[show abstract][hide abstract] ABSTRACT: Purpose: We tested whether rosiglitazone (RGZ), a peroxisome proliferator-activated receptor-γ agonist, can restore alveolar development and vascular growth in a rat model of bronchopulmonary dysplasia (BPD). Materials and Methods: A rat model of BPD was induced through intra-amniotic delivery of lipopolysaccharide (LPS) and postnatal hyperoxia (80% for 7 days). RGZ (3 mg/kg/d, i.p.) or vehicle was given daily to rat pups for 14 days. This model included four experimental groups: No BPD+vehicle (V), No BPD+RGZ, BPD+V, and BPD+RGZ. On D14, alveolarization, lung vascular density, and right ventricular hypertrophy (RVH) were evaluated. Results: Morphometric analysis revealed that the BPD+RGZ group had significantly smaller and more complex airspaces and larger alveolar surface area than the BPD+V group. The BPD+RGZ group had significantly greater pulmonary vascular density than the BPD+V group. Western blot analysis revealed that significantly decreased levels of vascular endothelial growth factor (VEGF) and its receptor VEGFR-2 by the combined exposure to intra-amniotic LPS and postnatal hyperoxia were restored by the RGZ treatment. RVH was significantly lesser in the BPD+RGZ group than in the BPD+V group. Conclusion: These results suggest that RGZ can restore alveolar and pulmonary vascular development and lessen pulmonary hypertension in a rat model of BPD.
Yonsei medical journal 01/2014; 55(1):99-106. · 0.77 Impact Factor
[show abstract][hide abstract] ABSTRACT: Background
For premature infants with advanced acute abdomen, creating a temporary enterostomy is believed to be an appropriate surgical management. However, there is no consensus regarding the timing of enterostomy reversal. The aim of this study was to determine the optimal timing for enterostomy closure (EC) by analyzing EC-related complications.
This was a retrospective study of preterm infants who underwent enterostomy for suspected acute abdomens and subsequent closure.
EC-related complications occurred in 35 of 54 infants (65%). A univariate analysis determined the following risk factors for EC-related complications: lower weight and younger age at the time of EC and a shorter stoma duration. In a multiple logistic regression analysis, the only significant risk factor was a weight under 2660 g at the time of the closure operation. Infants with EC-related complications were ventilated longer, were administered more vasopressors, and were more likely to undergo reoperation. Additionally, these infants required parenteral nutrition for a longer duration, had a longer length of hospital stay after EC, and had a significantly lower weight and height at a corrected age of 7–10 months than infants without EC-related complications.
Body weight may be one of the most important factors to consider for minimizing EC-related complications.
[show abstract][hide abstract] ABSTRACT: Amplitude-integrated electroencephalography (aEEG) allows continuous brain function monitoring at bedside.
This prospective cohort study was designed to longitudinally evaluate aEEG tracings at increased postmenstrual age (PMA) in preterm infants with intraventricular hemorrhage (IVH).
Biweekly aEEG recordings were performed on preterm infants <32weeks gestational age from 24 to 36weeks PMA. The tracings were evaluated according to a scoring system adapted from Burdjalov et al.
We analyzed 496 aEEG recordings in 105 preterm infants. The control group consisted of 42 infants with no IVH, whereas the IVH grade I, II, III, and IV groups consisted of 38, 8, 3, and 14 infants, respectively. There were significant differences in the cycling and total maturation scores among the IVH groups at 36weeks PMA (p=0.010 and p=0.006, respectively). The IVH-IV patients maintained low scores in their cycling as their PMA increased, in contrast to their continuity and amplitude scores. The risk factors affecting the aEEG maturation scores at 36weeks PMA in the IVH-IV patients included seizure events with the administration of antiepileptic drugs and the insertion of external ventricular drains (β=-0.679 and β=-0.418, respectively; p=0.003).
The low cycling scores persisted until 36weeks PMA in the IVH-IV group.
