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Archive for August, 2009

Pulmonary oedema and hyponatraemia after an ironman triathlon

24 Aug, 09 | by Emilia Demetriou

Excessive water drinking during sporting events is sometimes encouraged openly by colleagues and tacitly by the drinks industry. This case highlights the risk of drinking more fluid than you actually need. A reviewer commented “The article makes some excellent points and is very well written. It is an important contribution in the continuing fight to insure that EAHE is properly treated by physicians who may not understand the role of fluid overload and SIADH in this condition and the need to treat all cases with hypertonic saline until the serum sodium concentration has normalized. Had this been followed in this case the athlete would have recovered very quickly.”

Pulmonary oedema and hyponatraemia after an ironman triathlon

Unexpected visual gain with gene therapy suggests new areas for research

16 Aug, 09 | by Dr Dean Jenkins

A pseudo-fovea develops in the gene-therapy treated eye of a patient in a Leber’s Congenital Amaurosis trial. The authors show analysis of this region of cone function outside the fovea and compares it with the early fovea gain seen in the other patients in the trial.

“The unexpected late emergence of visual gain in [this] patient to spatially coded and sustained stimuli and a coincident change in preference for fixation from the fovea to the treated retinal region suggest a slow development of a pseudo-fovea and an underlying experience-dependent plasticity of the adult visual system. These results raise the possibility that this gene-based therapy may further improve visual function in an unexpected and useful way in previously untreatable congenital blindness.”1

1) Cideciyan AV, Hauswirth WW, Aleman TS, Kaushal S, Schwartz SB, Boye SL, Windsor EA, Conlon TJ, Sumaroka A, Roman AJ, Byrne BJ, Jacobson SG. Vision 1 year after gene therapy for Leber’s congenital amaurosis.  N Engl J Med. 2009 Aug 13;361(7):725-7.

Phase 1 trial of Talaporfin (Laserphyrin / NPe6) in neurofibromatosis treats first patient

15 Aug, 09 | by Dr Dean Jenkins

Not a full case report or a medical first but it is the first patient in a phase 1 trial of this intriguing, light-activated chemotherapy drug in children with plexiform neurofibromas.

“Light Sciences Oncology, Inc. (LSO) today announced the treatment of the first patient in an investigator-sponsored Phase 1 pediatric study of its novel light-activated drug Aptocine™ (talaporfin sodium) in neurofibromatosis type 1 (NF-1). Investigators at the Neurofibromatosis/Neuro-Oncology Clinic of Children’s Hospital of Philadelphia (CHOP) treated a pediatric patient with NF-1 and plexiform neurofibromas (PN) using Aptocine, which is also in late-stage development for solid tumors. The Neurofibromatosis/Neuro- Oncology Clinic is a program providing care and treatment for children with brain tumors and children with NF-1 and severe and/or life-threatening tumors.”

Heart transplant record holder dies of cancer

13 Aug, 09 | by Dr Dean Jenkins

“Heart transplant recipient (Tony Huesman) who lived a record 31 years with a single donated organ has died at age 51 of cancer, his heart still going strong, his widow said.”

Associated Press 12th August 2009

When he received the heart transplant in 1978 he was told he may have had 5 years to live. Since then survival has improved but graft survival is usually no more than 15 years1 which makes Mr Huesman’s achievement all the more unique.

1) Dayton JD, Kanter KR, Vincent RN, Mahle WT. Cost-effectiveness of pediatric heart transplantation. J Heart Lung Transplant. 2006 Apr;25(4):409-15. Epub 2006 Feb 8.

First internet-connected pacemaker? No.

11 Aug, 09 | by Dr Dean Jenkins

Recently noticed this claim. “NEW YORK (Reuters) – After relying on a pacemaker for 20 years, Carol Kasyjanski has become the first American recipient of a wireless pacemaker that allows her doctor to monitor her health from afar — over the Internet.

(Strangely it became a World first when taken up by Yahoo!

