The anatomy and feeding mechanism of the Japanese giant salamander (Andrias japonicus).

The fully aquatic Japanese giant salamander (Andrias japonicus) is a member of the Cryptobranchidae, and is currently distributed in western Japan, with other members of this group restricted to China and North America. Their feeding behaviour is characterized by a form of suction feeding that includes asymmetric movements of the jaw and hyobranchial apparatus. Previous studies on the North American species, Cryptobranchus alleganiensis, have suggested that this specialized jaw movement is produced by a flexible quadrate-articular joint combined with a loosely connected lower jaw symphysis including two small fibrocartilaginous pads. However, little is known about this feeding behaviour in the Asian species, nor have the three-dimensional asymmetric jaw movements been fully investigated in any member of Cryptobranchidae. In this study, we explore the asymmetric jaw movements in A. japonicus using three methods: (1) dissection of musculoskeletal structures; (2) filming of feeding behaviour to understand in which situations asymmetric feeding is used; (3) analysis of 3D movement of jaws and skull. In the third component, fresh (from frozen) specimens of A. japonicus were manipulated to replicate asymmetric and symmetric jaw movements, with the specimens CT scanned after each step to obtain the 3D morphology of the jaws at different positions. These positions were combined and their Euler angles from resting (closed) jaw position were calculated for asymmetric or symmetric jaw positions. Our filming revealed that asymmetric jaw movements are linked to the position of the prey in relation to the snout, with the jaw closest to the prey opening asymmetrically. Moreover, this action allows the salamander to simultaneously grasp prey in one side of the mouth while ejecting water on the other side, if the first suction attempt fails. The asymmetric jaw movements are performed mainly by rotation of the mandible about its long axis, with very limited lateral jaw movements. During asymmetric and symmetric jaw movements, the posterior ends of the maxilla and quadrate move slightly. The asymmetric jaw movements are permitted by a mobile quadrate-articular joint formed by wide, round cartilages, and by two small fibrocartilage pads within the jaw symphysis that act as cushions during jaw rotation. Some of these soft tissue structures leave traces on the jaws and skull, allowing feeding mode to be reconstructed in fossil taxa. Understanding cryptobranchid asymmetric jaw movement thus requires a comprehensive assessment of not only the symphysial morphology but also that of other cranial and hyobranchial elements.

A Classical Phenotype of Fabry Disease with Novel Mutation Found by Kidney Biopsy, A Case Report.

Fabry disease (FD) is a multi-organ disorder caused by a deficiency of alpha-galactosidase (α-GLA) or reduced activity of the enzyme due to mutations in the GLA gene on the X chromosome, making it an X-linked hereditary disease. A 37-year-old man previously diagnosed with sudden deafness and cardiac hypertrophy was referred to our department after an abnormal urine finding during a public health checkup. A renal biopsy revealed characteristic findings, and he was diagnosed with FD with a novel GLA abnormality (c.714dupT (p.I239Yfs*11)). We are currently administering enzyme replacement therapy (ERT) with agalsidase α. This case shows that a novel genetic abnormality in FD can be overlooked for 37 years, even in the presence of typical symptoms. The significance of a renal biopsy in diagnosing FD is emphasized, highlighting the crucial role of nephrologists.  DOI: 10.52547/ijkd.7595.

Evolutionary process toward avian-like cephalic thermoregulation system in Theropoda elucidated based on nasal structures.

It has long been discussed whether non-avian dinosaurs were physiologically closer to ectotherms or endotherms, with the internal nasal structure called the respiratory turbinate present in extant endotherms having been regarded as an important clue for this conundrum. However, the physiological function and relevance of this structure for dinosaur physiology are still controversial. Here, we found that the size of the nasal cavity relative to the head size of extant endotherms is larger than those of extant ectotherms, with that of the dromaeosaurid Velociraptor being below the extant endotherms level. The result suggests that a large nasal cavity accommodating a well-developed respiratory turbinate is primarily important as a thermoregulation apparatus for large brains characteristic of endothermic birds and mammals, and the nasal cavity of Velociraptor was apparently not large enough to carry out this role required for an endothermic-sized brain. In addition, a hypothesis that the enlargement of the nasal cavity for brain cooling has been associated with the skull modification in the theropod lineage toward modern birds is proposed herein. In particular, the reduction of the maxilla in derived avialans may have coincided with acquisition of the avian-like cephalic thermoregulation system.