Early human development 09/2013; · 2.12 Impact Factor
[show abstract][hide abstract] ABSTRACT: Abstract Aim: We investigated the combined effects of intra-amniotic lipopolysaccharide (LPS) and maternal betamethasone (BMZ) on alveolarization using a newborn rat model. Methods: LPS (1.0 μg/sac) or vehicle was injected into the amniotic sacs of pregnant rats and BMZ (170 μg/kg) or vehicle was injected intramuscularly into the pregnant rats twice at 8-h intervals on gestation day 20. The rat pups were delivered spontaneously after 2-2.5 days and raised until the measurements were taken. Bronchoalveolar lavage was performed on days 2 and 5, and morphometric analyses of the lungs were performed on days 5 and 14. Results: Intra-amniotic LPS significantly increased the neutrophils in the bronchoalveolar lavage fluid (BALF) on day 2, but double exposure to LPS and BMZ significantly alleviated the neutrophil increase in the BALF. On day 5, while the neutrophils in the BALF decreased in the animals exposed to LPS alone, the neutrophil numbers in the BALF were steady in the animals exposed to both LPS and BMZ. Morphometric analyses on days 5 and 14 revealed a significant disruption of alveolarization only in the animals exposed to both LPS and BMZ. Conclusions: Our results suggested that double exposure to maternal BMZ and intra-amniotic LPS induces sustained increase of neutrophils in the lungs and disrupts alveolarization.
Journal of Perinatal Medicine 08/2013; · 1.95 Impact Factor
[show abstract][hide abstract] ABSTRACT: OBJECTIVES:: The aim of this study was to investigate the population pharmacokinetics of theophylline in premature Korean infants and to assess the influence of clinical covariates. METHODS:: Blood samples were first obtained after 1 week of maintenance dosing and then acquired approximately 4 weeks after continuous dosing. The time points were just before dosing and 2, 4, or 6 hours (at randomly assigned time points) after dosing. Two single-nucleotide polymorphism markers, -3860 G>A (CYP1A2*1C) and -163C>A (CYP1A2*1F), were genotyped. Gestational age (GA), postnatal age (PNA), postconceptional age (PCA = GA + PNA), body weight (BW), height, serum AST, serum ALT, serum BUN, serum creatinine, oxygen support, sex, delivery mode, and CYP1A2 genotypes were used for covariate model building. External validation was analyzed using data from an additional 27 patients. RESULTS:: A total of 334 serum concentration measurements were made in 100 patients. A one-compartment absorptive model with first-order elimination was fitted to the data in NONMEM (version 7.1.2). The final model included the following parameters: Clearance (L/h) = 0.00492 × (BW) + 0.00646 × (PNA), and volume of distribution (L) = 1.53 × (BW). The addition of the CYP1A2*1C or CYP1A2*1F genotypes to the model did not improve the model. The external validation results confirmed the predictive performance without bias in the final model. CONCLUSIONS:: The selected covariates were generally consistent with previous studies. However, the mean volume of distribution was higher than the values reported in other population pharmacokinetic studies, which may have been due to the use of 2 sampling time points. The predictive performance was reasonably acceptable. Therefore, the present model may permit more accurate selection of doses to achieve target theophylline concentrations in premature infants.
Therapeutic drug monitoring 05/2013; · 2.43 Impact Factor
[show abstract][hide abstract] ABSTRACT: Preterm infants are vulnerable to the oxidative stress due to the production of large amounts of free radicals, antioxidant system insufficiency, and immature oligodendroglial cells. Reactive oxygen species (ROS) play a pivotal role in the development of periventricular leukomalacia. The three most common ROS are superoxide (O2(•-)), hydroxyl radical (OH(•)), and hydrogen peroxide (H2O2). Under normal physiological conditions, a balance is maintained between the production of ROS and the capacity of the antioxidant enzyme system. However, if this balance breaks down, ROS can exert toxic effects. Superoxide dismutase, glutathione peroxidase, and catalase are considered the classical antioxidant enzymes. A recently discovered antioxidant enzyme family, peroxiredoxin (Prdx), is also an important scavenger of free radicals. Prdx1 expression is induced at birth, whereas Prdx2 is constitutively expressed, and Prdx6 expression is consistent with the classical antioxidant enzymes. Several antioxidant substances have been studied as potential therapeutic agents; however, further preclinical and clinical studies are required before allowing clinical application.
