Cholesterol Emboli Syndrome in Type 2 Diabetes: The Disease History of a Case Evaluated with Renal Scintigraphy

BACKGROUND: Cholesterol crystal emboli syndrome (CCE) is an emerging disease, whose progression reflects the currently observed increase in cardiovascular diseases. Diagnostic criteria shifted from pathological to clinical criteria: creatinine increase, skin lesions, recent endovascular interventions and severe vasculopathy). Diabetes, hypertension and diffuse vascular disease are inter-linked, major risk factors. The role of imaging techniques in the diagnosis and treatment of the disease has been little investigated thus far. The AIM of this report is to describe a case exemplifying the potentials for renal scintigraphy in CCE, an emerging disease in type 2 diabetic patients. THE CASE: a 75 year-old, type 2 diabetic for over 15 years, obese, hypertensive white man was referred to the Nephrology Unit after an acute coronary syndrome. Stenosis of the left renal artery was diagnosed from the angiography. Serum creatinine (baseline: 1.9 mg/dl) increased after multiple angioplasties to 3.3 mg/dl, then slowly returned towards baseline (2.2 mg/dl), but rose, on referral, to 3.9 mg/dl, with an increase in acute phase reactants and peripheral livedo reticularis, a picture highly suggestive of CCE. The first renal scintiscan showed a reduction of the parenchymal phase, and a non-homogeneous parenchymal pattern in the right dominant kidney. The patient was started on corticosteroid therapy with a prompt decrease in creatinine; four days later (creatinine 2.5 mg/dl) a second scintiscan showed an improvement of the peak time and of the radionuclide parenchymal transit, and was further confirmed two months later (creatinine 2.2 mg/dl). No modification was detected in the left kidney, presumably mechanically "protected" from the cholesterol shedding by the stenosis. CONCLUSIONS: This is the first description of an imaging demonstration of the morpho-functional substratum to the rapid clinical response of corticosteroid therapy in a case of CCE and type 2 diabetes, underlining the potential of 99mTc-MAG3 dynamic scintiscan in this disease

Tailored Immunosuppression and Steroid Withdrawal in Pancreas-Kidney Transplantation

BACKGROUND: Recent improvements in simultaneous pancreas-kidney transplantation (SPK) and the striking decrease in acute rejection lead us to focus on the effects of long-term immunosuppression. AIM OF THIS STUDY: Evaluation of a policy of steroid withdrawal and tailored immunosuppression in pancreas-kidney patients treated in a single center. METHODS: review of the clinical charts in 9 SPK recipients (male/female = 5/4, median age 41 years, median follow-up 42 months), by the same operator, under supervision of the two usual caregivers. Therapeutic protocols. Induction phase: all patients received mycophenolate mophetil (starting dose: 2 grams), tacrolimus and steroids, 8 received Simulect, 1 received thymoglobulins. Maintenance therapy was slowly reduced, with the goal of steroid withdrawal. RESULTS: The therapeutic adjustments were mainly determined by two almost opposing elements: 1. Rapid adjustments in the case of side-effects (gastrointestinal problems, infections and neoplasia); 2. Slow tapering off in the case of good organ function. On the other hand, a switch to cyclosporine A and to rapamycine was considered in the case of chronic organ malfunction. By these means, over a median of 42 months follow-up, steroid withdrawal was slowly obtained in 6/9 patients (at a median time of 25 months). CONCLUSIONS: Within the limits of this small-scale study, a tailored immunosuppressive policy allows at least some "positively selected" patients to reach the "dream" of steroid withdrawal after SPK

Ankylosing spondylitis: a difficult diagnosis in patients on long-term renal replacement therapy

We report the case of a 48-year-old male, whose musculoskeletal manifestations, previously related to long-term renal replacement therapy (RRT), were diagnosed as ankylosing spondylitis when symptoms changed their pattern on daily hemodialysis (DHD). The patient started RRT in 1981; in 1985 he received a cadaver graft, which failed in 1987. Secondary hyperparathyroidism, amyloid geoids, bilateral carpal tunnel syndrome and high aluminium levels were present. Musculoskeletal pain, reported since 1986, involved feet, heels, hips, shoulders, hands, spine. Symptoms impairing daily life did not improve after parathyroidectomy. He developed chronic hypotension and recurrent atrial fibrillation. In 1994 and 1998, because of thoracic pain, coronarography was performed (normal on both occasions). In June 2000, DHD was started. Equivalent renal clearance increased from 9-12 to 15-17 mL/min. Well-being remarkably improved. In September 2000, musculoskeletal pain worsened and bilateral Achilles tendinitis occurred. The worsening of musculoskeletal symptoms despite the improvements in well-being and other dialysis related symptoms prompted a re-evaluation of the case. The diagnosis of ankylosing spondylitis was based on: history of plantar fasciitis, bilateral Achilles tendinitis, inflammatory spinal pain with limitation of lumbar spine mobility (positive Schober test), radiological evidence of grade 2 bilateral sacroiliitis, presence of HLA-B27. This diagnosis cast light on the episodes of chest pain, explained by enthesopathy at the costosternal and manubriosternal joints and atrial fibrillation, due to HLA-B27 associated impairment in heart conduction. This case exemplifies the difficulty of differential diagnosis of multisystem illness in patients with long RRT follow-up

Low-Protein Vegetarian Diet with Alpha-Chetoanalogues Prior to Pre-emptive Pancreas-Kidney Transplantation

BACKGROUND: Pre-emptive pancreas-kidney transplanttation is increasingly considered the best therapy for irreversible chronic kidney disease (CKD) in type 1 diabetics. However, the best approach in the wait for transplantation has not yet been defined. AIM: To evaluate our experience with a low-protein (0.6 g/kg/day) vegetarian diet supplemented with alpha-chetoanalogues in type 1 diabetic patients in the wait for pancreas-kidney transplantation. METHODS: Prospective study. Information on the progression of renal disease, compliance, metabolic control, reasons for choice and for drop-out were recorded prospectively; the data for the subset of patients who underwent the diet while awaiting a pancreas-kidney graft are analysed in this report. RESULTS: From November 1998 to April 2004, 9 type 1 diabetic patients, wait-listed or performing tests for wait-listing for pancreas-kidney transplantation, started the diet. All of them were followed by nephrologists and diabetologists, in the context of integrated care. There were 4 males and 5 females; median age 38 years (range 27.9-45.5); median diabetes duration 23.8 years (range 16.6-33.1), 8/9 with widespread organ damage; median creatinine at the start of the diet: 3.2 mg/dl (1.2-7.2); 4 patients followed the diet to transplantation, 2 are presently on the diet, 2 dropped out and started dialysis after a few months, 1 started dialysis (rescue treatment). The nutritional status remained stable, glycemia control improved in 4 patients in the short term and in 2 in the long term, no hyperkalemia, acidosis or other relevant side effect was recorded. Proteinuria decreased in 5 cases, in 3 from the nephrotic range. Albumin levels remained stable; the progression rate was a loss of 0.47 ml/min of creatinine clearance per month (ranging from an increase of 0.06 to a decrease of 2.4 ml/min) during the diet period (estimated by the Cockroft-Gault formula). CONCLUSIONS: Low-protein supplemented vegetarian diets may be a useful tool to slow CKD progression whilst awaiting pancreas-kidney transplantation