Biventricular function in preterm infants with patent ductus arteriosus ligation: A three-dimensional echocardiographic study.

BACKGROUND: The detailed hemodynamics after patent ductus arteriosus (PDA) ligation in preterm infants remain unknown. We aimed to clarify the effect of surgical ligation on left ventricular (LV) and right ventricular (RV) volume and function. METHODS: Echocardiography was performed in 41 preterm infants (median gestational age: 25 weeks) before and after PDA ligation. Global longitudinal strain was determined using three-dimensional speckle-tracking echocardiography. These values were compared with those in 36 preterm infants without PDA (non-PDA). RESULTS: Preoperatively, the PDA group had greater end-diastolic volume (EDV) and cardiac output (CO) in both ventricles, a higher LV ejection fraction (LVEF) (53% vs 44%) and LV global longitudinal strain, and a lower RVEF (47% vs 52%) than the non-PDA group. At 4-8 h postoperatively, the two groups had a similar LVEDV and RVEDV. However, the PDA group had a lower EF and CO in both ventricles than the non-PDA group. At 24-48 h postoperatively, the RVEF was increased, but the LVEF remained decreased, and LVCO was increased. CONCLUSIONS: PDA induces biventricular loading and functional abnormalities in preterm infants, and they dramatically change after surgery. Three-dimensional echocardiography may be beneficial to understand the status of both ventricles. IMPACT: Preterm infants are at high risk of hemodynamic compromise following a sudden change in loading conditions after PDA ligation. Three-dimensional echocardiography enables quantitative and serial evaluation of ventricular function and volume in preterm infants with PDA. PDA induces biventricular loading and functional abnormalities in preterm infants, and they dramatically change after surgery.

Respiratory function testing for guiding ventilator mode conversion in congenital diaphragmatic hernia.

INTRODUCTION: For patients with a congenital diaphragmatic hernia, conventional mechanical ventilation (CMV) and high-frequency oscillatory ventilation (HFOV) are used in initial ventilatory management. HFOV has recently been recommended as a rescue therapy; however, we use HFOV for initial ventilation management, with a preoperative challenge test for CMV conversion and respiratory function testing at the time of CMV conversion. We aimed to compare patient characteristics between CMV conversion- and HFOV-preferred treatment groups. METHODS: Ventilator settings and blood gases were retrospectively evaluated pre- and post-CMV conversion, and respiratory function tests for compliance of the respiratory system (Crs) and for resistance of the respiratory system (Rrs) were performed during the trial to CMV conversion. RESULTS: No differences were observed between the CMV conversion- and HFOV-preferred groups regarding gestational age, birth weight, and observed/expected lung area-to-head circumference ratios. The median Crs (ml/cmH2 O/kg) and Rrs (cmH2 Oï½¥kg/L/s) in the CMV conversion- and HFOV-preferred groups was 0.42 versus 0.53 (p = .44) and 467 versus 327 (p = .045), respectively. The pre and posttrial amount of change in blood gas levels and ventilator parameters in the CMV conversion- and HFOV-preferred groups were as follows: mean airway pressure, -2.0 versus 0 cmH2 O; partial pressure of carbon dioxide, 6.1 versus 2.9 Torr; alveolar-arterial oxygen difference, -39.5 versus -50 Torr; and oxygenation index, -1.0 versus -0.6; respectively. CONCLUSION: Respiratory function tests were useful in tailoring ventilator settings. Patients with high Rrs values responded better to CMV conversion.

Death of children with Down syndrome by gestational age and cause.