Lung evolution in vertebrates and the water-to-land transition.

A crucial evolutionary change in vertebrate history was the Palaeozoic (Devonian 419-359 million years ago) water-to-land transition, allowed by key morphological and physiological modifications including the acquisition of lungs. Nonetheless, the origin and early evolution of vertebrate lungs remain highly controversial, particularly whether the ancestral state was paired or unpaired. Due to the rarity of fossil soft tissue preservation, lung evolution can only be traced based on the extant phylogenetic bracket. Here we investigate, for the first time, lung morphology in extensive developmental series of key living lunged osteichthyans using synchrotron x-ray microtomography and histology. Our results shed light on the primitive state of vertebrate lungs as unpaired, evolving to be truly paired in the lineage towards the tetrapods. The water-to-land transition confronted profound physiological challenges and paired lungs were decisive for increasing the surface area and the pulmonary compliance and volume, especially during the air-breathing on land.

All life on Earth started out under water. However, around 400 million years ago some vertebrates, such as fish, started developing limbs and other characteristics that allowed them to explore life on land. One of the most pivotal features to evolve was the lungs, which gave vertebrates the ability to breathe above water. Most land-living vertebrates, including humans, have two lungs which sit on either side of their chest. The lungs extract oxygen from the atmosphere and transfer it to the bloodstream in exchange for carbon dioxide which then gets exhaled out in to the atmosphere. How this important organ first evolved is a hotly debated topic. This is largely because lung tissue does not preserve well in fossils, making it difficult to trace how the lungs of vertebrates changed over the course of evolution. To overcome this barrier, Cupello et al. compared the lungs of living species which are crucial to understand the early stages of the water-to-land transition. This included four species of lunged bony fish which breathe air at the water surface, and a four-legged salamander that lives on land. Cupello et al. used a range of techniques to examine how the lungs of the bony fish and salamander changed shape during development. The results suggested that the lungs of vertebrates started out as a single organ, which became truly paired later in evolution once vertebrates started developing limbs. This anatomical shift increased the surface area available for exchanging oxygen and carbon dioxide so that vertebrates could breathe more easily on land. These findings provide new insights in to how the lung evolved into the paired structure found in most vertebrates alive today. It likely that this transition allowed vertebrates to fully adapt to breathing above water, which may explain why this event only happened once over the course of evolution.

Feeding behaviour and functional morphology of the neck in the long-snouted aquatic fossil reptile Champsosaurus (Reptilia: Diapsida) in comparison with the modern crocodilian Gavialis gangeticus.

The extinct freshwater choristoderan reptiles Champsosaurus and Simoedosaurus are characterised by large body size and an elongated snout. They have often been considered as eco-analogues of crocodilians based on superficial similarities. The slender-snouted Champsosaurus has been described as a 'gavial-like reptile', which implies it feeds underwater with a lateral swipe of the head and neck, as in the living slender-snouted crocodilians such as Gavialis gangeticus. In contrast, the short-snouted Simoedosaurus is often compared with short-snouted living crocodilians and is considered to take single prey items. However, the neck mobility and flexibility needed for feeding movements are poorly understood even in extant crocodilians. This study explores the relationship between cervical morphology and neck flexion, focusing particularly on lateral and dorsal movements in G. gangeticus by comparison with shorter-snouted crocodilians. The paper also describes a method to estimate the maximum angle of neck dorsiflexion in choristoderes based on the cervical morphology of extant crocodilian species. Three indices were used in this study, of which Index 3 is newly proposed, to compare cervical morphology and intervertebral joint flexibility: (1) Enclosed zygapophyseal angles (EZA) as an index of dorsoventral/ bilateral flexibility, (2) moment arm (M) of dorsiflexor muscles as an Index of resistance against ventroflexion and (3) the orientations of zygapophysial facets for a maximum angle of dorsiflexion. These Indices were validated using µCT scanning of fresh specimens of G. gangeticus and Caiman latirostris in lateral and dorsal flexion. A unique mechanism of lateral flexion was identified in G. gangeticus that uses a combination of the following features: (1) lateral flexion mainly restricted to the anterior cervical vertebrae (v2/v3: high EZA, with more horizontal zygapophyses) and (2) high degree of dorsiflexion at the v3/v4 and v4/v5 joints with potential for dorsal flexibility through the middle-posterior neck, which is used in inertial feeding. In contrast, Champsosaurus and Simoedosaurus possess relatively short cervical vertebrae, as in short-snouted crocodilians. The middle-posterior cervical vertebrae of Champsosaurus are specialised for lateral flexion (high EZA), and there is only limited capacity for dorsiflexion throughout the neck. Like G. gangeticus, therefore, Champsosaurus may have used its slender snout to grab fish from shoals using lateral sweeping motions of the head and neck, but the movement is through the neck, not the craniocervical joint. However, inertial feeding is less likely to have occurred in this genus, and the aligned palatal dentition may have aided the lingual transport of prey into the mouth. Simoedosaurus, on the other hand, appears to have been less specialised, with a neck that combined lateral and dorsolateral flexion, a move that could have been effective in catching both terrestrial and aquatic prey. Where these two choristoderan genera occurred in the same place, they may have divided their niche by prey types.

