[Bilateral pneumothorax during awake fiberoptic intubation : A rare complication of bronchoscopy]. / Beidseitiger Pneumothorax bei fiberoptischer Wachintubation : Eine seltene Komplikation der Bronchoskopie.

An 86-year-old woman was scheduled for surgical treatment of a periprosthetic femoral fracture after a fall. Because of a known difficult airway due to multiple surgeries and radiation for cancer in the orofacial area, awake fiberoptic intubation was planned. During the fiberoptic maneuver the patient experienced a massive bout of coughing which resulted in large soft tissue emphysema and double-sided pneumothorax. This case reports a rare complication of fiberoptic intubation and airway management.

[Sentinel node mapping in head and neck squamous cell carcinoma]. / Sentinel-Node-Markierung bei Plattenepithelkarzinomen des Kopfes und Halses.

BACKGROUND: Commonly used staging procedures often cannot predict the absence of lymphatic micro- metastases in squamous cell carcinoma (SCC) of the head and neck. Therefore in many cases an elective neck dissection (ND) is necessary. In the surgical therapy of melanoma or breast cancer the presence of metastases can be evaluated securely by identification and examination of the sentinel lymph node (SLN). The type of surgical procedure is usually chosen in regard to the histopathological result. The present study evaluates the applicability of this concept for SCC of the head and neck. METHODS: Radioactive labeled micro-albumin particles were injected preoperatively around the tumor in 38 patients without proven metastases. Following the excision of the primary tumor the sentinel lymph node/s were detected and dissected and ND was completed. Histological examination of the tissue was performed to evaluate whether the SLN reflected the lymphatic status. RESULTS: In two cases (5.1 %) no SLN were detected. ND was completed in 32 cases. In nine cases (28.1 %) the SLN were infiltrated by the primary tumor. In 22 cases (68.8 %) SLN and ND revealed a N(0) stage. In one case (3.1 %) we could not identify a metastasis because of the anatomical closeness of the SLN to the primary. The negative predictive value was 96 %. CONCLUSION: Predictive value regarding metastases to the neck was higher with the detection of SLN than with conventional staging procedures. Whether the detection of a tumor-free SLN is an indication not to perform an elective neck dissection is a matter of discussion, especially under the aspect of the effective reduction of postoperative morbidity.

Angiotensin-converting enzyme (ACE) gene polymorphisms and familial occurrence of sarcoidosis.

OBJECTIVES: The aim of this study was to test for genetic linkage and association between polymorphisms of the angiotensin-converting enzyme (ACE) gene and familial occurrence of sarcoidosis. DESIGN, SETTING AND SUBJECTS: German families with more than one member suffering from sarcoidosis were contacted and a DNA bank was established. Sixty-two families (140 patients, 77 females and 63 males, and 104 unaffected relatives) were genotyped for the ACE gene insertion/deletion (I/D) polymorphism and for two flanking variable sites (ACE A-5466C and ACE 4656(CT)2/3). As controls, 100 DNAs from unrelated resident Caucasians (50 females, 50 males) were analysed. ACE allele and genotype frequencies were determined, and parametric linkage and affected sib pair analyses and transmission disequilibrium tests were performed. RESULTS: There was a striking over-representation of the ACE I/D genotype DD in patients with sarcoidosis and their families as compared with controls of the study and well founded genotype frequencies from the literature. The same was evident for the accompanying genotypes CC and 2,2 of the flanking polymorphisms. Linkage between the segregation of ACE alleles and the disorder within families was clearly excluded for simple models of inheritance. However, there was a suggestive but not significant (P = 0.06) excess of allele sharing amongst affected siblings. There was no transmission disequilibrium for any ACE allele or haplotype. CONCLUSIONS: ACE is involved in the pathogenesis of sarcoidosis, but the ACE polymorphisms are not an inherited main cause of the disease. They are more likely to modify the development of the disorder, and the ACE I/D genotype DD might be a promoter to clinical manifestation.

Familial sarcoidosis is linked to the major histocompatibility complex region.

