Bohan and Peter Criteria for diagnosing DM-PM

Bohan and Peter in 1975 defined DM and PM based upon the following features :

●Symmetric proximal muscle weakness

●Typical cutaneous eruption of DM (the only feature distinguishing DM from PM)

●Elevated serum muscle enzymes

●Myopathic changes on EMG

●Characteristic muscle biopsy abnormalities and the absence of histopathologic signs of other myopathies

Patients with the cutaneous eruption and at least three of the other four criteria met the requirements for definite DM according to these criteria, while requirements for definite PM were met by those with all four criteria other than the cutaneous features . Patients with findings indicating the presence of other disorders that may present similarly were excluded. Patients who did not meet these criteria but who lacked any of the exclusions could potentially have been diagnosed with possible or probable DM or PM, depending upon the number of criteria met.

Skin Findings in Dermatomyositis

Skin findings — Several distinct cutaneous eruptions, which are generally evident at the time of clinical presentation, occur in DM but not in PM . Other skin changes may occur in patients with PM and in patients with DM and are not specific to either disorder. Dermatologic manifestations may be prominent but can be quite subtle in some patients.

Characteristic dermatomyositis findings — Gottron's papules and the heliotrope eruption are the hallmark and likely pathognomonic features of DM. Gottron's sign, photodistributed erythema, poikiloderma, nailfold changes, scalp involvement, and calcinosis cutis are also characteristic and useful in distinguishing DM from PM.

●Gottron's papules – Gottron's papules are erythematous to violaceous papules that occur symmetrically over the extensor (dorsal) aspects of the metacarpophalangeal (MCP) and interphalangeal (IP) joints (picture 1A-C). In addition, these lesions may involve the skin between the MCP and IP joints, particularly when the eruption is prominent. Gottron's papules often have associated scale and may ulcerate. When scaling is present, the lesions may mimic psoriasis or lichen planus.

●Gottron's sign – Definitions used for Gottron's sign have varied in the literature. We define Gottron's sign as the presence of erythematous to violaceous macules, patches, or papules on the extensor surfaces of joints in sites other than the hands, particularly the elbows, knees, or ankles. By contrast, some authors have used the term Gottron's papules to refer to papules in these areas, reserving Gottron's sign for macular or patch-like lesions (picture 2) .

●Heliotrope eruption – The heliotrope eruption is an erythematous to violaceous eruption on the upper eyelids, sometimes accompanied by eyelid edema, which, at times, may be quite marked .

●Facial erythema – Patients may have midfacial erythema that can mimic the malar erythema seen in SLE . In contrast to those with SLE, patients with DM will often have involvement of the nasolabial fold, which can be helpful in distinguishing these two photosensitive midfacial eruptions.

●Photodistributed poikiloderma (including the shawl and V signs) – Poikiloderma refers to skin that demonstrates both hyperpigmentation and hypopigmentation, as well as telangiectasias and epidermal atrophy. In DM, patients may demonstrate poikiloderma in any photo-exposed site; however, classic areas of involvement are the upper back (shawl sign) and the V of the neck and upper chest. The poikiloderma in DM often presents with a violaceous hue. Early in the course of cutaneous disease, these areas may demonstrate only erythema rather than well-developed poikiloderma . The erythema may be macular (nonpalpable) or papular. In rare patients, these lesions become thickened and resemble papular mucinosis. The cutaneous eruption of DM is often associated with significant pruritus, which may assist in distinguishing its photo-exacerbated eruption from that of lupus erythematosus (LE).

●Holster sign – Patients with DM may also have poikiloderma on the lateral aspects of the thighs, referred to as the "Holster sign" . It is unclear why this cutaneous manifestation occurs on this classically photo-protected site.

●Generalized erythroderma – In rare patients, erythroderma may occur, which involves extensive cutaneous surface area, including areas that are less exposed to ultraviolet light.

●Periungual abnormalities – The capillary nail beds in DM may be erythematous and may show vascular changes similar to those observed in other systemic rheumatic diseases (eg, scleroderma and SLE). Abnormal capillary nail bed loops may be evident, with alternating areas of dilatation and dropout and with periungual erythema . In addition, cuticular overgrowth, sometimes termed "ragged cuticles," is characteristic and may be associated with hemorrhagic infarcts within the hypertrophic area . The degree of cuticular involvement is thought to reflect ongoing cutaneous disease activity, representing active vasculopathy .

●Psoriasiform changes in scalp – Changes in the scalp resembling seborrheic dermatitis or psoriasis occur in a high percentage of patients with DM . The scalp involvement in DM is diffuse, often associated with poikilodermatous changes and with prominent scaling. Scalp involvement may result in severe burning, pruritus, and/or sleep disturbance. In addition, severe pruritus may occur in patients without visible disease.

●Calcinosis cutis – The deposition of calcium within the skin, a finding known as calcinosis cutis, occurs commonly in juvenile DM. It is infrequent in adult DM. In children, calcinosis has been associated with a delay in treatment with glucocorticoids and/or immunosuppressive therapy. Calcinosis cutis, which is known to be very challenging to treat, may be seen in a variety of conditions, including SSc, particularly limited cutaneous SSc; SLE (rarely); and overlap connective tissue disorders. It may be more common in patients with DM with the anti-p140/anti-MJ autoantibody

Bhopalwala. H

Antisynthetase Syndrome

Antisynthetase syndrome — Up to 30 percent of patients with DM or PM have a constellation of clinical findings termed the "antisynthetase syndrome" . These findings include relatively acute disease onset, constitutional symptoms (eg, fever and weight loss), myositis, the Raynaud phenomenon, mechanic's hands, arthritis that is generally nonerosive, and ILD . Affected patients have antibodies to aminoacyl-transfer ribonucleic acid (tRNA) synthetase enzymes; the presence of one of these antibodies is highly specific for DM, PM, or ILD .

This syndrome can be further characterized as follows:

●Not all patients with antisynthetase antibodies or even those classified as having the antisynthetase syndrome have all manifestations of this syndrome. The syndrome is generally considered present in patients with an antisynthetase antibody plus two of the following features, which are elements of the syndrome: ILD, inflammatory myopathy, and inflammatory polyarthritis.

●This group of clinical findings or this general clinical picture is not specific for antisynthetase antibodies. Patients with other types of autoantibodies (eg, anti-PM-Scl or anti-U1 ribonucleoprotein [RNP] antibodies) can also present with these types of features. However, patients with antisynthetase antibodies generally have more prominent or severe myositis and ILD, and they usually lack some of the other clinical features seen in patients with these other autoantibodies.

●Some patients with antisynthetase antibodies have relatively little or no myositis, while ILD or other features are more prominent. The absence of myositis is seen more often with some antisynthetase antibodies than with others.

Bhopalwala. H

DM - PM (Abs associated with Malignancy)

Serum autoantibodies — Some serum antibodies in DM and PM confer a positive risk of malignancy, whereas others are associated with a negative risk.

●Positive risk – "Cancer-associated myositis" (CAM) in adults has been associated in several studies with antibodies to transcription intermediary factor (TIF)-1gamma (anti-p155, anti-p155/140) and with antibodies to nuclear matrix protein (NXP)-2 (anti-MJ or anti-p140) .

