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1.
Korean Journal of Pediatrics ; : S19-S24, 2016.
Article in English | WPRIM | ID: wpr-228471

ABSTRACT

Constitutional interstitial deletions of the long arm of chromosome 5 (5q) are quite rare, and the corresponding phenotype is not yet clearly delineated. Severe mental retardation has been described in most patients who present 5q deletions. Specifically, the interstitial deletion of chromosome 5q33.3q35.1, an extremely rare chromosomal aberration, is characterized by mental retardation, developmental delay, and facial dysmorphism. Although the severity of mental retardation varies across cases, it is the most common feature described in patients who present the 5q33.3q35.1 deletion. Here, we report a case of a de novo deletion of 5q33.3q35.1, 46,XY,del(5)(q33.3q35.1) in an 11-year-old boy with mental retardation; to the best of our knowledge this is the first case in Korea to be reported. He was diagnosed with severe mental retardation, developmental delay, facial dysmorphisms, dental anomalies, and epilepsy. Chromosomal microarray analysis using the comparative genomic hybridization array method revealed a 16-Mb-long deletion of 5q33. 3q35.1(156,409,412-172,584,708)x1. Understanding this deletion may help draw a rough phenotypic map of 5q and correlate the phenotypes with specific chromosomal regions. The 5q33.3q35.1 deletion is a rare condition; however, accurate diagnosis of the associated mental retardation is important to ensure proper genetic counseling and to guide patients as part of long-term management.


Subject(s)
Child , Humans , Male , Arm , Chromosome Aberrations , Chromosomes, Human, Pair 5 , Comparative Genomic Hybridization , Diagnosis , Epilepsy , Genetic Counseling , Intellectual Disability , Korea , Methods , Microarray Analysis , Phenotype
2.
Neonatal Medicine ; : 211-217, 2014.
Article in Korean | WPRIM | ID: wpr-53916

ABSTRACT

PURPOSE: The neonatal (NMR) or infant mortality rate (IMR) in South Korea is lower than that in the United States. We aimed to investigate the contributing factors to this difference in mortality rates. METHODS: The study populations consisted of five groups, namely group A, comprising live births in South Korea during 2009-2011 (n=1,383,806), and groups B (live births to Korean parents, n=107,309), C (Caucasian births, n=31,588,183), D (African-origin, n=4,381,664), and E (all live births, n=49,384,187) comprising various US live births during 2000-2011. Maternal characteristics, birth outcomes, and mortality rates in these five groups were compared according to birth weight (BW) and gestational age (GA). RESULTS: Maternal characteristics such as BW distribution and very low and low BW rates among infants in South Korea and those in the United States were quite similar. Both rates were significantly lower among the Korean live births than among Caucasian and African-origin live births in the United States. However, the mortality rates of these small birth weight groups were significantly higher in the Korean infants born in South Korea than in those born in the United States, or in Caucasian and African-origin infants born in the United States. Similar results were noted when analyzed according to gestational age. CONCLUSIONS: The NMR or IMR in South Korea is lower than that in the United States, primarily due to the overwhelming favorable BW and GA distribution, despite the lower BW- and GA-specific survival rates in the live births in South Korea than in the United States.


Subject(s)
Humans , Infant , Infant , Birth Weight , Racial Groups , Gestational Age , Infant Mortality , Korea , Live Birth , Mortality , Parents , Parturition , Survival Rate , United States
3.
Korean Journal of Pediatric Infectious Diseases ; : 225-230, 2014.
Article in English | WPRIM | ID: wpr-161263

ABSTRACT

Kawasaki disease (KD) is an immune-mediated disease which is a leading cause of acquired cardiovascular disease in developed country. Recently, tumor necrosis factor-alpha (TNF-alpha) blocker, infliximab has been considered a promising option for patients with refractory KD. Although chronic use of a TNF-alpha blocker could increase risk of opportunistic infections, a few studies have documented that use of infliximab was safe without serious adverse effects in patients with KD. We observed serious bacterial infection after infliximab treatment in an infant with refractory KD. Our patient was a 5-month-old male infant diagnosed with KD who did not respond to repeated doses of intravenous immunoglobulin. We effectively treated him with a single infusion of infliximab (5 mg/kg), but gram-negative (Acinetobacter lwoffii) septicemia developed after infliximab infusion. Therefore, we report a case of serious septicemia after treatment with infliximab, and suggest considering the risk of severe infection when deciding whether to prescribe infliximab to an infant with refractory KD.


