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Biomedical subjects

A Lanuza

Publications and source records attributed to A Lanuza.

15 recordsLinked to original sources

Lung transplantation in patients with x-linked agammaglobulinemia.

Lung transplantation is an established procedure to treat patients with end-stage lung disease. The criteria for recipient selection are broadening to include patients with congenital defects of the immune system, such as X-linked hypogammaglobulinemia (XLA). We report 2 cases of successful double lung transplantation in patients with XLA. The 2 men had developed bronchiectasis and end-stage lung disease despite early institution of intravenous immunoglobulin (IVIG) replacement therapy. Before transplantation, hypogammaglobulinemia was well controlled with IVIG in both patients. After transplantation, IVIG was administered every 48 hours during the first 10 days and then tapered slowly in the following weeks until returning to an every 3 weeks schedule. One patient has been followed up for 12 months and the other for 6 months. Lung function normalized in the first case and showed a restrictive pattern in the second one. Lung transplantation may be considered as a therapeutic option for patients with XLA and end-stage lung disease. Regular administration of IVIG overcomes the high risk of infections due to the severe immunodeficiency and the intensive immunosuppressive therapy.

Adult↗

Solitary fibrous tumour of the orbit. Report of a new case.

PURPOSE: Solitary fibrous tumour (SFT) of the orbit is a very rare lesion that may be misdiagnosed as fibrous histiocytoma, haemangiopericytoma, or other orbital tumour. We studied a case of SFT of the orbit in a 35-year-old woman. METHODS: The patient, with a 1-year history, reported a 'pressure' sensation behind her right eye, but no pain, vision changes, or other associated symptoms. Ocular examination was normal. Magnetic resonance imaging (MRI) showed a well-circumscribed medial mass in the right orbit. The tumour was excised. RESULTS: The tumour was a cellular spindle-cell neoplasm with a storiform pattern. The tumour cells were spindle-shaped with bland nuclei and rare mitoses. Immunostaining was positive for vimentin and CD34, but negative for cytokeratin (AE1-AE3), EMA, desmin, smooth muscle actin, S-100, CD31, CD45 and bcl-2. CONCLUSION: SFT can infrequently involve the orbit. The tumour must be removed entirely to avoid recurrences. Careful and continued follow-up is important because orbital recurrence may occur several years after the excision of the primary tumour.

Adult↗

Pattern of arborization of the motor nerve terminals in the fast and slow mammalian muscles.

A silver impregnation method and a morphometric approach were used to define differences existing in the motor nerve terminal branching pattern between a fast-twitch muscle (extensor digitorum longus) and a slow-twitch one (soleus) of the normal adult rat. Because no single measure can describe precisely all geometrical properties (ie both topology and metrics) of the nerve terminals, we evaluated morphologic parameters defining length and angular characteristics in the different terminal segments classified according to their centrifugal order. The main results indicate that the distal free-end segments in the extensor digitorum longus muscle are shorter and less divergent than in the soleus nerve terminals. The endings in the two muscles have different fractal dimensions. Findings are discussed in the context of the hypothetical mechanisms governing motor nerve terminal size and complexity.

Animals↗

Hepatic hamartoma in a newborn.

In a newborn infant with heart failure and hepatomegaly the radiological findings suggested hepatic haemangioendothelioma, but subsequently the tumour proved to be a hamartoma.

Angiography↗

The wide spectrum of the asphyxiating thoracic dysplasia.

Seven cases of A. T. D. are presented. Radiological findings were extraordinarily diverse. The prognosis of the disease is difficult for each individual case, because of the frequent pulmonary complications and cystic renal lesions are not always directly related to the severity of the bone changes. The authors believe that the term "thoracic-pelvic-phalangeal dysplasia" proposed by Langer, is the most adequate, since it points to the key factors in the diagnosis of the disease.

Asphyxia↗

[Cavernous hemangioma of the liver (author's transl)].

A three-month old asymptomatic infant was incidentally found to have an abdominal mass. Through standard radiological and vascular procedures it was defined as being of hepatic origin, vascular etiology and of benign prognosis. Differences among cavernous hemangioma, hepatoma, metastasis and hemangio-endothelioma are summarized. The importance of angiography is emphasized as an essential procedure previous to the surgical evaluation and therapy.

Angiography↗

[Urinary tract abnormalities with anorrectal malformations (author's transl)].

Thirty five patients with anorrectal malformations are reviewed. These are divided in high and low anomalies according to some simple clinical data, better than the drawing of reference lines to determinate the height of puborrectalis muscle. Malformations were associated in 13 cases with urinary tract estructural anomalies and in four cases with isolated vesico-ureteral reflux. Diagnosis of urinary tract infection was made in 14 patients, 12 of them with recto-urinary fistula. A point is made about the complete and early exploration of all these patients to prevent irreparable renal damage that could be developed.

Abnormalities, Multiple↗

[Indications of vascular exploration in the diagnosis of abdominal neuroblastoma (author's transl)].

The criteria on the indications of angiography are shown in the diagnosis of abdominal neuroblastoma, stressing its' utility in situations where conventional procedures do not supply an accurate diagnosis. Angiography happens to be fundamental for diagnosis retroperitoneal tumours which eventually result in a silent kidney as well as for the differential diagnosis of some hepatic tumours and Pepper's syndrome.

Abdominal Neoplasms↗

Indications for pneumoperitoneum in the diagnosis of congenital anomalies in the umbilical region.

Thirty pneumoperitoneograms were performed postmortem and 5 were performed for diagnosis: the technique itself, the radiological anatomy of the umbilical region, and the usefulness of the procedure in diagnosis are discussed. Pneumoperitoneum may be indicated in the investigation of a bleeding Meckel's diverticulum, in the exclusion or confirmation of remnants of the omphalomesenteric duct, in chronically moist lesions of the umbilicus resistant to symptomatic treatment, in suspected cases of non-communicating urachal cysts which cannot be diagnosed by cystogram, and in the differential diagnosis of abdominal tumours related to the umbilical region.

Abdominal Neoplasms↗

[Radiological diagnosis of primitive hepatic tumors in infants (author's transl)].

Six primitive hepatic tumours (two benign and four malignant) collected over a period of seven years, are studied. The symptoms were mild or absent in many of the cases, and all were discovered after investigation of hepatomegaly or abdominal mass. The importance of the co-ordinated use of radiological and gammagraphical findings is pointed out.

Angiography↗

[A study of mortality in patients with acute cerebrovascular pathology in the Castellon General Hospital].

OBJECTIVE: To analyse the hospital death rate caused by acute cardiovascular disorder and their factors conducted. METHODS: We made a retrospective review of all patients suffering from this pathology who had gone into the Casualty Ward of the Castellón General Hospital in the course of 1991: we also looked for all patients with acute cardiovascular disorder who had died in the hospital. We studied the risk factors for the patients referred using a univariant analysis, comparing those still living with those who had died. RESULTS: We find 514 patients diagnosed as having acute cardiovascular disorder, with an stroke/TIA incidence rate of 188 cases per 100,000 inhabitants. The proportion of referred patients was 52%. Among these, the death rate was 27.8%. Factors affecting the death rate were the age of the patients, the reduction in the level of consciousness, and lateral gaze at the start of the stroke. CONCLUSIONS: We believe that the high death rate in our selection was due to the severity of the strokes and the advanced age of the patients, as was in accordance with the methodology used in the gathering of data, which made it possible to include patients who died early.

Acute Disease↗