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Many women in quandary about HRT
Genes associated with periodic fevers highlighted at Dutch workshop
G
ene mutations associated with a number of periodic fevers took centre stage at the 2nd International Workshop on hyper-IgD and periodic fever syndrome (HIDS; Nijmegen, Netherlands; Dec 9–11). HIDS is a rare familial syndrome in which patients have attacks of fever, abdominal pains, arthralgias, skin lesions, and lymphadenopathy (http://hids.net). A unique characteristic of the syndrome is that patients have high concentrations of serum IgD. Recently, mutations in mevalonate kinase, a key enzyme in cholesterol synthesis, were independently discovered in patients with HIDS by Laurence Cuisset (Hôpital Cochin, Paris, France) and Hans Waterham (Academic Medical Center, Amsterdam, Netherlands), both of whom spoke at the workshop. “This is a textbook case of gene detection by use of forward and reverse genetics”, said Jos van der Meer (University Hospital St
Radboud Nijmegen, Netherlands), who discovered HIDS. Nevertheless, it is unclear how a defect in cholesterol synthesis could result in a periodic fever with a strong inflammatory response. Ian Todd and Richard McIntosh (Queen’s Medical Centre, Nottingham, UK) presented data on familial Hibernian fever (FHF), an autosomal dominant periodic fever. They described how they had pinpointed the gene for the tumournecrosis-factor (TNF) receptor as the causative gene for FHF. “There are now families from all over the world with the same genetic defect so I think that we should change the name of FHF to TNF-receptor associated periodic syndrome (TRAPS)”, argued Dan Kastner (National Institutes of Health, Bethesda, MD, USA), who also updated delegates on familial Mediterranean fever (FMF). With the better understanding of
these fevers comes the possibility of new treatments. For FMF, colchicine remains “the mainstay treatment”, said Avi Livneh (Sheba Medical Center, Tel Hashomer, Israel). For TRAPS, early results were presented on the use of injectable soluble TNF receptors. For HIDS, the potential use of thalidomide was discussed by Richard Powell (Queen’s Medical Centre, Nottingham, UK). “It might work, but neuropathy as a sideeffect precludes prolonged use”, he warned. Statins are another potential treatment for HIDS and a clinical trial on these is to start soon. Since the first HIDS workshop in 1995, much has been learnt about periodic fever syndromes. But, said Wietse Kuis (Wilhelmina Children’s Hospital, Utrecht, Netherlands), “much ground still needs to be covered”. Anna Simon, Joost P H Drenth
Many women in quandary about HRT
News in brief
ing either everybody should take it ne third of menopausal women or nobody should take it”, asserts are undecided about hormone Bastian. “But the decision is complireplacement therapy (HRT) and feel cated for the woman and her doctor, they are not getting enough informaand it needs to be individualised.” tion from their physicians to make In another study, Andrea LaCroix an informed choice, report US resear(Group Health Cochers. “We have no operative, Puget interest in having Sound, Seattle, women take it or WA, USA) and colnot—but we have leagues found that to help them make attitudes and beliefs a decision one way about HRT—rather or the other”, says than unbiased assLori Bastian of essment of risks and Durham Veterans benefits—are the Affairs Medical strongest correlates Center, NC, USA. More discussion needed both for a physiBastian and cocian’s prescription of HRT and for a workers studied 318 menopausal woman taking it (Women’s Health women participating in a larger study Issues 1999; 9: 306–18). For example, of decision-making about HRT. 176 only about half of 1083 menopausal had decided to use HRT, and 38 had women agreed that HRT reduced the decided against it; 104 were underisk of osteoporosis—the benefit cided. The undecided women were “most strongly supported by scientific less satisfied than their counterparts certainty”, says LaCroix. “I don’t with the information their health-care know if you can make an informed providers had given them about decision without knowing this.” HRT; they also understood less and “A big message here is that the evithought less about the risks and benedence keeps evolving; it’s a moving fits of HRT. In addition, they felt less target”, concludes LaCroix. “If we capable of making a decision about want people to make informed deciHRT that was right for them sions, they need access to the best (Women’s Health Issues 1999; 9: possible evidence as it emerges.” 330–37). “The information out there is strong and polarised, with forces sayMarilynn Larkin
Oral leishmaniasis treatment The oral phosphocholine analogue miltefosine has shown a cure rate of up to 97% in Indian patients with visceral leishmaniasis. All 120 patients who received 50, 100, or 150 mg miltefosine per day for 4 or 6 weeks had an initial parasitological cure; only six relapsed after 6 months. Gastrointestinal sideeffects were frequent but mild (N Engl J Med 1999; 341: 1795–800).
O
THE LANCET • Vol 354 • December 18/25, 1999
Mapping cancer mortality On Dec 8, the US National Cancer Institute published an updated Atlas of Cancer Mortality in the USA. The atlas covers the years 1950–94, and includes data from more than 3000 counties. As well as being printed, the atlas is also available in an interactive form on the internet at www.nci.nih.gov/atlas. A mouthful of microbes Those of us who are inclined to partake in the seasonal activity of kissing under the mistletoe may want to think twice this year. In studying the flora of the human subgingival crevice, researchers have identified 37 new bacterial phylotypes to add to the 500 strains already identified from the oral cavity (Proc Natl Acad Sci USA 1999; 96: 14547–52).