The sandwich structure of keratinous layers controls the form and growth orientation of chicken rhinotheca.

The upper beak bone of birds is known to be overlain by the rhinotheca, which is composed of the horny sheath of keratinous layers. However, the details of the structure and growth pattern of the rhinotheca are yet to be understood. In this study, the microstructure of the rhinotheca from chicken specimens of different growth stages (ranging from 1 to ~ 80 days old) was analyzed using a combination of thin section and scanning electron microscopy observations, and small-angle X-ray scattering analysis. We found that the rhinotheca comprises three different layers - outer, intermediate, and inner layers - throughout its growth. The outer layer arises from the proximal portion of the beak bone and covers the dorsal surface of the rhinotheca, whereas the intermediate and inner layers originate in the distal portion of the beak bone and underlie the outer layer. This tri-layered structure of the rhinotheca was also observed in wild bird specimens (grey wagtail, king quail, and brown dipper). On the median plane, micro-layers making up the outer and inner layers are bedded nearly parallel to the rostral bone at the base. However, more distally positioned micro-layers of the outer layer are more anteverted distally. The micro-layers of the intermediate layer are bedded nearly perpendicular to those of the outer and inner layers on the median plane. The growth of micro-layers in the intermediate layer adds thickness to the rhinotheca, which causes the difference in profile between the beak bone and the rhinotheca in the distal portion of the beak. Moreover, the entire intermediate layer grows distally as new proximal micro-layers form. The outer layer is dragged distally by the intermediate layer as a result of its distal growth, for the three layers are closely packed to each other at their boundaries. Furthermore, the occurrence of the intermediate and inner layers in the distal portion of the rostral bone may be because the distal end of the beak is frequently used and worn, and the rhinotheca therefore needs to be replaced more frequently at the distal end. The rhinotheca structure described here will be an important and useful factor in the reconstruction of the beaks of birds in extinct taxa.

How does the curvature of the upper beak bone reflect the overlying rhinotheca morphology?

The beak has independently been evolved accompanied by the edentulism in many tetrapod linages, including extant Testudinata and Aves, and its form and function have been greatly diversified. The beak is formed by beak bones and the overlying keratinous cover, although their profiles are different from each other. Therefore, it is difficult to reliably reconstruct the entire profile of the beak in extinct taxa, whose keratinous tissues are rarely preserved. For elucidation of the morphological relationship between beak bone and overlying keratinous cover, we compared the curvature distribution of the culminal profiles of the upper beak bone and the overlying keratinous cover (rhinotheca) with each other using CT-scan, in 66 extant testudinatan and avian specimens (Aves: 33 genera, 24 families; Testudinata: 12 genera seven families). In both, rhinotheca and beak bone, the curvature of the profile was nearly constant rostral to a certain point, which was defined as the transition point, and the transition points of the rhinotheca and beak bone were close to each other. The profiles of the rhinotheca and beak bone rostral to their transition point were different in curvature and length. However, the ratio between the curvatures of rhinotheca and the beak bone strongly correlated with the arc angle of the rostral culminal profiles of the beak bone. The upper beak profile in extinct taxa is expected to be reconstructed more reliably using the abovementioned relationship between the beak bone and the rhinotheca.

The first record of albanerpetontid amphibians (Amphibia: Albanerpetontidae) from East Asia.