Sarcoidosis is a systemic granulomatous disorder associated with high CD4+ cell activity, but no pathogen is detectable. Clustering in families occurs, and the existence of a genetic predisposition to sarcoidosis is widely accepted. The major histocompatibility complex (MHC) is believed to contribute to this susceptibility. Many studies testing this hypothesis have produced conflicting results. We have genotyped 122 affected siblings from 55 families for seven DNA polymorphisms that flank and cover the MHC region on chromosome 6, and for HLA-DPB1, a candidate gene for granulomatous disorders. Multipoint nonparametric linkage (NPL) analysis showed linkage (NPL score > 2.5; p < 0.006) for the entire MHC region with a maximum NPL score of 3.2 (p = 0.0008) at marker locus D6S1666 in the Class III gene cluster. There was a significant excess of marker haplotype sharing among affected siblings. However, the frequency of HLA-DPB1 alleles on 104 shared chromosomes did not differ from that of a control group of founders from the family panel. Transmission disequilibrium was found for allele DPB1*0201, but only nine families contributed to this result. We conclude that genes of the MHC are involved in the genetic predisposition to sarcoidosis, but HLA-DPB1 alone does not sufficiently explain this fact.

Microheterogeneity of acute-phase glycoproteins in patients with pulmonary sarcoidosis.

This study was designed to investigate qualitative changes in the carbohydrate side-chains of two acute-phase glycoproteins, alpha 1-acid glycoprotein (AGP) and alpha 1-antichymotrypsin (ACT), in 37 patients with pulmonary sarcoidosis. The glycosylation profile of AGP and ACT was studied using affinity immunoelectrophoresis with the lectin concanavalin A (conA). Serum concentration of soluble receptor for interleukin-2 (sIL-2R) and activity of serum angiotensin converting enzyme (ACE) were measured by specific enzyme-linked immunosorbent assay (ELISA) and enzyme kinetic assay, respectively. Rocket immunoelectrophoresis and nephelometric assay were used to determine serum concentration of AGP, ACT and C-reactive protein (CRP). In 11 patients with active disease, a decreased reactivity of AGP with conA was found as compared with controls (n = 44) and patients with nonactive sarcoidosis (n = 26). A similar tendency was seen with ACT. In the same group, increased concentrations of serum AGP and higher levels of sIL-2R were detected compared with patients with nonactive sarcoidosis. In the entire sarcoidosis group, there was a negative correlation between ACE activity and AGP and ACT affinity for conA (r = -0.6358, and r = -0.5019, respectively) and a positive correlation with sIL-2R level (r = 0.8241). In nine patients with elevated concentrations of serum CRP, no differences were found in disease activity and glycosylation profile of AGP and ACT when compared to patients with normal serum CRP. The results suggest that in active pulmonary sarcoidosis changes in the glycosylation pattern of acute-phase glycoproteins exist, which are similar in trend and magnitude to those found in other chronic inflammatory diseases. The synthesis and glycosylation of acute-phase proteins in pulmonary sarcoidosis are probably regulated independently.

[Value of 99mTc-MIBI scintigraphy in diagnostic localization of hyperparathyroidism. A prospective study]. / Zum Stellenwert der 99mTc-MIBI-Szintigraphie bei der Lokalisationsdiagnostik des Hyperparathyreoidismus. Eine prospektive Studie.

We evaluated the sensitivity of praeoperative parathyroid imaging using 99mTc-MIBI scintigraphy in planar as well as SPECT technique to detect and localize abnormal parathyroid glands in 36 patients with hyperparathyroidism. Seven out of these patients had been previously operated in the thyroid area. With a sensitivity of 76% (22/29) solid adenomas could be localized correctly. Surgical success was estimated by the weight of the adenomas, by histology and postoperative laboratory findings. Compared to other imaging procedures 99mTc-MIBI-scintigraphy seems to be most sensitive in detecting and localizing abnormal parathyroid glands, especially in previously operated patients. Praeoperative standard in nuclear medicine up to now is Tl-Tc-subtraction scintigraphy, which is technically more difficult, sensitive to artefacts and exposes the patient to more radiation. Based on our experience we would therefore suggest to screen all previously operated patients with 99mTc-MIBI scintigraphy on a routine basis.

