The differential diagnosis of asterixis is important for a Nephrologists- It is not always Uremia...
Here is another mystery electrolyte disorder with Detective Nephron
https://www.kidneynews.org/view/journals/kidney-news/14/12/article-p16_12.xml
Special post by
Lakshmi Kannan, MBBS, MD,
Department of Nephrology, Pikeville Medical Center
Adjunct Faculty, University of Pikeville Kentucky College of
Osteopathic Medicine
Kentucky, USA
Reference is here
1. Enhance competency based Nephrology education: This is in line similar to COCATs in cardiology.
I think this is a very important move as this will let us focus on the core topics in General Nephrology training. Overall, this is a win win for both fellows and programs
2. Individualize pathways for career goals: This is basically asking to create tracks so that each fellow can create a career niche. After basic general nephrology training, allow for time spent in various sub fields within nephrology( not an extra year of training).
Personally, I am all for this one and have been promoting this at our center for last 6 years. This allows for selection of tracks and focus for each fellow. It makes their fellowship unique from the peers. Focused tracks can make this happen. At our center we do the following tracks and give a certificate for each graduate. But each track has requirements they have to fulfil under a small curriculum within a curriculum. Yes, its time to just be creative over and over.
4. Emphasize training in home therapies- Need more intensive training in PD and HHD.
This is a MUST for all. I think this is important for our patients and our trainees. A win for the fellows. Not a sure win for all programs as some programs may suffer due to lack of patients -- not their fault as its a system's problem at some centers. But this may raise the bar to make sure there is enough faculty who are comfortable to teach PD and HHD and enough volume. Not sure you need a third year for this but rather most fellows graduating should be able to comfortable prescribing and managing PD and HHD. Some fellows who want an academic career may want an extra year of training at specialized centers.
5.Close gaps in current nephrology training. If there were gaps from the above 4 points, programs may need to partner with other societies to close those gaps.
I think this is a temporary solution. Eventually, this will evolve as most programs close the gaps
6. Promote well being of nephrology fellows.
This was important and finally made it to a priority. This will help with burnout in our fellows. Working with NP/PA and restructuring programs will help with this matter. We must not forget our faculty and attending well being as we work on fellows well being. Neither should suffer.
7. Prioritize diversity, equity, inclusion and health care justice
I think this is extremely important. We need more diverse applicants and applicant pool in Nephrology. Diversity brings ideas and promotion of our field forward.
8. Foster interprofessional practice.
This is important and our recent ASN Kidney News sept 2022 issue really highlights this. We are a kidney care team- all should work together.
9. Ensure interdisciplinary practice. Working closely with cardiology, oncology, hepatology and other fields in medicine is critical for our training.
This is a given but almost forgotten. Working with our colleagues closely will be important to foster collaboration and programs to help trainees for both sides. A classic example of this is centers that have created nephro-hospitalists, cardio-renal services etc.
10. Inspire lifelong learning
This is aspirational. This may happen but may not happen. This is individualized but if the program can create venues, programs to continue ongoing learning- this would be very helpful.
Overall, I applaud the entire ASN workgroup on this venture!
The Glomerular Center of Northwell now runs a special monthly GN chat to discuss fun and interesting case based discussions of glomerular disease topics. This is on 3rd Tuesday of each month at 8AM EST
Register in advance for this meeting:
https://northwell.zoom.us/meeting/register/tJElf-iqqzgoE9edbml-AI1dNR07Q1LQOL-m
After registering, you will receive a confirmation email containing information about joining the meeting.