●Negative risk – Conversely, the presence of myositis-specific (anti-synthetase antibodies, anti-Mi-2, anti-SRP) and myositis-associated antibodies (anti-RNP, anti-PM-Scl, anti-Ku) appears to be associated with a decreased risk of malignancy but an increased risk of interstitial lung disease in DM . More study is required to determine the utility of these autoantibodies for cancer screening in patients with myositis.

Around 30% of patients with Dermatomyositis develop Malignancy.

Around 5% of patients with Polymyositis develop malignancy.

Bhopalwala. H

Irregularly irregular rhythms

Quiz: Name the three types of irregularly irregular rhythms.

Answer:

Atrial fibrillation

Multifocal atrial tachycardia

Atrial flutter with variable conduction

That's all!

-IkaN

Baricitinib (Olumiant)

Mechanism of Action

Baricitinib inhibits Janus kinase (Jak) enzymes, which are intracellular enzymes involved in stimulating hematopoiesis and immune cell function through a signaling pathway. In response to extracellular cytokine or growth factor signaling, Jaks activate signal transducers and activators of transcription (STATs), which regulate gene expression and intracellular activity. Inhibition of Jaks prevents the activation of STATs and reduces serum IgG, IgM, IgA, and C-reactive protein

Use

Rheumatoid arthritis: Treatment of adult patients with moderately to severely active rheumatoid arthritis who have had an inadequate response to one or more tumor necrosis factor antagonist therapies.

Limitation of use: Use of baricitinib in combination with other JAK inhibitors, biologic DMARDs, or with potent immunosuppressants such as azathioprine and cyclosporine is not recommended.

Bhopalwala. H

Certolizumab (Cimzia)

Mechanism of Action

Certolizumab is a pegylated humanized antibody Fab’ fragment of tumor necrosis factor alpha (TNF-alpha) monoclonal antibody. Certolizumab binds to and selectively neutralizes human TNF-alpha activity. (Elevated levels of TNF-alpha have a role in the inflammatory process associated with Crohn disease and in joint destruction associated with rheumatoid arthritis.) Since it is not a complete antibody (lacks Fc region), it does not induce complement activation, antibody-dependent cell-mediated cytotoxicity, or apoptosis. Pegylation of certolizumab allows for delayed elimination and therefore an extended half-life

Use :

Ankylosing spondylitis: Treatment of adults with active ankylosing spondylitis (AS)

Crohn disease: Treatment of moderately to severely active Crohn disease in patients who have inadequate response to conventional therapy

Plaque psoriasis: Treatment of moderate to severe plaque psoriasis in adults who are candidates for systemic therapy or phototherapy

Psoriatic arthritis: Treatment of adult patients with active psoriatic arthritis

Rheumatoid arthritis: Treatment of adults with moderately to severely active rheumatoid arthritis (RA) (as monotherapy or in combination with nonbiological disease-modifying antirheumatic drugs [DMARDS])

Bhopalwala. H

Classification Criteria for Rheumatoid Arthritis

2010 ACR/EULAR criteria —

Using the 2010 ACR/EULAR classification criteria for RA, classification as definite RA is based upon the presence of synovitis in at least one joint, the absence of an alternative diagnosis that better explains the synovitis, and the achievement of a total score of at least 6 (of a possible 10) from the individual scores in four domains . The highest score achieved in a given domain is used for this calculation. These domains and their values are:

●Number and site of involved joints

•2 to 10 large joints (from among shoulders, elbows, hips, knees, and ankles) = 1 point

•1 to 3 small joints (from among the metacarpophalangeal joints, proximal interphalangeal joints, second through fifth metatarsophalangeal joints, thumb interphalangeal joints, and wrists) = 2 points

•4 to 10 small joints = 3 points

•Greater than 10 joints (including at least 1 small joint) = 5 points

●Serological abnormality (rheumatoid factor or anti-citrullinated peptide/protein antibody)

•Low positive (above the upper limit of normal [ULN]) = 2 points

•High positive (greater than three times the ULN) = 3 points

●Elevated acute phase response (erythrocyte sedimentation rate [ESR] or C-reactive protein [CRP]) above the ULN = 1 point

●Symptom duration at least six weeks = 1 point

In addition to those with the criteria above, which are best suited to patients with newly presenting disease, the following patients are classified as having RA:

●Patients with erosive disease typical of RA with a history compatible with prior fulfillment of the criteria above

●Patients with longstanding disease, including those whose disease is inactive (with or without treatment) who have previously fulfilled the criteria above based upon retrospectively available data

Fun fact : Hand ultrasound is done to evaluate for inflammation in RA, if it's not clinically evident.

Bhopalwala. H

SLE vs RA

The arthritis in SLE may look very similar to RA.

The main difference is that it is non erosive, unlike RA.

Pearls of wisdom :

When treating chronic conditions like Rheumatoid Arthritis, Osteoarthritis, SLE, Fibromyalgia, Psoriasis, and Psoriatic Arthritis, you've got to involve the patient in the care. You've got to explain to them that these are  chronic conditions with no cure. Goals should be damage control and remission.

A good strong patient doctor relationship when dealing with these conditions, works better than any pill on planet Earth.

Credits : Dr.G

Bhopalwala. H

Discoid Lupus Erythematosus

Discoid lupus erythematosus — It is estimated that 15 to 30 percent of patients with SLE develop DLE . Patients with localized or generalized DLE are estimated to have cross-sectional prevalences of concurrent SLE between 5 and 28 percent.

The presence of DLE lesions among patients with SLE may modify the risk of specific SLE features. Compared with SLE patients without DLE, those with DLE have increased risk for photosensitivity and leukopenia but decreased risk for serositis and arthritis. There is no obvious change in risk of nephritis despite variable reports of a "renal-protective effect" of the presence of discoid lesions among SLE patients.

Data on the risk for progression of DLE to SLE are limited to retrospective cohort studies, studies lacking power to detect statistical significance of potential markers of progression, and studies that do not address DLE specifically as a CLE subset. In these studies, progression to SLE has occurred in 0 to 28 percent of patients initially presenting with DLE . Progression to SLE often is delayed; in a Swedish-based population cohort study, 10 percent of patients with DLE who subsequently developed SLE did so within the first year and 17 percent developed SLE within the first three years . Two retrospective studies have suggested SLE develops within five years in 50 percent of the DLE patients who indeed go on to develop SLE . Risk factors for progression include an increasing number of clinical and serologic features of SLE: more widespread DLE lesions, arthralgias and arthritis, high antinuclear antibody (ANA) titers, leukopenia, and high erythrocyte sedimentation rates .

It is worth noting that patients with DLE and other mucocutaneous manifestations of LE may meet ACR classification criteria for SLE without having other end-organ disease . Revised classification criteria (the SLICC criteria) have been proposed to address some limitations of the ACR criteria.

