Beighton Score for Joint Hypermobility

Beighton score for joint hypermobility —

JHM should be evaluated in all patients suspected of JHS. JHM is ascertained by determination of their Beighton score, which depends on the presence of JHM in the hands, elbows, lumbar spine, and knees using specific examination techniques . One point is awarded for the ability to perform each of nine maneuvers (including four maneuvers tested bilaterally and evaluation of the spine). A score of 4 or more points represents generalized hypermobility. The specific maneuvers include:

●Passive apposition of the thumb to the volar aspect of the ipsilateral forearm

●Passive hyperextension of fingers, demonstrated by passive dorsiflexion of the fifth metacarpophalangeal joint to at least 90 degrees

●Hyperextension of the elbow to at least 10 degrees

●Hyperextension of the knee to at least 10 degrees

●Flexion of the spine with placement of the palms flat on the floor without bending the knees

The presence of JHM can be documented by an examination limited to those areas required for calculating the Beighton score, but an examination for JHM and joint stability that is adequate for fuller assessment of the patient and the formulation of treatment plans should also encompass the other joints, including the temporomandibular joints, shoulders, hips, cervical and thoracic spine, ankles, and feet.

In addition to determination of the Beighton score based upon the examination, the presence of generalized JHM, including its presence historically, may also be suspected in patients who answer ‘yes’ to two or more questions in a simple five-part questionnaire :

●Can you now (or could you ever) place your hands flat on the floor without bending your knees?

●Can you now (or could you ever) bend your thumb to touch your forearm?

●As a child did you amuse your friends by contorting your body into strange shapes OR could you do splits?

●As a child or teenager did your shoulder or kneecap dislocate on more than one occasion?

●Do you consider yourself double-jointed?

Bhopalwala. H

Standing up & speaking out - 2

Hello people

I am glad for your support. In reality when I first chose to speak up about it , I knew none would support me, I mean why would they.
They all had nothing to gain, but a lot to loose.

I have been with my guide for a long time , there were instances which would seem impossible, I mean how can one do this?
How can someone stoop this low ?

We students were in-charge of her ot slippers. Sketchers worth 2k. As soon as the operation theatre ended we had to keep track of getting it washed and keeping it in her locker.
The ambulance was her personal vehicle, ANC and paediatric patients were made to wait for hours, while the ambulance was used to get her tiffin, drop her students at school.

Whenever she had interviews, we were asked to wear new coats and wait outside her office, and come in one by one , while the camera was rolling and ask her questions and doubts.

The operatives where we would put our hand and leg on the weighing scale to increase the weight of the tumour.

None of this can be proved but it all happened.
Her students passed out years before all have gone through this.
We were like her jewellery to make her look pretty in public , and we were thrown away and stepped on in private.

But I can't take it anymore, and I feel anger and despair that how did I even allow her to treat me like this.
This is a shout out to all those times

Simpson's Grading for Brain Tumors.

Hello Everyone!

So my Neurosurgery residency diaries continue and I continue sharing as I learn.

Learnt about the Grades of Tumor resection while operating on a Glioma.

These are Simpsons Grades of Tumor resection and are correlated as the degree of surgical resection completeness with with symptomatic recurrence. 

Grade I-complete removal including resection of underlying bone and associated dura.

9% symptomatic recurrence at 10 years

Grade II-complete removal and coagulation of dural attachment.

19% symptomatic recurrence at 10 years

Grade III-complete removal without resection of dura or coagulation.

29% symptomatic recurrence at 10 years

Grade IV-subtotal resection of the tumor.

44% symptomatic recurrence at 10 years

Grade V-simple decompression with or without biopsy

100% symptomatic recurrence at 10 years.

That was it!

Let's Learn Together!

-Medha Vyas 

Difference Between Solitary and Singular Brain Metastasis.

Hello Guys!

So we were operating on a metastatic brain lesion the other day when My Consultant happened to ask me the question-  " What is the difference between Solitary and Singular Brain Metastasis?" Well I happened to have a vague idea and managed to blabber something, the actual definition goes as-

• A solitary brain metastasis is defined as the only known metastasis of a tumour in the whole body which happens to be
localised in the central nervous system.

• A singular brain metastasis is defined as a single cerebral metastasis with additional metastases in other organ systems.

Well it's a small nugget, may save you some embarrassing moments.

Let's Learn Together!
-Medha Vyas.

Residency in India: Harassment and speaking up against it

Let me be honest with you, when I first heard about the abuse of a resident by Dr. Rajashree Katke madam, I chose to ignore it.

"She's not that bad," I thought. Doesn't the resident know how the system works?

I'm embarrassed that the system has made me feel like it is okay for mentors to treat you like this. Mentors are supposed to guide you, inspire you. Not humiliate you at every chance they get.

I'm ashamed of myself because I'm used to how the government hospitals in India can get and used to not raising my voice against it.