Subject(s)
Humans , Infant , Male , Bacterial Infections , Cardiovascular Diseases , Developed Countries , Immunoglobulins , Mucocutaneous Lymph Node Syndrome , Opportunistic Infections , Sepsis , Tumor Necrosis Factor-alpha , Infliximab
4.
Korean Journal of Pediatrics ; : 542-545, 2014.
Article in English | WPRIM | ID: wpr-161256

ABSTRACT

Bickerstaff's brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of consciousness. It is similar to Miller Fisher syndrome, a variant of Guillain-Barre syndrome, in that they share features such as ophthalmoplegia and ataxia. The difference is that patients with Bickerstaff's brainstem encephalitis have impaired consciousness, whereas patients with Miller Fisher syndrome have alert consciousness and areflexia. Here, we report the case of a 3-year-old child who was diagnosed with Bickerstaff's brainstem encephalitis presenting typical clinical features and interesting radiological findings. The patient showed ophthalmoplegia, ataxia, and subsequent stuporous mentality. Brain magnetic resonance imaging revealed high signal intensity in the pons and cerebellum around the 4th ventricle on a T2-weighted image. He was successfully treated with intravenous immunoglobulin. Differentiation of Bickerstaff's brainstem encephalitis and Miller Fisher syndrome is often difficult because they possess many overlapping features. Brain magnetic resonance imaging may be helpful in diagnosing Bickerstaff's brainstem encephalitis, especially when lesions are definitely found.


Subject(s)
Child , Child, Preschool , Humans , Ataxia , Brain , Brain Stem , Cerebellum , Consciousness , Encephalitis , Guillain-Barre Syndrome , Immunoglobulins , Magnetic Resonance Imaging , Miller Fisher Syndrome , Ophthalmoplegia , Pons , Stupor
5.
Korean Journal of Perinatology ; : 285-294, 2011.
Article in Korean | WPRIM | ID: wpr-175190

ABSTRACT

PURPOSE: This study investigated the pattern of hematologic profile and eosinophilia for a month after birth in very low birth weight (VLBW) infants. METHODS: The medical records of 141 VLBW infants (birth weight, or =700/mm3). RESULTS: Overall, 50.4% of all infants had at least one instance of eosinophilia for a month after birth. There were 50.7% with moderate eosinophilia (1,000-2,999/mm3). White blood cell (WBC) counts and absolute neutrophil count (ANC) climaxed on 7th day of life, whereas eosinophilia mainly occurred on 21st day of life. The demographic data and perinatal characteristics of infants with and without eosinophilia were compared. Prevalence of eosinophilia was associated with gestational age and total parenteral nutrition on 21st day of life; total parenteral nutrition and transfusion on 28th day of life. Eosinophilia was closely associated with transfusion on logistic regression analysis (P<0.05). CONCLUSION: Eosinophilia in VLBW infants occurs mainly on 21st day of life. Eosinophil counts showed a separate trend different from WBC counts and ANC. Transfusion was significantly associated with eosinophilia.


