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Review Article
Nephrology (Genitourinary)
Atypical hemolytic uremic syndrome and eculizumab therapy in children
Seong Heon Kim, Hye Young Kim, Su Young Kim
Clin Exp Pediatr. 2018;61(2):37-42.   Published online February 28, 2018

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is...

Case Report
Nephrology (Genitourinary)
Recombinant Human Erythropoietin Therapy for a Jehovah's Witness Child With Severe Anemia due to Hemolytic-Uremic Syndrome
Da Eun Woo, Jae Min Lee, Yu Kyung Kim, Yong Hoon Park
Clin Exp Pediatr. 2016;59(2):100-103.   Published online February 29, 2016

Patients with hemolytic-uremic syndrome (HUS) can rapidly develop profound anemia as the disease progresses, as a consequence of red blood cell (RBC) hemolysis and inadequate erythropoietin synthesis. Therefore, RBC transfusion should be considered in HUS patients with severe anemia to avoid cardiac or pulmonary complications. Most patients who are Jehovah's Witnesses refuse blood transfusion, even in the face of life-threatening...

Shiga toxin-associated hemolytic uremic syndrome complicated by intestinal perforation in a child with typical hemolytic uremic syndrome
Hye Jin Chang, Hwa Young Kim, Jae Hong Choi, Hyun Jin Choi, Jae Sung Ko, Il Soo Ha, Hae Il Cheong, Yong Choi, Hee Gyung Kang
Clin Exp Pediatr. 2014;57(2):96-99.   Published online February 24, 2014

Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have...

A case of hemolytic uremic syndrome preceded by intussusception
Eun Young Ko, Joo Young Kim, Hye Jin Lee, Hyun Seung Lee, Ji Whan Han, Young Hoon Kim, Jin Tack Kim, Hae Il Cheong, Pil Sang Jang
Clin Exp Pediatr. 2011;54(4):176-178.   Published online April 30, 2011

Hemolytic-uremic syndrome (HUS) is the most common cause of acute renal failure in young children. It is classically characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and uremia. Further, not only is intussusception one of the differential diagnoses of HUS but it may also become a complication during disease progression. We report a case of HUS preceded by intussusception...

A case of atypical hemolytic uremic syndrome as an early manifestation of acute lymphoblastic leukemia
Dong Kyun Han, Hee Jo Baek, Young Kuk Cho, Chan Jong Kim, Myung Geun Shin, Hoon Kook, Tai Ju Hwang
Clin Exp Pediatr. 2010;53(2):253-257.   Published online February 15, 2010
Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children younger than 4 years and is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. HUS associated with diarrheal prodrome is usually caused by Shiga toxin-producing Escherichia coli O157:H7 or by Shigella dysenteriae, which generally has a better outcome. However, atypical cases show a...
Original Article
Multiple extrarenal manifestations in hemolytic uremic syndrome: A case report
Eugene Kim, So-Young Kim
Clin Exp Pediatr. 2007;50(12):1261-1265.   Published online December 15, 2007
Extrarenal manifestations of hemolytic uremic syndrome (HUS) have increasingly been recognized and may be major determinants of mortality and morbidity. Although microthrombi are often found in the pulmonary and coronary circulation, apparent lung and cardiac involvement are clinically infrequent. We describe here a 10-month-old boy with HUS who developed pulmonary hemorrhage, acute respiratory distress syndrome and dilated cardiomyopathy. Complete renal...
Review Article
Hemolytic uremic syndrome
Hye Won Park
Clin Exp Pediatr. 2007;50(10):931-937.   Published online October 15, 2007
The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however,...
Hemolytic anemia in pediatrics
Jeong Ok Hah
Clin Exp Pediatr. 2007;50(6):511-518.   Published online June 15, 2007
To understand the hemolytic anemia (HA) in children, the diagnostic approach and management of hereditary and acquired HA are described. The hereditary hemolytic anemia (HHA) can be classified according to the pathogenesis into three types : RBC membrane defects, hemoglobinopathies, and RBC enzymopathies. Clinical characteristics, laboratory findings and molecular defects of these three types are presented briefly. In Korea,...
