We examined the dynamics of dissolved inorganic nitrogen (DIN, nitrate + nitrite), dissolved inorganic phosphorus (DIP), and silicate (Si(OH) 4 ) in the northern shelf of the South China Sea in summer, which is under a complex hydrodynamic scheme largely shaped by river plume and coastal upwelling, along with the enhanced biological consumption of nutrients therein. The Pearl River plume, with high nutrient concentrations (, 0.1-14.2 mmol L 21 for DIN, , 0.02-0.10 mmol L 21 for DIP, and , 0.2-18.9 mmol L 21 for Si(OH) 4 ), occupied a large area of the middle shelf (salinity , 33.5). The nearshore area had high nutrient concentrations apparently sourced from subsurface nutrient-replete waters through wind-driven coastal upwelling. These nutrient levels were significantly elevated relative to those on the oligotrophic outer shelf where DIN, DIP, and Si(OH) 4 concentrations dropped to , 0.1 mmol L 21 , , 0.02-0.03 mmol L 21, and , 2.0 mmol L 21, respectively. A three end-member mixing model was constructed based on potential temperature and salinity conservation to assess biological consumption of inorganic nutrients, which was denoted by D and defined by the deviation from conservative mixing. In the coastal upwelling zone and deep chlorophyll maximum layer, the nutrient uptake ratio DDIN : DDIP was 16.7, which is the classic Redfield ratio. In contrast, in the river plume the uptake ratio was 61.3 6 8.7. We believed that an alternative non-DIP source likely contributed to this higher DIN : DIP consumption ratio in the river plume regime. Meanwhile, Si(OH) 4 showed predominant consumption in the river plume and a combination of regeneration and consumption along the path of the coastal upwelling current.

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Abstract. The East China Sea (ECS) and the South China Sea (SCS) are two major marginal seas of the North Pacific with distinct seasonal variations of primary productivity. Based upon field observations covering both the ECS and the northern SCS (NSCS) during December 2008-January 2009, we examined southward long-range transport of nutrients from the ECS to the northeastern SCS (NESCS) carried by the China Coastal Current (CCC) driven by the prevailing northeast monsoon in wintertime. These escaped nutrients from the ECS shelf, where primary production (PP) was limited in winter, might however refuel the PP on the NESCS shelf at lower latitude, where the water temperature remained favorable, but river-sourced nutrients were limited. By combining the field observation of nitrate+nitrite (NO 3 +NO 2 , DIN) with our best estimate of volume transport of the CCC, we derived a first-order estimate for DIN flux of 1430 ± 1024 mol s −1 . Under the assumption that DIN was the limiting nutrient, such southward DIN transport would have stimulated 8.84 ± 6.33 × 10 11 gC of new production (NP), accounting for 33-74 % of the NP or 14-22 % of PP in winter on the NESCS shelf shallower than 100 m.

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The East China Sea (ECS) and the South China Sea (SCS) are two major marginal seas of the north Pacific with distinct seasonal primary productivity. Based upon field observation in December 2008–January 2009 covering both the ECS and the northern SCS (NSCS) in wintertime, we examined southward long-range nutrient-transport from the ECS to the northeast SCS (NESCS) carried by the China Coastal Current (CCC) driven by the northeast prevailing monsoon. These nutrients escaped from the cold ECS shelf to refuel the primary production on the NESCS shelf where river-sourced nutrients were limited yet water temperature remained favorable. By coupling the field observation of nitrate + nitrite (DIN) with the volume transport of the CCC, we derived a first order estimate of DIN flux of ~ 1430 ± 260 mol s⁻¹. This DIN flux was ~ 7 times the wintertime DIN input from the Pearl River, a primary riverine nutrient source to the NSCS. By assuming DIN was the limiting nutrient, such southward DIN transport would have stimulated ~ 8.8 ± 1.6 × 10¹¹ gC of new production (NP), accounting for ~ > 58 ± 10% of the total NP or ~ 38 ± 7–24 ± 4% of primary production on the NESCS shelf shallower than 100 m

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The mutations in the ABCA3 (ATP-binding cassette transporter subfamily A member 3) gene could result in lethal respiratory distress syndrome (RDS) in neonates and interstitial lung disease (ILD) in infants and children. Here, we describe a full-term newborn who manifested respiratory distress 20 min after birth and then gradually developed hypoxemic respiratory failure and died on 53 days of life. A homozygous missense mutation (c.746C >T) was identified in exon 8 of ABCA3 gene in the neonate by next-generation sequencing, and the mutations were inherited from parents, respectively. This homozygous mutation is the first reported to date.

Perinatal lethal Gaucher disease (PLGD), a particular and serious form of type 2 Gaucher disease (GD), often causes lethality in utero or death within hours after birth. The typical clinical manifestations include non-immune hydrops fetalis (NIHF), premature birth, fetal growth restriction, fetal intrauterine death, or neonatal distress and rapid death after birth. Here, we present a premature neonate with GD whose main clinical manifestations included intrauterine growth retardation, anasarca, facial dysmorphia, ichthyosis, respiratory distress, hepatosplenomegaly, joint contractures, myoclonus, refractory thrombocytopenia, anemia, elevated levels of liver enzymes, bile acid and direct bilirubin, cholestasis, pulmonary hypoplasia, intracranial hemorrhage, and abnormal electroencephalogram. The activity of β- glucocerebrosidase was 0 in the peripheral white blood cells of the neonate. The sequencing analysis identified the presence of missense G234E and H413P heterozygous mutations in glucerebrosidase (GBA) exon 7 and 10, with the latter first observed to be associated with PLGD. This infant died at 73 days of age.