Antibodies to heat shock proteins in patients with pulmonary sarcoidosis.

Serum concentrations of IgG, IgA, and IgM antibodies to three heat shock proteins (HSPs)--ubiquitin, HSP70, and HSP90--were measured using ELISA in 37 patients with pulmonary sarcoidosis. When compared to healthy controls (n = 44), increased concentrations of IgA and IgG antibodies to ubiquitin were found in 13 (35.1%, p < 0.002) and 7 (18.9%, p < 0.05) patients, respectively. In 10 patients (27.0%) elevated concentrations of IgG antibodies to HSP70 were detected (p < 0.02), whereas IgA antibodies to this protein were found in 7 cases (18.9%, p < 0.05). IgM antibodies to ubiquitin and HSP70, and antibodies to HSP90 were not detected in patients' sera. The levels of antibodies to ubiquitin and HSP70 correlated well with each other within the given immunoglobulin class (r = 0.7391, p < 1E-5 and r = 0.9854, p < 1E-5 for IgG and IgA class, respectively). There was also a weak correlation between the level of IgG antibodies to HSP70 and both serum activity of angiotensin converting enzyme (SACE; r = 0.4668, p < 0.005) and serum level of soluble receptor for interleukin 2 (sIL-2-R; r = 0.4142), p < 0.02). A similar tendency was seen with IgG antibodies to ubiquitin. Furthermore, there was an association between the increased concentration of C-reactive protein (CRP) and increased levels of IgG antibodies to ubiquitin and HSP70 (p < 0.005 and p < 0.05, respectively). Our results suggest that antibodies to various HSPs are present in a subset of patients with sarcoidosis. The humoral immune response to HSPs relates probably to the immune activation and/or infection.

Diagnostic reliability of somatostatin receptor scintigraphy during continuous treatment with different somatostatin analogs.

In order to evaluate the diagnostic reliability of somatostatin receptor scintigraphy (SRS) in patients treated with somatostatin analogs, ten patients with metastatic neuroendocrine tumors were investigated before and during continuous treatment. Different somatostatin analogs were used for therapy: five patients received octreotide (Sandostatin, Sandoz, Switzerland) and five were treated with BIM 23014 (Lanreotide, Ipsen Biotech, France) within the scope of a clinical phase II study. The SRS findings were analyzed in terms of biodistribution of the labeled somatostatin analog and tumor visualization comparing the two studies in each patient. Whereas liver, spleen, and kidney uptake were decreased during octreotide treatment and increased on lanreotide therapy, tumor accumulation was intensified in all but one patient. Our results suggest that the diagnostic value of SRS is not necessarily restricted during treatment with somatostatin analogs. Indeed, tumor visualization may even be enhanced in this therapeutic setting.

[Somatostatin receptor scintigraphy. A new imaging procedure for the specific demonstration of carcinoids of the small intestine]. / Somatostatin-Rezeptor-Szintigraphie. Ein neues bildgebendes Verfahren zum spezifischen Nachweis von Dünndarmkarzinoiden.

Scintigraphy with a radiolabelled somatostatin analog represents a new highly specific approach in the diagnostic work-up of receptor-positive APUD tumours and their metastases. We present our preliminary results with somatostatin receptor scintigraphy in 15 patients with histologically proven midgut carcinoid. 5 out of 6 primary tumour sites (83%) and 90% of the known metastatic lesions could be detected; unknown metastatic lesions were seen in 5 patients. Compared with other nuclear medicine procedures somatostatin receptor scintigraphy is able to detect all tumour sites within hours. This advantage will promote the acceptance of this sensitive and specific imaging modality by the clinicians with regard to preoperative work-up and symptomatic therapy with a somatostatin analog.

[Excimer laser angioplasty in arterial obstructive disease. Clinical experience with guidewire-controlled catheter systems]. / Excimer-Laserangioplastie bei arterieller Verschlusskrankheit. Klinische Erfahrungen mit fürhrungsdrahtgesteuerten Kathetersystemen.

