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Biomedical subjects

J Fraga

Publications and source records attributed to J Fraga.

At least 73 records · Page 4Linked to original sources
PubMed1996

Porokeratotic eccrine ostial and dermal duct nevus of late onset: more frequent than previously suggested?

We describe the case of a 10-year-old boy with a porokeratotic eccrine ostial and dermal duct nevus (PEODDN) of late onset. The patient had an 8-year history of multiple keratotic papules on the dorsal surface, and multiple yellowish pitting lesions on the plantar surface of the right foot. Light-microscopic studies of both lesions showed multiple cornoid lamella-like parakeratotic columns, which exclusively arose over eccrine sweat ducts in which the acrosyringium was dilated. Although PEODDN is considered to be a congenital hamartoma of eccrine origin, a review of the literature showed us that the frequency of a late-onset variant may be as high as 26%.

Child↗
PubMed1996

Leukocytoclastic vasculitis in subacute cutaneous lupus erythematosus: clinicopathologic study of three cases and review of the literature.

BACKGROUND: Leukocytoclastic vasculitis associated with subacute cutaneous lupus erythematosus (SCLE) was observed by Sontheimer et al. in their first clinical series of patients with SCLE, although recent reports have suggested that its frequency was rare. OBJECTIVE: To evaluate the prevalence of cutaneous leukocytoclastic vasculitis (CLV) in patients with SCLE and to describe the clinicopathologic manifestations, response to treatment and prognosis of this subgroup of patients. METHODS: We reviewed all cases of SCLE seen at the Hospital de la Princesa from 1980 to 1995. RESULTS: Three (9%) of our 27 patients with SCLE exhibited CLV and SCLE. Purpura, maculoerythematous lesions, urticaria, nodules and necrotic lesions on the leg, trunk and palmoplantar regions were present in our patients with CLV and SCLE. Histologically, SCLE-associated CLV was a small-sized vessel leukocytoclastic vasculitis with intravascular thrombosis in the deep dermis and coexistence of lesions of CLV and SCLE. CONCLUSION: In our patients, CLV was self-limited and not associated with a worsened prognosis, with only cutaneous involvement in their further relapses of SCLE.

Adult↗
PubMed1996

Microscopic polyangiitis. A systemic vasculitis with a positive P-ANCA.

A young girl presented with a purpuric rash on lower limbs, fever, eosinophilia, peripheral neuropathy and progressive renal insufficiency. She developed vesicles, purpuric macules and papules on the head, several nodules on the palmar sides of hands and fingers, splinter haemorrhages, and a disfiguring, facial oedema. A renal biopsy specimen disclosed a focal and segmental necrotizing glomerulonephritis with crescents. Peripheral ANCA with antimyeloperoxidase specificity [P-ANCA (MPO)] was positive and cytoplasmic ANCA with PR3 specificity was negative. Treatment with prednisone and cyclophosphamide was started with a good clinical response, stabilization of renal insufficiency and disappearance of P-ANCA (MPO). Our case fulfils the diagnostic criteria for microscopic polyangiitis (microscopic polyarteritis, MPA), namely a segmental necrotizing and crescentic glomerulonephritis associated with extrarenal vasculitis involving small-sized vessels, without granulomas or asthma. This is a rare disease, which has a poor prognosis in the absence of aggressive therapy, and is infrequently reported in dermatological journals.

Adolescent↗
PubMed1996

Coexistence of diffuse plane normolipaemic xanthoma and amyloidosis in a patient with monoclonal gammopathy.

Diffuse plane normolipaemic xanthomatosis (DPNX) is a well-defined clinicopathological entity that characteristically presents with yellow-orange plaques in the periorbital areas, the neck, the upper trunk and the flexural folds. DPNX has been reported in association with lymphoproliferative disorders and, occasionally, with miscellaneous, probably coincidental disorders. A case of DPNX in a patient with an IgG lambda monoclonal gammopathy and systemic amyloidosis is reported. Clinical and histopathological findings revealed typical features of DPNX and amyloidosis coexisting in the cutaneous lesions. To our knowledge this is the first reported case showing coexistence of xanthoma and amyloidosis in cutaneous lesions in a patient with a monoclonal gammopathy.

