BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,skin changes(POEMS)syndrome is a rare paraneoplastic syndrome that encompass multiple systems.The most common clinical symptoms of POEMS syndrome are pro...BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,skin changes(POEMS)syndrome is a rare paraneoplastic syndrome that encompass multiple systems.The most common clinical symptoms of POEMS syndrome are pro-gressive sensorimotor polyneuropathy,organ enlargement,endocrine disorders,darkening skin,a monoclonal plasma cell proliferative disorder,and lymph node hyperplasia.The organomegaly consists of hepatosplenomegaly and/or lym-phadenopathy;cases of cardiomyopathy are rare.Diagnoses are often delayed because of the atypical nature of the syndrome,exposing patients to possibly severe disability.Therefore,identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients.lenalidomide and dexamethasone.CONCLUSION When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin,the POEMS syndrome is the most possible diagnosis.展开更多
BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor.The clinical manifestations of POEMS syndrome a...BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor.The clinical manifestations of POEMS syndrome are diverse.Due to the insidious onset and lack of specific early-stage manifestations,POEMS syndrome is easily misdiagnosed or never diagnosed,leading to delayed treatment.Neurological symptoms are usually the first clinical manifestation,while ascites is a rare symptom in patients with POEMS syndrome.CASE SUMMARY A female patient presented with unexplained ascites as an initial symptom,which is a rare early-stage manifestation of the condition.After 1 year,the patient gradually developed progressive renal impairment,anemia,polyserosal effusion,edema,swollen lymph nodes on the neck,armpits,and groin,and decreased muscle strength of the lower extremities.The patient was eventually diagnosed with POEMS syndrome after multidisciplinary team discussion.Treatment comprised bortezomib+dexamethasone,continuous renal replacement therapy,chest and abdominal closed drainage,transfusions of erythrocytes and platelets,and other symptomatic and supportive treatments.The patient’s condition initially improved after treatment.However,then her symptoms worsened,and she succumbed to the illness and died.CONCLUSION Ascites is a potential early manifestation of POEMS syndrome,and this diagnosis should be considered for patients with unexplained ascites.Furthermore,multidisciplinary team discussion is helpful in diagnosing POEMS syndrome.展开更多
Background:The detection of polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes (POEMS) syndrome at early stage is challenging for neurologists.Since polyneuropathy could be the first manifestation,i...Background:The detection of polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes (POEMS) syndrome at early stage is challenging for neurologists.Since polyneuropathy could be the first manifestation,it could be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP).The present study aimed to determine the clinical and electrophysiological features of POEMS syndrome to distinguish from CIDP.Methods:The data of a group of patients with POEMS (n =17) and patients with CIDP (n =17) in Zhongshan Hospital Fudan University from January 2015 to September 2017 were analyzed in this retrospective study.The clinical features,neurological symptoms,and electrophysiological findings were compared between the two groups.Results:Clinically,patients with POEMS demonstrated significantly more neuropathic pain in the lower extremities than patients with CIDP (58.8 % vs.11.8 %,P =0.01).Multisystem features like edema,skin change,organomegaly,and thrombocytosis were also pointed towards the diagnosis of POEMS syndrome.Electrophysiologically,terminal latency index (TLI) was significantly higher in patients with POEMS than that in patients with CIDP (median nerve:0.39 [0.17-0.52] vs.0.30 (0.07-0.69),Z =-2.413,P =0.016;ulnar nerve:0.55 [0.23-0.78] vs.0.42 [0.12-0.70],Z =-2.034,P =0.042).Patients with POEMS demonstrated a higher frequency of absent compound muscle action potential of the tibial nerve (52.9% vs.17.6%,P =0.031),less conduction block (ulnar nerve:0 vs.35.3%,P =0.018),and less temporal dispersion (median nerve:17.6% vs.58.8%,P =0.032) than CIDP group.The combination of positive serum monoclonal protein and high TLI (if either one or both were present) discriminated POEMS from CIDP with a sensitivity of 94.1% and 47.1% and specificity of 76.5% and 100.0%,respectively.Conclusions:POEMS syndrome could be distinguished from CIDP through typical clinical and electrophysiological characteristics in practice.The combination of serum monoclonal protein and high TLI might raise the sensitivity of de展开更多
