Loin Pain Hematuria Syndrome
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Loin Pain Hematuria Syndrome
- 1. Outcomes of Laparoscopic Nephrectomy with Renal Autotransplantation for The Loin Pain- Hematuria Syndrome Wisit Cheungpasitporn May 2016
- 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
- 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
Editor's Notes
- When renal biopsy is performed, some patients have a known cause of glomerular hematuria such as IgA nephropathy or vasculitis [2,8]. Such patients are considered to have secondary LPHS, while patients without signs of acquired underlying glomerular disease are considered to have primary LPHS.
- Renal biopsy in patients with primary LPHS shows evidence of glomerular hematuria, as manifested by red cells in the tubules. This finding is seen significantly more often in patients with LPHS than in normal kidneys from transplant donors (7.2 versus 1.6 percent of tubular cross-sections with red cells in a blinded assessment in our series of 34 patients), thereby precluding trauma from the biopsy as the cause [2].
- Somatoform disorder”- physical symptoms that mimic physical disease or injury for which there is no identifiable organic cause