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In hematology , hemophagocytic lymphohistiocytosis ( HLH ), also known as haemophagocytic lymphohistiocytosis ( British spelling ), and hemophagocytic or haemophagocytic syndrome , is an uncommon hematologic disorder seen more often in children than in adults. It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines . It is classified as one of the cytokine storm syndromes. There are inherited and non-inherited (acquired) causes of HLH.

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70-709: HLH may refer to: Biology and medicine [ edit ] Hemophagocytic lymphohistiocytosis , a blood disorder Basic helix–loop–helix , a structural motif in proteins Hectopsyllidae , a family of parasitic fleas Places [ edit ] Haydom Lutheran Hospital , in Manyara Region, Tanzania Ulanhot Yilelite Airport , in Inner Mongolia, China Hulan District , in Harbin, China; see List of administrative divisions of Heilongjiang Merrill (Marriner Wood) Hall,

140-454: A medical school attached to a university . In the US, medical school consists of four years. Hence, gaining a basic medical education may typically take eight years, depending on jurisdiction and university. Following completion of entry-level training, newly graduated medical practitioners are often required to undertake a period of supervised practice before their licensure , or registration ,

210-545: A European Certificate of Internal Medicine (ECIM) to facilitate the free movement of medical professionals with the EU are currently underway. The internal medicine specialist is recognized in every country in the European Union and typically requires five years of multi-disciplinary post-graduate education. The specialty of internal medicine is seen as providing care in a wide variety of conditions involving every organ system and

280-524: A compulsory subspecialty training (including acute internal medicine or a dual subspecialty including internal medicine). This latter stage of training is achieved through competition rather than just by yearly progress as the first years of postgraduate training. In the US, three organizations are responsible for the certification of trained internists (i.e., doctors who have completed an accredited residency training program) in terms of their knowledge, skills, and attitudes that are essential for patient care:

350-432: A decreased number of circulating red blood cells , white blood cells , and platelets . The bone marrow may show hemophagocytosis . The liver function tests are usually elevated. A low level of the protein albumin in the blood is common. The serum C reactive protein , erythrocyte sedimentation rate , and ferritin level are markedly elevated. In children, a ferritin above 10000 μg/L is very sensitive and specific for

420-578: A dorm at Brigham Young University in Provo, Utah, U.S.; see List of Brigham Young University buildings#Helaman Halls Other uses [ edit ] HLH Orion minicomputer, by High Level Hardware Ltd Harry Lloyd Hopkins (1890–1946), an American statesman; see George Racey Jordan#Congressional testimony Hillesheimite, a mineral; see List of mineral symbols#H Hala Air, an airline based in Sudan; see List of airline codes (H) Heavy-lift helicopter ,

490-636: A genetic cause of their disease, but rather HLH will be triggered by infection, malignancy, rheumatic disease, and/or certain treatments (as in the cytokine release syndrome associated with CAR T cell therapy ). Primary HLH is caused by high-penetrance variants in genes associated with the syndrome, and thus is part of the phenotype of several inborn errors of immunity (IEI) . The most common and best studied causes of Primary HLH are loss of function, (i.e. inactivating) mutations in genes that code for proteins cytotoxic T cells and NK cells use to kill targeted cells, such as those infected with pathogens like

560-418: A genetic impairment of granule-mediated cytotoxicity in patients, especially older children and adults, who meet any of the various criteria for HLH. Thus, like shock , one must simultaneously manage both the acute physiologic changes associated with HLH (like systemic inflammation, DIC , hepatitis, etc.) and look deeply for various underlying contributors. The International Histiocyte Society has collected

630-468: A holistic approach to care for the entire family unit. Internists also receive substantial training in various recognized subspecialties within the field and are experienced in both inpatient and outpatient settings. On the other hand, family medicine physicians receive education covering a wide range of conditions and typically train in an outpatient setting with less exposure to hospital settings. The historical roots of internal medicine can be traced back to

