Rapid Responses to:
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Rapid Responses: Rapid Responses published:
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Next Step
- John McCormack (8 September 2006)
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History of raw seafood consumption
- Petar I Ivanovski (8 September 2006)
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PUO - Young Woman
- Srinath Meadipudi (8 September 2006)
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Agree--Treat on spec!!
- Stephen R Workman (8 September 2006)
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A generalist takes a shot at it
- Ed Cooper (8 September 2006)
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being practical
- Elizabeth Howard (9 September 2006)
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haemophagocytic syndrome
- jonathan p kerr (10 September 2006)
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Common things are common
- RAGHAVA S K BHAMIDIMARRI (11 September 2006)
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Moderate Steroids why wait
- Michael L Loren (13 September 2006)
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Sure of the diagnosis ?
- Henry W Mannings (13 September 2006)
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Pyrexia of unknown origin
- Ramachandran Sivakumar, Spencer Ellis, spencer.ellis@nhs.net, Consultant physician and rheumatologist, Lister hospital, Stevenage (14 September 2006)
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Take another history
- Stephen J Katona (14 September 2006)
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Anaplasma phagocytophilum
- David Mitchell (14 September 2006)
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Lemierre syndrome
- David Mitchell (14 September 2006)
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Erythema marginatum ?
- Stephen J Katona (15 September 2006)
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C3 Nephritic Factor ?
- Stephen J Katona (15 September 2006)
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Did she go for a swim?
- Ben Ridwan (18 September 2006)
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fever of unknown origin
- Raul E. Marchena (19 September 2006)
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The patient perspective
- Spencer I Ellis, Ramachandran Sivakumar (19 September 2006)
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Fever of unknown origin ......Answers to the questions asked
- Dr Vinod kumar Kumar (20 September 2006)
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Reactive hemophagocytic syndrome (RHPS)
- Prasad R Koduri (24 September 2006)
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John McCormack, GP Rosmuc, Connemara, Ireland
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1) Request for second external opinion. Organise it immediately without quibble or any suggestion that treating physicians may feel hurt or undermined by the request. It is sometimes not enough to provide the best of care but to be seen to do so. The family need this as they need to know that everything that can be done is being done and they also need to feel that they have some input even if that is by requesting a second opinion. Their daughter is in extremis and they will not be able to shoulder the uncertainty that comes with a lack of diagnosis. I doubt that a second opinion will contribute to diagnosis beyond what is already being done. 2) features in support of adult onset Still's disease. PUO/arthropathy / inflammation / waxing and waning rash / benign lymphadenopathy / ferritin / hx sore throat /. To get this far I have had to read more on Still's disease. Features against stills disease- ?.My knowlege of this condition is limited and my experience of it is non existant 3) What do you do next. This woman is deteriorating rapidly. Doing nothing looks as if it will cause harm. Immediate immunosuppression. ? methylprednisalone bolus while awaiting methotrexate to start working. The worst immediate harm that large dose IV steroids could do is fan the flames of an undiagnosed disseminated TB infection (would anyone consider empirical anti TB rx in conjunction). TB is unlikely as progression/ deterioration is more rapid than one would expect. I am still unclear about the diagnosis and appropriate treatment and I hope the ultimate outcome is positive. Whether it was or was not I think it might be of some benefit to the family to direct them to the interactive case discussion on this site to allow them see the level of uncertainty surrounding their daughter's illness (amongst a wide range of specialities). good luck. Competing interests: None declared |
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Petar I Ivanovski, Pediatrician University Children's Hospital, Belgrade,Serbia
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In order to help the patient, I suggest heteroanamnestic data about consumption of raw seafood (ingestion of Vibrio vulnificus)during the patient's stay in Italy. Competing interests: None declared |
