Cookies help us deliver our services. By using our services, you agree to our use of cookies. Learn more

Loin Pain Hematuria Syndrome

What is Loin Pain Hematuria Syndrome ?

Loin Pain Hematuria Syndrome is the combination of flank pain and blood in the urine that is otherwise unexplained.

 

Loin Pain Hematuria Syndrome is the combination of flank pain and blood in the urine that is otherwise unexplained.
Acknowledgement of Loin Pain Hematuria Syndrome has not been added yet.
1.2http://www.uptodate.com/contents/loin-pain-hematuria-syndrome
Synonyms for Loin Pain Hematuria Syndrome has not been added yet.
The cause of LPHS is currently not known. One theory proposes that it is caused by a thin glomerular basement membrane and red blood cell (RBC) renal tubular congestion that leads to swelling of the kidney and distension of the renal fascia resulting in pain. Researchers have hypothesized that the syndrome may be due to blood vessel diseases of the kidney, spasms of the kidney vessels, or other bleeding disorders (coagulopathy). The hematuria in LPHS may be due to an abnormal (thick or thin) glomerular basement membrane. The glomerular basement membrane is a tissue in the kidney that filters the blood. An abnormal glomerular basement membrane may allow red blood cells into the urinary space. Because kidney stones are so common in people with LPHS, crystals in the kidney tubules may also play a part in bleeding and pain. Other speculations on cause include: IgA nephropathy. This is a condition in which small amount of a type of normal antibody (called IgA) get stuck in the kidney as it passes through in the bloodstream. This is a chronic condition, which sometimes goes away on its own but occasionally can cause damage to the kidneys. A related condition called IgM nephropathy can sometimes cause pain. Thin membrane disease. In this condition the membrane that filters the blood to make urine is too thin, and blood can pass across it in very small amounts. In a few cases of this condition, there is pain in the kidneys, usually occurring in attacks every so often. Although this condition can be painful, kidney failure does not seem to occur in the long term, so that the only real problem is the symptoms. Infection. In some cases, loin pain-haematuria syndrome occurs after a bladder infection with involvement of the kidney. Even when the infection has been treated and bugs can no longer be found in the urine, pain may persist for 6 months, or even longer in some cases. "Classic loin pain-haematuria syndrome". Some patients have none of the above diagnoses. In these cases there may be minor abnormalities on a kidney biopsy. Angiogram tests to look at the blood vessels in the kidney may show abnormal blood flow, perhaps causing a cramp like pain. The cause is not fully understood. It certainly is [more common] in women than in men, and there may be hormonal influences. Some women find the pain is worse at different times of their menstrual cycle, or comes on during pregnancy, or if they are taking [oral contraceptives]. It has also been reported to be caused by microscopic granules of Calcium Oxylate into the Glomerulus itself, causing blood vessels to rupture and increase the distention of the renal capsule This condition may persist for some years, and can be lifelong. Damage to the kidneys leading to kidney failure does not occur. However, because LPHS is unusual in patients older than 60 years, some clinicians believe that LPHS eventually resolves. At this time no cure has been found for this disease. LPHS is a debilitating disease due to chronic pain and the inability to know how to control the glomerular aspect. The pain of LPHS can be worsened by acts as simple as riding in the car and undertaking daily activities. Many people with this disease are unable to maintain employment due to the debilitating pain.[citation needed]
Loin Pain and Nausea are the most debilitating symptoms.
LPHS is considered a diagnosis of exclusion. The syndrome presents with hematuria (blood in the urine) and flank (a region of the lower back beneath the ribs and above the ilium) pain which can result from a number of causes. Nonglomular bleeding (e.g., urinary infection, tumor, or nephrolithiasis) must be excluded. Obstruction of the urinary tract should not be present, confirmed by at least two imaging procedures while pain is present. Diagnosis of loin pain-hematuria syndrome (LPHS) occurs when hematuria is present, recurrent or persistent pain is severe, and other causes of bleeding are excluded. Urine testing can be performed to detect microscopic levels of hematuria. Protein is also commonly found in the urine of patients with LPHS. Kidney biopsies are sometimes performed to look for evidence of glomerular hematuria, excess red blood cells in the kidney tubules, and to assess the width of the glomerular basement membrane. Hematuria ( more than 5 red blood cells per high power field) should be present in virtually every urinalysis and is typically characterized by dysmorphic red cells. Recurrent or persistent severe pain for six months or more, occurring in the costovertebral angles.
Diagnostic tests of Loin Pain Hematuria Syndrome has not been added yet
The treatment of LPHS varies considerably from centre to centre. As the condition is rare and poorly understood, a widely adopted standard of care is not existent. Treatment of loin pain-hematuria syndrome (LPHS) typically consists of pain management. Narcotics or oral opioids may be prescribed to help control pain. Patients with severe pain may need high-dose opioids daily or almost daily. Occasionally, people with LPHS require hospitalization for intravenous opioid therapy and control of nausea. Other treatments may include denervation, autotransplantation, renal neurectomy, or nephrectomy. Unfortunately symptoms often recur following these procedures. Limited evidence suggests that drugs that inhibit angiotensin may reduce the frequency and severity of episodes of loin pain and gross hematuria. Pain management with opiate and non-opiate analgesia is common. Angiotensin converting enzyme inhibitors are thought to be beneficial, as they reduce intraglomerular pressure and, presumably, reduce renal tubular congestion with RBCs. _Possible treatment regimens:_ *General* Angiotensin inhibition Reduce the risk of nephrolithiasis *Pain Control* Opioid therapy Inpatient therapy Intravenous opioid regimen Antiemetic drugs Pruritus management Maintenance therapy between pain exacerbations *Invasive therapy* Implantable drug delivery system Surgical renal denervation Renal autotransplantation Nephrectomy *Other invasive therapies* Pulse radio frequency Celiac plexus block Intraureteric capsaicin infusion Surgery (autotransplantation or nephrectomy) is thought by some to be of benefit in selected individuals and advocated in some centres, but usually considered the last resort. Physicians discourage surgery, as LPHS symptoms often re-occur after autotransplantation. Another treatment that has been known to help LPHS sufferers with their daily pain is a Spinal Cord Stimulator.
This condition may persist for some years, and can be lifelong. Damage to the kidneys leading to kidney failure does not occur.However, because LPHS is unusual in patients older than 60 years, some clinicians believe that LPHS eventually resolves. At this time no cure has been found for this disease. LPHS is a debilitating disease due to chronic pain and the inability to know how to control the glomerular aspect. The pain of LPHS can be worsened by acts as simple as riding in the car and undertaking daily activities. Many people with this disease are unable to maintain employment due to the debilitating pain.
Tips or Suggestions of Loin Pain Hematuria Syndrome has not been added yet.
References of Loin Pain Hematuria Syndrome has not been added yet.
Lphs Created by Donna56
Last updated 10 Apr 2014, 02:45 PM

