Kidney Failure-- It is very rare for kidney stones to cause kidney failure, although some factors produce a higher risk. They include the following:
According to the Merck Manual:
Prognosis and Treatment
"Eventually, end-stage renal disease usually develops. Decreasing the urinary concentration of cystine decreases renal toxicity". [12]
www.merck.com/mmpe/sec19/ch291/ch291b.html
What is crystalluria? Crystals in the urine
What causes cystine crystalluria? an inborn renal transport error.
Only persons with a rare metabolic disorder have abnormal excretions of cystine crystals. The abnormal excretions of cystine crystalluria (cystals in the urine) may cause chronic pain in some individuals.The chronic pain is most likely an irritation and inflammation of the bladder wall caused by the abnormal amounts of cystine crystals. It is generally may or may not be concurrent with hematuria (blood in the urine) and because of the compostion of the amino acid, cystine, will not be seen on radiology test or scans. The general region of pain is pelvic and lower back pain, and at times pain may become acute.
Indivar is a drugged used to treat AIDs/HIV patients that also causes medicinal crystalluria.
According to Brian Lipps, MD, "A cystine kidney stone usually presents with pain and passing of a stone, similar to other stones. Unlike other stones, some people complain of continual pain even after the acute episode has passed." [16]
Many cystinurics report chronic and often acute pain despite no hematuria (blood in urine) or despite an unremarkable CT scan. Cystine crystalluria is idiopathic by nature, and research on the effects of cystine crystals on the renal system have not fully been established. The pain associated with cystine crystalluria (as oppose to stone passage) is generalized to the pelvic region (bladder) and lower back region. The pelvic is "guarded-type" pain (tender to the touch). The lower back pain is distinctively different than the pain associated with stone passage or renal blockage. However, the pain is chronic and persistent. Another problem, cystine is massively excreted by cystinurics (cystinurics pass >400mg a day--this is the determining diagnostic factor of cystinuria). There is no known cure or any drug that can eliminate cystine excretions. Once excreted, there are three major drugs (captopril, tiopronin, or penicillamine) that produce different degrees of effectivness and results. All three drugs are commonly known to cause Nephrotic Syndrom or proteinuria (protein in the urine). This is well documented. [1]. Out of the three, captopril is the least effective, but produced less toxic side effects. Tipronin and penicillamine are not tolerated well by patients because of common, severe symptoms. There are no studys on the effect of cystine crystalluria or its' renal toxicity on the urinary tract. Unlike indinivar crystalluria, there is no notable renal sludging. Studies are needed to fully establish the effects of the massive cystine excretions on the urinary tract.
Renal toxicity of medications, massive cystines excretions, and the overall composition of cystine contribute to the chronic pain associated with this rare, inherited disease.
According to Dr.C.A.Jenner MB BS, FRCA, Cystinuria (cystine in the urine) is listed as one of the four named conditions or diseases which causes chronic bladder pain. (bladder infections, interstitial cystitis, cystinuria, and protatitis) Dr. Jenner defines bladder pain:
"Bladder pain is defined as the pain that emanates from the urinary bladder. The bladder is a hollow organ in the lower abdomen. It stores urine, the liquid waste produced by the kidney, which passes into the bladder through a tube called a urethra. Pain can also be felt in the abdomen, above the pubic bone, genitals, lower back and anus." [17]
Related Studies related cystine crystals:
Cystine Crystals/ Biopsies--Abstract
"We have biopsied the papillae of patients who have cystine stones asking if this stone type is associated with specific tissue changes. We studied seven cystine stone formers (SF) treated with percutaneous nephrolithotomy using digital video imaging of renal papillae for mapping and obtained papillary biopsies. Biopsies were analyzed by routine light and electron microscopy, infrared spectroscopy, electron diffraction, and micro-CT. Many ducts of Bellini (BD) had an enlarged ostium, and all such were plugged with cystine crystals, and had injured or absent lining cells with a surrounding interstitium that was inflamed to fibrotic. Crystal plugs often projected into the urinary space. Many inner medullary collecting ducts (IMCD) were dilated with or without crystal plugging. Apatite crystals were identified in the lumens of loops of Henle and IMCD. Abundance of interstitial Randall's plaque was equivalent in amount to that of non-SF. In the cortex, glomerular obsolescence and interstitial fibrosis exceeded normal. Cystine crystallizes in BD with the probable result of cell injury, interstitial reaction, nephron obstruction, and with the potential of inducing cortical change and loss of IMCD tubular fluid pH regulation, resulting in apatite formation. The pattern of IMCD dilation, and loss of medullary structures is most compatible with such obstruction, either from BD lumen plugs or urinary tract obstruction from stones themselves". [7], [15]
Studies on low-methionine diet information are conflicting. There is no study determining whether are not this is either effective or that patients are actually following such a diet.
