A Case of Omphalitis Revealing Alloimmune Neonatal Neutropenia.

Neutropenia, characterized by a decrease in peripheral blood neutrophil count less than 1500/µL, poses significant clinical challenges due to its association with recurrent infections. This paper presents a rare and intriguing case of alloimmune neonatal neutropenia (ANN), an uncommon variant of neutropenia instigated by the transplacental transfer of maternal anti-neutrophil antibodies that consequently induce opsonization and phagocytosis of the neonate's neutrophils within the reticuloendothelial system. The patient, an 18-day-old boy, was born at 36 weeks five days of gestation and weighed 2465 g, an attribute considered appropriate for gestational age (AGA). He experienced multiple episodes of skin and respiratory infections, coupled with delayed umbilical cord separation and demonstrated a significant reduction in neutrophil count. Despite these symptoms, the patient did not develop bacteremia and his condition improved with antibiotic therapy, leading to his discharge from the hospital. Crucially, both the patient and his mother tested positive for anti-HNA (human neutrophil alloantigen)-1a and anti-HNA-1b antibodies, indicative of a diagnosis of ANN. ANN is intriguing in its clinical course, where despite neutropenia, severe infections are relatively uncommon, and the majority of cases resolve spontaneously within several months post-birth as the maternal antibodies diminish. Nevertheless, there have been reports of moderate to severe infections, demanding clinical intervention and close patient monitoring. The patient in our case was treated with prophylactic antibiotics for six weeks, until a rise in neutrophil count was confirmed, stemming from the severity and recurrence of infections. The issue of using antibiotics and granulocyte colony-stimulating factor (G-CSF) agents in the treatment of ANN remains contentious, with contrasting reports regarding their efficacy and safety. The balance between the prospective therapeutic advantages, potential risks such as antibiotic resistance, and the possibility of inducing leukemia with long-term administration of G-CSF agents necessitates meticulous deliberation. This case underscores the crucial role of early recognition of ANN in neonates presenting with neutropenia. Prompt diagnosis enables a more targeted approach to treatment, reduction in unnecessary antibiotic administration, and specific testing, thus impacting the overall patient management and potentially improving outcomes. Furthermore, in the event of delayed umbilical cord separation in neonates, healthcare providers should consider ANN and other immunodeficiencies related to neutrophil functional abnormalities as potential diagnoses. This patient's story accentuates the need for further investigations to elucidate the precise etiology and pathogenesis of ANN, paving the way for improved diagnostic tools and effective therapeutic strategies.

A Case of Neonate with Split Cord Malformation Presenting with Hypoplasia of the Left Lower Extremity.

The frequency of split cord malformation (SCM) is approximately 1 in 5000 births; however, patients are rarely diagnosed with SCM in the neonatal period. Moreover, there have been no reports of SCM with hypoplasia of the lower extremities at birth. A 3-day-old girl was transferred to our hospital for a thorough examination of hypoplasia of the left lower extremity and lumbosacral abnormalities detected after birth. The spinal magnetic resonance imaging (MRI) revealed a split spinal cord in a single dural tube. Based on the MRI findings, the patient was diagnosed with SCM type II. Following discussions with the parents, pediatricians, neurosurgeons, psychologists, and social workers, we decided to perform untethering to prevent further neurological impairment after achieving a sufficient body weight. The patient was discharged on day 25 of life. Early diagnosis and intervention may improve the neurological prognosis in terms of motor function, bladder and bowel function, and superficial sensation; thus, clinicians should report infrequent findings that may lead to SCM diagnosis. SCM should be differentiated in patients with left-right differences in the appearance of the lower extremity, particularly in those with lumbosacral abnormalities.

Skeletal Muscle Insulin Resistance in a Novel Fetal Growth Restriction Model.

The abnormal fetal environment exerts long-term effects on skeletal muscle, and fetal growth restriction (FGR) is associated with insulin resistance in adulthood. In this study, we examined insulin resistance in early adulthood and insulin signaling in skeletal muscle using a novel FGR rat model. Ameroid constrictors (AC) were placed on the bilateral uterine and ovarian arteries of rats on day 17 of gestation; placebo surgery was performed on the control group. We measured body weight at birth, 4, 8, and 12 weeks of age and performed oral glucose tolerance tests at 8 and 12 weeks. Rats were dissected at 12 weeks of age. We examined the mRNA and protein expression of insulin signaling pathway molecules in skeletal muscle. FGR rats had a significantly lower birth weight than control rats (p = 0.002). At 12 weeks of age, the incremental area under the curve of blood glucose was significantly higher, and GLUT4 mRNA and protein expression in soleus muscle was significantly lower in the FGR group than in the control group. Reduced placental blood flow in the AC-attached FGR group caused insulin resistance and altered insulin signaling in skeletal muscles. Therefore, FGR causes skeletal muscle insulin resistance in early adulthood.

