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Three Cases of Urine Abnormalities Associated with Ketogenic Diet

Hahn HW, Kim KJ, Ha IS, Jung HI, Hwang YS, Choi Y

  • KMID: 1606287
  • J Korean Pediatr Soc.
  • 2001 Jun;44(6):709-713.
Ketogenic diet is a high-fat, low carbohydrate, low protein diet used in treatment of pediatric epilepsy since the 1920s. Currently it is used primarily to treat refractory childhood epilepsy. Few...
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A Case of Congenital Methemoglobinemia Due to NADH-Methemoglobin Reductase Deficiency

Kim DH, Lee JA, Lee SJ, Jo HS, Lee YK, Park JD, Kim BI, Choi JH, Lee DS, Cho HI, Park KR

  • KMID: 1606285
  • J Korean Pediatr Soc.
  • 2001 Jun;44(6):699-704.
Congenital methemoglobinemia is caused by NADH-methemoglobin reductase deficiency in more than half of the total reported cases. NADH-methemoglobin reductase deficiency is an uncommon hereditary disorder producing methemoglobinemia and cyanosis in...
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49, XXXXY Syndrome with Multicystic Kidney in a Neonate

Park EY, Moon SJ

  • KMID: 1606288
  • J Korean Pediatr Soc.
  • 2001 Jun;44(6):714-717.
The 49, XXXXY Klinefelter variant is distinctive; classic findings include mental retardation, hyp ogonadism and radioulnar synostosis. Its incidence is estimated to be 1/80,000 to 1/100,000 male births. We report a...
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A Case of Late Presentation of Traumatic Diaphragmatic Hernia in a Child

Jeong JY, Kim HM, Shin JK

  • KMID: 1606286
  • J Korean Pediatr Soc.
  • 2001 Jun;44(6):705-708.
Traumatic diaphragmatic injuries in infants and children are uncommon. Late presentation of such an injury is well recognised in adults but is exceptionally rare in children. Because of the increased...
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A Case of 18 Ring Chromosome

Bin JH, Song MY, Lee IG, Lee WB, Lee BC

  • KMID: 1606282
  • J Korean Pediatr Soc.
  • 2001 Jun;44(6):683-686.
A ring 18 chromosome(18r) karyotype is a rare disorder characterized by short stature, obesity, microcephaly, mental retardation, micropenis, cryptorchidism, hypertelorism, epicanthal folds, micrognathia, and small hands with short tapering fingers....
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Two Cases of Leigh Disease in Siblings

Kim SJ, Kim JE, Hwang HW, Mok JS, Lee DS, Kim DK, Choi SM, Kim WT

  • KMID: 1606289
  • J Korean Pediatr Soc.
  • 2001 Jun;44(6):718-722.
Leigh disease is a familial and degenerative disorder characterized by focal, bilateral, and usually symmetric lesions of the both gray and white matter in the brain and the spinal cord....
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The Optimum Dose of Oral Diazepam to Reduce Seizure Attack in Febrile Seizure Patients During Febrile Illness

Choi IJ, Choi JE, Lee JA, Jeong PJ, Kim SM, Jeong JH, Lee JH

  • KMID: 1606281
  • J Korean Pediatr Soc.
  • 2001 Jun;44(6):677-682.
PURPOSE: Febrile seizure is the most common type of seizure affecting 3-4% of children. The recurrence rate of febrile seizure is approximately 33%. About 16% of children with a febrile...
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Zellweger Syndrome : First Confirmed Neonatal Case in Korea

Kim SS, An YH, Chang YS, Jin DK, Park WS, Lee MH, Kim JW, Yoon HK, Kim BK

  • KMID: 1606284
  • J Korean Pediatr Soc.
  • 2001 Jun;44(6):694-698.
We describe below a case of Zellweger syndrome case with facial dysmorphism, profound hypotonia, and hepatomegaly. He died at the age of 2 months. Zellweger syndrome is a disease marked...
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A Case of Chronic Active Epstein-Barr Virus Infection with Coronary Aneurysm

Jeong H, Kim BS, Choi OJ, Yoo HW, Lim SD, Hong SJ

  • KMID: 1606283
  • J Korean Pediatr Soc.
  • 2001 Jun;44(6):687-693.
Chronic active Epstein-Barr virus infection(CAEBV) is a nonfamilial syndrome that shows a specific immunodeficiency for the Epstein-Barr virus(EBV). CAEBV is characterized by fever, lymphadenopathy, splenomegaly, hepatitis, interstitial pneumonitis, interstitial nephritis,...
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Risk Factors for Fatty Liver in Obese Children

Park KW, Lee JE, Ryu KH, Kim KH, Hong YM, Kim GH, Lee K, Yoo JH

  • KMID: 1606280
  • J Korean Pediatr Soc.
  • 2001 Jun;44(6):669-676.
PURPOSE: The incidence of obese patients has increased recently in Korea. Obesity is associated with hypertension, hyperlipidemia, insulin resistance, and fatty liver. Since recent investigators have revealed that not only...
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