These are the basic guideline for a low-methionine diet that I have be able to obtain:
There are a greater number fruits that are low in methionine.
There are fewer vegetables that are low in methionine.
The following should be strictly limited or restricted* when following a low-methionine diet :
Seafood*
Meat
Nuts and Seeds
Eggs and Dairy
Grains (pasta and breads)
Lunas (any beans including soy products like tofu)
NOTE:
Because Cystinurics has an inborn disease affecting the transport of Amino Acid, it is very difficult to avoid amino acids since every living organism (animals and plants. contains amino acids. Methionine is processed into cystine in the human body.
Before one eliminates anything from one's diet, one must first understand what exactly "it" means.
What is Methionine?
Answer according to : The Nutrition Notebook @ Springboard
|
Methionine is an essential amino acid. This means that it must be obtained through the diet in adequate quantities to meet the body's needs. Methionine is used in the manufacture of taurine, which is an important amino acid for cardiac function as well as serving as a brain neurotransmitter. Deficiencies of methionine which are found to be associated with a poor-quality dietary protein intake can result in taurine, cysteine, and one-carbon metabolite deficiencies. Insufficiencies of methionine can result in poor synthesis of phosphatidylcholine and other phospholipids. These substances are essential for nervous system function as well as prevention of blood cell stickiness. Supplementation with methionine is often seen in soy-based protein formulas to improve the protein quality. L-methionine supplementation of soy protein will raise its protein efficiency ratio by providing enhanced levels of this amino acid which is deficient in soy protein. Excessive intake of methionine can aggravate some forms of schizophrenia and encourage stuvite kidney stone formation in sensitive individuals. Therapeutic doses of methionine range between 500 and 1,000 mg per day. |
Method of Action
Methionine is converted to S-adenosyl methionine, which then serves as a methyl group donor for the synthesis of substances such as ethanolamine. Ethanolamine is further methylated in the body and converted to phosphatidylcholine, which is found in lecithin.
Methionine is also converted into homocysteine, which reconverted back to methionine through the trans-sulfuration pathway. Homocysteine should not build up in the body; if it does, it is associated with an increased risk to heart disease and atherosclerosis. The poor conversion of homocysteine to methionine is caused by vitamin B-6 deficiency in genetically susceptible individuals.
Methionine is incorporated into proteins. A major route of its metabolism involves conversion to S-adenosyl methionine (SAM). SAM is a key intermediate in the transsulfuration pathway, which results in the manufacture of diverse substances such as taurine and carnitine. SAM is converted to homocysteine, which can be reconverted to methionine, but adequate levels of vitamin B-6 are required. A genetic defect has been found which prevents proper conversion of homocysteine to methionine. This is associated with increased risk to atherosclerosis (coronary artery disease). This block can be overcome by administering higher levels of vitamin B-6 and/or betaine, which promote these sluggish enzymes and facilitate better conversion of homocysteine to methionine.
Plasma or urinary levels of homocysteine should be zero. Elevations indicated increased risk to coronary artery disease.
http://www.springboard4health.com/notebook/proteins_methionine.html
Restricting amounts of animal protein is encouraged in cystinurics.
Examples of vegetables that are high in methionine:
Spinach, Green Peas, cornBroccoli, Mushroom, Cauliflower, Avocado, Bean sprouts, and potatoes.
Related Study:
The Journal of Biological Chemistry: Cystinuria. The Effect of Amino Acids on the Excretion of Cystine., by W. C. Hess and M. X. Sullivan (1941)
Click here: http://www.jbc.org/cgi/reprint/142/1/3.pdf
[1] JAMA : The Journal of American Medical Association
(Membership Required)
[2] The Kidney Stone Book, Chapter 24 List of Medications
http://www.kidneystonesbook.net/meds.html
[3] KidneyStone.org
http://www.urologystone.com/CH07TreatmentOptions/otherMedication.html
[4] Kidney Stone Health Basis
[5] Uro/Stone Center:
urostonecenter.com
910.362.8765
http://www.urostonecenter.com/prevention.asp
[6] Kidney Stones: 09/2001/ What are Kidney Stones?
http://www.reutershealth.com/wellconnected/doc81.html
[7] Kidney International: Official Journal of the International Society of Nephrology
http://www.nature.com/ki/journal/v59/n5/full/4492224a.html
[8] Journal of Urology. 2001 Jun;165 (6pt 2): 2328:30.
