Centripetal Propagation of Vasoconstriction at the Time of Headache Resolution in Patients with Reversible Cerebral Vasoconstriction Syndrome.

BACKGROUND AND PURPOSE: Reversible cerebral vasoconstriction syndrome is characterized by thunderclap headache and diffuse segmental vasoconstriction that resolves spontaneously within 3 months. Previous reports have proposed that vasoconstriction first involves small distal arteries and then progresses toward major vessels at the time of thunderclap headache remission. The purpose of this study was to confirm centripetal propagation of vasoconstriction on MRA at the time of thunderclap headache remission compared with MRA at the time of reversible cerebral vasoconstriction syndrome onset. MATERIALS AND METHODS: Of the 39 patients diagnosed with reversible cerebral vasoconstriction syndrome at our hospital during the study period, participants comprised the 16 patients who underwent MR imaging, including MRA, within 72 hours of reversible cerebral vasoconstriction syndrome onset (initial MRA) and within 48 hours of thunderclap headache remission. RESULTS: In 14 of the 16 patients (87.5%), centripetal propagation of vasoconstriction occurred from the initial MRA to remission of thunderclap headache, with typical segmental vasoconstriction of major vessels. These mainly involved the M1 portion of the MCA (10 cases), P1 portion of the posterior cerebral artery (10 cases), and A1 portion of the anterior cerebral artery (5 cases). CONCLUSIONS: This study found evidence of centripetal propagation of vasoconstriction on MRA obtained at the time of thunderclap headache remission, compared with MRA obtained at the time of reversible cerebral vasoconstriction syndrome onset. If clinicians remain unsure of the diagnosis during early-stage reversible cerebral vasoconstriction syndrome, this time point represents the best opportunity to diagnose reversible cerebral vasoconstriction syndrome with confidence.

Inhibition of soybean lipoxygenase-1 by n-alcohols and n-alkylthiols.

A series of n-alcohols and n-alkylthiols with carbon chains from 2 to 12 were examined for the inhibition of soybean lipoxygenase-1 (L-1). The alcohol produces a competitive inhibition, the extent of which increases with an increase in the carbon number of alkyl chain up to 8. Whereas the inhibition of the alkylthiol is noncompetitive, the extent of which is almost independent from the carbon number. From the behavior of pKi dependence on the carbon number of the alcohol, the decyl group appears to be optimum to bind to L-1. The thermodynamic analysis for the inhibition based upon van 't Hoff equation indicates positive enthalpy and entropy changes for the binding of the alcohol to the enzyme and negative enthalpy and positive to negative entropy changes for that of the alkylthiol. These observations suggest that the alcohol inhibits L-1 by binding of the hydrophobic alkyl tail to the catalytic site of the enzyme by a hydrophobic interaction. The alkylthiol inhibits by binding of the nucleophilic sulfhydryl head to a polarizable region of the enzyme and the alkyl tail to a hydrophobic region of the enzyme free from the steric hindrance as an anchor.

Neuroradiologic Diagnosis of Minor Leak prior to Major SAH: Diagnosis by T1-FLAIR Mismatch.

BACKGROUND AND PURPOSE: In major SAH, the only method to diagnose a preceding minor leak is to ascertain the presence of a warning headache by interview; however, poor clinical condition and recall bias can cause inaccuracy. We devised a neuroradiologic method to diagnose previous minor leak in patients with SAH and attempted to determine whether warning (sentinel) headaches were associated with minor leaks before major SAH. MATERIALS AND METHODS: We retrospectively evaluated 127 patients who were admitted with SAH within 48 hours of ictus. Previous minor leak before major SAH was defined as T1WI-detected clearly bright hyperintense subarachnoid blood accompanied by SAH blood on FLAIR images that was distributed over a larger area than bright hyperintense subarachnoid blood on T1WI (T1-FLAIR mismatch). RESULTS: The incidence of warning headache before SAH was 11.0% (14 of 127 patients, determined by interview). The incidence of T1-FLAIR mismatch (neuroradiologic diagnosis of minor leak before major SAH) was 33.9% (43 of 127 patients). Of the 14 patients with warning headache, 13 had a minor leak diagnosed by T1-FLAIR mismatch at the time of admission. Variables identified by multivariate analysis as significantly associated with minor leak diagnosed by T1-FLAIR mismatch included 80 years of age or older, rebleeding after admission, intracerebral hemorrhage on CT, and mRS scores of 3-6. CONCLUSIONS: We conclude that warning headaches diagnosed by interview are not a product of recall bias but are the result of actual leaks from aneurysms.

