1.A Case of Early Manifested Long-term Complications in MODY(Maturity Onset Diabetes in Young) Patient.
Journal of Korean Society of Pediatric Endocrinology 1997;2(2):263-267
A 14-year old boy presented with myoclonic seizure with rightward deviation of eyeballs. Three years ago, he was diagnosed as diabetes necessitating insulin injection. At that time, his blood glucose was 448mg/dl, HbA1c 27.8%, serum C-peptide rose from 0.4 to 1.1ng/ml after glucagon, and 24 hour urine C-peptide was 6.7microg/day. Eye examination was normal. His maternal grandmother died of diabetes at 50 years old, and his mother's sister and his elder sister had NIDDM with oral hypoglycemics. But, he didn't control hyperglycemia himself since that time. On physical exam, his grasping power was decreased in right hand, and cataract was detected at the posterior pole of lenses in both eyes requiring surgery. EEG showed partial seizure disorder in left frontoparietal area, and MRI revealed cerebral infarction in left frontoparietal cortex. Sensory-motor polyneuropathy was noted in nerve conduction velocity. His neurologic symptom was improved gradually with insulin therapy, but nerve conduction velocity and MRI abnormalities did not improved after 6 months of follow-up. Although long-term diabetic complication is common in poorly controlled diabetes, very early manifested eye and nervous system complications like this case is extremely uncommon.
Adolescent
;
Blood Glucose
;
C-Peptide
;
Cataract
;
Cerebral Infarction
;
Diabetes Complications
;
Diabetes Mellitus, Type 2
;
Electroencephalography
;
Epilepsies, Partial
;
Follow-Up Studies
;
Glucagon
;
Hand
;
Hand Strength
;
Humans
;
Hyperglycemia
;
Hypoglycemic Agents
;
Insulin
;
Magnetic Resonance Imaging
;
Male
;
Middle Aged
;
Nervous System
;
Neural Conduction
;
Neurologic Manifestations
;
Polyneuropathies
;
Seizures
;
Siblings
2.Seasonal or Gender Effects on Cord Serum Insulin-like Growth factor-I Concentrations in Newborn Infants.
Ran NAMGUNG ; Chul LEE ; Dong Gwan HAN
Korean Journal of Perinatology 1997;8(1):27-31
Possible seasonal differences in serum Insulin-Like Growth Factor-I (IGF-I) concentrations have not been studied in newborn infants. Recently we demonstrated sea- sonal differences in bone mineral content (BMC) in newborn infants: lower BMC was present in summer vs. winter-born infants (J Pediatr Gastroenterol Nutr 1992; 15: 285). In a second stduy, higher serum osteocalcin, an index of bone formation, and lower BMC were found in summer vs. winter (J Pediatr Gastroenterol Nutr 1994; 19: 2207). We speculated that increased serum osteocalcin in summer could be an adaptive response to decreased bone mass. Since growth factors such as IGFs are local regulators of bone formation, we hypothesized that in summer-born infants, serum IGF-I will be higher than in winter, associated with high bone formation activity. Fifty-nine healthy, term appropriate for gestational age (AGA) infants were studied prospectively in winter (Jan-Mar, 29) and in summer (July-Sept., 30). Thirty infants were male, and 29 infants were female. Gestational ages and birth weights were not different by season(in summer, mean+SD, 39.61.1 wk, 3,471360 g,' in winter, 39.31.4 wk, 3,402 392 g). Cord serum IGF-I was measured by radioimmunoassay, modified from Furlanetto et al (1977), after acidification and sep-pack extraction of serum, and osteocalcin concentrations were determined by a kit radio-immunoassay. Cord serum IGF-I concentrations were not different by season of birth(summer vs. winter, 20.11.83 vs. 16.5 1.75 ng/mL, p=0.2). No gender differences were found: 18.21.8 vs. 18.2+1.8 ng/ mL in males vs. females. Serum osteocalcin was higher in summer vs. winter-born infants (8.22.3 vs. 4.951.58 ng/mL, p=0.009). BMC was different by season (87.2+ 14.5 vs. 94.1+16.4 mg/cm, p=0.02). Cord serum IGF-I was not related to serum osteocalcin and BMC. We conclude that serum IGF-I concentrations are not different by season or gender, and are not related to bone formation activity and BMC. Thus, IGF -I concentrations in serum are not seasonally regulated, nor associated with an index of bone formation activity.
