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Case Report
A Case of Congenital Agammaglobulinemia
Sang Yong Kim, Jong Hyun Kim, Jin Han Kang, Joon Sung Lee, Sang In Shim
Clin Exp Pediatr. 1995;38(1):99-103.   Published online January 15, 1995
Congenital agammaglobulinemia was described first among all human immunodeficiencies, and characterized by defect of B lymphocyte maturation and severe panhypogammaglobulinemia. These patients frequently acquire infections with high-grade extracellular pyogenic pyogenic organisms since infancy. We experienced a case of congenital agammaglobulinemia in a 10 years old male. He had history of recurrent pneumonia and purulent otitis media since late infancy. Also he...
Two Male Siblings with Pseudohypoaldosteronism Type I
Ran Lee, Sang Yong Kim, Sung Dong Choi, Seung Yun Chung, Jin Han Kang, Byung Churl Lee
Clin Exp Pediatr. 1994;37(2):262-268.   Published online February 15, 1994
Pseudohypoaldosteronism (PHA) is rare herediary salt wasting syndrome due to peripheral resistance to aldostrone. PHA type I, subdivided into isolated renal insensitivity to aldosterone of autosomal dominant inheritance and multiple target organ defect of autosomal recessive inheritance, and PHA type II show similar clinical manifestations except hypertension which is limited to type II. PHA type I is charaterized by hyponatremia, hyperkalemia,...
A Case of Wiskott-Aldrich Syndrome
Dong Un Kim, Seung Hoon Han, Jin Han Kang, Joon Sung Lee
Clin Exp Pediatr. 1993;36(3):439-446.   Published online March 15, 1993
Wiskott-Aldrich syndrome s an X-linked combined immunodeficency disorder characterzed by severely decreased number of platelets which are small in size, eczema resembling atopic dermatitis and recurrent infection. The serum of the patient contains elevated concentrations of IgA and IgE, whereas the IgG level is usually normal and IgM level is deecreased. The patient also shows skin test anergy and progressive...
Original Article
A case of hyperimmunoglobulin E syndrome.
Myeong Cheol Shinn, Sung Dong Choi, Byung Gyu Suh, Jin Han Kang, Joon Sung Lee
Clin Exp Pediatr. 1991;34(2):292-298.   Published online February 28, 1991
The hyperimmunoglobulin E syndrome is a primary immunodeficiency disorder characterized by serious recurrent bacterial infections of the skin and respiratory system in early life, and markedly elevated serum IgE level. These recurrent infections are generally subcutaneous abscesses caused by Staphylococcus aureus. Also, additional characteristrics of this syndrome include coarse facial appearance, chronic pruritic dermatitis and eosinophilia. The baisic immunologic defect mechanism in hyperimmunoglobulin E syndrome is...
A Case of Cold Urticaria.
Chul Ho Lee, Sung Ik Chang, Jin Han Kang, Joon Sung Lee
Clin Exp Pediatr. 1990;33(7):1012-1017.   Published online July 31, 1990
Cold urticaria is one of the most common forms of physical urticaria characterized by pruritus, hive formation, redness, swelling, angioedema and occasionally symptoms of hypotension after cold exposure. Usually the diagnosis can be ascertained with the use of an ice cube test or an ice water immersion test, in which a wheal reaction appears oh the exposed skin in 10 〜 20 minutes....
A Case of Systemic-Onset Juvenile Rheumatoid Arthritis with Multiple Complications.
Jong Deok Kim, Dong Joo Na, Jin Han Kang, Kyong Su Lee, Ki Yeal Sung
Clin Exp Pediatr. 1988;31(7):948-952.   Published online July 31, 1988
We recently experienced a case of systemic-onset juvenile rheumatoid arthritis with protracted and variable clinical course with multiple complications and sequeles including pericarditis, disseminated intravascular coagulation, severe ankylosis and bony fusion of neural arches of C2 through C6 vertebrae, and multiple chronic arthritis causing joint deformities at relatively young age of nine. A brief review of related literatures is also presented.
A Case of Acute Hepatic Porphyria.
Young Cheal Han, Sin Hang Joo, Jin Han Kang, Byung Churl Lee
Clin Exp Pediatr. 1988;31(2):253-257.   Published online February 28, 1988
Acute hepatic porphyria may be an inborn error of metabolism characterized biochemically by the excessive hapatic over-production and urinary excretion of porphyrin precursors and clinically by episode of acute neuro-visceral dysfunction. Acute porphyria is very rare in childhood. We experienced a case of acute hepatic porphyria in childhood age, confirmed by characterized clinical features of neuro-visceral dysfunction and biochemical findings in blood and urine...
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