700-772: A period of decline, was reemphasised by the Royal College of Physicians of London report from the Future Hospital Commission (2013). The European Board of Internal Medicine (EBIM) was formed as a collaborative effort between the European Union of Medical Specialists (UEMS) - Internal Medicine Section and the European Federation of Internal Medicine (EFIM) to provide guidance on standardizing training and practice of internal medicine throughout Europe. The EBIM published training requirements in 2016 for postgraduate education in internal medicine, and efforts to create

770-441: A syndrome of intense inflammation it needs to be differentiated from sepsis , which may be extremely challenging. The diagnosis of acquired, or secondary, HLH is usually made in association with infection by viruses, bacteria, fungi, or parasites or in association with lymphoma, autoimmune disease, or metabolic disease. Acquired HLH may have decreased, normal, or increased NK cell activity. A major differential diagnosis of HLH

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840-735: A term commonly used for a medical doctor who has obtained a medical degree but does not yet have a license to practice medicine unsupervised. In the United States and Commonwealth nations, there is often confusion between internal medicine and family medicine , with people mistakenly considering them equivalent. Internists primarily work in hospitals, as their patients are frequently seriously ill or require extensive medical tests . Internists often have subspecialty interests in diseases affecting particular organs or organ systems. The certification process and available subspecialties may vary across different countries. Additionally, internal medicine

910-577: A type of military helicopter Haklau Min , a variety of Min Chinese, by proposed ISO 639-3 code Topics referred to by the same term [REDACTED] This disambiguation page lists articles associated with the title HLH . If an internal link led you here, you may wish to change the link to point directly to the intended article. Retrieved from " https://en.wikipedia.org/w/index.php?title=HLH&oldid=1134697200 " Category : Disambiguation pages Hidden categories: Short description

980-491: Is Griscelli syndrome (type 2). This is a rare autosomal recessive disorder characterized by partial albinism, hepatosplenomegaly, pancytopenia, hepatitis, immunologic abnormalities, and lymphohistiocytosis. Most cases have been diagnosed between 4 months and 7 years of age, with a mean age of about 17 months. Three types of Griscelli syndrome are recognised: type 1 has neurologic symptoms and mutations in MYO5A . Prognosis depends on

1050-518: Is a heterogeneous autosomal recessive disorder found to be more prevalent with parental consanguinity. Secondary haemophagocytic lymphohistiocytosis (acquired haemophagocytic lymphohistiocytosis) occurs after strong immunologic activation, such as that which can occur with systemic infection, immunodeficiency, or underlying malignancy. Both forms are characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and haematologic alterations and death in

1120-721: Is also required. Internists may practice in Canada as generalists in Internal Medicine or serve in one of seventeen subspecialty areas. Internists may work in many settings including outpatient clinics, inpatient wards, critical care units, and emergency departments. The currently recognized subspecialties include the following: Medicine is mainly focused on the art of diagnosis and treatment with medication . The diagnostic process involves gathering data, generating one or more diagnostic hypotheses, and iteratively testing these potential diagnoses against dynamic disease profiles to determine

1190-606: Is also used. Methotrexate and vincristine have also been used. Other medications include cytokine targeted therapy . On 20 November 2018, the FDA approved the anti-IFN-gamma monoclonal antibody emapalumab (proprietary name Gamifant) for the treatment of pediatric and adult primary HLH. In October 2021 NHS England published Clinical Commissioning Policy: Anakinra for Haemophagocytic Lymphohistiocytosis (HLH) for adults and children in all ages , allowing Anakinra (a modified recombinant interleukin 1 receptor antagonist ) to be used in

1260-461: Is an autosomal recessive disease, hence each sibling of a child with familial HLH has a twenty-five–percent chance of developing the disease, a fifty-percent chance of carrying the defective gene (which is very rarely associated with any risk of disease), and a twenty-five–percent chance of not being affected and not carrying the gene defect. Patients with HLH, especially when untreated, may need intensive therapy . Therefore, HLH should be included in

1330-456: Is an important part of most post-graduate education programs, and many licensed physicians continue to be involved in research activities after completing post-graduate training. Inherent in any medical profession are legal and ethical considerations. Specific laws vary by jurisdiction and may or may not be congruent with ethical considerations. Thus, a strong ethical foundation is paramount to any medical profession. Medical ethics guidelines in

