Loin pain hematuria syndrome
Encyclopedia
Loin pain hematuria syndrome, also LPHS, is the combination of unilateral or bilateral flank pain
and microscopic
or macroscopic
amounts of blood
in the urine
that is otherwise unexplained.
. 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.
, as in thin basement membrane disease
, is proposed to be the characteristic finding on renal biopsy
, but not part of the syndrome definition.
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
.
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 .
is not existent.
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 RBC
s.
) is thought by some to be of benefit in selected individuals and advocated in some centres, but usually considered the last resort.
Many physicians discourage surgery
, as LPHS symptom
s often re-occur after autotransplantation.
Pain
Pain is an unpleasant sensation often caused by intense or damaging stimuli such as stubbing a toe, burning a finger, putting iodine on a cut, and bumping the "funny bone."...
and microscopic
Microscopic
The microscopic scale is the scale of size or length used to describe objects smaller than those that can easily be seen by the naked eye and which require a lens or microscope to see them clearly.-History:...
or macroscopic
Macroscopic
The macroscopic scale is the length scale on which objects or processes are of a size which is measurable and observable by the naked eye.When applied to phenomena and abstract objects, the macroscopic scale describes existence in the world as we perceive it, often in contrast to experiences or...
amounts of blood
Blood
Blood is a specialized bodily fluid in animals that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from those same cells....
in the urine
Urine
Urine is a typically sterile liquid by-product of the body that is secreted by the kidneys through a process called urination and excreted through the urethra. Cellular metabolism generates numerous by-products, many rich in nitrogen, that require elimination from the bloodstream...
that is otherwise unexplained.
Diagnosis
LPHS is considered a diagnosis of exclusionDiagnosis of exclusion
A diagnosis of exclusion is a medical condition reached by a process of elimination, which may be necessary if presence cannot be established with complete confidence from examination or testing...
. The syndrome presents with hematuria
Hematuria
In medicine, hematuria, or haematuria, is the presence of red blood cells in the urine. It may be idiopathic and/or benign, or it can be a sign that there is a kidney stone or a tumor in the urinary tract , ranging from trivial to lethal...
(blood in the urine) and flank (a region of the lower back beneath the ribs and above the ilium
Ilium (bone)
The ilium is the uppermost and largest bone of the pelvis, and appears in most vertebrates including mammals and birds, but not bony fish. All reptiles have an ilium except snakes, although some snake species have a tiny bone which is considered to be an ilium.The name comes from the Latin ,...
) pain which can result from a number of causes.
Relation to chronic pelvic pain
LPHS has considerable overlap with chronic pelvic pain syndrome.Relation to thin basement membrane disease
A thin glomerular basement membraneGlomerular basement membrane
The glomerular basement membrane is the basal laminal portion of the glomerulus. The Glomerular capillary endothelial cells, the GBM and the filtration slits between the podocytes perform the filtration function of the glomerulus, separating the blood in the capillaries from the filtrate that...
, as in thin basement membrane disease
Thin basement membrane disease
Thin basement membrane disease is, along with IgA nephropathy, the most common cause of asymptomatic hematuria. The only abnormal finding in this disease is a thinning of the basement membrane of the glomeruli in the kidneys...
, is proposed to be the characteristic finding on renal biopsy
Biopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
, but not part of the syndrome definition.
Prevalence
The condition is very rare and predominately affects females. Worldwide, only several hundred cases have been reported.Etiology
The cause of LPHS is currently not known. One theoryTheory
The English word theory was derived from a technical term in Ancient Greek philosophy. The word theoria, , meant "a looking at, viewing, beholding", and referring to contemplation or speculation, as opposed to action...
proposes that it is caused by a thin glomerular
Glomerulus
A glomerulus is a capillary tuft that is involved in the first step of filtering blood to form urine.A glomerulus is surrounded by Bowman's capsule, the beginning component of nephrons in the vertebrate kidney. A glomerulus receives its blood supply from an afferent arteriole of the renal...
basement membrane
Basement membrane
The basement membrane is a thin sheet of fibers that underlies the epithelium, which lines the cavities and surfaces of organs including skin, or the endothelium, which lines the interior surface of blood vessels.- Composition :...
and red blood cell
Red blood cell
Red blood cells are the most common type of blood cell and the vertebrate organism's principal means of delivering oxygen to the body tissues via the blood flow through the circulatory system...