BACKGROUND: We often encounter preterm infants with Down syndrome (DS) who die in the neonatal intensive care unit (NICU). In this study, we examined survival until NICU discharge and assessed the developmental prognosis of preterm infants with DS. METHODS: We retrospectively reviewed 416 infants with DS hospitalized during the past 27 years at our NICU. RESULTS: Death occurred in 8/20 (40%) infants at <32 weeks' gestation, 11/23 (48%) at 32-33 weeks, 9/99 (9%) at 34-36 weeks, and 9/274 (3%) at >36 weeks. In total, 84% of infants who died and 25% of those who survived had a non-reassuring fetal status (p < 0.001). Sex, small-for-gestational-age status, and postnatal transport were not associated with death. The main causes of death were bronchopulmonary dysplasia in 4/8 (50%) infants at <32 weeks' gestation, transient abnormal myelopoiesis in 11/20 (55%) and lymphatic dysplasia in 6/20 (30%) at 32-36 weeks, and varied causes at >36 weeks. Among survivors born at <34 weeks' gestation, 6/19 (32%) aged >2 years had moderate or severe cerebral palsy. CONCLUSIONS: These data on the high mortality and morbidity of preterm infants with DS may be useful for patient treatment and parent counseling in NICUs treating critically ill infants. IMPACT: Most infants with Down syndrome born at <34 weeks' gestation are born by cesarean section because of the non-reassuring fetal status. The mortality rate before discharge for infants with Down syndrome born at <34 weeks' gestation was 40%, and 30% of survivors developed moderate or severe cerebral palsy. The risk of death due to bronchopulmonary dysplasia and pulmonary hypertension was high in very preterm infants with Down syndrome despite the absence of chorioamnionitis. Infants with Down syndrome were born 1-2 weeks earlier than unaffected controls.

Common Blood Test Indices for Predicting Transient Abnormal Myelopoiesis-Related Mortality in Infants with Down Syndrome.

Transient abnormal myelopoiesis (TAM) can cause early death in children with Down syndrome, and liver failure is the most common cause of death. The aim of this single-center retrospective study was to identify a quantitative index for predicting TAM-related mortality at the time of diagnosis. Of the 462 children with Down syndrome admitted to our hospital from 1992 to 2021, we studied 12 infants with TAM-related death and 31 survivors who were diagnosed with TAM. In the death and survival groups, the median gestational ages were 34.9 and 37.1 weeks, respectively (p = 0.12). At diagnosis, the white blood cell (WBC) counts were 99.2 and 36.2 × 109/L (p = 0.011), the hemoglobin concentrations were 131 and 159 g/L (p = 0.009), and the serum albumin concentrations were 23 and 31 g/L (p < 0.001), respectively. The areas under the receiver operating characteristic curve for the abilities of the WBC count, hemoglobin, and serum albumin at diagnosis to predict survival were 0.75, 0.76, and 0.85, respectively. The serum albumin concentration threshold of 28 g/L at diagnosis had sensitivity of 0.79 and specificity of 0.82. Gestational age and serum albumin concentration were entered into a logistic regression model. The serum albumin concentration was an independent indicator of TAM-related death (adjusted odds ratio, 0.78; 95% confidence interval, 0.65-0.93; p = 0.005). In conclusion, a low serum albumin concentration at diagnosis may be a good predictor of TAM-related death.

Right to left ventricular volume ratio is associated with mortality in congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with high neonatal mortality. We performed this study to test the hypothesis that left ventricular (LV) and right ventricular (RV) volumes assessed by three-dimensional echocardiography may be associated with mortality in CDH. METHODS: This study was a single-center retrospective cohort study involving 35 infants with CDH. RV and LV end-diastolic volume (RVEDV and LVEDV, respectively) were measured by three-dimensional echocardiography and were corrected by birth body weight (BBW) on day 1. RVEDV/BBW, LVEDV/BBW, and LVEDV/RVEDV were compared between CDH survivors and non-survivors. Receiver-operating characteristic curve analysis was performed to assess the predictive ability for mortality of the echocardiographic parameters. RESULTS: Comparing CDH non-survivors (n = 6) with survivors (n = 29), respectively, RVEDV/BBW was significantly larger (2.54 ± 0.33 vs 1.86 ± 0.35 ml/kg; P < 0.01), LVEDV/BBW was significantly smaller (0.86 ± 0.21 vs 1.22 ± 0.33 ml/kg; P < 0.001), and LVEDV/RVEDV was significantly lower (0.34 ± 0.06 vs 0.66 ± 0.18; P < 0.001). The area under the curve for LVEDV/RVEDV was the largest (0.98). CONCLUSIONS: Three-dimensional echocardiographic volume imbalance between the RV and LV was remarkable in CDH non-survivors. The LVEDV/RVEDV ratio may be associated with mortality in CDH. IMPACT: Mortality with congenital diaphragmatic hernia (CDH) is high, and evaluating left and right ventricular structures and functions may be helpful in assessing the prognosis. Three-dimensional (3D) echocardiography indicated that the left ventricular end-diastolic volume/right ventricular end-diastolic volume ratio within 24 h after birth was associated with mortality in CDH infants. The usefulness of this ratio should be validated in prospective multicenter studies involving larger numbers of patients.