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Srinath Meadipudi, Staff Grade Woodend Hospital,NHS Grampian,Aberdeen,AB15 6XS
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This is a very unfortunate case and the distress experienced by the patient,parents/family members and the physicians treating her can be understandable. 1. I think we should respect the views of the parents and seek a second medical opinion.However, the family should be explained and should be aware that the patient is getting all the necessary care and we are doing all the investigations and treatment possible.Obviously,thats our main aim anyway.I think presenting the case here on BMJ is one of the best strategy of obtaining a second opinion which is really good and generating different opinions and suggestions.The other option is to have an interactive discussions through videoconferencing which is available at many teaching hospitals and to involve experts from both nationally and Internationally if possible and present the case for discussion. The presence of the parents at the conference would be helpful so that they can understand the gravity of the situation and it would also build up the trust on those who are treating the patient. 2. swinging fever associated with rash ( non pruritic), joint tenderness,age of onset,raised ferritin levels,multi organ involvement,Swelling of the lymph glands are all in favour of Stills Disease. Hypocomplimentaemia is against stills disease 3. I think she is getting the correct treatment at this stage and the main aim is to prevent multiorgan failure. In general, managing stills disease - Many symptoms of Still’s disease can be controlled with anti-inflammatory drugs or nonsteroidal anti-inflammatory drugs (NSAIDs – pronounced En-sedz). Cortisone medications such as prednisone are also used to treat the more severe symptoms of the disease. As Still’s disease often affects internal organs, some people with the disease require heart and lung medication as well as medication for diabetes. Pain medication is often necessary and used as required depending on the severity of symptoms. For patients with persistent illness, the medications for Still’s disease are similar to those used to treat rheumatoid arthritis. These include hydroxychloroquine (Plaquenil), penicillamine, azathioprine (Imuran), methotrexate and cyclophosphamide. Gold injections are also used. A new class of drugs called biological response modifiers (BRMs), including such drugs as Enbrel, Remicade, and Humira, is showing some promise for treating the disease. Re:http://www.arthritis.ca/types%20of%20arthritis/Stills/default.asp?s=1 Competing interests: http://www.arthritis.ca/types%20of%20arthritis/Stills/default.asp?s=1 |
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Stephen R Workman, Assistant Professor QEII HSC Halifax Nova Scotia Canada, B2H 3Y9
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Based on the following article I would estimate the pretest probability of Still's to be over 90%. The mortality rate without treatment is likely high--I would think it would be over 50%. Against this one must consider the risk of high dose steroids. I have never seen a patient die of high dose steroids, not that it does not happen; but I have seen many patients die of multi system failure. Almost any risk benefit calculation at this point would strongly favour treatment. In this case, and in many others, the quest for diagnostic certainty before initiating treatment is elusive and potentially dangerous. A second opinion would be welcome but should not delay the initiation of treatment. Rev Rhum Engl Ed. 1995 Dec;62(11):748-57. Links Adult Still's disease: part I. Manifestations and complications in sixty- five cases in France.Masson C, Le Loet X, Liote F, Renou P, Dubost JJ, Boissier MC, Brithmer L, Bregeon C, Audran M. Department of Rheumatology, Angers, France. DESIGN: a retrospective multicenter study conducted in France identified 65 cases of adult Still's disease. Data were recorded on a standardized questionnaire validated by the Inflammatory Joint Disease and Immunorheumatology Committee of the Societe Francaise de Rhumatologie. OBJECTIVES: (1) To compare clinical and laboratory findings in our patients with those reported in earlier studies, particularly two large series from Canada and Japan, respectively; (2) to describe the systemic and visceral complications associated with adult Still's disease. RESULTS: as compared with the two above-mentioned series, our study group included more patients who had experienced onset of their disease after the age of 35 years and fewer patients with involvement of the liver, spleen, or lymph nodes. Rates of occurrence of arthritis, myalgia, sore throat, pleuritis, pericarditis, and abdominal pain were significantly higher in the Canadian series than in the other two series. Arthritis was absent in one fourth of our patients. Life-threatening complications included "Still's hepatopathy", disseminated intravascular coagulation (with hemophagocytosis in some cases), and "Still's myocarditis". CONCLUSION: differences in the expression of adult Still's disease were found between patients from Canada, France, and Japan. Adult Still's disease can be responsible for life-threatening complications. PMID: 8869216 [PubMed - indexed for MEDLINE] Competing interests: None declared |
