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Objective. To characterize the genotype and phenotype of Chinese patients with congenital hyperinsulinism (CHI) caused by activating mutations in GLUD1, the gene that encodes mitochondrial enzyme glutamate dehydrogenase (GDH). Methods. The clinical data of glutamate dehydrogenase hyperinsulinism (GDH-HI) patients were reviewed, and gene mutations were confirmed by whole exome sequencing (WES) and Sanger DNA sequencing. Results. Twenty-six patients with GDH-HI heterozygous missense mutations were identified from 240 patients diagnosed as congenital hyperinsulinism over past 15 years. The median age at onset was 8 months (range: 1 day of life to 3 years). Seizure disorder was common in our cohort of patients (23/26). Four patients had normal serum ammonia levels; the median serum concentration was 101 μmol/L (range: 37–190 μmol/L). Hypoglycemic symptoms could be triggered by fasting or protein meals in all patients while blood glucose could be well controlled in all patients with diazoxide. Dosage of diazoxide could be reduced by protein restriction. Attempts to lower ammonia levels failed with different therapies such as protein restriction, benzoate, or N-carbamoyl glutamate. In follow-up, 15 of 26 patients had normal intelligence. Eleven patients developed epilepsy at the age of 6 months to 11 years. De novo mutations in GLUD1 were found in 24 cases, and dominant inheritance was observed in the other two; all were heterozygous. A total of 35% (9/26) patients carried c.1493C>T (p.S445L) mutation. Conclusions. Phenotypic heterogeneity of GDH-HI patients was observed within the Chinese cohort in the present study. The fact that most patients had a GLUD1 p. S445L mutation implies that this site could be a hotspot in Chinese patients. A high frequency of GDH-HI with normal ammonia has been reported in this study. Hence, GLUD1 mutational analysis may be an important method to differential diagnosis of GDH-HI from other diazoxide-responsive CHI in Chinese patients.

The counterpart of a previously described non-pterodactyloid pterosaur with an egg revealed the presence of a second egg inside the body cavity of this gravid female. It clearly shows that pterosaurs had two functional oviducts and demonstrates that the reduction of one oviduct was not a prerequisite for developing powered flight, at least in this group. Compositional analysis of one egg suggests the lack of a hard external layer of calcium carbonate. Histological sections of one femur lack medullary bone and further demonstrate that this pterosaur reached reproductive maturity before skeletal maturity. This study shows that pterosaurs laid eggs even smaller than previously thought and had a reproductive strategy more similar to basal reptiles than to birds. Whether pterosaurs were highly precocial or needed parental care is still open to debate.

In China, there is an urgent need for an efficient anaerobic digester to sustainably treat rice straw. In this study, a downward plug-flow anaerobic digester (DPAD) was designed in which the total working core is separated into three sections: an upper liquid zone, a lower liquid zone, and a solid-state bed (SSB) in the middle. A solid/liquid separation mechanism was designed to recirculate liquor and the discharged solid residue after complete digestion. The 70-L DPAD was run indoors for 100 d, time in which chopped rice straw (30 to 50 mm in length) was fed every 20 d. The digestion performance and biogas production were analysed to assess the feasibility for practice application. The results showed that the DPAD can control scum formation and offers a methane yield of 162.60 L/kg volatile solids, 21.1% higher than that of the control test. It was also found that straw was continuously and efficiently digested by the DPAD in 3 experimental stages. Methane production rates increased by 76.30%, 57.37%, and 13.33% on the second day compared to the first day, respectively, and then, all decreased as the substrate was gradually exhausted. Based on the results, it is clear that the DPAD is a promising solution for chopped straw digestion.