Dr. Steven Greenberg, the director of St. Francis’ Arrhythmia and Pacemaker Center, said the new technology helps him better treat his patients and will likely become the new standard in pacemakers.

He said the server and the remote monitor communicate at least once a day to download all the relevant information and alert the doctor and patient if there is anything unusual.

If there is anything abnormal, and we have a very intricate system set up, it will literally call the physician responsible at two in the morning if need be,” he said.

The wireless pacemaker, made by St. Jude Medical Inc., received FDA approval in July.

That sounds great … but it certainly isn’t much of a first other than for the St Francis’ centre.

At BMJ Case Reports we published a case of a web-connected implantable cardioverter defibrillator detecting digoxin toxicity a few months ago. That case report cites a paper that evaluated a German system where “devices have an embedded antenna for wireless transmissions of diagnostic information to a Service Center where messages are decrypted, stored as well as loaded on a protected website accessible to the attending physician through identity codes and a personal password“.

A so-called medical breakthrough story changes in the telling.

The Reuter’s story starts:

NEW YORK (Reuters) – After relying on a pacemaker for 20 years, Carol Kasyjanski has become the first American recipient of a wireless pacemaker that allows her doctor to monitor her health from afar — over the Internet.Reuters original

but has been misquoted by Yahoo! News as

NEW YORK (Reuters) – After relying on a pacemaker for 20 years, Carol Kasyjanski has become the world’s first recipient of a wireless pacemaker that allows her doctor to monitor her health from afar — over the Internet.Yahoo!’s misquoted version

‘First American’ becomes ‘World’s first’. Yahoo! News says it “does not write or edit any of the news on our site.” So I wonder how it got changed?

Are news websites in the best position to judge medical firsts?

A case of eyelash transplantation a first in the UK or not?

10 Aug, 09 | by Dr Dean Jenkins

“A woman from Greater Manchester has become the first person in the UK to undergo an eyelash transplant, surgical teams have claimed.

Louise Thomas, 19, from Stockport, had the treatment because she suffers from trichotillomania – obsessive plucking or pulling out hair.”

The surgery was performed by a private cosmetic surgery firm, Transform, who say they were the first to perform it in the UK having used a technique developed in the United States.

They are presumably describing single hair or follicular unit transplants but could this really be the first eyelash case in the UK? Perhaps the first for trichotillomania but what about alopecia or trauma?

Case reports of cancer in children on TNF blockers

9 Aug, 09 | by Dr Dean Jenkins

The US Food and Drug Administration (FDA) is requiring the manufacturers of Tumour Necrosis Factor (TNF) blockers to update the Boxed Warning in the prescribing information to alert healthcare professionals of an increased risk of lymphoma and other malignancies in children and adolescents treated with TNF blockers.

FDA ALERT [8/4/2009]

The FDA analysed 48 cases of malignancy in children taking TNF blockers and concluded that there was a small increased risk that health professionals and patients should be aware of. This is an example of post-marketing surveillance case reports highlighting adverse effects in newly licensed treatments which may not have been apparent in the randomised controlled trials prior to their launch.

Plant sterol enriched margarines increase cardiovascular risk?

8 Aug, 09 | by Dr Dean Jenkins

Maybe the assumption that natural substances can do no harm has again been shown to be wrong. Are these margarines good or bad for you?

A case published in BMJ Case Reports this week raises an interesting question about plant sterol-enriched margarines and cardiovascular risk. They reduce LDL cholesterol but do not seem to reduce cardiovascular risk. In this report a lady developed xanthelasma 18 months into a programme of cholesterol reduction with plant sterols.

Vergès B, Athias A, Petit JM, Brindisi MC. Extravascular lipid deposit (xanthelasma) induced by a plant sterol-enriched margarine. BMJ Case Reports 2009 [doi:10.1136/bcr.10.2008.1108]

The authors point out that elevated campesterol, a plant phytosterol and the cause of the xanthelasma in this case, may promote atherosclerosis. They go on to say that their observation raises concern about potential cardiovascular risk after long-term consumption of these margarines.

