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Two Cases of Idiopathic Dilatation of the Common Bile Duct.

Jin Bok SONG ; Kwang Kun HWANG ; Chung In CHA ; Kew Taek KIM

Journal of the Korean Pediatric Society.1977;20(2):136-143.

Choledochal cyst, or cystic dilatation of the common bile duct, is generally considered to be an uncommon indiopathic congenital condition that may produce symptoms in infancy but more of ten is discovered at some time during the first two decades of life. Female patients have outnumbered males 3 or 5 to1, and almost cases reported by many authors had more than two symptoms among the classic triad of abdominal mass, jaundice and upper abdominal pain. Authors would like to submit here with the brief review of the literature and report on 2 cases of idiopathic dilatation of the common bile duct that they had experiences among the Pediatric inpatient at Busan St. Benedict Hospital, and the diagnosis was established by clinical, laboratory, X-ray and surgical findings. Cases I in 14 year old girl was complained of severe abdominal distension, jaundice, pruritus and abdominal mass. On 7th admission day, exploratory laparatomy was done and choledochal cyst of 1722cm in size was confirmed and has been followed up for 1 year after Roux-en-Y choledochojejunsotomy without any complications. Cases II in 9 year old boy was complained of intermittent fever, jaundice, colicky abdominal pain and upper abdominal mass. On 6th admission day, exploratory laparatomy was done and choledochal cyst of 1015cm in size was confirmed and has been followed up for 1 year after choledochoduodenostomy with cholecystectomy without any complications.
Abdominal Pain ; Adolescent ; Busan ; Child ; Cholecystectomy ; Choledochal Cyst ; Choledochostomy ; Common Bile Duct* ; Diagnosis ; Dilatation* ; Female ; Fever ; Humans ; Inpatients ; Jaundice ; Male ; Pruritus

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A Case of Congenital Cretinism.

Kwang Kun HWANG ; Tae Ho LEE ; Chung In CHA ; Kew Taek KIM

Journal of the Korean Pediatric Society.1977;20(2):126-135.

A case of congenital hypothyroidism in 7 month old infant was presented. This baby was admitted because of growth retardation, feeding difficulties, and constipation. We diagnosed this case by clinical features as well as thyroid scintigram, X-ray survey for bone age and skeletal maturation and biochemical studies. The patient was treated with desiccated thyroid, but was died of intercurrent infection on 20th hospital day. The review of literature was made briefly.
Congenital Hypothyroidism* ; Constipation ; Humans ; Infant ; Thyroid Gland

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A Study of the Growth and Development of the Infants Participated in Kyungpook Well-Baby Contest.

Jin Gon JUN ; Jong Hwan KIM ; Doo Hong AHN ; Chung Hun CHOI

Journal of the Korean Pediatric Society.1977;20(2):115-125.

Yearly Well-Baby Contest has been held in order to recognize the importance pf the childanjx growth and development. The total number of the infants (6 months to 24 months) who participated in this Kyungpook regional contest, held on April 1976, were 149 comprising 130 infant boys and 19 infant girls. After throughful investigation of the several important parameters including their physical development (body length, weight, head and chest circumference), nutritional status (arm circumference, Kaup index, hemoglobin and signs of the vitamin D deficiency) and the feeding history, the following results were obtained. Comparing their body length and weight with Korean Children Growth Standard (K.S.) and Japanese Children Growth Standard (J.S.), their body length were superior to those of K.S. (3.11cm to 9.4cm) and J.S. (2.3cm to 6.27cm) and their body weight superior t those of K.S. (1.99kg to 4.41kg) and J.S.(1.56kg to 3.21kg). Their arm circumference were superior to those of Korean Mean Value (1973, Hong) by 1.57cm to 2.3cm and no significant difference was noted between both sex and each age groups. The Kaup index was above 20 in 15.4% of male infant and 21.1% of female infants. Mixed feeding was the most common form of the infant feeding comprising 41.6%, followed by breast feeding in 39.6% and bottle feeding in 18.8%. Weaning was started within 6months of age in 54.4% of the infants and the majority(92.6%) of the infants within 12 months of age. In one third of the infants (32.2%) there were signs of the vitamin D deficiency including Harrisonanjx groove, rachitic rosary etc., and no difference was noticed between different types of feeding. One third (32.9%) of the infants showed anemia, using the criteria of anemia as being hemoglobin less than 10.5mg%. Incidence was the highest among breast-fed infants (47.5%), followed by mixed-fed infants in 24.2% and bottle-fed infants in 21.4%. In 19.2% of the infants supplemental vitamin D was given and only 6.7% of the infants was given iron as supplement. The conclusion arrived from these results indicated that their physical growth was quite good but nutritional status was inadequate, represented in many infants sych as signs of the vitamin D deficiency and anemia. It is hoped that by education and guidance of the mothers, especially about the proper feeding method, infants growth and development could be improved further.
Anemia ; Arm ; Asian Continental Ancestry Group ; Body Weight ; Bottle Feeding ; Breast Feeding ; Child ; Education ; Feeding Methods ; Female ; Growth and Development* ; Gyeongsangbuk-do* ; Head ; Hope ; Humans ; Incidence ; Infant* ; Iron ; Male ; Mothers ; Nutritional Status ; Thorax ; Vitamin D ; Vitamin D Deficiency ; Weaning

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Study of the Serum Levels of Immunoglobulins in Normal Children.

