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P422 Poems syndrome: comparison of 2 cases in 20 years
SISO
Abstracts
I European
Journal
of Internal
vs. 23.08% p=O.19), and non-surgical patients (11.76% vs. 60.00%, p=O.O26). Conclusions: Higher prophylaxis awareness, unnecessary interventions in low risk patients, and less prophylaxis omissions suggest a higher alertness among physicians. The lack of adherence, particularly in VHR and MR patients, suggests that risk criteria only have a limited influence on physician behavior. New strategies should be considered.
P422 Poems syndrome: comparison of 2 cases E. Munez, I. Perales, A. Noblejas, M.J. (Madrid, E)
in 20 years Garcia Navarro,
F. Martin
Poems syndrome is a rare multisistemic disease associated with polyneruopathy. endocrinopathy, a monoclonal protein and skin changes, that occurs in the seting of a plasma cell dyscrasia. We describe 2 patients with poems syndrome diagnosed in 20 years. Case I: A 50.year-old woman with a history of raynaud phenomenon was seen in our hospital in 1977 for lower extremities weakness, paresthesias and fever of 38 “C. Physical examination showed hepatosplenomegaly, hands skin thickening, hirsutism, lower extremities edema and motor and sensory deficiency with archilles and patellar reflexes diminished. Lab studies were normal. Serum protein electrophoresis showed a monoclonal spike of iga lambda. Despite rectum and skin biopsies were negative, she was diagnosed of amiloidosis and treated with melphalan and prednisone with symptoms improvement. 2 years later, she was readmitted because of a bilateral symmetric motor and sensory distal lower limbs and diabetes mellitus. Bone marrow biopsy revealed myeloma and poems syndrome diagnoses was made. With same treatment she improved. Case 2: 41-year-old man with a history of polycytemia vera begins with lower extremities weakness and paresthesias with areflexia and hyperpigmentation. Electromyography study shows a motor and sensory polyneuropathy, both demyelination and axonal. Lab studies shows primary hypotiroidism, monoclonal gammapathy of igg lamda, and toraco-abdominal scan homogeneous hepatomegaly, and a litic, expansive lesion on ischiopubic area of 7 cm and another on sacro which biopsy suggests plasmacytoma. With the diagnosis of poems syndrome we began radiotherapy of solitary lesions with poor improvement of neurologic symptoms. Poems syndrome presentation is common, with polyneuropathy and monoclonal plasmaproliferative disorder as defining feature of poems. Treatment and prognosis depends of the extent of he underlying plasma cell disorder and its response to treatment. Even if the plasmacytoma responds completely, some symptoms such as neurologic defects and functional loss due to tightening of the skin, may be permantent
P423 Congenital asplenia with multiple additional the Ivemark syndrome R. Zarrabeitia, G. Santiago, A. Perez de1 Molino, Escalada, R. Arjona (Cantabria, E)
anomalies: J.A. Parra,
variant
of
B. Gutidrrez
Introduction: Heterotaxy syndrome (Ivemark or asplenia-polysplenia syndrome) is a heterogeneous group of diseases with abnormal symmetry and malposition of internal organs, sometimes with complex cardiac malformations. It is caused by the disturbance of the left/right axis in the early embrionic period and several cases of recessive, dominant and X linked inheritance, have been described. We present a patient with a non previosly reported association of anomalies in this syndrome. Case report: A girl aged 18 attended our service because of detection of Howell-Jolly bodies in a routine control of anovulatory treatment. She denied previous infectious diseases and there was no history of congenital
Medicine
14 (2003)
Sl-S159
defects in her family. The physical exploration was normal and also the current serum determinations except for the presence of Howell-Jolly and spiculed red cells. Abdominal ultrasound, angio CT scan and angio Nuclear Magnetic Resonance were performed and asplenia, pancreatic tail agenesia and bowel right position with colon situated in the left side were disclosed. Moreover an anomaly in the drainage of inferior cava vein was observed with absence of the final portion of this one, a prominent hemiazigos conecting with the superior cava and suprahepatic veins ending directly in the right atrium. The chest X-ray was normal and the ecocardiography did not find defects but the direct drainage of the suprahepatic veins in the right atrium.Vaccination against meningococcus, neumococcus and haemophilus was indicated. A prophylactic analysis and abdominal ultrasound were indicated to her sister showing no anomalies. The patient remains assymptomatic but aware of increased infectious risk. Discussion: This patient’s phenotype comprises a previosly undescribed combination of major and minor clinical features of the Ivemark syndrome. The incidence is very low. New babies born in families with a history of congenital visceral heterotaxy or other malformations associated with Ivemark syndrome, should be tested for asplenia, primarily by searching for Howell-Jolly bodies.
