Loin pain hematuria syndrome

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Loin pain hematuria syndrome
Classification and external resources
Gray1125.png
Sagittal section of the kidney and its capsule. Pain in LPHS is thought to result from distension of the kidney capsule.

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.[1]

Diagnosis[edit]

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.

Relation to chronic pelvic pain[edit]

LPHS has considerable overlap with chronic pelvic pain syndrome.

Relation to thin basement membrane disease[edit]

A thin glomerular basement membrane, as in thin basement membrane disease, is proposed to be the characteristic finding on renal biopsy,[2] but not part of the syndrome definition.

Prevalence[edit]

The condition is very rare and predominately affects females. Worldwide, less than two hundred one cases have been reported.

Etiology[edit]

The cause of LPHS is currently not known.[1] 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.[2] 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.[citation needed] Many people with this disease are unable to maintain employment due to the debilitating pain.[citation needed]

Treatment[edit]

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.

Conservative[edit]

Pain management with opiate and non-opiate analgesia is common. Angiotensin converting enzyme inhibitors are thought to be beneficial,[2] as they reduce intraglomerular pressure and, presumably, reduce renal tubular congestion with RBCs.

Surgical[edit]

Surgery (autotransplantation) is thought by some to be of benefit in selected individuals[3] and advocated in some centres, but usually considered the last resort.[4]

Many physicians discourage surgery, as LPHS symptoms often re-occur after autotransplantation.[4]

Differential diagnosis[edit]

See also[edit]

References[edit]

  1. ^ a b Pukenas BA, Zaslau S (2003). "Loin pain hematuria syndrome: case series". W V Med J 99 (5): 192–3. PMID 14959511. 
  2. ^ a b c Hebert LA, Betts JA, Sedmak DD, Cosio FG, Bay WH, Carlton S (1996). "Loin pain-hematuria syndrome associated with thin glomerular basement membrane disease and hemorrhage into renal tubules". Kidney Int. 49 (1): 168–73. doi:10.1038/ki.1996.23. PMID 8770964. 
  3. ^ Chin JL, Kloth D, Pautler SE, Mulligan M (1998). "Renal autotransplantation for the loin pain-hematuria syndrome: long-term followup of 26 cases". J. Urol. 160 (4): 1232–5; discussion 1235–6. doi:10.1016/S0022-5347(01)62503-5. PMID 9751325. 
  4. ^ a b Parnham AP, Low A, Finch P, Perlman D, Thomas MA (1996). "Recurrent graft pain following renal autotransplantation for loin pain haematuria syndrome". Br J Urol 78 (1): 25–8. doi:10.1046/j.1464-410x.1996.00455.x. PMID 8795395. 

External links[edit]