Triple Isomerism in 3D Covalent Organic Frameworks.

Isomerism in covalent organic frameworks (COFs) has scarcely been known. Here, for the first time we show 3D COFs with three framework isomers or polymorphs constructed from the same building blocks. All isomers were obtained as large (>10 µm) crystals; although their crystal shapes were distinctly different, they showed identical FT-IR and solid-state NMR spectra. Our structural analyses revealed unprecedented triple isomerism in 3D COFs (noninterpenetrated dia, qtz, and 3-fold interpenetrated dia-c3 nets). Furthermore, this Communication reports the first known COF with qtz topology for which the structure determination was based on Rietveld analysis. We achieved triple framework isomerism by reticulating a tetrahedral building block with a flexible junction and a linear building block with PEO side chains and by varying solution compositions. Our energy calculations, along with the discovery of interisomer transition, revealed that the isomer with qtz topology was a kinetic isomer. Thus, this simple yet little-explored concept of reticulating only flexible building blocks is an effective pathway to significantly broaden the diversity of 3D COFs, which have been proposed for a myriad of applications.

Prenatally diagnosed giant umbilical cord resulted from patent urachus in a monochorionic diamniotic twin with selective intrauterine growth restriction.

A long diffuse giant umbilical cord (GUC), caused by umbilical cord edema associated with a patent urachus, is an extremely rare anomaly. While patients with diffuse GUC appear to experience no significant complications and a good prognosis, little is known about their etiology and prenatal course. Here, we report the first case of prenatally diagnosed diffuse GUC resulted from patent urachus in a monochorionic diamniotic twin with selective intrauterine growth restriction. This case indicates that GUC is epigenetic and unrelated to multiple births.

Prenatally Detected Intrascrotal Testicular Teratoma in an Infant.

Although teratomas are the most common tumors in the testes of prepubertal children, prenatally detected testicular teratomas are extremely rare. To date, only 6 cases of prenatally detected testicular teratomas have been reported in the English literature. An intra-abdominal mass and an ipsilateral undescended testis were confirmed after birth in all 6 cases. Preservation of testicular tissue was not performed in any case. Herein, we report the first case of prenatally diagnosed intrascrotal testicular teratoma in an infant who underwent testis sparing surgery.

A case of a preschool child with a successful kidney transplant following the long-term administration of antibiotics to treat peritoneal dialysis-related ESI/peritonitis by Mycobacterium abscessus.

A preschool child with refractory peritoneal dialysis-related exit-site infection (ESI)/peritonitis caused by Mycobacterium abscessus (M. abscessus) received multidrug antibacterial therapy for 6 months and then successfully underwent living-donor kidney transplantation. The patient was a 2.7-year-old boy and the primary disease was bilateral hypo/dysplastic kidneys. Peritoneal dialysis (PD) was initiated at the age of 4 months. Purulent drainage from the PD catheter exit site was observed, and pus and PD effluent cultures were negative. Since living kidney transplantation was scheduled for 2 months later, the PD catheter was replaced. Due to dialysate leakage from the exit site, the new PD catheter was removed and hemodialysis was initiated. M. abscessus subsequently grew from the PD effluent and abscesses that formed at the exit site continued to present bacteria even after catheter removal; therefore, additional debridement was performed. He received combination treatment with antibiotics, amikacin, clarithromycin, imipenem/cilastatin sodium, and tigecycline, for 6 months. After a 4-month observation period without antibiotics, the patient underwent living-donor kidney transplantation. The post-transplantation course was uneventful without the recurrence of infection for 2 years. Although PD-related ESI/peritonitis caused by M. abscessus was intractable, PD catheter removal, multiple debridement, and 6-month antibiotic combination therapy led to improvements. Follow-up observations for 4 months after the cessation of antibacterial treatment confirmed no recurrence of M. abscessus infection, which allowed kidney transplantation. The establishment of an appropriate treatment strategy and observation period for M. abscessus infection ahead of kidney transplantation requires further case accumulation.