Juxtaglomerular cell tumor with retroperitoneal fibrosis and secondary immune-complex glomerulonephritis: a possible contribution of the renin angiotensin system to renal fibrosis.

We present a case of a 25-year-old woman with a renin-secreting juxtaglomerular cell tumor, retroperitoneal fibrosis associated with glomerular hypertrophy, glomerulonephritis, and marked tubulointerstitial alterations. Myofibroblasts, as shown by positive immunostaining for alpha-smooth muscle actin, were found along with transforming growth factor-beta (TGF-beta) in the interstitium of the tumor-free kidney. Regarding the pathogenesis of renal fibrosis and glomerular hypertrophy, this case may provide evidence not only experimentally but also clinically that the renin-angiotensin system plays an important role because angiotensin II is known to induce renal fibrosis associated with increased TGF-beta and the appearance of myofibroblasts.

Reactivity of soybean lipoxygenase-1 to linoleic acid entrapped in phosphatidylcholine vesicles.

The linoleic acids embedded in the SUVs of soy-PC, DMPC, and DPPC served as substrate for soybean lipoxygenase-1 (L-1). The initial velocity of the catalytic reaction and the concentration of the substrate showed a hyperbolic relation. The Km values of L-1 for the linoleic acids in soy-PC, DMPC, and DPPC vesicles were 0.07, 0.09, and 0.11 mM, respectively, being comparable with that for Tween-20 micellar linoleic acid. Soy-PC and DMPC competitively inhibited the enzyme with Ki values of 0.20 and 0.13 mM, respectively, whereas DPPC had no effect. DSC analysis revealed the phase separation of linoleic acid and DPPC in vesicles in the temperature range in which the enzyme reaction was carried out. This may account for the lack of inhibitory effect of DPPC on the enzyme. From the temperature dependence of the specific activity of the enzyme, the Ea values of the catalytic reaction were estimated to be 26.7 and 35.3 kJ.mol-1 for soy-PC and DPPC vesicles, respectively. For linoleic acid-DMPC vesicles, a two-phase temperature dependence of the activity across the transition temperature of the mixed vesicles was suggested.

Asymptomatic acute poststreptococcal glomerulonephritis following upper respiratory tract infections caused by Group A streptococci.

During an observation period of 1-2 years in 2 different districts in Japan, 104 patients were found to have upper respiratory infections caused by group A streptococci. Fourty-nine of these patients were followed prospectively to determine if renal involvement would occur. Twelve patients developed transient serum complement (CH50) depression and urinary abnormality, and 2 of these developed mild hypertension. The latent period was from 1-8 weeks after the streptococcal infection. Renal biopsies of the 12 patients with "asymptomatic" of "subclinical" acute poststreptococcal glomerulonephritis (APSGN) were examined by light, immunofluorescent and electron microscopy. Glomerular lesions ranged from mild proliferative changes to the classical pathology seen in APSGN. The 12 patients were followed for 10 years. Two of them developed persistent or intermittent hematuria, and renal biopsies obtained 4 years after the initial infection revealed mesangial proliferative glomerulonephritis without IgA deposits. The remaining patients showed no abnormal findings after the acute episode. These findings suggested that glomerular involvement after group A streptococcal infection is frequent and mesangial proliferative glomerulonephritis, which was found to develop in some, may rank with IgA nephropathy as a major cause of unexplained microscopic hematuria.

ANCA associated vasculitis allergica cutis (VAC) and mild proliferative necrotizing glomerulonephritis.

We report here the case of a 69-year-old male with vasculitis allergica cutis (VAC) accompanied by a nephrotic syndrome associated with serum perinuclear-staining anti-neutrophil cytoplasmic antibody (P-ANCA). Renal biopsy specimens showed mild proliferative glomerulonephritis with crescentic and necrotizing lesion in the glomeruli, while lesional skin biopsy specimens showed leukocytoclastic vasculitis in the deep dermis. Pauci-immune deposits were evident (deposits of immunoglobulins and complements were absent) by immunofluorescence microscopy in both tissues. The appearance of weakly positive P-ANCA associated with skin lesions and renal dysfunction occurred twice in this patient. Following corticosteroid administration, renal dysfunction was remarkably diminished and proteinuria decreased. P-ANCA titer were decreased, reaching the normal level. ANCA associated glomerulonephritis complicated by VAC has not been reported previously. In the present case, the vasculitis cutis and glomerulonephritis is assumed to be associated with the appearance of weakly positive P-ANCA.

