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Case Report
A Case of Congenital Megacystis Due to Non-Obstructive Urinary Retention Associated with Intestinal Malrotation and Hypoperistalsis
Tai Young Ham, Jeong Hoon Kim, Ji Hong Kim, Jae Seung Lee, Seok Ju Han, Choon Sik Yoon, Soon Won Hong
Clin Exp Pediatr. 2004;47(1):95-99.   Published online January 15, 2004
Congenital megacystis with bilateral hydroureteronephrosis is most commonly associated with posterior urethral valves, prune-belly syndrome, vesicoureteral reflux, or nonrefluxing, nonobstructive megaureters. Among the other cause of congenital megacystis, megacystis-microcolon-intestinal hypoperistalsis syndrome(MMIHS), which is characterized by megacystis, microcolon and hypoperistalsis of the intestines with resultant abdominal distension, is a rare cause of functional obstruction of urinary tracts in childhood. It was...
A Therapeutic Experience of Congenital Bilateral Neuroblastoma
Yeon Kyong Seo, Heung Sik Kim, Kun Young Kwon, Hee Jung Lee, Hong Hoe Koo
Clin Exp Pediatr. 2003;46(12):1279-1282.   Published online December 15, 2003
Neuroblastoma is the most common intraabdominal malignant tumor of childhood, with 40% arising from the adrenal gland. Bilateral adrenal involvement from synchronous development or metastatic spread of tumor is rarely seen in children with neuroblastoma. The patient was born with a spontaneous vaginal delivery. Birth weight was 3,200 g. Fetal ultrasonography showed a left adrenal cystic mass. At two weeks...
A Case of Micturition Syncope in a Child
Sun Youn Lee, Su Jeong Ryu, Deok Soo Kim, Young Hwue Kim, Tae Sung Ko, Jae Moon Kim
Clin Exp Pediatr. 2003;46(12):1274-1278.   Published online December 15, 2003
Syncope in children and adolescents have a common occurrence according for up to 15% before adulthood. Micturition syncope, a kind of situational syncope, can be considered a form of reflex syncope. It can typically occur in healthy young men after rising from bed in the early morning who experience sudden loss of consciousness during or immediately after urination. The mechanism...
A Case of Labial Adhesion
Ha Young Roh, Chan Kyun Oh, Kyung Ran Son, Jin Hwa Kook, Young Youn Choi
Clin Exp Pediatr. 2003;46(12):1271-12736.   Published online December 15, 2003
Labial adhesions are postnatal fusion of the labia minora in the midline of varying degrees. They are postulated to be the result of low estrogen levels in the prepubertal child and possibly of a chronic inflammatory process. Topical treatment with conjugated estrogens has been the mainstay of therapy. We experienced one case of labial adhesion in an infant who was...
A Case of Nasal T/NK-cell Lymphoma
Jung Bok Lee, In-sang Jeon, Ho Joon Im, Young Ha Oh, Ji Hye Kim
Clin Exp Pediatr. 2003;46(12):1266-1270.   Published online December 15, 2003
The advance of the immunobiology clarifies the nature of non-Hodgkin's lymphoma(NHL). In addition the proceed in the immunophenotyping renders the classification of NHL. According to the Revised European American Lymphoma(REAL) classification, classified by the etiologic factors, molecular biological characteristics, immunophenotype, cytogenetics and histologic feature, nasal T/NK-cell lymphoma(=angiocentric lymphoma) belongs to the category of peripheral T-cell and natural killer cell lymphoma....
A Case of Rickets Developed after Feeding on Sunsik for Seven Months
Jeong-A Yang, Kyung-A Jang, Hye-Won Park, Wook Jang, Man-Yong Han, Young-A Cho, Eun Lee
Clin Exp Pediatr. 2003;46(11):1143-1146.   Published online November 15, 2003
Rickets is a nutritional disorder which is caused either by deficiency of vitamin D or by a defective activation of vitamin D. In these days, even though the incidence of rickets has decreased through adequate nutritional support, we sometimes experience rickets in babies receiving a prolonged special diet as therapy for chronic diarrhea, or those subject to a in receiving...
