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Oral Proton Pump Inhibitor for Treatment of Congenital Chloride Diarrhea.

Hee Cheol JO ; Jong Seo YOON ; Joo Young JANG ; Young Bae SOHN ; Jang Hoon LEE ; Hae Il CHEONG ; Moon Sung PARK

Neonatal Medicine.2016;23(1):59-63. doi:10.5385/nm.2016.23.1.59

Congenital chloride diarrhea (CCD) is a rare autosomal recessive disease, which is characterized by electrolyte absorption defect due to impaired function of the Cl-/HCO3 - exchanger in the ileum and the colon. Its main features are profuse watery diarrhea, high fecal chloride concentration, and failure to thrive. Profuse watery diarrhea characterized by a high concentration of chloride in stools results in hypochloremia, hyponatremia, and dehydration with metabolic alkalosis. Early detection and therapeutic intervention can prevent life-threatening symptoms of CCD and growth failure. Recently, several therapies, such as proton pump inhibitors and butyrate, have been suggested for amelioration of diarrhea. Here, we report a case of CCD in a preterm male infant who was successfully treated with an oral proton pump inhibitor.
Absorption ; Alkalosis ; Butyrates ; Colon ; Dehydration ; Diarrhea* ; Failure to Thrive ; Humans ; Hyponatremia ; Ileum ; Infant ; Male ; Omeprazole ; Proton Pump Inhibitors ; Proton Pumps* ; Protons*

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Primary Adrenal Insufficiency in a Newborn With Adrenal Hypoplasia Congenita Caused by a Mutation of the DAX1 Gene.

Sun Hyoung PARK ; Yong Hee HONG ; Sung Shin KIM

Neonatal Medicine.2016;23(1):53-58. doi:10.5385/nm.2016.23.1.53

Adrenal hypoplasia congenita (AHC) is a rare inherited disorder of the adrenal gland caused by deletion or mutation of the dosage-sensitive sex-reversal AHC critical region on the X chromosome, gene 1 (DAX1) gene. The DAX1 gene is expressed in the adrenal cortex, the pituitary gland, the hypothalamus, the testis, and the ovary. Most affected infants present with failure to thrive, salt wasting, and hypoglycemic seizure in early life. Immediate mineralocorticoid and glucocorticoid replacement is essential. Most boys with AHC present with hypogonadotropic hypogonadism, resulting in failure to enter puberty and the need for testosterone treatment. However, a recent study revealed that the onset of puberty in boys with AHC can be variable, ranging from arrested or absent to precocious. We describe a case involving a newborn who presented with primary adrenal insufficiency due to a mutation of the DAX1 gene and was finally diagnosed with AHC.
Addison Disease* ; Adolescent ; Adrenal Cortex ; Adrenal Glands ; Adrenal Insufficiency ; Failure to Thrive ; Female ; Humans ; Hypogonadism ; Hypothalamus ; Infant ; Infant, Newborn* ; Ovary ; Pituitary Gland ; Puberty ; Seizures ; Testis ; Testosterone ; X Chromosome

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Bee Venom Exerts Neuroprotective Effects on Neuronal Cells and Astrocytes under Hypoxic Conditions Through MAPK Signaling Pathways.

Eun Joo LEE ; Bong Jae KIM ; Ji Eun JEONG ; Hai Lee CHUNG ; Eun Kyoung YANG ; Woo Taek KIM

