Clinical Heterogeneity of Neuronal Ceroid Lipofuscinosis Type 13: A Case Report and Systematic Review of Literature.

Objectives: In this study, we describe a 54-year-old Indian woman who presented with clinical features of Kufs syndrome A (KSA) and Kufs syndrome B (KSB), as well as neuropathologic and genetic findings consistent with neuronal ceroid lipofuscinosis type 13 (CLN13). Subsequently, we review the clinicopathologic features of 20 patients with CLN13 reported in the literature.

Methods: Data and imaging were obtained from the patient's medical records.

Oral administration of C37 inhibits colonization in chicks.

As the global population continues to grow, so too does the demand for poultry meat. However, the concurrent increase in the prevalence of drug-resistant bacteria has stimulated interest in the search for alternatives to antibiotics in poultry and livestock agriculture. One potential strategy is the use of probiotics.

Standardization of hemoglobin A and hemoglobin F: Achievements and perspectives.

The establishment of reference systems for the standardization of hemoglobin A (HbA) and fetal hemoglobin (HbF), both critical for improving diagnostic accuracy in conditions such as β-thalassemia and sickle cell disease, are described. Efforts were led by the IFCC and other groups to address and reduce the variability in laboratory measurements of these hemoglobins. This document outlines the production of certified reference materials (CRMs) for HbA and the development of a reference measurement procedure using isotope dilution mass spectrometry.

Prenatal magnetic resonance imaging of umbilical cord ulcer in a fetus with congenital small bowel atresia: A case report.

A 34-year-old woman (gravida 2, para 0) with suspected fetal small-bowel atresia on ultrasound underwent magnetic resonance imaging (MRI) at 33 weeks 3 days of gestation. In addition to small bowel atresia, a teardrop-shaped hematoma was observed adjacent to the umbilical cord associated with an umbilical cord ulcer (UCU). The fetus was delivered by induced labor on diagnosis of intrauterine fetal death.

Convergent and Scalable Second-Generation Synthesis of the Fully Functionalized HIJKLMN-Ring Segment of Caribbean Ciguatoxin C-CTX-1.

A highly convergent and scalable second-generation synthesis of the fully functionalized HIJKLMN-ring segment of Caribbean ciguatoxin C-CTX-1, the primary toxin responsible for ciguatera fish poisoning in the Caribbean Sea and the Northeast Atlantic regions, has been accomplished. Key aspects of the synthetic approach include the efficient syntheses of the HI- and KLM-ring fragments on gram scales, a convergent fragment coupling toward the HIJKLM-ring skeleton based on the Suzuki-Miyaura coupling strategy, and optimized iron hydride-catalyzed hydrogen atom transfer-mediated olefin coupling conditions for constructing the N-ring.