PubMed HealthSearch

Biomedical subjects

R Monforte

Publications and source records attributed to R Monforte.

At least 19 recordsLinked to original sources

Microvascular changes in skeletal muscle in idiopathic inflammatory myopathy.

Open deltoid muscle biopsy specimens from patients with idiopathic adult dermatomyositis, paraneoplastic dermatomyositis, childhood dermatomyositis, and idiopathic polymyositis, and from control patients were studied. Qualitative and morphometric capillary analysis by phase and electron microscopy was carried out. In the morphologic analysis the most striking difference was the presence of capillary damage and a higher capillary depletion in dermatomyositis as well as a higher capillary density in polymyositis. By electron microscopy, capillaries from patients with dermatomyositis showed mainly microtubuloreticular structures, loss of endothelial plasma membranes, and the appearance of abnormal cytoplasmic organelles. In contrast, capillaries from patients with polymyositis exhibited only minimal changes. By morphometric analysis, muscle capillaries in dermatomyositis had a significantly higher mean endothelial thickness than those in polymyositis. Finally, a significant topographic association between capillary damage and muscle fiber changes was observed only in patients with dermatomyositis. On the other hand, paraneoplastic dermatomyositis showed fewer structural and morphometric capillary changes than the other forms of dermatomyositis. We conclude that dermatomyositis is characterized by microvascular alterations that are absent in polymyositis. The topographic proximity of capillary changes to muscle fiber injury suggests that capillary damage may play a role in the pathogenesis of the muscle lesions observed in patients with dermatomyositis.

Capillaries

Abnormal sympathetic skin response in alcoholic subjects.

Although the impairment of autonomic function has been described in alcoholic subjects, there is no evidence for such a dysfunction to be due to damage of autonomic centers or peripheral small caliber fibers. To investigate the function of peripheral fibres of the autonomic nervous system, we have carried out an electrophysiological study of the sympathetic skin response (SSR) in the hands and feet in 70 alcoholic and in 33 non-alcoholic subjects. In the hand, SSR recordings were done simultaneously in the palm and finger tip by stimulating the median nerve at the wrist. A conventional electrophysiological study performed in the same patients was used to classify them according to the presence or absence of electrophysiological criteria for peripheral polyneuropathy (ECPN). In analyzing the results of the SSR test, we found absent responses in the finger tip with present responses in the palm in 18 patients, 13 of them with ECPN. Absent responses in the feet were observed in 37 patients, 28 of them with ECPN. From our findings, it is concluded that alcoholics suffer from a dysfunction of sympathetic sudomotor activity due to axonal involvement of the most distal segments of the nerves.

Adolescent

[Hypothyroid myopathy. A clinical and histologic prospective study of 19 patients].

Nineteen patients with primary hypothyroidism were prospectively studied before starting replacement therapy with thyroid hormones. Most patients had mild to moderate muscle symptoms: generalized muscle weakness, myalgia and cramps, and increased serum levels of muscle enzymes. In two patients these features were the motive for consulting the physician. The patients with muscular features had significantly higher plasma levels of lactic dehydrogenase and aldolase than asymptomatic patients. The major histological muscle findings were atrophy of type II fibers, abnormalities in the oxydative pattern of muscle cells, and glycogen deposition. In the biochemical investigations an increased muscle glycogen with reduced activity of the glycolysis and glucogenolysis enzymes were found. The patients with more marked histological abnormalities had lower levels of thyroid hormones, but the differences did not reach significant values. Five patients in euthyroid status after 12 months of therapy underwent a new biopsy. Although the remaining clinical and laboratory parameters had returned to normal, some of the histological muscle abnormalities persisted. This finding leads to the suspicion that the lack of thyroid hormones may induce irreversible structural damage in skeletal muscle.

Adult

Persistent low C3 levels associated with meningococcal meningitis and membranoproliferative glomerulonephritis.

Recurrent meningococcal meningitis is usually related to terminal complement factor deficiencies (C5-C8); however it is not frequent with isolated primary C3 deficiency. Similarly, membranoproliferative glomerulonephritis has been described in association with primary C3 deficiencies and the presence of C3 nephritic factor. We present a case of an 18-year-old woman with relapsing meningococcal meningitis in whom membranoproliferative glomerulonephritis and persistent low serum C3 levels were found. A detailed immunological study was performed, but no other abnormalities in the complement components were found. C3 Nef was also negative. Moreover, the familiar complement studies showed an asymptomatic C4 deficiency in her mother and borderline C3 levels in her sister. The presence of persistent low serum C3 levels in the absence of other immunological abnormalities suggests that this is the cause of the relapsing meningococcal infections and the glomerular disease of this patient. We suggest that a complement deficiency, including isolated C3 deficiency, should be ruled out in all cases of relapsing meningitis. Further, the possibility of glomerular disease should be carefully considered in these patients.

Adolescent

High ethanol consumption as risk factor for intracerebral hemorrhage in young and middle-aged people.

