hypoalbuminemic children were also examined. The tests revealed that starvation resulted in a significant reduction in serum amylase...
Early Reduction of Serum Amylase Activity in Children with Epidemic Encephalitis.
Suk Jung Chang, Eui Hyung Kim, Tai Ju Hwang, Chull Sohn
Clin Exp Pediatr. 1984;27(4):350-357.   Published online April 30, 1984
In 52 children aged form 5 to 13 years who were acutely starving from epidemic encephalitis, authors measured the level of amylase activity, BUN, GOT, GPT, alkaline phosphatase, total protein, albumin, globulin, creatinine in serum and GOT, GPT, amylase activity in CSF. And in another group of 7 children with acute viral hepatitis, GOT, GPT in serum and CSF were...
Case Report
Pancreatic Pseudocysts and Ascites as Complications of Chronic Pancreatitis.
Nam Su Kim, Hee Ju Kim, Kyoo Duck Lee, Yong Choi, Hyung Ro Moon
Clin Exp Pediatr. 1982;25(2):183-189.   Published online February 28, 1982
A case of pancreatic pseudocysts and bloody pancreatic ascites as complications of chronic pancreatitis in eleven-year-old girl was reported. She was admitted to the pediatric ward with chief complaints of abdominal distension and bloody ascites. About nine months prior to admission, when she was stuck by a bicycle, she had sustained a blow on her abdomen. She has been apparently...
Original Article
Serum Levels of Protein, Urea Nitrogen, Uric Acid, Creatinine and Serum Amylase Activity in Normal School Children.
Suck Pill Cho, Byung Wha Park, Hyung Suk Byun, Kyung Reyong Huh, Chull Sohn
Clin Exp Pediatr. 1982;25(2):148-152.   Published online February 28, 1982
Protein, and essential nutrient for the growth of children, is the chief nitrogenous substant in the serum of humans. For the purpose of establishment of the normal data for evaluation of nitrogen balance, we examined the serum levels of urea nitrogen, creatinine, uric acid and serum amylase activity as well as that of protein in 560 normal school children. The...
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