Clarification of acid site location in MSE-type zeolites by spectroscopic approaches combined with catalytic activity: comparison between UZM-35 and MCM-68.

MSE-type zeolites synthesized by different organic structure-directing agents (OSDAs), UZM-35 and MCM-68, were prepared. The location of Brønsted acid sites derived from the framework Al atoms and acidic properties were investigated based on 27Al MQMAS NMR and in situ IR techniques combined with the evaluation of the catalytic activity. We have successfully found a significant difference in the location of Brønsted acid sites in the MSE-type framework; 61 and 33% of acid sites were located at the 12-ring channel for MCM-68 and UZM-35, respectively. The differences in the location of the acid sites yielded their unique catalytic activities for the hydrocarbon cracking reactions, indicating that a well-chosen type of OSDAs for the synthesis is one of the possibilities for controlling the distribution of the framework Al atoms in the MSE-type framework.

Structure of alumina glass.

The fabrication of novel oxide glass is a challenging topic in glass science. Alumina (Al2O3) glass cannot be fabricated by a conventional melt-quenching method, since Al2O3 is not a glass former. We found that amorphous Al2O3 synthesized by the electrochemical anodization of aluminum metal shows a glass transition. The neutron diffraction pattern of the glass exhibits an extremely sharp diffraction peak owing to the significantly dense packing of oxygen atoms. Structural modeling based on X-ray/neutron diffraction and NMR data suggests that the average Al-O coordination number is 4.66 and confirms the formation of OAl3 triclusters associated with the large contribution of edge-sharing Al-O polyhedra. The formation of edge-sharing AlO5 and AlO6 polyhedra is completely outside of the corner-sharing tetrahedra motif in Zachariasen's conventional glass formation concept. We show that the electrochemical anodization method leads to a new path for fabricating novel single-component oxide glasses.

Bladder Cancer in a Long-term Survivor of the Prune Belly Syndrome.

Prune belly syndrome (PBS) is a rare but morbid complexity of congenital anomalies that consists of abdominal wall defect, bilateral cryptorchidism, and urinary tract dilation. With the recent advances in perinatal care, the life expectancy of patients with PBS has improved. Although renal and sexual outcomes are frequently discussed in the literature, little is known about urinary tract malignancies in adulthood. Herein, we present a case of advanced bladder cancer in a 38-year-old man with PBS. To the best of our knowledge, a malignant tumor arising in a nondefunctionalized or nonaugmented bladder in patients with PBS has not been reported previously.

Unilateral Fibrous Hyperplasia of the Labium Majus in Pre- and Early Pubertal Girls: A Report on 3 Cases.

BACKGROUND: In recent years, certain prepubertal cases of asymmetric enlargement of the labium majus came to be recognized as a physiological condition in response to hormonal stimulation. However, there exist only scattered case reports or a few case series on this entity, with different terminology. CASE: We present 3 cases of unilateral asymptomatic swelling of the labium majus in otherwise healthy pre- and early pubertal girls. Physical examination revealed painless, fluctuating, nontender bulging that was recognized for a few years on one side of the labium majus. Ultrasound and magnetic resonance imaging showed increased labial soft tissue without any masses. In all cases, surgical excision was performed for cosmetic request. Histopathological diagnosis was fibrous hyperplasia of the labium majus in all cases. Recurrence occurred in one of 3 cases a year after surgery. SUMMARY AND CONCLUSION: Due to the site- and age-specific non-neoplastic physiological condition, we recommend prepubertal unilateral fibrous hyperplasia of the labium majus (PUFHLM), as the terminology accurately reflects this entity. For the differential diagnosis of genital disorders in children, recognition of PUFHLM is important to avoid unnecessary biopsy or invasive procedures. Surgical excision for cosmetic reasons should not be considered because of the benign nature of this entity and a high recurrence rate in childhood and early adolescence.