1400-438: Is caused by IL-1 , IL-6 and TNF-alpha ; the cytopenia is due to the suppressive effect on hematopoiesis by TNF-alpha and TNF-gamma . TNF-alpha and TNF-gamma may also lead to inhibition of lipoprotein lipase or stimulate triglyceride synthesis . Activated macrophages secrete ferritin and plasminogen activator leading to hyperfibrinolysis . The blood count typically shows decreased numbers of blood cells —including

1470-530: Is different from Wikidata All article disambiguation pages All disambiguation pages Hemophagocytic lymphohistiocytosis HLH as defined by the HLH-04 criteria is a descriptive diagnosis. Its individual components are non-specific. The onset of HLH occurs before the age of one year in approximately 70 percent of cases. Familial HLH should be suspected if siblings are diagnosed with HLH or if symptoms recur when therapy has been stopped. Familial HLH

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1540-628: Is distinguished from family medicine in that the latter provides a broader model of care the includes both surgery and obstetrics in both adults and children. Accreditation for medical education and training programs in Australia is provided by the Australian Medical Council (AMC) and the Medical Council of New Zealeand (MCNZ). The Medical Board of Australia (MBA) is the registering body for Australian doctors and provides information to

1610-427: Is granted, typically one or two years. This period may be referred to as " internship ", "conditional registration", or " foundation programme ". Then, doctors may follow specialty training in internal medicine if they wish, typically being selected to training programs through competition. In North America, this period of postgraduate training is referred to as residency training , followed by an optional fellowship if

1680-469: Is monitored and accredited by the independent General Medical Council (which also maintains the specialist register). Doctors who have completed medical school spend two years in foundation training completing a basic postgraduate curriculum. After two years of Core Medical Training (CT1/CT2), or three years of Internal Medicine Training (IMT1/IMT2/IMT3) as of 2019, since and attaining the Membership of

1750-1153: Is more often referred to as macrophage activation syndrome (MAS) and occurs most frequently in the juvenile onset and adult onset forms of Still's disease and in systemic lupus erythematosus . It occurs rarely in juvenile idiopathic arthritis , juvenile Kawasaki disease , and rheumatoid arthritis . Secondary HLH also occurs rarely in immunodeficiency disorders such as severe combined immunodeficiency , DiGeorge syndrome , Wiskott–Aldrich syndrome , ataxia–telangiectasia , and dyskeratosis congenita ); and infections caused by EBV, cytomegalovirus , HIV/AIDS , bacteria , protozoa , fungi and SARS-CoV-2 . Secondary HLH may also result from iatrogenic causes such as bone marrow or other organ transplantations; chemotherapy; or therapy with immunosuppressing agents. About 33% of all HLH cases, ~75% of Asian HLH cases, and nearly 100% of HLH cases caused by mutations in SH2D1A (see X-linked lymphoproliferative disease type 1 ) are associated with, and thought to be triggered or promoted by, EBV infection. These cases of HLH are classified as belonging to

1820-402: Is not accepted as good practice with few exceptions. These exceptions include cross-coverage within a practice and certain public health urgent or emergent issues. The ethics of telemedicine including questions on its impact to diagnosis, physician-patient relationship, and continuity of care have been raised. However, with appropriate use and specific guidelines, risks may be minimized and

1890-542: Is recognized as a specialty within clinical pharmacy and veterinary medicine . The term internal medicine in English has its etymology in the 19th-century German term Innere Medizin . Originally, internal medicine focused on determining the underlying "internal" or pathological causes of symptoms and syndromes through a combination of medical tests and bedside clinical examination of patients. This approach differed from earlier generations of physicians, such as

1960-564: Is solely focused on adults and does not typically include surgery , obstetrics , or pediatrics . According to the American College of Physicians , internists are defined as "physicians who specialize in the prevention, detection, and treatment of illnesses in adults." While there may be some overlap in the patient population served by both internal medicine and family medicine physicians, internists primarily focus on adult care with an emphasis on diagnosis, whereas family medicine incorporates