Cystine Calculi in children: the results of a Metabolic Evaluation and Response to Medical Therapy
Department of Urology,
[9] Yale J Biol Med. 1981 Nov-Dec;54(6):439-46
[10] Patient Education Resources: Modern Medicine: healthy patients/healthy practice
http://www.modernmedicine.com/modernmedicine/article/articleDetail.jsp?id=108310
[11] Mission Pharmacal ® © 2006 MISSION PHARMACAL COMPANY
http://www.missionpharmacal.com/about/randd.aspx
[12] Cystinuria: Renal Transport Abnormalities: Merck Manual Professional
www.merck.com/mmpe/sec19/ch291/ch291b.html
[13]
[14] Urological Research, 1981;9(4):163-8.
"Cystine Crystalluria and Urinary Saturation in Cystine and Non-Cystine Stones"
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=7269003&dopt=Abstract
[15] Kidney International (2006) 69, 22272235. doi:10.1038/sj.ki.5000268; published online 17 May 2006
http://www.nature.com/ki/journal/v69/n12/abs/5000268a.html
[16] About: Kidney Stones "Cystine Kidney Stones" Your Guide to Kidney Diseases, Brian Lipp, MD
http://kidneydiseases.about.com/od/stonetypes/a/Overview0011.htm?terms=cystine+stones
[17] 15th October 2006 at 19:32 BST by Dr.C.A.Jenner MB BS, FRCA
http://www.londonpainconsultants.com/articles/2006/10/15/bladder_pain/
[18] Keck Graduate Institute of Applied Life Sciences
535 Watson Drive, Claremont, CA 91711 p (909) 607-7855 f (909) 607-8086
[19] Mayo Clinic: Clinical Trials (Experimental)
http://clinicaltrials.mayo.edu/clinicaltrialdetails.cfm?trial_id=100377
cystine calculi
Patients with cystine calculi have an autosomal recessive disorder of dibasic amino acid transport leading to decreased cystine resorption in the kidney. Only homozygote patients form cystine calculi and often present with stones during childhood. Calculi may be pure cystine or may be mixed with calcium oxalate. Cystine is poorly soluble at normal urinary pH and will readily form stones when levels rise above a concentration of 250 mg per L. Pure cystine stones are yellow and radiolucent or faintly radiopaque. A urinary cystine level of more than 250 mg per 24 hours (1,040 µmol per day) is diagnostic for the disorder
Dietary manipulation with a low-methionine diet is difficult and rarely successful. Hydration and administration of urinary alkalinizing agents such as potassium citrate are mainstays of therapy. However, it often is difficult to achieve adequate alkalinization with oral agents. If these measures are not effective, administration of cystine binders such as penicillamine (Cuprimine) and tiopronin (Thiola) can help prevent cystine calculi. Although these agents are effective, they can cause significant side effects such as gastrointestinal distress, rheumatologic symptoms, mental status changes, and skin rashes.30
Studies show that potassium citrate helps the formation of calcium and uric acid stones.
Where are the studies for cystine stones?
EMedicine.com:
http://www.emedicine.com/MED/topic3030.htm
Letter: Treatment of renal obstruction caused by cystine crystals or stones
JAMA, Aug 1974; 229: 1045.
Journal of American Medical Association
ABCs of Medical Management of Stones (3rd Revised Edition) et al Dr. Charles Pak
The Effect of aN Emergency Medicine Structured Data collection form in Aiding in Acute abdominal Pain examintation and diagnosis
http://www.sys.virginia.edu/hci/papers/CapstonePaper2.doc
Kidney International (2001) 59, 18211833; doi:10.1046/j.1523-1755.2001.0590051821.x
Without preventive treatment, calcium stones recur in 10% of patients within a year of the first attack, in 33% within five years, and in 50% by 10 years. Individual risk for recurrence, however, varies. For example, a 15-year old patient with inherited cystine stones has much higher than average odds for recurrence, while a middle-aged old man with a first calcium oxalate stone has a good chance of never passing another.
http://www.reutershealth.com/wellconnected/doc81.html
Miner Electrolyte Metab. 1994;20(6):414-23.
PMID: 7783705 [PubMed - indexed for MEDLINE]
Canadian Medical Association Journal (Webmasters Pick for Reading Material Cystinuria)
Can Med Assoc J. 1934 September; 31(3): 250255.
From the Departments of Urology and Metabolism, the
http://www.pubmedcentral.nih.gov/pagerender.fcgi?artid=403519&pageindex=1
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