Korean Journal of Pediatrics 03/2013; 56(3):107-11.
[show abstract][hide abstract] ABSTRACT: To develop an high resolution computed tomography (HRCT) scoring system for the assessment of bronchopulmonary dysplasia (BPD) and determine its usefulness as compared with the chest radiographic score.
Forty-two very low-birth-weight preterm infants with BPD (25 male, 17 female) were prospectively evaluated with HRCT performed at the mean age of 39.1-week postmenstrual age. Clinical severity of BPD was categorized as mild, moderate or severe. The HRCT score (0-36) of each patient was the sum of the number of bronchopulmonary segments with 1) hyperaeration and 2) parenchymal lesions (linear lesions, segmental atelectasis, consolidation and architectural distortion), respectively. We compared the HRCT scores with the chest radiographic scores (the Toce system) in terms of correlation with clinical severity.
The HRCT score had good interobserver (r = 0.969, p < 0.001) and intraobserver (r = 0.986, p < 0.001) reproducibility. The HRCT score showed better correlation (r = 0.646, p < 0.001) with the clinical severity of BPD than the chest radiographic score (r = 0.410, p = 0.007). The hyperaeration score showed better correlation (r = 0.738, p < 0.001) with the clinical severity of BPD than the parenchymal score (r = 0.523, p < 0.001).
We have developed a new HRCT scoring system for BPD based on the quantitative evaluation of pulmonary abnormalities of BPD consisting of the hyperaeration score and the parenchymal score. The HRCT score shows better correlation with the clinical severity of BPD than the radiographic score.
Korean journal of radiology: official journal of the Korean Radiological Society 01/2013; 14(2):350-360. · 1.32 Impact Factor
[show abstract][hide abstract] ABSTRACT: OBJECTIVE:: To evaluate clinical applicability of noninvasive hemoglobin (Hb) measurement with a pulse CO-oximeter in neonates. DESIGN:: Prospective comparison study. SETTING:: Neonatal ICU. PATIENTS:: Fifty-six preterm and term infants with median age = 20 days (range = 1-98 days) and median weight = 1,440 g (range = 530-4,230 g). INTERVENTIONS:: Hb measurements by Pulse CO-Oximetry (Masimo Radical-7) were recorded immediately prior to venous samplings. MEASUREMENTS AND MAIN RESULTS:: The collected data were compared with the corresponding venous Hb level obtained in laboratory testing, and a total of 137 data pairs were analyzed. Noninvasive Hb values measured with a pulse CO-oximeter were significantly correlated with the venous Hb levels (correlation coefficient, r = .758; p < .001). Hb values measured with a pulse CO-oximeter were higher than those measured with a laboratory hematology analyzer (13.3 ± 2.6 g/dL vs. 12.5 ± 3.1 g/dL). In terms of the agreement between the laboratory analyzer and the pulse CO-oximeter, 94.8% of the measurements fell within two standard deviations of the mean difference. CONCLUSION:: Noninvasive Hb measurements with Pulse CO-Oximetry provide clinically acceptable accuracy, and they were significantly correlated with laboratory Hb measurement in neonates. In terms of the clinical applicability, noninvasive Hb monitoring with a pulse CO-oximeter could be useful in the early detection of Hb changes in neonates.
