Comparison of the Incidences of Neonatal Morbidities by Different Criteria of Histologic Chorioamnionitis in Extremely Low Gestational Age Newborns

PURPOSE: The objective of the study was to compare the incidences of the neonatal morbidity, using the various criteria of histologic chorioamnionitis (HC) in the extremely low gestational age newborns (ELGANs), who were born before the 28 weeks of gestational age. METHODS: Sixty two ELGANs who were born and admitted to the Seoul National University Bundang Hospital from 2007 to 2011 were included. A total of seven criteria of HC were made according to the site within the placenta and the extent of the neutrophil infiltration. The incidence of the major neonatal morbiditiy was compared between the HC-exposed and the HC-unexposed ELGANs according to each criterion of HC. RESULTS: There were no significant differences in the birth weight, gestational age, and sex between the HC-exposed and the HC-unexposed ELGANs by any criteria. The incidence of respiratory distress syndrome (RDS) was significantly lower in the HC-exposed ELGANs only in the criterion of any extent of the umbilical cord inflammation (funisitis). The incidence of retinopathy of prematurity (ROP) was significantly higher in the HC-exposed ELGANs in the criterion of any extent of amnionitis or funisitis. The incidences of other major morbidities did not differ between the HC-exposed and the HC-unexposed ELGANs by any criteria. CONCLUSION: The presence of funisitis reflected the lung maturation effect of chorioamnionitis, as indicated by the decreased incidence of RDS. Among other major neonatal morbidities, only ROP was significantly associated with HC. The ROP occurred more frequently in the funisitis or amnionitis-exposed ELGANs.

The Time When the Metabolic Compensation for Hypercapnia Begin to Occur in Very Low Birth Weight Infants

PURPOSE: Lung injury from mechanical ventilation is one of the major pathogenetic factors of bronchopulmonary dysplasia. Permissive hypercapnia (PH) is one of the strategies for reducing lung injury. However, PH is frequently infeasible in very low birth weight infants (VLBWI) due to their immature renal compensation for respiratory acidosis. The purpose of this study was to identify time when metabolic compensation for hypercapnia begin to occur in VLBWIs. METHODS: Data were retrospectively collected from 82 VLBWI who were admitted to Seoul National University Bundang Hospital from January 2011 to December 2012. The postnatal day when the difference between actual bicarbonate and expected bicarbonate levels became less than 2.0 mmol/L consistently for the first time under hypercapnea (>40 mmHg) was defined as the time when metabolic compensation for hypercapnea occurred. RESULTS: Metabolic compensation for hypercapnea occurred on 9.1+/-3.9 postnatal day. The younger the gestational age (GA) was and the smaller the birth weight was, the later metabolic compensation for hypercapnea occurred. Late metabolic compensators (> or =9 days) were significantly younger in GA (P=0.001), lighter at birth (P=0.041), intubated longer (P=0.002), and less frequently afflicted with respiratory distress syndrome (P=0.036) compared to early metabolic compensators (<9 days). However, logistic regression analysis revealed only young GA was associated with late metabolic compensation with marginal significance (P=0.068). CONCLUSION: Metabolic compensation for hypercapnea occurred 9 days after birth on average. PH strategy for reducing lung injury should be considered after renal metabolic compensation for hypercapnea occurs in VLBWI.

The Clinical Characteristics and the Neurodevelopmental Outcomes of the Neonates with Cerebellar Hemorrhage

