loin pain hematuria syndrome

Outcomes of Laparoscopic Nephrectomy with Renal Autotransplantation for The Loin Pain-Hematuria Syndrome

Wisit Cheungpasitporn

May 2016

Disclosure• None

Burke JR. Pediatr Nephrol. 1996 Apr;10(2):216-20

Causes of Loin Pain- Obstructing nephrolithiasis

- Autosomal dominant polycystic kidney disease

- Renal cell carcinoma

- Recurrent renal thromboembolism, as with atrial fibrillation

Less common causes include:

- Endometriosis

- Left renal vein entrapment (nutcracker syndrome)

EPIDEMIOLOGY• < 500 cases reported to date

• An extremely rare disease (prevalence ~0.012%)

• First report 3 women in 1967

• 70% were women

• Almost all Caucasian

• Common in late 20s (range 10 - 60 years)

Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.

Signs &Symptoms in LPHS

• Location: unilateral or bilateral (less often) flank

• Radiation: abdomen, medial thigh or groin

• Characteristic: constant severe stabbing or dull aching pain

• Duration: hours

• Aggravating factor: gentle punch, exercise

• Associated findings: • Intermittent microscopic or gross hematuria• Nausea and vomitting• low-grade fever and dysuria (rare)• UTI is not present


Hematuria in LPHS• Dysmorphic RBCs

• Microscopic, but most patients experienced at least 1 episode of gross hematuria

• Gross hematuria - almost always accompanied by worsening pain, usually last a few days, but gross hematuria and pain can persist for weeks to months

• Between episodes of gross hematuria, UA typically shows microscopic hematuria; however, sometimes the hematuria clears up but the pain persists


Kidney function in LPHS• Mean serum creatinine 0.9 mg/dL• Protein excretion

• > 150 mg/d in 32%• > 500 mg/d in 6% • Maximum 1,635 mg/d


Diagnosis• Diagnosis of exclusion

Weisberg LS. Am J Nephrol. 1993;13(4):229-37.

Investigation: LPHS

Kidney Biopsy• Warranted if underlying acquired glomerular disease is

suspected.

• Renal biopsy in patients with primary LPHS • LM or IF

• normal glomeruli• RBC or RBC casts are present in the tubules.

• EM• thin or thick glomerular basement membranes

are noted in approximately 60 percent of specimens.

Kidney Biopsy- LPHS

Spetie DN et al.. Am J Kidney Dis 47(3):419-427, 2006.

7.2% vs 1.6%; P< 0.0001

PATHOPHYSIOLOGY- LPHS


Spetie DN et al.. Am J Kidney Dis 47(3):419-427, 2006.

PSYCHOLOGICAL MANIFESTATIONS• Psychogenic in nature

• Somatoform pain disorder • Drug-seeking behavior

• LPHS is a medical disorder and the psychiatric symptoms are a result of the accompanying pain rather than its cause

• A psychiatric evaluation is required to identify psychological issues potentially associated with pain to avoid improper surgical intervention


Treatment• ACE inhibitors/ARBs:

• Reduce frequency/severity of hematuria and pain by reducing glomerular hydrostatic pressure

• Case series:• 4 of 7 patients with LPHS treated with enalapril for 7-48

months had fewer/less severe episodes

• A metabolic stone workup • Chronic and acute pain control:

• Non-opioid therapy should be attempted first

Hebert LA. Kidney Int. 1996;49(1):168Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.

Blacklock AR. Br J Urol. 1989 Aug;64(2):203-4.

Surgical Renal Denervation

Sheil AG. Am J Kidney Dis. 1998 Aug;32(2):215-20.

LAPAROSCOPIC RETROPERITONEAL LIVE DONOR RIGHT NEPHRECTOMY

Gill IS. J Urol. 2000 Nov;164(5):1500-4.

LAPAROSCOPIC RETROPERITONEAL LIVE DONOR RIGHT NEPHRECTOMY

Gill IS. J Urol. 2000 Nov;164(5):1500-4.

• Gill et al. was the first to report the use of laparoscopic nephrectomy for kidney autotransplantation in 2 patients with LPHS, with both experiencing successful surgical outcomes.

Eisenberg ML. J Urol. 2008 Jan;179(1):240-3.

A total of 15 patients underwent autotransplantation for complex ureteral stricture disease and 4 underwent it for renal tumors. Median follow-up was 29 months (range up to 64).

Clinical and Safety Outcomes of Laparoscopic Nephrectomy with Renal Autotransplantation for The Loin Pain-Hematuria Syndrome: A 14-year Longitudinal Study

Adeel S. Zubair, BS, Wisit Cheungpasitporn, MD,

Stephen B Erickson, MD, Mikel Prieto, MD

http://onlinelibrary.wiley.com/doi/10.1111/jebm.12199/abstractPMID: 27186938

http://onlinelibrary.wiley.com/doi/10.1111/jebm.12199/abstract

Conclusion• Pain control and Quality of life is better after

renal autotransplantation by laparoscopic hand-assisted nephrectomy

• Despite postoperative risk, renal autotransplant seems to be safe for survival and renal outcomes

Kidney Autotransplantation• Selection criteria should include

• patients with severe pain requiring high doses of analgesics to control their pain

• those for whom extensive nonsurgical therapies have been unsuccessful

Questions & Discussion

loin pain hematuria syndrome

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