Micro-determinations of alpha-tocopherol in tissue lipids.

It has been shown that the determination of small amounts of tocopheral in tissue lipids by methods that use, in sequence, saponification, extraction, TLC and colorimetric assay, is greatly influenced by the concentration of soap in the saponification mixture. Soaps appear to interfere with the recovery of tocopherols during the subsequent extraction step. Based on this knowledge a method is described that was used to measure as little as 15 to 20 ng of tocopherol in 3 to 4 mg of lipid obtained from rat retinas. Presumably the method would be applicable to other tissues as well. The results show that the rat retina is readily depleted of tocopherol if none is included in the diet.

Use of epidural anesthesia in non-cardiac surgery in two cases complicated by hypertrophic cardiomyopathy.

Two patients with hypertrophic cardiomyopathy (HCM) who underwent non-cardiac surgery were managed successfully using epidural anesthesia as the principal intraoperative anesthesia. Epidural block was induced by inserting an epidural catheter from the lumbar vertebrae toward the caudal side. Epidural anesthesia is usually avoided in HCM patients because of the risk of a decreased preload or afterload causing stenosis in the left ventricular outflow tract. However, in surgery that requires an anesthesia level at L1 or below, which has little effect on sympathetic nerves, epidural anesthesia that maintains a stabilized hemodynamic state appears to be an excellent option.

Valsalva maneuver prevents guide wire trouble associated with 22-gauge safe guide.

UNLABELLED: The Safe guide is a central venous puncture needle that serves as both a pilot needle and as an introducer. A guide wire can be inserted into a vein through the side port at the hub of the 22-gauge Safe guides needle initially inserted as a pilot needle. However, guide wire insertion may fail due to kinking or locking at the side port. Increasing airway pressure to 20 cm H2O by squeezing a respiratory bag during insertion of the guide wire together with venous puncture was attempted to determine if would decrease guide wire trouble. SUBJECTS AND METHODS: A total of 120 patients scheduled for central venous catheterization by right internal jugular puncture were divided into two groups. Patients in group-A (n = 60) were catheterized by the conventional method and those in group-B (n = 60) were catheterized by applying the Valsalva maneuver. Three observations were made: 1) Frequency of cases in which blood back-flow occurred during withdrawal only and not upon advancement of the puncture needle. 2) Frequency of cases in which kinking and/or locking of the guide wire occurred at the hub during its insertion. And 3) the occurrence of complications. RESULTS: 1) The patency of the vein was preserved and blood back-flow was obtained during advancement of the puncture needle in all cases in which the Valsalva maneuver was applied. 2) The incidence of kinking and/or locking during insertion of the guide wire decreased from 16.7% to 3.4% by applying positive airway pressure during the Valsalva maneuver. And 3) complications were negligible. Additionally, the application of the Valsalva maneuver allowed successful guide wire insertion in 6 out of 9 cases (67%) in group-A, in which the initial attempt using the conventional method had failed. CONCLUSION: The application of positive airway pressure using the Valsalva maneuver may prevent the guide wire trouble associated with the 22-gauge Safe guide.

Modification of guide wire for 22-gauge safe guide.