Albanerpetontids are an enigmatic fossil amphibian group known from deposits of Middle Jurassic to Pliocene age. The oldest and youngest records are from Europe, but the group appeared in North America in the late Early Cretaceous and radiated there during the Late Cretaceous. Until now, the Asian record has been limited to fragmentary specimens from the Late Cretaceous of Uzbekistan. This led to speculation that albanerpetontids migrated into eastern Asia from North America in the Albian to Cenomanian interval via the Beringian land bridge. However, here we describe albanerpetontid specimens from the Lower Cretaceous Kuwajima Formation of Japan, a record that predates their first known occurrence in North America. One specimen, an association of skull and postcranial bones from a single small individual, permits the diagnosis of a new taxon. High Resolution X-ray Computed Microtomography has revealed previously unrecorded features of albanerpetontid skull morphology in three dimensions, including the presence of a supraoccipital and epipterygoids, neither of which occurs in any known lissamphibian. The placement of this new taxon within the current phylogenetic framework for Albanerpetontidae is complicated by a limited overlap of comparable elements, most notably the non-preservation of the premaxillae in the Japanese taxon. Nonetheless, phylogenetic analysis places the new taxon closer to Albanerpeton than to Anoualerpeton, Celtedens, or Wesserpeton, although Bootstrap support values are weak. The results also question the monophyly of Albanerpeton as currently defined.

Long-term outcomes of tonsillectomy for IgA nephropathy patients: A retrospective cohort study, two-centre analysis with the inverse probability therapy weighting method.

AIM: The effect of tonsillectomy on IgA nephropathy remains controversial. The aim of this study was to compare the effect of tonsillectomy on the outcome, end stage kidney disease (ESKD) and all-cause death in IgA nephropathy patients who did and did not undergo tonsillectomy. METHODS: All basic data were retrospectively gathered from patients who had undergone renal biopsies at two Japanese clinical centres. Two hundred and twenty-seven patients were eligible for the study, with a median age of 34 (Interquartile range (IQR): 25-43) years and median follow-up of 92 (IQR: 40-178) months. The primary endpoint was the composite outcome of the onset of ESKD and all-cause death before ESKD. We performed a Cox proportional hazard regression analysis after adjusting for patient characteristics using the inverse probability therapy weighting (IPTW) method and a Cox analysis using the Matching method. Similarly, we analyzed these outcomes in a mild cohort. RESULTS: We were unable to find any significant advantages of tonsillectomy in either analysis (IPTW and matching, HR: 0.40 (0.12-1.36) P = 0.072 and 0.78 (0.13-4.64) P = 0.786). However, in the mild cohort analysis, our data showed that the Tonsillectomy group tended to be less likely to reach the composite outcomes than the Not Tonsillectomy group with statistical significance (hazard ratio (HR), <0.001 [CI <0.001 to <0.001, P = 0.039]). CONCLUSION: In this study, our findings led us to conclude that performing tonsillectomy in an early and timely manner may have predisposition of less poor prognosis.

The palatal dentition of tetrapods and its functional significance.

The presence of a palatal dentition is generally considered to be the primitive condition in amniotes, with each major lineage showing a tendency toward reduction. This study highlights the variation in palatal tooth arrangements and reveals clear trends within the evolutionary history of tetrapods. Major changes occurred in the transition between early tetrapods and amphibians on the one hand, and stem amniotes on the other. These changes reflect the function of the palatal dentition, which can play an important role in holding and manipulating food during feeding. Differences in the arrangement of palatal teeth, and in their pattern of loss, likely reflect differences in feeding strategy but also changes in the arrangement of cranial soft tissues, as the palatal dentition works best with a well-developed mobile tongue. It is difficult to explain the loss of palatal teeth in terms of any single factor, but palatal tooth patterns have the potential to provide new information on diet and feeding strategy in extinct taxa.

Morphology and function of the palatal dentition in Choristodera.

Choristoderes are a group of extinct freshwater reptiles that were distributed throughout Laurasia from the Middle Jurassic to the Miocene. They are inferred to have had a lifestyle similar to that of extant gavialid crocodiles, but they differed from crocodiles in retaining an extensive palatal dentition. All choristoderes had teeth on the vomers, palatines and pterygoids, and teeth are rarely present on the parasphenoid. Palatal teeth are conical, as in the marginal dentition, and form longitudinal and transverse rows. Detailed examination of different genera shows that the orientation of the palatal tooth crowns changes with their position on the palate, supporting the view that they are involved in intra-oral food transportation, presumably in combination with a fleshy tongue. Moreover, observed variation in palatal tooth shape and the width of palatal tooth batteries may provide additional clues about diet. The European Simoedosaurus lemoinei has sharper palatal teeth than its North American counterpart, S. dakotensis, suggesting a preference for softer prey - a conclusion consistent with the more gracile teeth and narrower snout.