Changes in regional oxygen saturation of the kidney and brain of infants during hospitalization.

BACKGROUND: In pre-term infants, the postnatal changes in the regional oxygen saturation (rSO2) of the brain and kidney are unclear. METHODS: We performed a prospective observational study. We measured the cerebral/renal rSO2 ratio and recorded the associated clinical features of infants born at 23 to 41 weeks of gestation weekly from the early postnatal period to discharge. RESULTS: The median cerebral/renal rSO2 ratios (interquartile ranges) between birth and the expected date of birth were 1.13 (1.06-1.26) at 23-24 weeks (n = 7), 1.18 (1.10-1.32) at 25-26 weeks (n = 11), 1.24 (1.11-1.37) at 27-28 weeks (n = 9), 1.12 (1.05-1.19) at 29-30 weeks (n = 4), 1.11 (1.03-1.15) at 31-32 weeks (n = 5), 1.02 (0.98-1.06) at 33-34 weeks (n = 9), 0.98 (0.94-1.06) at 35-36 weeks (n = 19), and 0.95 (0.86-0.99) at 37-41 weeks of gestation (n = 22). The median cerebral/renal rSO2 ratio did not significantly change after birth, but with increasing gestational age, the cerebral/renal rSO2 ratio at the expected date of birth decreased (r = - 0.74, p < 0.001). Nephrotoxic drugs did not affect cerebral/renal rSO2 at the expected date of birth, after adjustment for clinical factors. CONCLUSIONS: Unlike in most infants born after the late pre-term period, the renal rSO2 remained lower than the cerebral rSO2 on the expected date of birth in infants born very pre-term.

Cytomegalovirus-related sepsis-like syndrome in very premature infants in Japan.

BACKGROUND: Very premature infants are at high risk of developing a symptomatic postnatal cytomegalovirus (CMV) disease, such as CMV-related sepsis-like syndrome (CMV-SLS). To address the limited data regarding its clinical features, a nationwide survey of CMV-SLS was conducted. METHODS: A questionnaire regarding CMV status and the clinical outcomes of CMV-SLS was sent to centers with reported cases of CMV-SLS. RESULTS: Twelve CMV-SLS cases, nine confirmed and three probable cases, were reported during the 3-year survey period. The median gestational age and birthweight were 25 weeks and 547 g, respectively. At disease onset, the median age was 49 days, and the corrected age was 31 weeks. Untreated breast milk was given in four cases (33%), whereas frozen breast milk was given in nine (75%). No specific symptoms and laboratory data regarding CMV-SLS were found. CONCLUSIONS: Very premature infants developed CMV-SLS after 1 month of age. There are no symptoms and signs specific for the diagnosis of CMV-SLS, so CMV-SLS should be considered as a differential diagnosis for premature infants who have unexplained sepsis-like symptoms during the convalescent phase.

Acute effect of hydrocortisone for respiratory deterioration in preterm infants: Oxygenation, ventilation, vital signs, and electrolytes.