Aged↗
PubMed1995

Reticular erythematous mucinosis associated with human immunodeficiency virus infection.

We report the case of a 56-year-old woman with reticular erythematous mucinosis (REM). During her workup infection with the human immunodeficiency virus (HIV) was detected. She developed a cerebral toxoplasmosis, salmonella sp. bacteremia and oral ulcerations with the presence of type I herpes simplex virus and cytomegalovirus. The relation of REM with the deposition of mucin in AIDS patients' bone marrow and HIV infection is discussed. To our knowledge, this is the first report where REM is associated with HIV disease.

Cytomegalovirus Infections↗
PubMed1993

Pharyngeal CT studies in patients with mild or severe upper airway obstruction during sleep.

The upper airway cross-sectional areas were studied with pharyngeal computed tomography (CT) at the nasopharyngeal, velopharyngeal, tongue base and hyoid bone levels in 119 consecutively investigated patients with a snoring complaint. According to their findings in an all-night static charge sensitive bed (SCSB) recording, the subjects were divided into four equally sized groups with increasing severity of nocturnal breathing disturbance. The body mass index (BMI) increased and the minimal cross-sectional area at the velopharyngeal level decreased consistently as a function of the severity of nocturnal breathing disturbance. The minimal cross-sectional area at the hyoid bone level showed a biphasic trend, with an initial decrease but a final increase, as the degree of nocturnal breathing disturbance aggravated. The results contradict the idea of gradually increasing anatomical narrowing of the upper airways in general as the nocturnal breathing disturbance exacerbates and support the concept of two anatomically determined entities of partial and complete upper airway obstruction during sleep.

Airway Obstruction↗
PubMed1993

Ulcerative colitis, primary biliary cirrhosis, and chronic pancreatitis: coincident or coexistent?

We report a 45-year-old woman with chronic ulcerative colitis complicated by primary biliary cirrhosis and concomitant chronic pancreatitis. The combination of primary biliary cirrhosis and ulcerative colitis has only rarely been reported, and the association of these diseases with chronic pancreatitis is even rarer. These systemic manifestations of ulcerative colitis should be looked for more often.

Adult↗
PubMed1992

[Small cell (oat cell) carcinoma of the prostate. Presentation of a case and review of the literature].

Prostate small cell carcinoma (SCC) is a rare and fatal condition over which countless enigmas hover both with regard to its origin and biological behaviour as well as to its management, the topic of this paper. We contribute the case of a 69-year old male diagnosed through transrectal biopsy and presenting lung, nodes and bone metastasis who underwent palliative UTR but died within four days of the intervention as a consequence of the metastatic condition. Microscopic and immunohistochemical findings characteristic of this type of tumour are described, emphasising the relevance of diagnosing this entity based on its poor prognosis and different therapeutic approach from that used for typical prostate adenocarcinoma.

Aged↗
PubMed1992

Importance of establishing appropriate local reference values for the screening of hemochromatosis: a study of three different control populations and 136 hemochromatosis family members. Hemochromatosis Clinical and Research Group.

Hemochromatosis is a human leukocyte antigen-linked (HLA-linked), potentially lethal disorder of iron metabolism with a high prevalence in white populations albeit an autosomal recessive mode of transmission. The diagnosis and treatment at early stages of the disease are critical to the prevention of the morbidity and mortality caused by the iron overload. After the identification of the first cases of hemochromatosis in Portugal, a screening program was started with a systematic search for the disease among family members of the patients, as well as in subjects from the normal population. In this study we analyze the results obtained with a total of 136 family members from 15 different families and 353 control subjects from three different villages, two in the north and one in the south of Portugal. We establish reference values for the biochemical tests used in the screening for iron overload and analyze the factors that affect those results. Besides sex-related differences, factors that were found to influence biochemical parameters most significantly included age and levels of daily alcohol intake. In addition, differences in iron status were identified between the populations from the regions in the north and the south of the country. We estimate, by HLA typing and family studies, a gene frequency for hemochromatosis of 0.14 that corresponds to a frequency of homozygotes and heterozygotes of 0.019 and 0.24, respectively.