A novel mutation in the SLCO2A1 gene, encoding a prostaglandin transporter, induces chronic enteropathy.

Chronic enteropathy associated with SLCO2A1 gene (CEAS) is caused by loss-of-function mutations in SLCO2A1, which encodes a prostaglandin (PG) transporter. In this study, we report a sibling case of CEAS with a novel pathogenic variant of the SLCO2A1 gene. Compound heterozygous variants in SLCO2A1 were identified in an 8-year-old boy and 12-year-old girl, and multiple chronic nonspecific ulcers were observed in the patients using capsule endoscopy. The splice site mutation (c.940 + 1G>A) of the paternal allele was previously reported to be pathogenic, whereas the missense variant (c.1688T>C) of the maternal allele was novel and had not yet been reported. The affected residue (p.Leu563Pro) is located in the 11th transmembrane domain (helix 11) of SLCO2A1. Because SLCO2A1 mediates the uptake and clearance of PGs, the urinary PG metabolites were measured by liquid chromatography coupled to tandem mass spectrometry. The urinary tetranor-prostaglandin E metabolite levels in the patients were significantly higher than those in unaffected individuals. We established cell lines with doxycycline-inducible expression of wild type SLCO2A1 (WT-SLCO2A1) and the L563P mutant. Immunofluorescence staining showed that WT-SLCO2A1 and the L563P mutant were dominantly expressed on the plasma membranes of these cells. Cells expressing WT-SLCO2A1 exhibited time- and dose-dependent uptake of PGE2, while the mutant did not show any uptake activity. Residue L563 is very close to the putative substrate-binding site in SLCO2A1, R561 in helix 11. However, in a molecular model of SLCO2A1, the side chain of L563 projected outside of helix 11, indicating that L563 is likely not directly involved in substrate binding. Instead, the substitution of Pro may twist the helix and impair the transporter function. In summary, we identified a novel pathogenic variant of SLCO2A1 that caused loss-of-function and induced CEAS.

Long-term renal tubular damage in intrauterine growth-restricted rats.

BACKGROUND: Intrauterine growth restriction (IUGR) has been shown to be associated with increased risk of renal disease or hypertension in later life. Glomerular dysfunction, however, has mainly been reported, and limited information is available to link IUGR with renal tubular damage. The aim of this study was therefore to investigate urinary markers of tubular damage in a rat model of IUGR induced by bilateral uterine artery ligation. METHODS: Pregnant Sprague-Dawley rats underwent bilateral uterine artery ligation, while the control group underwent sham surgery. RESULTS: Birthweight was reduced, and urinary ß2-microglobulin (ß2-MG)-, cystatin C (Cys-C)-, and calbindin-to-creatinine ratios were significantly higher at weeks 4 and 8 in the IUGR group compared with the control group. These urinary markers were not significantly different at week 16 between the two groups. Increased excretion of urinary ß2-MG, Cys-C, and calbindin was observed in IUGR rats at ≥8 weeks of age. CONCLUSION: Children born with IUGR are at increased risk for renal tubular damage.

Clinically silent seizures in a neonate with tuberous sclerosis.

Although seizures during infancy in patients with tuberous sclerosis complex are common, seizures in neonates are infrequent. Here, we report the clinical course and electroencephalography (EEG) findings of a neonate with tuberous sclerosis complex associated with clinically silent seizures. The patient was a girl in whom cardiac tumors were detected on fetal ultrasonography. Brain magnetic resonance imaging during the neonatal period showed subependymal and cortical tubers. Routine EEG indicated unexpected ictal changes with no noticeable clinical symptoms. Ictal EEG was associated with a subtle increase in heart rate and a brief increase in chin electromyogram. These changes were difficult to identify clinically. The patient later developed focal seizures and epileptic spasms and had severe psychomotor delay. The present case suggests the occurrence of clinically silent seizures before the appearance of epileptic spasms in infants with tuberous sclerosis, and that EEG is an option for neonates with a prenatal diagnosis.