PURPOSE: We present our experience of 14 neonates with cerebellar hemorrhage and describe the clinical characteristics and the neurodevelopmental outcomes. METHODS: Fourteen neonates were identified as having cerebellar hemorrhage, based on the brain MRI findings at the two university hospitals from January 2007 to July 2011. Twelve preterm infants with the brain MRI taken before the discharge, and 2 term infants having taken the brain MRI with birth asphyxia were enrolled. The electronic medical records of the infants were reviewed. RESULTS: Ten (71.4%) infants were found to have multifocal or lobar cerebellar hemorrhage involving both hemispheres. Three infants had unilateral lesions in the right hemisphere, and one infant had a left hemisphere lesion. Six infants (42%) had co-existing high grade IVH (> or =grade III); 6 infants (42%) had periventricular leukomalacia (PVL); and 6 infants (42%) had posthemorrhagic hydrocephalus (PHH). After discharge, 12 infants (85.7%) were followed at the outpatient clinics for at least 6 months. Two out of 9 infants (22.2%) with other neurological co-morbidities (IVH> or =grade III, PVL, or PHH) had cerebral palsy, and 5 infants (55.6%) had developmental delay assessed by the Bayley scales. None of the infants without other neurological co-morbidities had cerebral palsy or developmental delay. CONCLUSION: Neonatal cerebellar hemorrhage is often diagnosed incidentally on the brain MRI in the preterm infants. If there is no concomitant high-grade IVH, PVL or PHH, cerebellar hemorrhage seems to hardly accompany its own specific symptoms, and the neurodevelopmental outcomes seem to be favorable as well. However, further long-term, large scale studies are necessary.

The Significance of Maturation Score of Brain Magnetic Resonance Imaging in Extremely Low Birth Weight Infant

PURPOSE: The aim of this study was to investigate the effect of perinatal risk factors on brain maturation and the relationship of brain maturation and neurodevelopmental outcomes with brain maturation scoring system in brain MRI. METHODS: ELBWI infants born at the Seoul National University Children's Hospital from January 2006 to December 2010 were included. A retrospective analysis was performed with their medical record and brain MR images acquired at near full term. We read brain MRI and measured maturity with total maturation score (TMS). TMS is a previously developed anatomic scoring system to assess brain maturity. The total maturation score was used to evaluate the four parameters of maturity: (1) myelination, (2) cortical infolding, (3) involution of glial cell migration bands, and (4) presence of germinal matrix tissue. RESULTS: Images from 124 infants were evaluated. Their mean gestational age at birth was 27.1+/-2.1 weeks, and mean birth weight was 781.5+/-143.9 g. The mean TMS was 10.8+/-2.0. TMS was significantly related to the postmenstrual age (PMA) of the infant, increasing with advancing postmenstrual age (P<0.001). TMS showed no significance with neurodevelopmental delay, and with brain injury, respectively. CONCLUSION: TMS was developed for evaluating brain maturation in conventional brain MRI. The results of this study suggest that TMS was not useful for predicting neurodevelopmental delay, but further studies are needed to make standard score for each PMA and to re-evaluate the relationship between brain maturation and neurodevelopmental delay.

The relationship between Gly71Arg and TATA box polymorphism of UGT1A1 gene and prolonged hyperbilirubinemia of breast milk feeding infant in Korean / 소아과

PURPOSE: It has been known that breast milk cause prolonged unconjugated hyperbilirubinemia. UGT1A1 is a important gene of uridine diphosphate glucuronosyltransferase (UGT) which has a major role of bilirubin metabolism. These findings suggest that there is a relationship between UGT1A1 gene mutation and prolonged jaundice of breast feeding infant. The aim of study was to investigate whether a polymorphism of the UGT1A1 gene exist in prolonged hyperbilirubinemia of breast milk feeding Korean infant. METHODS: The genomic DNA was isolated from 50 full term Korean neonates, who had greater than a 10 mg/dL of serem bilirubin after 2 weeks of birth with no significant cause, and the other genomic DNA was isolated from 162 full term Korean neonates of the control population. Both group fed breast milk. We performed direct sequencing of TATA box and Gly71Arg polymorphism of the UGT1A1 gene. RESULTS: Two of the 50 neonates with hyperbilirubinemia had AA polymorphism, and 40 had GA polymorphism. Five of the 129 neonates of the control group had AA polymorphism, and 4 had GA polymorphism. The allele frequency of G>A polymorphism in the hyperbilirubinemia group was 44.0%; it was significantly higher than 5.4% of the control group. TATA box polymorpism was not different both group significantly. CONCLUSION: Our result indicated that Gly71Arg polymorphism is associated with the prolonged hyperbilirubinemia of breast milk-feeding infant in Korean, while TATA box polymorphism is not associated with the prolonged hyperbilirubinemia of breast milk-feeding infant in Korean.