2030-421: Is the hallmark of HLH. All genetic defects for familial HLH are related to granule -dependent cytotoxicity. This inability to remove infected and antigen-presenting cells and terminate the immune response leads to uncontrolled proliferation and activation of the immune system with release of excessive cytokines. These cells then infiltrate organs, releasing more cytokines, which gives the clinical picture. The fever

2100-747: The American Board of Internal Medicine , the American Osteopathic Board of Internal Medicine and the Board of Certification in Internal Medicine . In the UK, the General Medical Council oversees licensing and certification of internal medicine physicians. The Royal Australasian College of Physicians confers fellowship to internists (and sub-specialists) in Australia. The Medical Council of Canada oversees licensing of internists in Canada. In

2170-619: The Australian Health Practitioner Regulation Agency (AHPRA). Medical graduates apply for provisional registration in order to complete intern training. Those completing an accredited internship program are then eligible to apply for general registration. Once the candidate completes the required basic and advanced post-graduate training and a written and clinical examination, the Royal Australasian College of Physicians confers designation Fellow of

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2240-546: The Ayurvedic anthologies of Charaka . Internal medicine specialists, also referred to as general internal medicine specialists or general medicine physicians in Commonwealth countries, are specialized physicians trained to manage complex or multisystem disease conditions that single-organ specialists may not be equipped to handle. They are often called upon to address undifferentiated presentations that do not fit neatly within

2310-742: The Epstein-Barr virus (EBV) or the Dengue virus . These mutations include those in the following genes: UNC13D , STX11 , RAB27A , STXBP2 , LYST , PRF1 1, SH2D1A , BIRC4 , ITK , CD27 , and MAGT1 . Secondary HLH (sHLH) is associated with, and thought to be promoted by, malignant and non-malignant diseases that likewise weaken the ability of the immune system to attack EBV-infected cells. Malignant disorders associated with secondary HLH include T-cell lymphoma , B-cell lymphoma , acute lymphocytic leukemia , acute myeloid leukemia , and myelodysplastic syndrome . In rheumatic diseases, this syndrome

2380-768: The HScore , which can be used to estimate an individual's risk of HLH. In adults, soluble IL-2 receptor has been found to be a very sensitive marker for HLH, demonstrating 100% sensitivity for ruling out HLH below a cutoff of 2400 U/mL and optimal cutoff for ruling in at 2515 U/mL (sensitivity, 100%; specificity, 72.5%), with 93% specificity at >10 000 U/mL. The differential diagnosis of HLH includes secondary HLH and macrophage-activation syndrome or other primary immunodeficiencies that present with hemophagocytic lymphohistiocytosis, such as X-linked lymphoproliferative disease . Other conditions that may be confused with this condition include autoimmune lymphoproliferative syndrome . As

2450-471: The specificity and sensitivity of a particular test. An important part of this process is knowledge of the various ways that a disease can present in a patient.  This knowledge is gathered and shared to add to the database of disease profiles used by physicians. This is especially important in rare diseases. Communication is a vital part of the diagnostic process. The Internist uses both synchronous and asynchronous communication with other members of

2520-556: The 17th-century English physician Thomas Sydenham , known as the father of English medicine or "the English Hippocrates ." Sydenham developed the field of nosology (the study of diseases) through a clinical approach that involved diagnosing and managing diseases based on careful bedside observation of the natural history of disease and their treatment. Sydenham emphasized understanding the internal mechanisms and causes of symptoms rather than dissecting cadavers and scrutinizing

2590-551: The 19th century. During this time, internal medicine emerged as a field that integrated the clinical approach with the use of investigations. Many American physicians of the early 20th century studied medicine in Germany and introduced this medical field to the United States, adopting the name "internal medicine" in imitation of the existing German term. Internal medicine has historical roots in ancient India and ancient China . The earliest texts about internal medicine can be found in

2660-799: The Royal Australasian College of Physicians (FRACP). Basic training consists of three years of full-time equivalent (FTE) training (including intern year) and advanced training consists of 3–4 years, depending on specialty. The fields of specialty practice are approved by the Council of Australian Governments (COAG) and managed by the MBA. The following is a list of currently recognized specialist physicians. After completing medical school, internists in Canada require an additional four years of training. Internists desiring to subspecialize are required to complete two additional years of training that may begin after

