Complications of endovascular repair of juxtarenal aortic aneurysms
Sachinder Singh Hans, Mark F. Conrad in Vascular and Endovascular Complications, 2021
There are several leak points related to the bridging stents that are unique to fenestrated and branch type aortic repairs (B/FEVAR). This primarily includes type III leaks from the graft-branch interface and type 1c leaks from the branch-target vessel interface. Fortunately, with appropriate sizing and planning, these are relatively uncommon. In a series of 650 patients undergoing B/FEVAR with 1679 total branches, Mastracci et al. showed rates of branch-related endoleak requiring intervention of only 2.5% for renal, 3.9% for SMA, and 2.8% for celiac branches.18 Accurate diagnosis of these leaks is made difficult by the proximal location of the branches within the main body graft, as they occur early and simultaneously with type Ia endoleaks on both angiography and CT.59
Tumours of the oral cavity and pharynx
Anju Sahdev, Sarah J. Vinnicombe in Husband & Reznek's Imaging in Oncology, 2020
The most common nasopharyngeal malignancy, nasopharyngeal carcinoma (NPC), is classified by the World Health Organization (WHO) into three types: keratinizing squamous cell carcinoma (type I), non-keratinizing squamous cell carcinoma (type II), and undifferentiated carcinoma (type III). Type III is the most common and is endemic in regions of the world such as China, Southeast Asia, and North Africa. NPC is more common in males, arises at any age but is predominantly a disease of middle age, and is linked to the Epstein–Barr virus (EBV) and genetic and dietary factors. There is a four-fold increase in subjects with a first-degree family history of NPC (38). Common presenting symptoms include epistaxis, nasal blockage, and hearing loss secondary to dysfunction of the eustachian tube. However, early-stage disease can be clinically silent, and commonly NPC presents only in the more advanced stages when it has invaded deep structures, such as the brain and cranial nerves, or spread to lymph nodes in the neck.
Coagulation Theory, Principles, and Concepts
Harold R. Schumacher, William A. Rock, Sanford A. Stass in Handbook of Hematologic Pathology, 2019
The von Willebrand factor (vWf), which has been so intimately linked with factor VIII, is also involved in platelet adhesion. vWf is a series of multimers that show an impressive range in sizes, anywhere from 500,000 to 20,000,000 in molecular weight. These multimers are formed from a subunit that has a molecular weight of 270,000. The variety of multimers exist due to in-vivo proteolysis of disulfide-linked subunits. There are a number of genetic molecular defects of the von Willebrand factor, ranging from complete absence to multiple molecular variations. In general these can be broken down into three broad classifications: type I, in which there is a partial deficiency of the native species; type II, in which there are qualitative abnormalities in the structure; and type III, in which no plasma vWf can be detected (48). The primary platelet membrane receptor for vWf, GP Ib, apparently binds to vWf after vWf has bound to another surface, such as a vessel wall. Experimentally, it appears that platelet adhesion mediated through vWf occurs only at high shear stresses, but these particular experimental studies do not address all the issues of in-vivo platelet adhesion (49,50).
Low α-N-acetylgalactosaminidase plasma concentration correlates with the presence and severity of the bipolar affective disorder
Published in The World Journal of Biological Psychiatry, 2023
Schindler’s disease affects neurons; as the disease progresses, it manifests itself primarily with psychiatric and neurological symptoms. An autosomal recessive inherited metabolic disorder, Schindler’s disease is characterised by reduced α-NAGAL activity with abnormal levels of glycoproteins excreted in the urine. Two German siblings with α-NAGAL deficiency were first described in 1987. This disease is defined in three different types and although it has different clinics, neurological and psychiatric symptoms may occur. Type-I has a severe clinic. It is a progressive neurodegenerative disease and typically neuroaxonal dystrophy is present. Some serious neurological signs and mental disorders occur (Schindler et al. 1989). Type-II, also known as Kanzaki disease, has a milder course than Type-I. In this type, mental disorder can be seen with some skin lesions (Kanzaki et al. 1991). Apart from these, there is Type-III, which is defined as the intermediate type. Neurological symptoms such as seizures and psychiatric symptoms are seen in type-III (Sakuraba et al. 2004).
Could low α-N-acetylgalactosaminidase plasma concentration cause schizophrenia?
Published in The World Journal of Biological Psychiatry, 2023
Schindler's disease has different clinical manifestations, affecting neurons and progressing with many symptoms, particularly neurological and psychiatric ones. It is an inherited autosomal-recessive metabolic disease characterised by decreased α-NAGAL activity. It features abnormal urinary excretion of glycopeptides. Clinically quite heterogeneous, Schindler's disease is a severe phenotype, manifesting progressive neurodegenerative disease characterised by Type-I infantile neuroaxonal dystrophy. In Type-I disease, severe neurological symptoms such as psychomotor retardation, seizures, myoclonus, and mental disorders are seen (Schindler et al. 1989). On the other hand, Type-II disease, also called Kanzaki disease (Kanzaki et al. 1991), is late-onset with a milder course, angiokeratoma corporis diffusum, mild mental disorder. Type-III is the intermediate form and has a variable clinical manifestation, which may include psychomotor retardation, seizures, autism-like symptoms, and psychiatric symptoms (Sakuraba et al. 2004).
Aortic Valve Regurgitation: Pathophysiology and Implications for Surgical Intervention in the Era of TAVR
Published in Structural Heart, 2020
Filippo Ravalli, Alexander P. Kossar, Hiroo Takayama, Juan B. Grau, Giovanni Ferrari
In the diagnosis of AR, the surgical El Khoury classification is employed to differentiate between different types of AR, each with their own clinical manifestations.5 Accurate classification of the type of AR is essential for surgical treatment, as certain contraindications, such as severe calcification, active infection in the valve, extremely restricted cusp motion, severely dilated sinotubular junction (STJ), and decreased cusp height, preclude a durable repair.6,7 According to this classification paradigm, AR is characterized by 3 discrete types: Type I, Type II, and Type III. Type I AR indicates normal cusp motion with a dilatation of the ascending aorta (Type-Ia), aortic sinus (Type-Ib), aortic annulus (Type-Ic), and cusp perforation (Type-Id).5,7 These patients normally have relatively healthy valve leaflets and valve sparing procedures represent the gold standard for treatment. Type II AR is characterized by prolapse or excessive motion of the cusp. Type II AR can also be treated with a valve repair, as opposed to complete valve replacement, allowing for preservation of the patient’s native valve.6,7 Type III indicates restricted cusp mobility due to fibrosis and/or calcification.5,7 Type III AR constitutes a particularly significant patient cohort, as calcification can restrict cusp mobility and may necessitate complete replacement.
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