Safe guide is a central venous catheterization kit that serves as both pilot needle and introducer. With a single puncture, a guide wire can be introduced by inserting it through the side port of the 22-gauge needle. The advantage is that this needle can be placed within a blood vessel using no more force than is required to insert a pilot needle. However, the 0.018-inch guide wire is vulnerable to kinks and locking. Because the tip has been shaped into a sharp J-shaped angle, it can kink at the puncture site, and locking sometimes occurs when the guide wire is passed through the side port of the needle, or when the dilator is introduced. In order to resolve these issues, we modified the device by making an experimental guide wire with a gentler angle. In addition, we fortified the body of the wire without altering its thickness. We then investigated the effectiveness of our modifications. The subjects of the study were 120 patients, who required central venous catheterization. They were divided into 2 groups. The original J-type guide wire was used in one group (Group A: n = 60) and the modified guide wire in the other group (Group B: n = 60). Catheters were introduced by right internal jugular vein puncture. We observed the following: 1) incidence of back-flow appearing at withdrawal of the needle without back-flow during advancement, 2) incidence of kinking or locking of the guide wire when it was passed through the side port, 3) incidence of kinking of the guide wire at the puncture site when introducing the dilator, and 4) complications. The results were as follows: 1) back-flow appeared upon withdrawal in 3.4% of both groups; 2) kinking and locking occurred when passing the guide wire through the side port of the Safe guide needle in 16.7% of Group A and 1.7% of Group B; 3) kinking of the guide wire occurred when introducing the dilator in 5 % of Group A in contrast to 0% in Group B; 4) the only complication caused by the passing of the guide wire was accidental puncture of the common carotid artery, which occurred in 1.7% of both groups. No problems with the guide wire were noted in either group. The use of our modified guide wire decreased the incidence of kinking and locking of the guide wire when passing it through the side port. In addition, no guide wire kinking at the puncture site occurred when introducing the dilator. Issues associated with the original J-type guide wire were resolved by 1) changing the guide wire tip to a gentler angle, and 2) fortifying the guide wire by altering its composition.

[A better method to attach an endotracheal tube to the stylet of the Bullard laryngoscope].

The Bullard laryngoscope with its introducing stylet is useful in a variety of patients with airway problems, but it poses difficulties in some cases where an endotracheal tube (ETT) catches on the ary-epiglottic fold and cannot be advanced into the trachea. This difficult may be avoided by slightly angulating the tip of the ETT so that it is directed in a better alignment toward the rima glottis. The efficacy of the two methods of angulation was studied. One hundred and eight patients were randomly assigned to one of three groups. In group 1 (control, 36 patients), the ETT was attached naturally to the stylet with the bevel facing left. In group 2 (36 patients), the ETT was hooked on the distal end of the stylet at the Murphy eye, as recommended by the manufacturer. In group 3 (36 patients), the ETT was set over the stylet after being rotated 180 degrees so that the bevel was directed to the right. Intubation on the first attempt was successful in 56% of group 1, 83% in group 2 and 100% in group 3. The patients in groups 1 & 2 in whom first attempt failed were all successfully intubated on the second trial with the method used in group 3. This method (180 degrees rotation of the ETT on the stylet), is applicable to any ETT with or without the Murphy eye.

[Evaluation of the bite block wedged between the maxillary and mandibular molars].

The purpose of this study was to compare our original bite block (T-X Block) wedged between the maxillary and mandibular molars, with the standard gum bite block, in 200 patients whose tracheas were intubated. During emergence from isoflurane anesthesia, no trouble occurred in T-X Block group (n = 100). On the other hand, lip damage and ejection of the bite block were found in 11 and 10 cases, respectively, in gum bite block group (n = 100). As another study, an opening between the maxillary and mandibular incisor edges was measured with T-X Block placed in twenty patients under general anesthesia. The inter-incisal distances in one way of using it as a smaller wedge and in the other way as a bigger one were 21.6 +/- 2.4 and 25.2 +/- 2.6 mm, respectively. Those values were significantly larger than thickness of the gum bite block. T-X Block is very useful because its use causes no complications and makes it easier to insert a naso-gastric tube as well as to clean the oral cavity with suction by giving a larger opening of the mouth.

[Optimal length of nasopharyngeal airway and its correlation with height and body weight].

We studied 26 patients (11 males and 15 females) undergoing elective surgery under general anesthesia. The purpose of this study was to decide how long the length of nasopharyngeal airway should be by measuring distance A (permitting airway obstruction to be released), distance B (giving the most effective ventilation), and distance C (between nostril and arytenoid). The values of distance A in male group and female group were 12.73 +/- 0.85 cm and 11.70 +/- 0.75 cm, respectively. The values of distance B were 14.55 +/- 0.96 cm in male group and 13.93 +/- 1.12 cm in female group. The values of distance C were 18.84 +/- 0.90 cm in male group and 17.40 +/- 0.97 cm in female group. This showed that it is necessary to advance the nasopharyngeal airway about 2 cm from the distance A to give the most effective ventilation to the patients with airway obstruction. Therefore, most of standard nasopharyngeal airways commercially available are too short. In addition, the distance B has no correlation with height and body weight and it is difficult to predict the optimal length of the airway.