Birth Weight
;
Bone Density
;
Female
;
Gestational Age
;
Humans
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Infant
;
Infant, Newborn*
;
Insulin-Like Growth Factor I
;
Intercellular Signaling Peptides and Proteins
;
Male
;
Osteocalcin
;
Osteogenesis
;
Prospective Studies
;
Radioimmunoassay
;
Seasons*
3.Effect of gax gene on the expression of PCNA and CDK2 in vascular smooth muscle cells
Geng WANG ; Yaling HAN ; Boli RAN
Medical Journal of Chinese People's Liberation Army 1983;0(02):-
Objective To investigate the role of proliferating cell nuclear antigen (PCNA) and cyclin dependent kinase 2 (CDK2) in inhibition of vascular smooth muscle cells (VSMCs) proliferation by gax gene. Methods After being transfected by AdCMV-gax, the expression of gax, PCNA and CDK2 in VSMCs were assessed. The effect of gax overexpressions on VSMCs proliferation was observed by 3 H-thymidine incorporation. Results After AdCMV-gax was transfected to VSMCs, the level of Gax protein expression was significantly higher than that before transfection. PCNA and CDK2 expressions were decreased after VSMCs were transfacted with AdCMV-gax. The level of 3 H-thymidine incorporation was decreased significantly in with AdCMV-gax transfected VSMCs compared with that in non-transfected VSMCs. Conclusion The mechanism of inhibition of VSMCs proliferation by gax gene is related with depression of expression of PCNA and CDK2.
4.A clinical study on the efficacy of oral minirin in transsphenoidal surgery patients with central diabetes insipidus
Qi DONG ; Ran YI ; Fengping HAN
Clinical Medicine of China 2010;26(6):631-632
Objective To observe the effect of oral minirin in postoperative transsphenoidal surgery patients with central diabetes insipidus. Methods The changes in the urine volume and osomlality after two weeks of minirin medication (0. lmg, 3 times each day) were observed in 34 patients with central diabetes insipidus underwent transsphenoidal surgery. Results After two weeks of minirin therapy,the average daily urine volume decreased from 7985.40 ±410. 36 ml to 2277. 87 ± 328. 94 ml,and the average plasmas osmolarity increased from 301. 68 ± 3. 59 ml/d to 313. 26 ±4. 87 mOsm/ kg. No adverse reaction was observed during the therapy. Conclusions Minirin is effective and safe in the therapy of postoperative transsphenoidal surgery patients with central diabetes insipidus.
5.Protective effect of atorvastatin on radiation-induced endothelial cell injury
Xinze RAN ; Huaien ZHENG ; Fengchao WANG ; Xi RAN ; Aiping WANG ; Jing HAN ; Yanqi ZHANG ; Jun CHEN
Chinese Journal of Radiological Medicine and Protection 2009;29(2):129-132
Objective To explore the protective effect of atorvastatin on irradiated endothelium and the thrombomodulin(TM)expression.Methods Cultured human coronary artery endothelial cells(HCAEC)and human umbilical vein endothelial cells(HUVEC)were treated by atorvastatin at the final concentration of 10 μmol/ml for 10 min,and then irradiated with 2 and 25 Gy.Cell cycles status and TM expression were quantitatively measured by flow cytometry 24 hours after irradiation.Protein C activation in endothelial cells was also assessod.Results After administration with atorvastatin for 24 h,the TM expression increased by 77%,59% and 61% in normal control group,2 Gy group and 25 Gy group,respectively(t=27.395,26.420,58.065;P=0.000).The protein C levels decreased by 23% and 34% compared with the normal group post-irradiation to 2 and 25 Gy,but increased by 79% and 76% compared with the irradiated control group after administration with atorvastatin.The rates of cell apoptosis decreased by 6% and 16% in 2 Gy and 25 Gy groups,respectively after administration with atorvastatin for 24 h(t=4.178,17.863;P=0.000).Conclusions Atorva statin can protect endothelia cell from irradiation-induced apeptosis by increasing TM expression and protein C activation.