We examined the prevalence of high ethanol intake, hypertension, and other risk factors for intracerebral hemorrhage in a case-control study of 24 young and middle-aged patients with intracerebral hemorrhage. We recorded ethanol consumption, history of hypertension, liver disease, cigarette smoking, and mild or severe coagulation disorder in each case of intracerebral hemorrhage and in 48 control patients matched by sex and age. In univariate matched analyses, the frequencies of high ethanol intake (p = 0.009), hypertension (p = 0.05), and coagulation disorder (p = 0.05) were higher in the cases than in the controls. After controlling for possible confounding factors, we found that high ethanol intake and hypertension were the only independent risk factors for intracerebral hemorrhage (p = 0.02 and p = 0.05, respectively). The hemorrhagic lesion found in cases with a high ethanol intake tended to be located in the cerebral lobes (p = 0.01), contrasting with the typical basal ganglia location of hypertensive hematomas (p = 0.009). We conclude that chronic, high ethanol intake should be considered as an important risk factor for lobar hematomas in young and middle-aged people.

Adult

Immunohistochemical analysis of the immune reaction in the nervous system in paraneoplastic encephalomyelitis.

We examined frozen sections of frontal cortex, medulla, and dorsal root ganglia from a patient with small-cell lung cancer and paraneoplastic encephalomyelitis, involving the medulla and dorsal root ganglia, with a panel of antibodies reactive for IgG, IgM, C3, B cells, T cells, T cell subsets, macrophages, and class I and II (HLA-DR) major histocompatibility complex (MHC) antigens. We detected an antineuronal antibody (anti-Hu) in the serum and CSF of the patient and found deposits of IgG in the periphery of some neurons in dorsal root ganglia. The infiltrates were almost exclusively T cells with a predominance of CD8-positive cells. Neurons did not express class I or II MHC antigens. Satellite cells in the dorsal root ganglia from the patient and controls were HLA-DR-positive. These data indicate that CD8-positive T cells predominate in the inflammatory infiltrates of paraneoplastic encephalomyelitis. IgG deposits may be relevant in the damage of the sensory neurons.

Antibodies, Monoclonal

Myasthenia gravis after allogeneic bone marrow transplantation: report of a new case and pathogenetic considerations.

A 21-year-old caucasian man with T acute lymphoblastic leukemia underwent a bone marrow transplantation (BMT) and developed classic myasthenia gravis (MG) 46 months later. The association of almost all published cases with HLA B35 is discussed, as are the clinical aspects suggesting that BMT survivors are at risk for developing MG as part of the spectrum of chronic graft-versus-host disease.

Adult

[A community outbreak of Legionnaires' disease in Barcelona: clinical and microbiological study].

In February and March 1989 a community-acquired outbreak of legionnaires' disease developed in Barcelona, involving at least 56 patients (48 males and 8 females) with a mean age of 60 years (range 22-87). 70% were smokers, 20% alcohol abusers, 50% had chronic bronchitis and 20% were immunologically depressed. The most common signs and symptoms were: fever (100%), features of lung condensation (77%), cough (51%), stupor (27%), diarrhea (18%), thoracic pain (18%, hyponatremia (53%), increased serum level of hepatic enzymes (44%) or CK (37%), and renal failure (21%). Radiological involvement was bilateral in 30% of cases. In most patients the diagnosis was made by seroconversion (70%). Late seroconversion (between 4 and 14 weeks) was seen in 20 patients, whose age was significantly higher than that of patients with early seroconversion (p less than 0.02). All cases were caused by Legionella pneumophila serogroup 1. Forty-six patients (81%) were admitted to the hospital and 10 (18%) required tracheal intubation. Although all received erythromycin, seven patients died. Hypoxemia, leukopenia, hyponatremia and renal failure were associated with a higher mortality rate. However, after multivariate analysis renal failure appeared as the only independent prognostic variable. Finally, it was concluded that in the community-acquired outbreaks of pneumonia Legionella pneumophila infection should be ruled out.

Adult

[Legionellosis].

Explore the source record for details and available documents.

Humans

Chronic neutrophilic leukemia with marked myelodysplasia terminating in blast crisis.

A case of chronic neutrophilic leukemia (CNL) is reported. The patient had a history of bleeding, and showed sustained mature neutrophilic leukocytosis, hepatosplenomegaly, a high leukocyte-alkaline phosphatase score, elevated serum vitamin B12 and uric acid, and the presence of Döhle bodies in the neutrophils. In addition to the above typical features, marked myelodysplastic changes in the erythroid and megakaryocytic series were observed in the bone marrow. Three months after the diagnosis of CNL had been established, the hematological picture evolved into a blast crisis of monocytic type. Such findings give support to the statement of CNL as a distinct entity belonging to the spectrum of the myeloproliferative disorders.

Blast Crisis

Intestinal lymphoma in a patient with chronic lymphocytic leukemia of atypical phenotype: Richter's syndrome of unusual presentation.

A patient who developed an intestinal large-cell pleomorphic lymphoma during the course of untreated chronic lymphocytic leukemia (CLL) with an atypical phenotype (SIgG kappa) is reported. This is an unusual presentation of Richter's syndrome since RS with primary gastrointestinal involvement has only been described in two patients. In our case, immunological studies disclosed the same immunoglobulin (IgG kappa) in the large-cell pleomorphic lymphoma and on the surface of CLL cells, suggesting that both had arisen from the same clonal proliferation.

Aged