Biomedical subjects
Publications and source records attributed to C Messina.
The Authors report a case of benign mucous pemphigoid. The review of the literature has confirmed the rarity of this disease. After having discussed acquired diagnostic data, the Authors show reasons to justify the endoscopic surgical treatment. Furthermore, they underline the importance of associated medical therapy as well as follow-ups which patients must periodically undergo.
The acute systemic blood pressure, electrocardiographic and renal function responses to an intravenous bolus infusion of diltiazem (0.2 to 0.5 mg/kg) were evaluated in 18 subjects with mild to moderate essential hypertension. Although a significant blood pressure response occurred within 5 minutes, blood pressure returned to pretreatment levels within 1 to 3 hours. After drug infusion, a variety of rhythm and conduction disturbances were noted; the most important were transient prolongation of the PR Interval (first-degree atrioventricular block), a single episode of second-degree atrioventricular block (Mobitz I) associated with T-wave inversion and a transient episode of junctional escape rhythm and atrioventricular dissociation. Intravenous diltiazem had no consistent effect on glomerular filtration rate or effective renal plasma flow. Natriuresis and kaliuresis were observed only at the highest infusion dose. It is concluded that an alternative dosing regimen will be required if intravenous diltiazem is to be used safely and effectively to control blood pressure in patients with hypertensive disease.
Four patients with botulism were studied on admission and at different times after intoxication, using a battery of cardiovascular autonomic tests. The results were compared with clinical status and single-fiber electromyographic findings. In the early stage of intoxication, the control of heart-rate and blood-pressure responsivity was markedly impaired, as was the neuromuscular transmission. At follow-up, results of sympathetic tests normalized earlier than those of parasympathetic tests. The recovery of autonomic function was slower than that of neuromuscular transmission in three patients. Monitoring autonomic derangement in botulism adds further information on the course of the disease and may identify patients at risk for cardiac or respiratory arrest. Further clinical investigation can help in determining more precisely the autonomic sites where the toxin acts.
15 cases of acute leukemia (AL) displaying a TdT+, HLA-DR+ phenotype were studied; surface immunoglobulins, T cell markers and the common acute lymphoblastic leukaemia (c-ALL) antigen were negative, as were peroxidase and non-specific esterase cytochemical reactions. All cases were extensively investigated by conventional immunofluorescence (IF) and immunoperoxidase (IP), with a panel of monoclonal antibodies (MoAb), using both light and electron microscopy, and for ultrastructural myeloperoxidase (MPO). 8 cases, which were OKB2+, BA1+, B4+, J5- and BA2- by IF, expressed the J5 antigen in IP. These cases were therefore re-classified as ALL with a weak expression of the C-ALL antigen. The other 7 cases showed an OKB2-, BA1-, B4+, BA2+ phenotype at IF and were also positive for 1 or more anti-myeloid MoAb. These features were confirmed by IP study. 4 patients also presented ultrastructural positivity to MPO. These cases were considered as proliferations of early precursor cells capable of expressing both myeloid and lymphoid features. This study, while demonstrating the heterogeneity of TdT+, HLA-DR+ AL, suggests that the cell origin of many cases may be defined by extensive immunotyping at both IF and IP level. The prognostic and therapeutic implications of these findings are discussed, also in view of the poor prognosis often observed in the more undifferentiated cases of AL.
To assess the relationship between aging and autonomic control of heart rate and blood pressure, cardiovascular reflex tests were performed in 70 healthy volunteers in the age range 25-71 years. R-R interval variation, heart rate change with deep breathing, 30/15 ratio and blood pressure response to standing appeared significantly declining with age. For each test we calculated the P0.99 and P0.01 confidence limits for individual observations. On the other hand, Valsalva ratio and the blood pressure response to sustained handgrip appeared to be unrelated to age. These results suggest that there is an age-dependent degradation of the mechanisms involved in the cardiovascular reflexes. The assessment of age-adjusted normal values improves the criteria for delineating abnormal from normal results in individual testing of autonomic function.
Patients with primary immunodeficiency disorders were evaluated for three aspects of natural defense: natural killer (NK) cells which lyse HSV-infected fibroblasts [NK(HSV-FS)], NK cells which lyse K562 tumor targets [NK(K562)], and interferon-alpha generation. In addition, capacity to make interferon upon challenge with other commonly used inducers was also evaluated. Most patients with severe combined immunodeficiency disease (SCID) and deficits of both T- and B-cell function demonstrated normal NK function with one or both targets. Six of eight SCID patients generated interferon-alpha at or below the lower limit of normal while only two made clearly normal levels. Six of 10 patients with Wiskott-Aldrich syndrome (WAS) had normal NK(K562) and five of 10 generated normal levels of interferon-alpha but all had severely deficient NK(HSV-FS). Patients with Bruton's agammaglobulinemia demonstrated normal NK and interferon generation, as did patients with common variable immunodeficiency, even when subdivided into patients with T-cell proliferative deficiencies and those with only hypogammaglobulinemia. Natural defense parameters may help categorize patients with SCID and WAS and help define these heterogeneous diseases.