Pediatric Critical Care Medicine 11/2012; · 2.35 Impact Factor
[show abstract][hide abstract] ABSTRACT: Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant with ML II who presented with prenatal skeletal dysplasia and typical clinical features of severe secondary hyperparathyroidism at birth. A female infant was born at 37(+1) weeks of gestation with a birth weight of 1,690 g (<3rd percentile). Prenatal ultrasonographic findings revealed intrauterine growth retardation and skeletal dysplasia. At birth, the patient had characteristic features of ML II, and skeletal radiographs revealed dysostosis multiplex, similar to rickets. In addition, the patient had high levels of alkaline phosphatase and parathyroid hormone, consistent with severe secondary neonatal hyperparathyroidism. The activities of β-D-hexosaminidase and α-N-acetylglucosaminidase were moderately decreased in the leukocytes but were 5- to 10-fold higher in the plasma. Examination of a placental biopsy specimen showed foamy vacuolar changes in trophoblasts and syncytiotrophoblasts. The diagnosis of ML II was confirmed via GNPTAB genetic testing, which revealed compound heterozygosity of c.3091C>T (p.Arg1031X) and c.3456_3459dupCAAC (p.Ile1154GlnfsX3), the latter being a novel mutation. The infant was treated with vitamin D supplements but expired because of asphyxia at the age of 2 months.
Korean Journal of Pediatrics 11/2012; 55(11):438-44.
[show abstract][hide abstract] ABSTRACT: Background: Increased pro-inflammatory cytokines are suggested in the pathogenesis of necrotizing enterocolitis (NEC). The transcription factor, nuclear factor-ĸB (NF-ĸB), is a central regulator of inflammatory and immune responses, and recent rodent NEC models have shown that NF-ĸB may have a critical role in the disease processes that underlie NEC. Heat shock proteins have important functions in response to stress-related events in a variety of systems, including digestive organs. Objectives: We investigated whether heat shock pretreatment protects intestinal epithelial damage in the early NEC rat model. Methods: We generated human NEC-like lesions in neonatal rat ileum by administering oral endotoxin (10 mg/kg), intermittent 8% hypoxia, and hypertonic formula. Heat shock was administered by raising the chamber temperature to 42°C for 20 min, 3 h prior to endotoxin ingestion. Results: Heat shock pretreatment increased the expression of HSP70 in the ileal tissue and attenuated histological severity of early experimental NEC. NF-ĸB was activated in the ileal tissue of the NEC group and this activation was attenuated by heat shock pretreatment, which was determined by electrophoretic mobility shift assay and Western blot analysis of p50 in subcellular fractionated samples. Conclusions: Heat shock pretreatment reduced the incidence and severity of early experimental NEC in rats. A possible mechanism underlying this protective effect includes inhibition of NF-ĸB activation through increased HSP70 expression.
[show abstract][hide abstract] ABSTRACT: To determine whether neurally adjusted ventilatory assist (NAVA), a new method of mechanical ventilation that delivers pressure assistance that is proportional to the electrical activity of the diaphragm (EAdi), could lower the inspiratory pressure and respiratory muscle load in preterm infants supported with ventilators.
Twenty-six mechanically ventilated preterm infants were randomized to crossover ventilation with NAVA and synchronized intermittent mandatory ventilation (SIMV) with pressure support (PS) for 4 hours each in a randomized order. A 1-hour interval for washout was provided between the 2 modes of ventilation. The ventilator settings were adjusted to maintain similar levels of end-tidal partial pressure of CO(2). The ventilator parameters, vital signs, and gas exchange effects under the 2 ventilatory modes were compared.
Nineteen infants completed the 9-hour crossover comparison protocol. Peak inspiratory pressure (PIP), work of breathing, and peak EAdi with NAVA were lower than those in SIMV with PS. Calculated tidal volume to peak EAdi ratio and PIP to peak EAdi ratio were higher with NAVA. There were no significant differences in mean airway pressure, inspiratory oxygen fraction, and blood gas values. The measurements of vital signs did not differ significantly between the 2 modes.
NAVA lowered PIP and reduced respiratory muscle load in preterm infants at equivalent inspiratory oxygen fraction and partial pressure of CO(2) of capillary blood in comparison with SIMV with PS.