(RBC) renal tubular congestion that leads to swelling of the kidney
Kidney
The kidneys, organs with several functions, serve essential regulatory roles in most animals, including vertebrates and some invertebrates. They are essential in the urinary system and also serve homeostatic functions such as the regulation of electrolytes, maintenance of acid–base balance, and...
and distension of the renal fascia
Renal fascia
The renal fascia is a layer of connective tissue encapsulating the kidneys. The deeper layers below the renal fascia are, in order, the perinephric fat , renal capsule, and finally the parenchyma of the renal cortex...
resulting in pain
Pain
Pain is an unpleasant sensation often caused by intense or damaging stimuli such as stubbing a toe, burning a finger, putting iodine on a cut, and bumping the "funny bone."...
.
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
Glomerulus
A glomerulus is a capillary tuft that is involved in the first step of filtering blood to form urine.A glomerulus is surrounded by Bowman's capsule, the beginning component of nephrons in the vertebrate kidney. A glomerulus receives its blood supply from an afferent arteriole of the renal...
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 .
Treatment
The treatment of LPHS varies considerably from centre to centre. As the condition is rare and poorly understood, a widely adopted standard of careStandard of care
In tort law, the standard of care is the degree of prudence and caution required of an individual who is under a duty of care.The requirements of the standard are closely dependent on circumstances. Whether the standard of care has been breached is determined by the trier of fact, and is usually...
is not existent.
Conservative
Pain managementPain management
Pain management is a branch of medicine employing an interdisciplinary approach for easing the suffering and improving the quality of life of those living with pain. The typical pain management team includes medical practitioners, clinical psychologists, physiotherapists, occupational therapists,...
with opiate
Opiate
In medicine, the term opiate describes any of the narcotic opioid alkaloids found as natural products in the opium poppy plant.-Overview:Opiates are so named because they are constituents or derivatives of constituents found in opium, which is processed from the latex sap of the opium poppy,...
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 RBC
Red blood cell
Red blood cells are the most common type of blood cell and the vertebrate organism's principal means of delivering oxygen to the body tissues via the blood flow through the circulatory system...
s.
Surgical
Surgery (autotransplantationAutotransplantation
Autotransplantation is the transplantation of organs, tissues or even proteins from one part of the body to another in the same individual. Tissue transplanted by such "autologous" procedure is referred to as an autograft or autotransplant. It is contrasted with xenotransplantation and...
) is thought by some to be of benefit in selected individuals and advocated in some centres, but usually considered the last resort.
Many physicians discourage surgery
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
, as LPHS symptom
Symptom
A symptom is a departure from normal function or feeling which is noticed by a patient, indicating the presence of disease or abnormality...
s often re-occur after autotransplantation.
Differential diagnosis
- Kidney stones
- Nutcracker syndromeNutcracker syndromeIn medicine, the nutcracker syndrome — is a clinically manifest variant of nutcracker phenomenon, renal vein entrapment syndrome, or mesoaortic compression of the left renal vein. It results most commonly from the compression of the left renal vein between the abdominal aorta and superior...
- IgA nephropathyIgA nephropathyIgA nephropathy is a form of glomerulonephritis...
- CancerCancerCancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
of the genitourinary tract - classically not associated with pain - Chronic pelvic pain
- EndometriosisEndometriosisEndometriosis is a gynecological medical condition in which cells from the lining of the uterus appear and flourish outside the uterine cavity, most commonly on the ovaries. The uterine cavity is lined by endometrial cells, which are under the influence of female hormones...
External links
- Loin Pain - Patient UKPatient UKPatient UK or Patient.co.uk is an online resource providing information on health, lifestyle, disease and other medical related topics. The website's aim is to provide members of the public with up-to-date information on health related topics in the form of comprehensive leaflets which can be read...
- Loin pain hematuria syndrome - UpToDate.com.
- Loin pain hematuria syndrome statistics - geocities.com.
- LPHS talk and support - lifeinpain.com.