Measurement of cytokine levels in cerebrospinal fluid over time in neonatal Enterococcal meningitis.

Enterococcus faecalis is rarely involved in neonatal meningitis. Several studies have indicated that the cytokines related to bacterial infection may induce nerve cell damage; therefore, the cytokine levels in cerebrospinal fluid (CSF) could represent a valuable hallmark for rapid recognition of the disease and evaluation of the degree of neurological involvement. We analyzed cytokine levels in the CSF of a neonate with E. faecalis meningitis over time. Tumor necrosis factor-α (TNF-α) tended to be elevated during the acute phase of infection, and then decreased during the convalescent stage after treatment. CSF inflammatory cytokine measurement may provide important clues for predicting the development of complications in the host because some of these cytokines, such as TNF-α, can injure neurons.

Whole-exome sequencing of a unique brain malformation with periventricular heterotopia, cingulate polymicrogyria and midbrain tectal hyperplasia.

We report a case of an infant with unique and unreported combinations of brain anomalies. The patient showed distinctive facial findings, severe delay in psychomotor development, cranial nerve palsy and seizures. Brain magnetic resonance imaging performed at 5 days of age revealed complex brain malformations, including heterotopia around the mesial wall of lateral ventricles, dysmorphic cingulate gyrus, and enlarged midbrain tectum. The patient unexpectedly died at 13 months of age. Postmortem pathological findings included a polymicrogyric cingulate cortex, periventricular nodular heterotopia, basal ganglia and thalamic anomalies, and dysmorphic midbrain tectum. Potential candidate genes showed no abnormalities by traditional PCR-based sequencing. Whole-exome sequencing confirmed the presence of novel gene variants for filamin B (FLNB), guanylate binding protein family member 6, and chromosome X open reading frame 59, which adapt to the autosomal recessive mode or X-linked recessive mode. Although immunohistochemical analysis confirmed the expression of FLNB protein in the vessel walls and white matter in autopsied specimens, there may be functional relevance of the compound heterozygous FLNB variants during brain development.

Pulmonary artery size as an indication for thoracoscopic repair of congenital diaphragmatic hernia in neonates.

PURPOSE: We reviewed 24 consecutive cases of prenatally or immediately postnatally diagnosed left-sided congenital diaphragmatic hernia (CDH) to evaluate pulmonary artery (PA) size as an indication for thoracoscopic repair (TR). METHODS: CDH repair is planned once echocardiography confirms improvement in pulmonary hypertension. TR is chosen if cardiopulmonary status is stable more than 10 min in the decubitus position in the neonatal intensive care unit (NICU) under conventional mechanical or high frequency oscillatory ventilation (HFOV) with/without nitric oxide (NO) and the patient appears likely to tolerate manual ventilation during transfer to the operating room. Otherwise open repair (OR) is performed in NICU. Proximal right PA (RPA) and left PA (LPA) diameters measured at birth were assessed with respect to the type of repair. RESULTS: 10/24 had TR and 14/24 had OR. TR cases had significantly larger RPA/LPA diameters (3.52 ± 0.23 vs. 3.10 ± 0.56 mm, p < 0.05 for RPA; 3.04 ± 0.26 vs. 2.48 ± 0.37, p < 0.01 for LPA), and significantly less requirement for HFOV (70 vs. 100 %, p < 0.05) and NO (20 vs. 86 %, p < 0.01). Four TR required conversion to OR for technical reasons (n = 3) and cardiopulmonary instability (n = 1). CONCLUSIONS: TR can be considered when RPA/LPA diameters are larger than 3.0/2.5 mm, respectively, and cardiopulmonary status is stable without NO.

A severe form of epidermal nevus syndrome associated with brainstem and cerebellar malformations and neonatal medulloblastoma.

Here we report a boy with epidermal nevus syndrome associated with brainstem and cerebellar malformations and neonatal medulloblastoma. The patient had epidermal nevi and complicated brain malformations including macrocephaly with polymicrogyria, dysmorphic and enlarged midbrain tectum, enlarged cerebellar hemispheres with small and maloriented folia. The patient died after surgical resection of medulloblastoma which was newly recognized on MRI at 51 days of age. Postmortem pathological examinations showed very unique and bizarre malformation of the midbrain and hindbrain. The cerebellar cortex exhibited a coarse, irregular and bumpy surface, blurred border between the Purkinje cell layer and internal granule cell layer, and many foci of heterotopia in the cerebellar white matter. The brainstem showed multiple anomalies, including enlargement of superior colliculi, hypoplasia of pyramidal tracts and dysplasia of inferior olivary nuclei. The unusual constellation of brain malformations of our patient will widen the spectrum of epidermal nevus syndrome.

