Five patients with common variable immunodeficiency treated in our hospital between December 1979 and December 1990 were given six kinds of intravenous immunoglobulin preparations (pepsin treated, S-sulfonated, polyethylene glycol treated, pH4 treated, alkylated, and pH4.25 formulation preparation) for replacement therapy. Duration of the therapy ranged from 7.6 to 11 years. Incidences of fever and acute infections were variable among patients, but no significant differences were seen in the incidences among periods given each preparation. Three cases revealed abnormal pulmonary functions in tests. Adverse reactions were rarely seen in our study periods, and no severe reactions were observed. No significant differences were seen in incidences of adverse reactions. Postinfusion levels of serum complement slightly decreased from preinfusion levels. However, the decrease in complement was not related to any adverse reaction. No long-term complications such as transmission of hepatitis have been observed. Our data suggest that no obvious differences exist between the efficacy and safety of each IVIG preparation. Differences of efficacy of IVIG replacement therapy may be due to the variable pathophysiology of each patient.

Adolescent↗

PubMedDate not supplied

Effects of erucic acid therapy on Japanese patients with X-linked adrenoleukodystrophy.

Ten Japanese boys with childhood adrenoleukodystrophy (ALD), one adult patient with adrenomyeloneuropathy (AMN), and two presymptomatic ALD boys were treated with dietary erucic acid (C22:1) for more than 12 months; except in a case of childhood ALD patient who died 7 months after beginning erucic acid therapy. During erucic acid therapy, the serum levels of very long-chain fatty acid (VLCFA) (C24:0/C22:0) decreased within 1-2 months in all patients, and these levels in four of the patients decreased to the normal range. Neurological examination and MRI findings in all 10 of the childhood ALD patients showed progression of the disease while they were receiving the dietary therapy. However, the mean interval between the onset of awkward gait and a vegetative state in diet-treated patients was significantly longer than that in the untreated patients. One AMN patient showed slight improvement of spastic gait and lessened pain in the lower limbs due to spasticity. The two presymptomatic ALD boys remained intact on clinical examination and on MRI findings for 38 and 23 months, respectively, after starting the diet.

Adolescent↗

PubMedDate not supplied

Short latency somatosensory evoked potentials and 99mTc-HMPAO SPECT in a case of flunarizine-effective alternating hemiplegia in infancy.

Short latency somatosensory evoked potentials (SSEPs) and 99mTc-hexamethylpropylene amine oxime single photon emission computed tomography (99mTc-HMPAO SPECT) were examined in a patient with alternating hemiplegia in infancy (AHI) before and after flunarizine treatment. The low amplitude and elongation of the latency time at the C'4 level of the interictal SSEPs before treatment were ameliorated by flunarizine administration. The cerebral hypoperfusion in the right hemisphere observed on 99mTc-HMPAO SPECT before treatment improved, as seen by normal and symmetrical imaging, after flunarizine treatment. These observations suggest that AHI is caused by a functional disturbance in the cerebral area contralateral to the hemiplegia, and that SSEPs and 99mTc-HMPAO SPECT are useful for evaluating functional disturbance in the contralateral motoneurons and the effect of flunarizine treatment in AHI patients.

Evoked Potentials, Somatosensory↗

PubMedDate not supplied

Prenatal diagnosis of peroxisomal disorders. Biochemical and immunocytochemical studies on peroxisomes in human amniocytes.

Prenatal diagnoses of peroxisomal disorders, including peroxisome-deficient Zellweger syndrome, isolated deficiency of peroxisomal beta-oxidation enzyme and rhizomelic type chondrodysplasia punctata were investigated by means of the lignoceric acid oxidation assay, indirect immunofluorescence staining and pulse-chase experiments, using cultured amniocytes. Assessment of peroxisomal beta-oxidation activity by means of [1-14C]lignoceric acid oxidation is essential for the diagnosis of a single enzyme deficiency of peroxisomal beta-oxidation with detectable enzyme protein. For the diagnosis of Zellweger syndrome, the absence of peroxisomes was readily determined by immunofluorescence staining of only a few amniocytes. Evidence for abnormal processing of 3-ketoacyl-CoA thiolase leads to the diagnosis of rhizomelic chondrodysplasia punctata. All the fetuses were considered to be normal and the neonates were normal. Use of these methods requires only a small number of amniocytes and will facilitate the prenatal diagnosis of peroxisomal disorders.

