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MRCPCH Clinical Revision - more videos at http://mrcpch.paediatrics.co.uk
Revise for your MRCPCH Clinical exam, with videos and high quality content created by the London Paediatrics Trainees Committee.
Examiner: Jonathan Round
Candidate: Amitav Parida
Filming: Mary Chesshyre, Huey Miin Lee, Chris Kelly
Thank you to the Evelina Children's Hospital for allowing us to film during their MRCPCH Revision Course (https://www.guysandstthomaseve....nts.co.uk/mrcpch-cli
Dilation and curettage (D&C) is a procedure to remove tissue from inside your uterus. Doctors perform dilation and curettage to diagnose and treat certain uterine conditions — such as heavy bleeding — or to clear the uterine lining after a miscarriage or abortion. In a dilation and curettage — sometimes spelled "dilatation" and curettage — your doctor uses small instruments or a medication to open (dilate) your cervix — the lower, narrow part of your uterus. Your doctor then uses a surgical instrument called a curette to remove uterine tissue. Curettes used in a D&C can be sharp or use suction
The term labiaplasty refers to a procedure that reduces the length of the labia minora. It is the most commonly performed female genital plastic surgery procedure and it can relieve symptoms women experience from twisting and tugging of the labia. Women opt for surgery for a variety of reasons, including pain from twisting and tugging of the labia when riding a bike or during intercourse, itching and irritation, and self-consciousness.
Selective immunoglobulin A deficiency (SIgAD) is a primary immunodeficiency disease and is the most common of the primary antibody deficiencies.[1] Total immunoglobulin A deficiency (IgAD) is defined as an undetectable serum immunoglobulin A (IgA) level at a value < 5 mg/dL (0.05 g/L) in humans. Partial IgAD refers to detectable but decreased IgA levels that are more than 2 standard deviations below normal age-adjusted means.[2, 3] IgAD is commonly associated with normal B lymphocytes in peripheral blood, normal CD4+ and CD8+ T cells, and, usually, normal neutrophil and lymphocyte counts. Anti-IgA autoantibodies of the IgG and/or IgE isotype may be present. Peripheral blood may also be affected by autoimmune cytopenias, eg, autoimmune thrombocytopenia,[4, 5] and patients may have other autoimmune phenomena. IgA was first identified by Graber and Williams in 1952; ten years later, the first patients with IgAD were described. IgAD is a heterogeneous disorder, and the results of intensive study are beginning to elucidate genetic loci and molecular pathogenesis that contribute to various subtypes of this disorder. Several lines of evidence suggest that, in many cases, IgAD and common variable immunodeficiency (CVID) have a common pathogenesis, which is discussed further in Pathophysiology. Other data indicate different genetic risk factors. Family studies show variable inheritance patterns. Familial inheritance of IgAD occurs in approximately 20% of cases,[6] and, within families, IgAD and CVID are associated.[7, 8] Many IgAD patients are asymptomatic (ie, "normal" blood donors) and are identified by finding a laboratory abnormality, without any apparent associated clinical disease. Some patients with IgAD may have the following associated conditions: (1) deficits in one or more immunoglobulin G (IgG) subclasses (this accounts for 20-30% of IgA-deficient patients, many of whom may have total IgG levels within the normal range) or (2) a deficient antibody response to pneumococcal immunization (specific polysaccharide antibody deficiency [SPAD]). Some patients with IgAD later develop CVID, and family members of patients with CVID may have only selective IgAD. Characterization of the receptor for the transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI), encoded by the gene TNFRSF13B ( tumor necrosis factor receptor superfamily member 13B), suggests that people with the C104, A181E, and ins204A variants may be at risk for IgAD that progresses to CVID.[9] Primary IgAD is permanent, and below-normal levels have been noted to remain static and persist after 20 years of observation.[10] A recent report documents a rare case of reversion.[11] Environmental factors such as drugs or infections can cause IgAD, but this form is reversible in more than half the cases (see Causes). Although individuals with IgAD have largely been considered healthy, recent studies indicate a higher rate of symptoms. A 20-year follow-up study that compared 204 healthy blood donors with incidentally identified IgAD to 237 healthy subjects with normal IgA levels demonstrated that 80% of IgAD donors and 50% of control subjects had episodes of infections, drug allergy, or autoimmune or atopic disease. Severe respiratory tract infections occurred in 26% of IgAD subjects, in 24% of subjects with decreased IgA levels, and in 8% of control subjects; however, the incidence of life-threatening infections was not increased. IgAD is more common in adult patients with chronic lung disease than in healthy age-matched control subjects.[12] Patients with IgAD are at some increased risk of developing severe reactions after receiving blood products.[13, 14, 15] IgG anti-IgA antibodies may cause severe transfusion reactions if patients with IgAD are given whole blood; therefore, IgA-poor blood or washed red cells are preferred for those patients. IgA-deficient patients with immunoglobulin E (IgE)–class anti-IgA antibodies are at risk for anaphylaxis if they receive blood or intravenous immunoglobulin, but this situation is extremely rare. Individuals with such an unusual profile should receive only low IgA intravenous immunoglobulin preparations. However, caution must be used when administering IGIV to patients with IgAD if their anti-IgA status is unknown. A history devoid of previous blood product administration does not exclude the possibility of anti-IgA antibodies or adverse reactions. Fortunately, appropriate precautions can significantly reduce morbidity (see Treatment). Blood banks can use a simple ELISA screening approach to establish an IgAD blood donor poo
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Alexandra J. Golby, MD, Director, Image-guided Neurosurgery at Brigham and Women’s Hospital, discusses technological advancements to improve the precision of surgery to remove brain tumors.
It’s estimated that each year nearly 80,000 people are diagnosed with primary brain tumors and 100,000 with metastatic brain tumors. Nearly everybody is at risk for developing a brain tumor. Brain tumors can affect people from childhood to the last years of their lives. Men are slightly more affected than women and the causes of most brain tumors are not known.
There are a number of unique challenges in treating brain tumors. One challenge is that primary tumors can have indistinct margins that are difficult to see. Another challenge is that the tissue around a brain tumor is uniquely important and may impact things like language, visual and motor function.
The AMIGO Suite, opened in 2011 at Brigham and Women’s Hospital, is the Advanced Multimodality Image Guided Operating Suite. It's an NIH-funded national center which was developed with the goal of translating technological advances into improvements in surgical and interventional care for patients. In the AMIGO Suite, there is an intraoperative MRI scanner which can be brought in and out of the operating room during surgery to help surgeons visualize a patient’s tumor better.
Image-guided surgery uses the information obtained from advanced imaging and translates that into the planning and execution of surgery by acquiring high resolution and specialty structural images of the brain and also functional images of the brain. These images can be registered to one another and then to the patient's head during surgery. This allows surgeons to pinpoint the location of the tumor as well as the areas that we would like to preserve, areas that serve critical brain functions are located.
One of the big challenges, even with image-guided surgery, is that as we perform the surgery, the configuration of the brain is changing, and we call that brain shift. And it's due to changes in the brain itself and also as we remove tissue, things are constantly shifting and moving. When we're talking about doing brain tumor surgery, a few millimeters of movement can be a big difference. How to measure and track brain shift is an important area of research and a number of technologies are being studied to understand how to measure brain shift during surgery.
The development of various intraoperative imaging technologies allows surgeons to provide the most accurate surgical treatment for each individual patient.
Learn more about precision brain surgery at Brigham and Women’s Hospital:
https://www.brighamandwomens.o....rg/neurosurgery/brai