Adolescent↗
PubMed1991

Fiberoptic bronchoscopic diagnosis of pulmonary disease in 151 HIV-infected patients with pneumonitis.

In a prospective study the efficacy of fiberoptic bronchoscopy was evaluated in the diagnosis of infections with opportunistic pathogens, Kaposi's sarcoma and nonspecific interstitial pneumonitis in 171 episodes of pneumonitis in 151 HIV-infected patients. Samples were collected by suction through the inner aspiration channel of the bronchoscope (n = 164), telescoping plugged catheter (n = 117) and transbronchial lung biopsy (n = 82). A high incidence of infections with pyogenic bacteria (12%), Legionella spp. (5 %) and Mycobacterium tuberculosis were diagnosed (9%). Bronchoalveolar lavage demonstrated a high diagnostic rate in bacterial pneumonia (significance level greater than 10(5) cfu/ml) and a low degree (10%) of contamination (less than 1% squamous epithelial cells). Bronchoalveolar lavage was more effective than the telescoping plugged catheter in yielding a significant number of colonies in patients with bacterial pneumonia previously treated with antibiotics. Nondiagnosed pneumonitis was more frequent in intravenous drug abusers than in homosexual men (p less than 0.001).

Adult↗
PubMed1991

Why don't all heavy snorers have obstructive sleep apnea?

Patients with obstructive sleep apnea (OSA) and heavy snorers without apnea both show intrathoracic suction pressures during sleep that exceed their static upper airway closing pressures. Complete airway occlusion, however, occurs only in the former patient group. We hypothesized that the kinetic properties of the airflow would be different in these two types of patients because of differences in upper airway morphology. The pharyngeal computed tomography (CT) was used to measure the cross-sectional areas of the upper airways in 15 patients with OSA, 25 nonapneic heavy snorers, and 14 control subjects while they were awake. Nocturnal breathing was monitored with the static charge-sensitive bed (SCSB). The patients with OSA had a narrower airspace at the velopharyngeal (VP) level than the controls (p less than 0.01); the nonapneic snorers did not differ from the other groups. At the tongue base (TB) and the hyoid bone (HB) levels there was no difference between the OSA and the control groups, but the nonapneic snorers had narrower airways at both of these levels compared with control subjects (p less than 0.01) and at the hyoid bone level compared with the OSA group (p less than 0.05). The VP/HB ratio was the parameter that best distinguished the patients with OSA from the nonapneic snorers (lower in the OSA group, p less than 0.001). We suggest that airway collapse during sleep is favored by a narrow velopharynx associated with large hypopharynx. Some heavy snorers may not have an oropharyngeal collapse because the peak inspiratory suction pressure could already be damped down at the level of the relatively narrow hypopharyngeal airways.

Airway Resistance↗
PubMed1990

Hairy leucoplakia--a clinical, histopathological and ultrastructural study in 33 patients.

Thirty-three cases of hairy leucoplakia (HL) in HIV-positive patients were studied. The most frequent clinical presentation was the development of corrugated plaques on the lateral sides of the tongue without spiculations on the surface. In all biopsies, herpetic-type viral inclusions were found. In 12 out of 14 cases in whom an electromicroscopic study was carried out, only viral particles of the herpes group were found. Human papillomavirus (HPV) was not demonstrated, although by means of immunohistochemistry, positive labelling for HPV was found in 95% of the cases. The aetiopathological significance of these findings remains to be evaluated.

Adult↗