●Clinical manifestations – The classic findings of DLE are discrete, erythematous, somewhat indurated plaques covered by a well-formed adherent scale that extends into dilated hair follicles (follicular plugging). The plaques tend to expand slowly with active inflammation at the periphery and then heal, leaving depressed central scars, atrophy, telangiectasias, and hyperpigmentation and/or hypopigmentation . DLE most often involves the face, neck, and scalp but may also occur on the ears (particularly conchal bowls) and, less frequently, on the upper torso . Localized DLE is limited to sites above the neck. Generalized DLE refers to DLE occurring both above and below the neck.

Hypertrophic DLE is an uncommon clinical variant of DLE characterized by the development of hyperkeratotic, verrucous plaques

Fun Fact : SEAL, the famous singer suffers from DLE.

https://en.m.wikipedia.org/wiki/Kiss_from_a_Rose

Bhopalwala. H

Clinical Indications for Thalidomide

Use :

Erythema nodosum leprosum: Acute treatment of cutaneous manifestations of moderate to severe erythema nodosum leprosum; maintenance treatment for prevention and suppression of cutaneous manifestations of erythema nodosum leprosum recurrence

Limitation of use: Thalidomide is not indicated as monotherapy for erythema nodosum leprosum treatment in the presence of moderate to severe neuritis.

Multiple myeloma: Treatment of newly diagnosed multiple myeloma (in combination with dexamethasone)

Mechanism of Action :

Thalidomide exhibits immunomodulatory and antiangiogenic characteristics; immunologic effects may vary based on conditions. Thalidomide may suppress excessive tumor necrosis factor-alpha production in patients with erythema nodosum leprosum, yet may increase plasma tumor necrosis factor-alpha levels in HIV-positive patients. In multiple myeloma, thalidomide is associated with an increase in natural killer cells and increased levels of interleukin-2 and interferon gamma. Other proposed mechanisms of action include suppression of angiogenesis, prevention of free-radical-mediated DNA damage, increased cell mediated cytotoxic effects, and altered expression of cellular adhesion molecules.

Fun fact : Thalidomide causes congenital defects called phocomelia.

Bhopalwala. H

Labs in SLE

Laboratory testing — We obtain the following routine laboratory tests, which may provide diagnostically useful information:

●Complete blood count and differential may reveal leukopenia, mild anemia, and/or thrombocytopenia

●Elevated serum creatinine may be suggestive of renal dysfunction

●Urinalysis with urine sediment may reveal hematuria, pyuria, proteinuria, and/or cellular casts

In addition to the routine laboratories described above, we perform the following laboratory tests which support the diagnosis of SLE if abnormal:

●ANA

●Antiphospholipid antibodies (lupus anticoagulant [LA], IgG and IgM anticardiolipin [aCL] antibodies; and IgG and IgM anti-beta2-glycoprotein [GP] I)

●C3 and C4 or CH50 complement levels

●Erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) levels

●Urine protein-to-creatinine ratio

The ANA test is positive in virtually all patients with SLE at some time in the course of their disease . If the ANA is positive, one should test for other specific antibodies such as dsDNA, anti-Sm, Ro/SSA, La/SSB, and U1 ribonucleoprotein (RNP). In some labs, a positive ANA test by indirect immunofluorescence will automatically result in testing for such additional antinuclear antibodies that are often present in patients SLE.

●Anti-dsDNA and anti-Sm antibodies are highly specific for SLE, but anti-Sm antibodies lack sensitivity . Anti-dsDNA and anti-Sm antibodies are seen in approximately 70 and 30 percent of patients with SLE, respectively.

●Anti-Ro/SSA and anti-La/SSB antibodies are present in approximately 30 and 20 percent of patients with SLE, respectively; however, both antibodies are more commonly associated with Sjögren's syndrome.

●Anti-U1 RNP antibodies are observed in approximately 25 percent of patients with SLE, but they also occur in patients with other conditions and high levels are almost always present in patients with mixed connective tissue disease (MCTD).

●Antiribosomal P protein antibodies have a high specificity for SLE, but have low sensitivity for SLE. They also lack specificity for involvement of a particular organ system or disease manifestation.

If the initial ANA test is negative, but the clinical suspicion of SLE is high, then additional antibody testing may still be appropriate. This is partly related to the differences in the sensitivity and specificity among the methods used to detect ANA. A more detailed discussion on the techniques used to detect ANA is presented separately.

Fun fact : Anti-Sm antibody was actually named after a patient with Lupus, Mr. Smith.

Bhopalwala. H

Secukinumab (Cosentyx)

Use :

Ankylosing spondylitis: Treatment of active ankylosing spondylitis in adults.

Plaque psoriasis: Treatment of moderate to severe plaque psoriasis in adults who are candidates for systemic therapy or phototherapy.

Psoriatic arthritis: Treatment of active psoriatic arthritis in adults.

Mechanism of Action :

Secukinumab is a human IgG1 monoclonal antibody that selectively binds to the interleukin-17A (IL-17A) cytokine and inhibits its interaction with the IL-17 receptor. IL-17A is a naturally occurring cytokine involved in normal inflammatory and immune responses. Secukinumab inhibits the release of proinflammatory cytokines and chemokines.

Never treat a test, test the patient always #Dr. G

Bhopalwala. H

Ixekizumab (Taltz)

Use

Plaque psoriasis: Treatment of moderate to severe plaque psoriasis in adult patients who are candidates for systemic therapy or phototherapy.

Psoriatic arthritis: Treatment of active psoriatic arthritis in adult patients.

Mechanism of Action :

Ixekizumab is a humanized IgG4 monoclonal antibody that selectively binds with the interleukin 17A (IL-17A) cytokine and inhibits its interaction with the IL-17 receptor. IL-17A is a naturally occurring cytokine that is involved in normal inflammatory and immune responses. Ixekizumab inhibits the release of proinflammatory cytokines and chemokines.

Bhopalwala. H

Belimumab ( Benlysta)

Mechanism of Action :

Belimumab is an IgG1-lambda monoclonal antibody that prevents the survival of B lymphocytes by blocking the binding of soluble human B lymphocyte stimulator protein (BLyS) to receptors on B lymphocytes. This reduces the activity of B-cell mediated immunity and the autoimmune response.

Use :

Systemic lupus erythematosus: Treatment of adult patients with active, autoantibody-positive systemic lupus erythematosus (SLE) who are receiving standard therapy.

Limitations of use: Use is not recommended in patients with severe active lupus nephritis, severe active CNS lupus, or in combination with other biologics, including B-cell targeted therapies or intravenous (IV) cyclophosphamide.

Bhopalwala. H

Guttate Psoriasis

The papules and plaques of guttate psoriasis are usually less than 1 cm in diameter (giving rise to the name guttate, which means drop-like). The trunk and proximal extremities are the primary sites of involvement.

Guttate psoriasis typically occurs as an acute eruption in a child or young adult with no previous history of psoriasis. Less commonly, a guttate psoriatic flare occurs in a patient with preexisting psoriasis. There is a strong association between recent infection (usually streptococcal pharyngitis) and guttate psoriasis

Bhopalwala. H

Pustular Psoriasis

Pustular psoriasis — Pustular psoriasis is a form of psoriasis that can have life-threatening complications. The most severe variant (the von Zumbusch type of generalized pustular psoriasis) presents with the acute onset of widespread erythema, scaling, and sheets of superficial pustules . This form of psoriasis can be associated with malaise, fever, diarrhea, leukocytosis, and hypocalcemia. Renal, hepatic, or respiratory abnormalities and sepsis are potential complications.