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G
ene mutations associated with a number of periodic fevers took centre stage at the 2nd International Workshop on hyper-IgD and periodic fever syndrome (HIDS; Nijmegen, Netherlands; Dec 9–11). HIDS is a rare familial syndrome in which patients have attacks of fever, abdominal pains, arthralgias, skin lesions, and lymphadenopathy (http://hids.net). A unique characteristic of the syndrome is that patients have high concentrations of serum IgD. Recently, mutations in mevalonate kinase, a key enzyme in cholesterol synthesis, were independently discovered in patients with HIDS by Laurence Cuisset (Hôpital Cochin, Paris, France) and Hans Waterham (Academic Medical Center, Amsterdam, Netherlands), both of whom spoke at the workshop. “This is a textbook case of gene detection by use of forward and reverse genetics”, said Jos van der Meer (University Hospital St
Radboud Nijmegen, Netherlands), who discovered HIDS. Nevertheless, it is unclear how a defect in cholesterol synthesis could result in a periodic fever with a strong inflammatory response. Ian Todd and Richard McIntosh (Queen’s Medical Centre, Nottingham, UK) presented data on familial Hibernian fever (FHF), an autosomal dominant periodic fever. They described how they had pinpointed the gene for the tumournecrosis-factor (TNF) receptor as the causative gene for FHF. “There are now families from all over the world with the same genetic defect so I think that we should change the name of FHF to TNF-receptor associated periodic syndrome (TRAPS)”, argued Dan Kastner (National Institutes of Health, Bethesda, MD, USA), who also updated delegates on familial Mediterranean fever (FMF). With the better understanding of
these fevers comes the possibility of new treatments. For FMF, colchicine remains “the mainstay treatment”, said Avi Livneh (Sheba Medical Center, Tel Hashomer, Israel). For TRAPS, early results were presented on the use of injectable soluble TNF receptors. For HIDS, the potential use of thalidomide was discussed by Richard Powell (Queen’s Medical Centre, Nottingham, UK). “It might work, but neuropathy as a sideeffect precludes prolonged use”, he warned. Statins are another potential treatment for HIDS and a clinical trial on these is to start soon. Since the first HIDS workshop in 1995, much has been learnt about periodic fever syndromes. But, said Wietse Kuis (Wilhelmina Children’s Hospital, Utrecht, Netherlands), “much ground still needs to be covered”. Anna Simon, Joost P H Drenth
Many women in quandary about HRT
News in brief
ing either everybody should take it ne third of menopausal women or nobody should take it”, asserts are undecided about hormone Bastian. “But the decision is complireplacement therapy (HRT) and feel cated for the woman and her doctor, they are not getting enough informaand it needs to be individualised.” tion from their physicians to make In another study, Andrea LaCroix an informed choice, report US resear(Group Health Cochers. “We have no operative, Puget interest in having Sound, Seattle, women take it or WA, USA) and colnot—but we have leagues found that to help them make attitudes and beliefs a decision one way about HRT—rather or the other”, says than unbiased assLori Bastian of essment of risks and Durham Veterans benefits—are the Affairs Medical strongest correlates Center, NC, USA. More discussion needed both for a physiBastian and cocian’s prescription of HRT and for a workers studied 318 menopausal woman taking it (Women’s Health women participating in a larger study Issues 1999; 9: 306–18). For example, of decision-making about HRT. 176 only about half of 1083 menopausal had decided to use HRT, and 38 had women agreed that HRT reduced the decided against it; 104 were underisk of osteoporosis—the benefit cided. The undecided women were “most strongly supported by scientific less satisfied than their counterparts certainty”, says LaCroix. “I don’t with the information their health-care know if you can make an informed providers had given them about decision without knowing this.” HRT; they also understood less and “A big message here is that the evithought less about the risks and benedence keeps evolving; it’s a moving fits of HRT. In addition, they felt less target”, concludes LaCroix. “If we capable of making a decision about want people to make informed deciHRT that was right for them sions, they need access to the best (Women’s Health Issues 1999; 9: possible evidence as it emerges.” 330–37). “The information out there is strong and polarised, with forces sayMarilynn Larkin
Oral leishmaniasis treatment The oral phosphocholine analogue miltefosine has shown a cure rate of up to 97% in Indian patients with visceral leishmaniasis. All 120 patients who received 50, 100, or 150 mg miltefosine per day for 4 or 6 weeks had an initial parasitological cure; only six relapsed after 6 months. Gastrointestinal sideeffects were frequent but mild (N Engl J Med 1999; 341: 1795–800).
O
THE LANCET • Vol 354 • December 18/25, 1999
Mapping cancer mortality On Dec 8, the US National Cancer Institute published an updated Atlas of Cancer Mortality in the USA. The atlas covers the years 1950–94, and includes data from more than 3000 counties. As well as being printed, the atlas is also available in an interactive form on the internet at www.nci.nih.gov/atlas. A mouthful of microbes Those of us who are inclined to partake in the seasonal activity of kissing under the mistletoe may want to think twice this year. In studying the flora of the human subgingival crevice, researchers have identified 37 new bacterial phylotypes to add to the 500 strains already identified from the oral cavity (Proc Natl Acad Sci USA 1999; 96: 14547–52).
2141