ASXL1-related Bohring-Optiz syndrome complicated by persistent neonatal pulmonary hypertension and abnormal alveoli formation.

Bohring-Opitz syndrome (BOS) is a rare disease with a characteristic facial appearance and limb position. This report describes a case of BOS complicated by persistent pulmonary hypertension of the newborn (PPHN) and formation of abnormal alveoli that was confirmed by autopsy. A female neonate was born by cesarean section at 37 weeks and 2 days of gestation and found to have a nevus flammeus, exophthalmos, abnormal palate, retraction of the mandible, and a posture characteristic of BOS.

Continuous monitoring using thermography can capture the heat oscillations maintaining body temperature in neonates.

The body temperature of infants at equilibrium with their surroundings is balanced between heat production from metabolism and the transfer of heat to the environment. Total heat production is related to body size, which is closely related to metabolic rate and oxygen consumption. Body temperature control is a crucial aspect of neonatal medicine but we have often struggled with temperature measures.

Impact of hydrogen gas inhalation during therapeutic hypothermia on cerebral hemodynamics and oxygenation in the asphyxiated piglet.

We previously reported the neuroprotective potential of combined hydrogen (H) gas ventilation therapy and therapeutic hypothermia (TH) by assessing the short-term neurological outcomes and histological findings of 5-day neonatal hypoxic-ischemic (HI) encephalopathy piglets. However, the effects of H gas on cerebral circulation and oxygen metabolism and on prognosis were unknown. Here, we used near-infrared time-resolved spectroscopy to compare combined H gas ventilation and TH with TH alone.

Cerebral hemodynamic response during the resuscitation period after hypoxic-ischemic insult predicts brain injury on day 5 after insult in newborn piglets.

Perinatal hypoxic-ischemic brain injury of neonates remains a significant problem worldwide. During the resuscitation period, changes in cerebral hemoglobin oxygen saturation (ScO) have been identified by near-infrared spectroscopy (NIRS). However, in asphyxiated neonates, the relationship between these changes and brain injury is not known.

Cerebral hemodynamics during neonatal transition according to mode of delivery.

Cerebral haemodynamics during the immediate transition period in neonates may differ depending on whether delivery is vaginal or by caesarean section. However, these differences have never been confirmed by near-infrared time-resolved spectroscopy (TRS). Therefore, the purpose of this study was to compare cerebral blood volume (CBV) and cerebral haemoglobin oxygen saturation (ScO) between healthy term neonates by mode of delivery.

Hypothermia cannot ameliorate renal fibrosis after asphyxia in the newborn piglet.

Background: The effects of therapeutic hypothermia (TH) on renal function are not widely reported, especially in longer term animal models. The hypothesis of this study was that TH of the kidneys of hypoxic-ischemic newborn piglets would reduce pathological renal fibrosis.

Methods: Twenty-five newborn piglets obtained within 24 h of birth were classified into a control group (n = 5), an hypoxic insult with normothermia (HI-NT) group (n = 12), and an hypoxic insult with TH (HI-TH) group (33.

Cerebral blood volume increment after resuscitation measured by near-infrared time-resolved spectroscopy can estimate degree of hypoxic-ischemic insult in newborn piglets.

Neonatal hypoxic-ischemic encephalopathy is a notable cause of neonatal death and developmental disabilities. To achieve better outcomes, it is important in treatment strategy selection to categorize the degree of hypoxia ischemia and evaluate dose response. In an asphyxia piglet model with histopathological brain injuries that we previously developed, animals survived 5 days after insult and showed changes in cerebral blood volume (CBV) that reflected the severity of injuries.

Cerebral hemoglobin oxygenation in children with congenital heart disease.

Background: It is important to identify the pathological characteristics of cerebral circulation and oxygen metabolism at the bedside in children with congenital heart disease (CHD) to prevent neurodevelopmental impairments. The brain regional oxygen saturation index (rSO ) can be easily obtained at the bedside with near-infrared spectroscopy and has been widely used in the management of children with CHD in recent years.