Neonatal Medicine.2016;23(1):43-52. doi:10.5385/nm.2016.23.1.43

PURPOSE: Hypoxic-ischemic brain injuries influence the mechanisms of signal transduction, including mitogen-activated protein kinase (MAPK) that regulates gene expression through transcription factor activity. Several attempts have been made to use bee venom (BV) to treat neurological diseases. However, limited data are available for brain injuries such as neonatal hypoxic-ischemic encephalopathy (HIE) and neurodegenerative disorders. The purpose of this study was to investigate the neuroprotective effects of BV by determining the expression of activated MAPK pathways. METHODS: We examined activation and cell viability in hypoxia (1% O2, 5% CO2, 94% N2) in low glucose-treated (H+low G) neuronal cells and astrocytes in the presence and absence of BV. After they were subjected to hypoxic conditions and treated with low glucose, the cells were maintained for 0, 6, 15, and 24 h under normoxic conditions. RESULTS: Extracellular-signal-regulated kinases 1/2 (ERK1/2), p38 MAPK, and stress-activated protein kinases (SAPK)/Jun amino-terminal kinases (JNK) were activated in H+low G conditions. Particularly, phosphorylation of ERK1/2 was maximized 6 h after exposure to H+low G condition. BV specifically inhibited the phosphorylation of ERK1/2. However, BV had no effect on p38 MAPK or SAPK/JNK. In addition, BV improved neuronal cell and astrocytes viability following exposure to H+low G. CONCLUSION: ERK inactivation is known to mediate protective effects in hypoxic brain injury. Taken together, these results suggest that treatment with BV may be helpful in reducing hypoxic injury in neonatal HIE through the ERK signaling pathway.
Anoxia ; Astrocytes* ; Bee Venoms* ; Bees* ; Brain Injuries ; Cell Survival ; Gene Expression ; Glucose ; Hypoxia-Ischemia, Brain ; Neurodegenerative Diseases ; Neurons* ; Neuroprotective Agents* ; p38 Mitogen-Activated Protein Kinases ; Phosphorylation ; Phosphotransferases ; Protein Kinases ; Signal Transduction ; Transcription Factors

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The Incidence, Treatment and Risk Factors of Candidiasis in Very Low Birth Weight Infants.

Ha Young SHIN ; Ji Na PARK ; Yun Hye SHIN ; Da Young YUN ; Hyun A PARK ; Jong Hee HWANG

Neonatal Medicine.2016;23(1):35-42. doi:10.5385/nm.2016.23.1.35

PURPOSE: Candidiasis is an important morbidity among very low birth weight infants (VLBWI). There is a little data on the risk factors in VLBWI. This study was done to describe the incidence, treatment, and risk factors of candidiasis in VLBWI. METHODS: From September 2008 to December 2011, medical records of 130 infants with VLBWI in Inje University Ilsan Paik hospital neonatal intensive care unit (NICU) were reviewed retrospectively. Seventeen infants were diagnosed with candidiasis and treated with antifungal agent. Patients were divided into the candidiasis group (CAN, n=17), the bacterial sepsis group (BAC, n=34), and the non-sepsis group (Non-SEP, n=74). Demographic findings and factors associated with candidiasis were compared between these groups. RESULTS: The mean gestational age was significantly low in the CAN group, but birth weight was not significantly different between the groups. The maternal demographic findings were not significantly different between the groups. The incidence of respiratory distress syndrome (RDS) is higher in the CAN group compared to these groups (P<0.05). The durations of intubation and central venous line were significantly longer in the CAN group than in the other groups (P<0.05). In the logistic regression analysis, the duration of central venous line is the significant factor for candidiasis (P=0.003, odd ratio: 1.56, 95% confidence interval: 1.39-1.68). CONCLUSION: The incidence of candidiasis in VLBWI was 13.1 % and the risk factor for candidiasis was longer duration of central venous line in our study.
Birth Weight ; Candidiasis* ; Gestational Age ; Humans ; Incidence* ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care, Neonatal ; Intubation ; Logistic Models ; Medical Records ; Retrospective Studies ; Risk Factors* ; Sepsis

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Relation Between Absoute Nucelated Red Blood Cell Count at Birth and Retinopathy of Prematurity.