P424 Analysis of patient-doctor Internet in Switzerland M. Uhlmann, A. Fischer,
interaction S. Reichlin
on (Basel,
the
phone
and
via
the
CH)
Doctors regularly advise or care for patients over the phone. In addition, the number of dedicated service centres in Europe responding to patients’ requests and giving medical advice over the phone and via the Internet is increasing. The triage process is an important aspect in the evaluation of a medical problem. The decision on when, where and how to respond to the problem plays a major role in the patients consequent behaviour and influences the use of health care resources. In spite of growing demand for telemedical advice there are relatively few studies evaluating the use, quality and outcome of phone or Internet-based advice or triage services, People seeking help or requiring information can contact the Swiss telemedical advice centre Medgate around the clock. A specifically trained team of doctors responds to medical questions either via phone, e-mail or online. In this report we present demographic data of users and types of services provided between 01.01.2001 and 31.12.2002 and the results of a Likert-type questionnaire that were sent to a consecutive subgroup of patients. After exclusion of administrative calls, 47769 individuals were given advice during the study period. The three most frequently provided types were triage (59%). general medical counselling (32%) and second opinion requests (3%). Contacts via Internet represented a small (1%) but growing number of people seeking advice. Calls lasted 12 minutes on average, and 50% were received outside of regular office hours. Women aged 25-45 years made 60% of calls. The content covered the whole spectrum of medicine, with focus on general medicine, infectious diseases and paediahics. 12,681 patients answered the questionnaire (59% of recipients). 90% of respondents state to have followed the given advice, and were satistied with the service in 89-96% of cases, depending on the quality criteria. In conclusion, the data shows that telemedical advice is a growing demand in Switzerland, that the quality requirement of the callers is met and that the advice is generally adhered to.
P425 Primary biliary cirrhosis associated with vasculitis: a case report I. Lino, F. Bastida, A. Sousa, R. Matono, Guilherme (l?vora, Almada, P)
a central T. Alves,
nervous
system
I. Mendes,
L.
Abstracts
I European
Journal
of Internal
vs. 23.08% p=O.19), and non-surgical patients (11.76% vs. 60.00%, p=O.O26). Conclusions: Higher prophylaxis awareness, unnecessary interventions in low risk patients, and less prophylaxis omissions suggest a higher alertness among physicians. The lack of adherence, particularly in VHR and MR patients, suggests that risk criteria only have a limited influence on physician behavior. New strategies should be considered.
P422 Poems syndrome: comparison of 2 cases E. Munez, I. Perales, A. Noblejas, M.J. (Madrid, E)
in 20 years Garcia Navarro,
F. Martin
Poems syndrome is a rare multisistemic disease associated with polyneruopathy. endocrinopathy, a monoclonal protein and skin changes, that occurs in the seting of a plasma cell dyscrasia. We describe 2 patients with poems syndrome diagnosed in 20 years. Case I: A 50.year-old woman with a history of raynaud phenomenon was seen in our hospital in 1977 for lower extremities weakness, paresthesias and fever of 38 “C. Physical examination showed hepatosplenomegaly, hands skin thickening, hirsutism, lower extremities edema and motor and sensory deficiency with archilles and patellar reflexes diminished. Lab studies were normal. Serum protein electrophoresis showed a monoclonal spike of iga lambda. Despite rectum and skin biopsies were negative, she was diagnosed of amiloidosis and treated with melphalan and prednisone with symptoms improvement. 2 years later, she was readmitted because of a bilateral symmetric motor and sensory distal lower limbs and diabetes mellitus. Bone marrow biopsy revealed myeloma and poems syndrome diagnoses was made. With same treatment she improved. Case 2: 41-year-old man with a history of polycytemia vera begins with lower extremities weakness and paresthesias with areflexia and hyperpigmentation. Electromyography study shows a motor and sensory polyneuropathy, both demyelination and axonal. Lab studies shows primary hypotiroidism, monoclonal gammapathy of igg lamda, and toraco-abdominal scan homogeneous hepatomegaly, and a litic, expansive lesion on ischiopubic area of 7 cm and another on sacro which biopsy suggests plasmacytoma. With the diagnosis of poems syndrome we began radiotherapy of solitary lesions with poor improvement of neurologic symptoms. Poems syndrome presentation is common, with polyneuropathy and monoclonal plasmaproliferative disorder as defining feature of poems. Treatment and prognosis depends of the extent of he underlying plasma cell disorder and its response to treatment. Even if the plasmacytoma responds completely, some symptoms such as neurologic defects and functional loss due to tightening of the skin, may be permantent