Case Report
A case of dapsone syndrome
Yoo Jong Won, Ok Lan Kim, Seung Taek Yu, Young Wook Yoon, Du Young Choi
Clin Exp Pediatr. 2007;50(5):493-496.   Published online May 15, 2007
Diamino-diphenyl-sulfone (Dapsone) is widely used in the treatment of leprosy and a variety of blistering skin diseases. It sometimes has adverse side effects with common usual doses, such as skin, nervous system, gastrointestinal tract, liver, kidney and hematologic toxicity. One of these side effects is a rare but serious hypersensitivity reaction called dapsone syndrome, which occurs several weeks after the...
A Case of Rothmund-Thomson Syndrome with Pure Red Cell Aplasia, Autoimmune Hemolytic Anemia and Chronic Respiratory Infection
Jung Hyun Lee, Eun Seok Roh, Yoo Rah Hong, Jae Sun Park, Ghi Seok Seo, Bang Hur, Mi Hyang Kim
Clin Exp Pediatr. 2004;47(12):1351-1355.   Published online December 15, 2004
Rothmund-Thomson syndrome(RTS), or poikiloderma congenita, is a rare, multisystem disorder. It is inherited genetically as an autosomal recessive trait, occurring predominantly in females(1.4 : 1). The RTS is comprised of poikiloderma, short stature, sparse hair, juvenile cataracts, skeletal defects, dystrophic teeth and nails, photosensitivity, and hypogonadism. We report a case of RTS who died of bleeding from esophageal varices, pulmonary...
Atypical Hemolytic Uremic Syndrome Associated with Streptococcus pneumoniae Infection
Hyun Sug Lee, Ja Wook Koo, Sang Woo Kim, Hyun Soon Lee
Clin Exp Pediatr. 2004;47(2):217-222.   Published online February 15, 2004
Hemolytic uremic syndrome is the most common cause of acute renal failure in childhood. Atypical hemolytic uremic syndrome which is not usually associated with prodromal symptoms has a higher mortality rate and more tendency to progress to chronic renal failure. Children younger than two years of age are a more susceptible to pneumococcal infection and can develop more serious disease....
Original Article
A Clinical Study on Childhood Hemolytic Anemia According to Etiological Classification
Hae-Sik Kwon, Jung-Chul Kang, Sung-Chul Won, Seung-Hwan Oh, Chuhl-Joo Lyu
Clin Exp Pediatr. 2003;46(9):883-888.   Published online September 15, 2003
Purpose : The etiology of hemolytic anemia can be classified as either cellular or extracellular defects of red blood cells. The aim of this study was to investigate the clinical and laboratory findings of hemolytic anemia concerning its etiological classification. Methods : Clinical and laboratory findings of the patients with hemolytic anemia treated from January 1987 to May 2002 at Severance...
Case Report
A Case of Hemolytic Disease in a Newborn Due to Anti-Jkb
Dong-Kyun Park, Young-Min Kim, Chong-Woo Bae, Yong-Mook Choi, Woo-In Lee
Clin Exp Pediatr. 2003;46(7):718-721.   Published online July 15, 2003
The isoimmune hemolytic disease of newborn due to the incompatibility of minor blood groups is characterized by progressive neonatal hyperbilirubinemia and anemia caused by the IgG antibody transmitted from the mother to the fetus. Recently we had a case of hemolytic disease in a newborn due to anti-Jkb. There were no ABO and Rh(D) incompatibilities between mother and baby. The...