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Ed Cooper, Semi-retired pediatrician London
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The family have an absolute right to a second opinion. As a generalist myself I would be seeking help from an experienced rheumatologist, if possible from a tertiary centre. E-mail discussion, e.g. through the relevant specialty group in doctors.net, etc. is also a useful resource. Adult onset Still's disease, or any sort of Still's disease with disseminated intravascular coagulation, heart failure and renal failure, is rare; and here we have a 19-year-old whose life is in grave danger. In talking to the family, I would emphasise that Still's disease looks more and more likely to be the diagnosis. I would say that our simultaneous search for other possible diagnoses, by multiple tests for sometimes highly obscure or unlikely diseases, is a matter of leaving no stone unturned - in the patient's interest - rather than an admission that "we are baffled". Adult-onset Still's fits the picture best. We do not need to show certainty to show confidence, but we do need to show some confidence. By the way, is it a limitation of the particular assay used that we can only get serum ferritin as "very high" ">1500 ng/mL"? It looks from the literature as if an actual concentration (e.g. 9600 ng/mL or whatever) would be valuable, as the specificity of serum ferritin for adult Still's correlates with its magnitude. For the second opinion of the family's choice, we may conceivably have to manage some unfavourable contingencies. The family might insist on an infectious disease specialist rather than a rheumatologist. This consultant might show their expertise by producing for them an enormous list of very rare but not impossible conditions. Fair enough, but this will not really help the family. Worse, the family might take friends' recommendations and find a convincing but unorthodox practitioner who produces a firm diagnosis which the NHS team finds hard to swallow. This might need to be countered by third and fourth opinions called in by the hospital team, with a great deal of explanation always conducted without antagonism. Body language and voice control are vital skills here. As for the specific treatment of Still's disease, I would be just taking the advice of the senior rheumatologist or possibly a consensus of senior rheumatologists. I think pulsed methylprednisolone is likely to be the essence of this. I would not "cover" this with treatment for tuberculosis. The presentation is nothing like any form of tuberculosis. HIV testing is not a bad idea when we are doing so many tests for so many improbable infections. HIV testing should become a part of routine checking with very little fuss made of it in the 21st century. However, in this case the result will make no difference to management, it can certainly be deferred, it is the opportunistic infections themselved that we are searching for and that we would need to treat if we found them. But the diagnosis in this young woman is much more likely to be adult onset Still's disease. Competing interests: None declared |
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Elizabeth Howard, GP RM7
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1. I would support the family's request for a second opionion without feeling undermined by it. She has become rapidly and seriously unwell from a point of little/ no limitation and is continuing to deteriorate despite your efforts. The biggest question is whose opinion should she have? 2. My little oxford handbood of GP says that the high swinging temperatures, rash, arthritis, lymphadenopathy, raised CRP, raised neutrophils and negative auto antibodies all support Stills disease. But I would keep an open mind and be prepared to review the evidence regularly. 3. I would go with the above and throw in the high dose steroids WHILST WAITING for the second opinion. We know that steroids have a protective nature in sepsis and she has had heavy duty antibiotics. The inflammatory response needs tempered before it kills her. Many Parents are steroid phobic but a chat about how she is likely to deteriorate again if you don't act now should bring them round. 4. Save serum for retrospective analysis and do a summary sheet of investigations/ findings to aid reviews. Competing interests: None declared |
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jonathan p kerr, haematology research fellow Southampton University, so16 6yd
