Effectiveness of ultrasound-guided peripheral arterial cannulation in neonates, including very low birth weight infants who are conventionally difficult-to-cannulate: a case series.

The effectiveness of ultrasound-guided peripheral arterial cannulation (UGPAC) in children has been increasingly been reported. However, to the best of our knowledge, there have been no reports of UGPAC in neonates, including very low birth weight infants (VLBWIs). In this study, we aimed to retrospectively review the results of UGPAC in neonates, including VLBWIs, and assess its effectiveness. This case series was conducted in a tertiary neonatal intensive care unit (NICU) in Japan. We included neonates aged below 28 days who underwent UGPAC in our NICU between April 2021 and October 2022. We extracted the following data from medical records and analysed it retrospectively: patient age (days), postconceptional age, patient weight at the time of cannulation, number of punctures using the conventional technique before ultrasound guidance was performed and number of punctures with the ultrasound-guided technique until successful cannulation. A total of 27 UGPACs were performed in 19 neonates, including 14 cannulations in 10 VLBWIs. In infants weighing > 1500 g and VLBWIs, the success rate within the first three punctures was 100% (13/13) and 79% (11/14), respectively. Overall, 41% (11/27) of UGPACs were performed following failed punctures using conventional methods, with a 100% success rate within the first three attempts. In all cases, no apparent adverse events, such as hypothermia, were noted.  Conclusions: Our results suggest that UGPAC had a high success rate in neonates, including VLBWIs. Further studies are required to compare the effectiveness of UGPAC with conventional methods in neonates. What is Known: • The use of ultrasound guidance for arterial cannulation is recommended in children. • Ultrasound-guided peripheral arterial cannulation (UGPAC) in neonates, including very low birth weight infants (VLBWIs), has not been reported. What is New: • UGPAC in neonates, including VLBWIs, was performed with a high success rate; approximately 40% of UGPACs were performed after the failure of the conventional methods. • This study suggested the effectiveness of UGPAC in neonates, including VLBWIs.

Remodeling of venous drainage after the treatment of dural sinus malformation with arteriovenous shunts in a neonate: a case report and literature review.

Dural sinus malformations (DSMs) are rare congenital vascular diseases characterized by a giant venous pouch with or without arteriovenous shunts. We present a neonatal case of DSM that was diagnosed prenatally and treated via endovascular intervention in the early postnatal period. The patient presented with a large DSM involving the torcular Herophilion prenatal magnetic resonance imaging (MRI). Enlargement of the head circumference and respiratory failure rapidly progressed after birth. On the 5th day after birth, the neonate underwent endovascular occlusion via the umbilical artery. The arteriovenous shunt was occluded, and the reflux from the enlarged venous pouch to the dural sinus was decreased. No additional procedure other than ventriculoperitoneal shunting was required. The neonate's development slowly caught up to normal parameters. Follow-up MRI demonstrated the successful development of the venous drainage system. DSMs are characterized by an abnormally dilated dural sinus, which can block the venous return and ultimately increase intracranial pressure and cerebral ischemia. Long-term follow-up indicates that an abnormally developed dural sinus can be reconstructed by appropriate and timely treatment.

Assessment of Alcohol Exposure From Alcohol-Based Disinfectants Among Premature Infants in Neonatal Incubators in Japan.