The Journal of pediatrics 06/2012; 161(5):808-813.e2. · 4.02 Impact Factor
[show abstract][hide abstract] ABSTRACT: The aim of our study was to investigate the differential effects of dexamethasone (DXM) and hydrocortisone (HCS) on somatic growth and postnatal lung development in a rat model of bronchopulmonary dysplasia (BPD). A rat model of BPD was induced by administering intra-amniotic lipopolysaccharide (LPS) and postnatal hyperoxia. The rats were treated with a 6-day (D1-D6) tapering course of DXM (starting dose 0.5 mg/kg/day), HCS (starting dose 2 mg/kg/day), or an equivalent volume of normal saline. DXM treatment in a rat model of BPD induced by LPS and hyperoxia was also associated with a more profound weight loss compared to control and LPS + O(2) groups not exposed to corticosteroid, whereas HCS treatment affected body weight only slightly. Examination of lung morphology showed worse mean cord length in both LPS + O(2) + DXM and LPS + O(2) + HCS groups as compared to the LPS + O(2) alone group, and the LPS + O(2) + DXM group had thicker alveolar walls than the LPS + O(2) group at day 14. The HCS treatment was not significantly associated with aberrant alveolar wall thickening and retarded somatic growth. The use of postnatal DXM or HCS in a rat model of BPD induced by intra-amniotic LPS and postnatal hyperoxia appeared detrimental to lung growth, but there was less effect in the case of HCS. These findings suggest that effect of HCS on somatic growth and pulmonary outcome may be better tolerated in neonates for preventing and/or treating BPD.
Journal of Korean medical science 04/2012; 27(4):395-401. · 0.84 Impact Factor
[show abstract][hide abstract] ABSTRACT: Granulocyte colony-stimulating factor (G-CSF) is known to mobilize endothelial progenitor cells (EPCs) from bone marrow. EPCs reportedly promote neovascularization and participate in the repair of lung structure in adult animals.
We tested the hypothesis that G-CSF contributes to alveolar growth by increasing the production of angiogenic growth factor in the lungs of hyperoxia-exposed neonatal mice.
Neonatal mice were exposed to hyperoxia (80%) or room air (RA) for 7 days and treated with G-CSF (50 μg/kg/day) or vehicle for 5 days. Blood was subjected to flow cytometry to gate for CD45(dim/-)/Sca-1(+)/CD133(+)/vascular endothelial growth factor (VEGF) receptor-2 (VEGFR2) to define the EPC population at day 7.
The percentage of EPCs in the peripheral blood and VEGF and VEGFR2 levels in the lungs of neonatal mice exposed to hyperoxia were significantly reduced compared to those of mice kept in RA. G-CSF significantly increased EPCs in the peripheral blood, and VEGF and VEGFR2 levels in the lungs of both mice exposed to hyperoxia and mice kept in RA. G-CSF restored alveolarization inhibited by hyperoxia without altering normal alveolarization under RA.
G-CSF restored alveolarization inhibited by hyperoxia in the developing lungs and this alveolarization-enhancing effect of G-CSF is associated with mobilization of EPCs and upregulation of VEGF signaling.
[show abstract][hide abstract] ABSTRACT: Peroxiredoxins (Prdx), a family of antioxidant proteins, have important defensive roles in the degenerative brain diseases and neuronal cell death in adult subjects. However, little is known in the neonatal brain. Here, we studied the developmental expression of Prdxs and their response to dexamethasone in the perinatal rat brain. Prdx 1 expression increased during late gestations and peaked at postnatal-day 1, when its expression gradually decreased. Prdx 2 expression remained largely unchanged. Prdx 6 expression continually increased as growing. Using immunohistochemistry, each Prdx showed a strong expression in the cerebral cortex and hippocampus. Prdx 1 was strongly expressed in the corpus callosum. The dexamethasone injection increased the expression of Prdx 6. In conclusion, we reveal for the first time that Prdx 1, 2 and 6 are found in abundance in the perinatal rat brain and are differentially expressed during development. The expression of Prdx 6 was affected by dexamethasone treatment.
