Biomedical subjects
H Rousset
Publications and source records attributed to H Rousset.
[Systemic lupus erythematosus with disease onset after age 65].
OBJECTIVES: Systemic lupus erythematosus with disease-onset in the elderly has rarely been studied (only one report about 21 patients with disease onset at 65 and older). Is the management of this pathology modified in this population? METHODS: Seventeen hospitalised cases of lupus patients with disease onset at 65 or older are retrospectively reported between 1988 and 2000. The results are compared with those of younger subjects. RESULTS: The female to male ratio is 1.83. Mean age at disease onset is 71.9 +/- 3.5 years. Mean duration of follow-up is 3.5 +/- 2.4 years. Main initial symptoms are: deterioration of general status (41%), arthritis (35%), cutaneous manifestations (35%), thrombo-embolism (24%) and pleuritis (18%). Malar rash is uncommon (12%). Nephropathy is never a revealing symptom and is rarely serious during the disease's evolution. Like in neurologic manifestations, the etiology has to be discussed in relation to associated co-morbidities. Concerning haematologic features, lymphopenia is found in 82% of the cases with a questionable specificity. Antinuclear antibodies are constant, anti-dsDNA antibodies are found in 82% of the cases, antibodies to extractable nuclear antigens in 50%, and anticoagulant circulating activity in 59%. Prognosis is difficult to assess in such a limited series but 5-years survival probability is 83%. Glucocorticoid lead to 50% of major complications. CONCLUSIONS: This study focuses on the particular initial manifestations of systemic lupus erythematosus in the elderly (deterioration of general status, thrombosis, unusual cutaneous symptoms), and on the specificity of differential diagnosis and treatment.
[Inherited polycythemia].
PURPOSE: The majority of polycythemias occurs sporadically without any other familial case. Very occasionally polycythemia finds a familial support. This work is a review of the actual knowledge about inherited polycythemias. CURRENT KNOWLEDGE AND KEY POINTS: Although polycythemias linked to a anomaly of the haemoglobin affinity for oxygen are well understood (haemoglobin mutants with high oxygen affinity, 2-3 Diphosphoglycerate deficiency and methemoglobinemia), so called primary polycythemias (above all primary familial and congenital polycythemias) just begin to find an explanation for ten years (erythropoietin receptor gene mutation). FUTURE PROSPECTS AND PROJECTS: Progressively, the part of really idiopathic polycythemias is smaller and smaller. Although most of mechanisms to explain congenital polycythemias are understood, some of them are still unresolved (Chuvash polycythemia, the majority of primary familial and congenital polycythemias).
[Subsepsis hyperergica: another way of discussing Wissler-Fanconi syndrome?].
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[Unusual initial manifestations of celiac disease in adults].
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[Neurological manifestations of Whipple disease].
Whipple disease is an uncommon chronic bacterial infection due to Tropheryma whipplei. Clinical manifestations are protean (joint pain, fever, weight loss, abdominal pain, lymphadenopathies), and the diagnosis is often delayed. Although previously considered a late manifestation of Whipple disease, neurological involvement is now frequently the initial clinical manifestation and represents the greatest risk for long-term disability. All patients should be treated and monitored as if they had central nervous system disease even if they are asymptomatic. Neurological manifestations include dementia (56 percent), abnormalities of eye movements (33p. cent), involuntary movements (28 percent), seizures, hypothalamic dysfunction, myelopathy, ataxia and psychiatric manifestations. Uveitis, retinitis, optic neuritis and papilloedema may be found. 80 percent of the reported cases of neuro-Whipple had associated systemic symptoms or signs but many patients are presenting without concurrent intestinal manifestation. Thus, the disease may remain undiagnosed or misdiagnosed, as rheumatoid arthritis or sarcoidosis. Traditionally, the diagnostic procedure of choice is biopsy of the duodenal mucosa by demonstrating PAS-positive foamy macrophages. However, not all cases have small bowel infiltration and tissue obtained from sites clinically affected may be helpful. CT and MR images of the central nervous system are normal or not specific: atrophic changes, mass lesions, focal abnormalities and hydrocephalus. The application of a PCR assay against Tropheryma whipplei has transformed the diagnosis. Positive results have been obtained from several tissues and from CSF and PCR is more sensitive than other techniques. All patients must be treated with antibiotics which cross the blood-brain barrier. Most agree that initial treatment with a combination of parenteral penicillin and streptomycin for at least 14 days is appropriate, thereafter cotrimoxazole orally 3 times a day for at least one and probably for two years. Third generation cephalosporins, rifampicin and chloramphenicol have been used successfully. PCR is recognized to be a useful tool for monitoring progress but it is sometimes difficult to reverse established neurological defects.
[A potential etiology of elevated ferritin: hyperferritinemia-cataract syndrome].
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[Is alopecia areata a psychosomatic disease?].
INTRODUCTION: Destruction of hair follicles by lymphocytes induces alopecia aerata. Hence, immunological mechanisms are involved in this disease, as for numerous dermatoses. Nonetheless, alopecia aerata appears to be a psychosomatic disease. Is there any contradiction? CURRENT KNOWLEDGE AND KEY POINTS: Alopecia aerata often occurs after stress, particularly during mourning. Psychiatric disorders are more frequent in patients with alopecia than in healthy subjects. But these disorders might be secondary to the visible hair disease. Psychopathological mechanisms need to be clarified but alexithymia seems to be the key for understanding how stress could induce hair loss. In the skin (and the scalp) all functions are narrowly controlled by nerve fibers. Among these functions are hair growth and immunity. Immune cells and hair follicle cells possess receptors for neurotransmitters, which are synthesized by neuronal endings. When activated, these receptors are able to modulate cell properties. The same phenomena are described with stress-induced hormones. In alopecia aerata, like in numerous other diseases, psychosomatics and immunology are not opposed because immune cells are controlled by the nervous system through neurotransmitters. FUTURE PROSPECTS AND PROJECTS: Research needs to be thorough in both the fields of psychology and neurobiology. Psychotherapies or psychotropes appear to be useful in the treatment of alopecia aerata.
