Langerhans' cell granulomatosis in an adult: a 22-year follow up.

A 57-year-old patient with Langerhans' cell granulomatosis (LCG) is presented. During 22 years of treatment, there were five relapsing infiltrations at different sites of the skull bones, which were treated by surgery, local radiotherapy, and chemotherapy. During the last relapse, the right temporal bone was infiltrated by granuloma and the petrous bone was destroyed with an intimate spread to the internal carotid artery. After two palliative surgical resections and ineffective radiotherapy, 12 cycles of chemotherapy (vinblastine plus prednisolone) were applied and a clinical remission of the disease was achieved. Special attention is paid to the complexity of diagnosis and choice of therapy. It is concluded that the behavior of LCG may change with time, and assume an aggressive form of the disease. Chemotherapy is the treatment of choice for this type of multifocal malignant form of LCG. Prognosis of the disease is unpredictable.

First Croatian auditory brainstem implantation.

A deaf female patient was diagnosed with bilateral acoustic neurinomas. Diagnosis incorporated the standard audiological battery for sensorineural hearing loss, computed tomography and magnetic resonance imaging. The left side had been operated on four years previously in another clinic using the suboccipital approach. The auditory brainstem implant surgery was performed on the 'second side' using the same approach. It was an uneventful operation with good anatomy and no serious post-operative complications. Post-operatively, the patient performed exceptionally well, with up to 50 per cent of words recognized in the opened set and 85 per cent in the closed set, both without lip-reading.

Effect of cromolyn pre-treatment on capsaicin-induced rhinitis in rats.

The effect of cromolyn sodium local pre-treatment on capsaicin-induced rhinitis in rats was studied by analyzing tissue changes due to edema, inflammatory cell infiltration and IgA upregulation. Nasal mucosa samples were stained with hematoxylin-eosin and examined immunohistochemically with monoclonal IgA antibodies. Changes were analyzed at 6, 12 and 72 h after capsaicin treatment and were scored semiquantitatively. Results showed that local cromolyn pre-treatment modified all parameters observed in the nasal mucosa following capsaicin-induced rhinitis in the rats.

Dose-dependent protective effect of BPC 157 on capsaicin-induced rhinitis in rats.

Protection of BPC 157 on capsaicin-induced rhinitis was studied in Wistar rats for its effect on mastocyte infiltration, degranulation and inflammatory cell infiltration. Animals were pretreated with 10 microg/kg, 10 ng/kg or 2 ml saline i.p. and capsaicin (0.05 ml/nostril of 1750 nmol/l sol.) was applied intranasally. They were then euthanized at 1, 3 and 12 h after capsaicin provocation. Nasal mucosa was analyzed and scored for mastocyte infiltration, degranulation and inflammatory cell infiltration. BPC 157 pretreatment significantly prevented mastocyte infiltration at 1 h. Polymorphonuclear leukocyte infiltration was significantly reduced in rats pretreated with 10 microg/kg BPC 157. A dose-dependent effect of BPC 157 pretreatment was demonstrated only for polymorphonuclear leukocyte infiltration at 12 h.

Adaptation to oral breathing after anterior nasal packing.

We report the changes in blood gas tensions in a group of 17 patients during a five-day period of anterior nasal packing after endonasal surgery. Significant hypocapnia was observed in the early postoperative period, which was followed by significant hypoxemia within the first 48 h after surgery, and also shortly after removal of the nasal pack. Analyses of the changes in blood gas tensions suggested disturbances in the ventilation-perfusion ratio. Most subjects required a period of 48 h to adapt to oral breathing.

Exogenous and endogenous suppression of thyroid-stimulating hormone induces similar effects on thyroidal iodothyronines.

Thyroidal concentrations of T4 and T3 and the T4/T3 ratio were analyzed in the nodular and paranodular tissues from two groups of patients with suppressed TSH secretion. The first group consisted of 17 patients with nontoxic nodular goitre (NG), 8 of whom received long-term levothyroxine therapy to suppress TSH, while remaining 9 were untreated. The second group consisted of 10 patients with autonomously functioning thyroid adenoma (AFTA), in whom TSH secretion was suppressed due to the adenoma-induced increase in thyroid hormone concentrations. In nodular tissues of NG patients, thyroidal T4 and the T4/T3 ratio were significantly higher in treated than in untreated patients (0.34 +/- 0.05 vs. 0.15 +/- 0.02 mol T4/mol of thyroglobulin (Tg) and 10.9 +/- 1.2 vs. 5.2 +/- 0.7 respectively). Analysis of paranodular tissues of NG patients also revealed a higher T4/T3 ratio in treated patients (16.0 +/- 2.1 vs. 6.9 +/- 0.9), although thyroidal T3 and T4 concentrations in treated and untreated patients were similar. In AFTA patients, both T3 and T4 concentrations were higher in the adenoma than in paranodular tissues (0.14 +/- 0.04 vs. 0.02 +/- 0.005 mol T3/mol Tg and 1.08 +/- 0.32 vs. 0.26 +/- 0.06 mol T4/mol Tg), whereas the T4/T3 ratio was significantly higher in paranodular tissues (23.2 +/- 5.9 vs. 9.3 +/- 1.8). These results indicate that suppression of TSH induced either exogenously or endogenously results in an increase in the thyroidal T4/T3 ratio that reflects an increase in T4 and/or a decrease in T3 concentrations. These findings also support the notion that TSH preferentially stimulates thyroidal T3 production.

