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Two Cases of Systemic Amyloidosis Presenting with Abnormalities in Liver Function Tests

Park JH, Park CK, Yun YM, Hyun DW, Kim ES, Park SY, Jo CM, Tak WY, Kweon YO, Kim SK, Choi YW

  • KMID: 772566
  • Korean J Gastroenterol.
  • 2003 Oct;42(4):341-346.
Systemic amyloidosis results from the deposition of insoluble, fibrous amyloid proteins. It occurs mainly in the extracellular spaces of multiple organs and tissues including the kidney, heart, and liver. Although...
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Hepatoblastoma with budd-chiari syndrome in child: a case report

Park JM, Kim IO, Yeon KM

Diverse causes evoke Budd-Chiari syndrome including mallignant hepatic tumors such as hepatoblastoma inchildhood. The Budd-Chiari syndrome classically shows the symptom traid of ascites, hepatomegaly and abdominalpain. The radiologic findings include...
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A Case of Letterer - Siwe Disease

Kim YC, Chang CY

  • KMID: 1677691
  • Korean J Dermatol.
  • 1978 Jun;16(3):207-213.
Letterer-Siwe disease in an acutely onset, progressive and fatal disorder of RES, usually appears in male children, chareacterized by constitutional signs and extraos-seous lesions. This case occurred in a 18-day-old...
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Clinical, Biochemical, and Genetic Characterization of Glycogen Storage Type IX in a Child with Asymptomatic Hepatomegaly

Kim JA, Kim JH, Lee BH, Kim GH, Shin YS, Yoo HW, Kim KM

Glycogen storage disease type IX (GSD IX) is caused by a defect in phosphorylase b kinase (PhK) that results from mutations in the PHKA2, PHKB, and PHKG2 genes. Patients usually...
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A Case of Letterer-Siwe Disease in Adult

Lee SJ, Won DH, Choi GS, Koo SW, Lee JH, Kim YK

  • KMID: 2146483
  • Korean J Dermatol.
  • 2000 Dec;38(12):1688-1690.
Letterer-Siwe disease is a one of Langerhans cell histiocytosis and characterized by proliferation of Langerhans cells. It's clinical features are onset of infancy, hemorrhagic crusted papules and petechiae, hepatomegaly, lymphadenopathy,...
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A Case of Hepatoblastoma

Kwack YH, Son SC, Whang KT, Cho SH, Lee AH, Lee CM

  • KMID: 1663535
  • J Korean Pediatr Soc.
  • 1978 Apr;21(4):299-304.
We have experienced a case of hepatoblastoma in 22 months old male infant who presented with abdominal distention and hepatomegaly. Diagnosis was confirmed by X-ray of abdomen, liver scan and...
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A Case of Mauriac Syndrome

Kim YH, Lee KH, Yoo KH, Hong YS, Lee JW, Kim SK

  • KMID: 2193860
  • J Korean Soc Pediatr Endocrinol.
  • 1999 Jun;4(1):100-103.
In 1930s, Mauriac described a syndrome in diabetic children consisting of stunted growth, hepatomegaly, and delayed puberty. This syndrome was related to poorly controlled diabetes of long duration. A 14-year-old...
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A Case of von Gierke Disease

Joe YA, Song MY, Cho B, Lee SJ, Sung IK, Lee KS

  • KMID: 2335312
  • J Korean Pediatr Soc.
  • 1997 Dec;40(12):1756-1756.
von Gierke disease (type Ia glycogen storage disease) is an inherited disease associated with accumulation of glycogen in the liver, kidney, intestine and erythrocytes due to the defect of glucose-6-phosphatase...
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A Case of Berardinelli Lipodystrophy Syndrome

Hwang JS, Lim JS, Kim SY, Shim KS, Yang SW, Yu JS

  • KMID: 1529898
  • J Korean Soc Pediatr Endocrinol.
  • 1997 Sep;2(2):274-276.
Berardinelli lipodystrophy syndrome is a rare autosomal recessive disorder, characterized by loss of body fat, muscular hypertrophy, acanthosis nigricans, hepatomegaly, hyperlipidemia, insulin resistant diabetes, and elevated metabolic rate. The mechanism(s)...
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A Case of Hepatomegaly due to Diabetic Glycogenosis Reversed by Glycemic Control

Park J, Song DH, Park JS, Nam JY, Kim CS, Kim DM, Ahn CW, Cha BS, Lim SK, Kim KR, Lee HC, Kim HR, Park CI