Reported causes of pustular psoriasis include pregnancy (impetigo herpetiformis), infection, and the withdrawal of oral glucocorticoids. The term impetigo herpetiformis has been used to refer to pustular psoriasis of pregnancy.

Bhopalwala. H

Inverse Psoriasis

Inverse psoriasis — "Inverse psoriasis" refers to a presentation involving the intertriginous areas, including the inguinal, perineal, genital, intergluteal, axillary, or inframammary regions . This presentation is called "inverse" since it is the reverse of the typical presentation on extensor surfaces. This variant can easily be misdiagnosed as a fungal or bacterial infection since there is frequently no visible scaling.

Bhopalwala. H

Grading of Sacroiliitis on Imaging

Plain radiographs of the sacroiliac (SI) joints can be semiquantitatively graded based upon the presence of the characteristic radiographic findings :

●Grade 0: Normal .

●Grade 1: Suspicious changes .

●Grade 2: Minimal abnormality – Small localized areas with erosions or sclerosis, without alteration in the joint width . Erosions usually first appear on the iliac side.

●Grade 3: Unequivocal abnormality – Moderate or advanced sacroiliitis with erosions, evidence of sclerosis, widening, narrowing, or partial ankylosis .

●Grade 4: Severe abnormality – Total ankylosis

Bhopalwala. H

Classification Criteria for Axial Spondyloarthritis

Axial SpA — Classification criteria for axial SpA, including criteria for those without plain radiographic changes (nonradiographic axial SpA [nr-axSpA]) and with radiographic (radiographic axial SpA) changes of sacroiliitis, were based upon a large multicenter study [39]. In patients with a history of back pain of unknown origin, which was of at least three months' duration and which began before age 45, the classification criteria for axial SpA exhibited sensitivity of 83 percent and specificity of 84 percent [39]. This algorithm is as follows:

●The entry step is that the patient must have had back pain of any type for at least three months, and the age of onset must be less than 45 years.

●The second step consists of two arms that are evaluated separately based upon the presence either of sacroiliitis on imaging or of human leukocyte antigen (HLA)-B27:

•HLA-B27-positive patients – In patients who test positive for HLA-B27, at least two additional features of SpA from the list below are required for classifying a patient as having axial SpA (see 'SpA features' below)

•Sacroiliitis on imaging – In patients diagnosed with sacroiliitis based upon plain radiographs (structural changes) or magnetic resonance imaging (MRI) (subchondral bone marrow edema [BME]), at least one other feature of SpA from the list below should be present (see 'SpA features' below)

SpA features — The following are SpA features that contribute to the classification criteria for axial SpA (see 'Axial SpA' above):

●Inflammatory back pain – Several definitions for inflammatory back pain have been proposed. For classification purposes, inflammatory back pain can be defined as having at least four of the five following parameters [67]:

•Age of onset <40 years

•Insidious onset

•Improvement with exercise

•No improvement with rest

•Pain at night (with improvement upon arising)

●Other SpA features (each of equal weight) – Other SpA features include the presence of one or more of the non-spinal features noted below. The occurrence can be either before or at the time of evaluation for the following items listed: arthritis, heel enthesitis, uveitis, dactylitis, psoriasis, and inflammatory bowel disease. The features are:

•Arthritis – As diagnosed by a clinician

•Heel enthesitis – Spontaneous pain or tenderness at site of insertion of Achilles tendon or plantar fascia at the calcaneus diagnosed by a clinician

•Uveitis – Confirmed by an ophthalmologist

•Dactylitis – Diagnosed by a clinician

•Psoriasis – Diagnosed by a clinician

•Inflammatory bowel disease – Crohn disease or ulcerative colitis diagnosed by a clinician

•Good response to nonsteroidal antiinflammatory drugs (NSAIDs) – Within 24 to 48 hours

•Family history of SpA – Presence in first- or second-degree relatives of AS or acute anterior uveitis [37]

•Elevated C-reactive protein (CRP) – After exclusion of other causes for elevated CRP

Bhopalwala. H

Classification Criteria for Peripheral Spondyloarthritis

For classification as having peripheral SpA, the patient should NOT have concurrent inflammatory back pain. In that case, the axial SpA criteria should be used. However, inflammatory back pain in the past is considered as a contributing SpA feature. There are two steps in the algorithm for classifying peripheral SpA:

The entry step is that the patient should have, at the time of being seen, at least one of the following three findings:

●Arthritis

●Enthesitis (spontaneous pain or tenderness at any enthesis)

●Dactylitis

If the patient satisfies the entry criteria, the patient should show (or have had in the past) at least one of the features of SpA in Group A (below) or at least two of the features of SpA in group B (below):

●Group A SpA features:

•Uveitis – Confirmed by an ophthalmologist

•Psoriasis – Diagnosed by a clinician

•Crohn disease or ulcerative colitis – Diagnosed by a clinician

•Preceding infection – Urethritis/cervicitis or diarrhea within one month prior to onset of arthritis/enthesitis/dactylitis

•HLA-B27

•Sacroiliitis on imaging

●Group B SpA features:

•Arthritis – Diagnosed by a clinician

•Enthesitis – Diagnosed by a clinician

•Dactylitis – Diagnosed by a clinician

•Inflammatory back pain in the past

•Family history of SpA – Presence in first- or second-degree relatives of AS and acute uveitis

Bhopalwala. H

How to Define Inflammatory Back Pain

●Inflammatory back pain – Several criteria have been proposed for defining IBP ; we use the "ASAS [Assessment of SpondyloArthritis International Society] expert criteria" to identify patients with IBP . The criteria for IBP are met if at least four of the following five features are present and IBP is described as "suggested" in the presence of three of five features:

•Onset of back discomfort before the age of 40 years

•Insidious onset

•Improvement with exercise

•No improvement with rest

•Pain at night (with improvement upon arising)

The presence of four of the five features listed above has a sensitivity and specificity of 80 and 74 percent, respectively, for an inflammatory cause of the back pain among patients with chronic back pain of unclear origin and onset of the back pain at <45 years of age who were evaluated by local rheumatologists . However, when IBP is present, and considered independently of other factors, the probability of AS among patients with chronic back pain increases only from 5 percent to 14 to 16 percent .

Bhopalwala. H

Rhupus

Rhupus – The term rhupus has been used to describe patients with overlapping features of both SLE and RA. Whether rhupus is clinically and immunologically a distinct entity, a true overlap of SLE and RA, or a subset of patients with SLE remains a matter of debate. In addition to having serologies consistent with both SLE and RA, some patients classified as rhupus may have an erosive arthropathy that is atypical for SLE

Bhopalwala. H

Kikuchi's disease

●Kikuchi's disease – Kikuchi's disease is a benign and usually self-limited form of histiocytic-necrotizing lymphadenitis. Clinical features at presentation include lymphadenopathy as well as fever, myalgias, arthralgias, and, less commonly, hepatosplenomegaly. Associations with SLE have been reported, but the clinical course is usually favorable with spontaneous remission often occurring within four months. The diagnosis of Kikuchi's disease is based on a lymph node biopsy, which reveals a histiocytic cellular infiltrate.