Increase in fetal pulmonary artery diameters during late gestation is a predictor of outcome in congenital diaphragmatic hernia with liver herniation.

AIM: Liver herniation (LH) in congenital diaphragmatic hernia (CDH) may not be a reliable prognostic indicator. We measured pulmonary artery (PA) diameters in CDH + LH as an alternative. METHODS: Of 41 consecutive cases of prenatally diagnosed left-sided CDH treated from 2002 to 2010, 19 had CDH + LH and 22 had CDH - LH. Ultrasonography and magnetic resonance imaging were used to assess LH and echocardiography to measure PA diameters during the third trimester (fetal; 32-34 weeks), at birth, and on day 2 of life. RESULTS: In CDH + LH survivors (9/19; 47%), fetal right PA (RPA) diameters were significantly larger than in nonsurvivors (2.58 ± 0.56 vs 1.82 ± 0.35 mm; P < .01), but left PA (LPA) diameters were not (1.73 ± 0.38 vs 1.59 ± 0.22). In survivors, fetal RPA was greater than 2 mm in all but one case, and both PA diameters increased significantly by birth (RPA, 2.58 ± 0.56 vs 3.52 ± 0.54; LPA, 1.73 ± 0.38 vs 2.60 ± 0.40; both P < .01). Final diameters at birth in survivors were at least 2.5 and 2.0 mm, respectively. In nonsurvivors, both PAs were significantly smaller (RPA, 3.52 ± 0.54 vs 2.04 ± 0.31; LPA, 2.60 ± 0.40 vs 1.68 ± 0.18; P < .01), with no observed increase by birth. Survival in CDH - LH was 82% (18/22). CONCLUSION: PA diameter appears to be correlated with prognosis in infants with CDH + LH.

Pulmonary artery size has prognostic value in low birth weight infants with congenital diaphragmatic hernia.

AIM: The aim of this study was to examine the relationship between birth weight, pulmonary artery (PA) size, and outcome in congenital diaphragmatic hernia (CDH) to establish if PA size has prognostic value. METHODS: The subjects for this study were 39 consecutive left-sided CDH patients treated at our institution according to the same protocol from 2002 to 2009. Other CDH patients with concurrent anomalies that eventually caused death or who became symptomatic more than 6 h after birth were excluded. Birth weight was used to create two groups; low birth weight (LBW; birth weight ≤ 2,500 g; n = 15) and normal birth weight (NBW; birth weight >2,500 g; n = 24). Right PA (RPA) and left PA (LPA) were measured by echocardiography (EC) during late pregnancy (fetal; gestational age (GA): 32-34 weeks), and on days 0, and 2 of life and compared. RESULTS: Mean birth weights were significantly different between the two groups; however, gender, mean GA, and outcome were similar. In LBW survivors, RPA was significantly larger than in non-survivors. PA size was not related to outcome in NBW. CONCLUSION: We are the first to show that RPA size has prognostic value in low birth weight infants with left-sided CDH.

Effects of parenteral soybean oil lipid emulsion on the long-chain polyunsaturated fatty acid profile in very-low-birth-weight infants.

AIM: Conventional soybean lipid emulsions contain no docosahexaenoic acid (DHA) or arachidonic acid (AA). We investigated the relationship between blood DHA and AA status in 27 very-low-birth-weight (VLBW) infants with or without parenteral lipid emulsion. METHODS: Sixteen infants received parenteral lipid emulsion, and 11 infants were control group. The fatty acid composition of the erythrocyte membrane was analysed at birth and at 2 weeks of age. RESULTS: No significant difference in AA levels was observed in the lipid emulsion group between the two time points, whereas the AA levels at 2 weeks were significantly lower than at birth in the control group. The DHA levels in both groups at 2 weeks were significantly lower than at birth, but no group differences were observed at both time points. CONCLUSION: The use of parenteral soybean oil lipid emulsions in VLBW infants in the postnatal period may prevent the decline in the AA level but does not appear to influence the DHA level.

Indications for thoracoscopic repair of congenital diaphragmatic hernia in neonates.

