MR lymphangiography of lymphatic abnormalities in children and adults with Noonan syndrome.

Noonan syndrome is associated with complex lymphatic abnormalities. We report dynamic-contrast enhanced MR lymphangiography (DCMRL) findings in children and adults with Noonan syndrome to further elucidate this complex disease spectrum. A retrospective evaluation of patients with confirmed Noonan syndrome and clinical signs of lymphatic dysfunction undergoing DCMRL between 01/2019 and 04/2021 was performed. MRL included T2-weighted imaging (T2w) and DCMRL. Clinical history/presentation and genetic variants were recorded. T2w-imaging was evaluated for central lymphatic abnormalities and edema distribution. DCMRL was evaluated regarding the presence of cisterna chyli/thoracic duct, lymphatic leakages, pathological lymphatic reflux and abnormal lymphatic perfusion. The time from start of contrast-injection to initial enhancement of the thoracic duct venous junction was measured to calculate the speed of contrast propagation. Eleven patients with Noonan syndrome with lymphatic abnormalities (5 female, 6 male; 7 infants, 4 adults; mean age 10.8 ± 16.4 years) were identified (PTPN11 n = 5/11 [45.5%], RIT1 n = 5/11 [45.5%], KRAS n = 1/11 [9%]). Patients had a chylothorax (n = 10/11 [91%]) and/or pulmonary lymphangiectasia [dilated pulmonary lymph vessels] (n = 9/11 [82%]). Mediastinal/pulmonary edema was depicted in 9/11 (82%) patients. The thoracic duct (TD) was (partially) absent in 10/11 (91%) cases. DCMRL showed lymphatic reflux into intercostal (n = 11/11 [100%]), mediastinal (n = 9/11 [82%]), peribronchial (n = 8/11 [73%]), peripheral (n = 5/11 [45.5%]) and genital lymphatics (n = 4/11 [36%]). Abnormal pulmonary/pleural lymphatic perfusion was seen in 8/11 patients (73%). At infancy peripheral/genital edema was more prevalent in patients with RIT1 than PTPN11 (n = 3/5 vs. n = 0/5). Compared to patients with PTPN11 who had fast lymphatic enhancement in 4/5 patients, enhancement took markedly longer in 4/5 patients with RIT1-mutations. Thoracic duct dysplasia, intercostal reflux and pulmonary/pleural lymphatic perfusion are characteristic findings in patients with Noonan syndrome presenting with chylothorax and/or pulmonary lymphangiectasia. Central lymphatic flow abnormalities show possible phenotypical differences between PTPN11 and RIT1-mutations.

[Classification of pancreatitis]. / Klassifikation der Pankreatitis.

BACKGROUND: Both, acute and chronic pancreatitis represent complex disease patterns, whose effective treatment is based on structured diagnostics and therapy by a multi-professional team. There are different systems for an improved objectivity in the classification of the severity of the disease OBJECTIVES: Overview of the common classifications of acute and chronic pancreatitis. MATERIALS AND METHODS: Literature research of currently used classifications of acute and chronic pancreatitis. Evaluation of the current chronic pancreatitis guideline. RESULTS: For acute pancreatitis, the modified Atlanta Classification and the "determinant-based" classification are most widely used. These classifications are complemented by clinical risk scores such as the Marshall Score and the SOFA Score. In chronic pancreatitis, the image-based Cambridge classification was established. For clinical assessment further classifications such as the ABC classification and the M­ANNHEIM classification are applied evaluating leading symptoms such as pain, exocrine and endocrine pancreatic insufficiency.

The accuracy of video imaging for mixed dentition and adolescent treatment.

The purpose of this study was to evaluate the accuracy of computerized video imaging in predicting the soft tissue outcome of growth modification treatment for skeletal Class II malocclusions. Pretreatment and posttreatment cephalometric and facial photographic records of 22 mixed dentition (8 to 10 years old) and 20 adolescent (12 to 14 years old) patients were digitized, and the known outcomes were compared with computer-generated VTOs and video images. The predicted video images were found to be reasonably accurate for the mixed dentition group, but unacceptable for the adolescent group. When graded by a panel of judges, orthodontists were far more critical of the findings than their lay counterparts. These results emphasize the potential of video imaging as a communication medium, rather than as a diagnostic tool for growing patients.

[Is transvenous cardiac pacemaker implantation with X-ray control associated with a dangerous radiation exposure? (author's transl)]. / Führt die transvenöse Herzschrittmacherimplantation unter Röntgenbildkontrolle zu einer gefährlichen Strahlenbelastung

From July to December 1974 we measured the radiation exposure of the hands of the operator and assistant with 69 implantations of permanent cardiac pacemakers and recorded a dose of 0,5 mrem/min, this corresponds to 4 mrem/patient.