Methods: To determine if the rSO before or after CHD surgery is a good predictor of cerebral oxygen metabolism, we investigated the impact of different clinical variables on the correlation between rSO and reference values under steady ratios of hemoglobin oxygen saturation in the internal jugular vein (SjvO ) or femoral artery (SaO ) (0.

Intravenous Edaravone plus Therapeutic Hypothermia Offers Limited Neuroprotection in the Hypoxic-Ischaemic Newborn Piglet.

Background: Therapeutic hypothermia (TH) is a standard therapy for neonatal hypoxic-ischaemic encephalopathy. One potential additional therapy is the free radical scavenger edaravone (EV; 3-methyl-1-phenyl-2-pyrazolin-5-one).

Objectives And Methods: This study aimed to compare the neuroprotective effects of edaravone plus therapeutic hypothermia (TH + EV) with those of TH alone after a hypoxic-ischaemic insult in the newborn piglet.

Hydrogen ventilation combined with mild hypothermia improves short-term neurological outcomes in a 5-day neonatal hypoxia-ischaemia piglet model.

Despite its poor outcomes, therapeutic hypothermia (TH) is the current standard treatment for neonatal hypoxic-ischaemic encephalopathy (HIE). In this study, due to its antioxidant, anti-inflammatory, and antiapoptotic properties, the effectiveness of molecular hydrogen (H) combined with TH was evaluated by means of neurological and histological assessments. Piglets were divided into three groups: hypoxic-ischaemic insult with normothermia (NT), insult with hypothermia (TH, 33.

Relationship between prolonged neural suppression and cerebral hemodynamic dysfunction during hypothermia in asphyxiated piglets.

Objectives: Hypothermia (HT) improves the outcome of neonatal hypoxic-ischemic encephalopathy. Here, we investigated changes during HT in cortical electrical activity using amplitude-integrated electroencephalography (aEEG) and in cerebral blood volume (CBV) and cerebral hemoglobin oxygen saturation using near-infrared time-resolved spectroscopy (TRS) and compared the results with those obtained during normothermia (NT) after a hypoxic-ischemic (HI) insult in a piglet model of asphyxia. We previously reported that a greater increase in CBV can indicate greater pressure-passive cerebral perfusion due to more severe brain injury and correlates with prolonged neural suppression during NT.

Amounts and compositional analysis of glycosaminoglycans in the tissue of fish.

We isolated GAGs from the inedible parts; head, skin, internal organs, fins, scales and spine, of atlantic mackerel (Scomber scombrus), japanese jack mackerel (Trachurus japonicus), pacific bluefin tuna (Thunnus orientalis), yellowfin sole (Limanda aspera), broadbanded thornyhead (Sebastolobus macrochir), golden threadfin bream (Nemipterus virgatus), and nile tilapia (Oreochromis niloticus). We also investigated deep-sea fish, eelpouts (Bothrocara hollandi, Lycodes toyamensis, and Lycodes nakamurae), rough snailfish (Careproctus trachysoma), and squids (Watasenia scintillans, Enoploteuthis chunii, and Berryteuthis magister). Enzymatic digestion of the GAGs enabled a compositional analysis of CS, DS, and HA including the sulfation patterns of CS and DS, as well as the amount of each GAG.

Synthesis and interaction with midkine of biotinylated chondroitin sulfate tetrasaccharides.

Regiospecifically sulfated chondroitin sulfate repeating tetrasaccharides, CS-OO, GlcAβ-GalNAcβ-GlcAβ-GalNAcβ;CS-EE, GlcAβ-GalNAc(4S6S)β-GlcAβ-GalNAc(4S6S)β; and CS-AA, GlcAβ-GalNAc(4S)β-GlcAβ-GalNAc(4S)β, having biotin linked with a hydrophilic linker at the reducing terminal were synthesized effectively by a coupling of the corresponding disaccharide units and regioselective sulfation. CS-EE showed greater affinity for midkine than CS-AA and CS-OO.