Electron-phonon coupling enhancement and displacive magnetostructural transition in SrCr2 As2 under magneto-Raman spectroscopy.

A Raman spectroscopy study on high quality single crystals of SrCr2 As2 (SCA) in the temperature T range 4 K < T < 300 K and high applied magnetic fields up to H = 9 T is presented. The chromium B 1g phonon analysis reveals two anomalous shifts in the frequency, the first below T = 250 K at H = 0 T in the saturated AFM G-type order likely due to an enhanced electron-phonon coupling by the magnetic order, whereas the second anomaly occurs above H = 4 T at T = 4 K likely as a consequence of a magnetostructural displacive transition. Renormalization of the electronic Raman spectra in both studies reveals a decrease in the electronic density of states with decreasing T and increasing H, respectively, with consequent changes in the Fermi surface, which are intrinsically related to the observed anomalies.

Evidence of precursor orthorhombic domains well above the electronic nematic transition temperature in Sr(Fe1-x Co x )2As2.

Raman scattering, synchrotron x-ray diffraction, specific heat, resistivity and magnetic susceptibility measurements were performed in Sr(Fe1-x Co x )2As2 [[Formula: see text]] single crystals with superconducting critical temperature [Formula: see text] K and two additional transitions at 132 and 152 K observed in both specific heat and resistivity data. A quasielastic Raman signal with B 2g symmetry (tetragonal cell) associated with electronic nematic fluctuations is observed. Crucially, this signal shows maximum intensity at [Formula: see text] K, marking the nematic transition temperature. X-ray diffraction shows evidence of coexisting orthorhombic and tetragonal domains between [Formula: see text] and [Formula: see text] ∼ 152 K, implying that precursor orthorhombic domains emerge over an extended temperature range above [Formula: see text]. While the height of the quasielastic Raman peak is insensitive to [Formula: see text], the temperature-dependence of the average nematic fluctuation rate indicates a slowing down of the nematic fluctuations inside the precursor orthorhombic domains. These results are analogous to those previously reported for the LaFeAsO parent oxypnictide (Kaneko et al 2017 Phys. Rev. B 96 014506). We propose a scenario where the precursor orthorhombic phase may be generated within the electronically disordered regime ([Formula: see text]) as long as the nematic fluctuation rate is sufficiently small in comparison to the optical phonon frequency range. In this regime, the local atomic structure responds adiabatically to the electronic nematic fluctuations, creating a net of orthorhombic clusters that, albeit dynamical for [Formula: see text], may be sufficiently dense to sustain long-range phase coherence in a diffraction process up to [Formula: see text].

Spin-electron-phonon excitation in Re-based half-metallic double perovskites.

A remarkable hardening (~30 cm(-1)) of the normal mode of vibration associated with the symmetric stretching of the oxygen octahedra for the Ba(2)FeReO(6) and Sr(2)CrReO(6) double perovskites is observed below the corresponding magnetic ordering temperatures. The very large magnitude of this effect and its absence for the antisymmetric stretching mode provide evidence against a conventional spin-phonon coupling mechanism. Our observations are consistent with a collective excitation formed by the combination of the vibrational mode with oscillations of Fe or Cr 3d and Re 5d occupations and spin magnitudes.

Exonic deletion of CASP10 in a patient presenting with systemic juvenile idiopathic arthritis, but not with autoimmune lymphoproliferative syndrome type IIa.

Systemic juvenile idiopathic arthritis (s-JIA) is a rare inflammatory disease classified as a subtype of chronic childhood arthritis, manifested by spiking fever, erythematous skin rash, pericarditis and hepatosplenomegaly. The genetic background underlying s-JIA remains poorly defined. To detect copy number variations, we performed single nucleotide polymorphism (SNP) array analysis in 50 patients with s-JIA. We found a 13-kb intragenic deletion of CASP10 in one patient. RT-PCR of the mRNA extracted from the patient's lymphoblastoid cells revealed that CASP10 mRNA was truncated. Sequencing the mRNA revealed that this deletion resulted in a frame shift with an early stop codon. CASP10 is known as a causative gene for autoimmune lymphoproliferative syndrome (ALPS) type IIa, another childhood syndrome of lymphadenopathy and splenomegaly associated with autoimmune haemolytic anaemia and thrombocytopenia. TCR αß(+) CD4/CD8 double-negative T cells in the peripheral blood as a diagnostic marker of ALPS were not high in this patient and lymphocyte apoptosis induced by anti-Fas antibody was normal, denying ALPS in the patient. The father and a sister of the patient showing no symptoms of ALPS or s-JIA, also had the same deletion. Furthermore, we found no other mutations of CASP10 in the other 49 s-JIA patients. These data suggest that the pathogenic significance of CASP10 mutations should be carefully evaluated in s-JIA or even ALPS type IIa in further studies.

