Response of sympathetic nervous system activity to exercise in patients with congestive heart failure

To investigate the serial sympathetic nervous system response to exercise, plasma norepinephrine (NE) and epinephrine (E) concentrations were measured at rest, during each stage of treadmill exercise, and immediately and 5 minutes after exercise in 68 congestive heart failure (CHF) patients (NYHA functional class I 24, II 25, III 19) and 30 normal subjects. Circulatory responses of NYHA class II patients increased at early stages of exercise. Systolic blood pressure and double product at peak exercise were significantly lower in NYHA class III patients. Plasma NE response of NYHA class I patients was similar to that of normal subjects. However, plasma NE at rest, and during and after exercise were significantly higher in NYHA classes II and III patients than in normal subjects and NYHA class I patients (peak NE (pg ml-1); Normals: 547 +/- 37, I: 535 +/- 53, II: 867 +/- 87, III: 1033 +/- 157). There was no significant difference in plasma E levels among the four groups. NE response to exercise was augmented according to the severity of heart failure, which suggested compensatory activation of sympathetic nervous system activity. Circulatory responses were reduced in NYHA class III patients despite the exaggerated compensatory activation of the sympathetic nervous system. Blunted circulatory responses to increased NE concentration in NYHA class III patients might relate to a decreased cardiac responsiveness to sympathetic activity in severe CHF patients.     

A case of four coronary artery fistulae originating from three vessels associated with aneurysm

A case of an aneurysm associated with four coronary artery fistulaeoriginating from three vessels is reported. The patient, a 52-year-oldwoman, had chest heaviness and palpitations. Coronary arteriographyreveaed the four fistulae originating from three coronary vesselswith an aneurysm draining into the left ventricle and the mainpulmonary artery.The patient's symptoms were relieved afterfistulectomy and the aneurysmectomy, suggesting that a coronarysteal phenomenon through the fistulae was the cause of her symptoms.This case is of interest because of its rarity, since this isthe first case report of an aneurysm associated with four coronaryartery fistulae from three vessels.     

Donepezil increases regional cerebral blood flow in a case of Korsakoff syndrome

Cholinesterase inhibitors improve cognitive functions and regional cerebral blood flow (rCBF) in mild to moderate Alzheimer's disease (AD). Recently, the possibility has been suggested that in patients with dementia other than AD cholinesterase inhibitors are useful for the treatment of their cognitive impairment and behavioral symptoms. In the present study we report the effectiveness of donepezil for a patient with Korsakoff syndrome. The patient was a 65‐year‐old man who gradually showed disturbances in memory, attention and concentration. The etiology was considered to be alcoholism. He would indulge in drinking and repeatedly asked the same questions over and over. He also developed emotional fluttering and would not settle down. On admission, he showed a severe memory deficit, disorientation and momentary confabulation. It was obvious that the patient was inactive and not spontaneous. He also manifested disinhibited behaviors. The patient's confabulation gradually diminished in hospital. He had no focal neurological deficit. Cranial magnetic resonance imaging (MRI) did not reveal any abnormality. Single photon emission computed tomography (SPECT) with 99 m‐TC‐ hexamethyl propylamenamine oxide (HMPAO) indicated hypoperfusion in the left thalamus, left basal ganglia and bilateral medial temporal lobes. In order to improve his cognitive function, the patient was treated donepezil. An evaluation of the effects of donepezil on rCBF was performed with the images showing a remarkable improvement in cerebral perfusion in the right medial temporal, the left thalamus and basal ganglia. However, the patient's memory impairment, disorientation, disinhibition, and stereotypical behavior and utterance were not improved. As an explanation, it was speculated that his neural network might be damaged so severely that there was no room for improvement by the acetylcholinesterase inhibitors.     

Clinical and Experimental Studies on Teratogenic Mechanisms of Congenital Absence of Digits in Longitudinal Deficiencies*

ABSTRACT: In order to clarify teratogenic mechanisms of congenital absence of the digits in longitudinal deficiencies, ulnar deficiency, radial deficiency, central polydactyly, osseous syndactyly and cleft hand (central deficiency) were analyzed. Then, the same anomalies induced in rat fetuses with myleran were analyzed. In ulnar and radial deficiencies, the more severe was the degree of finger deficit, the more severe was the arrest of the forearm bone. The critical periods of these anomalies were earlier than those of other anomalies. Ulnar deficiencies were induced only in Gun: Wistar rats and radial deficiencies only in WKAH/Hkm rats. A genetic factor may influence the teratogenesis of ulnar and radial deficiencies. From histological analysis of tibial deficiency, it may be suggested that the cause of absence of digits in longitudinal deficiency is closely related to a deficit of mesenchymal cells. The analysis of skeletal changes of central polydactylies, osseous syndactylies and cleft hands suggested that there seemed to be the cleft hand formation process from osseous syndactylies and central polydactylies. These anomalies were often observed in both hands in various combinations. They could be induced by the same treatment at the same developmental stage in rats. The teratogenic mechanism of cleft hand seemed to be failure of induction of digital rays in the hand plate.     

ENDOTOXIN CLEARANCE BY EXCHANGE BLOOD TRANSFUSION IN SEPTIC SHOCK NEONATES

ABSTRACT. The role of exchange transfusion with fresh whole blood in septic shock infants was evaluated. Endotoxin was positive in 8 of 10 infants and completely removed from 6 by exchange transfusion. All 6 infants with negative endotoxins after exchange transfusions survived, but 2 with positive reactions died despite the exchange transfusion. Staphylococcus aureus was cultured in the blood from 2 infants with negative reactions and one of them survived. These findings suggested that the clearance of endotoxin has an important role in exchange transfusion among septic shock neonates.     

Solitary Epicranial Neurofibroma with Neurofibromatosis Type 1-Related Germline Mutation: Case Report

A 33-year-old male became aware of a painless soft mass in the left occipital region. His medical and family history were unremarkable for neurofibromatosis type 1 (NF1) or other genetic disorders. Physical examination showed no signs of NF1. Neurological and ophthalmological examinations found no abnormality. Cranial computed tomography showed an isodense mass located subcutaneously with irregular deformities in the adjacent occipital bone. Magnetic resonance (MR) imaging demonstrated that the lesion, 7.5 × 5.5 cm in diameter, was hypointense both on T₁- and T₂-weighted images and intensely enhanced after gadolinium infusion. The patient requested to remove the large mass. The subcutaneous tumor was well circumscribed, encapsulated, and less vascular, and resected en bloc. The histological diagnosis was neurofibroma without findings of cell atypia, whereas genomic exploration identified abnormal gains in NF1 gene, and resultant absence of neurofibromin, a protein coded on NF1 gene. Solitary neurofibromas in “clinically” non-NF1 patients may originate from the genomic changes in NF1 gene.