Subject(s)
Humans , Infant , Infant, Newborn , Blood Cell Count , Eosinophilia , Eosinophils , Gestational Age , Infant, Very Low Birth Weight , Intensive Care, Neonatal , Leukocytes , Logistic Models , Medical Records , Neutrophils , Parenteral Nutrition, Total , Parturition , Prevalence
6.
Journal of the Korean Society of Neonatology ; : 391-394, 2011.
Article in Korean | WPRIM | ID: wpr-59449

ABSTRACT

Neonatal lupus erythematosus (NLE) is a rare disease characterized by typical clinical features and the transplacental passage of maternal autoantibodies, particularly anti-SSA/Ro. The major clinical manifestations are cutaneous lupus lesions, congenital heart block, hematological disorders, and hepatobiliary diseases. We report a case of NLE presenting with multiple round and oval target-like erythematous skin lesions and abnormal liver function, born to a clinically asymptomatic mother whose diagnosis was made retrospectively only after her newborn's diagnosis. Both the infant and the mother were positive for the anti-SSA/Ro and anti-SSB/La antibodies.


Subject(s)
Humans , Infant , Antibodies , Autoantibodies , Heart Block , Liver , Lupus Erythematosus, Systemic , Mothers , Rare Diseases , Retrospective Studies , Skin
7.
Korean Journal of Pediatrics ; : 655-660, 2009.
Article in Korean | WPRIM | ID: wpr-163698

ABSTRACT

PURPOSE: To compare birth weight between infants with a single umbilical artery (SUA) and normal infants, investigate the associated anomalies of infants with SUA and isolated SUA (no abnormality of external appearance on birth, except SUA), and determine the prognosis of infants with isolated SUA. METHODS: Live-born infants with SUA (n=59) detected by physical examination from among 15,193 live births in seven university hospitals in Korea between January 1, 2004, to August 1, 2007, were reviewed retrospectively, with 236 normal infants serving as the control group. RESULTS: A statistical difference was observed between the groups in birth weight and in vitro fertilization. The incidence of infants with SUA was 0.37%. Congenital malformations were observed in 21 infants with cardiovascular (n=15, 25.4%), gastrointestinal (n=2, 3.4%), genitourinary (n=9, 15.3%), neuromusculoskeletal (n=6, 10.2%), central nervous system (n=1, 1.7%), chromosomal (n=1, 1.7%), and other (n=3, 5.1%) abnormalities. There were 49 (83.1%) infants with isolated SUA in this study population; among them, the associated congenital malformations were cardiovascular (n=6, 12.2%) and genitourinary (n=6, 12.2%) abnormalities. Two infants with cyanotic heart disease were operated and four infants with acyanotic heart disease showed improvements without any treatment. Six infants with genitourinary abnormalities on renal ultrasound had mild hydronephrosis without further consequences. CONCLUSION: The incidence of structural abnormalities in the cardiovascular and genitourinary systems is high and the genitourinary anomalies associated with isolated SUA have relatively good prognosis.


Subject(s)
Humans , Infant , Birth Weight , Central Nervous System , Fertilization in Vitro , Heart Diseases , Hospitals, University , Hydronephrosis , Incidence , Korea , Live Birth , Parturition , Physical Examination , Prognosis , Retrospective Studies , Single Umbilical Artery , Umbilical Arteries , Urogenital Abnormalities , Urogenital System
8.
Journal of the Korean Society of Neonatology ; : 75-79, 2008.
Article in Korean | WPRIM | ID: wpr-86432

ABSTRACT

PURPOSE: There are many causes of hematochezia in neonates and during early infancy. Cow`s milk allergy is considered to be the most common cause of bloody stools in infants; however, cow`s milk allergy is diagnosed in relatively few patients. In a significant proportion of case investigations, the etiology of hematochezia is not determined. The objectives of this study were to evaluate the clinical course and prognosis of patients with hematochezia of unknown etiology. METHODS: The patients were <4-month-old infants with hematochezia in whom infectious and surgical etiologies had been excluded. We investigate retrospectively the clinical course and laboratory finding. We also perform 2-years follow-up study and evaluate prognosis of the patients. RESULTS: In all study patients, RAST milk was negative. All patients had self-limited hematochezia. In 2-years of follow-up, the prognosis of patients was good. There were no differences at 2-year regarding allergic tendencies of the patients, as determined by the increase in total eosinophil count. CONCLUSION: Hematochezia in neonates and during early infancy is generally a benign and self-limiting symptom. Based on 2-years of follow-up, the prognosis of the patients is good.


