Renal tubular acidosis in Sjögren's syndrome: a case series.

BACKGROUND: The exact frequency of distal and proximal renal tubular acidosis (RTA) in Sjögren's syndrome is unknown. Other features of Sjögren's syndrome like polyuria, glomerular manifestations, familial occurrence and pregnancy are not widely reported. The aim was to prospectively study the clinical features and outcome of distal and proximal RTA in Sjögren's syndrome and also report on other renal manifestations of Sjögren's syndrome. METHODS: The present study is a prospective consecutive case series of patients who presented with a history suggestive of RTA and Sjögren's syndrome. All patients were followed for 1 year. The diagnosis of RTA was by fractional excretion of bicarbonate. The diagnosis of Sjögren's syndrome was according to the American-European classification system [modified by Tzioufas and Voulgarelis: Best Pract Res Clin Rheumatol 2007;21:989-1010]. RESULTS: The total number of RTA patients diagnosed during this period was 149. Sjögren's syndrome accounted for 34.8% (52 of 149) of RTA patients. The important symptoms and laboratory parameters were oral and ocular symptoms in 23 (44.2%), dental caries in 12 (23%), body pains in 47 (90.3%), mean serum pH 7.202 ± 0.03, mean serum bicarbonate, 14.03 ± 1.66 mmol/l, and mean urine pH, 7.125 ± 0.54. There were 30 (57.6%) patients with distal RTA and 22 (42.3%) patients with proximal RTA. CONCLUSIONS: The clinical implication of the present study is that RTA is a common feature of Sjögren's syndrome. It may be missed if the presentation is not due to oral and ocular symptoms. The present study is also the only one with a 1-year follow-up.

"True" mycotic aneurysm of the anastomotic site of the renal allograft artery.

The incidence of vascular complications after renal transplant as reported varies from 3.5% to 14%. Pseudoaneurysm formation at the site of the anastomosis is a rare complication, and only a few cases have been reported. There also were only a few reports of "true" mycotic aneurysms of the renal allograft artery. We present 2 patients with true mycotic aneurysmal formation of the renal allograft artery after a renal transplant. Both patients presented with fever and increasing serum creatinine levels. Cultures from aneurysm tissue samples have grown Aspergillus flavus. Both patients were subjected to an allograft nephrectomy, and amphotericin was given.

Fanconi syndrome following honeybee stings.

A 32-year-old gentleman was attacked by honey bees about 8 months and immediately afterwards his eyelids, cheeks and pinnae became swollen, red, and tender. However, the patient did not develop any renal or serum sickness symptoms and his physical examination and laboratory investigations were normal. He recovered completely. A week later, while working on his farm, he experienced a sudden loss of muscular tone in all four limbs without losing his consciousness. The medical examination subsequently revealed flaccid quadriparesis associated with a serum potassium of 2.1 mEq/L. He was also found to have hyperchloremic metabolic acidosis with normal anion gap and preserved ability to acidify urine to a pH of 5.5. These findings were suggestive of proximal renal tubular acidosis (Fanconi syndrome). Other abnormalities like hypophosphataemia, hypouricemia, renal glucosuria and high urinary excretion of calcium, phosphorus and uric acid further supported the diagnosis of proximal tubular dysfunction. The renal biopsy revealed dense lymphocytic interstitial infiltrate, a feature often seen in Sjogren's syndrome, in which at least 50% of patients fail to acidify urine. In our patient, thorough search for other causes of proximal renal tubular acidosis was negative.

End-stage renal disease patients on hemodialysis: a study from a tertiary care center in a developing country.

The exact number of patients with chronic renal failure requiring renal replacement therapy in developing world is not known. Unlike the developed world, most developing countries lack renal registries. This study was initiated to know demographic and clinical data of end-stage renal disease (ESRD) patients presenting to maintenance hemodialysis (MHD) at a government funded tertiary care centre in a developing country. A prospective analysis of all new ESRD patients attending to hemodialysis at our centre from 2004 to 2007 had been done. There were 237 new hemodialysis patients during a three-year period. Males were 153 and females were 84, with the mean age 44.92 years. Diabetes mellitus (31.22%) was the most common cause of ESRD. Only 29.95% of patients had education on renal replacement therapy. 65.40% patients had emergency hemodialysis. Internal jugular catheter was the most common form of vascular access at initiation of hemodialysis. Arteriovenous fistula was secured in 29.95% of patients at presentation. Catheter-related infection appeared in 13.55% of patients on catheter. The most common infection in dialysis patients was urinary tract infection (37.14%). Renal transplantation was opted by 9.7% patients and continuous ambulatory peritoneal dialysis in 20.25% and 103 (43.45%) were lost to follow up. The rest (8.86%) continued on MHD. There were 42 (17.72%) deaths over a three-year period. The present study provided the information of the practice of hemodialysis, its population characteristics and outcomes from a developing country.

Kikuchi-Fujimoto disease associated with systemic lupus erythematosus.

A cervical lymph node biopsy from a 38-year-old woman initially revealed necrotising lymphadenitis. Her case is presented herein. An exhaustive examination that included renal biopsy did not suggest systemic lupus erythematosus (SLE). She was diagnosed with Kikuchi-Fujimoto Disease (KFD) and was treated with prednisone. One year later, a renal biopsy performed for renal failure revealed Class IV SLE. It was proposed that lymphadenitis in this KFD patient should be considered as SLE so that the SLE would be properly treated. In our patient, this hypothesis was partially correct, because even though SLE could not be verified at initial presentation, it evolved into full SLE after a year interval.

Right atrial thrombus due to internal jugular vein catheter.

Right atrial thrombus is rare complication of hemodialysis central venous catheter. Literature survey revealed 49 documentations of right atrial thrombus due to a central venous catheter. We report a 58-year-old type 2 diabetic, hypertensive, end-stage renal disease patient, who 2 months after initiation of hemodialysis through a right internal jugular vein catheter, developed clinical features suggestive of pulmonary thromboembolism. An echocardiography revealed presence of a serpentine thrombus in right atrium. The internal jugular vein catheter was removed and unfractionated heparin was initiated. At the end of 6 weeks he was symptom free. We compared conservative treatment with surgery for RAT. Conservative management with central venous catheter removal and anticoagulation therapy is not inferior to the surgery.