The snout reflex appeared to be electrophysiologically present in a great majority of normal subjects. Nevertheless, a facilitation of the R2 responses of the snout reflex was found in parkinsonian patients while in pseudobulbar patients an augmentation of the R1 took place. The snout reflex, therefore, seemed to behave in a similar way as the blink reflex. In order to explain the reported findings the authors took into account the different effects of the lesions of the lateral and ventral motor system upon the oligo- and multisynaptic reflexes.
Data about the influence of hemispheric lesions on the blink reflex are conflicting. 21 hemiplegic patients and 11 control subjects were investigated. The duration, latency and electric area of electrically evoked blink reflex responses were evaluated by common electromyographic techniques. A depression of the ipsilateral and the consensual late response after stimulation of the paretic side was the most evident finding. However, also a certain increase of the early response and a depression of the late response of the paretic side independent of the side of stimulation emerged. Concerning the parameters taken into account, the evaluation of the latency period seems to be the most significant and reliable. Determination of the electric area provides additional useful data which, however, may easily lead to mistakes.
An Italian male aged 50 years with oculopharyngeal muscular dystrophy is reported. Eleven of his relatives, over a period of three generations, had ptosis, dysphagia, nasal voice and difficulty in walking. The distribution of muscle weakness in the propositus and in one of his sisters was proximal in the upper, but distal in the lower limbs, confirming the existence of a relationship between oculopharyngeal dystrophy and distal myopathy. The first muscle biopsy appeared normal except for some round-cell collections, whereas the second one, 5 years later, showed marked dystrophic changes. Some patients with oculopharyngeal dystrophy may apparently pass through a secondary muscular inflammatory stage.
The ability of an association of three steroid hormones to influence the reinnervation process and the trophism of rabbit muscles denervated by crush of the sciatic nerve was investigated. The beginning of reinnervation was established with electromyographic recordings from the tibialis anterior muscle. The distance from the site of crushing to the point where the motor nerve enters the tibialis anterior muscle was then measured in each animal, and the nerve regeneration velocity (mm/day) was calculated: a slightly but significantly higher (P less than 0.001) mean value was found in treated animals compared with untreated ones. When soleus and extensor digitorum longus (EDL) muscles were histochemically examined 50 days after lesion, a larger mean diameter of type 2c fibers was found in treated than in untreated animals, pointing out a possible useful effect of the treatment. On the contrary, the size reduction of EDL type 2b fibers was more pronounced in treated rabbits, indicating a catabolic influence of the drugs on this fiber type.
In 6 cases of mild botulinum intoxication, conventional EMG and single fibre EMG (SFEMG) were performed on admission to our ward (about 15 days after ingestion of the toxin) and 4, 8 and 14 weeks after admission. In 4 cases, conventional EMG resulted in abnormal findings; and they normalized 4 weeks later. On the first examination, SFEMG revealed in all cases but one the occurrence of potential pairs with abnormal jitter (above 50 mus). The % of the potential pairs with abnormal jitter ranged in different cases from 17% to 44%. Some of the potential pairs with abnormal jitter showed blockings; the occurrence of blockings was not strictly related to jitter value. Mean jitter value and % of potential pairs with abnormal jitter became progressively reduced with increasing time after intoxication. Nevertheless, in 4 cases slightly abnormal findings were still present after 4 months. The data obtained in the basal condition are in agreement with those reported by others. SFEMG findings relate fairly well to conventional EMG data and clinical status. SFEMG has proved to be a very sensitive method for studying the neuromuscular transmission defect in botulism and in obtaining further information on the course of the syndrome.
Psychological distress in parents of children with acute lymphatic leukemia was evaluated by means of the Symptom Distress Checklist. This scale was administered twice: within a few days after the child's admission to hospital and 8 months later. Twenty-five consecutive, unselected subjects were compared with controls matched for age, sex, marital status and social class. At the first evaluation the sample presented higher mean scores than the controls for anxiety (P less than 0.005), depression (P less than 0.005), sleep disturbances (P less than 0.005) and obsessions (P less than 0.05). An 8 months' follow-up confirmed the persistence of anxiety (P less than 0.05), sleep disturbances (P less than 0.05) and above all depression (P less than 0.005).
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99mTechnetium pyrophosphate (99mTc PYP) muscular scanning was carried out in 28 patients affected by neuromuscular diseases. To express the muscle tracer retention quantitatively, the soft tissue to bone uptake ratio was calculated in the thigh and in the lower leg. Significantly increased ratio values were found in all the patients with polymyositis and in most patients with different types of muscular dystrophy, while high values were seen in a few subjects with myasthenia gravis and with neurogenic disorders. These findings are compared with those of previous studies, and the factors playing a role in determining some discrepant observations are discussed. The use of 99mTc muscular scanning in the investigation of patients with suspected inflammatory myopathy is suggested.
Peripheral blood mononuclear cells from patients with essential mixed cryoglobulinemia were investigated for the ability to induce suppressor cell activity (S.C.A.) following in vitro exposure to Concanavalin A (Con A). The generation of Con A-S.C.A. is significantly impaired in essential mixed cryoglobulinemia compared to the healthy controls. No correlation was found between Con A-S.C.A. and the clinical or laboratory parameters of the disease. This impaired ability to induce Con A-S.C.A. in essential mixed cryoglobulinemia reflects a functional T-cell defect, which may play a role in the pathogenesis of the disease.
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