Wednesday, May 28, 2008

What is Loin Pain Hematuria Syndrome?

by Marc F. Brazie, MD
Division of Nephrology
University of Maryland Medical Center, Baltimore, MD

What is Loin Pain Hematuria Syndrome (LPHS)?
LPHS is a rare kidney disorder causing attacks of severe flank pain and blood in the urine.

Who gets LPHS?
LPHS was first reported in three patients in 1967 who were found to have recurrent attacks of severe flank pain and blood in the urine (hematuria) in whom no other explanation could be found [2]. Since that time, it has been reported in several hundred people worldwide, although it is likely that there are many more unreported cases. It is more common in women (70% of all cases) [3-5], and has been seen mostly in the United States, Great Britain, Australia, and Canada, suggesting a white predominance. While it can start at almost any age, most patients will first develop symptoms in their 20’s [6].

How do I know if I have LPHS?
The predominant features of LPHS are recurrent flank pain and blood in the urine. The pain can occur on only one side or both, and may radiate to the abdomen, groin, or inner thigh. Pain episodes may be associated with low-grade fevers and a burning discomfort with urination. The blood in the urine may be overt (called “macroscopic hematuria”), or only detectible on a urine dipstick test or by examining the urine through a microscope (called “microscopic hematuria”). Up to 47% of patients with LPHS also have a history of kidney stones [7], but stones must be absent during pain episodes for LPHS to be diagnosed.

What causes LPHS?
As LPHS is a rare disorder, not much is known about the underlying causes. It is thought that there may be a problem with the glomerular basement membrane (GBM), which is the blood “filter” in the kidney, causing bleeding into the kidney tubules. These tubules can then become blocked by the blood cells, causing swelling and increased pressure in the kidney. This in turn leads to stretching of the outer layer of the kidney, called the “capsule,” and pain. Indeed, studies examining biopsies of patients thought to have LPHS have shown that in over 50% of patients, the GBM is abnormally thick or thin [7].

What should I do if I think I might have LPHS?
There are many disorders that can cause flank pain and blood in the urine, and as there is no specific test to confirm LPHS, it is considered a diagnosis of exclusion. It is very important to have a thorough evaluation by a kidney doctor (nephrologist) to be sure there is no other cause for the symptoms. This evaluation may involve laboratory tests, radiology studies, or even a kidney biopsy.

What can be done for LPHS?
Again, as LPHS is a rare disorder, it is not clear what the optimal treatment is. What is known is that patients with LPHS tend to have normal kidney function, which does not deteriorate over time. Thus, treatment is aimed at pain control and improvement in quality of life. Multiple therapies have been tried, all with mixed results. These include:
• Medications such at angiotensin-converting enzyme inhibitors (a family of blood pressure medicines which reduce the pressure in the kidney and have been shown to be of benefit to some patients [8]), non-steroidal anti-inflammatory drugs (such as ibuprofen, naproxen, or ketorolac), and narcotic pain medications.
• Nerve block procedures to decrease the pain signal from the effected kidney, and
• Surgery to strip the nerves from the kidney, “autotransplant” the kidney (in which it is removed from its normal position and reinserted in the lower abdomen – this has been shown to relieve pain in up to 70% of patient in some reports [3,4]), or in extreme cases, remove the kidney altogether.

It is best to discuss all the treatment options with your doctor, and ideally as part of a “multidisciplinary” approach involving primary care doctors, nephrologists, psychiatrists, and chronic pain specialists.