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I just wondered if this might be virus associated haemophagocytic lympho-histiocytosis. In particular, the need anaemia, thrombocytopaenia, hypofibrinogenaemia, swinging fever, rash, lymph node and hyperferritinaemia. It would be useful to know the results of triglyceride levels. I remember a similar case in a teenage girl - the diagnosis is an uncertain one, and the treatment is complex and potentially dangerous. Has she had a bone marrow aspirate performed? Even if she has it may be worth repeating, since the first marrow is often negative. There's more information at http://www.histio.org/site/apps/nl/content2.asp?c=kiKTL4PQLvF&b=1850031&ct=2592057 . It's an important diagnosis to make, since effective treatment exists (Blood, 1 October 2002, Vol. 100, No. 7, pp. 2367-2373), Best of luck, Paul Kerr Competing interests: None declared |
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RAGHAVA S K BHAMIDIMARRI, SPECIALIST REGISTRAR- GENERAL MEDICINE HD2 1NX
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Having gone through other responses, I don't intend to repeat things. I think Infection vs Autoimmune is the summary of this story. The checklist:
Competing interests: None declared |
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Michael L Loren, Private Practice Lee's Summit, Missouri 64064
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The serum ferritin is likely acting as an acute phase reactant/ and totally nonspecific: high with infection and, malignancy and auto-immune arthritis. This rapid deterioration without a clear infectious agent needs empiric from the hip treatment: Steroids in mod to high doses to cover the "Still Disease" and decrease the cytokine storm, multiple antibiotics to cover what we can possibly treat. In the US, lyme disease a spirochetal disease, commonly missed usually results in death with the same pattern: renal and respiratory failure. Competing interests: None declared |
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Henry W Mannings, locum general practitioner NR18 9TF
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1. As other respondents, I would wholeheartedly agree for a second medical opinion especially in view of her extreme condition. Despite receiving excellent supportive care, the diagnosis remains elusive.In this situation, it would be arrogant to suggest that no one else would be able to shed any further light on the problem even if this was the case.The most important thing to ensure is that in the parents eyes everything possible has been done to procure the survival of their daughter. 2.Most of the features besides low complement levels support the diagnosis of Stills disease but the paucity of articular manifestations as well as lack of myalgia makes me uncomfortable with a definitive diagnosis of Stills. 3.The chronicity of the illness producing DIC suggests a persisting trigger which maybe of external origin i.e. infection or internal origin i.e. autoimmune disease or a malignancy releasing antigens such as leukaemia or lymphoma.In view of this i would ask for a bone marrow aspirate which may direct us towards a malignancy or possibly an infection such as leishmaniasis. The latter is endemic in southern europe spread by sandfly bites and can produce severe illness although the lack of splenomegaly would be unexpected. It may be worthwhile re-examining the lymph node biopsy with this in mine. Competing interests: None declared |
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Ramachandran Sivakumar, Consultant Physician, Ipswich hospital sivasiva51@hotmail.com Ipswich, Spencer Ellis, spencer.ellis@nhs.net, Consultant physician and rheumatologist, Lister hospital, Stevenage
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We are delighted with the number of responses our case report has elicited. Regarding the query about the absolute value of ferritin, serum ferritin assay available in our centre could not quantify the exact value above 1500. Further, Drs Elizabeth Howard and Ed Cooper are absolutely right in raising whose opinion should be sought as a second opinion given the multiorgan involvment? Should it be a rheumatologist, cardiologist, microbiologist,nephrologist etc? We would welcome any suggestions in this regard. Competing interests: None declared |
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Stephen J Katona, GP Herefordshire OOH / locum
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Referral to another, provides a fresh start with eyes unclouded... Take another history from the patient by herself and explain that anything she says is in confidence. What did she do / where did she go whilst on holiday (walking in the mountains - ?Lyme disease). Any other travel ? Explore whether she is at risk of a sexually transmitted disease (?Neisseria Gonorrhoea in view of painful knee / Syphilis - fleeting rash) , or shared needles in the past. Does she use tampons, and could one be retained ? Is there a family history of severe infections, or problems dealing with them. Does she has a vaginal discharge ? Did she develop DIC shortly after being giving antibiotics (excessive bacterial kill can lead to massive cytokine release and dic/multiorgan failure - cf. the Jarisch Herxheimer reaction after syphilis is treated with penicillin). A better description of the rash - was it itchy, could it have been erythema nodosum. Were blood cultures taken after antibiotics, and how many sets have been taken. Have TB cultures been