The relation between angiotensin converting enzyme (ACE) gene polymorphism and neonatal hyperbilirubinemia in Korea / 소아과

PURPOSE: Human angiotensin converting enzyme (ACE) gene shows an insertion/deletion polymorphism in 16 intron, and three genotypes are determined by whether a 287 bp fragment of the DNA is present or not; II, ID and DD genotype. DD genotype has been suggested as a risk factor of chronic nephrotic disease such as IgA nephropathy and diabetic nephropathy, various cardiovascular diseases and several other diseases. ACE activity increases in acute hepatitis, chronic persistent hepatitis, chronic active hepatitis and cirrhosis. On the other hand, patients with fatty livers have normal ACE activity. This study was designed to find out the relation between polymorphsims of the ACE genes and neonatal hyperbilirubinemia in Koreans. METHODS: The genomic DNA was isolated from 110 full-term Korean neonates who had hyperbilirubinemia with no obvious causes (serum bilirubin?12 mg/dL) and 164 neonates of a control population (serum bilirubin?12 mg/dL). We performed polymerase chain reaction (PCR) to see the allele of the ACE gene. Electrophoresis was done in the PCR products in 1.5 percent agarose gel, and then DNA patterns were directly visualized under ethidium bromide staining. RESULTS: ACE genotypes in the hyperbilirubinemia group are as follows; 26.36 percent for II, 53.64 percent for ID, 20.00 percent for DD, 0.532 for I allele and 0.468 for D allele. These distributions were not significantly different from those in the control group; 24.39 percent for II, 51.83 percent for DI, 23.78 percent for DD, 0.503 for I allele and 0.497 for D allele. CONCLUSION: In this study, ACE gene polymorphism was detected in the neonatal hyperbilirubinemia and control group. The most frequent genotype was ID. Our results indicate that the ACE gene polymorphism is not associated with the prevalence of neonatal hyperbilirubinemia in Koreans.

A case of congenital neurocutaneous melanosis / 소아과

Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi and benign pigment cell tumors of the leptomeninges. Neurocutaneous melanosis is thought to represent an error in the morphogenesis of embryonal neuroectoderm. We experienced a neonate who presented with giant, dark colored pigmented nevi covering chest, abdomen, neck and arms, with satellite lesions. Magnetic resonance image showed a nodular hyperintense lesion in the amygdala of the right temporal lobe, and T1-weighted images showed hyperintensities in the adjacent leptomeninges. We report a rare case of neurocutaneous melanosis with a brief review of related literature.

The Effect of Reoperation on the Patency Rate of Arteriovenous Fistula for Hemodialysis

PURPOSE: We designed this study to find out the risk factors affecting the patency rate after creation of an arteriovenous fistula (AVF) for hemodialysis. METHODS: Between March 1997 and December 2001, a total of 397 AVF operations in 314 patients were performed by a single surgeon using the same surgical technique and principles. One hundred and forty cases (35.3%) were reoperations due to previous AVF failure. Artificial vessels (=PTFE, polytetrafluoroethylene) were used for the AVF in 39 cases (9.8%). The sex, age, diabetes mellitus, previous operation history and type of dialysis center were considered as a possible risk factors affecting the patency rate. RESULTS: After a mean follow-up of 18.3 months, there were 63 cases of patient death, 15 of kidney transplantation, and 104 of AVF failure identified. The overall patency rates of 1, 2 and 3 year were 76.2, 70.9, and 66.9%, respectively. From the multivariate analysis, the previous operation history was the only significant factor affecting the patency rate of AVF (P=0.001, odd ratio=2.58). Although the diabetic patient (odd ratio=1.43) and artificial vessel groups (odd ratio=1.678) showed high odd ratios, these factors were not statistically significant (P>0.05). From the univariate analysis, the reoperation cases also showed a significantly inferior patency rate compared to the first operation cases (1 year patency rate was 65.2% and 83.7%, P=0.001). However, the diabetes and types of vessel showed no significant differences in patency rates. CONCLUSION: A reoperation of an AVF creation was the most significant risk factor affecting the patency rate after its original creation. The first attempt AVF creations showed-favorable patency rates. To improve the overall patency rate of an AVF creation, a more delicate preoperative study and accurate surgical technique will be required at the time of the first AVF operation.