[CLINICAL AND IMAGING FINDINGS OF ACUTE EPIDIDYMITIS IN CHILDREN].

(Objective) The etiology of acute epididymitis in children remains poorly understood. Several studies have demonstrated that urine tests are negative in the majority of children with acute epididymitis, and the condition is self-limiting. The need for radiological evaluation of the urinary tract in children with acute epididymitis is still debatable. The aim of this study was to describe clinical and imaging findings in children with acute epididymitis. (Methods) We identified 47 children with acute epididymitis at our institute between 2017 and 2021.We retrospectively reviewed their clinical features and radiological and laboratory data. All children underwent ultrasonography of the kidney and urinary tract. (Results) Median patient age was 9 years (range, 6 months-16 years) and 60% of the cases occurred between the ages of 7 and 12 years. Thirteen children (28%) had a past history of genitourinary malformations. The common malformations were hypospadias in eight children and bladder dysfunction in three. Ultrasound revealed no new urinary tract abnormalities in the remaining 34 children. Urinalysis were performed in 27 children, nine of whom (33%) had pyuria. Urine culture was positive in two children. Of the nine children with genitourinary malformations, eight had pyuria. All 18 children without genitourinary malformations had a negative urinalysis except for one patient (p< 0.0001). (Conclusions) Acute epididymitis is a common cause of acute scrotum in pediatric patients. In this study, one-third of acute epididymitis cases presented pyuria, and about 30% had a past history of genitourinary malformations. The presence of pyuria was associated with a past history of genitourinary malformations. For children with no previous genitourinary malformations, routine use of ultrasound for the detection of urinary tract abnormalities is questionable due to the low yield.

Modified Li7P3S11 Glass-Ceramic Electrolyte and Its Characterization.

Li7P3S11 glass ceramics have high conductivities competitive with liquid electrolytes, making them good candidates as solid-state electrolytes for all-solid-state lithium-ion batteries. However, the metastable nature and performance of Li7P3S11 glass ceramics remain mysterious. Herein, modified Li7P3S11 glass ceramics with compositions of 70Li2S-30P2S5 were prepared via two-step mechanical milling and thermal annealing. Li7P3S11 glass ceramics synthesized using the conventional method (mechanical milling and thermal annealing) were again ball-milled to obtain amorphous 70Li2S-30P2S5 with a peculiar glass structure. Further thermal annealing was carried out to crystallize the glass. The obtained crystalline phase was analogous to the original Li7P3S11 phase, but the conductivity was enhanced by a factor of 1.7. Based on 31P solid-state nuclear magnetic resonance (NMR) spectroscopy, the Li7P3S11 phase contained an additional PS43- unit. A rational deconvolution procedure for the 31P solid-state NMR spectra based on crystalline Li7P3S11 was developed and applied to the samples. The analysis can resolve the additional crystalline PS43- unit in the Li7P3S11 structure. Based on two-dimensional double-quantum 31P NMR spectroscopy, the additional PS43- unit is located adjacent to the P2S74- unit, suggesting that P2S74- is divided into two PS43- units in the Li7P3S11 phase. The flip motion of Li+ was also investigated based on the 7Li spin-lattice relaxation time. The independent activation energy of spin-lattice relaxation with respect to temperature in the Li7P3S11 phase was attributed to a conduction path between the two PS43- units. The findings provide a synthetic route that can be used to develop metastable solid-state electrolytes.

Optically active covalent organic frameworks and hyperbranched polymers with chirality induced by circularly polarized light.

Axial chirality was induced by circularly polarized light to covalent organic frameworks as well as hyperbranched polymers composed of bezene-1,3,5-triyl core units and oligo(benzene-1,4-diyl) as linker units where variation in induction efficiency was rationally interpreted in terms of internal rotation dynamics studied through CPMAS 13C NMR experiments including CODEX measurements.