Bhopalwala. H

Psoriatic Arthritis

Diagnostic criteria for Psoriatic Arthritis :

●CASPAR criteria – The CASPAR study concluded that a patient with an inflammatory musculoskeletal disease (peripheral arthritis, spondylitis, or enthesitis) can be classified as having PsA if a total of at least three points is accumulated from the presence of the following list of features (each of which is assigned a certain number of points):

•Skin psoriasis that is:

-Present – two points, OR

-Previously present by history – one point, OR

-A family history of psoriasis, if the patient is not affected – one point

•Nail lesions (onycholysis, pitting) – one point

•Dactylitis (present or past, documented by a rheumatologist) – one point

•Negative rheumatoid factor (RF) – one point

•Juxtaarticular bone formation on radiographs (distinct from osteophytes) – one point

These classification criteria should facilitate studies in PsA and may function well in diagnosing PsA, given sensitivity and specificity in four studies, including two of early arthritis patients, ranging from 91 to 100 percent and 97 to 99 percent, respectively [90-94]. However, these criteria can only be applied to individuals who demonstrate evidence for inflammatory musculoskeletal disease (peripheral arthritis, axial disease, or enthesitis).

Rheumatology has a lot of Rheum (Room) for Internal Medicine.

Bhopalwala. H

Orotic aciduria

Hello Awesomites!
The question has helped me to learn the pyrimidine biosynthesis and urea cycle better. Felt like sharing it.

Let's get started.

OROTIC ACIDURIA= Excessive excretion of orotic acid in urine.

Orotic aciduria is caused by defect in either UMP synthase enzyme or Ornithine trancarbomylase (OTC) enzyme.

When UMP synthase is defective, orotic acid builds up and the synthesis of nucleic acid is impaired, leading to deficient hematopoiesis and growth. Orotic aciduria is associated with megaloblastic anemia due to decreased pyrimidine synthesis. This megaloblastic anemia is refractory to Vitamin B12, folic acid supplementation. This megaloblastic anemia present in infants.

SYMPTOMS

 Lethargy

 Fatigue

Incessant crying due to orotic acid crystals in ureter produces colicky pain

Growth retardation

SIGNS

Tachycardia and faint murmur due to hyperdynamic circulation in anemia.

LABORATORY INVESTIGATION:

Urine analysis show orotic acid crystals.

Peripheral blood smear show hypochromic megaloblastic anemia and hypersegmented neutrophils.

We can distinguish this increase in orotic acid secondary to OTC deficiency from hereditary orotic aciduria by looking at blood ammonia levels and the BUN (blood urea nitrogen).

HYPERAMMONAEMIA is present in OTC deficiency.

TREATMENT

Administration of uridine, which is converted to UMP, will bypass the metabolic block and provide the body with a source of pyrimidine. Uridine triacetate is a drug approved by FDA to be used in the treatment of hereditary orotic aciduria.

Have a great day.

-Upasana Y. :)

RACE protocol in Atrial fibrillation.

Hello! This is a short post regarding RACE protocol. Before starting it, here are some high yielding points regarding Atrial fibrillation.

Most common cause of embolic stroke: Non rheumatic Atrial fibrillation.
Examination shows
1) Pulse: Fast
2) Rhythm: Irregular
3) Pulse deficit more than 10/ min is diagnostic of Atrial fibrillation.

Ecg finding:

1) No P wave
2) R-R interval keep varying

R. A. C. E. protocol is followed in treatment of the Atrial fibrillation.

1) Rate control :

Short acting beta blockers are given to decrease heart rate. Decreasing heart rate leads to increase filling time of the heart. This in turn leads to increase diastolic time. It Increases end diastolic volume, stroke volume, cardiac output and increase BP.

2)Anticoagulation:

Atrial fibrillation causes increase clot formation in heart. Clots can be detected by Transesophageal Echo.

3) Rhythm control:

Antiarrythmic drugs are given to control rhythm.
Chemicals cardioversion is drugs used to terminate ectopic foci.

4) Electrical cardioversion:

If chemical cardioversion fails, go for electrical cardioversion. In this technique patient is sedated and DC shock of 200J biphasic is given.

That's it!

-Demotional bloke

Amiodarone and thyroid dysfunction

Amiodarone, a class III antiarrhythmic drug is associated with a number of side effects, including thyroid dysfunction (both hypo- and hyperthyroidism)

AMIODARONE INDUCED HYPERTHYROIDISM:
There are two types of amiodarone-induced thyrotoxicosis (AIT):

Type I: This type is typically seen in patients with preexisting multinodular goiter or latent Graves disease, the excess iodine from amIODarone results in enhanced thyroid hormone production.

Type II: In type II AIT there is destructive thyroiditis caused by the drug itself that results in excess release of T4 and T3(There is no hormone production). It typically occurs in patients without underlying thyroid disease.

AMIODARONE INDUCED HYPOTHYROIDISM:
1)Normally after exposure to an iodine load (eg, radiocontrast), iodine transport and thyroid hormone synthesis are transiently inhibited to prevent normal individuals from becoming hyperthyroid(the Wolff-Chaikoff effect). Normally patients escape this Wolff-Chaikoff effect and come back to normal within a few weeks, but patients with pre-existing subclinical thyroid disease fail to escape and develop hypothyroidism.

2)Amiodarone also inhibits 5'-deiodinase which is responsible for the peripheral conversion of T4 to T3. So there is a decrease in T3 production.

-Srikar Sama

Wolff-Parkinson's White syndrome

Hello! This post is regarding WPW syndrome. It is an Autosomal recessive disorder.
In Normal heart, conduction is from SA node to cardiac myocytes via AV node and Purkinje fibers. In WPW syndrome, SA node transfers electric activity directly to cardiac myocytes. This transfer is done by 'Bundle of Kent'.  Since AV node is responsible for delay of the conduction in normal heart, skipping of the AV node causes excitation prior to the expected time. Hence low Cardiac output is sign in WPW syndrome.

Investigation: ECG done

Findings in ECG.

1) Since AV node is skipped in conduction, short P-R interval is seen.

2) q wave is responsible for conduction in septal region, here also it is skipped in ECG (Remember conduction directly from SA node to cardiac myocyte!). At the same time we see Delta waves.

What are Delta waves?
-Change in the upswinging of the 'R' wave.

3) PJ interval is normal

What is PJ interval?
-J point is the point where S wave ends.
Starting point of P wave to J point is called as PJ interval.

(Remember: Segments does not include waves, interval loves waves! Example: ST segment is from end of S to start of T wave)

Now, as we can figure it out PJ interval is PR + qRS
PR becomes shorter and qRS becomes  broader in WPW syndrome. qRS is broader because conduction of cardiac myocytes is slower than Purkinje fibers. (Hope you remember clue sentence here - conduction from SA node directly to cardiac myocytes!) Hence PJ interval is normal.

Treatment:

Flecainide given orally is DOC.
Treatment of choice is Radio-frequency ablation.