Free radical research 12/2011; 46(3):231-9. · 2.22 Impact Factor
[show abstract][hide abstract] ABSTRACT: This study aimed to investigate the associations of combined parental low educational level and combined parental unemployment on the risk of preterm birth (PTB) in Korea.
Data on 427,857 singleton births were obtained from the National Birth Registration (NBR) database in 2003 and analyzed. Parental education and parental employment status were combined as exposure for analysis. Place of birth, sex, marital status, parental age and parity were included for analysis of unconditional multiple logistic regressions. PTB was defined as birth before a gestational age of 37 complete weeks.
Group of the lowest educational level, below high school, had the highest odds of PTB in both father and mother in multivariable analysis [odds ratio (OR) 1.15 and 1.16, respectively]. After combining parental educational status for the multivariable analysis, the highest probability of PTB was in families where both parents had below college level education (OR 1.22). As for paternal employment, the multivariable analysis showed an increased rate of PTB occurred where the father was unemployed (OR 1.11). After combining the employment status of both parents, the multivariable analysis revealed that PTB was only significant in families where both parents were unemployed (OR 1.09).
We found that combined parental low educational level and combined parental unemployment increased the likelihood of preterm birth.
International Journal of Public Health 11/2011; 57(2):253-60. · 1.99 Impact Factor
[show abstract][hide abstract] ABSTRACT: Meconium obstruction (MO) of prematurity can result in increased morbidity or mortality and prolonged hospitalization if not diagnosed and treated appropriately.
The aims of our study were to identify the incidence and risk factors associated with MO and to review the treatment outcomes.
A retrospective analysis was undertaken of 101 extremely low birth weight infants (ELBWIs) who were born between January 1, 2007, and April 1, 2009, at Seoul National University Hospital. Prenatal and neonatal factors were compared between the MO and control groups. The treatment outcomes were also reviewed.
Twenty-two (22%) patients were diagnosed as having MO. Eighteen of these patients (82%) had prenatal risk factors for MO. Respiratory distress syndrome was more prevalent in the MO group than in the control group (p = 0.001). Overall, 17 of the 22 patients (77%) were relieved with medical treatment and the rest underwent ileostomy. The times to full enteral feeding did not differ between the medically treated group and the control group. However, the surgically treated group required more time to achieve full enteral feeding, and some patients had persistent gastrointestinal problems.
MO is not a rare condition in ELBWIs, and the majority of ELBWIs have prenatal risk factors. Medical management was effective, and medically manageable cases had good prognoses for subsequent feeding, whereas some surgically managed cases had persistent gastrointestinal problems.
[show abstract][hide abstract] ABSTRACT: Despite the potential importance of pulmonary artery hypertension (PAH) in preterm infants with bronchopulmonary dysplasia (BPD), little is known about the risk factors for PAH.
To investigate the risk factors for PAH in preterm infants with BPD.
Infants diagnosed with BPD were assigned to the PAH group or non-PAH group except for infants with mild BPD who had no PAH. PAH was diagnosed on the basis of echocardiograms demonstrating elevated right ventricle pressure beyond the postnatal age of 2 months. Logistic regression analysis was done for the multivariate assessment of the risk factors for PAH in preterm infants with moderate or severe BPD.
A total of 98 infants among 145 infants with BPD were divided into a PAH group (n = 25) or non-PAH group (n = 73), while the remaining 47 infants had mild BPD with no PAH. Among the study patients, survival rate of the PAH group was significantly lower than that of the non-PAH group. Infants with PAH had more severe cases of BPD and underwent longer durations of oxygen therapy, conventional or high-frequency ventilation, and hospitalization compared to those without PAH. Low 5-min Apgar scores (≤6; relative risk (RR) 6.2; 95% confidence interval (CI) 1.4-28.0; p = 0.017) and oligohydramnios (RR 7.7; 95% CI 2.0-29.6; p = 0.030) were found to be significant risk factors for PAH according to multivariate analysis.