Congenital perineal urethrocutaneous fistula without rupture in a neonate.

Congenital anterior urethrocutaneous fistula (CAUF) is a rare anomaly, commonly observed in the mid-shaft or more distal position of the penis. It can be seen in the perineum in anorectal anomalies or in cases of duplicated urethra. However, isolated CAUF opening into the perineum has not been reported. Herein, we present a case of congenital anterior urethral diverticulum and possible perineal urethrocutaneous fistula of an otherwise healthy neonate.

Sequential delithiation behavior and structural rearrangement of a nanoscale composite-structured Li1.2Ni0.2Mn0.6O2 during charge-discharge cycles.

Lithium- and manganese-rich layered oxides (LMRs) are promising positive electrode materials for next-generation rechargeable lithium-ion batteries. Herein, the structural evolution of Li1.2Ni0.2Mn0.6O2 during the initial charge-discharge cycle was examined using synchrotron-radiation X-ray diffraction, X-ray absorption spectroscopy, and nuclear magnetic resonance spectroscopy to elucidate the unique delithiation behavior. The pristine material contained a composite layered structure composed of Ni-free and Ni-doped Li2MnO3 and LiMO2 (M = Ni, Mn) nanoscale domains, and Li ions were sequentially and inhomogeneously extracted from the composite structure. Delithiation from the LiMO2 domain was observed in the potential slope region associated with the Ni2+/Ni4+ redox couple. Li ions were then extracted from the Li2MnO3 domain during the potential plateau and remained mostly in the Ni-doped Li2MnO3 domain at 4.8 V. In addition, structural transformation into a spinel-like phase was partly observed, which is associated with oxygen loss and cation migration within the Li2MnO3 domain. During Li intercalation, cation remigration and mixing resulted in a domainless layered structure with a chemical composition similar to that of LiNi0.25Mn0.75O2. After the structural activation, the Li ions were reversibly extracted from the newly formed domainless structure.

Realizing the Single-Phase Spinel-Type Sodium Titanium Oxide with the Li4Ti5O12-like Structure for Building Stable Sodium-Ion Batteries.

Sodium titanium oxide with a spinel-type structure is suitable for the stable sodium-intercalation host for the negative electrode of sodium-ion batteries, such as the spinel-type lithium titanium oxide (Li4Ti5O12, LTO) material for lithium-ion batteries. Recently, this has been partly discovered as the Na3LiTi5O12 (NTO) phase in the LTO particle. However, the single-phase NTO material has never been obtained, preventing accurate material characterizations and applications. Here, we successfully realized the NTO material with the single-phase by the chemical sodium insertion-extraction process. The chemical sodium-inserted LTO material is well converted to the pure NTO phase in the single particle level, via following chemical oxidation by water. The purified material was about 97 mol % of NTO as the single-phase spinel structure with a = 8.746 Å. The basic lattice framework of the prepared NTO was confirmed to be the same as that of the LTO. The single-phase NTO electrode shows 0.8 V versus Na+/Na of the Na-insertion and extraction potential, and 99.4% of Na-insertion capacity with 99.7% of Coulombic efficiency during 200 cycles of the Na-ion half-cell experiment. Further, the Na2Fe2(SO4)3/NTO full-cell shows 3 V-class stable charge-discharge character during 100 cycles. This excellent stability of Na-insertion and extraction properties of single-phase NTO extends the range of constructing safe and stable high-voltage oxide-based sodium-ion battery cells for practical use.

[A CASE OF SPLENOGONADAL FUSION PRESENTING NON-PALPABLE TESTIS AND PROXIMAL HYPOSPADIAS].

