Osteomyelitis of the maxilla in a patient with Malignant Infantile Osteopetrosis

Osteopetrosis is characterized by a considerable increase in bone density resulting in defective remodeling, caused by failure in the normal function of osteoclasts, and varies in severity. It is usually subdivided into three types: benign autosomal dominant osteopetrosis; intermediate autosomal recessive osteopetrosis; and malignant autosomal recessive infantile osteopetrosis, considered the most serious type. The authors describe a case of chronic osteomyelitis in the maxilla of a 6-year-old patient with Malignant Infantile Osteopetrosis. The treatment plan included pre-maxilla sequestrectomy and extraction of erupted upper teeth. No surgical procedure was shown to be the best to prevent the progression of oral infection. Taking into account the patient's general condition, if the patient develops severe symptomatic and refractory osteomyelitis surgery should be considered. The patient and his family are aware of the risks and benefits of surgery and its possible complications.

Long-evolution ascites in a patient with constrictive pericarditis

Constrictive pericarditis (CP) is an uncommon disease resulting from chronic pericardial inflammation, fibrosis and calcification. Since there are atypical forms of presentation, with subtle or nonexistent cardiorespiratory symptoms, diagnosis may be challenging and difficult. Recurrent ascites in patients with congestive hepatopathy due to constrictive pericarditis is, in most cases, reversible after pericardiectomy. Nevertheless, development of persistent liver dysfunction may be a long-term complication. The present case describes a 23 years old man with growth delay, dyspnoea and long evolution ascites, whose exhaustive etiological investigation led to diagnosis. Afterwards the patient underwent elective surgery with symptom and general condition improvement. Ascites differential diagnosis and its association with constrictive pericarditis are briefly reviewed in this article.

Outcomes of a general hospital-based Home Parenteral Nutrition (HPN) program: report of our experience from a 26-year period

Background: Home parenteral nutrition (HPN) was introduced in Spain in the late 1980s. Our hospital was a pioneering medical centre in this field. Aim: Analyze outcomes of our HPN program. Methods: Retrospective study of patients receiving HPN between 1986-2012. Study variables are expressed as frequency, mean ± SD (range), median [interquartile range]. Parametrics, non-parametrics test and survival analysis (p < 0.05) were applied. Results: 91 patients (55 females and 36 males, mean age: 50.6 ± 5 yrs.) who received HPN for an accrual period of 55,470 days (median: 211 days [range: 63-573]) were included. The most prevalent underlying condition was cancer (49.5%), with the commonest HPN indication being short bowel syndrome (41.1%). The most frequently used catheter type was the tunneled catheter (70.7%). The complication rate was 3.58/1,000 HPN days (2.68, infection; 0.07, occlusion; 0.07 thrombosis; and 0.59, metabolic complications). Complications were consistently associated with both the underlying condition and HPN length. Infections were most frequent within the first 1,000 days of HPN. Liver disease incidence was related to HPN duration. HPN could be discontinued in 42.3% of patients. Ten-year survival rate was 42%, and varied across the underlying conditions. Conclusions: In the present series, the commonest reason for HPN was cancer. Our complication rate is in keeping with that reported in the literature. The overall survival rate was 42%, and varied across the underlying conditions.