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Contributors: A Bansal (U of Pennsylvania) | P Dedhia

(U of Cincinnati) | A Elebiary (Lahey Clinic) | X Vela (U
of El Salvador) | D Thomson (ECU) | P Jawa (ECU) | S
Sridharan (Lister Hospital, UK) | F Iannuzsella (IRCCS,
Italy) | D Mitema (Johns Hopkins U) | Malvinder Parmar
(Northern Ontario, Canada) | Wisit Cheungpasitporn
(Mayo)

I ssue 3

Volume 2

Year 2015

URL http://goo.gl/QDSB5B

#Kidney
KONNECTI ON
Editor: Tejas Desai | Chief: Cynthia Christiano | Free subscription by emailing myFellowship@ecu.edu

THE TRAGIC CASE OF "CREATININA"

WHERE EARNING A LIVING COULD EARN YOU AN EARLY DEATH
The prevalence of CKD in
developing countries is known to
be increasing in part due to the
burden of emerging
non-communicable diseases such
as diabetes mellitus, hypertension,
and obesity. These CKD risk
factors are often associated with
adoption of a more sedentary
lifestyle. In Central America,
however, CKD is currently
manifest in epidemic proportions
and is being observed in a rather
peculiar geo-demographic
population in the absence of
diabetes and/or hypertension.
This CKD of unknown origin is
currently termed ?Meso-American
Nephropathy?, and it has been
gaining some well-deserved
attention recently (CNN, NPR,
and NBC have carried out
features recently), given the
concern that it has probably
contributed to the premature death
of more than 20,000 young men
in the affected regions.
Let?s attempt to break down

by D. Mitema (@mitemadog)

Mesoamerican Nephropathy. First, ?Meso-America?

for the purposes of the this discussion encompasses
the geographic regions of Southeastern Mexico,
Guatemala, Belize, El Salvador, Honduras, Nicaragua,
Costa Rica, and Panama. (Figure 1, source:
Wikipedia). From a geodemographic standpoint, the

mortality amongst men has led to

a nickname for the community of
survivors: ?La Isla de Viudas,? or
?The Island of Widows.?

How do these patients present to

clinicians? For starters, the disease
is quite asymptomatic until late, as
is the case with many causes of
CKD. Patients typically present
with impairment in their
glomerular filtration rate (in the
absence of diabetes and
hypertension per case definition),
with additional studies showing
rather bland urinary sediment and
non-nephrotic range proteinuria
typically <1g/24hrs. There is
progressive decline in eGFR and
many patients progress to ESRD,
observed nephropathy shows preponderance for young though the precise natural history
men (male to female ratio is 3-4:1) typically age 30-50 of the disease is yet to be defined.
years, exposed to working under hot conditions in
There are limited studies looking
predominantly agricultural communities in the low
at the histopathology of the
lying coastal areas. In the lowlands of the Nicaraguan disease, though the predominant
pacific coast, the condition is colloquially referred to as finding seems to be
?creatinina?, and it is the leading cause of death in
tubulo-interstitial scarring, with
young men working in sugar cane plantation. In
some case series also
Chichigalpa, Nicaragua, the significantly high
demonstrating glomerular

9 A common method of classifying systemic

vasculitides relies upon this blood vessel characteristic

ischemia and glomerulosclerosis.

using plasma exchange in patients with granulomatosis

with polyangiitis who suffer from severe kidney damage
(Cr 5.7 mg/dl) or alveolar hemorrhage

10 Acronym of the trial that showed potential benefit of

8 The former name for Granulomatosis with Polyangitis

The specific cause for MeN remains elusive, though

it is speculated to be multifactorial. The Report
from the First International Research Workshop on
MeN? (available at http://goo.gl/o6A66Y) has
proposed the following framework for development
of MeN:

7 Acronym of the scoring system used to assess the

degree of remission or relapse of a small vessel vasculitis

1 PR3-ANCA is positive 75% of the time in this type

of small vessel vasculitis

6 Acronym of the trial that compared oral versus

intravenous cyclophosphamide as induction therapy
against granulomatosis with polyangiitis

5 A large percentage of Churg-Strauss patients suffer

with this

4 The pathogenic molecule in anti-GBM disease

Down

granulomtosis with polyangiitis

3 ANCAs cause tissue damage by activating monocytes
and these cells

11 Acronym of the trial that compared azathioprine to

cyclophosphamide for maintenance therapy in
2 These antibodies are not necessarily positive in
vasculitic disorders, nor are they necessarily negative in
healthy patients

Across

ANSWERS

HOW WELL DO YOU KNOW...ANCA ASSOCIATED VASCULITIS

("Creatinina" continued)

Currently, one theory that seems to be gaining some

traction is that volume depletion (some workers in
Nicaragua lose up to 2.6kg per day after working in
the hot sun!) coupled with dehydration and
hyperosmolarity trigger chronic activation of
vasopressin and the aldose reductase pathway. The
end result of the chronic activation of these
pathways has recently been shown to contribute to
renal injury. To date, no specific nephrotoxin
(heavy metals, pesticides etc) has been
demonstrated to be the causative agent. Much work
still needs to be done to identify the cause of this
epidemic of CKD in Central America.
Treatment strategies in the resource limited setting
are mainly conservative. Many patients cannot
afford renal replacement therapies, and as a result
main focus at this time is on prevention. There are
now measures in place to provide sugar-cane
workers with hydration, rest, and shade in addition
to screening them intermittently for kidney disease
while additional studies are undertaken to establish
the causative agent/mechanism for this disease. For
those students/residents/fellows interested in
additional resources on this topic, please visit the
following links:
- regionalnephropathy.org
- storify.com/nephondemand/meso-american-nephropathy