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Biomedical subjects

I Marie

Publications and source records attributed to I Marie.

At least 73 records · Page 4Linked to original sources
PubMed2000

Susac's syndrome or retinocochleocerebral vasculopathy: a misdiagnosed and overlooked disorder.

We report a case of Susac's syndrome (retinocochleocerebral vasculopathy) that was initially successfully treated with steroids. The patient had a recurrence of the disorder 2 years after the onset of the first clinical manifestations; this was treated with steroids and both calcium channel blockers and aspirin. Our findings underline the importance of recognizing patients with retinocochleocerebral vasculopathy at an early stage so that it may be accurately managed and, therefore, result in both decreased morbidity and disability related to severe sequelae. We also stress the need for patients with Susac's syndrome to have both close and long-term follow-up in order to disclose both symptomatic and asymptomatic recurrences of the disorder.

Journal Article↗
PubMed1999

Hypertrophic osteoarthropathy can indicate recurrence of Whipple's disease.

We report the case of a patient with Whipple's disease (WD) who developed hypertrophic osteoarthropathy (HOA) characterized by digital clubbing, periostosis of the tubular bones, and polysynovitis. The HOA disclosed the recurrence of the patient's WD, since polymerase chain reaction (PCR) analysis clearly demonstrated the presence of Tropheryma whippelii in the synovial fluid from the patient's left knee. Initiation of appropriate antibiotic therapy resulted in complete healing of all clinical rheumatologic manifestations within 2 months and in disappearance of radiographic bone changes at 7-month followup. We suggest that HOA be included within the spectrum of rheumatologic manifestations of WD, and that an evaluation for WD should be considered in patients, especially middle-aged men, presenting with HOA even without gastrointestinal symptoms. PCR analysis may be useful in accurate diagnosis and management of early WD with unusual clinical manifestations, and may contribute to decreased morbidity and mortality.

Anti-Bacterial Agents↗
PubMed1999

[Pulmonary involvement in systemic scleroderma. Part I. Chronic fibrosing interstitial lung disease].

INTRODUCTION: Chronic pulmonary interstitial fibrosis is the most frequent respiratory manifestation in systemic sclerosis, occurring in 80% of cases. It remains a severe complication of the disease and is the primary cause of mortality related to respiratory insufficiency in 20 to 60% of cases. CURRENT KNOWLEDGE AND KEY POINTS: The date of onset of interstitial lung disease remains undetermined, and only in rare cases does it reveal the presence of systemic sclerosis. The clinical signs are only observable at a later stage, when at least 50% of the lung parenchyma is affected. The methods of choice adopted for early diagnosis of this disease are high resolution computed tomography and pulmonary functional investigations; they should be carried out during the preliminary investigation and at follow-up once a year. Moreover, high resolution computed tomography also provides prognostic data, for there is a correlation between the type of lesion and its severity as determined by high resolution computed tomography and by histological findings. The value of other methods of investigation, in particular bronchoalveolar lavage, has not yet been clearly established. The association of cyclophosphamide and corticoids is currently being evaluated (indications, administration modalities, duration), and this combination may be the most effective treatment. FUTURE PROSPECTS AND PROJECTS: Interstitial lung disease is one of the major causes of morbidity and mortality in systemic sclerosis. Early diagnosis and management of this disease is therefore of utmost importance.

Adrenal Cortex Hormones↗
PubMed1999

[Pulmonary involvement in systemic scleroderma. Part II. Isolated pulmonary arterial hypertension, bronchopulmonary cancer, alveolar hemorrhage].

INTRODUCTION: Pulmonary interstitial fibrosis is the most frequent cause of lung disease in systemic sclerosis. However, other pulmonary complications exist, including lung cancer, alveolar hemorrhage, and in particular isolated pulmonary arterial hypertension, which is still considered the bête noire as regards this disease. CURRENT KNOWLEDGE AND KEY POINTS: The prevalence of pulmonary arterial hypertension has been reported to range from 5 to 60% in cases of systemic sclerosis; isolated pulmonary arterial hypertension has been principally observed in subjects with a ten-year history of limited forms of the disease. As the patient remains asymptomatic for a long period, with nonspecific respiratory clinical manifestations, the diagnosis is made at a much later stage in the course of the disease. The diagnostic method of choice is echocardiography-doppler, which should be performed during the preliminary investigation, and at follow-up. The prognosis is poor, and patient survival rate at 2 years after onset of symptoms amounts to 40%. To date, no curative therapy for pulmonary arterial hypertension has yet been found. FUTURE PROSPECTS AND PROJECTS: A knowledge of the mechanisms involved in the development of isolated pulmonary arterial hypertension is essential to the determination of new and relevant therapeutic strategies. Vasodilatory treatment, notably calcium channel blockers, prostacyclin and analogs such as iloprost, may be effective at an early stage of the disease before the appearance of permanent vascular damage.

Echocardiography, Doppler↗
PubMed1999

[Pulmonary lymphangioleiomyomatosis: an often fortuitous diagnosis. A case report].

INTRODUCTION: Lymphangioleiomyomatosis is an uncommon disorder of unknown origin, which exclusively occurs in women of reproductive age. The condition is characterized by proliferation of immature smooth muscle cells throughout the lungs, i.e., in the peribronchial, perilymphatic, and perivascular areas. This results in obliteration of the respiratory tract and in the development of cysts. Lymphangioleiomyomatosis has a poor prognosis due to both numerous lung complications and progression of the disease to respiratory failure. EXEGESIS: We report the case of a patient in whom lymphangioleiomyomatosis was fortuitously diagnosed from chest CT scan, itself performed for the diagnosis of pulmonary embolism. This case is therefore of particular interest. CONCLUSION: Our results suggest that the prevalence of lymphangioleiomyomatosis is probably underestimated due to its clinical latency and the absence of specific laboratory tests. Therefore, the development of non-invasive radiological methods should permit early diagnosis of the disease.

Adult↗
PubMed1999

[Involvement of the small intestine in systemic scleroderma].

INTRODUCTION: Though impairment of the gastrointestinal tract is commonly encountered in patients with systemic sclerosis, the most frequent abnormalities are esophageal and anorectal disorders. Involvement of the small intestine is also common, reaching a 40-80% prevalence. It often leads to life-threatening complications. CURRENT KNOWLEDGE AND KEY POINTS: The occurrence of small intestine impairment and its potential relationships with other organ impairment is still unknown. However, it rarely indicates the existence of the disease (10%) which remains asymptomatic for a long period. As clinical symptoms are non-specific and radiological tests (upper intestinal tract barium meal, gastrointestinal transit times of radiolabeled meal, computerized tomography scan) not sensitive enough to detect the symptoms, diagnosis of small intestine impairment is delayed, i.e., when severe complications such as malabsorption or pseudo-obstruction are present. The physiopathology of small intestine disorders is still unclear, leading to both collagenous fibrosis and atrophy of muscle fibers. As well, its treatment is difficult. FUTURE PROSPECTS AND PROJECTS: Knowledge of the mechanisms at the origin of small intestine impairment in the course of systemic sclerosis is important for the development of efficacious therapies. Manometry of the small intestine would be a useful tool to assess the various motor abnormalities that may occur in patients presenting with systemic sclerosis associated with either malabsorption or pseudo-obstruction. It would also provide a useful test in selecting patients whose treatment require somatostatin analogs.

CREST Syndrome↗