Come learn GN in a coffee style manner with experts in the field- Purva Sharma, Jordan Rosenstock and Kenar Jhaveri
Concept map of AKI associated with chronic liver disease. Made using biorender.com
Inspired by an article in CJASN
Northwell Nephrology is offering both a traditional two year general nephrology fellowship as well as a new fellowship that includes a third year fellowship specializing in glomerular diseases and onconephrology. The traditional two year fellowship can be applied for through this link
A candidate for the Galdi Fellowship will have completed internal medicine residency training, a general nephrology fellowship and demonstrate the highest level of performance and scientific and clinical potential. Candidates for the Galdi Fellowship would be carefully vetted based upon academic and other indications suggesting that advanced training as a Galdi Fellow would enable their ability to become one of a select few international leaders in glomerular diseases and onco-nephrology. The Galdi Fellowship will last for one academic calendar (July through June) year. A new fellow will be recruited each year.Training in glomerular kidney diseases is through the Northwell Nephrology Center for Glomerular Diseases directed by Drs. Kenar Jhaveri and Purva Sharma. The fellow will work in the Glomerular Disease Center and be exposed to all aspects of glomerular disease management including a rotation with Division of Rheumatology for extra training in SLE and ANCA vasculitis. The fellow will also have the opportunity to participate in ongoing clinical trials in glomerulonephritis at the Glomerular Disease Center.
Onconephrology overlaps to an extent with glomerular diseases. Fellows gain experience both in the clinic and hospital with world renowned leaders Rimda Wanchoo and Kenar Jhaveri. Rotations will also include with hematology and oncology teams dealing with multiple myeloma, renal cell cancer and bone marrow transplant services. In addition, the fellow will have rotations with our nephropathologists as well.
Currently we are accepting applications from current nephrology fellows or recent graduates for the Galdi Fellowship for start date of July 2023. In addition we are accepting applications from internal medicine residents for the general nephrology fellowship starting July, 2023 with a third year Galdi fellowship starting in July, 2025.
For inquiries regarding the advanced fellowship program, please email Dr. Kenar Jhaveri at kjhaveri@northwell.edu
The application should include1. CV of the applicant2. Two recommendation letters (one must be from the Nephrology Program Director of Chief)3. A Personal Statement on the reasons for joining this fellowship.
Thrombotic Microangiopathy- what’s in a name?
This is a common conversation:
“ The kidney biopsy confirms TMA”. Great- we should ask hematology to help
treat..
Hematology—“ but there are no signs of microangiopathic hemolytic anemia.. no schistocytes
on smear—no need to treat”
Another conversation
“ The kidney biopsy confirms TMA”. But there are no micro thrombi on the kidney biopsy.
This is likely from HTN—treat the HTN. “
but the patient has a complement factor H mutation..”.. hmm..
The presentation of TMA can be as mild as HTN only, or AKI
on CKD or HTN with CKD or nephritic syndrome or nephrotic syndrome or just
nephrotic range proteinuria with AKI. The clinical presentation is very varied.
I have seen it all.
The problem with TMA starts with the nomenclature. Personally, I have a problem with a series of
diseases in the kidney called TMA—some have angiopathy only, some have
endotheliosis, some have both and some have additional microthrombi but at the
end we call all of them TMA.
Most of TMA cases are without thrombosis, only rarely would
you see true thrombi. Frank thrombosis
is more common in (catastrophic) APLAS associated TMA in the kidney.
Some TMA tends to be more glomerular classically in pregnancy related, for example,
with endothelial cell swelling = endotheliosis). And some TMA tends to be more arterial
(thrombotic angiopathy rather than microangiopathy). Or we might be
catching them at various points in time. A continuous process..
The pathology is also variable and hence should be considered to be defined perhaps with what is noted on it.
Endothelial injury with angiopathy
Endothelial injury with micro thrombi
Endothelial injury with endotheliosis predominant
The cause of the injury than can be defined better based on
history of the patient and then divided into categories as per the syndromes of
TMA or better called “endothelial injury”- such as ADAMTS13 mediated, complement
mediated, Drug induced ( immune vs toxic), Shiga toxin mediated, metabolism
mediated, coagulation mediated and so forth. This figure is from the classic NEJM article by George et al in 2014.
A new agent has been found to cause AKI and AIN—Vonoprazan. A recent paper in Kidney International is the first to describe this from Japan. The authors used the National reporting database of drug toxicities in Japan to assess this and compared it to PPIs—JADER database. See visual ab from the recent paper.
What is vonoprazan?
Vonoprazan, a
potassium-competitive acid blocker possessing a new mechanism of action.