PURPOSE: We reviewed 26 consecutive cases of congenital diaphragmatic hernia (CDH) prospectively to establish selection criteria for successful thoracoscopic CDH repair (TR). METHODS: Five preoperative deaths were excluded, leaving 21 subjects. TR was only considered once pulmonary hypertension (PH) improved on echocardiography, and if cardiopulmonary status was stable in the decubitus position in the neonatal intensive care unit (NICU) under conventional mechanical or high-frequency oscillatory ventilation (HFOV) with/without nitric oxide (NO) for at least 10 min as a marker for tolerating surgery and manual ventilation was possible for transfer to the operating room. All other patients had open repair (OR). RESULTS: 8/21 had TR and 13/21 had OR. There were significant differences between TR and OR for prenatal diagnosis (37.5 vs. 84.6%, p < 0.05) and earlier surgery (1.4 ± 0.8 vs. 2.5 ± 1.1 days after birth, p < 0.05), respectively. Intraoperative HFOV was required in all OR and 3 TR (p < 0.01). NO was required in 1 TR and 10 OR (p < 0.01). Organ herniation was significantly less in TR (50 vs. 100%, p < 0.01 for stomach; 0 vs. 54%, p < 0.05 for liver). Three TR required conversion to OR because of technical difficulties. One OR died from deteriorating PH. CONCLUSIONS: Our selection criteria for TR would appear to be safe and reasonable.

Altered gene expression in umbilical cord mononuclear cells in preterm infants with periventricular leukomalacia.

AIM: Periventricular leukomalacia (PVL) is one of the most important causes of adverse outcome of preterm infants. We hypothesized that inflammatory or some other specific pathways will have been activated at birth in preterm infants who later develop PVL. The aim of this study is to examine the difference in mRNA expression in umbilical cord blood according to the presence or absence of PVL. METHODS: A total of 61 umbilical cord blood samples were collected from preterm infants with gestational age less than 33 weeks together with the patients' medical information during perinatal period. RNA expression patterns in the collected cord bloods were analyzed by microarray. On the basis of cranial ultrasonography and brain MRI examination, 3 infants (4.9%) were diagnosed as cystic PVL and selected as the subjects. Five patients who showed similar perinatal factors to those of infants with PVL but did not show PVL were selected as the normal control. RESULTS: Five of the 15 up-regulated genes are coding ribosomal proteins, and another encodes a translation elongation factor. Three of the 7 down-regulated genes encode proteins that may be related to immune response and/or inflammation. CONCLUSIONS: Up-regulation of the ribosomal proteins may indicate an activation of lymphocytes during the fetal period.

Association between iron status and neurodevelopmental outcomes among VLBW infants.

OBJECTIVE: Our purpose was to evaluate iron status and neurodevelopmental outcomes in infants with and without extrauterine growth restriction (EUGR). METHODS: This observational study evaluated 38 medically stable premature infants, with birth weights below 1500g. Iron status was determined by measuring venous levels of Hb, Fe, and serum ferritin. The infants were divided into EUGR and non-EUGR groups. At a corrected age of 18months, neurodevelopmental outcomes were checked using the Bayley scales, and body weight, body length, and head circumference were measured. RESULTS: Hb levels at corrected ages of 1 and 3months and iron at a corrected age of 1 and 9months were significantly lower in the EUGR group compared with those of the non-EUGR group. There was no significant difference in the MDI score between the groups, but the PDI score at a corrected age of 18months was significantly lower in the EUGR group. We found a positive correlation between the serum level of Fe at 1month of age and PDI score at 18months of age. Head circumference at a corrected age of 18months did not differ between two groups, although body weight and length were lower in the EUGR group. CONCLUSIONS: Developmental outcome in preterm infants at a corrected age of 18months may be influenced by nutritional factors, including iron status, during their early life.

Estimation of postoperative fat absorption using the 13C mixed-triglyceride breath test in children with choledochal cyst.

No previous studies have focused on postoperative fat malabsorption in children with choledochal cyst (CC) who undergo cyst excision and Roux-en-Y (RY) hepatico-jejunostomy (HJ), a combination of procedures that can lead to the non-physiological mixture of food and bile juice. To examine the effect of RYHJ with cholecystectomy on the fat absorption ability of children with CC, we estimated postoperative fat-absorption ability using the carbon 13-labeled mixed triglyceride (13C-MTG) breath test. Twelve postoperative children with CC and 12 normal control children were administered 13C-MTG orally and asked to give breath samples at six time points: once before the 13C-MTG ingestion and at five 60-min intervals postingestion. Fecal chymotrypsin activity and fecal fat excretion were also measured. The delta value of breath 13CO2 at 3, 4, and 5 h after administration and the 5-h cumulative breath 13CO2 were significantly lower in the CC children than in the controls. There were no significant differences in the fecal chymotrypsin activity or fecal fat excretion of the two groups. Conclusion. Occult fat malabsorption occurs in patients with CC after RYHJ, even in the absence of clinical symptoms or abnormal laboratory data.