Acetyl-CoA C-Acyltransferase↗

PubMedDate not supplied

p53-dependent hyperthermic enhancement of tumour growth inhibition by X-ray or carbon-ion beam irradiation.

To elucidate p53-dependency on combined treatment with radiation and hyperthermia, growth inhibition and apoptosis were analysed using transplantable human tumour. Human head and neck squamous cell carcinoma (HNSCC) cells carrying different p53 genes were transplanted into the thigh of nude mice. When the mean diameter of tumour reached 5-6 mm, the tumours were exposed to X-rays (2 Gy) or Carbon-ion (C-) beams (1 Gy) followed by heating at 42 degrees C for 20 min. Tumour growth inhibition was evaluated by measuring the diameters of tumour. The induction of apoptosis and accumulation of apoptosis-related proteins were also analysed by immunohistochemical staining. Synergistic enhancement of tumour growth inhibition by hyperthermia was observed in wild-type p53 tumours treated with X-rays or C-beams but not in mutant p53 tumours. The incidence of apoptotic cells and activated-caspase-3-positive cells after combined treatment with them were significantly high in wild-type p53 tumours compared with that in mutant p53 tumours. The hyperthermic enhancement of tumour growth inhibition by X-ray- or C-beam-irradiation was p53-dependent, suggesting that it might be highly correlated with p53-dependent apoptosis.

Animals↗

PubMedDate not supplied

Role of slice thickness in MR imaging of the internal auditory canal.

The relative efficacy of post-Gd-DTPA 5 and 3 mm axial T1-weighted images was compared in the detection of lesions in the internal auditory canal and cerebellopontine angle. One hundred twenty consecutive patients were prospectively evaluated with 5 mm axial T1-weighted slices. If these were negative or questionable. 3 mm axial slices were immediately obtained as the next sequence. Eighteen percent of cases were positive and in none of these was the 5 mm study normal. However, in 22 negative cases and two positive cases, a 3 mm study was necessary for confirmation. Five millimeter axial scanning post Gd-DTPA is recommended as the initial study for detection of masses in the internal auditory canal and cerebellopontine angle. Because this study requires fewer acquisitions than 3 mm sections and can be done satisfactorily on low and midfield systems, there are potential time- and cost-saving benefits to this approach.

Cerebellar Diseases↗

PubMedDate not supplied

Electromechanical effects of caroverine, a new slow-channel blockade, on the SA node cells of rabbit and atrial muscle fibers of rabbit and guinea pig.

The effects of caroverine on electrical activity of isolated rabbit sinoatrial (SA) node cells and atrial muscle fibers and on contractile force of atrial muscle preparations were examined. In spontaneously firing SA node cells, caroverine (1 X 10(-7) M-1 X 10(-5) M) decreased the action potential amplitude (APA) and the maximal rate of depolarization (MRD) in a concentration-dependent manner. However, the spontaneous firing cycle length of these cells was not prolonged significantly with the drug except at a high concentration. In constantly driven atrial muscle fibers, caroverine at the same concentration range shortened the 30% repolarization time coupled with a depression of the plateau phase of action potentials. The effects of caroverine on the developed tension (DT) of atrial muscle were compared with that of verapamil. The 50% effective dose (ED50) for inhibition on atrial DT was 1 X 10(-5) M for caroverine and 8 X 10(-8) M for verapamil. Caroverine as well as verapamil had a frequency-dependent inhibitory action on atrial DT, which indicates that both of the drugs have an influence on the kinetics of slow channel of cardiac fibers. In conclusion, it is suggested that caroverine has only a small negative inotropic effect while electrophysiological effects are similar to slow-channel blockers.

Action Potentials↗