Aplastic Crisis Secondary to Parvovirus B19 Infection
Yang Joon Park, Dae Kyun Koh, Jin Hee Oh
Clin Exp Pediatr. 2003;46(11):1139-1142.   Published online November 15, 2003
Human parvovirus(HPV) B19 infection causes erythema infectiosum in children, sometimes red cell aplastic crisis with hemolytic anemia and chronic bone marrow failure in immunocompromised hosts. HPV B19 is directly cytotoxic for erythroid progenitor cells and inhibits erythropoiesis. Infrequently, HPV B19 inhibits hematopoiesis of three cell lineages and causes transient pancytopenia in patients with hemolytic disorders. We report three patients with...
A Case of Keratitis, Ichthyosis and Deafness(KID) Syndrome
Lan Kim, Dong Hwan Lee
Clin Exp Pediatr. 2003;46(11):1135-1138.   Published online November 15, 2003
KID syndrome was named after the initials of the major three symptoms of the disease; keratitis, ichthyosis, and deafness. The syndrome was first introduced by Dr. Burns in 1915 who described one of his patients with those symptoms. In 1981, Dr. Skinner and his colleagues reported 17 patients who had keratitis, ichthyosis, and deafness. They also called the disease KID...
A Case of Infantile Spasm Associated with Acute Renal Failure and Kwashiorkor after Ketogenic Diet
Young-Myoung Kim, Tae-Hong Kim, Jin-A Jung, Kyu-Geun Hwang
Clin Exp Pediatr. 2003;46(11):1131-1134.   Published online November 15, 2003
The ketogenic diet is a high-fat, low-protein, low-carbohydrate diet developed in the 1920s for the treatment of difficult-to-control seizures. Despite advances in both the pharmacotherapy and the surgery of epilepsy, many children continue to have difficult-to-control seizures. In this situation, a ketogenic diet should be considered as an alternative therapy. However, less attention has been paid to associated adverse events...
A Case of Acute Pancreatitis in a Neuroblastoma Patient after Retinoic Acid Therapy
Yoo Jin Jeong, Yeon Kyong Seo, Heung Sik Kim, Hee Jung Lee
Clin Exp Pediatr. 2003;46(11):1128-1130.   Published online November 15, 2003
Retinoic acid has been used successfully as a differentiating agent in acute promyelocytic leukemia and neuroblastoma. However, some adverse effects have been recognized, such as headaches, dry skin and retinoic acid syndrome, a life threatening acute cardiorespiratory disorder. Acute pancreatitis with hyperlipidemia has rarely been reported. We experienced a case of acute pancreatitis with hyperlipidemia in a neuroblastoma patient after...
Arthritis in the Subacute Stage of Kawasaki Disease after Responding to Intravenous Immunoglobulin Treatment
Kyung-Yil Lee, Jin-Hee Oh, Dea-Kyun Koh
Clin Exp Pediatr. 2003;46(11):1124-1127.   Published online November 15, 2003
We evaluated the clinical and laboratory characteristics of five children with Kawasaki disease who had showed arthritis after responding to intravenous immunoglobulin(IVIG) treatment. Age distribution was between 13 months and six years of age(mean 3.2?.6 years). There were two males and three females. Arthritis occurred when acute symptoms were subsiding, with the average onset on day 5.8?.8 after final IVIG...
A Case of Perinatal Varicella Infection
Jeong A Rho, Young Il Rho, Eun Young Kim, Sang Kee Park
Clin Exp Pediatr. 2003;46(10):1047-1050.   Published online October 15, 2003
Maternal varicella resulting in viremia may transmit the virus to the fetus by either transplacental spread, or by ascending infection from lesion in the birth canal. The characteristic symptoms consist of skin lesions in dermatomal distribution, eye diseases, neurological defects, and limb hypoplasia. Varicella of the newborn is a life-threatening illness that may occur when a newborn is delivered either...