Subject(s)
Humans , Infant , Infant, Newborn , Eosinophils , Follow-Up Studies , Gastrointestinal Hemorrhage , Milk , Milk Hypersensitivity , Prognosis , Retrospective Studies
9.
Journal of the Korean Society of Neonatology ; : 68-74, 2006.
Article in Korean | WPRIM | ID: wpr-70655

ABSTRACT

PURPOSE: The aim of this study was to identify usefulness of simple oral and gastric pH measurement using pH paper on detection of symptomatic gastroesophageal reflux in neonates. METHODS: This prospective study included a total of 66 neonates born at Konyang University Hospital from June 2004 to June 2005. Each neonate's oral and gastric pH levels measured with pH paper at 6 hourly intervals. Suspected gastroesophageal refluex neonates were studied 24-hr lower esophageal pH monitoring or upper GI series and confirmed. We compared oral and gastric pH between symptomatic gastroesophageal reflux (GER) group and asymptomatic (control) group. RESULTS: GER group consist of 12 neonates and control group consist of 54 neonates. Oral and gastric pH were 5.4+/-0.6, 2.9+/-0.5 in GER group, 6.0+/-0.3, 3.9+/-0.9 in control group, the differences between two groups were significant (P<0.05). All neonates of GER group were corfirmed gastroesophageal reflux by 24-hr lower esophageal pH monitoring or upper GI series studies. Our data indicate as a predictor for significantly symptomatic gastroesophageal reflux, at oral pH 5.75, has a sensitivity 92%, specificity 89%, positive predictive value of 65%, and negative predictive value of 98%. The difference between oral and gastric pH (oral pH-gastric pH) was not significant in each group. CONCLUSION: In neonates with symptomatic gastroesophageal reflux oral and gastric pH were significantly lower than asymptomatic neonates. Oral and gastric pH were related with clinically significant symptoms of gastroesophageal reflux. We suggest that pH measurement could be a possible simple screening test of symptomatic gastroesophageal reflux.


Subject(s)
Humans , Infant, Newborn , Esophageal pH Monitoring , Gastroesophageal Reflux , Hydrogen-Ion Concentration , Mass Screening , Prospective Studies , Sensitivity and Specificity
10.
Korean Journal of Pediatrics ; : 617-622, 2006.
Article in Korean | WPRIM | ID: wpr-151859

ABSTRACT

PURPOSE: High-Sensitivity C-reactive protein(hs-CRP) has been recognized as a very useful and sensitive predictor of the future risk of myocardial infarction. But the clinical significance of hs-CRP in children remains uncertain. To confirm the existence of obesity-induced vascular inflammation and the association between metabolic syndromes and elevation of CRP in children, we investigated the relationship among CRP, obesity, blood pressure(BP), and serum lipids in schoolboys. METHODS: Twenty-eight obese(BMI 29.61+/-3.29 kg/m2) and 93 non-obese(BMI 18.99+/-2.21 kg/m2) boys aged 14 years were examined. Serum CRP levels was measured by the high sensitive latex turbidimetric immunoassay and subjects with CRP levels below 0.3 mg/dL were adopted to avoid the influence of acute infection. RESULTS: Obese children had significantly higher hs-CRP levels than their non-obese group(0.104+/-0.075 vs. 0.054+/-0.005 mg/dL). In the obese group, BMI, systolic blood pressure, diastolic blood pressure, apolipoprotein B, atherogenic index, and triglyceride were significantly higher than in non-obese. The BMI, diastolic blood pressure, apolipoprotein E, atherognic index, and triglyceride showed positive correlation with log CRP by simple regression. Multiple regression analysis indicated that BMI and apolipoprotein E were strongly related to CRP. CONCLUSION: This study revealed that obese children tended to have higher levels of serum hs-CRP, BP elevation and dyslipidemia than the control group and that BMI and apolipoprotein E were strongly related to CRP. These results indicate that obesity related metabolic syndrome can be developed in children.