Vonoprazan inhibits acid secretion in the cells of the gastric wall. The
inhibitory effect of vonoprazan on H+, K+-ATPase is perhaps over 300 times
greater than that of lansoprazole.
In Japan, this drug was approved for use for acid reflux in 2015. In the US, this drug has been FDA approved for esophageal esophagitis in association with H pylori recently in May 2022. A recent meta-analysis also found that vonoprazan is non inferior to PPIs as therapy for GERD but in the subgroup for severe erosive esophagitis- it was more effective.
The mechanism of action of vonoprazan is that it competes with potassium ions for the reversible inhibition of H+- K+-ATPase, whereas PPIs act by binding covalently to the gastric H+, K+-ATPase via disulfide bonds. Having a H+, K+-ATPASe in the kidney have any impact? Not sure?
Another interesting finding from another study showed
increase tacrolimus levels when this agent is used- a caution in our GN and
transplant patients.
As we learn more about this agent in the US, we need to be vigilant!
FSGS is a tough diagnosis and often confusing to the Nephrologist. Classifications in FSGS also have been very confusing and challenging. Several years ago, the pathology based classification had entered all textbooks.
Is this classification clinically useful? Not sure it is to most nephrologists? If I have a tip variant FSGS, or Perihilar, does it tell me anything re the cause and outcome? Maybe- but mostly not.
In 2007, there was a movement towards changing the concept to more podocytopathy based. ( see below- recreated using biorender).
Not sure if this is useful either but it really asked a fundamental question re how we are seeing these spectrum of diseases we term FSGS.
The most useful to me personally is classifying the FSGS presentation into
1) primary vs secondary cause
2) nephrotic syndrome vs nephrotic range proteinuria
While not 100% in most cases, nephrotic syndrome and FSGS usually is going to have a primary cause( sparing some genetic causes and viruses). In addition, what is also helpful from a pathology standpoint is not the LM, but the EM--
3) Is there diffuse or partial foot process effacement?- Usually the former responds to treatment better with steroids or other immunosuppression and later is more likely a secondary cause. It may also aid in looking for a secondary cause.
This figure from a JASN paper by De Vriese et al is very helpful indeed.
So FSGS really should be described more in terms of primary vs secondary causes and EM findings to help in treatment decisions. Classically, your "permeability or immune mediated" FSGS should respond to treatment and would fit under nephrotic syndrome, diffuse foot process effacement and classically your primary FSGS. Secondary FSGS from various causes like low nephron mass, obesity, viral , meds- all classically would have nephrotic range proteinuria and sporadic foot process effacement on EM. That being said, some genetic causes of FSGS would be seen to have diffuse foot process effacement as well. Genetic FSGS is an important one to keep in mind and screening for genetic causes should be done: young patient, family hx, resistant to treatment, aiding in post transplant risk. etc.
KDIGO GN 2021 guidelines summarize this nicely
APOL-1 plays an important cause and role here and this slide can summarize the primary and the second hit concept with APOL-1 related FSGS
We should not forget --nonspecific scarring on renal biopsies. FSGS should also be differentiated from focal segmental scarring that develops in immune-mediated GN (e.g., Membranous Nephropathy, IgA Neph, ANCA-associated GN, and lupus nephritis) as a result of post-inflammatory scarring of necrotizing or proliferative lesions. This happens a lot and this should not be treated as FSGS.
In summary, FSGS has come a long way and finally we are seeing some changes in the way we are describing it.. Best 3 ways to categorize FSGS is clinically and EM based.
1. Primary vs Secondary
2. Nephrotic syndrome vs nephrotic range proteinuria
3. Diffuse foot process effacement vs partial foot process effacement
Here is a comprehensive concept map of various known and rare causes of edema. I am sure I am missing some other causes.
A survey done of renal fellows training during the pandemic has now been published.
The link is here https://data.asn-online.org/reports/fs_21/
Some key take home messages of this overall positive survey on our field.
1. Over 80% of fellows felt their program had successfully maintained education and conferences via video and over 80% felt that they were ready for independent practice.
