A Rare Cause of Leg Ulcer: Calcinosis Cutis as a Part of CREST Syndrome.

Leg ulcers are not a disease themselves, they are a symptom of a disease. To manage them properly, finding the correct diagnosis of the disease is mandatory. Our case is a model to underline that leg ulcers are a significant burden for the patient, because leg ulcer got ahead of the patient's other serious complaints such as Raynaud's phenomenon or sclerodactyly. Furthermore, our patient is also a model, and an attentive clinical evaluation is inevitable to find the correct diagnosis. We present our case aiming to underline the significance of full dermatologic examination in each ulceration to discover underlying disease.

Anemia and leukopenia in a long-term parenteral nutrition patient during a shortage of parenteral trace element products in the United States.

Recently, drug shortages in the United States have affected multiple components of the parenteral nutrition (PN) solution. A 62-year-old patient with systemic sclerosis who was dependent on home PN due to intestinal dysmotility developed anemia and leukopenia approximately 4 months after parenteral copper was withheld from her PN solution due to drug shortages. The patient was not able to tolerate a sufficient amount of oral multivitamins with trace elements due to severe dysphagia. Her serum copper and ceruloplasmin concentrations were undetectable, confirming the diagnosis of severe copper deficiency. The hematological abnormalities promptly resolved with copper supplementation. This report emphasizes the importance of close monitoring for nutrient deficiencies during drug shortages and supplementing with oral or enteral nutrition when feasible, particularly in high-risk patients such as those with intestinal malabsorption or short bowel syndrome who are dependent on long-term PN.

[Double-lung transplantation in 15 patients with pulmonary hypertension]. / Trasplante bipulmonar en hipertensión pulmonar. Una serie de 15 pacientes.

BACKGROUND: Pulmonary hypertension is a serious disease that, in its terminal phase, requires lung transplantation. PATIENTS AND METHODS: A retrospective study was undertaken of 15 patients with pulmonary hypertension who underwent lung transplantation between 1994 and 2004. Clinical data recorded before the procedure and during follow-up were reviewed. RESULTS: Pulmonary hypertension was reported as idiopathic in 8 patients (53%) and related to consumption of toxic oil in 2. The remaining causes were documented as chronic peripheral pulmonary embolism, histiocytosis X, venoocclusive disease, scleroderma, and simple corrected congenital heart defect in 1 patient each. The mean values of the hemodynamic variables were 100, 50, and 67 mm Hg for systolic, diastolic, and mean pulmonary artery pressure, respectively; 2.63 L/min for cardiac output; and 20.9 Wood units for total pulmonary resistance. The mean time between diagnosis of pulmonary hypertension and lung transplantation was 5.9 years (range, 0.4-20 y). Seven patients were in functional class III and 8 in functional class IV. The mean 6-minute walk distance was 204 m (range, 0-360 m). Four patients (26%) died during the during the perioperative period and 9 (60%), 7 (46%), and 6 (40%) were still alive at 1, 3, and 5 years, respectively. CONCLUSIONS: Double-lung transplantation is a therapeutic option that, in certain cases, has similar outcomes to those achieved with the most aggressive medical treatment for pulmonary hypertension.

Úlceras acrales y fenómeno de Raynaud en un adolescente / Digital ulcerations and Raynaud´s phenomenon in an adolescent

El síndrome de Crest es una variedad de esclerodermia sistémica; concretamente, una forma limitada. Se considera una forma incompleta de la enfermedad, que presenta un curso más benigno, dado que las afectaciones renal y pulmonar no son sus principales características. Los síntomas fundamentales del síndrome de Crest, que conforman su acrónimo,son: calcinosis, fenómeno de Raynaud, dismotilidad esofágica, esclerodactilia y telangiectasias. Presentamos un caso cuyo inicio se produjo durante la adolescencia, hecho muy poco frecuente, que, sin embargo, presenta un mejor pronóstico que en la edad adulta

Crest síndrome is a type of limited systemic scleroderma. It is considered to be an incomplete form of the disease that has a more benign course, as renal and pulmonary involvement are not is principal characteristics. The major symptoms of Crest syndrome, the initials of which give it its name, are calcinosis, Raynaud´s phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia. We report a case with onset during adolescence, a very uncommon circumstance which however, is associated with a better prognosis than that involving adult onset

Progressive interstitial renal fibrosis due to Chinese herbs in a patient with calcinosis Raynaud esophageal sclerodactyly telangiectasia (CREST) syndrome.

A 58-year-old woman with calcinosis Raynaud esophageal sclerodactyly telangiectasia (CREST) syndrome presented with slowly progressive renal dysfunction. She was normotensive with normal plasma renin activity and lacking symptoms of vasculitis. Mild proteinuria was of tubular origin, but serological tests and an absence of sicca symptoms excluded the possibility of Sjögren's syndrome. Light microscopic study of renal biopsy showed interstitial fibrosis with ectasia and degeneration of proximal tubule and lymphocyte infiltration. There were no remarkable changes in the glomeruli. Chromatographic analysis of the Chinese herbs regimen that she had been taking for several years demonstrated aristolochic acid. She was diagnosed as Chinese herbs nephropathy. Therapy with oral prednisolone was markedly effective in improving renal function and anemia. To our knowledge, this is the first report of Chinese herbs nephropathy complicating connective tissue disease. It is important to consider the possibility of Chinese herbs nephropathy when patients treated with Chinese herbs develop renal dysfunction.