Subject(s)
Child , Humans , Apolipoproteins , Blood Pressure , C-Reactive Protein , Dyslipidemias , Immunoassay , Inflammation , Latex , Myocardial Infarction , Obesity , Triglycerides
11.
Journal of Korean Society of Pediatric Endocrinology ; : 205-208, 2006.
Article in Korean | WPRIM | ID: wpr-26280

ABSTRACT

Hypocalcemia is due to Hypoparathyroidism, Vitamin D deficiency, Hypomagnesemia, Inadequate calcium intake. The benefits of breast-feeding are well established. There are no need to supply calcium or Vitamin D in breast-fed infant. We report a case of infantile hypocalcemia caused by Vitamin D deficiency in exclusively breast-fed infant. He had no hypocalcemic symptom and hypocalcemia was found incidentally by routine laboratory tests during pneumonia treatment. He was presented with a low serum calcium level and 1,25(OH)2 Vit D3 level and high PTH. He was improved by Calcium and Vitamin D supplement. After then his mother continued breast feeding exclusively and resisted to feed her baby weaning food. During follow up period, hypocalcemia was recheked after discontinuation of vitamin D supplement. At 11 months of age, the calcium level was normal without vitamin D supplement after he had eaten weaning food. This report describes a case of hypocalcemia induced by vitamin D deficiency in exclusively breast-fed infant, with review of the literature.


Subject(s)
Humans , Infant , Breast Feeding , Calcium , Follow-Up Studies , Hypocalcemia , Hypoparathyroidism , Mothers , Pneumonia , Vitamin D Deficiency , Vitamin D , Vitamins , Weaning
12.
Korean Journal of Pediatrics ; : 539-544, 2005.
Article in Korean | WPRIM | ID: wpr-109132

ABSTRACT

PURPOSE: The aim of this study was to investigate the pathophysiologic role of serum E-selectin, vascular endothelial growth factor(VEGF)-induced cell adhesion mollecule in Kawasaki disease(KD) and to look for the evidence of direct relationship between the plasma levels of soluble E-selectin and the incidence of coronary artery lesion(CAL). METHODS: Changes in plasma levels of sE-selectin(n=98) over time were measured by enzyme-linked immunosorbent assay(ELISA) in 23 patients with acute KD and 25 age-matched febrile children. RESULTS: Compared with control values, the peak levels of plasma sE-selectin were significantly elevated(mean+/-S.E.:22.89+/-12.53 ng/mL vs 10.65+/-3.42 ng/mL, P=0.01) in KD. 5 patients with CAL, plasma sE-selectin levels before treatment were higher than in 18 patients without CAL(mean+/-S.E.:39.43+/-15.08 ng/mL and 19.00+/-8.32 ng/mL, respectively; P=0.01). Plasma sE-selectin declined rapidly in the majority of KD patients regardless of the presence of CAL. Plasma sE-selectin levels after treatment and convalesent period were similar in KD patients with and without CAL. The plasma levels sE-selectin were correlated with those of white blood cell count(r=0.299, P<0.05), CRP(r=0.430, P<0.05), serum albumin(r=-0.483, P<0.05), serum protein(r=-0.502, P<0.05) and hemoglobin(r=-0.372, P<0.05) not with those of ESR, platelet, or duration of fever. There were significant differences in the initial level of serum sE-selectin between KD with and without CAL(mean+/-S.E.:39.44+/-15.08 ng/mL vs. 19.00+/-17.18 ng/mL) in multivariated linear tests. CONCLUSION: Plasma sE-selectin levels were significantly higher in KD than in other febrile illness. Higher plamsa levels of sE-selectin may have potential as a predictor of CAL in patients with KD.