I forgot how deeply I condemned such practices - people like her are one of the reasons why I didn't want to do my residency in India.

I worked as a CMO under Dr. Rajashree Katke and I've seen students, residents, medical officers, nurses, paramedical staff and even peons be verbally abused by her at some point. I'm confident none of them will come forward. (I'm also confident that I'll never get my experience letter after writing this, the one I asked for multiple times but never got signed because I didn't show up with an expensive gift.)

People who are asking for proof: There'll be no proof. I wish I recorded her disrespecting people. There'll be no proof of her refusing to teach if her demands aren't met. I can recall residents waiting for her outside her office. Isn't someone's word proof? Is it not evidence?

I'm glad the resident spoke up. Residency is tough. Isn't there too much work already for doctors? Is it too much to expect humane behavior from our mentor?

To the resident:
Maybe you weren't an unlucky girl.

Maybe it was fate who wanted you to be in that position, so that you can raise your voice against such people. You are the reason that hopefully, this abuse will end.

And no other resident will have to deal with this ever again.

Thanks for raising your voice.

Nakeya

This post is supporting a resident and our dear author, Sakkan. She has been doing her residency with a verbally abusive mentor. 

She decided to speak up against it and without our support, no action will be taken. Read more here:

https://m.facebook.com/story.php?story_fbid=1062008630649068&id=750938228422778

https://www.medicowesome.com/2019/02/standing-up-speaking-out.html

https://www.thehindu.com/news/cities/mumbai/resident-docs-seek-action-against-jj-professor/article26325048.ece

https://www.freepressjournal.in/mumbai/mumbai-mard-puts-forth-demands-to-jj-hospital-administration/1464928

https://www.loksatta.com/mumbai-news/jj-the-hospital-dr-rajshree-katke-again-once-in-controversy-1843259/

https://m.timesofindia.com/city/mumbai/medical-pupil-senior-doctor-treating-me-like-maid/articleshow/68055883.cms

https://m.navbharattimes.indiatimes.com/metro/mumbai/crime/exploitation-allegation-by-resident-doctors-of-jj-hospital/articleshow/68054261.cms

Standing up & speaking out

Hello everyone.
I used to be an author here.
But haven't been active since I got into post-graduation.

I want to tell the story of my journey.

During MBBS the most wonderful thing I ever witnessed was a childbirth with all the gore , the scream, the horridness.
But the moment the child was born my heart would sing " Innocence" by Avril Lavigne

Alas after hours and hours of studying day and night, missing out on life, missing out on religion & social life, missing out on your friends..it payed of and I scored a seat.
And I took in OBGY

I chose my hospital, cause I wanted to be near to my home. It was very tempting to pick up other options, but I choose to be in Mumbai , cause I had spent too many years away from home.

The next big question after choosing the hospital is who you get as a guide. My department had a chit system, through which I got allotted my recent guide.
Now being from Mumbai I was aware of the type of person she is, my cousin had worked under her , so I was very well aware of it all.
But my naseeb was a bit too short and I picked her name in the chit. I was devastated.
But I had no option, so I decided to buckle up and just stick with it for the next three years. After all my home was here, I felt falsely assured.

In first year of residency, I was a bystander , silently observing what my seniors went through day and night.
I quietly empathized , sometimes even asking my seniors to just be calm, to not let her affect them this much. That it would pass, that it was okay.

Things weren't that different when I became a year senior.
Every day was a terror, waking up , sleeping at night every minute my guide thoughts would torment me.
She would call late at night, sometimes even 11pm, to know where I was. If I failed to attend she would send my juniors to my room to know where was I.

She judged and demanded that my clothes, my hair , my makeup every thing should be to her standards, sometimes going to the extent of even calling me a kaamwalibai / maid in public.
She would make me sit outside her chambers from 9am to 7pm, just so that I was always available for her vip visitors.
After 7pm she would take me to her home for her entertainment and gossip buddy.
Relieving me late night at 9 /9:30 pm.
I had to rush to see my ward work, only for her to not listen to my informing till late night.

It was an accepted norm to give her gifts, either an esbeda bag or a silk sari , a min budget of 5k and not less. The first thing she saw on the gifts was the price tag.

If I fell short I would be chucked out of the operation theatre.
I had to suck up to her. I had to. I mean she is my guide yes.
I did it all. All that was necessary

But not anymore.

I have worked under teachers who made me a better person, who taught me self respect, who gave me the confidence to stand tall.

But she affected me to an extent , that I felt more like a slave, a slave hungry for cutting and operatives.
I doubted myself, I hated myself, I cried silently.
I stopped buying new clothes and being myself.
It was a common norm within my seniors to not take bath and change clothes, so she wouldnt take us out to her conferences to hold her purse, I followed too for a time being.

I have a raised a voice now. I just want to be heard.
To all the medicos , there will be times when you think that it is okay to compromise , that it is a profession where you need to dance around your seniors.
No one deserves to be treated this way.

Have pride in what you are.

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