Percutaneous transluminal laser angioplasties (PTLA) were performed in nine patients (7 males, 2 females, aged 52-81 years) with peripheral vascular disease, stages IIB-IV (Fontaine's classification), with 75% stenosis (5 patients) or occlusion (4 patients) in the area of the superficial femoral artery. Two catheter systems were used. One was a monofiber catheter (600 microns) with eccentric guidewire, the other a multifiber catheter with 12 concentrically arranged fibres (200 microns each) and a central guidewire. An XeCl-excimer laser (308 nm), with a pulse duration of 50 ns and pulse frequency of 15-30 Hz, served as the laser source. It was possible in all patients to reduce the stenosis of 75-100% to 35-70% or reopen the occlusion without laser-associated complications. To remove the residual stenosis PTLA was followed by balloon dilatation in seven patients and by application of a Simpson atherectomy catheter in one patient: these measures reduced the degree of stenosis to 20-40%.

[The vena cava superior syndrome in sarcoidosis]. / Vena cava superior-Syndrom bei Sarkoidose.

We report the first observation of clinical manifestations of vena cava superior syndrome (VCSS) associated with sarcoidosis. Twenty-four years after the first signs of the disease had been noted, mediastinal lymphomas penetrating the wall of the vena cava superior caused complete obstruction. It is most unusual for the vessel wall to be destroyed in this way, which explains why VCSS is often missed in sarcoidosis. The obstructed vessel was resected and successfully replaced by a Gore-Tex prosthesis. The importance of VCSS for the differential diagnosis is pointed out. Two further peculiarities are the simultaneous occurrence of elevated intraocular pressure and VCSS, and the familial incidence.

Sarcoidosis complicated by HTLV III-infection: steroid therapy in combination with thymostimulin.

Report on a patient suffering from severe lung sarcoidosis and HTLV III-infection. Under combined treatment with cortisone and thymostimulin the patient recovered surprisingly well. The X-ray showed a frank improvement of the lung alterations, and in addition an extensive normalization of leucocytes and lymphocytes could be noticed. No side-effects could be observed under this treatment.

Immuno-cytological blood tests in cases of sarcoidosis.

Monocytosis, occurring in the majority of sarcoidosis cases in the bloodstream, and an increased number of natural killer cells are a manifestation of an increased cellular defence by bone marrow cells. The antigen-offering function of natural killer cells, macrophages and monocytes, should be specially emphasized. While the majority of sarcoidosis patients show an absolute lymphocyte count of less than 1500 cells/microliter during the usual clinical blood tests, the determination of lymphocyte subpopulations by means of monoclonal antibodies displayed a different behaviour of the lymphocyte subpopulations, especially a substantial reduction of T-cells, while B-lymphocytes were at normal levels. The assumption, that sarcoidosis immunology is characterized by an imbalanced homeostasis in the sense of a decrease in cellular and an increase in humoral immune defence, can no longer be maintained. We are rather dealing with an increased immunological activity of the cellular and humoral immune defence. In addition, the investigation of the lymphocyte subpopulations in the blood allows a judgement on the existing activity of the disease. The statement of a helper-suppressor ratio of less than 2.0 suggests progress of disease and indicates therefore drug therapy. Our immunological findings also explain the difference of the prognosis, which is more favourable for women than for men.

[Current status of clinical and scientific sarcoidosis research 100 years later]. / Stand der klinischen und wissenschaftlichen Sarkoidose-Forschung nach 100 Jahren.

According to today's knowledge the definition of sarcoidosis is formulated as follows: Sarcoidosis is a systemic disorder induced by inhalation, to be interpreted as a reactionary disease with genetic predisposition. The inducing noxa are still unknown. Sarcoidosis is characterized: a) by its specific course appearing as radiologically visible stages, b) immunologically by a disturbed balance between cellular and humoral defence reaction, c) in view of the pathological anatomy by the epitheloid cell granuloma. The clinical pictures have to be differentiated between two forms of this disease: an acute course and an asymptomatically starting chronic course. The clinical phenomenology shows great variety as to the course and the predominantly injured organs. Concerning the nature of sarcoidosis nevertheless we have a precise idea today. The diagnosis is in most cases without problems. Corticoid therapy is the only effective treatment.