Subject(s)
Child , Humans , Blood Platelets , Cell Adhesion , Coronary Vessels , E-Selectin , Fever , Incidence , Leukocytes , Mucocutaneous Lymph Node Syndrome , Plasma
13.
Journal of the Korean Society of Neonatology ; : 172-178, 2005.
Article in Korean | WPRIM | ID: wpr-56299

ABSTRACT

PURPOSE: The purpose of this study was to measure the oral and gastric pH among preterm and term infants by the simple experimental method using pH paper. METHODS: This prospective study included a total of 60 neonates born at Konyang University Hospital from June 2004 to September 2004. Each neonate's oral and gastric pH levels were measured with pH paper at 6 hourly intervals on the first and the fifth day of life. 15 preterm infants were examined until twenty first day of life. RESULTS: The oral and gastric pH of 60 neonates were 6.3+/-0.5 and 4.1+/-1.0 on the first day and 6.0+/-0.3 and 3.9+/-0.9 on the fifth day, respectively. The preterm infant had lower oral and gastric pH of 6.4+/-0.6 and 4.3+/-1.1 on the first day and 6.0+/-0.2 and 4.2+/-1.0 on the fifth day, respectively in comparison to those of term infant's oral and gastric pH of 6.3+/-0.4, 4.0+/-0.9 on the first day and 6.1+/-0.3 and 3.7+/-0.8 on the fifth day, respectively. The gastric pH's of preterm infant on the first, fifth and twenty first day of life were 4.6+/-1.4, 4.3+/-1.0 and 4.2+/-1.0, respectively. CONCLUSION: This study reports the value of neonates' oral and gastric pH on the first and fifth day of life. The average gastric pH of neonate is higher than that of adult's and preterm infant's gastric pH is higher than term infant's. The gastric pH of preterm infant decreased in time.


Subject(s)
Humans , Infant , Infant, Newborn , Gastroesophageal Reflux , Hydrogen-Ion Concentration , Infant, Premature , Prospective Studies
14.
Journal of the Korean Child Neurology Society ; : 235-240, 2004.
Article in Korean | WPRIM | ID: wpr-205921

ABSTRACT

Primary central nervous system atypical teratoid/rhabdoid tumors are rare and extremely aggressive malignancies of early childhood. These tumors are most common in infants less than 2 years of age. Diagnosis is based on distinctive light microscopic and immunohistochemical findings, coupled with a molecular genetic analysis. A histologic features of these tumors are epithelial and/or mesenchymal components in addition to rhabdoid, with or without neuroepithelial fields. The expression of the epithelial membrane antigen, vimentin, and the smooth muscle actin are characteristic of these tumors. Treatment includes surgery, chemotherapy, and radiotherapy. Prognosis is poor despite of an aggressive therapy. We report one case of an atypical teratoid/rhabdoid tumor in a young child. She is an 18 month-old-girl who presented with central type facial palsy. After extensive surgery she improved transiently but relapsed immediately. Her condition was not permitted to receive radiotherapy or chemotherapy. Thereafter, with phrenic nerve palsy, she suffered from recurrent episodes of pneumonia and respiratory difficulties. Finaly, she was expired three months after the diagnosis and treatment.


Subject(s)
Child , Humans , Infant , Actins , Central Nervous System , Cerebellum , Diagnosis , Drug Therapy , Facial Paralysis , Molecular Biology , Mucin-1 , Muscle, Smooth , Paralysis , Phrenic Nerve , Pneumonia , Prognosis , Radiotherapy , Vimentin
15.
Korean Journal of Physical Anthropology ; : 153-161, 2004.
Article in English | WPRIM | ID: wpr-205190