2. Over 80% of fellows saw patients virtually as outpatients and a small number during the inpatient rotations.
3. Burnout was high though during the pandemic ( women more than men)
4. Overall employment perceptions improved from years prior
5. More fellows RECOMMENDED nephrology as a field this time around( the silver lining of the pandemic)
6. Where are fellows going? Nearly 90% start a clinical position, and 2% or so did general internal medicine. Remaining were industry, other fields and joint fields with nephrology
7. Median starting salary was 200,000 US$. Interestingly, IMGs got a higher base pay compared to USMGs. NO difference in female vs male salaries( a big win for Nephrology)
9. Most fellows chose nephrology during residency. Sadly only 6% want to do a career in research.
As we wish everyone Happy New Year in 2022, the year has not really started off happy for many in the NY area( or most of the United States and the world). Omicron variant is flourishing over mankind.
In March 2020, I had witnessed one of the most horrific moments of my career and life as we all saw death and sobering misery in NYC. But at the same time, human kind and all health care had a mission and calling to somehow combat this virus.
2 years later now Jan 2022, we are back in a similar situation. I was on service in early Dec 2021 and life was "covid" normal with mostly non covid admissions and a good mix of interesting nephrology consult cases. Conferences were hybrid and we were doing relatively ok.
Fast forward 3 weeks, and life has changed again. More COVID19 patients in the hospital, some sick, some not. More PPE again.. cafeterias restricting folks on eating, visiting not allowed, people scared again to talk to each other. This time around, there is less fear but more fatigue. This time around, there is sickness but not fear of death. There is more disruption. Disruption everywhere...
This wave is different. I call it the wave of disruption. More nurses, PA, NP, physicians are out and coverage and planning for coverage is challenging. Luckily most have mild symptoms and are returning to work. This wave is causing more cancellations due to disruptions and not due to fear. This wave is causing more delay in health care due to personnel out due to mild covid symptoms or a positive PCR than sickness.
This wave is different as there are more incidental PCR positive findings in both inpatient and outpatient world and we are testing so frequently. The patient who comes in for a fall and femur fracture by chance is found to have PCR + in the ER. This wave is not the SOB, DOE coming in with oxygen requirements for acute COVID. Don't get me wrong, there are some who are coming in with that as well.
This wave is different, it's the wave of " We know COVID treatment better". We know steroids and remdesivir work and avoid intubation if not necessary and we are doing it.. We are doing an amazing job discharging patients and keeping death rates low.. This wave is more disruptive and harm will happen due to shortened and shrinking staff in health care.
This wave is different as there is minimal to no AKI. There is less lung involvement and hence less AKI ( perhaps). Early treatment maybe making a difference. Most of this wave is going to be outpatient phone calls from dialysis units, transplant patients turning positive and what do we do... Most of the phone calls I am receiving are from patients turning positive either because they tested for a trip or have mild symptoms. This will overwhelm the outpatient practices. Virtual visits are back to decrease the disruption again.. This wave will cause dialysis patient placement issues. Cohorts and special units may be possible in 2020 and 2021 but this wave, omicron is everywhere-- perhaps cohort the non COVID ones maybe a better option.
Phone calls from patients, friends, co-workers and family members are constantly telling me- I am positive. Omicron seems inescapable. This variant is everywhere.. NY is again an epicenter for this wave and leading the front in the US( not a proud moment). And this is despite our vaccination rates.
While, this Jan 2022 seems gloom and doom, we have achieved so much in the last 2 years.
Vaccination in record time for almost all age groups, preventing severe cases and death; RCTs showing how some medications and therapies work well such as steroids and perhaps in some cases remdesivir. We have learnt Acute PD again, we have learnt to juggle immunosuppression for GNs and transplant patients. We have learnt to transplant in a pandemic. We have learnt to multi task and do hospitalist work again. Despite the anger and distress in the world, we have learnt to become more human again and help each other more.
While I am not a trained immunologist, I am hoping that Omicron stays mild and takes over Delta and Delta won't have any human hosts left and this would be a silver lining and perhaps an end to the pandemic. With so many people infected ( despite vaccination), endemic status maybe in sight..
Let's hope that 2022 is the year of mankind and not the virus!