Posted by Donna56
10 Apr 2014, 02:45 PM

*does anyone on here have any information about LPHS, anything would be helpful,I live in Louisiana and can't find a doctor to help,please anyone could you help me

Community Resources
Title Description Date Link

Clinical Trials


Cords registry

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.

Enrolling is easy.

  1. Complete the screening form.
  2. Review the informed consent.
  3. Answer the permission and data sharing questions.

After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.

Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.

Visit sanfordresearch.org/CoRDS to enroll.

Community Leaders

 

Expert Questions

Ask a question

Community User List

I'm a 47 yr. old woman with LPHS as a pending disorder. I've had hematuria, and severe flank pain for six yrs. without alternate medical diagnosis. I've been through many doctors, and have had many...
I was diagnosed with LPHS when I was 17 years old, just a few weeks shy of my 18th birthday (2010). I've had symptoms and I've searched for answers since I was 11 years old (2003). I also have...
I am the mother of a 23 year old son that had LPHS and we need help and information
I suffer from Loin Pain Hematuria Syndrome.
I am a fibromyalgia sufferer, but that's hardly rare. However, my sweet middle daughter has Loin Pain Hematuria Syndrome, complicated by acute kidney disease, kidney stone disease, lupus, and...
Married and a Mom of a child with LPHS - Loin Pain Hematuria Syndrome.

 

 

It is a rare and presently incurable disorder effecting the kidneys with flank pain and blood in urine and...

Start a Community


Don't See Your Condition On Rareshare?

Start your own! With a worldwide network of 8,000 users, you won't be the only member of your community for long.

FAQ


Have questions about rareshare?

Visit our Frequently Asked Questions page to find the answers to some of the most commonly asked questions.

Discussion Forum

Lphs

Created by Donna56 | Last updated 10 Apr 2014, 02:45 PM


Communities

Our Communities

Join Rareshare to meet other people that have been touched by rare diseases. Learn, engage, and grow with our communities.

FIND YOUR COMMUNITY
Physicians

Our Resources

Our rare disease resources include e-books and podcasts

VIEW OUR EBOOKS

LISTEN TO OUR PODCASTS

VIEW OUR GUIDES

Leaders

Our Community Leaders

Community leaders are active users that have been touched by the rare disease that they are a part of. Not only are they there to help facilitate conversations and provide new information that is relevant for the group, but they are there for you and to let you know you have a support system on Rareshare.