  • KMID: 1837630
  • J Korean Soc Endocrinol.
  • 2004 Apr;19(2):223-228.
Diabetes mellitus is well known to be associated with various structural and functional liver abnormalities. If diabetic patients are accompanied by hepatomegaly or abnormal findings from a liver function...
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Secondary Amyloidosis Involving Liver in Rheumatoid Arthritis Treated with Methotrexate and Low dose Prednisolone

Han YJ, Song CH, Sheen DH, Yang SS, Lee JY, Lim YS, Song YW

  • KMID: 2334768
  • J Korean Rheum Assoc.
  • 2000 Sep;7(3):291-296.
Amyloidosis is a heterogenous group of often fatal disorders characterized by extracellular deposition of a proteinaceous material with a unique fibrillar form in various tissues and organs. Presenting with severe...
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A Case of Glycogen Storage Disease with Hypertrophic Cardiomyopathy

Kim DH, Kang SW, Park WJ, Jang KA, Choi JH, Kim W, LEE SH, Hong GR

Glycogen storage diseases are a heterogeneous group of metabolic disorder affecting multiple organ system: liver, skeletal muscle, heart and brain. Clinical features include: short status, hepatomegaly, hypoglycemia, dyslipidemia and rare...
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Pleuropulmonalry amebiasis

Lee DH, Ra WY, Suh SJ, Woo WH

Nineteen cases o pleuropulmonary amebiasis was proved by needle aspiration or surgery as complication ofamebic liver abscess during the period from June, 1969 to August, 1971 at Kyungpook National University...
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A Case of Chilaiditi's Syndrome Presenting with Vomiting

Yoon YH, Rho YI, Moon KR

  • KMID: 2275161
  • Korean J Pediatr Gastroenterol Nutr.
  • 2002 Mar;5(1):88-90.
Chilaiditi's sign is a radiographic term used when the hepatic flexure of colon is seen interposed between the liver and right hemidiaphragm. Mostly asymptomatic, Chilaiditi's syndrome can present with abdominal...
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Toxic Hepatitis following Halothane Anesthesia

Im WM, Lee CB

  • KMID: 2357528
  • Korean J Anesthesiol.
  • 1979 Dec;12(4):466-468.
A 17 year old girl underwent a surgery of spine fusion for a tuberculous cervical spine (C(4~5) ) under halothane-02 anesthesia. On the 1st postoperative day, sudden high fever(39.7 degrees...
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Cranial Metastasis of Hepatocellular Carcinoma: Report of Three Cases

Lee HS, Kang SS, Kim SH, Lee JH, Woo JH

  • KMID: 2187776
  • J Korean Neurosurg Soc.
  • 1991 Sep;20(9):785-792.
The authors describe three cases of hepatocellular carcinoma presenting with the initial manifestation of an intracranial lesion without primary hepatic symptoms and signs. The only clue of hepatic dysfuntion was...
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Glycogenic hepatopathy in a Korean girl with poorly controlled type 1 diabetes mellitus

Jeong HR, Shim YS, Kim YB, Lee HS, Hwang JS

Glycogenic hepatopathy (GH) is a rare complication of type 1 diabetes mellitus. We report the case of a 13-year-old diabetic female with poorly controlled blood sugar levels who presented with...
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A Case of Congenital Rubella Syndrome

Yoon KC, Moon KH, Joo CU, Cho SC, Kim JS

  • KMID: 1838625
  • J Korean Soc Neonatol.
  • 1998 Jun;5(1):104-108.
We experienced a case of congenital rubella syndrome manifesting patent ductus arteriosus, petechiae, microcephaly, intrauterine growth retardation, and hepatomegaly in one-day-old female newborn infant. Congenital rubella syndrome was confirmed by...
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Stasis Dermatitis: A Case Report

Cho CK, Woo JS, Woo TH

  • KMID: 1683354
  • Korean J Dermatol.
  • 1969 Aug;7(1):71-72.
A case of stasis dermatitis with hepatomegaly is reported. The patient is 50 year old male. About 3 years ago a dark-brownish macule on the left shin was appeared with...
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A case of multiple hepatic adenomas and gout with glycogen storage disease type Ia

Lee DG, Park SH, Choi JM, Seo JH, Lee JS, Yang MJ, Kim GT

  • KMID: 2081644
  • Korean J Med.
  • 2009 Apr;76(Suppl 1):S1-S5.
Glycogen storage disease (GSD) type Ia is a rare inherited metabolic disease characterized by glucose-6-phosphatase (G6Pase) deficiency, which results in many metabolic problems, such as fasting hypoglycemia, lactic acidosis, hyperuricemia,...
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