Hypertensive crisis in young woman with Takayasu arteritis

A young woman who was experiencing repeated convulsions was admitted. The patient's brain magnetic resonance image revealed reversible posterior leukoencephalopathy. Blood pressure fluctuated at times to more than 200 mmHg, and the measurement of the right and left upper arms differed by approximately 70 mmHg. Enhanced computed tomography revealed stenotic lesions of some arteries including the left renal artery. Such findings led to an initial diagnosis of Takayasu arteritis and hypertensive encephalopathy caused by renovascular hypertension. A percutaneous transluminal renal angioplasty was successfully performed. The patient's blood pressure returned to normal value without the use of antihypertensive drugs.     

Suprasellar Germinoma Masquerading As Lymphocytic Hypophysitis Associated With Central Diabetes Insipidus,Delayed Sexual Development,and Subsequent Hypopituitarism

We report the case of an 18-year-old woman with suprasellar germinoma masquerading as lymphocytic hypophysitis, which was associated with central diabetes insipidus, delayed sexual development, and hypopituitarism. Magnetic resonance imaging revealed an enlarged pituitary gland, thickened pituitary stalk, and a mass lesion in the inferior hypothalamus; these findings are typical of lymphocytic hypophysitis. Despite the administration of prednisolone therapy for 3 months, an enlarged irregular cystic mass lesion developed in the pituitary stalk and inferior hypothalamus. Open cranial surgery of the posterior pituitary revealed the presence of a germinoma. Therefore, chemotherapy and stereotactic radiation therapy were administered, resulting in complete remission of the germinoma. This case illustrates that the presence of an intrasellar mass lesion in association with pituitary stalk thickening can often cause difficulties in differential diagnosis. We believe that lymphocytic hypophysitis in pubertal children may be the first sign of a host reaction to an occult germinoma.     

Organocatalytic asymmetric assembly reactions for the syntheses of carbohydrate derivatives by intermolecular Michael-Henry reactions

Given the significance of carbohydrates in life, medicine, and industry, the development of simple and efficient de novo methods to synthesize carbohydrates are highly desirable. Organocatalytic asymmetric assembly reactions are powerful tools to rapidly construct molecules with stereochemical complexity from simple precursors. Here, we present a simple and robust methodology for the asymmetric synthesis of pyranose derivatives with talo- and manno- configurations from simple achiral precursors through organocatalytic asymmetric intermolecular Michael–Henry reaction sequences. In this process, (tert-butyldimethylsilyloxy)acetaldehyde 1 was successfully utilized in two ways: as a donor in a highly selective anti-Michael reaction and as an acceptor in a consecutive Henry reaction. Varied nitroolefins served as Michael acceptors and varied aldehydes substituted for 1 as Henry acceptors providing for the construction of a wide range of carbohydrates with up to 5 stereocenters. In these reactions, a catalyst-controlled Michael reaction followed by a substrate-controlled Henry reaction provided 3,4-dideoxytalose derivatives 6 in a highly stereoselective manner. The Henry reaction was affected by addition of a simple base such as triethylamine: A complex chiral base was not necessary. 3,4-Dideoxymannose derivatives 7 were produced by simply changing the base from triethylamine to 1,8-diazabicyclo[5.4.0]undec-7-ene. Extension of this methodology to a syn-Michael initiated sequence was also successful. A mechanistic discussion is provided to explain the unusual substrate-induced stereoselectivity of the Henry reaction.     

Ift88 is involved in mandibular development

The mandible is a crucial organ in both clinical and biological fields due to the high frequency of congenital anomalies and the significant morphological changes during evolution. Primary cilia play a critical role in many biological processes, including the determination of left/right axis patterning, the regulation of signaling pathways, and the formation of bone and cartilage. Perturbations in the function of primary cilia are known to cause a wide spectrum of human diseases: the ciliopathies. Craniofacial dysmorphologies, including mandibular deformity, are often seen in patients with ciliopathies. Mandibular development is characterized by chondrogenesis and osteogenesis; however, the role of primary cilia in mandibular development is not fully understood. To address this question, we generated mice with mesenchymal deletions of the ciliary protein, Ift88 (Ift88^fl/fl;Wnt1Cre). Ift88^fl/fl;Wnt1Cre mice showed ectopic mandibular bone formation, whereas Ift88 mutant mandible was slightly shortened. Meckel's cartilage was modestly expanded in Ift88^fl/fl;Wnt1Cre mice. The downregulation of Hh signaling was found in most of the mesenchyme of Ift88 mutant mandible. However, mice with a mesenchymal deletion of an essential molecule for Hh signaling activity, Smo (Smo^fl/fl;Wnt1Cre), showed only ectopic mandibular formation, whereas Smo mutant mandible was significantly shortened. Ift88 is thus involved in chondrogenesis and osteogenesis during mandibular development, partially through regulating Sonic hedgehog (Shh) signaling.