Compensatory mechanisms induced by high oropharyngeal airway resistance in rats.

Acid base balance changes were observed during 72 h following bilateral nasal obstruction in rats. Mouth breathing caused acute respiratory acidosis and marked aerophagia, leading to spontaneous death of the experimental animal 80 to 90 h postoperatively. Stenotic oropharyngeal airway, due to palatal-epiglottic approximation, is supposed to be responsible for respiratory insufficiency in the nose obstructed rats. The compensatory changes in respiratory mechanics caused by high oropharyngeal airway resistance, together with some possible reflex changes, may have caused either air swallowing or aspiration. As changes in acid base balance parameters did not show breakdown of the compensatory mechanisms during the first 72 h postoperatively, it is supposed that the increased air volume in stomach and guts, causing elevation of the diaphragm and paralytic ileus, contributed to the experimental animals' death.

Relationship between T4, T3 and T4/T3 ratio in thyroid tissue, thyroid and peripheral veins in patients with nontoxic nodular goiter.

The relationship between T4, T3 and T4/T3 ratio in thyroid nodules/paranodular tissues, thyroid and peripheral veins has been investigated in 26 patients with nontoxic nodular goiters; eleven of them were treated with l-thyroxine 150 micrograms daily. A significant correlation between iodothyronine concentrations and T4/T3 [corrected] ratio in paranodular thyroid tissues and thyroid effluents was found in both groups of patients. By contrast, the correlation between these parameters in the nodule and thyroid veins was poor, which implies that the thyroid hormone pattern in the thyroid veins on the side of nodular lesion is predominantly controlled by the release of the hormones from paranodular healthy tissue. A close dependence of the serum T4/T3 ratio on the values of iodothyronines and their ratio in thyroid tissues and thyroid veins was observed in nontreated, but not in treated patients. Conversely, in the latter group, the thyroidal T4/T3 ratio in paranodular tissue, but not in the nodule, was found to be dependent on the serum T4/T3 ratio, suggesting that paranodular thyroid tissue more readily responds to 1-thyroxine-inhibited TSH secretion. The results demonstrate that 1. the serum thyroid hormone pattern under physiological conditions is dependent on the intrathyroidal T4/T3 ratio, and 2. minor alterations in the serum thyroid hormones may secondarily change the thyroidal T4/T3 ratio, presumably by their effect on TSH secretion.

Increased thyroidal T4/T3 ratio in nodular and paranodular tissues of nontoxic goiter following suppressive treatment with thyroid hormones.

The effect of suppressive treatment with thyroid hormones on thyroidal iodothyronines and T4/T3 ratio in nodular and paranodular tissues was investigated in 12 patients with nontoxic goiter. Results were compared to those from 11 nontreated patients. Continuous thyroid hormone administration produced a significant increase in thyroidal T4 and T4/T3 ratio in nodular tissues while T3 remained unchanged. In paranodular tissues a significant rise of T4/T3 ratio, an insignificant increase in T4 and a decrease in T3 were observed following the administration of thyroid hormones. The results are very similar to those obtained in paranodular tissue of autonomously functioning thyroid nodule, and are probably the consequence of suppressed TSH secretion, as TSH predominantly stimulates the synthesis of T3 and/or thyroidal T4 monodeiodination.

Thyroidal thyroxine and triiodothyronine in autonomously functioning thyroid nodule and paranodular tissue.

Thyroidal thyroxine (T4), triiodothyronine (T3), thyroglobulin (Tg) and T4/T3 ratio were investigated in nodular and paranodular tissue from 16 patients with autonomously functioning thyroid adenomas. The concentration of T4 and T3 in the nodule were 97.7 +/- 20.5 (Mean +/- SE) and 10.2 +/- 2.4 micrograms/g wet weight (w.w.). Both iodothyronines were significantly lower in paranodular tissue (22.6 +/- 4.8 and 1.45 +/- 0.32 micrograms/g w.w., respectively), but with disproportionately decreased T3 which resulted in T4/T3 ratio (25.4 +/- 6.4) higher than in adenoma tissue (11.2 +/- 1.6). In patients with high normal or supranormal serum T3 concentration, thyroidal concentration of T3 in adenoma tissue was higher (15.6 +/- 5.0 vs. 6.3 +/- 1.2 micrograms/g w.w.) and T4/T3 ratio lower (8.05 +/- 2.1 vs. 13.2 +/- 1.9) than in patients with normal serum T3. The results suggest that thyroid release of T3 from adenoma is relatively higher than T4 in patients with autonomously functioning thyroid nodules and increased peripheral T3.

[Successful decompression of the facial nerve in facial spasm (author's transl)]. / Erfolgreiche Dekompression des N. facialis beim Gesichtskrampf

A case of successful surgical treatment of facial spasm by seventh nerve decompression is reported in a female patient who failed to improve by usual methods of treatment. Prior to surgery, tomography of the facial canal was obtained, and revealed an osseous spine involving the posterior wall of the canal. During surgery, this spine was uncovered and removed with pneumatized retrofacial air cells. The patient's spasm disappeared completely following surgery. The present case indicates that the possibility of osseous spines impinging on the facial nerve should be considered in all cases of facial spasm.