Submission: Tips for Step 2 CK

Hello All,

I am currently preparing for my step 2 CS exam. I gave my step 2 CK in June 2018 and scored >250.

Here are the resources I used-

1) Onlinemeded lectures+MTB

2) U world Q bank

3) NBME /UWSA

Here is what I. Used to do-

Listen to Online meded lectures and take notes on MTB but I did not read them again. I just listened to OME lectures  2nd time while exercising.

I printed the pdf file circulating with UWorld tables and Followed listening lectures of online meded  with doing questions of Usmle World and taking notes on Tables file.

Then I used to revise whole system I did in the week on weekends

Initially I started with one system a week and in the end I did 2 systems in a week.

Some important points to note-

1) U world and Online meded are the basic resources. 

2) Listen to all the online meded lectures  before solving U world Qs. It helps alot and makes the process of going through Usmle world Qs a lot easier.

3) Memorise Usmle World tables on your tips. 

4) Every option of Usmle world Qs is important. Go through not only the right one but also the wrong options properly.

5) I used to give a NBME every 3-4 weeks to track my progress and gave UWSA in the end. I started with 200’s and went upto 250’s.

-Parneet kaur

Mechanistic insights regarding Lesch-Nyhan syndrome

Hello friends! Let's refresh our biochemistry knowledge today.

Lesch-Nyhan syndrome is characterized by choreoathetosis, dystonia, hyperuricemia, gout, self mutilatory behavior especially self-biting of fingers, and intellectual disability due to HGPRTase mutations.

So how do HGPRTase mutations actually cause dystonia and other extrapyramidal signs and symptoms?

1.)  For the synthesis of dopamine tetrahydrobiopterin as a cofactor for tyrosine hydroxylase is required.
Tetrahydrobiopterin itself is derived by a series of reactions in which GTP cyclohydrolase is a rate-limiting enzyme.
Now HGPRTase deficiency causes depletion of GTP thereby ultimately depleting tetrahydrobiopterin.

In fact, GTP cyclohydrolase mutations are known to cause dopa-responsive dystonia and phenotype similar to Lesch-Nyhan syndrome.

2.)  Secondly, dopamine receptors are linked to G-protein coupled receptors which alternate between GTP-GDP bound states, yet another link between GTP depletion and perturbation of dopamine signaling.

3.) Adenosine deficiency due to the reduction in salvage may adversely affect the role of adenosine as a neuroprotective agent.

Lastly, in Lesch Nyhan syndrome no characteristic imaging abnormalities are seen but reduced dendritic arborizations in the caudate nucleus, putamen, and nucleus accumbens are thought to underlie clinical manifestations.

So to summarise, Lesch Nyhan syndrome can be considered as one of the basal ganglia disorders with Wilson's disease and Huntington's disease being the other notable ones.

Have a great day!

-Kirtan Patolia

Submission :Dexmedetomidine and Bradycardia

-By Parneeet Kaur

Submission: Thyroid Acropachy

Let's go through quick review regarding Thyroid Acropachy! 

1)It is an uncommon finding of Graves disease.

2)It is a triad of clubbing+swelling of soft tissue of digits + periosteal reaction of extremity bones. 

3)It is usually associated with Thyroid Ophthalmopathy and Dermopathy.

X ray findings-Hands and feet involvement,soft tissue swelling, fluffy, asymmetric periosteal reaction

Skin biopsy- Fibroblast activation and GAG deposition.

Differentials: 

1)Pulmonary Osteoarthropathy-.

2)Symmetric periosteal reaction  -can involve long bones of forearms and legs

Treatment-
No Specific treatment available, Treatment directed at associated Ophthalmopathy and Dermopathy using Local corticosteroids and systemic immunosuppressive therapy.

By Parneet kaur

Cyanide poisoning

Cyanide is a mitochondrial toxin that causes death within minutes to hours of exposure.

PATHOPHYSIOLOGY:

1)Cyanide avidly binds to the ferric ion (Fe3+) of complex IV thus inhibiting the Electron transport chain.

2)The cell must then switch to anaerobic metabolism of glucose to generate ATP.Anaerobic metabolism leads to the formation of lactic acid and the development of metabolic acidosis.

TREATMENT:

1)Sodium nitrite and Amyl nitrite induce formation of methemoglobin. Cyanide has high affinity to metHb. This provides an attractive alternative binding site for cyanide which makes the ETC free.

2)Sodium thiosulfate can be given which converts cyanomethemoglobin to thiocyanate and metHb. Thiocyanate is then renally excreted and metHb can be converted back to normal Hb by using methylene blue.

3) Hydroxocobalamin, a precursor of vitamin B12, avidly binds to intracellular cyanide (with greater affinity than cytochrome oxidase) forming cyanocobalamin. This molecule is stable and readily excreted in the urine.

-Srikar Sama

Early morning workout and weight loss.

To reduce weight, early morning exercise is recommended but question is why?
Let's get back to basics before answering this question.

Body has three sources of blood glucose to maintain level uniformly.
1) Food.
2) Liver Glycogen.
3) ‎Gluconeogenesis.

Now, Liver Glycogen can provide energy for around 12-18 hours. Gluconeogenesis uses lots of energy to maintain blood glucose level. Between dinner and breakfast we have gap of around 12 hours. This mean before taking breakfast  liver glycogen stock is null! And body is using now gluconeogenesis to maintain blood glucose level and as you know it's going to take hell lots of ATPs to maintain it. Also, exercise uses lots of energy. Hence both in turn helps in reducing body weight.

What is wrong with evening workout?

Suppose a person has taken lunch around 2 pm and he's working out around 5-6 pm. Which stores will be used by body to maintain glucose level - food obviously! Hardly any Liver glycogen is used up. Also extra food will be stored.

That's all!

-Demotional bloke

Authors diary: Goodbyes

It's heart breaking when you leave work in the evening and show up 10 hours later only to find that the patient you were taking care of passed away during the night.

You don't get to say goodbyes. But my wish for you is that you get to give your condolences 💔 

-IkaN 

USMLE Step 3 CCS sheet guide

Hey guys,

So I recently took my USMLE Step 3 and I gathered some of this from various resources. I think this may be of some help to the beginners.

Let's get started!