BACKGROUND: Preterm infants with severe bronchopulmonary dysplasia require rescue therapy with glucocorticoids, and hydrocortisone is increasingly replacing dexamethasone. The standard for rescue therapy is unclear. AIM: To quantify the short-term effects of respiratory rescue hydrocortisone of 4 mg/kg/day for 3 days. STUDY DESIGN: Retrospective single-center study. SUBJECTS: Ventilator-dependent infants born at <28 weeks of gestation with an increased oxygen demand to maintain the target oxygen saturation at 88% to 95% >1 week after birth. OUTCOME MEASURES: Ventilator settings, SpO2/FiO2 ratio, heart rate, and blood parameters within 24 h before and 228 h after starting hydrocortisone. RESULTS: Twenty-five infants (median gestational age, 25.1 weeks) received hydrocortisone at a median age of 16 days. The median pre-therapy SpO2/FiO2 was 297 (interquartile range, 265-320) and began to rise after 12 h of administration, reaching 307 (interquartile range, 278-335). The increase in SpO2/FiO2 peaked from the third day to 3 days after therapy (median range, 341-356). SpO2/FiO2 decreased thereafter and remained unchanged from 6 and 7 days after therapy (median range, 304-314). The pCO2 level (median range, 49-53 mmHg) did not change significantly. The heart rate significantly decreased from -4 to -6 beats/min from the first day to 1 day after therapy. Systolic blood pressure increased by a median of 4 to 8 mmHg after therapy. Blood electrolytes and glucose were similar after therapy. CONCLUSION: Rescue hydrocortisone administration improved oxygenation without particular adverse effects at the stage of respiratory deterioration in preterm infants.

Clinical features of very-low-birthweight infants with congenital heart disease.

BACKGROUND: Few studies have investigated the developmental prognosis of very-low-birthweight (VLBW) infants with congenital heart diseases (CHDs). This study aimed to determine the mortality and morbidity, including the developmental prognosis, of VLBW infants with CHD. METHODS: This single-center, retrospective cohort study included VLBW infants admitted to the neonatal intensive care unit from January 2006 to December 2011. Perinatal records were reviewed for CHD diagnosis, treatment details, comorbidities, mortality, and long-term neurodevelopmental outcomes. The characteristics and neurological developmental quotients at around the age of 3 years were compared among the following three groups of VLBW infants with CHDs: biventricular circulation without intervention (without surgery), biventricular circulation with intervention (catheter intervention or one-stage surgery), and single-ventricular circulation (Fontan-type multiple-stage surgery). RESULTS: Among a total of 449 VLBW infants admitted during this period, 45 (10.0%) infants had CHDs, including 25 infants with congenital abnormalities (chromosomal abnormalities and/or multiple anomalies). All 13 infants who died before discharge had congenital abnormalities. The incidence rates of comorbidities were not higher in VLBW infants with CHDs than in those without CHDs. The developmental quotients of the no-surgery, catheter intervention or one-stage surgery, and Fontan-type multiple-stage surgery groups were 87.2 ± 10.9, 91.3 ± 4.7, and 63.7 ± 8.6, respectively. CONCLUSIONS: The neurological development at around the age of 3 years in VLBW infants with biventricular circulation was in the borderline-to-normal range; however, that in infants with single-ventricular circulation was poor. Further studies are needed to comprehend the neurological development of VLBW infants with CHDs better.

Acute respiratory effect of transpyloric feeding for respiratory exacerbation in preterm infants.

OBJECTIVES: Gastroesophageal reflux may exacerbate chronic lung disease in preterm infants. We evaluated the short-term effects of transpyloric feeding on respiratory status in preterm infants during mechanical ventilation. METHODS: We retrospectively collected data from the hospital information management system. To evaluate the effect of transpyloric feeding on oxygenation, we compared changes in SpO2/FiO2 ratios before and after commencing transpyloric feeding by a piecewise linear regression model. RESULTS: We examined 33 infants (median gestational age, 25.4 weeks; median birth weight, 656 g) who underwent transpyloric feeding. All tubes were placed at the bedside without fluoroscopy. No cases of unsuccessful placement, gastroduodenal perforation, or tracheal misinsertion occurred. Transpyloric feeding began at a median age of 18 (interquartile range, 15-23) days. Mean SpO2/FiO2 (±SD) ratios were 391 (±49), 371 (±51), 365 (±56), and 366 (±53) 72-96 h before, 0-24 h before, 48-72 h after, and 96-120 h after starting transpyloric feeding, respectively. The rate of change per hour of SpO2/FiO2 ratios increased 48-120 h after compared with 0-96 h before transpyloric feeding (0.03 [95% confidence interval, -0.10 to 0.17] vs. -0.29 [-0.47 to -0.12]) (p=0.007). No apparent changes occurred in the mean airway pressure, amplitude pressure, or pCO2. CONCLUSIONS: Transpyloric feeding during mechanical ventilation can prevent the deterioration of oxygenation without major complications at the stage of respiratory exacerbation in preterm infants.

