[Ten Cases of Cryptotia Corrected using Ono's Technique]. / Zehn Fälle von Kryptotie, korrigiert mit der Ono Technik.

Cryptotia is a very rare condition of ear deformity found in Europe which is more common in Asian populations. The upper ear portion is hidden and fixed in a pocket of skin of the mastoid. Conservatively, this deformity can be treated by molding of the posterior sulcus starting early after birth. Various surgical procedures are described in the literature. We successfully operated on a total of 10 ears of 9 patients with the technique described by Ichiro Ono in 1995. Here, a triangular flap is raised above the ear to reconstruct the posterior sulcus together with a rhomboid-shaped flap in the anterior part of the ear in order to elongate the helix. Among all patients, regular contours and a stable and successful outcome was observed.

Recurrent cranial fibroblastic neoplasm in a male adolescent: case report and review of the literature.

Paediatric fibroblastic tumours are rare neoplasms, of which cranial fasciitis is the most common. We present a case of a male 7-year-old suffering from a cranial tumour preceded by a mild trauma. The tumour recurred despite radical resection within 8 months. Histologically, neither tumour could be classified as any published pathological entity. Both lesions were described as cellular fibroblastic neoplasms; in addition, the recurrent tumour featured a prominent myxoid matrix. In the 12 months following resection of the second tumour, no further disease recurrence has occurred.

Quantification of faults resulting from disregard of standardised facial photography.

Medical photography is an important part of documentary procedures in plastic surgery both for quality assurance and for protecting surgeons from possible medico-legal claims filed by patients. Photo documentary standards were established to make photos reliable and to enable comparisons between pre- and post-operative views of a patient, different operative procedures and between results achieved by different surgeons. Despite their obvious importance, especially in the documentation of subtle surgical procedures on the face such as rhinoplasty, photo documentary standards continue to be disregarded. In this article, we analyse and quantify faults caused by even small deviations from photo documentary standards. Photos of 10 healthy, young adults (n = 10) taken in accordance with established standards were compared to photos with the similar types of faults in positioning of the head (±10° deviation from the standard), camera angles (±10° deviation from standard) or mimic expression (slight smile). A computer-assisted facial analysis was performed and the effects of faulty photo documentation on the final result were calculated. The analysis showed, for instance, that a +10° fault in positioning of the head causes a mean shortening of the nasal dorsum by 32.34% (p<0.001) and of the front by 15.33% (p = 0.001), whereas the chin is elongated by 6.82% (p = 0.004). A -10° fault elongates the nasal dorsum by 11.93% (p<0.001) and shortens the upper lip by 22.50% (p = 0.007) and the chin by 13.67% (p = 0.001). Deviations from standardised camera angles lead to similar mistakes, but to a lesser extent. A mistake in mimic expression leads to a shortening of the upper lip by 23.20% (p = 0.006) and also to a clear cheek lift. We show that slight deviations from photo documentary standards result in dramatic faults in the final outcome.

Glucocorticoid-induced apoptosis and glucocorticoid resistance: molecular mechanisms and clinical relevance.

The ability of glucocorticoids (GC) to efficiently kill lymphoid cells has led to their inclusion in essentially all chemotherapy protocols for lymphoid malignancies. This review summarizes recent findings related to the molecular basis of GC-induced apoptosis and GC resistance, and discusses their potential clinical implications. Accumulating evidence suggests that GC may induce cell death via different pathways resulting in apoptotic or necrotic morphologies, depending on the availability/responsiveness of the apoptotic machinery. The former might result from regulation of typical apoptosis genes such as members of the Bcl-2 family, the latter from detrimental GC effects on essential cellular functions possibly perpetuated by GC receptor (GR) autoinduction. Although other possibilities exist, GC resistance might frequently result from defective GR expression, perhaps the most efficient means to target multiple antileukemic GC effects. Numerous novel drug combinations are currently being tested to prevent resistance and improve GC efficacy in the therapy of lymphoid malignancies.

Glucocorticoid receptor heterozygosity combined with lack of receptor auto-induction causes glucocorticoid resistance in Jurkat acute lymphoblastic leukemia cells.

Glucocorticoids (GC) induce apoptosis in malignant lymphoblasts, but the mechanism of this process as well as that of the clinically important GC resistance is unknown. We investigated GC resistance in Jurkat T-ALL cells in which ectopic GC receptor (GR) restores GC sensitivity, suggesting deficient GR expression. Jurkat cells expressed one wild-type and one mutated (R477H) GR allele. GR(R477H) ligand-binding-dependent nuclear import, as revealed by live-cell microscopy of YFP-tagged GR, was unaffected. Transactivation and transrepression were markedly impaired; however, GR(R477H) did not act in a dominant-negative manner, that is, did not prevent cell death, when introduced into a GC-sensitive cell line by retroviral gene transfer. Contrary to another GR heterozygous, but GC-sensitive, T-ALL model (CCRF-CEM), Jurkats expressed lower basal GR levels and did not auto-induce their GR, as revealed by 'real-time' RT-PCR and immunoblotting. Absent GR auto-induction could not be restored by transgenic GR and, hence, was not caused by reduced basal GR levels. Thus, inactivation of one GR gene results in haploinsufficiency if associated with lack of GR auto-induction.