Kidney SMART: For medical students

ASN initiative to spark research interest in nephrology for medical students.  After completing one year course in medical school, students can attend a one week renal physiology course with great sessions.
And then they get to attend the ASN kidney week during their later part of medical school.
It mirrors from the fellows course at Mount Desert Island Biologic Labs.
Check out the official website at Kidney SMART

ANCAs and Alpha-1 antitrypsin- any link?

Alpha 1- antitrypsin (AAT) is a major inhibitor of proteinase 3.  In one with AAT deficiency, it has been postulated that there may be an increase in proteinase 3 activity due to inability of AAT to inactivate the proteinase 3.  This imbalance may lead to ANCA vasculitis.  See below for some interesting aspects of this association. One study looked at an association of the allele of AAT deficiency and relation to ANCA disease. While a cause and effect cannot be proven, this is an interesting association. As early as 1993, this assocaition was encountered and studied. Interestingly, another study that looked at AAT inhibitor phenotypes and levels examined in 40 ANCA positive cases of systemic vasculitis, an excess of PiZ and PiS alleles were associated with the development of pulmonary haemorrhage and alpha-1-proteinase inhibitor levels were lower in the subgroup with pulmonary haemorrhage. However, this allelic imbalance and reduced alpha-1-proteinase inhibitor level was not confined to antiproteinase 3 positive patients and did not appear to be associated with other organ involvement or disease severity.

Post by

-Mala Sachdeva, MD

Clinical Case 65: Answers and Summary

A 34 YEAR OLD FEMALE ON HEMODIALYSIS GETS PREGNANT. HOW WOULD YOU ADJUST THE PRESCRIPTION ON DIALYSIS TO HAVE THE BEST OUTCOME FOR MOTHER AND BABY?

Change to Peritoneal dialysis

  7 (9%)

 

Since the largest data suggests poor outcomes, would suggest to not continue pregnancy

  2 (2%)

 

Daily dialysis for total of 12-15 hours per week

  38 (50%)

 

Three times a week dialysis as her regular prescription

  6 (7%)

 

Three times a week dialysis but goal pre-dialysis BUN<35-40mg/dl

  23 (30%)

 

Pregnancy has been reported in dialysis patients. Over 70% of 80 pregnancies reported in one large series, had resulted in surviving infants and no maternal deaths. The largest case series to date of pregnant HD patients is 52 patients over 20 years. In that experience, HD was performed daily but total weekly treatments were shorter( 12-15 hours per week). UF was avoided and over 85% of pregnancies ended up with surviving infants. Most were pre terms. BUN concentration is <35mg/dl. In other words, pregnancy can be successfully tried in HD patients in the right circumstances and in the experienced centers. Most answered the question correctly. 

Consult Rounds: Distal RTA and Sjorgren's Syndrome

Distal renal tubular acidosis in Sjorgren’s Syndrome (SS):

1. One of the mechanisms is an absence of the H-ATPase pump on intercalated cells in the collecting duct.  

2. Also, Sjogren's syndrome (SS) leads to autoantibodies directed against carbonic anhydrase II.

This leads to less proton excretion.

3. Severe hypokalemia might also suggest that there is a combined proximal and distal RTA.
4. Full blown fanconi syndrome has been described in SS as well.

5. Severe hypokalemia can occur in SS despite no RTA and is thought to be due to tubular damage induced sodium wasting with subsequent increased distal sodium delivery.  

6. Chronic hypokalemia can lead to a nephrogenic diabetes insipidus(NDI)

7. Regarding NDI, the largest series is an Italian series 21% of patients were noted to have an abnormal urinary concentrating ability. Lymphocytic infiltrates of the collecting duct might be the cause. 

8. A nice attending rounds in CJASN discusses hypokalemic metabolic acidosis. 

Ethics in Dialysis practices

" You stole my patient when she was admitted to a hospital I don't go to". " How come all my dialysis patients are being taken away by the other group in town as they open a new unit?"

These are concerns and ethical issues that many nephrologists in practice face as competition arises between practicing groups. A recent CJASN article highlights many issues that we face ethically when such issues arise.  This paper is almost as close to a policy statement re such unethical practices that are business minded and not patient centered.
Key points that are discussed have to deal with how one group can refrain from soliciting other groups patients and keep their business interests aside while taking care of patients.

