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991.
JA da Costa Nery PAM Schreuder P Castro Teixeira de Mattos L Vieira de Mendonça R Tebaldi Tardi S de Mello RD Azulay LF Lehman B Naafs 《Journal of the European Academy of Dermatology and Venereology》2009,23(2):150-156
Background The question was raised as to why 'obvious' signs of leprosy, Hansen's disease (HD), are often missed by medical doctors working in a HD endemic area.
Methods This study describes a small sample of patients who were diagnosed with HD during their hospital admission and not before. The discussion is whether the typical early signs and symptoms of HD are just not recognized, or whether unusual presentations confuse the attending physician.
Results A total of 23 HD patients were hospitalized during the study period, of which 6 (26%) were only diagnosed with HD during their admission. All were classified as lepromatous leprosy (LL) with a history of signs and symptoms of HD. In nearly all patients, a suspicion of HD might have been raised earlier if a careful history and dermato-neurological examination had been done.
Conclusions Multibacillary (MB) HD, especially close to the lepromatous end of the spectrum, may mimic other diseases, and the patient can not be diagnosed without a biopsy or a slit skin smear examination. Clinicians working in a HD endemic area (Rio de Janeiro) do not always include HD in their differential diagnosis, especially when the clinical presentation is unusual. HD should be considered in all patients with skin lesions not responding to treatment, especially when they have neurological deficits, and live or have lived in an HD endemic area. Due to the increase in global travel and immigration, doctors in low endemic areas need to consider HD as a possible diagnosis.
None declared 相似文献
Methods This study describes a small sample of patients who were diagnosed with HD during their hospital admission and not before. The discussion is whether the typical early signs and symptoms of HD are just not recognized, or whether unusual presentations confuse the attending physician.
Results A total of 23 HD patients were hospitalized during the study period, of which 6 (26%) were only diagnosed with HD during their admission. All were classified as lepromatous leprosy (LL) with a history of signs and symptoms of HD. In nearly all patients, a suspicion of HD might have been raised earlier if a careful history and dermato-neurological examination had been done.
Conclusions Multibacillary (MB) HD, especially close to the lepromatous end of the spectrum, may mimic other diseases, and the patient can not be diagnosed without a biopsy or a slit skin smear examination. Clinicians working in a HD endemic area (Rio de Janeiro) do not always include HD in their differential diagnosis, especially when the clinical presentation is unusual. HD should be considered in all patients with skin lesions not responding to treatment, especially when they have neurological deficits, and live or have lived in an HD endemic area. Due to the increase in global travel and immigration, doctors in low endemic areas need to consider HD as a possible diagnosis.
Conflicts of interest
None declared 相似文献
992.
Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington’s disease brain nuclei
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Marcela V. Karpuj Hideki Garren Hilda Slunt Donald L. Price James Gusella Mark W. Becher Lawrence Steinman 《Proceedings of the National Academy of Sciences of the United States of America》1999,96(13):7388-7393
The protein huntingtin (htt), aggregated in neuronal nuclear inclusions, is pathognomonic of Huntington's disease (HD). Constructs, translated in vitro from the N terminus of htt, containing either polyQ23 from a normal individual, or polyQ41 or polyQ67 from an HD patient, were all soluble. Transglutaminase (TGase) crosslinked these proteins, and the aggregations did not have the staining properties of amyloid. More TGase-catalyzed aggregates formed when the polyglutamine domain of htt exceeded the pathologic threshold of polyQ36. Furthermore, shorter htt constructs, containing 135 aa or fewer, formed more aggregates than did larger htt constructs. TGase activity in the HD brain was increased compared with the control, with notable increases in cell nuclei. The increased TGase activity was brain specific. In lymphoblastoid cells from HD patients, TGase activity was decreased. TGase-mediated crosslinking of htt may be involved in the formation of the nonamyloidogenic nuclear inclusions found in the HD brain. The staining properties of nuclear inclusions in the HD brain revealed that they were not amyloid. 相似文献