Effects of Cyclosporin A and Mycophenolate Mofetil on Expression of Transforming Growth Factor-beta 1 and Endothelin-1 Following Renal Ischemia-reperfusion in Rats / 대한신장학회잡지

PURPOSE: Nephrotoxicity of cyclosporin A (CsA) remains a major obstacle for the clinical use of this potent immunosuppressant. It is likely that the transforming growth factor-beta(TGF-beta) and endothelin-1 (ET-1) play a central role in initiation and/or progression of CsA induced nephropathy in renal ischemia-reperfusion injury. It is proposed that the new immunosuppressive drug mycophenolate mofetil (MMF) reduces the incidence of acute rejection in comparison with azathioprine. Thus, the aim of the present study was to analyze the expression of TGF-beta and ET-1 in renal ischemia-reperfusion injured rats and to see the effect of CsA or MMF on the expression of these mediators. Effects of coadministration of CsA and MMF were also evaluated. METHODS: Sprague-Dawley rats (N=60) performed right nephrectomy were classified into five groups according to experimental methods. Control group underwent right nephrectomy. After the right nephrectomy, to induce renal ischemia, the left renal vascular pedicle was occluded for 30 minutes with vascular clamps in all experimental groups. After 30 minutes, the clamps were removed to undergo reperfusion. In control group, ischemic injury wasn't done. CsA group was administered CsA (10 mg/kg/ day, S.C) after the operation. CsA and MMF group was coadministered CsA (10 mg/kg/day, S.C) and MMF (10 mg/kg/day, P.O). MMF group was administered MMF (10 mg/kg/day, P.O). After 7 days, the left kidney was removed and processed for histological, immunohistochemical, immunofluorescent and molecular analyses for TGF-beta 1 and histological, immunohistochemical, immunofluorescent analyses for ET-1. RESULTS: The immunohistochemical and immunofluorescent expression density for TGF-beta1 in CsA group was higher than control and other experimental groups. The immunohistochemical and immunofluorescent expression density and mRNA production for ET-1 in CsA group was higher than control and other experimental groups. The expression density for TGF-beta1 and ET-1 in CsA and MMF group was less than in CsA group. The expression density for TGF-beta 1 and ET-1 in MMF group was less than in CsA group and CsA and MMF group, was similar to ischemia-reperfusion group. CONCLUSION: These results suggest that MMF does not seem to have nephrotoxic effects, and seems to have a reno-protective effect from CsA induced nephrotoxicity in the ischemia-reperfusion model. The present study might partially explain that MMF is going to contribute to the improvement of the survival rate of the transplanted kidney associated with acute and/or chronic rejection and to the reduction of CsA dosage and its complications.

Pneumatosis Intestinalis Associated with Immune-suppressive Agents in a Case of Minimal Change Disease

We report treatment of a 38-year-old man with minimal change disease (MCD) who developed pneumatosis intestinalis (PI) during administration of immune-suppressive agents. His immunosuppressive medication had been tapered to 15 mg/day of prednisolone. MCD was steroid-resistant type. Abdominal examination and laboratory studies were not clinically remarkable. Radiologic findings were consistent with PI. Abnormal air accumulation was noted in the bowel, peritoneum, mediastinum and retroperitoneum. Conservative therapy with oxygen and metronidazole improved the PI symptoms. In 1993, a case of PI with nephrotic syndrome following steroid treatment was reported in Japan. However this is only the second case reported in the literature, and the first in English.

