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Biomedical subjects

R Simón

Publications and source records attributed to R Simón.

29 records · Page 2Linked to original sources
PubMed1982

[Idiopathic pulmonary hemosiderosis: anatomoclinical study of 2 cases].

Two children with idiopathic pulmonary hemosiderosis are described. Both of them presented initially a clinical picture of recurring iron deficiency anemia, before pulmonary symptoms and signs made it possible to establish correct diagnosis. In one case diagnosis was considered only when an overwhelming pulmonary hemorrhage occurred, leading to the patient's death. At necropsy, areas of fresh alveolar hemorrhage and hemosiderin loaded macrophages were found, as well as moderate to severe interstitial fibrosis and hyperplastic alveolar cells. There was not disruption of the basal alveolocapillary membrane.

Anemia, Hypochromic↗
PubMed1980

Leukaemoid reaction and eosinophilia in "inflammatory fibrous histiocytoma".

A case of retroperitoneal inflammatory fibrous histiocytoma associated to a marked granulocytic blood reaction and high eosinophilic count is reported. The paraneoplastic nature of the haematological findings and their probable prognostic role are discussed. Ultrastructurally the tumor does not differ significantly from "non inflammatory" fibroxanthosarcomas.

Aged↗
PubMedDate not supplied

[Vascular malformations of the central nervous system in children].

OBJECTIVE: To present the experience of vascular malformations (VM) of the central nervous system (CNS) in children obtained in our centre. Since these lesions do not often present clinically in childhood, there are few series described in the literature. PATIENTS AND METHODS: We made a retrospective review of the clinical histories of patients with VM of the CNS shown on MR, angiography and/or morbid anatomy, who were aged 15 years or under. Patients with Sturge-Weber or Von Hippel-Lindau syndromes were excluded. RESULTS: We describe 32 patients with VM of the CNS. Twenty four had arteriovenous malformations (AVM), 5 had cavernous malformations (CM), 2 had malformations of Galeno's vein (MGV) and one patient had a congenital fusiform arterial aneurysm. Twenty two children (68%) presented clinically with hemorrhages. This was the form of presentation in 20 patients with AVM. In 4 of the 5 patients with CM the first sign was epileptic crisis. The two patients with MGV presented with macrocephaly, in one neonate it was associated with heart failure and in on older baby with hydrocephaly. The patient with a congenital arterial aneurysm presented with paroxystic episodes of facial pain. CONCLUSIONS: The AVM are the VM which most frequently cause symptoms in childhood.

Adolescent↗
PubMedDate not supplied

[Spinal tumors in infancy. A report of 48 cases].

INTRODUCTION: Spinal tumours in infancy are an infrequent oncological disorder. The clinical features, usually of insidious onset, are alterations in gait and a painful spine. OBJECTIVE: To analyze the different histological types of spinal tumours seen in infancy and their form of presentation in our series. PATIENTS AND METHODS: We reviewed the clinical histories of 48 patients with intraspinal tumours, aged under 15 years, whose reports of histological diagnosis had been sent to our centre. RESULTS: Of the patients studied, 17 were girls (35.4%) and 31 boys (64.6%) with an average age of 7.7 years. The histological diagnoses made most frequently were astrocytomas (22.9%) and lipomas (18.8%) followed by metastases (12.5%), ependymomas (8.3%) and Edwing's sarcoma (8.3%). The clinical features were present prior to diagnosis for between 1 and 6 months in 13 patients, and for less than one month in 9 patients. The presenting symptoms were alterations in gait and back pain in most patients. Exploratory tests were related to the involvement of long vias and second motor-neurone lesions. The main topographical findings were: in the axial plane the lesions were extradural (23 patients) and in the sagittal plane there was dorsal involvement (34 patients). CONCLUSIONS: Insidious, progressive alterations in gait together with continuous, nocturnal back pain are valuable data when a serious spinal disorder is to be suspected. Early diagnosis should be based on neuroimaging tests, essentially MR, in the patients in whom spinal cord involvement is considered.

Adolescent↗
PubMedDate not supplied

[Spinal cord infarction/ischemia].