Expression of immunoglobulin E-dependent histamine-releasing factor in idiopathic nephrotic syndrome of childhood.

Concanavarin-A (conA)-stimulated peripheral blood mononuclear cells (PBMNC) from patients with idiopathic nephrotic syndrome (INS) produce putative factors that increase vascular permeability. These factors are expressed in the nephrotic phase but are reduced in the convalescent phase. To identify the genes that are expressed only in the nephrotic phase, we performed cDNA subtraction using conA-stimulated PBMNC from three patients with INS. We isolated several gene transcripts in all three subtracted cDNA libraries. Among these genes, IgE-dependent histamine-releasing factor (HRF) was overexpressed in the nephrotic phase not only at the mRNA level but also at the protein level in another 10 patients with INS. Moreover, we found increased secretion of HRF from conA-stimulated PBMNC in the nephrotic phase. The results suggest that HRF is involved in the pathogenesis of idiopathic nephrotic syndrome.

Contingent negative variation in children with orthostatic dysregulation.

BACKGROUND: Children with orthostatic dysregulation (OD) appear to have hypodynamia, as well as the symptoms described in the OD criteira. Hypodynamia, which is greatly influenced by motivation, volition and concentration, is unexceptionally recognized in their everyday life. It has been suggested that the symptoms and hypodynamia aggravate considerably the quality of life (QOL) of children with OD. The purpose of this study was to distinguish the characteristics of contingent negative variation (CNV) and post imperative negative variation, which may reflect the level of attention and motivation in children with OD. METHODS: Twelve patients with OD aged 10-15 years and 23 age-matched healthy children were included. The CNV was recorded from Fz, Cz and Pz linked to earlobes during 30 trials consisting of a warning stimulus and an imperative stimulus with an interstimulus interval (ISI) of 2 s and an intertrial interval (ITI) of 10 s. The imperative stimulus of each trial required a button to be pressed. RESULTS: The untreated children with OD did not have a significantly smaller CNV amplitude than healthy children. Children with OD treated with midodrine and autonomic training had a significantly larger CNV amplitude than the untreated children, in the area of early, late and total CNV at the three sites. CONCLUSION: The present study confirms that children with OD have diminished motivation and deterioration of concentration, which cause hypodynamia in everyday life. Treatment for OD improves the symptoms, diminished motivation and deterioration of concentration, consequently restoring dynamia. Treatment for OD should be recommended to ameliorate QOL of children with OD.

[Abscess of the abdominal wall following penetration of the descending colon: a rare complication of a ventriculoperitoneal shunt].

A case of abscess of the abdominal wall associated with a ventriculoperitoneal (V-P) shunt following penetration into the descending colon is reported. A 49-year-old male was diagnosed as having hypertensive intracerebellar hematoma with hydrocephalus, and he was treated by V-P shunt. One year later he experienced left hypochondrial pain with rebound tenderness during a period of one week. He was doing well until 4 years and 10 months after, when barium enema examination and colonoscopy incidentally demonstrated that the abdominal catheter had penetrated into the descending colon. However, he had no symptoms. He remained in good health during a period of observation. A year later he was readmitted to our department with an abscess of the abdominal wall. Plain radiograph of the abdomen demonstrated disconnection of the shunt catheter and it was thought that the abdominal catheter had been passed via the rectum. At operation a subcutaneous abscess was found and cultures grew proteus mirabilis. We discussed the mechanism of the developing of the abscess on the abdominal wall following penetration of the bowel. It is believed that abscess of the abdominal wall may occur due to spontaneous extraction of a previously inserted shunt catheter which has penetrated into the bowel.

Two cases of giant serpentine aneurysm.

Giant serpentine aneurysm (GSA) is an entity defined on radiological and pathological grounds as a giant, partially thrombosed aneurysm containing tortuous vascular channels. We have had the opportunity to study two patients with GSAs, which has allowed for a complete comparative anatomical and radiological study. This report emphasizes the etiology of the GSAs. Twenty-two patients with GSAs have been reported in the literature, of which pathological studies were done in 10. In most of these, the aneurysm was found to be filled with an organized thrombus, but in our patients the aneurysm was filled with relatively new clot. The aneurysm enlarged and a change in the tortuous vascular channel was observed over a period of 1 year in the first patient, whereas a globoid aneurysm developed into a GSA in the brief period of just 2 weeks in the second patient. This rapid transformation of a globoid aneurysm into a GSA is of particular interest when the etiology of GSAs is considered. Our patients therefore shed some interesting light on the possible pathophysiology of GSAs. That is, the bloodstream may change dynamically in a giant aneurysm and may become a serpentine channel under conditions that lead to a "Coanda effect."