Importance: The risk of premature infants in neonatal incubators exposed to evaporated alcohol from alcohol-based disinfectants (ABDs) is unknown. Objective: To assess alcohol concentrations in the peripheral blood of premature infants and neonatal incubators. Design, Setting, and Participants: A quality improvement study comparing 2 different populations before and after introduction of ABD practice (ABD-PRAC) was conducted in a neonatal intensive care unit of a single tertiary hospital in Japan. Participants included premature infants who were born before 34 weeks of gestational age and received medical care in neonatal incubators. The study consisted of 3 periods: (1) September 1, 2020, to August 1, 2021 (prospective observation of pre-ABD-PRAC, (2) August 2 to August 22, 2021 (introduction of ABD-PRAC to medical staff and parents in the neonatal intensive care unit), and (3) August 23, 2021, to March 31, 2022 (prospective observation of post-ABD-PRAC). No follow-up studies were initiated. Interventions: An ABD-PRAC that aimed to reduce alcohol evaporation from ABDs inside neonatal incubators was instituted: (1) place alcohol preps in the incubator just before use and remove them from the incubator as soon as possible and (2) withhold placing hands into the incubators until 60 seconds after using ABDs for disinfection (applied only to family members). Main Outcomes and Measures: Blood alcohol concentration and evaporated alcohol concentrations in neonatal incubators. Results: Disinfectant practice was assessed among 28 infants during the pre-ABD-PRAC (17 infants [10 girls]; median gestational age at birth, 29.4 [IQR, 26.3-30.3] weeks) and post-ABD-PRAC (11 infants [3 girls]; median gestational age at birth, 30.0 [IQR, 25.3-32.2] weeks) study periods. The median blood alcohol concentration was 7.0 (IQR, 5.4-9.3) mg/dL pre-ABD-PRAC and 4.2 (IQR, 2.5-7.2) mg/dL post-ABD-PRAC. The median evaporated alcohol concentration inside neonatal incubators during pre-ABD-PRAC during the day was 23.6 (IQR, 15.9-36.5) ppm and, at night, was 13.2 (IQR, 8.9-19.4) ppm; during post-ABD-PRAC, the concentration was 9.4 (IQR, 6.0-16.0) ppm during the day and 5.7 (IQR, 3.6-9.7) ppm at night. The introduction of ABD-PRAC at 22 weeks' corrected gestational age was associated with a lower blood alcohol concentration in premature infants: regression coefficient value, -8.3 (95% CI, -12.0 to -4.7). Conclusions and Relevance: In this study, alcohol evaporated from ABDs was absorbed by premature infants in neonatal incubators. The findings suggest that introduction of ABD-PRAC was associated with lower alcohol concentrations in neonatal incubators and in the blood of premature infants.

A Neonate with a Diagnosis of Pontocerebellar Hypoplasia Type 6 Treated with Biotin and Developed Biotin Interference with Laboratory Thyroid Function Tests.

BACKGROUND The interference of biotin administration with thyroid function tests has been reported; however, it remains unclear in clinical practice. In this report, we present the case of a neonate with a diagnosis of pontocerebellar hypoplasia type 6 (PCH6) treated with biotin who developed biotin interference with laboratory thyroid function tests. CASE REPORT A 1-day-old male infant with hypothermia, tachypnea, and lactic acidosis had a suspected diagnosis of mitochondrial disease. Biotin and several vitamins were administered to improve his condition. On day 14, his laboratory tests revealed a free triiodothyronine level of 4.7 pg/mL, free thyroxine level of 3.7 ng/dL, thyroid-stimulating hormone level of 0.07 µIU/mL, and thyroid-stimulating hormone receptor antibody (TRAb) level of 37.6 IU/L, suggesting Graves' disease. No goiter or tachycardia developed. The maternal thyroid function was not measured during pregnancy, while the maternal TRAb was negative on the same day. After methimazole administration, the patient's thyroid function normalized, and methimazole was therefore discontinued. All thyroid function tests were conducted using immunoassay methods with avidin and biotin. Later, reduced oxygen consumption under aerobic conditions in skin fibroblasts and compound heterozygous variants of the mitochondrial arginine tRNA synthetase gene were identified, and the patient was diagnosed with PCH6. CONCLUSIONS In this case, the clinical symptoms and physical findings were incompatible with the thyroid function. These laboratory findings could have mimicked Graves' disease due to the biotin interference with immunoassays. Therefore, caution is required when evaluating similar cases.

A novel case of congenital hepatic arterio-veno-portal shunts with umbilical vein aneurysm.

A term male infant presented with congenital hepatic arterio-veno-portal shunts. A mass-like lesion in the left lobe of the liver received blood supply from not only the umbilical vein, but also the hepatic and inferior intrahepatic arteries, communicating with the hepatic and portal veins in a complicated manner, with an umbilical vein aneurysm. The blood flow of the arterio-veno-portal shunts spontaneously and gradually declined from the neonatal period to six years of age. Although mild high-output cardiac failure had developed, no life-threatening events or health problems originating from portosystemic shunts, such as pulmonary artery hypertension and hepatopulmonary syndrome, were observed. However, this report shows that scrupulous follow-up to identify pulmonary artery hypertension and hepatopulmonary syndrome should be continued because complete resolution of the arterio-veno-portal shunts was not obtained in this case.

Nationwide survey of late-onset hemolysis in very low birthweight infants.