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Milani Junior R; Jorge MT; de Campos FP; Martins FP; Bousso A; Cardoso JL; Ribeiro LA; Fan HW; Franca FO; Sano-Martins IS; Cardoso D; Ide Fernandez C; Fernandes JC; Aldred VL; Sandoval MP; Puorto G; Theakston RD; Warrell DA 《QJM : monthly journal of the Association of Physicians》1997,90(5):323-334
The jararacucu, one of the most dreaded snakes of Brazil, southern Bolivia,
Paraguay and northeastern Argentina, is a heavily-built pit viper which may
grow to a length of 2.2 m. Up to 1000 mg (dry weight) of highly-lethal
venom may be milked from its venom glands on a single occasion. It has
accounted for 0.8% to 10% of series of snake bites in Sao Paulo State,
Brazil. We examined 29 cases of proven jararacucu bites recruited over a
20-year period in two Sao Paulo hospitals. Severe signs of local and
systemic envenoming, (local necrosis, shock, spontaneous systemic bleeding,
renal failure) were seen only in patients bitten by snakes longer than 50
cm; bites by shorter specimens were more likely to cause incoagulable
blood. Fourteen patients developed coagulopathy, six local necrosis
(requiring amputation in one) and five local abscesses. Two became shocked
and four developed renal failure. Three patients, aged 3, 11 and 65 years,
died 18.75, 27.75 and 83 h after being bitten, with respiratory and
circulatory failure despite large doses of specific antivenom and
intensive-care- unit management. In two patients, autopsies revealed acute
renal tubular necrosis, cerebral oedema, haemorrhagic rhabdomyolysis at the
site of the bite and disseminated intravascular coagulation. In one
survivor with chronic renal failure, renal biopsy showed bilateral cortical
necrosis; the patient remains dependent on haemodialysis. Effects of
polyspecific Bothrops antivenom were not impressive, and it has been
suggested that anti-Bothrops and anti-Crotalus antivenoms should be given
in combination.
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999.
Ursula B. Wandl Otto Kloke Marianne Nagel-Hiemke Thomas Moritz Reinhard Becher Bertram Opalka Wilhelm Holtkamp Heinrich Bartels Siegfried Seeber Norbert Niederle 《British journal of haematology》1992,81(4):516-519
Between March 1988 and July 1990, 28 adults with chronic myelogenous leukaemia (CML) were treated with a combination of recombinant human interferon (IFN) alpha-2b s.c. (initial dose 4 x 10(6) U/m2) and recombinant human IFN gamma s.c. (50 micrograms totally) daily. All patients were in chronic phase disease and had been treated previously with chemotherapy or bone marrow transplantation. A complete haematologic remission was achieved in three patients (11%), a haematologic remission in 12 patients (43%), and a partial haematologic remission in seven patients (25%). Six patients did not respond to this schedule. Acute side-effects were flu-like symptoms, fever and chills. During long-term treatment six patients developed polyarthralgia. Haematotoxicity WHO grade III occurred in three patients, and WHO grade IV in two patients. One patient developed psychosis, and in another patient an exacerbation of a pre-existing sarcoidosis was observed. We conclude that this combination is tolerable and effective in inducing haematological remissions in pretreated CML patients. 相似文献
1000.
Hemopoietic cells in chronic granulocytic leukemia (CGL) frequently contain a chromosome translocation involving chromosome 22 and another autosome, usually number 9. The translocated chromosome 22 is known as the Philadelphia (Ph) chromosome. The appearance of a second Ph chromosome is the most common cytogenetic abnormality in CGL signaling the blastic phase. For 6 yr we serially studied a man with atypical CGL whose marrow cells were marked by a translocation from chromosome 18 to chromosome 11 [46XY,t(11;18)(q23;q12)]. Three months prior to blast transformation there appeared an extra copy of the marker chromosome 18: 47XY,t(11;18)(q23;q12),+(18p11 leads to 18q12). This man presents a new cytogenetic pattern of clonal evolution in CGL. The pattern is analogous to that of the Ph chromosome and is characterized by a balanced chromosomal rearrangement and the subsequent acquisition of an extra copy of the small translocation chromosome immediately prior to blast transformation. 相似文献