[Inaugural symptoms of Horton's disease in a series of 260 patients].
PURPOSE: Horton disease or 'giant cell arteritis' is a known entity in its typical form; the difficulty in diagnosis is due to the atypical signs and symptoms. METHODS: We review 260 medical files presenting Horton disease between 1979 and 1999 in five different departments: three internal medicine departments, one rheumatology department and one geriatric department. RESULTS: The study shows a female domination with a mean age of 75 years. Temporal artery biopsy was done on all patients. Ten patients presented a vascular manifestation. The neurological manifestation was the first symptom in four patients. Five patients had cutaneous symptomatology, with positive temporal artery in three cases. Renal manifestation was present in two patients. Two symptoms are important to discuss because of their frequency: the cough and the peripheral arthritis. We found nine observations with arthritis affecting large joints and responding to nonsteroidal antiinflammatories with positive temporal artery biopsy in seven patients, and 21 observations manifesting by cough without radiological signs; in 57% of cases the temporal artery biopsy was positive, and the cough regressed with corticoids. CONCLUSION: These atypical symptoms have to be known to make a diagnosis and to begin a corticotherapy as soon as possible.
[Errando discimus: diagnostic error].
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[Serum markers in breast cancer and colorectal cancer (1997)].
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[Hereditary diseases of metabolism in the adult: a new challenge for the future].
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[Prevalence of hypertransaminasemia in untreated celiac disease].
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[Asymptomatic nodular thyroiditis].
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[Hereditary hemochromatosis].
INTRODUCTION: Hereditary hemochromatosis is a fairly common disease in the Caucasian population, with a prevalence estimated at between 1.5 to 3/1,000 inhabitants. Over the past few years, its symptomatology has altered; at present, its clinical aspect with diabetes mellitus, cirrhosis, and darker skin pigmentation only constitutes 10% of new cases of this disease. CURRENT KNOWLEDGE AND KEY POINTS: In 1996, the discovery of the C282Y mutation in the HFE gene radically altered the diagnostic approach to hereditary hemochromatosis. At present, any patient admitted with an isolated case of asthenia, or with arthralgia or hypertransaminasemia should be examined via transferrin-saturation testing: if the transferrin saturation coefficient is > 45%, then the presence of the C282Y mutation should be investigated to confirm the diagnosis of hemochromatosis. A liver biopsy is no longer necessary to establish the diagnosis, but this is still useful in cases of possible cirrhosis, which is the main risk factor for hepatocellular carcinoma. Phlebotomy remains the sole recommended treatment, and should be undertaken in a case-specific manner. Family screening should be carried out for all first-degree relatives for every new case that is diagnosed. FUTURE PROSPECTS AND PROJECTS: The discovery of the HFE gene has permitted hereditary hemochromatosis to be easily differentiated from other forms of hepatic iron overload including a new syndrome, dysmotabolic hepatosiderosis. Casos of homozygotic C282Y without hepatic iron overload have been described, but the clinical outcome of some of these cases requires further study, and adds to the controversy on whether systematic population screening should be made available.
[Atypical hypoglycemia encephalopathy revealing pancreatic insulinoma. A case report].
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[Clinical or biological symptoms leading to the search for amyloidosis].
INTRODUCTION: The extracellular, multifocal, disseminated or diffuse localization of amyloidosis accounts for the diversity of clinical presentations and late diagnosis. CURRENT KNOWLEDGE AND KEY POINTS: However, early diagnosis is important, as new drugs have been recently introduced. Both clinical picture and biology help guide diagnosis, including either kidney, heart, skin or neurologic involvement associated with monoclonal gammapathy (primary idiopathic amyloidosis AL); underlying inflammatory or infectious disease, familial Mediterranean fever with proteinuria (secondary amyloidosis AA); cardiac, neurologic or ocular involvement (heredofamilial amyloidosis); carpal tunnel syndrome, joint pain (amyloidosis of hemodialysis). Furthermore, amyloid fibrils are identified on salivary gland biopsy. FUTURE PROSPECTS AND PROJECTS: Due to the introduction of new specific drugs aimed at curing various amyloidoses, early diagnosis is important. Chemotherapy and hematopoietic stem cell transplantation are promising regarding AL amyloidosis, while liver transplantation has proven remarkably successful in heredofamilial amyloidosis. Progress in molecular biology should allow identification of various forms of familial amyloidosis.
[Primary parvovirus B19 infection in immunocompetent adults: clinical and biological manifestations. Retrospective study of 16 patients].
PURPOSE: The spectrum of clinical and biological expressions of parvovirus B19 infection is incompletely described. METHODS: We report 16 retrospective cases of primary parvovirus B19 infection that occurred in immunocompetent adults and were diagnosed by serology. RESULTS AND CONCLUSION: Most cases conformed to the classical descriptions of parvovirus-induced arthropathy (transient polyarthritis often accompanied by erythema, dysesthesia and pruritus). However, the diagnosis of primary parvovirus B19 infection should also be considered in other clinical and biological situations such as edema, acute anemia or cytopenia, vascular purpura, neuro-ophthalmologic manifestations, and hepatitis. Parvovirus infection may mimic diseases such as lupus erythematosus or systemic necrotizing vasculitis. The risk for confusion with both diseases is increased, as immunological abnormalities such as hypocomplementemia, when they are systematically searched for, prove to be common.