A 9-month-old boy, having a history of cardiac surgery in neonatal period, was referred to our department for evaluation of genital anomalies. The antenatal course was uneventful, except for unknown gender. His family history was unremarkable. He was delivered at full term, and his birth weight was 3,510 g. Physical examination revealed proximal hypospadias and a non-palpable testis on the left side. Chromosome studies showed a normal male karyotype with positive SRY. At the age of 14 months, he underwent hypospadias repair. Three months later, left testicular exploration was performed along with orchidopexy of an ascending testis on the contralateral side. As nothing was found through an inguinal incision on the left side, laparoscopy was indicated. Laparoscopic observation revealed a small dark reddish mass cranially connected to the left hypoplastic testis that was located high in the left iliac fossa. The epididymis and vas deference looked abnormal, and detachment to the testis was apparent. Testicular vessels were undifferentiated from the mass. Therefore, the left testis was excised with the mass. Histopathological examination confirmed the testis and spleen tissue, and the diagnosis of splenogonadal fusion was made postoperatively.

Variation of Gonadal Dysgenesis and Tumor Risk in Patients With 45,X/46,XY Mosaicism.

OBJECTIVE: To describe the gonadal features of patients with 45,X/46,XY mosaicism, and to evaluate the prevalence of gonadal tumor in different phenotypes. MATERIALS AND METHODS: The medical records of consecutive patients with 45,X/46,XY karyotype or its variants who had undergone gonadal biopsy or gonadectomy at a single institute between 1996 and 2017 were retrospectively reviewed. RESULTS: Of 34 patients with 45,X/46,XY mosaicism, a unilateral dysgenetic testis and a contralateral streak gonad was detected in 20 patients (59%), bilateral streak gonads in 9 (26%), and bilateral dysgenetic testes in 5 (15%). A gonad composed of both streak and dysgenetic testicular portions was observed in 7 gonads of 6 patients. All streak gonads were removed, and bilateral gonadectomy was performed in 15 patients raised as girls. Pathologic examination revealed gonadal tumors in 6 of the 34 (18%) patients, including a gonadoblastoma in 7 gonads among 5 patients and an association of dysgerminoma with gonadoblastoma in 1 gonad. All 6 patients who developed gonadal tumor had female genitalia. Postoperative course was uneventful except 1 boy. A seminoma was developed in his soritaly scrotal testis at the age of 16 years. CONCLUSION: The prevalence of gonadal tumor in patients with 45,X/46,XY mosaicism may vary according to the phenotype, and high in patients with female phenotype. Considering the increased risk of gonadal tumors in such patients, early investigation and individual management, including prophylactic gonadectomy, are recommended. In male patients, a close follow-up of the preserved testes is mandatory until adulthood.

Effect of Drug-Polymer Interactions through Hypromellose Acetate Succinate Substituents on the Physical Stability on Solid Dispersions Studied by Fourier-Transform Infrared and Solid-State Nuclear Magnetic Resonance.

The present study evaluated the specific intermolecular interactions between carbamazepine (CBZ) and substituents of hypromellose acetate succinate (HPMC-AS), as well as the mechanism of inhibition of recrystallization of solid dispersions (SDs) using Fourier-transform infrared (FTIR) and solid-state nuclear magnetic resonance (NMR) spectroscopy. CBZ and HPMC derivatives, including HPMC, hypromellose acetate (HPMC-A), and hypromellose succinate (HPMC-S), were spray-dried to prepare CBZ/polymer spray-dried samples (SPDs). CBZ/HPMC SPD and CBZ/HPMC-A SPD recrystallized within 10 days at 60 °C and 0% relative humidity, whereas CBZ/HPMC-S SPD maintained its amorphous state for a longer period. FTIR and solid-state NMR measurements using 13C cross polarization (CP), 1H single-pulse, and 1H-15N CP-based heteronuclear single quantum correlation filter experiment with very fast magic angle spinning (MAS) at 70 kHz identified molecular interactions in CBZ/polymer SPDs. Although the HPMC backbone and substituents did not interact notably with CBZ and disrupt CBZ-CBZ intermolecular interactions (formed in the amorphous CBZ), acetate and succinate substituents on HPMC-A and HPMC-S disrupted CBZ-CBZ intermolecular interactions through formation of CBZ/polymer interactions. The acetate substituent formed a hydrogen bond with the NH2 group of CBZ, whereas the succinate substituent formed molecular interactions with both the C═O and NH2 groups of CBZ. Formation of relatively strong molecular interactions between CBZ and the succinate substituent followed by disruption of CBZ-CBZ intermolecular interactions effectively stabilized the amorphous state of CBZ in CBZ/HPMC-S SPD. The correlation between CBZ-polymer interactions and ability of polymers to effectively inhibit CBZ recrystallization is reflected in various commercial HPMC-AS. For example, HPMC-AS LF grade, containing higher amounts of the succinate group, was found to effectively inhibit the recrystallization of CBZ through strong molecular interactions as compared with the HPMC-AS HF grade. The present study demonstrated that a detailed investigation of molecular interactions between the drug and the polymer using FTIR and solid-state NMR spectroscopy could contribute to a suitable selection of the SD carrier.