6.Microvessel Quantification, Expression of p53 Protein and MIB-1 in Colorectal Adenoma and Carcinoma.
Tae Jung JANG ; Jung Ran KIM ; Han Ik BAE
Korean Journal of Pathology 1997;31(1):40-50
Angiogenesis is a crucial step in tumor growth and progression. Scarce data is available on angiogensis in gastrointestinal tumors. We studied 16 normal colon, 44 adenomas and 29 carcinomas to evaluate angiogenesis in colorectal tumors and to assess the correlation among p53 protein, proliferative activity and other clinical prognostic parameters. Endothelial cells were immunostained with an anti-Factor VIII mAb; in each case three microscopic fields(x 200) were counted: average number of the three fields was defined as microvessel density (MVD). p53 protein expression was 45.5%(20/44) in adenomas, and 79.3%(23/29) in carcinomas (p<0.01). p53 protein expression of carcinomas was 57.1%(4/7) in diploid tumors, 100%(8/8) in aneuploid tumors (p=0.07), 100%(8/8) in well differentiated tumors, and 50%(2/4) in poorly differentiated tumors (p=0.09). MIB-1 score was 2.3+/-0.7(38) in adenomas, 3.4+/-0.5(29) in carcinomas (p<0.01). There was no significant correlation between p53 protein and MIB-1 score. MVD was 10.4+/-4.1(16) in the normal mucosa, 21.5+/-7.9(39) in the adenomas, 35.3+/-9.7(26) in carcinomas (normal versus adenomas, p<0.01; adenomas versus carcinomas, p<0.01). MVD was 25.8+/-5.4(2) in carcinomas confined to mucosa, and 36.1+/-9.6(24) in carcinomas with transmural invasion. The higher MIB-1 score was in carcinomas the more MVD increased but there was no statistical significance (r=0.38, p=0.055). MVD of carcinomas was not associated with nodal metastasis, p53 expression, and DNA ploidy. p53 protein and MIB-1 expression are useful methods for the evaluation of malignancy, and tumor angiogenesis is an early event in a colorectal tumor but MVD does not correlate with prognostic parameters except for the tumor depth.
Adenoma*
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Aneuploidy
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Colon
;
Colorectal Neoplasms
;
Diploidy
;
DNA
;
Endothelial Cells
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Microvessels*
;
Mucous Membrane
;
Neoplasm Metastasis
;
Ploidies
7.Postnatal Weight Curve in Low Birth Weight(Appropriate for Gestational Age) Infants.
Kyoung Mi PARK ; Ran NAMGUNG ; Chul LEE ; Dong Gwan HAN
Journal of the Korean Pediatric Society 1989;32(5):606-619
No abstract available.
Humans
;
Infant*
;
Parturition*
8.Clinical and Ultrasonographic Study on Prenatal Brain Damage inNewborn Infants.