In relevant emergency cases (order the ones that are relevant):
ABC:
Airway
Breathing
Circulation

P.O. ICESS:
Pulse oximetry
Oxygen
Intravenous line and fluids (Please remember to type NSS/Normal Saline or Dextrose, etc. CCS software won't take orders that are less than 3 characters!)
Cardiac monitor
ECG
Sugar (fingerstick)
Suction

For chest pain add MONA:
Morphine
Oxygen
Nitrates
Aspirin
~~~
Then order relevant Physical Exam (In Office cases, you'd want to order most physical exams and in the Emergency Department cases, you'd want to order more symptom-based system specific exam)
~~~
Laboratory orders: CBC LFT ICU PAX
Complete Blood Count (CBC), ESR
Basic Metabolic Profile (BMP)
Cardiac enzymes (if not ordered earlier)

Liver Function Tests (LFT) or Lipid profile
FOBT
TFT

Imaging (CT/MRI/USG/etc) Iron profile Immunologic tests (HIV/HepB/HepC/Rubella/etc)
Cultures (Blood/urine/fluid/etc)
Urine (routine, microscopy, culture & sensitivity)

Pregnancy test (urine) & Pap test (if female) PT/INR PTT d-Dimer PFT
Amylase ABG
Xray
~~~
You can now forward the time to get some results or decide whether the location needs to be changed
~~~
CCC
Comfort: if the patient is in pain- give NSAIDs/Morphine based on the situation; vomiting- antiemetic; etc
Cure: if you suspect a particular infection-give antibiotic; if it's an MI- angiography v/s other management options; etc
Consult: you may want to order a Psych or Surgery or OBGYN or any other relevant consult based on the case
~~~
If the patient needs to get admitted on floors or ICU:  ADIC

Activity: Bed rest/ ambulation
Diet
Input/output charting
Compression stockings