Sung Woo CHO ; Seung Hyun LEE ; Yeon Kyun OH

Neonatal Medicine.2016;23(1):29-34. doi:10.5385/nm.2016.23.1.29

PURPOSE: The aim of this study is to prove the association between potential fetal hypoxia and retinopathy of prematurity (ROP) development and absolute nucleated red blood cell (aNRBC) is used to evaluate it in premature infants without any hypoxic or ischemic history. METHODS: Medical records of 43 premature infants with ROP who were admitted to the neonatal intensive care unit at Wonkwang University Hospital from January 2004 to December 2014 were analyzed retrospectively. We excluded 15 infants who had a confounding medical condition that could have increased the aNRBC count. Finally, 28 premature infants affected by ROP were enrolled and compared with 28 pair-matched controls. The aNRBC counts at birth in these infants were compared. Statistical analysis was performed with a paired t-test for continuous data, and a Fisher's exact test for categorical data. P<0.05 was considered significant. RESULTS: There were no significant differences in perinatal characteristics such as gestational age (GA), birth weight (BWt), Apgar scores, premature rupture of membrane (>24 hours), prenatal betamethasone, surfactant or respiratory distress syndrome between the ROP and the control infants. In addition, neither group differed in major morbidities such as patent ductus arteriosus, periventricular leukomalacia, intraventricular hemorrhage (> or =Grade 2), or bronchopulmonary dysplasia. Regardless of the severity of ROP, the aNRBC counts at birth in premature infants with ROP were not higher than in the control infants. CONCLUSION: The aNRBC counts at birth may not be related directly to the development of ROP.
Betamethasone ; Birth Weight ; Bronchopulmonary Dysplasia ; Ductus Arteriosus, Patent ; Erythrocyte Count* ; Erythrocytes* ; Fetal Hypoxia ; Gestational Age ; Hemorrhage ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Intensive Care, Neonatal ; Leukomalacia, Periventricular ; Medical Records ; Membranes ; Parturition ; Retinopathy of Prematurity* ; Retrospective Studies ; Rupture

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Clinical Findings According to Feeding Diets in Very Low Birth Weight Infants: Human Breast Milk versus Bovine Milk-Based Formula.

Sang Hyun LEE ; Jae Hyun PARK ; Chun Soo KIM ; Sang Lak LEE

Neonatal Medicine.2016;23(1):23-28. doi:10.5385/nm.2016.23.1.23

PURPOSE: We aimed to compare the clinical findings, including morbidity, duration of parenteral nutrition, and length of hospital stay between very low birth weight infants (VLBWIs) fed bovine milk-based formula (BOV) and VLBWIs fed human breast milk (HBM) in a neonatal intensive care unit (NICU). METHODS: VLBWIs admitted to the NICU of Dongsan Medical Center, Keimyung University, were enrolled. Infants born from March to August 2014 (n=28) were fed BOV (the BOV group), and those born from September to December 2014 (n=18) were fed HBM (the HBM group). Pasteurized (heating at 62.5degrees C for 30 minutes) donor human milk was used if the mother's own milk was not available because of insufficient breast milk production. RESULTS: The gestational age (28.0+/-1.7 weeks vs. 27.8+/-1.4 weeks) and birth weight (1,055+/-265 g vs. 1,175+/-187 g), of the infants in the BOV and HBM, groups were similar. In addition, perinatal characteristics were similar between the groups. The duration of parenteral nutrition (36.4 days vs. 24.1 days, P=0.038), length of hospital stay (74.3 days vs.61.1 days, P=0.037), and incidence of nosocomial sepsis (53.6% vs. 22.2%, P=0.035), significantly differed between the BOV and HBM groups. Furthermore, the frequency of feeding intolerance was higher in the BOV group than in the HBM group, but this difference was not significant. Perinatal cytomegalovirus infection was not detected in any of the infants fed pasteurized donor human milk. CONCLUSION: Human-breast-milk-based diet for VLBWIs significantly reduces the incidence of nosocomial sepsis, duration of parenteral nutrition, and length of hospital stay.
Birth Weight ; Breast* ; Cytomegalovirus Infections ; Diet* ; Gestational Age ; Humans ; Humans* ; Incidence ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care, Neonatal ; Length of Stay ; Milk ; Milk, Human* ; Parenteral Nutrition ; Sepsis ; Tissue Donors

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Clinical Significance of Newborn Gastric Inflammatory Mediators.