ABSTRACT

The pogo mouse is an autosomal recessive ataxic mutant that arose spontaneously in the inbred KJR/MsKist strain derived originally from Korean wild mice. The ataxic phenotype is characterized by difficulty in maintaining posture and the consequent inability to walk straight. In our previous study about pogo mice cerebellum, we reported the Purkinje cell abnormalities and ectopic expression of tyrosine hydroxylase (TH) in Purkinje cell. In this study, we have provided an abnormal expression of NPY in ataxic mutant pogo mice for the first time. There was increased immunoreactivity for NPY in Purkinje cell of ataxic pogo (pogo/pogo) mice compared to those of heterozygote non-ataxic pogo mice (pogo/+, control group). In our previous study, TH is also expressed abnormally in Purkinje cells of ataxic mutant pogo (pogo/pogo) mouse cerebellum. To compare the expression patterns of TH and NPY within some Purkinje cell using double immunofluorescence, most of NPY-immunoreactive Purkinje cells in the ataxic pogo mice are TH-immunoreactive Purkinje cells. However, all of TH-immunoreactive Purkinje cells are not express the NPY. These data reveal that abnormal NPY-immunoreactivity in the ataxic pogo (pogo/pogo) cerebellum is restricted to a subset of cells within the ectopic TH-immunoreactive Purkinje cell subset. These results further suggest that Purkinje cell abnormalities contribute to motor ataxia in the ataxic pogo mouse.


Subject(s)
Animals , Mice , Ataxia , Cerebellum , Fluorescent Antibody Technique , Heterozygote , Neuropeptide Y , Neuropeptides , Phenotype , Posture , Purkinje Cells , Tyrosine 3-Monooxygenase
16.
Journal of the Korean Society of Pediatric Nephrology ; : 250-255, 2004.
Article in Korean | WPRIM | ID: wpr-46789

ABSTRACT

Wilson s disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson s disease. However, nephrotoxicity can occur after D-penicillamine treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syndrome in a 3-year-old girl with Wilson s disease 3 weeks after initiation of D-penicillamine.


Subject(s)
Child, Preschool , Female , Humans , Brain , Cornea , Glomerulonephritis, Membranous , Liver , Nephrosis, Lipoid , Nephrotic Syndrome , Penicillamine , Trientine , Zinc Sulfate
17.
Journal of the Korean Society of Neonatology ; : 225-229, 2004.
Article in Korean | WPRIM | ID: wpr-15028

ABSTRACT

An H-type tracheoesophageal fistula is a rare congenital condition of which incidence is approximately 1: 50, 000-80, 000 births. Although patient usually has recurrent respiratory symptoms such as choking episodes and cyanotic spells, its rarity and atypical symptoms may delay its detection. Besides, conventional esophagogram often fail to find the defect. We reported a case of H-type tracheoesophageal fistula, diagnosed by tube esophagram with infant in the prone position. During the test, contrast medium was injected while the catheter is slowly withdraws from lower esophagus.


Subject(s)
Humans , Infant , Airway Obstruction , Catheters , Esophagus , Incidence , Parturition , Prone Position , Tracheoesophageal Fistula
18.
Journal of the Korean Society of Neonatology ; : 65-71, 2004.
Article in Korean | WPRIM | ID: wpr-172764

ABSTRACT

PURPOSE: Newborns may undergo many kinds of minor procedures. Since painful events during procedures may cause adverse effects on growth and/or development, nonpharmacological way to relive pain have been extensively studied including oral glucose and pacifiers. This study was undertaken to evaluate the analgesic effects of orally administered glucose and pacifiers in term neonates during minor procedures using validated behavioral pain rating system. METHODS: Randomized prospective studies with 81 term infants were assigned to one of three treatment groups: placebo (2 ml sterile water), glucose (2 ml 30% glucose), and pacifier (2 ml 30% glucose followed by a pacifier) during heelsticks. Intensity of pain was assessed by Douleur Aigue Nouveau-ne scale (DAN scale). RESULTS: Median pain scores (interquartile) during minor procedure and after 30 seconds were 7 (6-10), 4 (3-8) for sterile water; 7 (3-8), 1 (0-5) for 30% glucose; 4 (2-6), 1 (0-1) for 30% glucose and pacifiers, respectively. P values for comparisons of sterile water versus 30% glucose, and 30% glucose versus 30% glucose plus pacifiers were 0.019, 0.035 for during the procedure and 0.006, 0.034 for after 30 seconds, respectively. CONCLUSION: The analgesic effect of oral glucose during minor procedure is clinically apparent in full term neonates. Pacifiers plus glucose have a synergetic analgesic effect in neonates. These simple and safe interventions should be widely used during minor procedures in term neonates.