Kook In PARK ; Dong Gwan HAN ; Ran NAMGUNG ; Chul LEE
Journal of the Korean Pediatric Society 1994;37(10):1364-1375
To determine the incidence of prenatal brain damage, and evaluate the clinical and neurosonographical characteristics, we prospectively examined 508 newborn infants with intracranial ultrasound within the first day of life who admitted to the NICU of Severance Hospital from June 1990 to January 1992 and reviewed maternal or neonatal medical records. We found 12 cases (2.4%) of fetal brain lesions and ten of which had antenatal periventricularintraventricular hemorrhage and posthemorrhagic hydrocephalus. One of 10 infants had focal parenchymal hemorrhage, 1 had diffuse parenchymal hemorrhage with a porencephalic cavity, 1 had multicystic periventricular leukomalacia with spongiform cerebromalacia, and 1 had multicystic periventricular leukomalacia. Another 2 infants showed multicystic periventricular leukomalcia and multicystic encephalomalacia with ventriculomegaly respectively. Of 12 infants with prenatal brain damage, 7 were full-term, 5 were preterm, 9 were appropriate-for-gestational age, 2 were small-for-gestational-age, 7 were male, and 9 were delivered vaginally. Ten of 12 infants had perinatal asphyxia and five of which showed severe asphxia. Ten of 12 cases had significant materanl histories (three of which had preterm labor, three had premature rupture of amniotic membrane, one had preeclampsia, one had frequent upper respiratory tract infection and influenza, one had herb medication, and one had mental retardation). Only one infant with prenatal brain damage was asymptomatic and ll infants exhibited a few clinical signs during the neonatal period (all 5 infants had respiratory distress symptom, 4 infants had multiple congenital anomalies, 2 infants showed janudice and one infant had seizure). Of 9 infants who were taken electroencephalogram, 7 infants showed abnormal findings and four of 9 infants taken brainstem auditory evoked potential test exhibited abnormal response. Cerebral palsy and mental retardation were documented in two infants, 5 infants were lost on follow-up examination, and 5 infants were discharged against doctor's advices and died. This study confirms that some drain damage is prenatal and these lesions are associated with the development of cerebral palsy. therefore, prenatal brain damage can not be attributed to obstetrical events and neonatal care, We recommend that a fetal neurosonographic examination should be done in the last trimester of all pregnancies, especially in the presence of significant obstetric history or suspected fetal malformations and neonatal brain sonogaraphy be done within the first week of life. These examination are justified because they would allow early intervention to help offset possible neurologic deficits, would help prepare parents and pediatricians for possible limitations, and would prevent lawsuits and protect against malpractice allegations. But, it is not clear that every newborn infants need an ultrasound scan, since detection of prenatal brain damage would be of little benefit to the patients and enormous cost of routinely examining all pregnancies would be required.
Amnion
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Asphyxia
;
Brain*
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Cerebral Palsy
;
Early Intervention (Education)
;
Electroencephalography
;
Encephalomalacia
;
Evoked Potentials, Auditory, Brain Stem
;
Female
;
Follow-Up Studies
;
Hemorrhage
;
Humans
;
Hydrocephalus
;
Incidence
;
Infant*
;
Infant, Newborn
;
Influenza, Human
;
Intellectual Disability
;
Leukomalacia, Periventricular
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Male
;
Malpractice
;
Medical Records
;
Neurologic Manifestations
;
Obstetric Labor, Premature
;
Parents
;
Pre-Eclampsia
;
Pregnancy
;
Pregnancy Trimester, Third
;
Prospective Studies
;
Respiratory Tract Infections
;
Rupture
;
Ultrasonography
9.Exogenous Surfactant Replacement Therapy of Hyaline Membrane Disease: A controlled clinical trial.
Ran NAMGUNG ; Chul LEE ; Kook In PARK ; Dong Gwan HAN
Journal of the Korean Pediatric Society 1990;33(1):22-35
No abstract available.
Humans
;
Hyalin*
;
Hyaline Membrane Disease*
;
Infant, Newborn
10.Clinical Observation on Guillain-Barre Syndrome in Children.
Byung Ran YUN ; Hye Kyung HAN ; Yong Seung HWANG
Journal of the Korean Pediatric Society 1989;32(1):52-61
No abstract available.
Child*
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Guillain-Barre Syndrome*
;
Humans