Young Don KIM ; Soon Sup SHIM ; Hye Sim KANG ; Young Hee MAENG

Neonatal Medicine.2016;23(1):16-22. doi:10.5385/nm.2016.23.1.16

PURPOSE: We performed this study to determine (1) whether the levels of inflammatory mediators, including matrix metalloproteinase-9 (MMP-9) and intercellular adhesion molecule-3 (ICAM-3), in gastric fluid (GF) in premature newborns are associated with those in amniotic fluid (AF) in their mothers and (2) whether the levels of the inflammatory mediators in newborn GF are associated with the presence of intrauterine inflammation (IUI). METHODS: Sixty-two pairs of pregnant women and their premature newborns born at <35 weeks' gestation by cesarean delivery were enrolled in this study. AF and newborn GFs were obtained during cesarean section procedures. Levels of MMP-9, ICAM-3, interleukin-6 (IL-6), interleukin-8 (IL-8), and tumor necrosis factor-alpha (TNF-alpha) were measured and compared between the AF and newborn GFs in each dyad, according to the presence or absence of chorioamnionitis (CA), preterm prelabor rupture of membranes (PPROM), and preterm labor (PTL). RESULTS: The levels of MMP-9, ICAM-3, IL-6, IL-8, and TNF-alpha in newborn GF were significantly correlated with those in AF in each dyad. The premature newborns and their mothers with CA had significantly higher GF MMP-9, IL-8, and TNF-alpha levels than those without CA. Those with PPROM or PTL showed similar findings in terms of GF MMP-9, IL-8, and TNF-alpha levels. CONCLUSION: The newborn GF immediately after birth can be a useful alternative source of information on whether a premature infant has been exposed to IUI at the time of delivery.
Amniotic Fluid ; Cesarean Section ; Chorioamnionitis ; Female ; Humans ; Infant, Newborn* ; Infant, Premature ; Inflammation ; Interleukin-6 ; Interleukin-8 ; Matrix Metalloproteinase 9 ; Membranes ; Mothers ; Obstetric Labor, Premature ; Parturition ; Pregnancy ; Pregnant Women ; Rupture ; Tumor Necrosis Factor-alpha

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Deaths in the Neonatal Intensive Care Unit between 2002 and 2014.

Ga Young PARK ; Sung Shin KIM

Neonatal Medicine.2016;23(1):8-15. doi:10.5385/nm.2016.23.1.8

PURPOSE: To report the causes and patterns of death among infants admitted to our neonatal intensive care unit (NICU) over a 13-year period. In addition, we analyzed trends regarding the type of end-of-life care provided. METHODS: All of the neonates who died at the Soonchunhyang University Bucheon Hospital between January 1, 2002, and December 31, 2014, were identified. The causes and circumstances of death were extracted from individual medical records. Trends in mortality were compared between two time periods: 2002 to 2007 and 2008 to 2014. RESULTS: Of the 5,223 admissions to our NICU, 97 neonates died. The overall mortality rate was 1.9%. The most common cause of death was sepsis (15%). At a lower gestational age, infants died of extreme prematurity and complications of prematurity. Among term infants, the principal cause of death shifted to hypoxic ischemic encephalopathy and asphyxia. A total of 63 infants (64.9%) received maximal intensive care, and 34 infants (35%) had redirection of intensive care. During this period, the proportion of death after redirection of care increased from 30.6% to 39.6%. Infants decided to forgo life-sustaining care before death had significantly lower gestational ages and lower birth weights (30.5 vs. 27.1 weeks, P=0.005; 1,528 vs. 1,063 g, P=0.025). CONCLUSION: Infection remained an important cause of death for neonate, particularly for preterm infants. The proportion of infants who had redirectoin of care before death was increased, suggesting that quality-of-life should be considered an important factor in the decision-making process for the infant, parents, and medical staff.
Asphyxia ; Birth Weight ; Cause of Death ; Gestational Age ; Gyeonggi-do ; Humans ; Hypoxia-Ischemia, Brain ; Infant ; Infant, Newborn ; Infant, Premature ; Critical Care ; Intensive Care, Neonatal* ; Medical Records ; Medical Staff ; Mortality ; Parents ; Sepsis ; Terminal Care

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A 12-Year Retrospective Study of Survival Rates and Outcomes of Very Low Birth Weight Infants in a Single Center.