Sung Won PARK

Journal of the Korean Pediatric Society.1977;20(2):106-114.

The concentrations of three immunoglobulins (IgG, IgA, and IgM) were studied in sera of 307 healthy children from the age of one month to 16 years. The method used was a single radial immunodiffusion technique, with the use of commercially available antibody agar plates. The results obtained were compared with those found in umbilical cord sera and with the average levels in healthy adults. The average concentration of IgG was lowest at the age of 3 months, i.e. about 40 percent of adult level. At the age of one year the average level of IgG was 50 percent of adult level and approached the adult level at the age of 6~8 years (Fig. 1,4 and 5 and Table 1). In cord sera IgA could only be demonstrated in three neonates out of 20. At the age of one year an average IgA level of about 25 percent of adult level was found and the adult level was reached at age of 12 to 16 years(Fig. 2, 4 and 5, and Table 1). IgM was detected in all cord sera with an average concentration of 15 percent of adult level. At the age of 3 months the IgM values were about 50 percent of adult level and at the age of one year approximately 70 percent(Fig. 3,4 and 5, Table 1).
Adult ; Agar ; Child* ; Humans ; Immunodiffusion ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Immunoglobulins* ; Infant, Newborn ; Umbilical Cord

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A Case of Secondary Hemosiderosis and Hepatic Fibrosis in a Pateint with Acute Myelogenous Leukemia.

Hyoung No KIM ; Jae Won HUH ; Jae Sun PARK

Journal of the Korean Pediatric Society.1998;41(3):420-424.

Chronic iron overload is associated with life-threatening complications, such as cardiomyopathy, liver cirrhosis, diabetes, hypothyroidism, hypoparathyroidism and hypogonadism. We experienced a case of secondary hemosiderosis with hepatic fibrosis in a 8-year-old boy who had been transfused 56 pints of packed red blood cells for a supportive therapy of acute myelogenous leukemia for a 27-month period. Intramuscular injection of Desferroxamine (40mg/kg/ day) was done for 9 months, however, there was no sign of improvement in hepatic size, consistency, and in liver biopsy findings. Except for mild thrmobocytopenia which seems to be the result of hypersplenism, the boy remained in remission for 3 2/3 years. We think, the prompt use of Desferroxamine therapy may be needed to avoid iron overload, if patients recieved multiple transfusions and high serum ferritin level.
Biopsy ; Blood Transfusion ; Cardiomyopathies ; Child ; Deferoxamine ; Erythrocytes ; Ferritins ; Fibrosis* ; Hemosiderosis* ; Humans ; Hypersplenism ; Hypogonadism ; Hypoparathyroidism ; Hypothyroidism ; Injections, Intramuscular ; Iron Overload ; Leukemia, Myeloid, Acute* ; Liver ; Liver Cirrhosis ; Male

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A Case of Congenital Nephrotic Syndrome due to Diffuse Mesangial Sclerosis.

Jung Jin YU ; Hae Il CHEONG ; Hyun Soon LEE ; Yong CHOI ; Dong Kyu JIN

Journal of the Korean Pediatric Society.1998;41(3):415-419.

Diffuse mesangial sclerosis (DMS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. Infants with DMS develop nephrotic syndrome before 2 years of age and progress to end stage renal disease within 3 years of age. The authors experienced a case of isolated DMS in a 4-month-old male infant who had nephrotic syndrome for 1 month. The diagnosis was confirmed on the basis of clinical, laboratory, pathological and molecular genetic findings. This is the 3rd case report of DMS in our country and the 1st case report of isolated DMS confirmed by molecular genetic study.
Denys-Drash Syndrome ; Diagnosis ; Humans ; Infant ; Kidney Failure, Chronic ; Male ; Molecular Biology ; Nephrotic Syndrome* ; Pathology ; Sclerosis*

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A Case of Alagille Syndrome.

Eun Kyung HWANG ; Gwang Hoon LEE ; Eell RYOO ; Kang Ho CHO ; Gil Hyun KIM ; Hak Soo LEE ; Ji Hye KIM ; Sung Hae PARK ; Hee Sup KIM

Journal of the Korean Pediatric Society.1998;41(3):410-414.