P423 Congenital asplenia with multiple additional the Ivemark syndrome R. Zarrabeitia, G. Santiago, A. Perez de1 Molino, Escalada, R. Arjona (Cantabria, E)
anomalies: J.A. Parra,
variant
of
B. Gutidrrez
Introduction: Heterotaxy syndrome (Ivemark or asplenia-polysplenia syndrome) is a heterogeneous group of diseases with abnormal symmetry and malposition of internal organs, sometimes with complex cardiac malformations. It is caused by the disturbance of the left/right axis in the early embrionic period and several cases of recessive, dominant and X linked inheritance, have been described. We present a patient with a non previosly reported association of anomalies in this syndrome. Case report: A girl aged 18 attended our service because of detection of Howell-Jolly bodies in a routine control of anovulatory treatment. She denied previous infectious diseases and there was no history of congenital
Medicine
14 (2003)
Sl-S159
defects in her family. The physical exploration was normal and also the current serum determinations except for the presence of Howell-Jolly and spiculed red cells. Abdominal ultrasound, angio CT scan and angio Nuclear Magnetic Resonance were performed and asplenia, pancreatic tail agenesia and bowel right position with colon situated in the left side were disclosed. Moreover an anomaly in the drainage of inferior cava vein was observed with absence of the final portion of this one, a prominent hemiazigos conecting with the superior cava and suprahepatic veins ending directly in the right atrium. The chest X-ray was normal and the ecocardiography did not find defects but the direct drainage of the suprahepatic veins in the right atrium.Vaccination against meningococcus, neumococcus and haemophilus was indicated. A prophylactic analysis and abdominal ultrasound were indicated to her sister showing no anomalies. The patient remains assymptomatic but aware of increased infectious risk. Discussion: This patient’s phenotype comprises a previosly undescribed combination of major and minor clinical features of the Ivemark syndrome. The incidence is very low. New babies born in families with a history of congenital visceral heterotaxy or other malformations associated with Ivemark syndrome, should be tested for asplenia, primarily by searching for Howell-Jolly bodies.
P424 Analysis of patient-doctor Internet in Switzerland M. Uhlmann, A. Fischer,
interaction S. Reichlin
on (Basel,
the
phone
and
via
the
CH)
Doctors regularly advise or care for patients over the phone. In addition, the number of dedicated service centres in Europe responding to patients’ requests and giving medical advice over the phone and via the Internet is increasing. The triage process is an important aspect in the evaluation of a medical problem. The decision on when, where and how to respond to the problem plays a major role in the patients consequent behaviour and influences the use of health care resources. In spite of growing demand for telemedical advice there are relatively few studies evaluating the use, quality and outcome of phone or Internet-based advice or triage services, People seeking help or requiring information can contact the Swiss telemedical advice centre Medgate around the clock. A specifically trained team of doctors responds to medical questions either via phone, e-mail or online. In this report we present demographic data of users and types of services provided between 01.01.2001 and 31.12.2002 and the results of a Likert-type questionnaire that were sent to a consecutive subgroup of patients. After exclusion of administrative calls, 47769 individuals were given advice during the study period. The three most frequently provided types were triage (59%). general medical counselling (32%) and second opinion requests (3%). Contacts via Internet represented a small (1%) but growing number of people seeking advice. Calls lasted 12 minutes on average, and 50% were received outside of regular office hours. Women aged 25-45 years made 60% of calls. The content covered the whole spectrum of medicine, with focus on general medicine, infectious diseases and paediahics. 12,681 patients answered the questionnaire (59% of recipients). 90% of respondents state to have followed the given advice, and were satistied with the service in 89-96% of cases, depending on the quality criteria. In conclusion, the data shows that telemedical advice is a growing demand in Switzerland, that the quality requirement of the callers is met and that the advice is generally adhered to.
P425 Primary biliary cirrhosis associated with vasculitis: a case report I. Lino, F. Bastida, A. Sousa, R. Matono, Guilherme (l?vora, Almada, P)
a central T. Alves,
nervous
system
I. Mendes,
L.