2730-490: The Royal College of Physicians , physicians commit to one of the medical specialties: Many training programmes provide dual accreditation with general (internal) medicine and are involved in the general care to hospitalised patients. These are acute medicine, cardiology, Clinical Pharmacology and Therapeutics, endocrinology and diabetes mellitus, gastroenterology, infectious diseases, renal medicine, respiratory medicine and often, rheumatology. The role of general medicine, after

2800-456: The US, two organizations are responsible for certification of subspecialists within the field: the American Board of Internal Medicine and the American Osteopathic Board of Internal Medicine . Physicians (not only internists) who successfully pass board exams receive "board certified" status. The following are the subspecialties recognized by the American Board of Internal Medicine . The American College of Osteopathic Internists recognizes

2870-455: The Western world typically follow four principles including beneficence , non-maleficence , patient autonomy, and justice . These principles underlie the patient-physician relationship and the obligation to put the welfare and interests of the patient above their own. The relationship is built upon the physician obligations of competency, respect for the patient, and appropriate referrals while

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2940-405: The absence of treatment. A subtype of primary HLH where the inflammation is limited to the central nervous system has been described. The current (2008) diagnostic criteria for HLH are 1. A molecular diagnosis consistent with HLH. These include the identification of pathologic mutations of PRF1, UNC13D, or STX11. OR 2. Fulfillment of five out of the eight criteria below: In addition, in

3010-443: The benefits including increased access to care may be realized. Ethical considerations in financial include accurate billing practices and clearly defined financial relationships.  Physicians have both a professional duty and obligation under the justice principle to ensure that patients are provided the same care regardless of status or ability to pay.  However, informal copayment forgiveness may have legal ramifications and

3080-822: The best course of action for the patient. Data may be gathered directly from the patient in medical history-taking and physical examination . Previous medical records including laboratory findings, imaging, and clinical notes from other physicians is also an important source of information; however, it is vital to talk to and examine the patient to find out what the patient is currently experiencing to make an accurate diagnosis. Internists often can perform and interpret diagnostic tests like EKGs and ultrasound imaging (Point-of-care Ultrasound – PoCUS). Internists who pursue sub-specialties have additional diagnostic tools, including those listed below. Other tests are ordered, and patients are also referred to specialists for further evaluation.  The effectiveness and efficiency of

3150-427: The case of familial HLH, no evidence of malignancy should be apparent. Not all five out of eight criteria are required for diagnosis of HLH in adults, and a high index of suspicion is required for diagnosis as delay results in increased mortality. The diagnostic criteria were developed in pediatric populations and have not been validated for adult HLH patients. Attempts to improve diagnosis of HLH have included use of

3220-455: The class of Epstein–Barr virus–associated lymphoproliferative diseases and termed EBV+ HLH . Five genetic subtypes (FHL1, FHL2, FHL3, FHL4, and FHL5) are described, with an estimated overall prevalence of one in 50,000 and equal gender distribution. Molecular genetic testing for four of the causative genes, PRF1 (FHL2), UNC13D (FHL3), STX11 (FHL4), and STXBP2 (FHL5), is available on a clinical basis. Symptoms of FHL are usually evident within

3290-413: The complexity involved in explaining the treatment of diseases that are not localized to a single organ, there has been some confusion surrounding the meaning of internal medicine and the role of an "internist". Although internists may serve as primary care physicians , they are not synonymous with " family physicians ", "family practitioners", " general practitioners ", or "GPs". The training of internists

3360-450: The diagnosis of HLH, however, the diagnostic utility for ferritin is less for adult HLH patients. The serum fibrinogen level is usually low and the D-dimer level is elevated. The sphingomyelinase is elevated. Bone marrow biopsy shows histiocytosis . Primary HLH, also known as familial haemophagocytic lymphohistiocytosis (FHL) or familial erythrophagocytic lymphohistiocytosis,

3430-580: The differential diagnosis of intensive care unit patients with cytopenia and hyperferritinemia . Patients in the earlier stages of HLH are frequently hospitalized at internal medicine wards. HLH clinically manifests with fever , enlargement of the liver and spleen , enlarged lymph nodes , yellow discoloration of the skin and eyes , and a rash . Laboratory findings may include elevated triglyceride levels, low fibrinogen levels, transaminitis , and elevated ferritin levels (among others). The vast majority of patients who meet these criteria will not have