INTRODUCTION: Vascular pathology is considered to be uncommon, with a much lower incidence than cerebral vascular pathology. PATIENTS AND METHODS: We reviewed the clinical histories of patients with a clinical diagnosis of spinal cord infarction-ischemia attended by the Neurology Department and the Neuropaediatric section at our centre between 1990 and 1997. We analyzed the clinical and investigational findings and compared them with those in the literature. RESULTS: We studied the cases of 12 patients (nine adults and three children). There was only one case in which no etiological factor of any type was found to justify the spinal vascular condition. All the children had had surgery to repair coarctation of the aorta. The most frequent clinical presentation was of paraparesis-paraplegia with signs of involvement of the anterior spinal artery. MR was done in all cases, except in that of an adult who was diagnosed on arteriography. The adults were studied by means of MR during the first 24-48 hours after onset of the condition. In five cases this was normal. In two of the latter cases, MR was repeated and alterations were seen in one whilst the other was not satisfactory. In the other three cases MR was not repeated since in two there was clinical improvement and in the other excessive technical problems. On discharge the children had not improved at all although the six adults improved completely or almost completely. CONCLUSIONS: In general, our findings are comparable to those in the literature. We consider that the diagnosis is basically clinical and differential diagnosis should be made with other spinal disorders. MR is helpful, although early use does not show changes which help in diagnosis.

Adult↗
PubMedDate not supplied

[Gamma-sarcoglycanopathy:two new cases in a gypsy family family in Spain].

INTRODUCTION: Included under the heading of limb girdle muscular dystrophy is a heterogeneous group of myopathies which share the same phenotype characteristics. The illness is of early onset, progressive and basically involves muscles of the shoulder and pelvic girdles. Recent identification of muscle proteins and the genes which codify them has led to new classification of these conditions according to their genetic characteristics. It is currently accepted that there are two major groups: the dominant and recessive forms. The latter includes type 2C limb girdle muscular dystrophy associated with chromosome 13, where the gene for gamma-sarcoglycan is found. This protein belongs to the glycoprotein complex associated with dystrophin. Recently a new mutation has been identified, the C283Y, exclusive to the Gypsy race, which affects this gene and therefore the alterations in gamma-sarcoglycan produced by it. CLINICAL CASE: We describe two patients, Gypsy brothers, who complained of myopathy, which they had had for some years, compatible with this condition and in whom the C283Y mutation had recently been detected. CONCLUSIONS: We describe another Spanish Gypsy family, all members of which have the characteristic mutation. We emphasize the importance of genetic studies in all cases of myopathy which have not been fully diagnosed.

Biopsy↗
PubMedDate not supplied

[Acute cerebrovascular accident associated with drepanocytosis complicated by pneumococcal meningitis in two children].

INTRODUCTION: Between 6% and 17% of the patients with drepanocytosis will have an acute cerebrovascular accident (ACVA). Precipitating factors have been described including bacterial meningitis, upper respiratory tract obstruction, dehydration, intense exercise, postoperatively, and hypoxia due to altitude. CLINICAL CASES: We report two Negro children with drepanocytosis who, at the ages of 8 and 20 months, had pneumococcal meningitis complicated by an ACVA. The 20 month old boy had been previously diagnosed as having drepanocytosis; the 8 month old girl was diagnosed when she was admitted to hospital with meningitis. In both cases the clinical features of the ACVA were focal epileptic seizures followed by hemiplegia. On cranial CT and MR regions of focal ischaemia of the hemisphere were observed. The boy of 20 months made an excellent recovery with no long term sequelae. The 8 month old girl had severe permanent sequelae: hemiparesia, blindness, mental deficiency and epileptic encephalopathy. CONCLUSIONS: We present two Negro children with drepanocytosis who had ischemic ACVAs, a common complication of the disease, during the course of pneumococcal meningitis but with very different clinical courses. Recent increases in immigration will mean that previously rare illnesses will be more commonly seen in our environment, and should be considered in the differential diagnosis.

Anemia, Hemolytic↗