requested ? Re-examine including fundi. Is there any evidence of a peripheral neuropathy ? What does the throat look like. What does examination of the knee show ? Look for roth spots, splinters, oslers nodes, pustules, petechiae etc. Is her spleen enlarged/tender (due to deposition of immune complexes) - examine in right lateral position. A high CRP makes SLE unlikely (although vasculitis in SLE could cause the CRP to rise a bit). The low C3 and C4 is therefore probably due to consumption rather than deficiency. Immune complex formation would be the usual mechanism, accounting for arthralgia (knee), myalgia, renal failure, and abnormal GGT. The high CRP and neutrophil would suggest a bacterial infection although neutrophils can rise purely due to a significant stress response. What does the differential count show - is there a lymphopenia, or eosinophilia ? Ferritin is simply an acute phase reactant, and is measured far more rapidly by the CRP. It takes time to make specific antibodies to an infection, so much of the initial serology including ASOT could have been falsely negative. Is the CK significantly raised (ie. thousands ) to suggest a myositis ? The ASOT in particular needs to be repeated. It is after all, a RISE in the titre which confirms the presence of a streptococcal infection, and there is a lot going for rheumatic fever/endocarditis following a strep sore throat. Take note of the appearance of a reactive lymph node on the ride of the neck. Is this in the correct position to be secondary to the sore throat ? ASOT needs a least a week to rise and will be at its highest 3-4 weeks after the sore throat. Remember that lab tests or laboratory workers are not infallible. If in doubt, repeat the test. Without the above additional information I think the most likely
diagnosis is some form of bacterial infection.
In order of likelihood:
While investigating I would start blind broad spectrum antibiotics capable of covering many atypical infections (discuss with a microbiologist) and keep away from immunosuppressive drugs. Competing interests: None declared |
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David Mitchell, Department of microbiology Trinity College, Dublin
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This case is becoming most interesting. The working diagnosis still appears to be Still's disease. I have to confess that I have never seen a case of Still's disease become as complicated as this before - but I am not a rheumatologist. I previously have listed most of the possible infectious causes and have seen them ruled out in the on going presentation. Now reading of this deterioration in the clinical condition I am now dredging the depths of the more unusual infectious causes. One possibility that has not been mentioned yet is human granulocytic anaplasmosis. Since this is a condition likely to be known only to infectious disease specialists and microbiologists I will provide some backgound. The organism has been recognised as a vetinary pathogen since 1932 and as a human pathogen since 1990 initially in the US but since then also in Europe. Like Lyme disease it is undiagnosed. The organism is spread by ticks of the Ixodes genus. The usual vector in Europe is I. ricinus. The usual reservoir are small rodents. Dogs, cats, horses, sheep and deer are also infected. Transmission in ticks is by the transstadal route rather than the transovarial. The organism is located in the salivary glands of the tick and is injected during feeding. Clinical presentation is variable. Fever is the most common feature with raised liver enzymes, rash, respiratory infiltrates, arthitis and gastroentestinal disturbances reported. Complications include septic or toxic shock-like syndrome, coagulopathy, atypical pneumonitis/acute respiratory distress syndrome (ARDS), acute abdominal syndrome, rhabdomyolysis, myocarditis, acute renal failure, hemorrhage, brachial plexopathy, demyelinating polyneuropathy, cranial nerve palsies and opportunistic infections. Fatal cases have been reported. There have been less than one hundred cases reported in Europe to date. In studies seroprevelence seems to lie between 6 and 20% suggesting that asymptomatic infection is common. One difficulty with these figures is that we are currently unsure of the rate of cross reactivity of this organism with other pathogens. The organism infects polymorphs. A blood film will show what appear to be gram positive cocci with the polymorph. The diagnosis can be easily missed as a result. The mention of multiple blood films negative for malaria to my way of thinking increases this possibility here but I may be reading too much into this. The organism causes multiple changes to the polymorphs metabolism that are still being worked out. This include a down regulation of the apotosis mechanisms. The pathogensis of the disease seems to involve distrubances of various cytokines and interference with the metabolism of the polymorphs. The organism belongs to the group of organisms known as the Rickettsiae. This group has undergone considerable taxonomic revisions in the last few years and these revisions