Moon Sun JEONG ; Seok Ho YOON ; Yeon Kyung LEE ; Sun Young KO ; Son Moon SHIN

Neonatal Medicine.2016;23(1):1-7. doi:10.5385/nm.2016.23.1.1

PURPOSE: To investigate the outcomes and survival rates of very low birth weight infants (VLBWI) born over a 12-year period in a single center. METHODS: A retrospective review of 613 VLBWI born from January 2000 to December 2011 was performed. We compared the incidence, survival rate, and morbidity of infants classified according to their birth weight or gestational age for the following periods: Period I (2000-2002), Period II (2003-2005), Period III (2006-2008), and Period IV (2009-2011). RESULTS: The incidence of VLBWI was 0.7%, while the overall survival rate was 94.9%. The survival rates were 92.8%, 92.9%, 95.9%, and 97.5% for periods I, II, III and IV, respectively; the rates improved significantly over time (P<0.05). The average survival rate of extremely low birth weight infants was 85.9%, and it tended to increase over successive periods (P=0.06). The overall incidence of respiratory distress syndrome was 63.6%; patent ductus arteriosus; 43.6%; bronchopulmonary dysplasia, 30.5%; retinopathy of prematurity (> or =grade III), 8.5%; sepsis, 6.5%; cystic periventricular leukomalacia, 3.8%; necrotizing enterocolitis (> or =grade II), 3.4%; and intraventricular hemorrhage (> or =grade III), 2.3%. A significant decrease was seen in some clinical parameters: the time to start feeding, duration of parenteral nutrition, and duration required to reach full enteral feeding in every successive three-year period (P<0.01). CONCLUSION: The overall survival rate of VLBWI born between January 2000 and December 2011 was 94.9%. This survival rate was found to increase significantly in each successive three-year period starting January 2000.
Birth Weight ; Bronchopulmonary Dysplasia ; Ductus Arteriosus, Patent ; Enteral Nutrition ; Enterocolitis, Necrotizing ; Gestational Age ; Hemorrhage ; Humans ; Incidence ; Infant* ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Leukomalacia, Periventricular ; Parenteral Nutrition ; Retinopathy of Prematurity ; Retrospective Studies* ; Sepsis ; Survival Rate*

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A Case of Incontinentia Pigmenti with Multiple Brain Infarction.

Tae Hee KIM ; Young Jin CHOI ; Hyun Kyung PARK ; Chang Ryul KIM ; Hyun Ju LEE

Neonatal Medicine.2013;20(2):228-232. doi:10.5385/nm.2013.20.2.228

Incontinentia pigmenti (IP) is a rare X-linked dominant disease that is typically lethal to males and usually affect female patients. IP is a neurocutaneous disorder, involving the ectodermal tissues such as the skin, eyes, teeth, hair, and central nervous system. The pathogenesis of IP is linked to the gene mutation in the NF-kappa B essential modulator (NEMO) on chromosome Xq28. We experienced one case of newborn with multiple vesiculobullous skin lesions over the entire body after birth. Skin biopsy and histologic studies revealed suspected IP stage I and the genetic analysis of the NEMO confirmed IP diagnosis. A brain MRI showed multiple cerebral infarctions and the infant has shown delayed development in follow-up clinic.
Biopsy ; Brain ; Brain Infarction ; Central Nervous System ; Cerebral Infarction ; Ectoderm ; Eye ; Female ; Follow-Up Studies ; Hair ; Humans ; Incontinentia Pigmenti ; Infant ; Infant, Newborn ; Male ; Neurocutaneous Syndromes ; NF-kappa B ; Parturition ; Skin ; Tooth

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