A Case of Rathke’s Cleft Cyst Associated with Transient Central Adrenal Insufficiency and Masked Diabetes Insipidus

A 73-year-old woman admitted to our hospital because of headache, poor appetite, malaise, weight loss, and vomiting was found to have central adrenal insufficiency and thyrotoxicosis due to silent thyroiditis. Polyuria developed after replacement with glucocorticoid (masked diabetes insipidus), which was controlled with nasal administration of desmopressin. Magnetic resonance imaging of the brain showed a large cystic pituitary mass (18 × 18 × 12 mm) extending suprasellarly to the optic chiasm. Transsphenoidal surgery revealed that the pituitary tumor was Rathke’s cleft cyst. Following surgery, replacement with neither glucocorticoid nor desmopressin was needed any more. Therefore, it is suggested that Rathke’s cleft cyst is responsible for the masked diabetes insipidus and the central insufficiency. Furthermore, it is speculated that thyrotoxicosis with painless thyroiditis might induce changes from subclinical adrenal insufficiency to transiently overt insufficiency

Impaired Relaxation in Induced Pluripotent Stem Cell-Derived Cardiomyocytes with Pathogenic TNNI3 Mutation of Pediatric Restrictive Cardiomyopathy

Wang R., Hasegawa M., Suginobe H., et al. Impaired Relaxation in Induced Pluripotent Stem Cell-Derived Cardiomyocytes with Pathogenic TNNI3 Mutation of Pediatric Restrictive Cardiomyopathy. Journal of the American Heart Association 13, e032375 (2024); https://doi.org/10.1161/JAHA.123.032375.BACKGROUND: Restrictive cardiomyopathy (RCM) is characterized by impaired diastolic function with preserved ventricular contraction. Several pathogenic variants in sarcomere genes, including TNNI3, are reported to cause Ca²⁺ hypersensitivity in cardiomyocytes in overexpression models; however, the pathophysiology of induced pluripotent stem cell (iPSC)-derived cardiomyocytes specific to a patient with RCM remains unknown. METHODS AND RESULTS: We established an iPSC line from a pediatric patient with RCM and a heterozygous TNNI3 missense variant, c.508C>T (p.Arg170Trp; R170W). We conducted genome editing via CRISPR/Cas9 technology to establish an isogenic correction line harboring wild type TNNI3 as well as a homozygous TNNI3-R170W. iPSCs were then differentiated to cardio-myocytes to compare their cellular physiological, structural, and transcriptomic features. Cardiomyocytes differentiated from heterozygous and homozygous TNNI3-R170W iPSC lines demonstrated impaired diastolic function in cell motion analyses as compared with that in cardiomyocytes derived from isogenic-corrected iPSCs and 3 independent healthy iPSC lines. The intracellular Ca²⁺ oscillation and immunocytochemistry of troponin I were not significantly affected in RCM-cardiomyocytes with either heterozygous or homozygous TNNI3-R170W. Electron microscopy showed that the myofibril and mitochondrial structures appeared to be unaffected. RNA sequencing revealed that pathways associated with cardiac muscle development and contraction, extracellular matrix-receptor interaction, and transforming growth factor-β were altered in RCM-iPSC-derived cardiomyocytes. CONCLUSIONS: Patient-specific iPSC-derived cardiomyocytes could effectively represent the diastolic dysfunction of RCM. Myofibril structures including troponin I remained unaffected in the monolayer culture system, although gene expression profiles associated with cardiac muscle functions were altered

Study on Water Science

In this study, we intended to clarify the effect of various treatments such as magnetism, ultraviolet light irradiation and ultrasonic wave treatment on the acidic electrolyzed aqueous solutions and alkali halide aqueous solutions. The results have indicated that the available chloride concentration of acidic electrolyzed aqueous solution was decreased by these treatments. It was proved that these treatments increased hydroxyl radicals and bound water in the alkali halide aqueous solution

Study on Water Science

In this study, we intended to clarify the effect of various treatments such as magnetic and ultraviolet light irradiation on acidic electrolyzed aqueous solutions and alkali halide aqueous solutions. The results indicated that the available chloride concentration of acidic electrolyzed aqueous solution was decreased by these treatments. It was proved that hydroxyl radicals and bound water varied by these treatments in the alkali halide aqueous solution. It was proved that the most effective treatment in aqueous solutions relates to the salt concentration in both the acidic electrolyzed aqueous solution and the alkali halide aqueous solution

A 106 year monthly coral record reveals that the East Asian summer monsoon modulates winter PDO variability

The Pacific Decadal Oscillation (PDO) is a dominant climate mode in the Pacific Ocean and thought to be related to seasonal to decadal changes in sea surface conditions. Colonies of long-living Porites coral, widely used to reconstruct monthly to century-scale tropical sea surface temperature and sea surface salinity records, were discovered near Koshiki Island, Japan (31 degrees N, 129 degrees E). A monthly resolved, 106 year 18O record revealed that distinct decadal-scale variability was significantly correlated with the PDO index. Our comparison showed 1 to 3 years lead-lag correlation of summer coral 18O with the winter PDO index, suggesting that the East Asian summer monsoon (EASM) may act as the driving force of winter PDO variability over the last 100 years. Cross-spectral analysis between the winter PDO index and summer coral 18O suggested that recent and future global warming may lead to a more frequent and/or stronger teleconnection between EASM and PDO

A Case of Uterine Lymphangioleiomyomatosis Complicated by Tuberous Sclerosis Complex

Lymphangioleiomyomatosis (LAM) is one of the presentations of perivascular epithelioid cell neoplasm that is frequently complicated by tuberous sclerosis complex (TSC). Here, we report an uncommon case of uterine LAM treated with everolimus, which is a mechanistic target of rapamycin (mTOR) inhibitor. A 42-year-old female patient (gravida 0) with a history of TSC presented with abdominal pain. Pelvic magnetic resonance imaging showed multiple masses in the uterine myometrium, suggesting tumors that may contain internal hemorrhagic components. The lesions were suspected as the root cause of her symptoms. After everolimus was administered for a previously diagnosed renal angiolipoma, her uterine tumors temporarily decreased in size. Subsequently, laparoscopic hysterectomy and bilateral salpingectomy were performed since she could not tolerate everolimus for a long period due to the medication’s side effects. Furthermore, the patient was diagnosed with LAM through histopathological examination after surgical resection. Therefore, it is advisable to suspect and investigate uterine LAM when a patient with a history of TSC presents with irregular genital bleeding or abdominal pain. Moreover, mTOR inhibitors may be a treatment option, in addition to surgery, in cases of uterine LAM exacerbation