3500-449: The disease and are based on the likelihood that someone with the disease has a particular symptom.  A list of potential diagnoses is termed the "differential diagnosis" for the patient and is typically ordered from most likely to least likely, with special attention given to those conditions that have dire consequences for the patient if they were missed. Epidemiology and endemic conditions are also considered in creating and evaluating

3570-518: The first few months of life and may even develop in utero . However, symptomatic presentation throughout childhood and even into young adulthood has been observed in some cases. The five subtypes of FHL are each associated with a specific gene: Nearly half of the cases of type 2 familial hemophagocytic lymphohistiocytosis are due to bi-allelic PRF1 mutations. The underlying causes, either inherited or acquired, lead to an unchecked immune response when exposed to triggers. Impaired NK-cell cytotoxicity

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3640-603: The following subspecialties: In the United Kingdom, the three medical Royal Colleges (the Royal College of Physicians of London, the Royal College of Physicians of Edinburgh and the Royal College of Physicians and Surgeons of Glasgow ) are responsible for setting curricula and training programmes through the Joint Royal Colleges Postgraduate Training Board (JRCPTB), although the process

3710-563: The incorporation of scientific principles into medical practice in the 1800s, while family medicine emerged as part of the primary care movement in the 1960s. The training and career pathways for internists vary considerably across different countries. Many programs require previous undergraduate education prior to medical school admission. This " pre-medical " education is typically four or five years in length. Graduate medical education programs vary in length by country. Medical education programs are tertiary -level courses , undertaken at

3780-411: The internal workings of the body. In the 17th century, there was a shift towards anatomical pathology and laboratory studies, and Giovanni Battista Morgagni , an Italian anatomist of the 18th century, is considered the father of anatomical pathology. Laboratory investigations gained increasing significance, with contributions from physicians like German physician and bacteriologist Robert Koch in

3850-477: The internist decides to train in a subspecialty. In most countries, residency training for internal medicine lasts three years and centers on secondary and tertiary levels of health care , as opposed to primary health care . In Commonwealth countries, trainees are often called senior house officers for four years after the completion of their medical degree (foundation and core years). After this period, they are able to advance to registrar grade when they undergo

3920-418: The list of diagnoses. The list is dynamic and changes as the physician obtains additional information that makes a condition more ("rule-in") or less ("rule-out") likely based on the disease profile.  The list is used to determine what information will be acquired next, including which diagnostic test or imaging modality to order.  The selection of tests is also based on the physician's knowledge of

3990-439: The medical care team, including other internists, radiologists, specialists, and laboratory technicians.  Tools to evaluate teamwork exist and have been employed in multiple settings. Communication to the patient is also important to ensure there is informed consent and shared decision-making throughout the diagnostic process. Treatment modalities generally include both pharmacological and non-pharmacological, depending on

4060-415: The patient requirements include decision-making and provides or withdraws consent for any treatment plan.  Good communication is key to a strong relationship but has ethical considerations as well, including proper use of electronic communication and clear documentation. Providing treatment including prescribing medications based on remote information gathering without a proper established relationship

4130-474: The pooled proportion are fever 97.2%, hepatomegaly 70.2%, splenomegaly 78.4%, thrombocytopenia 90.1%, anemia 76.0%, and serum ferritin ≥500 μg/L 97.1%. The case fatality rate is 14.6% among dengue hemophagocytic lymphohistiocytosis patients. Internal medicine Internal medicine , also known as general internal medicine in Commonwealth nations , is a medical specialty for medical doctors focused on

4200-607: The prevention, diagnosis, and treatment of internal diseases in adults. Medical practitioners of internal medicine are referred to as internists , or physicians in Commonwealth nations. Internists possess specialized skills in managing patients with undifferentiated or multi-system disease processes. They provide care to both hospitalized ( inpatient ) and ambulatory (outpatient) patients and often contribute significantly to teaching and research. Internists are qualified physicians who have undergone postgraduate training in internal medicine, and should not be confused with " interns ",