[A case of extra-adrenal pheochromocytoma with frostbite in extremities].

We describe a patient who presented frostbite in extremities in addition to characteristic symptoms, such as severe hypertension, sweating attacks, palpitations and headache. The patient was eventually diagnosed as having single extra-adrenal pheochromocytoma. The frostbite in extremities rapidly resolved after the removal of the tumor as well as other characteristic clinical symptoms. It is speculated that this frostbite might have been induced by severe continuous constriction of peripheral artery and loss of heat by frequent sweating attacks. Regarding cutaneous symptoms in this disease, pallor, acrocyanosis and cold extremities are commonly found. However, it seems that typical frostbite associated with pheochromocytoma has not been reported so far.

[PGE1 and dopamine administration in a pediatric patient during hepatectomy].

A 2-year-old male underwent medial inferior hepatectomy for the treatment of metastatic tumors. Due to congenital hepatoblastoma, at 6-months of age, right lobectomy of his liver had been performed. To protect liver functions, PGE1 and dopamine were administered during the surgery. Although half of his circulating blood volume was lost, his perioperative hemodynamics was stable, with no development of postoperative liver dysfunction. PGE1 and dopamine may thus be beneficial in pediatric hepatectomy.

[Identification and analysis of immune cells infiltrating into the glomerulus and interstitium in lupus nephritis].

This study was performed to investigate the role of cell-mediated immunity in lupus nephritis (LN). Frozen sections from 38 patients with LN were examined by indirect immunoalkaline-phosphatase labeling using monoclonal antibodies to identify the immune cells infiltrating into the interstitium and glomerulus. 14 patients showed minor glomerular abnormality (MGA), 9 had mesangial LN (MesLN), 12 had diffuse proliferative LN (DPLN) and 3 had membranous LN (MLN). Monocyte/macrophage and helper/inducer T cells infiltrated in the interstitium predominantly, but intraglomerular infiltration of these cells was rare. Monocyte/macrophage and suppressor/cytotoxic T cell levels were significantly higher (p < 0.05) in the interstitium in DPLN patients and monocyte/macrophage level was significantly higher (p < 0.05) in MesLN patients than in MGA patients. In the interstitium, serum creatinine level was highly correlated with infiltrations of suppressor/cytotoxic T cell, monocyte/macrophage (p < 0.01), pan T cell and total leucocyte (p < 0.05). Clinical activity score was correlated with suppressor/cytotoxic T cell (p < 0.001), monocyte/macrophage and pan T cell (p < 0.01). These results suggest that suppressor/cytotoxic T cell and and monocyte/macrophage may play an important role in the progression of lupus nephritis.

Pathogenic GLUT9 mutations causing renal hypouricemia type 2 (RHUC2).

Renal hypouricemia (MIM 220150) is an inherited disorder characterized by low serum uric acid levels and has severe complications such as exercise-induced acute renal failure and urolithiasis. We have previously reported that URAT1/SLC22A12 encodes a renal urate-anion exchanger and that its mutations cause renal hypouricemia type 1 (RHUC1). With the large health-examination database of the Japan Maritime Self-Defense Force, we found two missense mutations (R198C and R380W) of GLUT9/SLC2A9 in hypouricemia patients. R198C and R380W occur in highly conserved amino acid motifs in the "sugar transport proteins signatures" that are observed in GLUT family transporters. The corresponding mutations in GLUT1 (R153C and R333W) are known to cause GLUT1 deficiency syndrome because arginine residues in this motif are reportedly important as the determinants of the membrane topology of human GLUT1. Therefore, on the basis of membrane topology, the same may be true of GLUT9. GLUT9 mutants showed markedly reduced urate transport in oocyte expression studies, which would be the result of the loss of positive charges in those conserved amino acid motifs. Together with previous reports on GLUT9 localization, our findings suggest that these GLUT9 mutations cause renal hypouricemia type 2 (RHUC2) by their decreased urate reabsorption on both sides of the renal proximal tubule cells. However, a previously reported GLUT9 mutation, P412R, was unlikely to be pathogenic. These findings also enable us to propose a physiological model of the renal urate reabsorption via GLUT9 and URAT1 and can lead to a promising therapeutic target for gout and related cardiovascular diseases.