A report of an adult case of tubulointerstitial nephritis and uveitis (TINU) syndrome, with a review of 102 Japanese cases.

BACKGROUND: Although TINU syndrome is characterized by idiopathic TIN with bilateral anterior uveitis, few reports have provided a comprehensive summary of the features of this disorder. Previous reports have suggested that many Japanese patients had HLA-A2 and -A24 (7), but there is no evidence. CASE REPORT: A 44-year-old female was referred to our hospital due to renal dysfunction in March 2012. After admission, her symptoms improved spontaneously without medication within 2 weeks. In the outpatient clinic, she was diagnosed with idiopathic bilateral anterior uveitis in May, and her renal dysfunction relapsed in November. A renal biopsy showed diffuse TIN. We made a diagnosis of TINU syndrome because we could not explain the origin, and treated her with a systemic corticosteroid. Her renal function and ocular symptoms have been improving. The patient had HLA-A24, -B7, -DR1, -C*07: 02 and -DQB1*05: 01: 01. We collected 102 Japanese cases in PubMed, Ovid MEDLINE, and the Japanese Medical Abstracts Society and compared our case with the previous cases. CONCLUSIONS: This disorder affects primarily young females (median age, 14 years), and the most common symptom is fever (44/102 cases). We conducted a statistical analysis using contingency table and Pearson's chi-square test, for HLA-A2 and A24, and calculated the odds ratio (OR). There are no significant differences (A2 was present in 7/22 cases and in 19/50 controls, p value (P) 0.61, OR 0.76 (95% confidence interval (CI)) 0.27-2.2; A24 was present in 10/22 cases and in 33/50 controls, P 0.10, OR 0.43, CI 0.16-1.2).

New material of the enigmatic reptile Khurendukhosaurus (Diapsida: Choristodera) from Mongolia.

New material of the enigmatic diapsid Khurendukhosaurus is described from the Mongolian type locality, Khuren Dukh, providing additional data on the vertebral column, pelvis, and hind limb. It confirms the choristoderan status of the genus and permits a more detailed phylogenetic analysis that supports a relationship between Khurendukhosaurus and the long-necked Asian Hyphalosauridae. The existence of tall caudal neural spines implies that Khurendukhosaurus was a deep-tailed swimmer. This and the open sacral costocentral sutures suggest a primarily aquatic lifestyle.

[Exacerbation of acute leukemia bearing isolated i(17q) along with proliferation of blasts with high BMI-1 expression].

We report a case of acute leukemia with an isolated isochromosome 17q karyotypic abnormality, which may be transformed from myeloproliferative disease (MPD)/myelodysplastic syndrome (MDS). A 69-year-old male patient with 27% of blasts in the peripheral blood underwent hematological examinations including cytochemical staining of cells such as myeloperoxydase (MPO), surface marker study on blasts, chromosomal test and bcr-abl mRNA analysis. The cytological and molecular findings (MPO-positive, myeloid marker CD13 expression (67.3%) and megakaryocytic marker CD41 expression (24.8%)) indicated that the blasts were consistent with myeloid leukemic cells partially committed to megakaryocytes. He was diagnosed as having leukemic transformation from MPD/MDS based on history of leukocytosis and thrombocytosis, isolated i(17q), bcr-abl negative, hepatosplenomegaly, increased eosinophil/basophil count and cytologic dysplasia. Positivity of BMI-1 in CD34+ blasts was 25.8% at the diagnosis and anti-leukemic drugs including anthracyclines were effective for his disease control during 6 months. However, the CD34+ cells turned out to highly express BMI-1 (83.1%), and leukemic cells started to increase progressively following which the leukemic cells failed to respond efficiently to any anti-leukemic drugs. Thus, expression of BMI-1 was well correlated with the disease progression, growth ability of blasts and resistance to anti-cancer drugs, indicating that BMI-1 positivity in CD34+blasts is an excellent molecular marker for disease progression and prognosis in such patients.