[A case showing angiographically moyamoya vessels combined with intracranial calcification].

This case was 36-year-old female with the past history of pulmonary tuberculosis on her 10 years old. During the treatment for pulmonary tuberculosis, transient tetraparesis appeared, and improved completely. She was admitted to our hospital, complaining sensory disturbance of left face and arm on July 25, 1983. Plain skull roentogenogram showed calcification at the base of brain. Computerized tomography (CT scan) showed dilatation of lateral and third ventricles. Cerebral angiograms revealed an occlusion of the right internal carotid artery and the left middle cerebral artery, the stenosis of the basilar artery and bilateral posterior cerebral arteries, transdural anastomosis (so-called vault moyamoya), leptomeningeal anastomosis and moyamoya vessels at the base of brain. Four days after the admission, left hemiplegia appeared suddenly. Because the findings of CT scan and angiogram showed no change, conservative treatment was performed and motor disturbance improved. Biopsy of the calcified mass was carried out and histological findings suggested an old inflammation. Based on the clinical course and examination, it was considered that moyamoya vessels of this case had been formed as a result of tuberculous meningitis. Up to now, only 6 cases with moyamoya vessels at the base of brain coursed by tuberculous meningitis Up to now, only 6 cases with moyamoya vessels at the base of brain coursed by tuberculous meningitis have been reported. In our case, neurological symptoms appeared 26 years after tuberculous meningitis and occlusive changes were seen in bilateral carotid system and vertebrobasilar system.

[Giant optic glioma--case report (author's transl)].

A 3 1/2-year-old girl with a huge optic glioma was reported. On February 26, 1978, she was hospitalized for signs of increased intracranial pressure, namely headache, vomiting and consciousness disturbance. Before admission she did not complain of her visual disturbance. A huge mass lesion in the subfrontal-suprasellar region was found by neuroradiological examination. The operation was performed on March 7, 1978, and the tumor arising from the right optic nerve, about 170 grams in weight, was totally removed in piecemeals. Histopathological diagnosis was pilocytic astrocytoma. Immediately after operation diabetes insipidus and hypernatremia developed, but two months later these symptoms disappeared. Post-operative CT scan demonstrated no mass lesion in the subfrontal-suprasellar region. After radiation therapy, she was discharged with slight left hemiparesis on August 31, 1978. Though her right eye was blind, visual acuity remained 0.2 in the left eye. No other neurologic deficits could be found.

An analysis of follow-up results of 1000 intracranial saccular aneurysms with definitive surgical treatment.

The authors report a follow-up review of 1000 cases of intracranial definitive surgery for saccular aneurysms. The prognosis for such surgical cases is discussed. Postoperative results at least 6 months after discharge from the hospital were analyzed in 876 (93.3%) of the 939 surviving patients. The longest follow-up period was 14 years and 5 months, with an average of 3 years and 7 months. At the time of discharge, there were 543 excellent results, 186 good, 117 fair, 93 poor, and 61 deaths. The chief findings were as follows: 1) Most of the patients determined as "excellent" or "good" at discharge were able to return to normal life; most of the deaths or instances of worsened condition found in the follow-up study were due to new lesions. 2) Fully 62% of the cases determined as "fair" at discharge were found in an improved state at the follow-up study, having returned to normal life. 3) Only 19% of cases determined as "poor" at discharge had improved to the point where a return to normal life was possible, the majority having died or remaining in poor condition.

[Postmortem examination of patients with non-surgically treated ruptured aneurysms (author's transl)].

The is no doubt that radical surgery has been successful in preserving the lives of patients with ruptured aneurysms. On the other hand, no matter how successful we have been we cannot and must not forget the patients who died without undergoing surgery. Therefore, we investigated from clinical course, laboratory work, including angiography and autopsy, 38 cases who died during hospitalization in order to elucidate the causes of death in the natural course and to find the possibilities of minnimizing the number of non-operative fatalies. We classified the causes of death into 3 groups: intracranial hemmatomas, brain ischemia due to vasospasm, and aggravation of the general condition. From the study, it was concluded that most of the lives could have been saved, if appropriate diagnosis and treatment had been made as soon as possible after the initial hemorrhage.