Tips suggested are:

1. Rescual ( don't get involved in care of the other group's patients)
2. Avoid soliciting
3. Full transparency to the patient if you have to get involved.
4. Avoid self referrals to one's unit or office
5. Provide a collegial environment( while competition is good, we all went into this profession for patient benefit)

Glad an article to this regard is published in nephrology. Its worth applauding the authors on a topic that is often faced by many of us in a competitive environment; and to highlight that such tactics that are often used are unethical and remove us far far away from our professional oath.

Communication Skills Training for Dialysis Decision-Making and End-of-Life Care in Nephrology

Communication is an essential component of nephrology care, yet nephrologists receive little training in communication. We developed a communication workshop for nephrology fellows, NephroTalk, to address common communication topics encountered including:  giving a diagnosis; discussing the risks and benefits of treatment options; and addressing end-of-life decision-making, especially in elderly, medically complex patients. Our curriculum, modeled after OncoTalk a successful communication skills program for oncology fellows, was comprised of didactic and practice sessions with simulated patients and nephrology cases.

The workshop consisted of one-half day divided into two sessions. Sessions addressed common communication scenarios in nephrology: delivering bad news and defining goals of care when the patient is doing poorly. For each session, an overview presentation highlighted the skills to be practiced including a faculty demonstration of the skills. After each overview presentation, fellows were divided into small groups each led by a facilitator for skills practice using standardized patients. For each practice session, the facilitator followed a reflective process-oriented framework that focused on identifying the practicing fellow’s goal and providing the tools to accomplish this goal.

The skills taught included the following: giving information using Ask-Tell-Ask; recognizing and responding to emotion using the NURSE acronym; open-ended questions to elicit care goals and end-of-life preferences; and using “wish” statements to respond to unrealistic goals.

Twenty-two fellows participated in the workshop from University of Pittsburgh and Duke University. We measured perceived preparedness using pre- and post- workshop surveys. Overall, perceived preparedness following training increased for all communication challenges including; delivering bad news, expressing empathy, and discussing dialysis initiation and withdrawal. Fellows rated the course highly and recommended it to other fellows. Qualitative comments highlighted how the training would impact future practice: “Listen more intently, limit use of medical terminology further, give patients more opportunity to express feelings.”

NephroTalk is an interactive communication workshop to enhance nephrology fellow communication skills using didactics and practice sessions to address common communication tasks in nephrology. From this work, future direction would involve disseminating our curriculum to other institutions and enhancing the education of nephrology educators and attendings. 

For the a full article on this, check out: 

http://www.ncbi.nlm.nih.gov/pubmed/23143502

http://www.ncbi.nlm.nih.gov/pubmed/23078102

Special post by

Jane Schell MD

MGRS: Monoclonal Gammopathy of RENAL SIGNIFICANCE: A new name for an old entity to define treatment

What happens with we find renal pathology findings and they confirm a monoclonal strain of B cell clone. A bone marrow is done and there is MGUS revealed. Is that now MGUS really undetermined or insignificant.  A new term now referred to MGUS disorders with renal biopsy findings as MGRS( monoclonal gammopathy of renal significance). These patients are hard to treat as they are never classified as having a hematologic disease. They are usually classified as MGUS with MIDD or MGUS with MPGN.  Other diseases that have been identified to be consistent with monoclonality are fibrillary, immunotactoid and certain cryoglobulinemias. 

A lot of the MGUS patients with renal disease have been receiving no treatment or undertreated given the confusion. No one receives standard therapy for MM at the time of diagnosis.

How do we treat these disorders? A recent article by Leunget al in Blood summarizes some suggestions: Treating the underlying clone, myeloma-based treatments have shown more response rates although lymphoma based treatments have been used as well.  The authors think that these disorders don’t require treatment from a “tumoral” standpoint but from a renal deterioration standpoint it’s needed. Hence the term MGRS fits better for this entity.

Diseases that are now associated with MGRS( or could have been classified)

1.     MIDD

2.     AL amyloidosis

3.     Fibrillary GN

4.     Type I and II Cryoglobulinemic GN

5.     Immunotactoid GN

6.     GOMMID

7.     Proliferative GN with monoclonal deposits

8.     MPGN