Ectopic ACTH-syndrome due to a neuroendocrine tumour of the appendix.

Ectopic ACTH production causes 10% of Cushing's syndromes. The diagnostic workup is difficult, can last more than 6 months (> 50% of cases), and the underlying tumour is still frequently not located (12%). Carcinoid tumours of the appendix are frequent and are revealed in 0.3% of patients undergoing routine appendectomy. However, neuroendocrine tumours of the appendix with ACTH production are an extremely rare entity. Here we report the case of a female patient with clinically overt Cushing's syndrome due to ectopic ACTH-production from a carcinoid tumour of the appendix. During the diagnostic workup, repeated endocrine tests, multiple different imaging modalities and frequent and lengthy hospitalisations were necessary. Wrongly, even a neurosurgical pituitary exploration was performed. After 12 months from the initial admission, the tumour was finally detected by an ¹8F-fluoro-L-dihydroxyphenylalanine (¹8FDOPA PET) and an appendectomy followed by right hemicolectomy were performed. The patient recovered rapidly and the symptoms from the hypercortisolism were no more present.In this case, we discuss the multitude of problems, which may delay the diagnosis and the pitfalls, that should be avoided in order to locate the tumour and to initiate adequate therapy as early as possible. Furthermore, our case demonstrates the complexity of diagnostic procedures, which demand most of the times a multidisciplinary approach. In this setting, regular follow-ups in short time intervals and the use of novel imaging techniques can finally cut the diagnostic "Gordian knot".

[Transition clinics in endocrinology and diabetology for the chronically ill in Germany. A recent inquiry]. / Ubergangssprechstunden für chronisch Kranke in Endokrinologie und Diabetologie in Deutschland. Eine aktuelle Erhebung.

BACKGROUND AND OBJECTIVES: Transferring adolescent patients with endocrine disorders from paediatric to adult care units depends on regional facilities with varying efficiency. A nationwide system of transition clinics in Germany is actually not available, despite demands by physicians and patients. PATIENTS AND METHODS: We conducted a survey for the 36 German university hospitals to examine the regional existence and characteristics of actual transition methods. In a second questionnaire patients were asked to report their individual transition periods. RESULTS: Despite physician's demand in the majority of hospitals, there are only eight transition clinics nationwide. On average 56 patients attended these clinics yearly. Most patients suffered from disorders of pituitary, thyroid or adrenal gland (each 76 %) and diabetes mellitus type 1 (82 %). The perception of teamwork between the paediatric and adult care units was better in hospitals with transition clinics. Moreover, there was more scientific cooperation in those hospitals. Attendance to medical care and patient's compliance was enhanced by establishing transition clinics in 82 % and 70 %, respectively. Questioning affected patients we found attendance to the paediatric team up to the age of twenty with substantial difficulties finding an adequate adult care unit. Patients were more contend with paediatric than adult quality of physicians care (mark 1.6 versus 2.4). 82 % of patients judged establishing transition clinics as "urgently needed". CONCLUSION: In conclusion there are transition clinics in one quarter of German university hospitals. A more widespread installation of transition clinics is medical reasonable and is demanded by patients.

Macrowell cultures identify a subpopulation of neonatal rat dorsal root ganglionic neurons displaying nerve growth factor independent survival.

Dorsal root ganglionic (DRG) neurons of the newborn rat in vitro die by apoptosis within 24-48 h unless nerve growth factor (NGF) is added. Using a novel cell culture system (macrowell), we identified a neuronal subpopulation displaying NGF-independent survival in vitro. Neurons were grown on glass coverslips at standard cell density in different volumes of defined medium (standard: 500 microl; macrowell: 10 ml). In standard culture, 40% of neurons survived in the presence of NGF whereas there was no survival under control conditions. In macrowell culture, however, about 15% of neurons survived even in the absence of NGF. Addition of NGF to these cultures increased survival up to 65%. Neurons surviving independent of NGF in macrowell culture were heterogeneous in size and were lacking the low-affinity NGF receptor.

Cell death of neonatal rat sensory neurons is prevented by culture at clonal density.

We studied the trophic requirements of neonatal rat sensory neurons in single neuron and standard culture (9400 neurons/cm2). In agreement with previous studies, we found that survival of sensory neurons in standard culture is strictly dependent on nerve growth factor (NGF). Contrary to this, however, no difference between NGF-treated and untreated cultures with respect to survival and neurite formation was evident when neurons were plated as single cells. Approximately 70% of singly-seeded sensory neurons survived for 2 days independent of supply with NGF. Survival of large, mid-sized, and small sensory neurons in single neuron culture demonstrated that this phenomenon is not confined to a neuronal subpopulation. We speculate that survival in single neuron culture is due to the absence or inactivation of yet unidentified cell death-inducing factors that in standard culture are overridden or suppressed by NGF.