Subclinical pulmonary abnormalities in childhood-onset systemic lupus erythematosus patients.

OBJECTIVE: The aims of this study were to analyze the pulmonary function of childhood-onset systemic lupus erythematosus (cSLE) patients and to identify possible correlations between the high-resolution computed chest tomography (HRCT) score, disease activity, disease cumulative damage, and the participants' quality of life. METHODS: Forty cSLE patients, median age: 14.1 years (range: 7.4-17.9), underwent spirometry and plethysmography. Carbon monoxide diffusing capacity (DLCO), HRCT, disease activity, disease cumulative damage, and quality of life were assessed. RESULTS: Pulmonary abnormalities were evident in 19/40 (47.5%) cSLE patients according to spirometry/DLCO. Forced expired volume in one second (FEV1%) was the parameter most affected (30%). The HRCT showed some abnormality in 22/30 patients (73%), which were minimal in 43%. Signs of airway affects were found in 50%. Twelve patients were hospitalized due to cSLE-related pulmonary complications before the study began (median discharge: 2.1 years earlier). Total lung capacity (TLC%), vital capacity (VC%), forced vital capacity (FVC%), and FEV1% were significantly lower in the group with hospitalization compared to the group without hospitalization (p = 0.0025, p = 0.0022, p = 0.0032, and p = 0.0004, respectively). Of note, DLCO was positively correlated with disease duration (r = +0.4; p = 0.01). The HRCT-score was negatively correlated with FEV1/VC (r = -0.63; p = 0.0002), FEV1 (r = -0.54; p = 0.018), FEF25%-75% (r = -0.67; p < 0.0001), and HRCT-score was positively correlated with resistance (r = +0.49; p = 0.0056). CONCLUSIONS: Almost half of patients with cSLE had subclinical pulmonary abnormalities, especially airway abnormalities. The cSLE-related pulmonary complications seem to determine long-term functional damage.

A case of carcinoma arising in the intrapancreatic terminal choledochus 12 years after primary excision of a giant choledochal cyst.

A 27-yr-old woman with carcinoma developing in the intrapancreatic terminal choledochus 12 yr after the initial excision of giant choledochal cyst with Roux-en-Y hepaticojejunostomy is reported. Despite the generally accepted concept that the presence of bile is associated with malignancy in the remnant biliary ducts after excision of a choledochal cyst, this is the first report of a malignant growth developing in the terminal choledochus which had been free from the bile exposure long after the excision of the choledochal cyst. Long-term follow-up of the patient is advisable even after operation for primary resection of the choledochal cyst.

Granular cell tumors of the esophagus: a report of two cases and review of the literature.

Two patients with granular cell tumors of the esophagus are presented. Both were men, 54 and 51 years old, respectively, and without tumor-related complaints. Diagnosis was made by esophagogram and endoscopic biopsy. The two patients were treated with a left thoracotomy and local excision of the tumors. A review of the world literature was carried out and data of general interest were collected. The symptoms and management of esophageal granular cell tumors, as well as histogenesis, are discussed.