A Study of the Dialysis Associated Pericarditis in Patients on Undergoing Maintenance Hemodialysis / 대한신장학회잡지

BACKGROUND: The accurate pathogenetic mechanism of dialysis associated pericarditis remain uncertain, but its clinical significance is very important from a therapeutic and prognostic point of view. Clinical features of the disease weren't reported well in Korea. So, we analyzed the clinical characteristics of the dialysis associated pericarditis. METHODS: Thirty-five patients were included in this study. The study group was divided into two groups (a minimal group and moderate to large group) according to the amount of the pericardial effusion on echocardiogram. We reviewed and compared the clinical appearance, laboratory data and echocardiogram findings. RESULTS: There were 18 cases (51.4%) of minimal amount pericardial effusion and 17 cases (48.5%) of moderate to large amount. Cardiac tamponade developed in 2 patients. There was a significant difference in urea reduction rate between minimal group (42+/-20.%) and moderate to large group (24.3+/-21.2%) (p<0.05). A time to pericardial effusion development from dialysis initiation was 3.6+/-8.3 months, 17.6+/-21.0 months, respectively and the difference showed statistical significance (p<0.05). The Left ventricular dimension at systole was 4.8+/-0.7 cm, 3.8+/-1.1 cm, respectively. This difference is statistically significant (p<0.05). In the minimal amount group, 16 (89.9%) cases were treated conservatively and 2 cases (10.1%) were treated surgically. In the moderate to large amount group, 10 cases (58.8%) were treated surgically. CONCLUSION: These results show that the dialysis associated pericarditis with moderate to large amount of pericardial effusion have lesser urea reduction rate, longer dialysis treatment period and require further surgical approach than minimal amount group. We suggest that the sufficient and adequate dialysis and the regular echocardiography are needed. This need is more eminent as the duration of dialysis become longer. It is possible to prevent the development of pericarditis and its complication.

A Study of the Dialysis Associated Pericarditis in Patients on Undergoing Maintenance Hemodialysis / 대한신장학회잡지

BACKGROUND: The accurate pathogenetic mechanism of dialysis associated pericarditis remain uncertain, but its clinical significance is very important from a therapeutic and prognostic point of view. Clinical features of the disease weren't reported well in Korea. So, we analyzed the clinical characteristics of the dialysis associated pericarditis. METHODS: Thirty-five patients were included in this study. The study group was divided into two groups (a minimal group and moderate to large group) according to the amount of the pericardial effusion on echocardiogram. We reviewed and compared the clinical appearance, laboratory data and echocardiogram findings. RESULTS: There were 18 cases (51.4%) of minimal amount pericardial effusion and 17 cases (48.5%) of moderate to large amount. Cardiac tamponade developed in 2 patients. There was a significant difference in urea reduction rate between minimal group (42+/-20.%) and moderate to large group (24.3+/-21.2%) (p<0.05). A time to pericardial effusion development from dialysis initiation was 3.6+/-8.3 months, 17.6+/-21.0 months, respectively and the difference showed statistical significance (p<0.05). The Left ventricular dimension at systole was 4.8+/-0.7 cm, 3.8+/-1.1 cm, respectively. This difference is statistically significant (p<0.05). In the minimal amount group, 16 (89.9%) cases were treated conservatively and 2 cases (10.1%) were treated surgically. In the moderate to large amount group, 10 cases (58.8%) were treated surgically. CONCLUSION: These results show that the dialysis associated pericarditis with moderate to large amount of pericardial effusion have lesser urea reduction rate, longer dialysis treatment period and require further surgical approach than minimal amount group. We suggest that the sufficient and adequate dialysis and the regular echocardiography are needed. This need is more eminent as the duration of dialysis become longer. It is possible to prevent the development of pericarditis and its complication.

