Audit of donor centre: guidelines by the World Marrow Donor Association Quality and Regulation Working Group.

According to the Standards of the World Marrow Donor Association (WMDA) 2020 [1] unrelated stem cell donor registries are responsible for compliance of their donor centres with these Standards. To ensure high stem cell product quality and high standards for safety and satisfaction of voluntary unrelated stem cell donors, we present here guidelines for audits of donor centres (DC) that can be used by new and established donor registries. They have been developed for registries relying on independent national or international DCs for the recruitment and management of Unrelated Donors (UD) for verification typing (VT)/extended tying (ET), work up processes and Hemopoietic Progenitor Cell (HPC) donation.

Receptor tyrosine kinase EphA7 is required for interneuron connectivity at specific subcellular compartments of granule cells.

Neuronal transmission is regulated by the local circuitry which is composed of principal neurons targeted at different subcellular compartments by a variety of interneurons. However, mechanisms that contribute to the subcellular localisation and maintenance of GABAergic interneuron terminals are poorly understood. Stabilization of GABAergic synapses depends on clustering of the postsynaptic scaffolding protein gephyrin and its interaction with the guanine nucleotide exchange factor collybistin.

Organisation and control of neuronal connectivity and myelination by cell adhesion molecule neurofascin.

The neuronal cell adhesion molecule neurofascin is expressed in highly complex temporally and spatially regulated patterns. Accordingly, many different functions have been described including control of neurite outgrowth, clustering of protein complexes at the axon initial segments as well as at the nodes of Ranvier and axoglial contact formation at paranodal segments. At the molecular level, neurofascin provides a link between extracellular interactions of many different interaction partners and cytoskeletal components or signal transduction.

A comprehensive small interfering RNA screen identifies signaling pathways required for gephyrin clustering.

The postsynaptic scaffold protein gephyrin is clustered at inhibitory synapses and serves for the stabilization of GABA(A) receptors. Here, a comprehensive kinome-wide siRNA screen in a human HeLa cell-based model for gephyrin clustering was used to identify candidate protein kinases implicated in the stabilization of gephyrin clusters. As a result, 12 hits were identified including FGFR1 (FGF receptor 1), TrkB, and TrkC as well as components of the MAPK and mammalian target of rapamycin (mTOR) pathways.

Neurofascin: a switch between neuronal plasticity and stability.

Neurofascin (NF) is a cell surface protein belonging to the immunoglobulin superfamily (IgSF). Different polypeptides of 186, 180, 166 and 155 kDa are generated by alternative splicing. Expression of these isoforms is temporally and spatially regulated and can be roughly grouped into embryonic, adult and glial expression.

[Spontaneous occurrence of a factor VIII inhibitor in glomerulonephritis and immunoglobulin a deficiency].

A 24-year-old patient with isolated IgA deficiency and a 3-year history of minimal change glomerulonephritis with nephrotic syndrome developed acute hemorrhagic diathesis. A spontaneous inhibitor of factor VIII was diagnosed. Therapy with substitution and plasmapheresis was without prolonged effect.

[Clinical picture, differential diagnosis, and conservative therapy of biliary obstruction (author's transl)].

The history, clinical picture, and laboratory values are clues in the diagnosis of biliary obstruction. The next diagnostic procedure for the differentiation between intra- and extrahepatic obstruction is an ultrasound study. Bile duct dilatation, cholelithiasis, hydrops, and empyema can be detected quickly and reliably.

[General amyloidosis associated with Hashimoto-hypothyroidism (author's transl)].

We are reporting a case of perireticular as well as pericollageneous amyloidosis developing in a 65 year old patient with Hashimoto-hypothyroidism. The data presented suggest the presence of two primary, not interrelated disorders. Amyloid deposits were found in liver, rectum and bone marrow biopsies.

[Syndrom of hypercalcemia in a case of hyperthyroidism, primary hyperparathyroidism and cancer of the gall-bladder (author's transl)].

A hypercalcemia syndrom in the case of a 63 year old female patient is reported on, who simultaneously suffered from hyperthyroidism and suspected primary hyperparathyroidism. During autopsy an ectopic (ventral mediastinum) parathyroid adenoma and a gall bladder carcinoma were discovered. Each of these three diseases can induce hypercalcemia by themselves.

Studies on steroids in urine of the male newborn.

Four different methods of isolation and purification were utilized to study steroids in urine of male newborns which was collected during the first 5 days of life. These methods included celite column, ion exchange column and thin-layer chromatography, solvolysis and enzyme hydrolysis with beta-glucuronidase and aryl sulfatase. Procedural losses were evaluated by using radioactive internal standards.