Subject(s)
Humans , Infant , Infant, Newborn , Glucose , Pacifiers , Prospective Studies , Water
19.
Korean Journal of Anatomy ; : 457-462, 2003.
Article in English | WPRIM | ID: wpr-650743

ABSTRACT

The purpose of this study is to identify the differences of zebrin II expression between ataxic pogo and normal Balb/C mouse cerebellum. Zebrin II is expressed by subsets of Purkinje cells that form an array of parasagittal bands that extend rostrocaudally throughout the cerebellar cortex, separated by similar bands of Purkinje cells that do not express zebrin II. Zebrin II immunoreactivity was localized in the perikarya of Purkinje cells, and the dendrites. Distribution of zebrin II-immunoreactive Purkinje cells were very similar pattern in pogo and Balb/C mouse cerebellum. But, in the lobule III, distribution of zebrin II expression was different between pogo and Balb/C mouse cerebellum. In lobule III of Balb/c mouse cerebellum, 10~15 zebrin II-immunoreactive Purkinje cells were observed and clustered to form a parasagittal bands. On the other hand, zebrin II expressions of lobule III in pogo mouse cerebellum showed a little different patterns. In lobule III of pogo mouse cerebellum, three bilateral zebrin II immunoreactive parasagittal band were observed. P1 band was almost same with lobule III of Balb/C mouse cerebellum. But, P2 bands were composed of 50~60 Purkinje cells which were immunoreactive with zebrin II. These kind of thickening in zebrin II expression of pogo mouse cerebellum may be due to the genetical difference. Furthermore, these results may provide useful information with further ataxic pogo mice cerebellum studies.


Subject(s)
Animals , Mice , Cerebellar Cortex , Cerebellum , Dendrites , Hand , Immunohistochemistry , Purkinje Cells
20.
Pediatric Allergy and Respiratory Disease ; : 109-121, 2001.
Article in Korean | WPRIM | ID: wpr-60190

ABSTRACT

PURPOSE: Cytokines are known to play a major role in mediating many of the immunological and pathological findings of allergic disease. Many studies on allergen-specific T cell clones isolated from atopic individuals have shown that these cells produce cytokines of the Th2 phenotype. The purpose of the present study was to investigate cytokine patterns of T lymphocyte induced by Dermatophagoides pteronyssinus in atopic asthma. METHODS: Freshly isolated peripheral blood mononuclear cells of Dp(+) asthmatic children, Dp(-) asthmatic children, nonasthmatic children, and normal adults were stimulated with Dp antigen and examined for the induction of IFN-gamma, IL-2, IL-4, and IL-5 mRNA using RT-PCR. RESULTS: The lymphoproliferative response to crude Dp antigen was maximized at Dp concentration of 2.5 microgram/mL. The lymphoproliferative response was low in children including Dp(+) asthmatics, Dp(-) asthmatics and nonasthmatics, but that of normal adults was high. The expression of IFN-gamma was not so different in each group, but the expression of IL-4 was least in adults, and that of IL-5 was most prominent in Dp(+) asthmatic children. In adult healthy group, IFN-gamma expression without IL-4 and IFN-gamma without IL-5 was significantly higher than those in children. IL-5 expression with or without IFN-gamma was the highest in Dp(+) asthmatic children. IL-4 and IL-5 or IL-5 without IL-4 expression were observed more frequently in Dp(+) asthmatic children than in any other groups. Th2 response was most prominent in Dp(+) asthmatics, and Th1 response was most prominent in normal adult group. CONCLUSION: In conclusion, we observed significantly prominent Th2 response in Dp(+) asthmatic children, especially that of IL-5.


Subject(s)
Adult , Child , Humans , Asthma , Clone Cells , Cytokines , Dermatophagoides pteronyssinus , Gene Expression , Interleukin-2 , Interleukin-4 , Interleukin-5 , Lymphocytes , Negotiating , Phenotype , Pyroglyphidae , RNA, Messenger
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