Aggressive Infantile Fibromatosis in an Iraqi child treated in the UK

7 Aug, 09 | by Dr Dean Jenkins

The case of three-year-old Saif Basim with Agressive Infantile Fibromatosis is in the news this week. The young boy was flown from Iraq to be treated by British surgeons since the appropriate facilities were not available in Baghdad.

Royal Jordanian airlines paid for the family’s flight to the UK but the British Association of Oral and Maxillofacial Surgery now needs to raise the funds to pay for the operation that members of their society performed.

Management of this size of tumour in a child is clearly a surgical challenge and the team are to be congratulated on their work.

An unusual neurological presentation after exposure to snails.

7 Aug, 09 | by Emilia Demetriou

The reviewer (Dr Marion Woods, Royal Brisbane and Women’s Hospital, Australia) commented:

“The case of Angiostrongylus cantonensis infection with the clinical manifestations of eosinophilic meningo-encephalitis and myeloradiculitis that caused lower limb weakness and bladder dysfunction described in this issue of the journal is the likely consequence of ingestion of a very high worm burden of the L3 infectious larval forms of this rat nematode. The L3 larval content of slugs and snails varies considerably with some snails, such as the African giant land snail, Achotina fulica, containing up to 90,000 L3 larvae (a likely lethal dose). [1] The syndrome of myeloradiculitis with lower limb weakness and bladder dysfunction is a common manifestation of canine Angiostrongylus cantonensis infection in Australia with ingestion of snails and slugs the likely source of infection. [2] Treatment directed at reducing inflammation (corticosteroids) produces better outcomes than treatments directed at killing adult worms in the central nervous system. Our practice is to use a tapering dose of corticosteroids (60 mg/day prednisone for 5-7 days).

For clinicians who have the rare opportunity to intervene after snail or slug ingestion but prior to neurological symptoms, animal studies have shown that drugs such as albendazole (human dose 10 mg/kg/day if > 60 kg, 15 mg/kg/day if < 60 kg) can effectively kill L3 larval stages before they reach the central nervous system. [3] Usually the occurrence of eosinophilic meningitis prompts the question about slug and snail ingestion. Snails and slugs that are infected constantly exude L3 larvae in their wake onto salad vegetables and other raw foods stuffs that may be ingested by unwary future patients. In humans, the median incubation time between ingestion and meningitis symptoms is 11 days with a range of 6-31 days. [4] In animal models, up to half of the ingested L3 larvae are found in the brain as soon as 3 days post ingestion with the majority of L3 larvae found in the brain by day 7 post ingestion. By 30 days nearly all of the worms have migrated from the brain to the pulmonary arteries to become adults. [5]

[1] Wallace GD, Rosen L. Studies on eosinophilic meningitis. V. Molluscan hosts of Angiostrongylus cantonensis on Pacific Islands. The American journal of tropical medicine and hygiene. 1969 Mar;18(2):206-16.

[2] Mason KV. Canine neural angiostrongylosis: the clinical and therapeutic features of 55 natural cases. Australian veterinary journal. 1987 Jul;64(7):201-3.

[3] Lan KP, Wang CJ, Lai SC, Chen KM, Lee SS, Hsu JD, et al. The efficacy of therapy with albendazole in mice with parasitic meningitis caused by Angiostrongylus cantonensis. Parasitology research. 2004 Jul;93(4):311-7.

[4] Slom TJ, Cortese MM, Gerber SI, Jones RC, Holtz TH, Lopez AS, et al. An outbreak of eosinophilic meningitis caused by Angiostrongylus cantonensis in travelers returning from the Caribbean. The New England journal of medicine. 2002 Feb 28;346(9):668-75.

[5] Wallace GD, Rosen L. Studies on eosinophilic meningitis. VI. Experimental infection of rats and other homoiothermic vertebrates with Angiostrongylus cantonensis. American journal of epidemiology. 1969 Mar;89(3):331-44. “

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