Calciphylaxis in dialysis patients, a severe disease poorly responding to therapies: report of 4 cases.

Calciphylaxis or calciphic uremic arteriolopathy (CUA) is a rare syndrome characterized by the deposition of calcium within the walls of small and medium size vessels in the dermis and in the subcutaneous tissue. The disease mainly affects patients with end-stage renal disease. We report here our experience with 4 cases of calciphylaxis in dialysis patients. The main predisposing factor observed in our 4 patients was warfarin use (2 patients, 50%), while local traumas and diabetes were respectively present in only one patient. None of our patients was obese. Lower legs were the most frequently involved site of CUA (3/4 patients, 75%). In our experience biopsy was crucial to achieve a correct diagnosis and did not cause aggravation of the ulcers. Therapeutic approach was multimodal: mainly hyperbaric oxygen therapy, cinacalcet and sodium thiosulphate. Although many recent case reports have shown exceptional results and healing with the use of sodium thiosulphate, we did not experience any change in the poor prognosis of our patients with the use of this drug, at a dosage of 5 g thrice weekly endovenously.

[Description of hospital discharges in patients with systemic lupus erythematosus]. / Descripción de las altas hospitalarias en pacientes con lupus eritematoso sistémico.

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease that mainly affects young women and whose mortality is increasing for this age group. OBJECTIVES: We used the national registry of Hospital discharges in Spain based on the study of the Minimum Basic Data Set (MBDS) to analyze hospital discharges of patients whose diagnosis included that of LES. MATERIAL AND METHODS: A cross-sectional, descriptive study was performed of all episodes coded as having LES using ICD-9-MC coding system of the patients hospitalized within the period 2005-2008. RESULTS: A total of 5,464 episodes were identified, 1,855 (33%) as main diagnosis and 3,609 (66%) as secondary diagnosis. Patients having LES the main diagnosis were younger (41.56 ± 17.55 vs 56.07 ± 19.01 years; P < .001), had fewer elective admittances (62.5 vs 84.8%; P<.001), lower comorbidity as measured by the Charlson's index (Charlson>2; 18 vs 35%; P<.001) and lower mortality (1.1 vs 5.4%; P < .001). CONCLUSIONS: Patients admitted to internal medicine departments in Spain with a diagnosis of LES accounts for 0.3% of the total. Two different groups of patients are identified. The first group was younger, had lower comorbidity and were in the early phases of diagnosis and/or treatment. The second group was more numerous, older, with a higher comorbidity, with admittances frequently related to infections or cardiovascular complications and higher mortality rate.

[MEN type 1 and chronic renal failure: a rarely reported association]. / MEN tipo 1 ed insufficienza renale: una associazione poco conosciuta.

BACKGROUND: Multiple endocrine neoplasia type 1 (MEN 1), or Wermer's syndrome, is a rare autosomal dominant genetic syndrome characterized by tumors or hyperplasia involving the pituitary, parathyroid, and pancreatic islet cells. Association between MEN 1 and nephrocalcinosis is well known, though data published in medical literature regarding Wermer's syndrome and chronic renal failure relation are still rare. CASE: A 70-year-old Caucasian female patient had a history of primitive hyperparathyroidism, prolactinoma, glucagonoma, adrenal adenoma and pulmonary neuroendocrine neoplasia. She presented at our clinic first when she was 62 years old because of hypertension, nephrolithiasis (calcium oxalate) and diabetes mellitus treated with oral agents. During the eight-year follow-up she developed chronic renal failure (serum creatinine 1.8 mg/dL, glomerular clearance 35 mL/min) and partial thrombosis of abdominal aortic wall. CONCLUSIONS: Although the association between renal failure and MEN 1 is rarely reported, patients affected by Wermer's syndrome have several risk factors of decreasing renal function such as hypertension, nephrolithiasis and diabetes mellitus. Moreover, hyperparathyroidism in MEN 1 is clinically similar to the kidney failure condition; indeed, diffuse hyper-plasia of more than one gland is common. Vitamin D should not be administered to these patients. Nephrologists should be involved in MEN 1 follow-up, with the aim to prevent kidney failure development by correcting risk factors.

Displacement of the inferior vena cava as a factor limiting catheter performance in long-term hemodialysis patients.

We report a case of a lady affected by autosomal dominant polycystic kidney disease who had been on hemodialyis for 24 years. She has exhausted all options for arterious-venous fistula. The presence of an acquired anatomical abnormality was an obstacle in order to get appropriate blood flow from standard tunnelled femoral catheters. The enlarged right kidney was pushing the inferior vena cava to the left side of the abdomen, and the abnormality was demonstrated by phlebography. Only after placing a cuffed catheter 53 cm long in her left femoral vein we could dialyze efficiently. Venography is mandatory before placing a cuffed catheter especially in uremic patients with long history of access failure, because it saves costs.

Role of B-type natriuretic peptide in cardiovascular state monitoring in a hemodialysis patient with primary amyloidosis.

BACKGROUND: Cardiac involvement occurs in up to 50% of patients with primary or A amyloidosis (ALA) and is associated with very poor prognosis. B-type natriuretic peptide (BNP) has been proposed as a guide for treatment of heart failure patients and as an index of myocardial dysfunction in patients with ALA. Data about BNP dosage for cardiovascular monitoring of patients with ALA on renal replacement therapy are lacking. CASE: A 64 year old Caucasian man was admitted because of nephrotic syndrome in July 2003. Renal diagnosis was ALA. Melphalan and prednisolone were given but renal function worsened and in April 2004 standard bicarbonate hemodialysis was started. In March 2004 thalidomide was added to his therapy. During the follow-up ejection fraction was stable and was 65% on the contrary E/A ratio gradually increased and overtook 1. BNP plasma levels were increased and the values recorded during the follow-up were: 2505 pg/mL in October 2003 (normal reference values<100), 1827 in April 2004, 4006 in June 2004, 5000 in September 2004, 3750 in January 2005 and 1920 in April 2005. In September 2005 BNP was 3380 pg/mL. The patient was still alive after a follow-up longer than two years. CONCLUSION: In ALA patients a powerful prognostic role of BNP has been reported whose expression is increased in ventricular myocytes of patients with cardiac involvement. BNP level monitoring does not appear to be superior to standard echocardiography in evaluating cardiovascular status of uremic patients with ALA.