A Case of 46 XX Male Syndrome / 대한내분비학회지

The 46, XX male syndrome is rare disease that is characterized by a phenotypic male who has a 46, XX female karyotype. Since the first report by de la Chapelle and associates in 1964, several cases have been reported, but it is still a rare entity. Recently we examined a 20-year-old XX male who had the symptoms of gynecomastia, an infantile appearance of the external genitalia, scanty pubic hair, no Adams apple, and no axillary hair. We presently describe a patient with the 46, XX male syndrome who showed a 46, XX karyotype on chromosomal study and review the literatures.

The Significance of Monocyte Chemoattractant Protein-1 and Interleukin-6 in Maintenance Hemodialysis Patients with Arteriovenous Fistula Obstruction / 대한신장학회잡지

Dysfunction of vascular access remains a leading cause of morbidity in patients receiving maintenance hemodialysis, accounting for 10-25% of their hospitalization. The main cause of vascular access dysfunction is thrombosis which is related to venous stenosis. The pathogenetic mechanism of venous stenosis includes cytokines, coagulation factors, growth factors and adhesion molecules. Two such cytokines, monocyte chemoattractant protein-1(MCP-1) and interleukin-6(IL-6), are known to be important in vascular smooth muscle cell proliferation. MCP-1 secreted by vascular endothelial cells, suppresses vascular smooth muscle cell proliferation. In contrast, IL-6 is secreted by monocytes, and it stimulates vascular smooth muscle cell proliferation. The followings are the results of our investigation into the plasma levels of MCP-1 and IL-6, as well as the clinical features of 70 patients currently receiving hemodialysis. 1) The patients were divided into two groups, a group with a history of vascular access obstruction (obstructed group) and a group with no such history (non-obstructed group). The mean age of the patients in the obstructed group was 48.1+/-14.2 years and that of the non-obstructed group, 53.2+/-13.7 yerars. The male-to-female ratios were 12 : 8 and 25 : 25 respectively. The average number of obstructions was 1.5+/-0.7. 2) The average duration of dialysis treatment was 17.2+/-42.2 months for the obstructed group and 28.5+/-35.2 months for the non-obstructed group. The dosage of heparin and erythropoietin given during dialysis, the amount of ultrafiltration, average blood pressure, hematocrit, platelet count, and the levels of albumin, total cholesterol, triglyceride, calcium, phosphorus, glucose, iPTH and creatinine were analysed, but there was no meaningful statistical difference between the two groups, There was, however, a statistically significant(p<0.05) difference in the levels of HDL-cholesterol between the two groups : In the obstructed group, an average level of HDL-cholesterol was 34.4+/-8.5 mg/dL whereas in the non-obstructed group, was 41.3+/-13.7 mg/dL. 3) The obstructed group had a MCP-1 level of 403.2+/-233.9 pg/mL and the non-obstructed group had a level of 271.8+/-124.1pg/ml, which is a meaningful statistical difference(p<0.01). Although the IL- 6 level was 10.3+/-24.3 pg/mL in the obstructed group and 5.0+/-8.1 pg/mL in the non-obstructed group, there is not significant difference because the distri bution of the sample values was widely scattered. In conclusion, MCP-1 levels showed higher levels in the group that had a history of vascular access obstruction perhaps as a result of over-secretion stimulated by A-V fistula obstruction. The higher IL-6 levels(without statistical meaning) in the same group may have relevance to vasclar access obstruction, but additional studies with a larger population are needed to clarify any relationship.

A Giant Hydronephrosis in Adult / 대한신장학회잡지

The term of giant hydronephrosis in adults, is applied when the collecting system contains more than 1,000 mL of fluid. The commonest cause of asymptomatic giant hydronephrosis in early adult life is congenital ureteropelvic stenosis. Giant hydronephrosis in adults is uncommon and often clinically misdiagnosed, because clinical characteristics are vague and generalized symptom. We presented a giant hydronephrosis associated with ureter stricture on 31 years old male.

Two cases of silicone- induced pulmonary embolism / 결핵

No abstract available.