BACKGROUND: In Japan, some cases of late-onset acute hemolysis in very low birthweight (VLBW) infants have been reported. These cases had common features but the cause of hemolysis was unknown. The incidence and prognosis of this disease are also unknown. However, there are only few reports of such hemolytic episodes in countries other than Japan. Thus, this study aimed to examine the incidence and clinical course of late-onset acute hemolysis and to establish it as a new disease concept. METHODS: A nationwide prospective survey was conducted from 2011 to 2015 as a rare disease surveillance project of the Japan Society for Neonatal Health and Development. RESULTS: Twenty-four cases were confirmed. The median (range) gestational age, birthweight, and onset of hemolytic episodes were 26 weeks and 2 days (23 weeks and 4 days-31 weeks and 2 days), 898 g (627-1,416 g), and 19 days after birth (9-33 days), respectively. Phototherapy, blood transfusion, and exchange transfusion were required in 22 (96%), 24 (100%), and 7 (29%) cases, respectively. During the observation period, no recurrence of the hemolytic episode occurred. All patients survived; however, one case developed kernicterus and suffered severe neurological sequelae. CONCLUSIONS: In this study, at least 1 out of 1,259 VLBW infants developed hemolysis at 9-33 days after birth in Japan. Owing to the risk of kernicterus, this disease should be recognized as among the important pathological conditions of VLBW infants, suggesting the need to manage jaundice and anemia until 5 weeks after birth.

A Follow-Up Report of an Infant Born to a Mother Receiving Tamoxifen.

Tamoxifen, an estrogen receptor antagonist, is contraindicated in pregnant women due to its teratogenic activity. In the present study, we report the case of an infant whose mother received tamoxifen for breast cancer while unaware of the pregnancy. The infant, born at 29 weeks and 6 days of gestational age with a birth weight of 1664 g, had no congenital anomalies. This case presents detailed information on the development of an infant with placental transfer of tamoxifen. The infant has grown and developed normally throughout a 5-year follow-up period, but long-term vigilance continues.

Neonatal outcomes of very low birthweight infants born to mothers with hyperglycaemia in pregnancy: a retrospective cohort study in Japan.

OBJECTIVE: To examine the mortality and morbidities of very low birthweight (VLBW, <1500 g) infants of mothers with hyperglycaemia in pregnancy. DESIGN AND SETTING: We conducted a retrospective cohort study using data from the Neonatal Research Network of Japan, a nationwide registry of VLBW infants (2003-2012). PATIENTS: We studied 29 626 infants born at 23 to 32 weeks without major congenital anomalies, of which 682 (2.3%) infants were from pregnancies affected by maternal hyperglycaemia. MAIN OUTCOME MEASURES: The primary outcome was hospital mortality. Secondary outcomes were neonatal morbidities and their anthropometric values. Associations between maternal hyperglycaemia and each outcome were observed for the overall period, and statistical tests for interaction were conducted to assess whether they differed before or after the adoption of the International Association of Diabetes in Pregnancy Study Group (IADPSG) guidelines in 2010 for the diagnosis of gestational diabetes mellitus. RESULTS: Overall, hospital mortality (4.1% vs 5.2%), composite outcomes of mortality and severe morbidity (54.2% vs 60%), and anthropometric values were not significantly different between infants of mothers with or without hyperglycaemia in pregnancy. However, the incidence of respiratory distress syndrome (RDS) in VLBW infants from mothers with hyperglycaemia was significantly higher than those from mothers without it only before (relative risk (RR) 1.09, 95% CI 1.00 to 1.19) and not after (RR 0.97, 95% CI 0.83 to 1.11) the adoption of the IADPSG guidelines. CONCLUSION: VLBW infants born to mothers with hyperglycaemia in pregnancy do not seem to be at higher risk of mortality and morbidities, except for RDS only before the adoption of the IADPSG guidelines.

Diagnosis and clinical presentation of hemoglobin Kirksey.

More than 1000 hemoglobin (Hb) variants have been identified. Hb Kirksey, a rare Hb variant involving mutation at codon 94 of α2-globin, is associated with low oxygen affinity. To our knowledge, there is no case report on Hb Kirksey in the literature, and, accordingly, the clinical features of patients with Hb Kirksey are currently unknown. We report here the case of a neonate who presented with asymptomatic low oxygen saturation on pulse oximetry (SpO2 ) just after birth, together with clinical analysis of the sister and cousin. Based on the presentations, Hb Kirksey does not seem to be associated with clinical abnormalities. Discrepancy between SpO2 and partial pressure of arterial oxygen (PaO2 ) detected on arterial blood gas analysis provided a clue to the diagnosis. Hb variants should be suspected and arterial blood gases should be measured in pediatric cases of unexplained low SpO2 .