Alagille syndrome is characterized by chronic cholestasis, posterior embryotoxon, skeletal abnormalities, cardiovascular abnormalities, and a typical face with prominent forehead and pointed chin. Its histological feature includes paucity of interlobular bile ducts. We experienced a 49-day-old female infant presenting with frequent upper respiratory tract infection and persistent jaundice. She had a typical face and chronic cholestasis. Echocardiograms revealed peripheral pulmonary stenosis. The histological examination of liver revealed paucity of interlobular bile ducts.
Alagille Syndrome* ; Cardiovascular Abnormalities ; Chin ; Cholestasis ; Female ; Forehead ; Humans ; Infant ; Jaundice ; Liver ; Pulmonary Valve Stenosis ; Respiratory Tract Infections

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Two Cases of Leigh Disease.

Seong Hun KIM ; Soo En PARK ; Ju Seok LEE ; Sang Ook NAM ; Yeong Tak LIM

Journal of the Korean Pediatric Society.1998;41(3):405-409.

Leigh Disease, or subacute necrotizing encephalopathy (SNE), is a degenerative disorder characterized by lesions of the gray and white matter in the bran and spinal cord. The pathogenesis was known as mitochondrial enzyme defect of the respiratory chain system. We experienced 2 cases of Leigh disease. The first case, a seven-month old girl who was presented with weak respiration and failure to thrive, showed lactic acidemia and increased lactic acid in CSF fluid, high signal intensity in the bilateral putamen and head of caudate of nucleus at T2 weighted MR imaging. The second case, a 3-year-old girl with ataxic gait and bilateral ptosis also showed lactic acidemia, increased lactic acid in CSF fluid and high signal intensity in the bilateral basal ganglia. Respiratory difficuly developed in both cases and died within 1 month after visiting our hospital. The diagnosis was made by lactic acidosis and specific MRI finding. We report these cases with a brief review of its related literature.
Acidosis, Lactic ; Basal Ganglia ; Child, Preschool ; Diagnosis ; Electron Transport ; Failure to Thrive ; Female ; Gait ; Head ; Humans ; Lactic Acid ; Leigh Disease* ; Magnetic Resonance Imaging ; Putamen ; Respiration ; Spinal Cord

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Two Cases of Factor XI Deficiency in Sisters.

Yun Kyeong CHO ; Jae Young LIM ; Yang Suk JUNG ; Chan Hoo PARK ; Hyang Ok WOO ; Byung Kiu PARK ; Hee Shang YOUN

Journal of the Korean Pediatric Society.1998;41(3):401-404.

We experienced two cases of factor XI deficiency in a 5 yearr 7 month-old girl, whose chief complaint was coke-colored gross hematuria, and her 9-year-old elder sister. The laboratory findings were prolonged aPTT and prominent deficiency of factor XI. The gross hematuria of the index case was identified due to acute poststreptococcal glomerulonephritis. So we report two cases of factor XI deficiency with a brief review of the related literature.
Child ; Factor XI Deficiency* ; Factor XI* ; Female ; Glomerulonephritis ; Hematuria ; Humans ; Infant ; Siblings*

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A Case of VATER association Variant with a 46, XY, inv (6)(q11q16) Inversion.

Dong Ho PARK ; Dae Bong JUNG ; Eun Seok YANG ; Yeong Bong PARK ; Sang Kee PARK

Journal of the Korean Pediatric Society.1998;41(3):396-400.

VATER association of vertebral defects, imperforate anus, tracheoesophageal (T-E) fistula with esiphageal atresia, and radial and renal dysplasia are utilized to identify this complex. Other anomalies are vascular anomalies (cardiac defects and single umbilical artery), and prenatal and postnatal growth deficiency, defects of external genital and urinary tract as well as bone defects (preaxial lower extremity anomalies, supernumerary digits, rib anomaly). This pattern of malformation generally occurs sporadically in an otherwise normal family, and may occur as a part of a broader pattern, such as the trisomy 18, del (4p, 6q, and 13q) syndromes, and sirenomelia. As inversion of chromosome does not lose of genetic materials, phenotypic abnormalities does not occur. But rare cases have been reported. We report a neonate who had multiple congenital anomalies, including the VATER association defects associated with an unusual chromosomal abnormality, a paracentric inversion of chromosome 6 : 46, XY, .inv (6)(q11q16).
Anus, Imperforate ; Chromosome Aberrations ; Chromosomes, Human, Pair 6 ; Ectromelia ; Fistula ; Humans ; Infant, Newborn ; Lower Extremity ; Ribs ; Trisomy ; Urinary Tract

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