~~~

If the patient is scheduled to undergo a procedure: ABC PIN

Antibiotics

Blood grouping and crossmatching

Consent

PT/ PTT

INR

NPO

~~~

When you get your 2-minute screen: Counsel and order follow-up labs!

You may want to counsel them on their diagnosis, lifestyle habits, medication adherence/ compliance/ side effects and so on.

Hope this helps!

Stay awesome :)

-Rippie

Lifestyle modifications for managing hypertension

Hey guys,

Happy New Year!

Let's get started on lifestyle modifications for treating or managing hypertension.

We Decide to Eat less Salt & drink less Alcohol!

Weight loss: Reduce BMI to <25 
DASH: Diet high in fruits and vegetables and low in saturated fat and total fat
Exercise: 30minutes/day for 5-6 days/week
Dietary Sodium: <3 g/day
Alcohol: 2 drinks/day in men and 1 drink/day in women

The effect of these interventions is in descending order, with weight loss having an impact of about 5-20 mmHg lowering per 10 kg weight loss and reducing alcohol intake can lower BP by 2-4 mmHg!

Remember: If a patient's BMI is already lower than 25, you don't have to ask them to reduce weight any further for this therapeutic effect. Instead, you ask them to switch over to DASH diet!

Hope this is helpful!

Stay awesome!
-Rippie

In short: Vasopressin in the ICU

Hello!

Here are some quick points + mnemonics on Vasopressin!

1. Effects are preserved during hypoxia and severe acidosis and catecholamine-resistant states.

Mnemonic: Vasopressin presses when other pressors can't press the vasculature anymore.

2. Vasopressin decreases norepinephrine requirement.

3. Onset: fast, offset: fast.
Mnemonic: VasopressIN is IN and OUT fast.

4. It is often weaned off last in patients on multiple pressors for the same reason.

5. Used in:
- Refractory hypotension (potentiates the actions of over vasoconstrictors)
- Esophageal variceal bleed
- Cardiac arrest
Non ICU indications: vWD, DI, hemophilia

That's all!

-IkaN

In short: Dexmedetomidine and bradycardia

If a patient who is intubated and sedated develops bradycardia, go through the sedatives list - it might give you a hint on what is causing the bradycardia.

Dexmedetomidine (Precedex) is notorious for causing bradycardia. Another sedative associated with bradycardia is propofol.

That's all!

Will update this post at a later date. What you can do if you are free: Read up on it, write a small post on it and email it to us so we can post it and learn from you :)

-IkaN

Authors diary: In short

Hello!

I am planning to write short one line posts on things that I learn in the everyday.

Pediatrics residency series: 1. Intro

Hey Medicowesomites :) Happy new year everyone (writing this post on 1-1-19)

Residency is time-consuming as you know. It’s been a long time since I last posted but I came back to tell you that I will be starting a new series on “Pediatrics Residency”. Useful apps, cases and other things will be discussed.

Stay tuned :)

-Murad

Hair tansplant or follicular transplant

Hello Awesomites!

This is going to be fun. :D

Q. A male diagnosed with AGA (Androgenetic alopecia) came to me with grade 3 alopecia. Asking me that he is frustrated from taking medication and heard of hair transplant surgery. What advice would you give him?

A.I have seen lot of misconception regarding this concept. Hair transplant doesn't mean actual hair. We take follicle from occiput. Why? Because it is not responsive to androgen as there is no Androgen receptor.

Hair transplant is for already bald area. Androgen receptor blockade is given for remaining vellus hair. So that means hair transplantation surgery is not substitute for minoxidil/finasteride. For grade 3 AGA alopecia patient can undergo hair transplantation for bald area but have to take medication for remaining vellus hairs.
This is for AGA alopecia. Scarring alopecia won't show good response with hair transplantation surgery as much as AGA alopecia,

Q. AGA is genetic alopecia. So why don't it appear at birth itself?
A. At birth, androgen receptor is present but insensitive. When genetic component become active, the receptor become sensitive and balding occur.

Have a great day ahead.
Upasana Y. :)

Warfarin: a procoagulant or anticoagulant?

Hello Awesomites!

No doubt ! Warfarin is an oral anticoagulant.
Confused? Don't be. :D

Warfarin inhibits reduction of Vitamin K to its active form and leads to depletion of the vitamin K-dependent clotting factors II,VII,IX and X, and protein C,S and Z.

Because of the rapid depletion of the anti-coagulant Protein C and a slower depletion of factor II, patients might develop increased hypercoagulability during the first few days of warfarin therapy. So warfarin is combined with a parenteral initially. Treatment of DVT/PE with warfarin requires overlap therapy/bridging therapy with a parenteral anticoagulant (UFH,LMWH, or pentasaccharide) for atleast 4-5 days and until the INR reaches atleast 2.0.

The starting dose of warfarin depends on many factors. During warfarin therapy INR monitoring should occur frequently during the first month of warfarin therapy (e.g.twice weekly for 1-2 weeks, then weekly for 2 weeks, then less frequently).

Have a great day ahead.
Upasana Y. :)

ARNI

Hello Awesomites !

I have something with weird titles. :D

ARNI stands for Angiotensin receptor-neprilysin inhibitor.

This is combination of ARB Valsartan and neprilysin inhibitor Sacubitril recently approved for use in patients with HFrEF and NYHA Class II-IV symptoms.

NEPRILYSIN:- It is a neutral endopeptidase involved in the degradation of vasoactive peptides including natriuretic peptides, bradykinin, adrenomedullin. Inhibition of neprilysin increased the availability of these peptides, which exert favorable effects in HF.

Effects of Natriuretic peptides are-
1.Vasodilation
2.Lower blood pressure
3.Reduced sympathetic tone
4.Reduced aldosterone levels
5.Natriuresis/Diuresis

In a large trial, this agent was shown to be superior to enalapril in reducing death and rehospitalization among NYHA class II-IV patients with HFrEF.

Have a great day ahead.
Upasana Y. :)

Vestibulo ocular reflex

The vestibulo-ocular reflex is a reflex, where activation of the vestibular system causes eye movement. This reflex functions to stabilize images on the retinas during head movement by producing eye movements in the direction opposite to head movement, thus preserving the image on the center of the visual field. For example, when the head moves to the right, the eyes move to the left, and vice versa. Since slight head movement is present all the time, VOR is necessary for stabilizing vision.

Circuit:

1)It starts in the vestibular system, where semicircular canals get activated by head rotation and send their impulses via the vestibular nerve and end in the vestibular nuclei in the brainstem.In addition the hair cells of opposite ear are inhibited because endolymph in that ear flows away from hair cells.

2)From these nuclei, fibers cross to the contralateral cranial nerve VI nucleus.

3a)There they synapse with 2 additional pathways. One pathway projects directly to the lateral rectus of the eye via the abducens nerve.
  b) Another nerve tract projects from the abducens nucleus by the medial longitudinal fasciculus to the contralateral oculomotor nucleus, which contains motorneurons specifically activating the medial rectus muscle of the eye through the oculomotor nerve. 

4)For instance, if the head is turned clockwise, then excitatory impulses are sent from the semicircular canal on the right side via the vestibular nerve to the right vestibular nuclei in the brainstem. From this nuclei excitatory fibres cross to the left abducens nucleus.There they project and stimulate the lateral rectus of the left eye via the abducens nerve. In addition, by the right medial longitudinal fasciculus, fibers cross and go to right oculomotor nuclei, they activate the medial rectus muscles on the right eye. As a result, both eyes will turn counter-clockwise.

-Srikar Sama

Basics of Fat necrosis.

Hello, let's dissect fat necrosis in this post.

Fat necrosis is seen where fat concentration is more or lipase concentration is more.
Example- Injury to Breast or Omentum tissue or in Acute pancreatitis with gall stones or alcohol intake.

Alcohol intake leads to activation of lipase enzyme. This lipase enzyme converts lipids to fatty acids. Always remember fatty acids loves calcium! This love affair leads to formation of "Fatty acids - Calcium complex formation". This is called as "Saponification".
This gives yellow - white chalk like color. This helps surgeon to identify fat necrosis.

For prognosis we use serum calcium level. Why?

Suppose there is severe pancreatitis. This leads to more activation of the lipase enzyme. This leads to formation of the fatty acids. More fatty acids, more saponification. Hence less calcium level in serum!

Low calcium level suggest bad prognosis!

Chediak Higashi syndrome.

Hello! This is Ultra short post regarding Chediak higashi syndrome! Hope you like it.

In normal person, when bacteria is engulfed by WBCs, they are carried to lysosome enzyme by LYST protein.
LYST protein stand for Lysosomal transfer protein.

Defect in LYST protein causes Chediak Higashi syndrome. It is autosomal recessive disorder.
No LYST protein so no phagocytosis of macrophages. Hence recurrent infections.

Clinical features:

1) Recurrent infections.

2) Absence of Melanin - Albinism.
LYST also helps in transfer of melanin to superficial layer of skin

3) Decrease in Myelin formation - Delayed conduction.

4) Hemorrhage.
LYST helps in maturation of megakaryocytes to platelets.

Confirmation: Incomplete digestion of bacteria leads to formation of  "Giant granules inside cell"

Mnemonic:
CHEDIAK

C- CNS involvement
HE- Hemorrhage
DI- Decrease immunity
A-Albinism

That's it!

-Demotional bloke.

Horner Syndrome

Horner syndrome is a classic neurologic syndrome whose signs include miosis, ptosis, and anhidrosis.

NEUROANATOMY - Horner syndrome can result from a lesion anywhere along a three-neuron sympathetic pathway that originates in the hypothalamus:
●The first-order neuron descends caudally from the hypothalamus to the first synapse, which is located in the cervical spinal cord (levels C8-T2, also called ciliospinal center of Budge).

●The second-order neuron travels from the sympathetic trunk over the lung apex. It then ascends to the superior cervical ganglion, located near the bifurcation of the common carotid artery.

●The third-order neuron from superior cervical ganglia then ascends within the adventitia of the internal carotid artery, through the cavernous sinus. In the orbit and the eye, the oculosympathetic fibers innervate the iris dilator muscle as well as Müller's muscle, a small smooth muscle in the eyelids responsible for a minor portion of the upper lid elevation and lower lid retraction.

First-order syndrome - Lesions of the sympathetic tracts in the brainstem or cervicothoracic spinal cord can produce a first-order Horner syndrome.
The most common causes are:
(a)occlusion of PICA, which produces Horner syndrome as part of the Wallenberg syndrome.
(b)Brown-Séquard syndrome above T1, patient may present with ipsilateral Horner syndrome due to damage of oculosympathetic pathway.

Second-order syndrome — Second-order or preganglionic Horner syndromes can occur with trauma or surgery involving the spinal cord, thoracic outlet, or lung apex.Other causes include pancoast tumor involving the lung apex.

Third-order syndrome — Third-order Horner syndromes often indicate lesions of the internal carotid artery such as an arterial dissection, thrombosis, or cavernous sinus aneurysm

CLINICAL FEATURES -The classic signs of a Horner syndrome are ptosis, miosis, and anhidrosis.
1)The ptosis occurs as a result of paralysis of the Müller's muscle.
2)The degree of anisocoria is more marked in the dark than in light.
3)Anhidrosis is present in central or preganglionic (first- or second-order) lesions because the sympathetic fibers responsible for facial sweating branch off at the superior cervical ganglion along the external carotid artery and its branches.
4)Horner syndrome is also a common feature of cluster headache.

SOURCE-UpToDate, Kaplan.

-Srikar Sama.

True or False #10

Hallux valgus is also known as bunion. T or F

T

●Hallux valgus (HV) deformity (ie, bunion) is a common, potentially debilitating deformity consisting of lateral deviation of the hallux on the first metatarsal . The etiology is unknown. The deformity is more common among women and shod populations.

●Although HV is easily recognized by clinical examination, radiographs may be necessary to determine the presence of articular damage . Neither radiographic nor clinical appearance provides the basis for surgical referral, which is determined by patient pain and disability.

●There is little evidence that conservative treatments are useful in the treatment of HV. Nevertheless, we suggest patients without debilitating symptoms avail themselves of conservative therapies before being referred for surgery.

Possible treatments include:

•Shoe modification: wide, low-heeled shoes, or specially altered shoes with increased medial pocket for first metatarsophalangeal (MTP) joint to minimize deforming forces

•Orthoses to improve support and alignment

•Night splinting to improve toe alignment

•Stretching and/ormobilization/manipulation to maintain joint mobility

•Medial bunion pads to prevent irritation

•Ice applied after activity to reduce inflammation
•Analgesics: acetaminophen or NSAIDs

●We suggest that patients with severe pain or dysfunction and those whose symptoms do not improve under a conservative treatment regimen be referred for surgical repair.

Approximately 150 surgical procedures for the correction of HV deformity have been described. Few prospective, randomized trials evaluating these procedures have been performed. Patients should be referred to a foot surgery specialist with experience repairing HV deformity.

●Managing patient expectations about surgery is important. Patients should understand that 10 to 25 degrees of valgus angulation is normal at the MTP joint, and that resolution of postoperative pain and swelling may require several months. Most patients will remain unable to fit into narrower shoes.

Do not forget to look up pictures of how a bunion looks.

Over and out.