Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Recently, the role of a rapid increase in serum osmolality in the inhibition of postnatal ductal closure has garnered attention. This study evaluated the efficacy of high-humidity care in preventing the onset of patent ductus arteriosus (PDA) in extremely premature infants. METHODS AND RESULTS: The high-humidity group (HHG) comprised 28 infants (240to 276weeks gestational age) recruited prospectively within 6 h after birth between July 2019 and September 2021; these infants were cared for in 90% humidity for the first 72 h of life. The incidence of PDA within the first 7 days of life and the rate of increase in serum sodium concentrations were compared between the HHG and a conventionally managed historical control group (CG; 29 infants born in 2016-2017). Twelve (43%) infants in the HHG and 22 (76%) in the CG developed PDA (P=0.016). Multivariate logistic regression analysis revealed that high-humidity care was effective in reducing the incidence of PDA onset (odds ratio 0.265; 95% confidence interval 0.078-0.907). The rate of increase in serum sodium concentrations was significantly lower in the HHG than CG (median 0.29 [interquartile range 0.21-0.39] vs. 0.46 [interquartile range 0.32-0.62] mEq/L/h, respectively; P<0.001). CONCLUSIONS: High-humidity care for the first 72 h of life may help reduce the onset of PDA in extremely preterm infants by avoiding rapid increases in serum sodium concentrations.

DOI： 10.1253/circj.CJ-24-0705

PubMed

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Georg Thieme Verlag KG  

Objective Liver evaluation is essential in preterm infants because of exposure to hepatotoxic drugs, the effects of parenteral nutrition, and their organ immaturity. The clinical significance of shear wave elastography (SWE) which measures tissue elasticity, is unclear in preterm infants. For SWE application to liver evaluation in preterm infants, we examined the postnatal course and factors associated with changes.

Study Design We prospectively measured liver SWE values every other week in 37 preterm infants born at 23 to 35 weeks gestation and 12 term infants born after 36 weeks gestation.

Results The median early postnatal liver SWE value was 1.22 (interquartile range, 1.19–1.26) m/s. The correlations of liver SWE values with gestational age and birth weight were r = −0.18 (p = 0.23) and r = −0.21 (p = 0.157), respectively. The median liver SWE values from birth to 36 to 38 postmenopausal weeks were 1.22 (1.17–1.24) m/s at &lt;28 weeks gestation (n = 9), 1.21 (1.18–1.25) m/s at 28 to 29 weeks gestation (n = 11), 1.24 (1.21–1.28) m/s at 30 to 31 weeks gestation (n = 8), and 1.21 (1.20–1.24) m/s at ≥32 weeks gestation (n = 9). There was no change over time in any gestational age group (p = 0.158). The median liver SWE values were 1.22 (1.17–1.25) m/s (n = 10) and 1.22 (1.19–1.25) m/s (n = 27) for small- and appropriate-for-gestational-age infants, respectively (p = 0.93). The correlations of abnormally high serum concentrations of direct bilirubin (&gt;1.0 mg/dL) and alanine aminotransferase (&gt;12 IU/L) with liver SWE values were r = 0.37 (p = 0.041) and r = 0.21 (p = 0.35), respectively.

Conclusion Liver SWE values may be useful for the evaluation of liver damage with cholestasis in preterm infants because they remain constant regardless of gestational age and birth weight and do not change over time or with a deviation of body size.

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File： AJP_24oct0619最終版.pdf

DOI： 10.1055/a-2490-3259

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Language：English   Publishing type：Research paper (scientific journal)  

Nail-patella syndrome (NPS) is a hereditary disease caused by pathogenic variants in LMX1B and characterized by nail, limb, and renal symptoms. This study revealed a likely pathogenic LMX1B variant, NM_002316.4: c.723_726delinsC (p.Ser242del), in Japanese twins with clubfoot. The patients' mother, who shared this variant, developed proteinuria after delivery. p.Ser242del is located in the homeodomain of the protein, in which variants that cause renal disease tend to cluster. Our findings highlight p.Ser242del as a likely pathogenic variant, expanding our knowledge of NPS.

DOI： 10.1038/s41439-024-00266-z

PubMed

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Language：English  

Vitamin K deficiency bleeding (VKDB) in neonates is a significant disorder that causes skin, gastrointestinal, and intracranial hemorrhaging. Early-onset VKDB occurs within 24 hours of birth, and its prognosis is poor due to severe hemorrhage. The causes of early-onset VKDB include maternal intake of warfarin and anticoagulants, and maternal vitamin K deficiency. We report the case of a neonate with early-onset VKDB born to a mother with Crohn's disease. The neonate developed severe cerebellar hemorrhage on the day of birth and subsequent noncommunicating hydrocephalus requiring a ventriculoperitoneal shunt. The mother had a 14-year history of Crohn's disease and short bowel owing to intestinal resection. She was in complete remission during pregnancy according to the Crohn's Disease Activity Index. Endoscopic examination performed shortly before pregnancy revealed inflammatory findings in the residual small intestine. Her blood tests at delivery showed an elevated prothrombin induced by vitamin K deficiency or antagonist II (PIVKA-II) level of 26,900 mAU/mL. A definitive protocol to prevent early-onset VKDB in mothers with Crohn's disease complicated by a short bowel is lacking. Administering vitamin K to mothers with elevated PIVKA-II levels before delivery may help prevent early-onset VKDB.

DOI： 10.1055/a-2219-5024

PubMed

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Language：Japanese   Publisher：(一社)日本輸血・細胞治療学会  

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Language：Japanese   Publisher：神奈川県医師会  

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Language：Japanese   Publisher：(一社)日本新生児成育医学会  

J-GLOBAL

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Language：Japanese   Publisher：(一社)日本周産期・新生児医学会  

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Language：Japanese   Publisher：神奈川県医師会  

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Language：Japanese   Publisher：(一社)日本周産期・新生児医学会  

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Language：Japanese   Presentation type：Poster presentation  

File： 2012年未熟児新生児学会学術集会ポスター完成版.pdf

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Language：Japanese   Presentation type：Symposium, workshop panel (nominated)  

File： 2015年周産期新生児学会学術集会改.pptx

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Language：Japanese   Presentation type：Oral presentation (general)  

File： 2014年未熟児新生児学会学術集会改.pptx

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Language：Japanese   Presentation type：Oral presentation (general)  

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Language：English   Presentation type：Poster presentation  

File： 2018年Weinstein ポスター 最終版.pdf

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