Clinical Presentation of Preterm Infants with Ventricular Septal Defect.

Ventricular septal defects (VSDs) are the most common congenital heart diseases; however, case reports of preterm infants with VSD are limited. The aim of this study is to share our experience with preterm infants with VSD and to record their short-term outcomes. Between January 2000 and December 2017, 32 preterm infants with VSD were admitted to our neonatal intensive care unit at gestational age < 32 weeks. Of these, 9 were excluded by exclusion criteria. The size and location of the VSD, details of treatment, and neonatal prognosis were retrospectively reviewed from the medical records. Among the 23 preterm infants, the median gestational age was 29.4 weeks (25.0-31.3 weeks) and the median birthweight was 924 g (524-1,526 g). There were 9 infants with VSD < 2 mm and 14 infants with VSD ≥ 2 mm. For the 9 infants with VSD < 2 mm, no medical or surgical treatments for VSDs were undertaken. Of the 14 infants with VSD ≥ 2 mm, 8 (57.1%) underwent medical and surgical treatment. Surgical treatment was performed more frequently in infants with VSD ≥ 2 mm than in those with VSD < 2 mm (P = 0.007). In preterm infants, the presence of VSD ≥ 2 mm increases the risk of surgical interventions and significant patent ductus arteriosus. It is important to encourage treatment for preterm infants with VSD ≥ 2 mm, including surgical interventions, in cooperation with pediatric cardiologists.

Ultrasound-Guided Umbilical Venous Catheter Insertion With Alignment of the Umbilical Vein and Ductus Venosus.

Previous studies have highlighted the importance of confirming the position of an umbilical venous catheter (UVC) tip by an ultrasound (US) examination. However, methods for preventing insertion into the portal circulation under US guidance have not yet been established. We report 15 cases in which a UVC was successfully passed through the ductus venosus by compressing the upper abdomen near the portal sinus of the liver to align the umbilical vein and ductus venosus under US guidance. The UVC was inserted into the correct position in 14 of the 15 neonates (93%) without complications.

Reliability of real-time continuous glucose monitoring in infants.

BACKGROUND: Neonatal hypoglycemia is a common and treatable risk factor for neurological impairment. Real-time continuous glucose monitoring (RT-CGM) can show glucose concentration in real time. Using an RT-CGM alarm, physicians can be alerted and intervene in hypoglycemia. No reports, however, have evaluated the reliability of RT-CGM at low glucose levels in infants. This study therefore investigated the difference between blood glucose (BG) and RT-CGM sensor data at low glucose levels and assessed the optimum method of using a hypoglycemic alarm in infants. METHODS: We enrolled infants whose glycemic management was difficult. We calculated the mean absolute difference (MAD) and mean absolute relative difference (MARD) between BG and RT-CGM sensor data. We compared the MAD and MARD between the low BG fluctuation and high BG fluctuation groups. RESULTS: We used RT-CGM for 12 patients (29 times) and investigated 448 pairs of BG and RT-CGM sensor data. The MAD between these pairs was 9.3 ± 8.9 mg/dL, and the MARD was 11.5%. The MAD at low glucose was 7.7 ± 6.0 mg/dL, and the MARD was 16.2%. The MAD and MARD were 6.8 ± 5.4 mg/dL and 7.8% in the low fluctuation group and 10.1 ± 9.5 mg/dL and 12.7% in the high fluctuation group, respectively. CONCLUSIONS: The difference between BG and RT-CGM sensor data changes with the degree of fluctuation in BG. When physicians set the hypoglycemic alarm, consideration of this difference and a change in the alarm setting according to the degree of fluctuation in BG may be useful.

Blood potassium and urine aldosterone after doxapram therapy for preterm infants.