A Case of Craniofrontonasal Dysplasia Diagnosed at Birth
Jeong A Rho, Young Il Rho, Kyung Rye Moon, Young Bong Park, Sang Kee Park, Eun Young Kim
Clin Exp Pediatr. 2003;46(10):1044-1046.   Published online October 15, 2003
Craniofrontonasal dysplasia(CFND), a rare congenital syndrome, is characterized by varying degrees of frontonasal dysplasia, craniosynostosis, and variable extracranial abnormalities. It was first reported by Cohen in 1979. The inheritance pattern is not straightforward. Although all modes of Mendelian inheritance have been suggested, the most plausible explanation is that this is an X-linked condition with the unusual situation of complete expression...
A Case of Henoch-Schonlein Purpura Nephritis Complicating Encephalopathy Accompanied by Hypertension and Cerebral Vasculitis
Hee Ra Choi, Eo Jin Kim, Myoung Bum Choi, Jae Young Lim, Chan Hoo Park, Hyang Ok Woo, Hee Sang Youn
Clin Exp Pediatr. 2003;46(10):1040-1043.   Published online October 15, 2003
Henoch-Shonlein purpura(HSP) is a systemic small-vessel vasculitis that primarily affects the skin, gastrointestinal tract, joints, and kidneys. The nervous system may be involved, less commonly than other organs. When the central nervous system(CNS) was involved, headache, changes in mental status, seizures, and focal neurologic deficits have been reported. Hypertension, uremic encephalopathy, metabolic abnomalities, electrolyte abnormalities, or cerebral vasculitis were suggested...
A Case of Sandifer Syndrome
Mira Lee, Soo Hyun Kim, Yong Joo Kim, In Joon Seol
Clin Exp Pediatr. 2003;46(10):1036-1039.   Published online October 15, 2003
Sandifer syndrome is a rare manifestation of gastroesophageal reflux in children, occurring in association with abnormal movement of the head and neck and recurrent episodes of seizure after feeding. This syndrome may be misdiagnosed as infantile seizure and musculoskeletal disorder and may be associated with failure to thrive, anemia, aspiration pneumonia, and esophagitis. In this paper, Sandifer syndrome in a...
A Case of Pseudohypoparathyroidism in a Premature Infant
Jong Il Yang, Jang Won Seo, Ji Young Kim
Clin Exp Pediatr. 2003;46(10):1032-1035.   Published online October 15, 2003
In pseudohypoparathyroidism as reported by Albright in 1942, the parathyroid gland can normally synthesize and secrete parathyroid hormone(PTH). Pseudohypoparathyroidism has a similar biochemical finding with hypoparathyroidisms like hypocalcemia and hyperphosphatemia due to target tissue resistance to PTH. Administered PTH does not raise the serum levels of calcium and urinary phosphate. PTH activates G-protein in peripheral tissue and adenylate cyclase through...
A Case of Hemophagocytic Lymphohistiocytosis in a Child with Systemic Lupus Erythematosus
Ja Young Hwang, Suk Man No, Jin Lee, Pil Sang Jang, Young Hoon Kim, Jin Tack Kim, Joon Sung Lee
Clin Exp Pediatr. 2003;46(10):1029-1031.   Published online October 15, 2003
Hemophagocytic lymphohistiocytosis is a reactive disorder characterized by a generalized non-malignant histiocytic proliferation with prominent hemophagocytosis by stimulated histiocytes in the bone marrow and reticuloendothelial systems resulting in pancytopenia and liver dysfunction. Several diseases including infection, malignancy and autoimmune disease are known to be causative disorders. This case demonstrated histiocytic hemophagocytosis in the bone marrow, resulting in pancytopenia during treatment...