Original Article
Comparison of Two Rapid Antigen Detection Tests for Diagnosis of Group A Streptococcal Pharyngotonsillitis
Seung Kyu Song, Mi Ae Hong, Kyung Chang Oh, Seung In Ahn, Mi Hyon Tae, Hye Jung Shin, Jin Keun Chang, Sung Ho Cha
Clin Exp Pediatr. 2002;45(8):973-979.   Published online August 15, 2002
Purpose : Recently, a number of rapid antigen detection tests have been available to diagnose group A streptococcal pharyngotonsillitis. The purpose of this study was to determine the sensitivity, specificity and consistency of the two rapid antigen detection tests. Methods : Among the patients who visited our clinic from November 2001 to February 2002, 61 patients who had clinical findings of...
Clinical Features of Hemolytic Uremic Syndrome in Children
Ju Eun Lee, Su Yung Kim
Clin Exp Pediatr. 2000;43(11):1473-1479.   Published online November 15, 2000
Purpose : Hemolytic uremic syndrome(HUS) is the most common cause of renal failure in childhood in western countries, but it has been a relatively rare disease in Korea. To analyze the clinical and epidemiologic characteristics of HUS in Korea, we reporte our experience. Methods : We reviewed retrospectively the medical records of 9 patients who were diagnosed the hemolytic uremic syndrome...
Case Report
A Case of Common Variable Immunodeficiency with Autoimmune Hemolytic Anemia
Kyung-Yil Lee, Yon-Joo Lee, Sang-Won Cha, Dong Joon Lee, Ji-Whan Han, Kyung-Tae Whang, Joong-Gon Kim
Clin Exp Pediatr. 2000;43(1):117-122.   Published online January 15, 2000
Common variable immunodeficiency(CVID) is a heterogeneous collection of disorders with hypogammaglobulinemia with recurrent bacterial infections and high incidence of autoimmune disorders as its hallmark. We report a 7-year-old girl suffering from CVID with Coombs' test positive hemolytic anemia. She had been relatively well until 23-months old when she was admitted to Taejon St. Mary' s Hospital with pneumonia 5 years ago. Afterwards, she had...
Original Article
Nationwide Survey on Group A beta-Hemolytic Streptococcal Carrier Rate in Normal Korea School Children in 1996
Sung-Ho Cha, Yong-Mook Choi, Sei-Won Yang, Dong-Hwan Lee, Chong-Guk Lee, Chang-Ho Hong
Clin Exp Pediatr. 1998;41(5):585-592.   Published online May 15, 1998
Purpose : The accuracy of bacteriologic diagnosis of beta-hemolytic streptococcal pharyngotonsillitis depends on the degree of carrier rate in that area. It is the purpose of this study to obtain the carrier rate from seven different areas and to find results and epidemiologic factors. Methods : Throat swab was obtained from the tonsillar fossa of normal school children during March through...
Relationship between Antistreptolysin O Titer and Number of Colonies from Throat Swab Culture Plate in Group A Beta-hemolytic Streptococcal Carriers and Normal School Children
Sungho Cha
Clin Exp Pediatr. 1997;40(4):480-488.   Published online April 15, 1997
Purpose : To know the true carrier rate and the immunologic responses(antistreptolysin O :ASO) to beta-hemolytic streptococci which obtained from normal school children with or without carrying streptococci. And to study the clinical meaning of the number of organisms found in culture. Methods : One hundred ninty-eight first grade of elementary school children were included. I obtained throat swab culture from...
Comparison of The Efficacy of the Fiberoptic Phototherapy and Conventional Phototherapy for Neonatal Hyperbilirubinemia
Hye Jung Min, Gwi Sook Kim, Soo Kyung Lee, Il Kyung Kim, Ho Sung, Chang Hee Choi
Clin Exp Pediatr. 1996;39(8):1076-1083.   Published online August 15, 1996
Purpose : We performed this study in order to compare the feasibility and efficacy of the fiberoptic phototherapy with conventional phototherapy and double phototherapy with single phototherapy for the treatment of neonatal hyperbilirubinemia while watching for any possible side effects of the system. Methods : During the period of February 1994 till June 1995, randomized controlled study was performed. 130 healthy term infants with non-hemolytic...