Significance of pulmonary artery size and blood flow as a predictor of outcome in congenital diaphragmatic hernia.

AIM: To determine if pulmonary artery size and blood flow have prognostic value in congenital diaphragmatic hernia (CDH). METHODS: Twenty-eight consecutive left-sided CDH patients treated according to a standard protocol with high frequency oscillatory ventilation (HFOV) + nitric oxide (NO) had right and left pulmonary artery (RPA, LPA) diameters, LPA/RPA diameter (L/R) ratios, and PA blood flows examined by echocardiography (EC) on days 0, 2, and 5 after birth and compared prospectively. RESULTS: Twenty-two patients (78.6%) survived. Of these, 15 required NO (NO-s), and seven did not (non-NO-s). All six patients that died required NO (NO-d). RPA in the NO-d group was significantly smaller than in the NO-s or non-NO-s groups on day 0 (2.90 +/- 0.41 vs. 3.40 +/- 0.49 or 4.01 +/- 0.43; P < 0.01, respectively). LPA in the NO-d group was significantly smaller than in the non-NO-s on day 0 (2.13 +/- 0.45 vs. 3.39 +/- 0.34; P < 0.01). L/R ratios in NO subjects were significantly smaller (NO-s 0.74 +/- 0.11; NO-d 0.73 +/- 0.11) than in non-NO-s subjects (0.84 +/- 0.03) on day 0 (P < 0.01). PA diameters and L/R ratios did not change significantly from day 0 to day 5 in all three groups. There was LPA flow on day 0 in all non-NO-s subjects, but none in all NO subjects. In the NO-s group, LPA flow was confirmed in 87% (13/15) on day 2 and in 100% on day 5, however, there was no LPA flow from day 0 to day 5 in any of the NO-d group. CONCLUSION: Our data indicate that PA diameters on day 0 and LPA flow are strongly prognostic in left-sided CDH and L/R ratio would appear to be a simple highly reliable indicator of the necessity for NO therapy.

Management of pulmonary hypertension in congenital diaphragmatic hernia: nitric oxide with prostaglandin-E1 versus nitric oxide alone.

AIM: Prostaglandin-E1 (PGE1) is used at most centers for treating pulmonary hypertension (PH) in congenital diaphragmatic hernia (CDH) because it has been regarded as effective. The aim of this study was to investigate the role of PGE1 for treating PH in CDH. METHODS: We reviewed 49 CDH cases with echocardiography-proven PH. PH was treated with PGE1 and nitric oxide (NO) and high frequency oscillatory ventilation (HFOV) from 1997 to 2001 (PG + NO; n = 19) and with NO and HFOV from 2002 to 2007 (NO; n = 30). RESULTS: Subject demographics, severity of PH, and presence of other anomalies were not significantly different between the two groups. In the PG + NO group, 12/19 (63.2%) survived (PG + NO-s) and 7/19 (36.8%) died (PG + NO-d). In the NO group, 21/30 (70.0%) survived (NO-s) and 9/30 (30.0%) died (NO-d). Survival rates were not significantly different. In the NO-s group, spontaneous closure of the ductus arteriosus (DA) was significantly earlier compared with the PG + NO-s group (P < 0.01; 4.0 +/- 0.9 vs. 9.5 +/- 2.2 days after birth). DA diameters were significantly larger in groups that died compared with groups that survived (P < 0.01), and PH persisted in groups that died. In the NO-s group, surgery was possible significantly earlier compared with the PG + NO-s group (P < 0.01; 3.75 +/- 0.67 vs. 6.12 +/- 0.78 days after birth). No NO-s case developed a PH crisis even though PGE1 was not used. Hospital stay was significantly shorter in the NO-s group compared with the PG + NO-s group (P < 0.05; 39.9 +/- 19 vs. 53.2 +/- 23 days). CONCLUSION: Nitric oxide alone would appear to simplify the management of CDH with PH and provide better outcome.