Effects of superficial temporal artery to middle cerebral artery bypass on postoperative infarction rates among young children (≤ 5 years old) with moyamoya disease.

Revascularization surgery for young children with moyamoya disease (MMD) is challenging. Although indirect revascularization is preferred because of the technical difficulty in direct anastomosis, higher risks of postoperative infarction remain a problem. We aimed to investigate the effect of superficial temporal artery to middle cerebral artery (STA-MCA) bypass on postoperative outcomes during the acute postoperative period in young children ≤ 5 years old with MMD.

Long-Term Effects on Preventing Stroke after Endovascular Treatment or Bypass Surgery for Intracranial Arterial Stenosis.

Background: Intracranial arterial stenosis (ICAS) is an important cause of ischemic stroke worldwide due to its higher risk of recurrence with medical therapy. Although some large randomized studies failed to show the superiority of surgical treatment compared with medical therapy, the results of medical therapy are not sufficient. There are patients who still benefit from surgical treatment.

Tumor Volume Decrease via Feeder Occlusion for Treating a Large, Firm Trigone Meningioma.

Trigone meningiomas are considered a surgical challenge, as they tend to be considerably large and hypervascularized at the time of presentation. We experienced a case of a large and very hard trigone meningioma that was effectively treated using initial microsurgical feeder occlusion followed by surgery in stages. A 19-year-old woman who presented with loss of consciousness was referred to our hospital for surgical treatment of a brain tumor.

Development of Pyriform Sinus Suctioning Programs for Aspiration Pneumonia Prevention During the Acute Stroke.

Aspiration due to dysphagia is a factor associated with pneumonia during acute stroke. In such cases, it is likely that secretions in the pyriform sinuses enter the laryngeal inlet. The present study was based on the idea that it is possible to reduce aspiration pneumonia by periodically suctioning and removing such secretions (pyriform sinus suctioning), a study was conducted in a single facility.

Ruptured partially thrombosed anterior inferior cerebellar artery aneurysms: two case reports and review of literature.

Aneurysms arising from the distal anterior inferior cerebellar artery (AICA) are very rare. When the parent artery is an AICA-posterior inferior cerebellar artery (PICA) variant, occlusion of the artery, even distal to the meatal loop, leads to a significant area of cerebellar infarction. We report two cases of ruptured partially thrombosed distal AICA aneurysms.

Fungal Internal Carotid Artery Aneurysm Treated by Trapping and High-Flow Bypass: A Case Report and Literature Review.

We report a case of unruptured fungal internal carotid artery (ICA) aneurysm and review the pertinent literature. A 79-year-old man presented with decreased visual acuity on the right side, and he was diagnosed with retrobulbar optic neuritis. Medical treatment with steroids resulted in Aspergillus meningoencephalitis spreading to the bottom of bilateral frontal lobes, caused by an intracranial extension of sphenoid sinusitis.

Outcomes in two Japanese adenosine deaminase-deficiency patients treated by stem cell gene therapy with no cytoreductive conditioning.

Objective: We here describe treatment outcomes in two adenosine deaminase (ADA)-deficiency patients (pt) who received stem cell gene therapy (SCGT) with no cytoreductive conditioning. As this protocol has features distinct from those of other clinical trials, its results provide insights into SCGT for ADA deficiency.

Patients And Methods: Pt 1 was treated at age 4.

Determination of the deletion breakpoints in two patients with contiguous gene syndrome encompassing CYBB gene.

X-linked chronic granulomatous disease is a primary immunodeficiency caused by mutations in CYBB. Although large deletions involving CYBB are known to cause contiguous gene syndrome (CGS), only a few patients have been studied precisely at the molecular levels. Our study determined the deletion breakpoints in two patients with CGS involving CYBB by array comparative genomic hybridization and the following PCR and DNA walking studies.

A 5-year-old boy with unicentric Castleman disease affecting the mesentery: utility of serum IL-6 level and (18)F-FDG PET for diagnosis.

Castleman disease (CD) is a rare lymphoproliferative disorder of unknown etiology. It is quite difficult to diagnose CD without typical localized signs or symptoms. We present a 5-year-old boy with unicentric plasma cell CD in the mesentery, which was too small to be detected by any conventional imaging.

Stem cell transplantation in primary immunodeficiency disease patients.

Background: Primary immunodeficiency diseases (PID) are rare but have a high associated risk of death from overwhelming infection in early childhood. Stem cell transplantation (SCT) can be curative for PID, but standardized protocols for each disease have not yet been established.

Methods: Between May 1995 and May 2005, nine patients diagnosed with a PID received SCT at the Department of Pediatrics, Hokkaido University Hospital.

Epstein-Barr virus-associated B-cell lymphoma in a patient with DNA ligase IV (LIG4) syndrome.

A 14-year-old Japanese girl with a progressing combined immunodeficiency had developed non-Hodgkin's diffuse large B cell lymphoma. Her molecular analysis showed a compound heterozygote of novel mutations in the LIG4 gene, M249V substitution and a five nucleotides deletion from nucleotide position 1,270-1,274. She had also a set of characteristic clinical features of LIG4 syndrome.

Epitope mapping of anti-alpha-fodrin autoantibody in juvenile Sjögren's syndrome: difference in major epitopes between primary and secondary cases.

Objective: Juvenile Sjögren's syndrome (SS) is an early-onset type of SS. Autoantibody against the N-terminal 120 kDa form of a-fodrin is a specific and sensitive disease marker for both juvenile and adult SS. We investigated the initial and major determinants of a-fodrin in SS.