1. Dube GK, Hamilton SE, Ratner LE, Nasr SH, Radhakrishnan J. Loin pain hematuria syndrome. Kidney Int. 2006; 70: 2152-2155.
2. Little PJ, Sloper JS, deWardener HE. A syndrome of loin pain and haematuria associated with disease of peripheral renal arteries. Q J Med. 1967; 36: 253-259.
3. Sheil AG, Chui AK, Verran DJ et al. Evaluation of the loin pain/hematuria syndrome treated by renal autotransplantation or radical renal neurectomy. Am J Kidney Dis. 1998; 32: 215-220.
4. Chin JL, Kloth D, Paulter SE, Mulligan M. Renal autotransplantation for the loin pain-hematuria syndrome: long-term followup of 26 cases. J Urol. 1998; 160: 1232-1235.
5. Greenwell TJ, Peters JL, Neild GH, Shah PJ. The outcome of renal denervation for managing loin pain haematuria syndrome. BJU Int. 2004; 93: 818-821.
6. Weisberg LS, Bloom PB, Simmons RL, Viner ED. Loin pain hematuria syndrome. Am J Nephrol. 1993; 13: 229-237.
7. Spetie DN, Nadasdy T, Nadasdy G, et al. Proposed pathogenesis of idiopathic loin pain-hematuria syndrome. Am J Kidney Dis. 2006; 47: 419-427.
8. Hebert LA, Betts JA, Sedmak DD, et al. Loin pain-hematuria syndrome associated with thin glomerular basement membrane disease and hemorrhage into renal tubules. Kidney Int. 1996; 49: 168-173.

Our Story

About four months ago, our 20 year old daughter Sarah discovered she had a rare kidney disorder called Loin Pain Hematuria Syndrome (LPHS). This disorder was discovered by
Dr. Kevin Ferentz, Director & Chief of Clinical Operations Associate Professor and
Dr. Marc Brazie, Nephrologist at the University of Maryland-Family Medicine Department. This Department along with the Nephrology and Pain Management Departments have been fantastic in trying to help Sarah. There is no cure, but the many ideas that come from the University of Maryland Hospital – Family Medicine Department will lend a hand in helping Sarah through it all! Take a moment and read an explanation of LPHS and its treatments written by Dr. Marc Brazie. Hopefully, with more testing, trials, and research there will be more to offer the 700 plus patients that endure this painful disorder.

We have been with our daughter through it all and it’s been very, very difficult and painful. As a parent, there is a heartache that you cannot even imagine when you see your child in such pain and there is nothing you can do! But with the help of both the Family Medicine and Nephrology Departments and the support and comfort of our incredible doctors, Dr. Ferentz and Dr. Brazie, we believe Sarah can and will be helped! Just recently, Dr. Thelma Wright joined these two doctors and is also aiding Sarah in providing pain management of this disorder. These doctors outshine any doctors that we have dealt with through this whole experience. Most people don't know of this disorder, most doctors don't either. But both Dr. Ferentz & Dr. Brazie, who discovered this disorder, have excelled at always being by her side when she needed them and going above & beyond to help her through this ordeal. During Sarah’s five day stay and the kidney biopsy she had to endure, they treated her with the respect, understanding, care, and consideration that she so needed.

We have a couple of friends (they know who they are) who over this past couple of weeks and during the most trying of times have shown us what friendship is all about. We are grateful to them for their support of Sarah and our family and treasure their friendship. We are reminded of them by a song by Tracey Lawrence “You find out who your friends are” and it’s so true! Everyone is busy, but our friends, business associates, and clients have taken the time to provide us with comfort. We hug each of you and thank you!

Because we see what they have been doing and are so touched by their dedication, we want to lend a hand in offering the Department other means to help Sarah and others like her with rare kidney disorders/diseases and other health issues. So, we are asking from the bottom of our hearts for your help! We have set up a PayPal account to collect donations. All funds raised will go to the University of Maryland Hospital – Family Medicine and its specialized department. Unfortunately, because we are not non-profit, but parents trying to do what they can to help their child, we are unable to provide a receipt for those of you wanting to use this as a tax deduction. But we can give you our heartfelt thanks and utmost gratitude for helping our daughter and others!!

We would appreciate it if you would help spread the word by sharing this information via email and word of mouth. Our special website and our rolling advertisement will be hitting the streets in a week or so. This will be the first outdoor mobile advertising fundraiser on wheels and we hope it will gain a lot of attention to help our daughter with her rare disorder and Dr. Ferentz at University of Maryland - Family Medicine. He and his department exhibit the compassion, care and consideration that every parent of a sick child needs. They have taken care of our daughter as if she were their own. We so appreciate all that they have done and will continue to do to help Sarah - who wants to become a nurse someday - through this and to gain her life back.

We have been blessed by these doctors and by the many friends, families, and businesses that have offered to help us raise funds! Thank you for the taking time to donate!