seem likely to continue. In broad strokes the organism is a gram negative coccobacillus of the alpha division of the Enterobacteriaceae and appears to be an obligate intracellular pathogen. Diagnosis may be made by examination of blood smears may be used to look for characteristic morulae (microcolonies). The diagnosis should be confirmed by PCR, immunostaining or serology. Because of its intracellular location choice of antibiotics is limited. Current recomendations are for 10-14 days of deoxycycline. Given how ill this young woman was I would be very tempted to add in rifampacin or rifabutin since these are active against most bacteria and penetrate most eukaryotic cells. I am not aware of any reports supporting this suggestion but it seems unlikely to do any additional harm. These latter two drugs do have interactions with other medication so a review of the pharmacology would be indicated before using either of them. To answer the questions raised in the article 1. I would agree that a second opinion would be very helpful here. I would suggest a review by infectious diseases/microbiology and rheumatology if the patient was not being care for by either of these. I would also very very tempted to ask for permission to present this at a grand round. If you put enough doctors in a room and present a case it is very rare that someone has never seen anything like this before. 2. Here I will defer to my rheumatological collegues. 3. I think supportive care while a diagnosis is being considered would seem sensible. Others here have suggested immunosupression. I am not entirely happy that an infectious cause has been ruled out and that immunosupression might not be the best way forward. That having been said given that rheumatological opinion still favours Still's disease and that this woman is very ill I would be inclined to defer to my rheumatological collegues opinion here. Competing interests: None declared |
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David Mitchell, Department of Microbiology Trinity College, Dublin
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This condition was first described in the Lancet in 1936. It is a pyemia secondary to a sore throat and is usually caused by Fusobacterium necrophorans or Fusobacterium nucleatum. The syndrome itself is pharyngitis followed by sepsis complicated by internal jugular vein thrombosis and infectious metastatic abscesses. The condition has been reported world wide and is considered a rare diagnosis. It does not fit with the 'insect bite' but that may be taking Osler's dictum too far. I would be very disturbed if the blood cultures which we have been assured were consistently negative failed to grow anything and this turned out to be the diagnosis. If the patient was on antibiotics before this would make growing the organism less likely but I hope this was taken into consideration. The diagnosis is still possible in spite of the negative blood cultures. The sore throat in the presentation was mentioned but not emphasised. Normally in this condition the throat abcess is much more painful than a usual sore throat. There is no mention of jugular vein thromobosis or evidence of metastatic abcesses. Treatmemt is with metronidazole, supportive care and surgery. ENT opinion should always be sought in such a case. On the balance I would find this diagnosis improbable given the negative blood cultures and seemingly atypical presentation but I am grateful for the other poster for raising this possibility. Competing interests: None declared |
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Stephen J Katona, GP OOH, locum
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'A rash not evident during ward rounds' A rash that comes and goes in the early stages of rheumatic fever. http://www.gpnotebook.co.uk/simplepage.cfm?ID=-2140471290 I would seriously consider managing this as a case of rheumatic fever. Plenty of criteria have been met, we just need proof of streptococcal infection. http://www.gpnotebook.co.uk/simplepage.cfm?ID=- 1294991341&linkID=29747&cook=yes I'd ask the microbiologist to see if the technicians could do another ASOT titre today (or other specific test) or send serum by courier to a lab that could. A combination of penicillin and prednisolone wouldn't be a bad start today, and see if the CRP has fallen tomorrow. Competing interests: None declared |
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Stephen J Katona, OOH / locum Hereford