The present study shows that oligohydramnios is a specific risk factor for PAH in preterm infants with moderate or severe BPD.
[show abstract][hide abstract] ABSTRACT: Currently, bronchopulmonary dysplasia (BPD) occurs almost exclusively in pre-term infants. In addition to prematurity, other factors like oxygen toxicity and inflammation can contribute to the pathogenesis. This study aimed to compare urinary inflammatory and oxidative stress markers between the no/mild BPD group and moderate/severe BPD group and between BPD cases with significant early lung disease like respiratory distress syndrome (RDS) ('classic' BPD) and with minimal early lung disease ('atypical' BPD). A total of 60 patients who were a gestational age < 30 weeks or a birth weight < 1250 g were included. Urine samples were obtained on the 1(st), 3(rd) and 7(th) day of life and measured the levels of leukotriene E(4) (LTE(4)) and 8-hydroxydeoxyguanosine (8-OHdG). The 8-OHdG values on the 3(rd) day showed significant correlation to duration of mechanical ventilation. The 8-OHdG levels on the 7(th) day were the independent risk factor for developing moderate/severe BPD. In 'classic' BPD, the 8-OHdG values on the 3(rd) day were higher than those of 'atypical' BPD. In 'atypical' BPD, the LTE(4) values on the 7(th) day were higher than the values in 'classic' BPD. These results suggest that oxidative DNA damage could be the crucial mechanism in the pathogenesis of current BPD and the ongoing inflammatory process could be an important mechanism in 'atypical' BPD.
Free radical research 06/2011; 45(9):1024-32. · 2.22 Impact Factor
[show abstract][hide abstract] ABSTRACT: McKusick-Kaufman syndrome (MKS) is an autosomal recessive multiple malformation syndrome characterized by hydrometrocolpos (HMC) and postaxial polydactyly (PAP). We report a case of a female child with MKS who was transferred to the neonatal intensive care unit of Seoul National University Children's Hospital on her 15th day of life for further evaluation and management of an abdominal cystic mass. She underwent abdominal sonography, magnetic resonance imaging, genitography and cystoscopy which confirmed HMC with a transverse vaginal septum. X-rays of the hand and foot showed bony fusion of the left third and fourth metacarpal bones, right fourth dysplastic metacarpal bone and phalanx, right PAP and hypoplastic left foot with left fourth and fifth dysplastic metatarsal bones. In addition, she had soft palate cleft, mild hydronephroses of both kidneys, hypoplastic right kidney with ectopic location and mild rotation, uterine didelphys with transverse vaginal septum and low-type imperforated anus. She was temporarily treated with ultrasound-guided transurethral aspiration of the HMC. Our patient with HMC and PAP was diagnosed with MKS because she has two typical abnormality of MKS and she has no definite complications of retinal disease, learning disability, obesity and renal failure that develop in Bardet-Biedl syndrome, but not in MKS until 33 months of age. Here, we describe a case of a Korean patient with MKS.
Korean Journal of Pediatrics 05/2011; 54(5):219-23.
[show abstract][hide abstract] ABSTRACT: Fetus-in-fetu (FIF) is a rare congenital condition in which a fetiform mass is detected in the host abdomen and also in other sites such as the intracranium, thorax, head, and neck. This condition has been rarely reported in the literature. Herein, we report the case of a fetus presenting with abdominal cystic mass and ascites and prenatally diagnosed as meconium pseudocyst. Explorative laparotomy revealed an irregular fetiform mass in the retroperitoneum within a fluid-filled cyst. The mass contained intestinal tract, liver, pancreas, and finger. Fetal abdominal cystic mass has been identified in a broad spectrum of diseases. However, as in our case, FIF is often overlooked during differential diagnosis. FIF should also be differentiated from other conditions associated with fetal abdominal masses.
Korean Journal of Pediatrics 03/2011; 54(3):133-6.