A Case of Influenza-associated Encephalopathy
Yeoni Song, Chang Hwan Choi, Jong Woon Choi, Se Young Kim, Hyun Soo Kim, Yeol Kim, Dong Jin Im
Clin Exp Pediatr. 2003;46(10):1024-1028.   Published online October 15, 2003
Influenza-associated encephalopathy is regarded as one of the major neurologic disease entities along with those of Reye syndrome, acute necrotizing encephalopathy, and myelitis which are known to be related to influenza virus, mostly type A. And it is being actively researched in Japan as it has caused a tremendous increase in the number of deaths from 1997 to 2002, but...
A Case of Recurrent Herpes Simplex Virus Disease of a Preterm Infant, Who Needed Continuous Oral Acyclovir Suppressive Therapy
Sung Seok Kim, Ki Woong Hong, Eun Ryoung Kim, Young-Don Kim, Kyoo Man Lee
Clin Exp Pediatr. 2003;46(9):939-949.   Published online September 15, 2003
Neonatal herpes simplex virus(HSV) infections result in significant morbidity and mortality. Although acyclovir treatment has improved survival, severe neurological sequelae can occur in the majority of survivors. HSV infections limited to the skin, eyes and mouth(SEM) can cause neurologic impairment. A direct correlation exists between the development of neurologic deficits and the frequency of cutaneous HSV recurrences. National Institutes of...
A Case of Canavan Disease
Young Ho Son, Tae Gyu Hwang, Jong Beom Sinn
Clin Exp Pediatr. 2003;46(9):934-938.   Published online September 15, 2003
Canavan disease, also known as van Bogaert-Bertrand disease, is a rare autosomal recessive disorder characterized by early an onset and a progressive spongyform degeneration of the brain, associated with an edema of the central nerve system, intramyelinic swelling and neurologic symptoms. This disorder is most prevalent in people of Ashkenazi Jewish descent but has been observed in other ethnic groups....
A Case of Carbohydrate Pneumonitis after Ingestion of Thinner
Eul Soon Kim, Joon Soo Park
Clin Exp Pediatr. 2003;46(9):930-933.   Published online September 15, 2003
Hydrocarbon pneumonitis is an inflammatory change in the lungs caused by ingestion or inhalation of household products that contain hydrocarbons. We experienced a rare case of hydrocarbon pneumonitis with a pulmonary hemorrhage in a 3-year-old girl after ingestion of paint thinner which has a high mortality. She was admitted due to dyspnea, vomiting, hemoptysis, and needed to support mechanical ventilation....
A Case of Intestinal Lymphangiectasia
Hyung Eun Yim, Min Ji Jung, Kee Hwan Yoo, Young Sook Hong, Joo Won Lee, Soon Kyum Kim
Clin Exp Pediatr. 2003;46(9):921-925.   Published online September 15, 2003
Intestinal lymphangiectasia, one of the protein-losing gastroenteropathies, is an uncommon disease characterized by dilated intestinal lymphatics, enteric protein loss, edema, hypoalbuminemia, and lympocytopenia. Small bowel biopsy and CT have been used to confirm the diagnosis of intestinal lymphangiectasia. Small bowel biopsy shows collections of abnormal dilated lacteals in submucosa with distortion of villi and CT findings have been described as...
A Case of Herpetic Whitlow with Bacterial Superinfection
Ju-Young Chung, Sang-Woo Kim
Clin Exp Pediatr. 2003;46(9):918-920.   Published online September 15, 2003
Herpes simplex virus infection of the hand in children occurs after auto-inoculation from herpetic gingivostomatitis or herpes labialis. Herpetic whitlow should be suspected based on clinical signs. Diagnosis can be made by PCR or virus culture. Many misdiagnosed cases suggests that this disease is not sufficiently known. Surgical interventions may be harmful and should be avoided. We report a case...
