Meconium Ileus, Distal Intestinal Obstruction Syndrome, and Other Gastrointestinal Pathology in the Cystic Fibrosis Patient.

Cystic fibrosis is an autosomal-recessive defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene located on chromosome 7 that affects 1 in 2500 live White births. Defects in the gene lead to abnormally thick secretions causing chronic obstruction in the respiratory and gastrointestinal tracts. Common gastrointestinal pathology in children with cystic fibrosis includes meconium ileus in infancy and distal intestinal obstruction syndrome in childhood and exocrine pancreatic insufficiency, constipation, and rectal prolapse. This article describes the presentation, diagnosis, and management of these conditions in patients with cystic fibrosis, from birth to adulthood.

Classification System for Inferior Vena Cava (IVC) Appearance Following Percutaneous IVC Filter Retrieval.

OBJECTIVE: There is no classification system for describing inferior vena cava (IVC) injuries. The objective of this study was to develop a standardized grading system for venographic appearance of the IVC following percutaneous IVC filter retrieval. METHODS: A classification system for the appearance of the IVC on cavograms following percutaneous IVC filter removal was developed consisting of two grading elements; luminal characteristics and extravasation. Luminal narrowing from 0% up to 50% from any cause is grade 1; narrowing between 50 and 99% is grade 2; occlusion is grade 3; and avulsion is grade 4. Absence of extravasation is grade A, contained extravasation is grade B, and free extravasation is grade C. This system was then applied retrospectively to pre- and post-IVC filter retrieval cavograms performed at a single institution from October 2004 through February 2019. RESULTS: 546 retrieval attempts were identified with 509 (93.2%) filters successfully retrieved. 449 cases (88.2%) had both pre-retrieval and post-retrieval imaging appropriate for application of the proposed classification system. Inter-rater reliability was 0.972 for luminal characteristics, 0.967 for extravasation, and 0.969 overall. Consensus grading demonstrated a distribution of 97.3% grade 1, 1.3% grade 2, 1.3% grade 3, and 0.0% grade 4 for post-retrieval luminal characteristics. For extravasation classification, 96.4% of the cases were classified as grade A, 2.7% grade B, and 0.9% grade C. CONCLUSION: A classification system was developed for describing IVC appearance after IVC filter retrieval, and retrospectively validated using a single center dataset.

Investigations into the carbonic anhydrase inhibition of COS-releasing donor core motifs.

Carbonyl sulfide (COS) releasing scaffolds are gaining popularity as hydrogen sulfide (H2S) donors through exploitation of the carbonic anhydrase (CA)-mediated hydrolysis of COS to H2S. The majority of compounds in this emerging class of donors undergo triggerable decomposition (often referred to as self-immolation) to release COS, and a handful of different COS-releasing structures have been reported. One benefit of this donation strategy is that numerous caged COS-containing core motifs are possible and are poised for development into self-immolative COS/H2S donors. Because the intermediate release of COS en route to H2S donation requires CA, it is important that the COS donor motifs do not inhibit CA directly. In this work, we investigate the cytotoxicity and CA inhibition properties of different caged COS donor cores, as well as caged CO2 and CS2 motifs and non-self-immolative control compounds. None of the compounds investigated exhibited significant cytotoxicity or enhanced cell proliferation at concentrations up to 100 µM in A549 cells, but we identified four core structures that function as CA inhibitors, thus providing a roadmap for the future development of self-immolative COS/H2S donor motifs.

Spectroscopic investigation of the reaction of metallo-protoporphyrins with hydrogen sulfide.

Hydrogen sulfide (H2S) is the most recently discovered gasotransmitter molecule joining nitric oxide and carbon monoxide. In addition to being biologically important gases, these gasotransmitters also provide distinct modes of reactivity with biomimetic metal complexes. The majority of previous investigations on the reactivity of H2S with bioinorganic models have focused on Fe-based porphyrin systems, whereas investigations with other metals remains underinvestigated. To address this gap, we report here an examination of the reactions of H2S, HS-, and S8 with MgII, CuII, CoII, ZnII, CrII, SnIV, and MnII/III protoporphyrins.