Acute Late-Onset Cirrhosis in Zellweger Spectrum Disorder.

Zellweger spectrum disorders (ZSDs) are known to present with variable hepatic manifestations ranging from benign hepatosplenomegaly and elevated liver enzymes to advanced liver cirrhosis with hepatocellular carcinoma. However, the progression of liver disease in ZSD patients over time is poorly characterized due to scarcity of the disease. Herein, we report a case of newly diagnosed liver cirrhosis in a ZSD patient with rapid progression and fatal outcome to demonstrate key clinical learning points.

Peroral cholangioscopy: Update on the state-of-the-art.

Peroral cholangioscopy (POC) is an endoscopic procedure that allows direct intraductal visualization of the biliary tract. POC has emerged as a vital tool for indeterminate biliary stricture evaluation and treatment of difficult biliary stones. Over several generations of devices, POC has fulfilled additional clinical needs where other diagnostic or therapeutic modalities have been inadequate. With adverse event rates comparable to standard endoscopic retrograde cholangioscopy and unique technical attributes, the role of POC is likely to continue expand. In this frontiers article, we highlight the existing and growing clinical applications of POC as well as areas of ongoing research.

Applications, Limitations, and Expansion of Cholangioscopy in Clinical Practice.

Peroral cholangioscopy (POC) provides minimally invasive, direct endoscopic visualization of the biliary ductal system for both diagnostic and therapeutic purposes. POC has benefited from a number of technologic advances since its first introduction several decades ago. These advances have led to improved utility and expanded functionality, making POC an integral part of managing various bile duct diseases and disorders. Over time, the clinical role of POC has expanded. Novel applications and capabilities are being increasingly appreciated and developed. This article provides an overview of the current state of POC, with a particular focus on digital single-operator cholangioscopy and its strengths, limitations, advances, and emerging applications.

Bilateral thigh methicillin-resistant Staphylococcus aureus necrotising fasciitis in a man with newly diagnosed Human Immunodeficiency Virus (HIV).

Methicillin-resistant Staphylococcus aureus (MRSA) necrotising fasciitis (NF) is rare and constitutes a medical and surgical emergency. We report a case of a 53-year-old man with type-2 diabetes mellitus and newly diagnosed HIV infection who presented with 1 week of progressively worsening bilateral sharp thigh pain. On presentation, he was febrile, tachycardic and had bilateral thigh erythema and mild tenderness without open wounds. He had leucocytosis, lactic acidosis and acute kidney injury. Urgent incision and drainage (I&D) was performed for suspected NF. Blood cultures and I&D were positive for MRSA. Biopsy of bilateral thighs showed NF. He was treated with wound washout and 6 weeks of intravenous antibiotics. After undergoing skin graft for wounds, he was discharged to physical rehabilitation. Here we report severe sepsis from bilateral thigh MRSA NF in a patient with newly diagnosed HIV.

Thin-film optical notch filter spectacle coatings for the treatment of migraine and photophobia.

Previous evidence suggests optical treatments hold promise for treating migraine and photophobia. We designed an optical notch filter, centered at 480nm to reduce direct stimulation of intrinsically photosensitive retinal ganglion cells. We used thin-film technology to integrate the filter into spectacle lenses. Our objective was to determine if an optical notch filter, designed to attenuate activity of intrinsically photosensitive retinal ganglion cells, could reduce headache impact in chronic migraine subjects. For this randomized, double-masked study, our primary endpoint was the Headache Impact Test (HIT-6; GlaxoSmithKline, Brentford, Middlesex, UK). We developed two filters: the therapeutic filter blocked visible light at 480nm; a 620nm filter was designed as a sham. Participants were asked to wear lenses with one of the filters for 2weeks; after 2weeks when no lenses were worn, they wore lenses with the other filter for 2weeks. Of 48 subjects, 37 completed the study. Wearing either the 480 or 620nm lenses resulted in clinically and statistically significant HIT-6 reductions. However, there was no significant difference when comparing overall effect of the 480 and 620nm lenses. Although the 620nm filter was designed as a sham intervention, research published following the trial indicated that melanopsin, the photopigment in intrinsically photosensitive retinal ganglion cells, is bi-stable. This molecular property may explain the unexpected efficacy of the 620nm filter. These preliminary findings indicate that lenses outfitted with a thin-film optical notch filter may be useful in treating chronic migraine.