A Case of Repeated Ventricular Tachycardia Caused by Cardiac Rhabdomyomas in an Infant with Tuberous Sclerosis
Hee Jung Joo, Min Seob Song, Tae Gyu Hwang, Chul Ho Kim
Clin Exp Pediatr. 2003;46(9):913-917.   Published online September 15, 2003
Cardiac rhabdomyomas are relatively uncommon and associated with tuberous sclerosis in 40-50% cases. We report a 10-month-old infant with tuberous sclerosis who presented with ventricular arrythmias and status epilepticus. There were hypopigmented macules on the body, periventricular calcifications, renal cyst and cardiac rabdomyomas just below the aortic valve. The patient required resection of left ventricular subaortic masses due to sustained...
A Case of Klippel-Trenaunay Syndrome Combinded with Sturge-Weber Syndrome
Mi Young Kim, So Young Lee, Na Yeon Kim, Sun Ju Lee, Won Duck Kim, Sung Min Cho, Dong Seok Lee, Doo Kwun Kim, Sung Min Choi
Clin Exp Pediatr. 2003;46(9):909-912.   Published online September 15, 2003
Klippel-Trenaunay syndrome is a rare mesodermal phakomatosis characterized by cutaneous haemangiomata(usually unilateral and involving an extremity), venous varicosities and osseous and soft tissue hypertrophy, of the affected limb. Sturge-Weber syndrome, also a mesodermal pharkomatosis, is characterized by a port-wine nevus, which is present from birth and covers the face and cranium in the territory of the first division of the...
A Case of 21-Monosomy with Holoprosencephaly(Semilobar Type)
So Young Lee, Sung Min Cho
Clin Exp Pediatr. 2003;46(8):831-835.   Published online August 15, 2003
Holoprosencephaly of unknown definite causes, has been associated with several chromosome abnormalities involving the autosomes and the sex chromosomes. The most commonly reported associations include dup(3p), del(7q), deletions of chromosome 13, trisomy 13, trisomy 18, and triploidy. In previously reported cases in Korea, none were associated with chromosome 21 anomalies. In conclusion, we reported the first case of holoprosencephaly(semilobar type)...
A Case of ARCI Syndrome with Hypoplasia of Corpus Callosum and Heart Anomaly
Eo-Jin Kim, Young-Ran Yoon, Min-Hae Lee, Ki-Su Kang, Jae-Young Lim, Myoung-Bum Choi, Chan-Hoo Park, Hyang-Ok Woo, Hee-Shang Youn
Clin Exp Pediatr. 2003;46(8):826-830.   Published online August 15, 2003
ARCI syndrome consists of arthrogryposis, renal tubular acidosis, cholestatic jaundice and icthyosis. We experienced an ARCI syndrome case with corpus callosum hypoplasia and atrial septal defect. This case had oral feeding difficulty, multiple joint contracture, renal tubular acidosis and neurogenic muscular atrophy at neonatal period. At two months of age, icthyosis and cholestatic jaundice were diagnosed. The case was hospitalized...
A Case of Congenital Malaria
Eun Jin Lee, Hyen Ho Chang, Chong Guk Lee, Chong Rae Cho
Clin Exp Pediatr. 2003;46(8):821-825.   Published online August 15, 2003
Malaria is known to have been eradicated for a few decades through the persistent efforts of the national health program in South Korea. However, malaria caused by Plasmodium vivax has started to reappear incidiously among military personnel near to the De-militarized Zone since 1993. From that time on the number of malarial cases have increased abruptly year by year. However,...
A Case of Ileocolic Type Intussusception Associated with Hematemesis
Young Hee Kim, Seung Soo Kim, Eun Sook Suh
Clin Exp Pediatr. 2003;46(7):722-725.   Published online July 15, 2003
Intussusception is a common cause of lower intestinal bleeding from five to 11 months of age. Typical symptoms are paroxysmal colicky pain, irritability, vomiting, abdominal mass and currant jelly stool, but hematemesis is rare. Though the most common type of intussusception is ileocecal type, it is rarely accompanied with hematemesis. Up until the present, hematemesis has been observed rarely but...
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...
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