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Immune complex deposition would normally lower the C4 rather than the C3, so it might be worth testing for C3 Nephritic Factor. Severe sepsis in itself, is also a recognized cause of low c3 and c4. Hypocomplementaemia of poststreptococcal acute glomerulonephritis is associated with C3 nephritic factor (C3NeF) IgG autoantibody activity. Fremeaux-Bacchi V, Weiss L, Demouchy C, May A, Palomera S, Kazatchkine MD. Department of Immunology, INSERM U28, Hopital Broussais, Paris, France. We have observed that decreased plasma levels of C3 in the serum of three children with poststreptococcal acute glomerulonephritis (PSAGN) at the time of presentation were associated with the presence of C3NeF activity in purified serum IgG from the patients. C3NeF activity was determined using a sensitive assay measuring the ability of patients' IgG to stabilize a preformed cell-bound alternative pathway convertase complex. C3NeF activity of patients' IgG decreased within weeks as plasma levels of C3 progressively returned to normal values. C3NeF activity became undetectable within 1-4 months following normalization of plasma C3 levels. Our observations suggest that early alternative pathway-dependent hypocomplementaemia, a cardinal feature of PSAGN, is mediated by the transient expression of C3NeF autoantibody activity by patients' IgG. PMID: 7708258 [PubMed - indexed for MEDLINE] Competing interests: None declared |
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Ben Ridwan, Medical microbiologist UMC Utrecht, the Netherlands
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I didn't see anyone suggesting Leptospirosis (Weil's syndrome). I would try to confirm the diagnosis by antigen testing, serology and try to culture it from the urine or CSF. Competing interests: None declared |
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Raul E. Marchena, Paediatrics Hospital Infantil de Infectologia, Guatemala, CA 01009
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I think this young lady could have SLE (lupus) due to the clinic manifestations at the begining and the evolution, with kidney involvement. Competing interests: None declared |
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Spencer I Ellis, Consultant Physician and Rheumatologist Lister Hospital, Stevenage, Herts SG1 4AB, Ramachandran Sivakumar
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Written by the patient with the support of Dr Ellis. I have had the opportunity to read all the responses. It is interesting now to see how many different possible causes there might have been for the problems that I was suffering from. I learnt from my parents after I had been moved from intensive care that they had found it difficult to accept that all the investigations were negative after nearly four weeks in hospital. This meant that the doctors were unsure of a definite diagnosis although one was given as the most likely. In the absence of a clear answer, they were concerned about a missed diagnosis and losing me, their youngest daughter. There was never any doubt in their minds that a second opinion could only be helpful to all concerned. Competing interests: None declared |
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Dr Vinod kumar Kumar, consultant in Medicine G B PANT Hospital, New Delhi-110002
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1.Differential diagnosis should also include Malaria,Dengue ,Rocky mountain spotted fever and Ehrlichiosis in view of fever and rashes in a 19years old girl.I strogly feel about the possiblity of rocky mountain spotted fever in view of type and distribution of rashes. There is a definite history of insect/mosquito bite in Italy. May be a tick bite ??? Epidemiology also supports rocky mountain spotted fever. Absence of leucopenia and thrombocytopenia means that the possiblity of Ehrlichiosis is less likely.Tests for malarial parasite are negative. Dengue serology should be done though the total wbc count is against the possiblity. 2.Skin biopsy from the rash and immunofluorescence staining,acute and convalescent serology for R. Rickettsii. 3.Patient and relatives should be assured and told that team of doctors is trying to do its best.(IF the diagnosis is correct it is treatable with Doxycycline 100mg per oral or IV BD for 5 days. Competing interests: None declared |
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Prasad R Koduri, Consultant hematologist Lincolnwood, IL. ISA 60712
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The diagnostic considerations in this patient would include reactive hemophagocytic syndrome complicating Adult onset Stills disease, and malignant lymphoma with hemophagocytosis ("primry bone marrow lymphoma" and hepato-splenic T-cell lymphoma, and angiotropic lymphoma T or B with hemophagocytosis). Hyponatremia, hypofibrinogenemia, and unexplained drops in the hemoglobin and thrombocytopenia suggest a considertion of RHPS. A review of the lymph node biopsy and bone marrow biopsy for evidence of hemophagocytic histiocytosis and for evidence of lymphomatous infiltrate as well as a skin biopsy would be helpful. Serum ferritin values over 100,000 ng/ml have only been reported in RHPS and in patients with Stills disease. A diagnosis of RHPS in this young woman with multiorgan dysfunction would be an indication for etoposide therapy for stabilizing her clinical condition followed by appropriate therapy for the underlying condition. To save time, I find it useful to alert the laboratory to do serial dilutions of the serum as needed and report the final value for serum ferritin in situations where I suspect extreme hyperferritinemia. Competing interests: None declared |
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