Human-derived hair follicle stem cells and hydrogen sulfide on focal cerebral ischemia model: A comparative evaluation of radiologic, neurobehavioral and immunohistochemical results.

The present study investigated the effects of intracerebral human-derived hair follicle stem cells (HFBSCs), whether alone or in combination with hydrogen sulfide (H2S) in a rat model of focal cerebral ischemia. The rats were randomly assigned into 4 groups (n = 10): Control (phosphate buffered saline (PBS)), Group A (at 24 h post-middle cerebral artery occlusion(MCAo), stereotaxic intracerebral, 1,0 × 106, total 10 µL HFBSCs), Group B (3-14 d post-MCAo, intraperitoneal (i.p.), 25 µM/kg/day H2S), Group AB (HFBSCs + H2S). Cranial magnetic resonance images were recorded on postoperative 1st and 28th days. Three dimensional analysis was performed to calculate the infarct volumes. Rotarod and cylinder tests were performed after MCAo and finally all rats were euthanized by cardiac perfusion at 28 days after MCAo for immunohistochemical analysis. The reduction in infarct volumes of rats receiving HFBSC was significant. The cranial infarct volume on the postoperative 28th day was significantly higher in the group in which H2S was administered alone compared to the HFBSC alone group. All animals showed steadily improved spontaneous locomotor activity from day 7 post-MCAo on rotarod test, from day 1 on cylinder test, but showed no significant differences at all times. In all groups, the grading scores of CD34, CD5, CD11b and GFAP immunohistochemical markers did not differ significantly. In conclusion, intracerebral HFBSC treatment after 24 h of ischemic stroke may be an effective way to reduce the cranial infarct volume, whereas H2S treatment alone or in combination with HFBSC may not be sufficient for ischemic brain injury.

Assessment of the MRI and Behavioral Test Results in a Focal Cerebral Ischemia-Reperfusion Model in the Rat after Separate and Combined Use of Mouse-Derived Neural Progenitor Cells, Human-Derived Neural Progenitor Cells and Atorvastatin.

AIM: To assess the efficacy of Neural progenitor cell (NPC) transplantation in ischemic stroke, and to investigate whether atorvastatin enhances therapeutic potency of NPC after stroke. MATERIAL AND METHODS: The focal cerebral ischemia-reperfusion model was performed by transient occlusion of middle cerebral artery. Rats were assigned randomly to receive intracerebral transplantation of mouse NPC alone (mNPC), human NPC alone (hNPC), mouse NPC plus oral atorvastatin (mNPC+A), human NPC plus oral atorvastatin (hNPC+A), oral atorvastatin alone, or intracerebral Dulbecco"s Modified Eagle"s medium injection (control group). Adhesive removal, rotarod, cylinder tests, and magnetic resonance imaging (MRI) were used for assessment of rats during 4 weeks. After sacrification on 28th day, rats were investigated by immunofluorescent staining. RESULTS: The hNPC and mNPC groups showed significantly improved functional outcome and reduced infarct area ratio compared with the control group. The hNPC group had significantly better performance and lower infarct area ratio than the mNPC group. Addition of atorvastatin to stem cell therapy significantly improved functional outcome, although it did not affect the infarct area ratio on MRI. Anti-inflammatory response in the infarct area was higher in the mNPC group. NPC transplantation significantly reduced the amount of microglia and a significant increase in the amount of astrocytes. CD8a+ T lymphocyte and granzyme B activities were not detected in any of the subjects. CONCLUSION: Both hNPC and mNPC treatments significantly improved functional outcome, and reduced infarct area ratio after stroke. Atorvastatin enhanced the therapeutic potency of NPCs, including neurological improvement.

Coexistence of two rare genetic disorders: Kartagener syndrome and familial Mediterranean fever.

Primary ciliary dyskinesia (PCD) is a rare disease, predominantly inherited as an autosomal recessive, with ciliary dysfunction leading to impaired mucociliary clearance, chronic airway infection and inflammation. Situs inversus totalis occurs in ~50 % of PCD patients and it is known as Kartagener syndome. Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent attacks of fever and peritonitis, pleuritis, arthritis, or erysipelas-like skin disease. FMF is caused by mutations in the MEFV gene which is located on chromosome 16p13.3. p.M680I, p.M694 V, p.M694I, p.V726A on exon 10 and p.E148Q on exon 2 are the most common mutations among FMF patients and these constitute 85 % of all. Homozygosity of R202Q polymorphism is strongly associated with FMF. We would like to present a case of Kartagener syndrome accompanied by FMF with R202Q polymorphism. Our case is the first in the literature indicating the accidental coexistence of FMF and Kartagener syndrome.