Investigations of the influence of external nose deformities on nasal airflow.

BACKGROUND: Abnormalities of the external shape of the nose are often felt as cosmetically disturbing. In many cases an additional hampering of the respiratory function of the nose is seen and causes pathological nasal airflow patterns. For the functional outcome of aesthetic-surgery of the nose, knowledge of nasal flow is essential. In the medical literature there are some discrepancies between the relationship of nasal shape and nasal flow. We investigated typical airflow patterns in different abnormalities of the external nose. METHODS: We performed fluid dynamic experiments on exact, anatomical nasal models and functional nasal models (so-called modified Mink boxes). We investigated the inspiratory flow pattern in nose-models with typical variations of the shape of the external nose. RESULTS AND CONCLUSIONS: There were typical airflow patterns for every external nasal abnormalities. The normal nose shows a disturbance of the streamlines over the entire nasal cavum. Under physiological flow velocities we find laminar and turbulent flow. The pathological variations of the nasal shape show mostly different airflow patterns and characteristics. The main reason for that is a deformed or anatomically false configurated inflow area (vestibulum, isthmus and anterior cavum). During rhinoplasty the reconstruction of the nasal inflow area has to be taken into account.

Liver transplantation in a patient with Budd-Chiari syndrome secondary to factor V Leiden mutation.

A point mutation in the factor V Leiden gene is the most common hereditary thrombophilic state and an important risk factor for Budd-Chiari syndrome. We report on a patient with Budd-Chiari syndrome secondary to factor V Leiden mutation, who underwent successful liver transplantation. Following liver transplantation, his thrombophilic state was corrected and he did not require anticoagulation therapy. There has been no recurrent venous thrombosis for 14 months after transplantation. Although his activated protein C sensitivity was normal, showing the normalization of protein C-factor V interaction, PCR analysis demonstrated that heterozygosity for factor V Leiden mutation was still present. We suggest checking resistance to activated protein C, rather than PCR analysis of factor V Leiden mutation in patients with Budd-Chiari syndrome after liver transplantation; the presence of the second does not effect clinical outcome.

Hemophagocytic syndrome after living-related liver transplantation.

Hemophagocytic syndrome (HPS) is a life-threatening hematological disorder in immunocompromised patients. Although the number of patients with HPS following liver transplantation is scarce the outcome is usually fatal. We report a patient who developed HPS following living-related liver transplantation (LRLT) and was treated successfully by a combination of intravenous (IV) immunoglobulin (Ig) and granulocyte colony-stimulating factor (GCSF).