Bacteriological Survey of beta-Hemolytic Streptococci from the Throats of Elementary School Children in Chinju -Compared with the Results of Children in Kangwon, Chungnam and Seoul-
Seon-Ju Kim, Kook-Young Maeng, Hyang-Im Lee, Yun-Kyong Cho, Hee-Sang Yun
Clin Exp Pediatr. 1996;39(2):238-245.   Published online February 15, 1996
Purpose : Throat culture for streptococcal pharyngitis is a gold standard to diagnose, but it may be misleading to interpret due to the carriers. The isolation rates of beta-hemolytic streptococci (BHS) and Streptococcus pyogenes in the school children in Chinju were investigated and compared with those of Kangwon, Chungnam and Seoul previously reported. Methods : Throat cultures were taken from the...
Case Report
A Case of Poststreptococcal Reactive Polyarthralgia
Sung Ho Cha, Byong Soo Cho, Taekyu Hame
Clin Exp Pediatr. 1993;36(11):1635-1637.   Published online November 15, 1993
Poststreptococcal reactive arthritis/arthralgia is characterized as an evidence of group A beta hemolytic streptococcal infection and does not fulfill the modified Jones criteria for a diagnosis of acute rheumatic fever. We had used to meet the patients with incomplete acute rheumatic fever who had more than 3 items of minor Jones criteria, or arthralgia or arthritis with one or two...
Original Article
Clincal Study on 12 Cases of Neonatal Group B β-Hemolytic Streptococcal Meningitis
Won Jin Kim, Sang Woog Lee, Sang Lak Lee, Myung Sung Kim, Chin Moo Kang
Clin Exp Pediatr. 1993;36(11):1507-1515.   Published online November 15, 1993
A clinical study was made on 12 cases of neonatal Group Bβ-hemolytic streptococcal(=GBS)meningitis, who were admitted to the department of Pediatrics, Dong-San Hospital, Keimyung University during the period of 3 years from Aug 1989 to Jul 1992. The following results were obtained 1) GBS was cultured in 12 cases(57.1%) among 21 neonatal meningitis admitted during the same period. 2) Male and female ratio...
Case Report
A Case of Wilson Disease Associated with Hemolytic Anemia and Cholelithiasis
Kyeong Cheol Yoon, Yong Hwa Shin, Ho Seek Ahn, Sung Won Kim
Clin Exp Pediatr. 1992;35(11):1573-1577.   Published online November 15, 1992
Wilson disease is an autosomal recessive abnormality in the hepatic excretion of copper that results in toxic accumulation of the metal in liver, brain, and other organs. Hemolytic anemia frequently complicates the courses of Wilson disease and may be the initial clinical feature of Wilson disease. Since hemolysis may be preceding the onset of hepatic manifestation and Kayser-Fleischer ring is...
Original Article
A Clinical Aspect of the Hemolytic Uremic Syndrome
Hye Won Park, Tae Sun Ha, Il Soo Ha, Hae Il Cheong, Yong Choi, Kwang Wook Ko
Clin Exp Pediatr. 1992;35(7):909-920.   Published online July 15, 1992
We reviewed the medical records of 14 children (8 girls, 6 boys), diagnosed as hemolytic uremic syndrome at Seoul National University Children뭩 Hospital from 1981 to 1990. The age at presentation ranged from 1 month to 10 years, with a mean age of 2.7 years. Only eight (57.1%) of the children had diarrheal prodrome and five (35.7%) had grossly bloody...
A case of pancytopenia associated with mycoplasmal pneumonia.