OBJECTIVE: We often encounter infants who developed hypokalaemia following low-dose doxapram for apnea of prematurity (AOP). AIMS: To determine changes in blood potassium (K+) levels after doxapram administration. STUDY DESIGN: We studied infants born before 30 weeks gestation. Doxapram (0.1-0.3 mg/kg/h) in addition to methylxanthines was used to treat AOP refractory to methylxanthines. RESULTS: Twenty-five infants received doxapram were studied. Fifty-two percent developed hypokalemia (<3.0 mEq/L) during doxapram administration. Time after starting doxapram to nadir blood K+ (<3.0 mEq/L) level was 11 days. Blood K+ levels normalized after 5 days of stopping doxapram administration. Data at 10 days before and after and at the time of doxapram administration were, respectively: lowest blood K+ level: 3.9, 3.0, and 3.6 mEq/L; urine aldosterone: 90, 206, and 146 pg/µg creatinine. Blood pH, blood pressure and urine volume were similar. CONCLUSIONS: Doxapram-induced hypokalemia may be due to an inappropriate increase in aldosterone levels.

Association between cord blood cystatin C levels and early mortality of neonates with congenital abnormalities of the kidney and urinary tract: a single-center, retrospective cohort study.

BACKGROUND: Some fetuses with congenital abnormalities of the kidney and urinary tract (CAKUT) have severe renal dysfunction during the prenatal period that can result in oligohydramnios, pulmonary hypoplasia, and death following birth. We hypothesized that cord blood cystatin C (CysC) levels are elevated in neonates who have life-threatening pulmonary hypoplasia and oligohydramnios due to severe renal dysfunction. In this study we compared cord blood CysC levels between a non-survivor group with CAKUT and a survivor group. METHODS: This was a single-center, retrospective cohort study conducted between January 2007 and December 2015. Eighty-seven neonates who were prenatally diagnosed with CAKUT were included in the study. Cord blood CysC and creatinine levels were compared between the survivor and non-survivor groups at discharge from hospital. RESULTS: Of the 87 neonates enrolled in the study, 67 survived and 21 died before discharge. Median cord blood CysC levels were higher in the non-survivor group than in the survivor group (4.28 vs. 1.96 mg/L, respectively; p < 0.001). Cord blood creatinine levels were not significantly different between the two groups. In patients with oligohydramnios (n = 28), cord blood CysC levels were significantly higher in the non-survivor group than in the survivor group (4.28 vs. 2.23 mg/L, respectively; p = 0.002). CONCLUSIONS: In this study population, cord blood CysC levels were significantly higher in the non-survivor group with CAKUT than in the survivor group. These results suggest that cord blood CysC levels may be a good marker of the severity of renal dysfunction at birth.

Temporal Relationship between Serum Levels of Interleukin-6 and C-Reactive Protein in Therapeutic Hypothermia for Neonatal Hypoxic-Ischemic Encephalopathy.

Objective C-reactive protein (CRP) is a useful marker of neonatal infection. Recent studies have shown that neonatal therapeutic hypothermia delays an elevation of CRP in infants with hypoxic-ischemic encephalopathy (HIE). This study investigated the time difference of peak levels of serum CRP and other inflammatory responses during therapeutic hypothermia. Study design We prospectively studied the serial serum data of CRP, interleukin-6 (IL-6), procalcitonin (PCT), and complete blood counts during the first week of life in HIE infants receiving therapeutic hypothermia. Results We identified 22 infants who received therapeutic hypothermia between August 2013 and July 2015. No infants developed clinically overt infections. The peak of serum levels of IL-6, PCT, and CRP were postnatal days 1, 2, and 4, respectively. White blood cells, neutrophils, and platelet counts gradually decreased from days 1 to 7. Early postnatal serum levels of IL-6 correlated with CRP on day 4 (IL-6 on day 2; r = 0.78, p < 0.001). Conclusion The peak value of CRP on day 4 might reflect the early production and secretion of IL-6 rather than an actual infection. Serial measurement of IL-6 might help avoid invasive sepsis workup and unnecessary change of antibiotics in infants.

Genotype-phenotype relationship in Japanese patients with congenital central hypoventilation syndrome.

