Hemangioma of the Mitral Valve and Aortomitral Curtain in an Adolescent.

A hemangioma of the mitral valve and aortomitral curtain was incidentally discovered in an adolescent. The lesion was surgically excised, and the mitral valve was reconstructed with complete preservation of valvular function. Pertinent principles of multimodality imaging-based diagnosis and nuances of operative management of this rare, vascular tumor are discussed.

Electroconvulsive therapy in the treatment of catatonia in a patient with Budd Chiari syndrome: a case report.

Catatonia may manifest as an independent entity or as a feature of a neuropsychiatric or medical illness. While electroconvulsive therapy (ECT) is the gold standard treatment for catatonia, it is typically administered if the patient's symptoms fail to respond to benzodiazepines. We describe the case of a 22-year-old male with Budd Chiari induced cirrhosis and no prior psychiatric history, who presented with symptoms of psychosis and hepatic encephalopathy, was treated in the ICU for multi-factorial delirium, developed symptoms of catatonia that failed to respond to lorazepam, ultimately requiring ECT with a favorable response.

Trapping Anions within Stacks of Tetra-Urea Macrocycles.

Designing molecular receptors that bind anions in water is a significant challenge, and an even greater difficulty lies in using these receptors to remove anions from water without resorting to the hazardous liquid-liquid extraction approach. We here demonstrate an effective and synthetically simple strategy toward these goals by exploiting ion-pair assembly of macrocycles. Our anion binding ensemble consists of an octa-chloro tetra-urea macrocyclic anion receptor (ClTU), which forms water-dispersible aggregates, and a tetra-cationic fluorescent dye 5,10,15,20-tetrakis(1-methyl-4-pyridinio)porphyrin (TMPyP4), which provides Coulombic stabilization and fluorescence reporting of anion binding in an ion-pair assembly.

Five-Year Results With Patisiran for Hereditary Transthyretin Amyloidosis With Polyneuropathy: A Randomized Clinical Trial With Open-Label Extension.

Importance: There is a lack of long-term efficacy and safety data on hereditary transthyretin amyloidosis with polyneuropathy (hATTR-PN) and on RNA interference (RNAi) therapeutics in general. This study presents the longest-term data to date on patisiran for hATTR-PN.

Objective: To present the long-term efficacy and safety of patisiran in adults with hATTR-PN.

Minimal invasive biopsies are highly sensitive for amyloid detection in hereditary transthyretin amyloidosis with polyneuropathy.

Objective: To assess the effectiveness of labial minor salivary gland biopsy (LSGB) alone or in combination with punch skin biopsy (SB) for the detection of amyloid deposits in hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN).

Methods: In this single-center retrospective study, Congo red staining of minimal invasive LSGB (4 mm) and SB (3 mm) was assessed in ATTRv-PN patients consecutively evaluated between 2012 and 2023.

Results: Histopathological data of 171 ATTRv-PN, including 49 early-onset p.