Young Mee Yoo, Beom Soo Park, In Sang Jeon, Hee Young Shin, Hyo Seop Ahn, Se Jung Sohn
Clin Exp Pediatr. 1991;34(6):826-831.   Published online June 30, 1991
Mycoplasma pneumoniae infection is manifestated as pneumonia and extra-pulmonary symptoms such as hematologic, neurologic, gastrointestinal, musculoskeletal, dermatologic and cardiac manifes- tations. Among the hematologic changes, hemolytic anemia is most frequently seen. Throm- bocytopenic purpura, lymphocytosis or rarely lymphopenia, elevated erythrocyte sedimentation rate, positive direct Coombs test, reticulocytosis and DIC are known hematologic features. This 12 years old female patient was presented with pneumonia and pancytopenia....
A Case of Autoimmune Hemolytic Anemia Associated with Chronic Hepatitis in Children.
Kwan Mo Choi, Kyeong Hun Cha, Eun Yeong Kwak, Kyung Rae Moon, Yeong Bong Park
Clin Exp Pediatr. 1990;33(9):1288-1293.   Published online September 30, 1990
Recently, We experienced a case of autoimmune hemolytic anemia in a 13 year old female patient associated with chronic hepatitis. This female child manifested severe pallor, jaundice, abdominal pain and fever. This diagnosis was made on the bases of typical clinical features, C.B.C., blood chemistry, coombs test, warm antibody, immunoelectrophoresis, peripheral blood smear and bone marrow examination. The patient improved with steroid therapy. We report this...
Relationship between anaphylactoid purpura and Beta-Hemolytic Streptococcal Infection.
Ji Eun Lee, Young Sook Kang, Joon Sik Kim, Sung Ho Kim, Chin Moo Kang
Clin Exp Pediatr. 1990;33(9):1231-1236.   Published online September 30, 1990
Relationship between anaphylactoid purpura and beta-hemolytic streptococcal infection was obser- ved and analyzed through clinical manifestation and labortory findings in 146 cases of allergic purpura, hospitalized at Dongsan hospital, Keimyung University during the period from January, 1981 to May, 1987. The following results were obtained. 1) The age distribution of anaphylactoid purpura showed a peak incidence between 2 to 7 years of age and male preponderance was...
A Case of Hemolytic Uremic Syndrome.
Seung Cheol Lee, Chong Guk Lee, Seong Hwan Kim, Sang Woo Kim, Hyun Soon Lee
Clin Exp Pediatr. 1990;33(8):1140-1147.   Published online August 31, 1990
A female child aged 7 months was admitted because of high fever, vomiting and stuporous conciousness and revealed to have hemolytic uremic syndrome. Anuria persisted 13 days and required peritoneal dialysis 4 times a day during the anuric period. Her kidney function recovered somewhat after dialysis but neurologic complications of marked hydrocephalus and brain atrophy remained.
The Effect of Oral Vitamin E on Hemolytic Anemia of the Premature Infants.
Hwa Kyoung Oh, Kwang Sik Yoo, Yong Sil Chi, Myung Jin Kim, Mi Na Lee
Clin Exp Pediatr. 1989;32(11):1469-1473.   Published online November 30, 1989
The study was designed to evaluate the effect of vitamin E on hemolytic anemia of premature infants. Sixty infants delivered in Cheil Hospital from March 1987 to December 1988 with birth weight below 2500 gm or gestational age below 37 weeks were studied. Thirty infants received oral vitamin E 25 IU/day until their birth weight doubled. Thirty infants served as control. The results were...
A Case of Thiopental-Induced Hemolytic Anemia After Open Heart Surgery.
J H Lee, K H Cho, K C Lee, J W Lee, S K Kim
Clin Exp Pediatr. 1989;32(5):730-734.   Published online May 31, 1989
We experienced a case of thiopental-induced hemolytic anemia with impairment of renal function after open heart surgery. The diagnosis was suggested by clinical features, CBC, urinalysis, transfusion reaction study and chemistry, and finally established by immunohematologic study. We detected antibody against thiopental in the patient’s serum (end-titer:1:16). The review of literature was made briefly.
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