Negative dielectric constant of water confined in nanosheets.

Electric double-layer capacitors are efficient energy storage devices that have the potential to account for uneven power demand in sustainable energy systems. Earlier attempts to improve an unsatisfactory capacitance of electric double-layer capacitors have focused on meso- or nanostructuring to increase the accessible surface area and minimize the distance between the adsorbed ions and the electrode. However, the dielectric constant of the electrolyte solvent embedded between adsorbed ions and the electrode surface, which also governs the capacitance, has not been previously exploited to manipulate the capacitance. Here we show that the capacitance of electric double-layer capacitor electrodes can be enlarged when the water molecules are strongly confined into the two-dimensional slits of titanium carbide MXene nanosheets. Using electrochemical methods and theoretical modeling, we find that dipolar polarization of strongly confined water resonantly overscreens an external electric field and enhances capacitance with a characteristically negative dielectric constant of a water molecule.

Chiral crystal-like droplets displaying unidirectional rotational sliding.

The self-assembly of organic molecules into supramolecular materials with structural ordering beyond the nanometre scale is challenging. Here, we report the spontaneous self-assembly of a chiral discotic triphenylene derivative into millimetre-sized droplets. The structure of the droplets is characterized by high positional and orientational ordering and a three-dimensional integrity similar to that of single crystals. Notwithstanding, these assemblies slide when placed on a vertical substrate demonstrating their fluid nature. X-ray imaging shows that during the sliding process the internal crystal-like structure is maintained and that the droplets undergo clockwise or counterclockwise unidirectional rotation, depending on the chirality of their molecular components. Rheological measurements suggest that this rotational behaviour might result from the distinct yield stress between the (R)- and (S)-enantiomers. Overall, our findings demonstrate that molecular chirality can determine the movement direction of a supramolecular structure, thus expanding the fundamental understanding of the structure and dynamics of soft materials.

[LOCALIZED CYSTIC DISEASE OF THE KIDNEY IN A 6-YEAR-OLD GIRL].

Localized cystic disease of the kidney (LCDK) is a rare, non-hereditary, non-progressive benign cystic renal condition. It is found primarily in adults, and is rarely reported in children. To date, only 5 prepubertal cases of LCDK have been reported in the literature. In this report, we present a case of LCDK that was difficult to differentiate from malignant renal tumor in a 6-year-old girl. Nephron-sparing surgery could not be performed.A 6-year-old girl with no past medical history presented with gross hematuria and right-sided abdominal pain. An abdominal ultrasound at a local hospital showed multiple variable-sized cysts throughout the right kidney. She was referred to our hospital for further evaluation. Computed tomography demonstrated that the region between cysts was slightly enhanced in some part of the lower pole and was diagnosed III in the Bosniak classification. Nephrectomy was performed for possible cystic renal neoplasm. After surgery, a diagnosis of LCDK was made by histopathological examination.