4270-535: The primary diagnosis. Additional treatment options include referral to specialist care including physical therapy and rehabilitation.   Treatment recommendations differ in the acute inpatient and outpatient settings. Continuity of care and long-term follow-up is crucial in successful patient outcomes. Aside from diagnosing and treating acute conditions, the Internist may also assess disease risk and recommend preventive screening and intervention.  Some of

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4340-756: The providing professional courtesy may have negatively impact care. Physicians must disclose all possible conflicts of interest including financial relationships, investments, research and referral relationships, and any other instances that may subjugate or give the appearance of subjugating patient care to self-interest. Other foundational ethical considerations include privacy, confidentiality, accurate and complete medical records, electronic health records , disclosure, and informed decision-making and consent. Electronic health records have been shown to improve patient care but have risks including data breaches and inappropriate and/or unauthorized disclosure of protected health information. Withholding information from

4410-526: The published consensus management documents for the many contexts in which HLH occurs and they host full-text versions. Most patients who meet HLH criteria will have secondary cases. Treatment for these patients should focus on the underlying contributors. Additionally, treatment of the inflammation of HLH itself is often required. While optimal treatment of HLH is still being debated, current treatment regimes usually involve high dose corticosteroids , etoposide and cyclosporin . Intravenous immunoglobulin

4480-686: The scope of a single-organ specialty, such as shortness of breath, fatigue, weight loss, chest pain, confusion, or alterations in conscious state. They may manage serious acute illnesses that affect multiple organ systems concurrently within a single patient, as well as the management of multiple chronic diseases in a single patient. While many internal medicine physicians choose to subspecialize in specific organ systems , general internal medicine specialists do not necessarily possess any lesser expertise than single-organ specialists. Rather, they are specifically trained to care for patients with multiple simultaneous problems or complex comorbidities. Due to

4550-475: The severity of neurologic manifestations. Type 2 has mutations in RAB27A and haemophagocytic syndrome, with abnormal T-cell and macrophage activation. This type has a grave prognosis if untreated. Type 3 has mutations in melanophilin and is characterized by partial albinism. This type does not pose a threat to those so affected. HLH is a description of an immunophysiologic state in time. It can be dangerous to infer

4620-459: The specialist referral process is an area of potential improvement. Determining which pieces of information are most important to the next phase of the diagnostic process is of vital importance. It is during this stage that clinical bias like anchoring or premature closure may be introduced. Once key findings are determined, they are compared to profiles of possible diseases.  These profiles include findings that are typically associated with

4690-561: The third year of internist training. The Royal College of Physicians and Surgeons of Canada (RCPSC) is a national non-profit agency that oversees and accredits medical education in Canada. A full medical license in Internal Medicine in Canada requires a medical degree, a license from the Medical Council of Canada , completion of the required post-graduate education, and certification from the RCPSC. Any additional requirements from separate medical regulatory authorities in each province or territory

4760-608: The tools available to the Internist include genetic evaluation. Internists also routinely provide pre-operative medical evaluations including individualized assessment and communication of operative risk. Training the next generation of internists is an important part of the profession.  As mentioned above, post-graduate medical education is provided by licensed physicians as part of accredited education programs that are usually affiliated with teaching hospitals. Studies show that there are no differences in patient outcomes in teaching versus non-teaching facilities. Medical research

4830-482: The treatment of HLH. The prognosis is guarded with an overall mortality of 50%. Poor prognostic factors included HLH associated with malignancy, with half the patients dying by 1.4 months compared to 22.8 months for non-tumour associated HLH patients. Secondary HLH in some individuals may be self-limited because patients are able to fully recover after having received only supportive medical treatment (i.e., IV immunoglobulin only). However, long-term remission without

4900-424: The use of cytotoxic and immune-suppressive therapies is unlikely in the majority of adults with HLH and in those with involvement of the central nervous system (brain and/or spinal cord). The first case report of HLH was published in 1939 under the term "Histiocytic Medullary Reticulosis". A second report would come out in 1952 that would rename the disorder that same year. A systematic review recently reported

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