Loin Pain Hematuria Syndrome

For education purpose only

1. Outcomes of Laparoscopic Nephrectomy with
Renal Autotransplantation for The Loin Pain-
Hematuria Syndrome
Wisit Cheungpasitporn
May 2016

2. Disclosure
•None

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

4. 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)

5. 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.

6. 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
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.

7. 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
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.

8. 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
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.

9. Diagnosis
•Diagnosis of exclusion

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

11. 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.

12. Kidney Biopsy- LPHS
Spetie DN et al.. Am J Kidney Dis 47(3):419-427, 2006.
7.2% vs 1.6%; P< 0.0001

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

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

15. 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
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.

16. 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):168
Taba Taba Vakili S et. al. Am J Kidney Dis. 2014;64(3):460-72.

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

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

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

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

21. LAPAROSCOPIC RETROPERITONEAL
LIVE DONOR RIGHT NEPHRECTOMY
Gill IS. J Urol. 2000 Nov;164(5):1500-4.

22. 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.

23. 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).

24. 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

25. http://onlinelibrary.wiley.com/doi/10.1111/jebm.12199/abstract
PMID: 27186938

26. 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

27. 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

28. Questions & Discussion