Two almost complete long-tailed pterosaurs from the Linglongta, Jianchang County, western Liaoning, China, are described and represent new taxa referred to the non-pterodactyloid clade Wukongopteridae. Kunpengopterus sinensis gen. et sp. nov. differs from other members of this clade mainly by the rounded posterior region of the skull, thick lacrimal process of the jugal and lack of a bony premaxillary crest. This species further shows a soft tissue crest above the frontal, a comparatively larger wing finger, and the proximal segment of the second pedal phalanx of the fifth toe shorter than in other wukongopterids. The second new species is referred to the genus Darwinopterus, D. linglongtaensis sp. nov. based on the posterior region of the skull. It further differs from other wukongopterid pterosaurs by the thin lacrimal process of the jugal, foramen on nasal process rounded, and by having the second pedal phalanx of the fifth toe less curved (115°). Several differences among the Wukongopteridae can be found in the dentition and the feet, suggesting that they might have occupied slightly different ecological niches. The long-tailed Changchengopterus pani is tentatively referred to this clade and new diagnosis for the wukongopterids Wukongopterus lii and Darwinopterus modularis is provided.Dois novos pterossauros de cauda longa procedentes de Linglongta, Jianchang County da região oeste de Liaoning, China, são descritos e referidos ao clado Wukongopteridae. Kunpengopterus sinensis gen. et sp. nov. difere dos demais integrantes desse clado basicamente pela região mais arredondada da parte posterior do crânio, pelo espesso processo lacrimal do jugal e pela ausência de uma crista óssea premaxilar. Esta espécie apresenta uma crista formada por tecido mole acima da região frontal e possui o dedo alar proporcionalmente maior do que nos demais wukongopterídeos. A segunda espécie é referida ao gênero Darwinopterus, D. linglongtaensis sp. nov., com base na região posterior do crânio. Difere dos deais pterossauros wukongopterídeos por possuir o processo lacrimal do jugal delgado, o forâmen no processo nasal arredondado e pela condição menos curvada da segunda falange do quinto dígito do pé. As principais diferenças entre os Wukongopteridae são encontradas na dentição e nos pés, o que sugere que estes ocupavam nichos ecológicos ligeiramente distintos. O pterossauro de cauda longa Changchen-gopterus pani também é tentativamente referido a esse clado. Novas diagnoses para os wukongopterídeos Wukongopterus lii e Darwinopterus modularis são apresentadas.

A new long-tailed pterosaur, Wukongopterus lii gen. et sp. nov, is described based on an almost complete skeleton (IVPP V15113) representing an individual with an estimated wing span of 730 mm. The specimen was discovered in strata that possibly represent the Daohugou Bed (or Daohugou Formation) at Linglongta, Jianchang, Liaoning Province, China. Wukongopterus lii is a non-pterodactyloid pterosaur diagnosed by the first two pairs of premaxillary teeth protruding beyond the dentary, elongated cervical vertebrae (convergent with Pterodactyloidea), and a strongly curved second pedal phalanx of the fifth toe. The specimen further has a broken tibia that indicates an injury occurred while the individual was still alive. Taphonomic aspects provide indirect evidence of an uropatagium, supporting the general hypothesis that at least all non-pterodactyloid pterosaurs show a membrane between the hind limbs. A phylogenetic analysis including most non-pterodactyloid pterosaurs shows that Wukongopterus lii gen. et sp. nov. lies outside the Novialoidea, being cladistically more primitive than the Rhamphorhynchidae and Capylognathoides. This analysis differs from previous studies and indicates that more work is needed before a stable picture of non-pterodactyloid pterosaur relationships is achieved.Um novo pterossauro de cauda longa, Wukongopterus lii gen. et sp. nov., é descrito baseado em um esqueleto quase completo (IVPP V15113) de um indivíduo com abertura alar estimada em 730 mm. O exemplar foi encontrado nas camadas Daohugou (ou Formação Daohugou) em Linglongta, Jianchang, Província de Liaoning, China. Wukongopterus lii é um pterossauro não-pterodactilóide diagnosticado pela presença de dois pares de dentes pré-maxilares posicionados antes do início do dentário, vertebras cervicais alongadas (convergente com os Pterodactyloidea) e a segunda falange do quinto dígito do pé fortemente curvada. Este espécime também apresenta uma tíbia quebrada indicando que a quebra ocorreu com o animal em vida. Evidências tafonômicas apresentam dados indiretos da presença de um uropatágio, corroborando com a hipótese de que pelo menos os não-pterodactilóides possuíam uma membrana entre os seus membros posteriores. Uma análise filogenética incluindo vários pterossauros não-pterodactilóides resulta no posicionamento de Wukongopterus lii gen. et sp. nov. fora dos Novialoidea, sendo cladisticamente mais primitivo do que os Rhamphorhynchidae e Campylognathoides. Esta nova análise filogenética difere de resultados anteriores, indicando que mais trabalhos são necessários até que uma estabilidade da relação de parentesco entre os pterossauros não-pterodactilóides seja alcançada.

Aims. To determine whether multiple daily injections (MDIs) or continuous subcutaneous insulin infusion (CSII) contributes to better glucose control in children with different type 1 diabetes duration. Methods. Subjects were grouped according to early (≤1 year after disease onset; 1A) or late (1–3 years after onset; 2A) MDIs/CSII treatment initiation. Corresponding control groups (1B, 2B) received insulin injections twice daily. Results. HbA1c levels were consistently lower in group 1A than in group 1B (6 months (T2): 7.37% versus 8.21%; 12 months (T3): 7.61% versus 8.41%; 24/36 months (T4/T5): 7.61% versus 8.72%; all P