Tenosynovial Giant Cell Tumor (TSGCT) of the hip: MRI accuracy and results of surgical treatment.

Tenosynovial Giant Cell Tumor (TSGCT) or formerly pigmented villonodular synovitis (PVNS) is a rare nonmalignant tumor of the synovia seldom affecting the hip. MRI and surgical resection are the gold standards in its diagnosis and treatment. However, the accuracy of MRI is unknown, and only few reports on its surgical treatment results exist. The goal of the study was to investigate the MRI accuracy, results after surgical treatment, and natural history of untreated MRI-diagnosed hip TSGCT. Twenty-four consecutive patients with suspected TSGCT on hip MRI, between December 2006 and January 2018, were identified from our medical database. Six refused to participate. About 18 patients with a minimal follow-up of 18 months were enrolled. Charts were reviewed for histopathology results, specific treatment and recurrence. At the last follow-up, all patients had a clinical (Harris Hip Score [HHS]) and radiological examination (x-ray and MRI). Out of 18 patients with suspected TSGCT on MRI, with a mean age of 35y (range 17-52), 14 had surgi- cal resection and 4 refused surgery 1 of whom had a CT-guided biopsy. Out of 15 cases with biopsies, in 10 TSGCT was confirmed. Three surgically-treated patients showed recurrence on MRI after 24, 31 and 43 months. Two non-treated patients showed progression after 18 and 116 months. At the last follow-up (65 m; range 18-159), the mean HHS with or without recurrence was 90 and 80pts (ns). Operative vs. non-operative treatment showed HHS of 86 and 90pts (ns). In the conservatively-treated group, HHS with and without progression was 98 and 82pts (ns), respectively. MRI-suspected TSGCT of the hip was confirmed with biopsy in two-thirds of the cases. Surgical treatment showed recurrence in more than one-third of the patients. Two out of four untreated patients showed progression of the TSGCT-suspected lesion.

[Vocational training of future GPs in Swiss hospitals: the view of chief physicians]. / Weiterbildung von angehenden Hausärzten an stationären Weiterbildungsstellen: Sicht der Chefärzte.

BACKGROUND: We sought to assess the way future general practitioners are promoted by their chief physicians. METHODS: Semi quantitative questionnaires sent to 54 chief physicians (hospitals in cantons Zurich and Berne). RESULTS: 42 (77.8%) questionnaires were returned. 41 (97.6%) chief physicians asked the assistant physicians about their intended career. 23 (54.8%) discussed with future general practitioners (GPs) specific learning objectives. 32 (76.2%) chief physicians would have appreciated an official catalogue of learning objectives for future GPs. A GP-specific traineeship was lacking in most of the hospitals. CONCLUSIONS: The upcoming shortage of future GPs could be inhibited by an appropriate GP curriculum during the vocational training.

[Vocational training of future GPs in Swiss hospitals: the view of assistant physicians]. / Weiterbildung von angehenden Hausärzten an stationären Weiterbildungsstellen: Sicht der Assistenzärzte.

BACKGROUND: We sought to assess views of young physicians, performing vocational training in hospitals, regarding primary care. METHODS: Semi quantitative questionnaires sent to 535 assistant physicians (hospitals in cantons Zurich and Berne). RESULTS: 318 (62.4%) questionnaires were returned. 143 (45.0%) of the assistant physicians considered becoming a general practitioner (GP). There from, 58 (40.3%) decided in their undergraduate phase, 56 (38.9%) decided postgraduate. They felt generally supported by supervisors. A lack of specific learning objectives and hospital GP-traineeships were revealed. CONCLUSIONS: Our study shows that the decision for a career in primary care is made in equal parts undergraduate and postgraduate. These future and potential GPs should be attracted by an appropriate GP curriculum during their vocational training.

[Case report from the Department of Pediatrics, University of Berne, Switzerland]. / Purpura Henoch-Schönlein. Falldemonstration aus der kindermedizinischen Poliklinik der Med. Universitäts-kinderklinik Bern.

Classic purpura of Henoch-Schönlein is described in a seven years old girl. Prominent clinical markers of this disease entity are: cutaneous, non thrombozytopenic, purple skin lesions, swollen and painful joints, micro- or macrohematuria and abdominal pain. No causal therapy exists; for ambulatory control of painful symptoms non steoidal anti-inflammatory agents and steroids should not be prescribed (danger for intestinal hemorrage). Prognosis in childhood is good, development of chronic glomerulopathy can occur.

[The interesting case from the pediatric polyclinic]. / Der interessante Fall aus der kindermedizinischen Poliklinik.

An eleven year old girl from Turkey was diagnosed to have a periodic fever syndrome. The diagnosis of familial mediterranean fever was made by molecular analysis of a mutation in the MEFV-Gen which codes for pyrin. The disease is well-known in the mediterranean area and belongs to the periodic fever syndromes. These syndromes are discussed for their differential diagnosis focused to childhood.

[An interesting case in the pediatric polyclinic. Crohn disease with involvement of the distal esophagus, stomach and duodenum]. / Der interessante Fall aus der kindermedizinischen Poliklinik. Morbus Crohn mit Befall des distalen Oesophagus, Magen und Duodenum.

Weight loss, day-time sleep and loss of appetite were leading symptoms in a thirteen years old girl, in whom consecutively the diagnosis of Crohn's disease of the stomach and the duodenum was made after endoscopy and biopsy were performed. There were no complaints of diarrhea, abdominal pain or other symptoms suspicious for this chronic inflammatory bowel disease.

High morbidity and mortality in cystic fibrosis patients compound heterozygous for 3905insT and deltaF508.