Examine the genotype-phenotype relationship in Japanese congenital central hypoventilation syndrome (CCHS) patients and estimate the incidence of CCHS in Japan. Subjects were 92 Japanese patients with PHOX2B mutations; 19 cases carried 25 polyalanine repeat expansion mutations (PARMs); 67 cases carried 26 or more PARMs; and 6 had non-PARMs (NPARMs). We collected clinical data in all patients and estimated the development or intelligent quotients only in the patients carrying 25 PARM. The estimated incidence of CCHS was greater than one case per 148 000 births. Polyhydramnios was observed in three cases. Twelve infants exhibited depressed respiration at birth. In 19 cases carrying 25 PARM, the male-to-female ratio was ~3, no cases had Hirschsprung disease; 7 cases (37%) developed hypoventilation after the neonatal period, and 8 cases (42%) had mental retardation. In other 73 cases carrying 26 or more PARMs or NPARMs, male-to-female ratio was equal; patients frequently complicated with Hirschsprung disease and constipation, and all patients presented with hypoventilation in the neonatal period. Clinical symptoms were severe in most patients carrying long PARMs and NPARMs. In 25 PARM, additional genetic and/or epigenetic factors were required for CCHS development and male sex is likely a predisposing factor. The patients carrying 25 PARM frequently had mental retardation likely because they were not able to receive appropriate ventilation support following a definitive diagnosis owing to subtle and or irregular hypoventilation. Molecular diagnosis provides a definitive diagnosis and enables to receive appropriate ventilator support.

Increased Incidence of Late-Onset Circulatory Collapse after Changing Clinical Practice: A Retrospective Investigation of Causative Factors.

OBJECTIVE: Acute primary profound circulatory failure responsive to glucocorticoid therapy after the first week of age in preterm infants is termed late-onset circulatory collapse (LCC). This study was performed to identify factors that notably increased the incidence of LCC after various management practices were changed. STUDY DESIGN: We retrospectively studied the clinical characteristics of infants (<29 weeks' gestation) before (n=26) and after (n=35) implementing the following practice changes: stress reduction, conservative replacement of thyroid hormone, positive antenatal glucocorticoid administration, sedation with fentanyl (<7 days after birth), and hydrocortisone therapy for hypotension. RESULTS: After the aforementioned changes, the incidence of LCC increased from 4 to 43%, and that of intraventricular hemorrhage decreased from 42 to 9%. Antenatal glucocorticoids (75 vs. 20%), fentanyl (94 vs. 53%), and hydrocortisone (63 vs. 31%) (<2 weeks of age) were given to infants with LCC and non-LCC. After the practice changes, infants with LCC had lower serum sodium levels than did infants without LCC at 7 to 14 days of age. CONCLUSION: Relative hyponatremia was an early sign of imminent LCC. In addition to adrenal prematurity, the antenatal administration of glucocorticoids and fentanyl, which influence adrenal function, might increase the incidence of LCC.

Premature rupture of membranes and neonatal respiratory morbidity at 32-41 weeks' gestation: a retrospective single-center cohort study.

AIM: To ascertain whether premature rupture of membranes (PROM) independently affects the risk of neonatal respiratory morbidity at 32-41 weeks' gestation because previous reports have given insufficient consideration to the mode of delivery and labor onset. METHODS: Data on 4,629 consecutive singleton infants were retrospectively collected. Respiratory morbidity was limited to respiratory distress syndrome and transient tachypnea of the newborn, both of which are related to prematurity. Delivery modes were divided into four groups based on the existence of PROM and of labor onset, and the respiratory morbidity was examined according to the number of weeks of gestational age. Multivariate analysis including PROM and delivery mode was conducted to examine the association of respiratory morbidity. RESULTS: Respiratory morbidity or a positive pressure requirement delivered after PROM and intact amniochorionic membranes accompanied by labor were similar at all weeks. Around 37 weeks, the absence of labor onset was associated with a risk of respiratory morbidity or positive pressure requirement. Significant respiratory risk was not associated with the incidence of PROM (adjusted odds ratio [aOR], 0.98; 95% confidence interval [CI], 0.52-1.83), interval from rupture to delivery (aOR, 1.00; 95% CI, 0.99-1.01), clinical chorioamnionitis, induction management, pregnancy-related complications, or neonatal sex. Delivery by Cesarean section and early gestational age presented a significant risk for respiratory morbidity. CONCLUSIONS: Neither PROM nor latency after PROM at 32-41 weeks affected neonatal respiratory morbidity. Avoiding Cesarean section instead of simply increasing the time to delivery may help to reduce respiratory morbidity.