Genotype-phenotype association in cystic fibrosis (CF) is difficult because of heterogeneous disease expression. The genotype-phenotype correlation for the 3905insT mutation in comparison to deltaF508 was studied here. Thirty CF patients compound heterozygous for 3905insT were compared to clinical presentation of matched patients homozygous for deltaF508 (1960-1997). Sweat tests, age at diagnosis, at death and at onset of Pseudomonas aeruginosa colonization were analysed. Chrispin-Norman scores and pulmonary function forced expiratory volume in one second (FEV1) determined severity of lung disease. Twenty-five of the patients with 3905insT had deltaF508 as a second mutation and five had another rare mutation. At the age of 15 yrs, 60% of patients with 3905insT had an FEV1 < 60% predicted in comparison to 25% of patients with deltaF508 (p<0.05). Age at death and cumulative survival rate was significantly lower (p<0.05) in the 3905insT than in the deltaF508 group (20.3 and 24.0 yrs, respectively). Age at onset of P. aeruginosa colonization was not different in the study groups. Sweat chloride concentrations were lower in patients homozygous for deltaF508 (105.63+/-15.3 mmol L(-1)) than in patients with 3905insT (119.9+/-22.1 mmol x L(-1)) (p<0.05). Patients compound heterozygous for 3905insT have similar high morbidity and mortality to patients homozygous for deltaF508.

[Swiss registry for patients with cystic fibrosis: design, programming, implementation and first examples of use]. / Schweizerisches Register für Patienten mit zystischer Fibrose: Planung, Programmierung, Ausführung und erste Beispiele der Anwendung.

The Swiss Registry for Cystic Fibrosis (SRCF) was designed to collect demographic, clinical and therapeutic data from patients with cystic fibrosis (CF) in Switzerland. It was designed, programmed and implemented for standalone application in Swiss cystic fibrosis centres. It is part of the European Registry for Cystic Fibrosis (ERCF), which has been implemented in Europe to collect data on the use and safety of dornase alpha (Pulmozyme) in the treatment of cystic fibrosis. At the time of first evaluation 245 cystic fibrosis patients are registered, their mean age is 13 years, and 17% are over 18. In larger databases in Germany or North America we observe comparable demographic data, similar degrees of severity and similar therapeutic approaches to those in Swiss cystic fibrosis patients. The aim of the Swiss Registry is to cover the maximum possible number of cystic fibrosis patients from this country.

Staphylococcus aureus septicaemia in a patient with cystic fibrosis.

UNLABELLED: Although bacterial colonisation of bronchi may occur from early childhood onwards, infections extending beyond the lungs are uncommon in patients with cystic fibrosis. A 12-year-old boy with cystic fibrosis, receiving oral corticosteroids for 3 weeks because of allergic bronchopulmonary aspergillosis, experienced pneumonia and septicaemia caused by Staphylococcus aureus. He was treated with flucloxacillin, ticarcillin-clavulanate, aztreonam, cefazolin and rifampin according to resistance testing of S. aureus cultured from the blood. On day 25 the patient finally had recovered. CONCLUSION: Systemic steroid therapy for allergic bronchopulmonary aspergillosis may favour life-threatening systemic bacterial infection which is rare in the immunocompetent patient with cystic fibrosis.

[Inhibition of renal carbonic anhydrase as a respiratory stimulant-- an obsolete indication?]. / Hemmung der renalen Carboanhydrase als Atemstimulans--eine obsolete Indikation?

Inhibition of carbonic anhydrase in general or specific inhibition of one the different isoenzymes results in a significant metabolic acidosis due to renal bicarbonate loss. The increase of arterial pCO2 stimulates central and peripheral chemoreceptors and enhances ventilation. The inhibition of carbonic anhydrase as a respiratory stimulant is an accepted measure for the prevention of acute mountain sickness, has been used for a restricted number of subjects with sleep-disordered breathing or chronic hypoxaemic lung disease. The few indications and the narrow therapeutic index restrict the use of carbonic anhydrase blockers as stimulants for ventilation.

Nutrition and lung function in cystic fibrosis patients: review.

Complex interactions between nutrition, skeletal and respiratory muscle function and energy expenditure in cystic fibrosis patients exist. Malnutrition significantly contributes to muscle weakness in patients with chronic obstructive pulmonary disease of the adult or in cystic fibrosis in childhood. Together with a measurable increase in resting energy expenditure the malnutrition, as a consequence of pancreatic insufficiency, leads to pulmonary deterioration. Whether pulmonary disease, pancreatic insufficiency, increased energy expenditure or insufficient intake of nutrition are the starters for the destructive circle or whether the basic defect is responsible for some of the components interacting with each other remains to be determined.

Effect of 3 weeks' rehabilitation on neutrophil surface antigens and lung function in cystic fibrosis.

Neutrophil leukocytes have been shown to be the predominant cells in inflammatory airway infiltrates of cystic fibrosis (CF) patients. The aim of this study was to investigate the effect of rehabilitation on neutrophil surface antigen expression and lung function in healthy controls and stable CF patients with moderately severe disease. The absolute number of neutrophils and the level of surface marker expression on neutrophils were elevated in 12 CF patients compared with eight healthy controls. The level of neutrophil surface marker expression was similar in bronchoalveolar lavage fluid from CF patients who underwent bronchoscopy for diagnostic or therapeutic reasons. After 3 weeks' rehabilitation, there was a significant reduction in the expression of CD11b (complement receptor type 3), CD13 (aminopeptidase N), CD32 (low-affinity Fc gamma chain receptor II), and CD35 (complement receptor type 1) in only the CF patients. At the same time, lung function improved significantly. The increase in forced vital capacity correlated significantly with the decrease in CD32 level. These results demonstrate that rehabilitation in a specialized clinic can reduce the neutrophil-dominated inflammation and improve the lung function of stable CF patients with moderately severe disease even without changing any medications.