AIM:To assess the feasibility of volumetric intensity-modulated arc radiotherapy (VMAT) in patients with limited polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. METHODS:...AIM:To assess the feasibility of volumetric intensity-modulated arc radiotherapy (VMAT) in patients with limited polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. METHODS:A 70-year-old male with histologically confirmed osteosclerotic myeloma was treated in our department in July 2010 with VMAT. Fourty-six Gray in 23 fractions were given on three bone lesions. Doses delivered to target volume and critical organs were compared with a tridimensional conformal radiotherapy (3D-RT) plan. Treatment was well tolerated without any side effects.RESULTS:VMAT improved dose homogeneity within the target volume, as compared to 3D-RT (standard deviations:2.9 Gy and 1.6 Gy for 3D and VMAT, respectively). VMAT resulted in a better sparing of critical organs. Dose delivered to 20% of organ volume (D20) was reduced from 22 Gy (3D-RT) to 15 Gy (VMAT) for small bowel, from 24 Gy (3D-RT) to 17 Gy (VMAT) for bladder and from 47 Gy (3D-RT) to 3 Gy (VMAT) for spinal cord. Volumes of critical organs that received at least 20 Gy (V20) were decreased by the use of VMAT, as compared to 3D-RT (V20 bladder:10% vs 99%; V20 small bowel:6% vs 21%). One year after treatment completion, no tumor progression has been reported. CONCLUSION:VMAT improved dose distribution as compared to 3D-RT for limited osteosclerotic myeloma, with better saving of critical organs.展开更多
Solitary pituitary metastasis is a rare phenomenon in human neoplasms. We report a case of lung cancer with the initial manifestation of endocrinopathy resulting from pituitary metastasis. The patient's initial di...Solitary pituitary metastasis is a rare phenomenon in human neoplasms. We report a case of lung cancer with the initial manifestation of endocrinopathy resulting from pituitary metastasis. The patient's initial diagnosis was a poorly differentiated carcinoma, however, morbid anatomy revealed a definite diagnosis of large cell neuroendocrine carcinoma(LCNEC). Clinical physicians should be aware of potential initial manifestations such as endocrine abnormalities including panhypopituitarism and diabetes insipidus due to solitary pituitary metastasis. This case demonstrates that an endocrine abnormality such as panhypopituitarism could be an initial manifestation of LCNEC.展开更多
Diabetes mellitus is a chronic disease with a world wide distribution. Use of herbal remedies has been on increase with World Health Organization estimating that 80 percent of the world’s population presently uses so...Diabetes mellitus is a chronic disease with a world wide distribution. Use of herbal remedies has been on increase with World Health Organization estimating that 80 percent of the world’s population presently uses some form of herbal medicine for some aspect of primary health care. Objectives of this study were therefore to determine the perceptions people with diabetes mellitus have towards herbal remedies, to determine the extent to which they use herbal remedies and also to establish whether there is any association between the perceptions people have on herbal remedies and use of herbal remedies. The study was carried out in Murang’a District, in Mathioya and Kangema Constituencies where five community health units were purposively selected to participate in the study based on their level of establishment in community health strategy. Data was collected using interview schedules. SPSS was used for data analysis. Significant findings from this study were: a significant number of the respondents (15%) were diagnosed with diabetes mellitus when already admitted in the wards prior to which period they had no idea that they were diabetic, over 86% of those interviewed were given information on diabetes management on diagnosis and they attend hospital clinics for follow-up regularly and therefore this means that the reason for seeking alternative modes of treatment is not due to lack of information on diabetes but due to other reasons, 12.4% of those interviewed admitted using herbal remedies as part of their management of diabetes. Recommendations made following the study were: the government of Kenya through Ministry of Health should encourage rigorous screening of clients and population in general for diabetes to ensure diabetes is diagnosed early and put under appropriate management and that the government of Kenya through Ministry of Health should put up a campaign educating diabetic patients on the potential dangers associated with combining herbal remedies with contemporary medicines due to their interac展开更多
BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a plasma cell proliferative disorder.The syndrome is characterized by elevate...BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a plasma cell proliferative disorder.The syndrome is characterized by elevated plasma cells,platelets,and vascular endothelial growth factor levels.Although heart disease rarely occurs in POEMS syndrome,the death rate increases sharply after heart failure.We report a patient who initially presented with an endocrine disease and developed congestive heart failure related to POEMS syndrome 9 years later.CASE SUMMARY A 23-year-old woman with no history of menstruation and a 9-year history of type I diabetes reported feeling breathless after activities.She could not lie down and rest at night.Three months prior,she experienced pain and increased tension in her left thigh accompanied by tenderness and edema in both lower extremities.The chief complaint upon hospital admission was that blood sugar has increased for more than 9 years,pain in the left thigh,and edema in both legs for more than 2 mo.After a multisystem evaluation,she was diagnosed with POEMS syndrome.Her echocardiogram showed left ventricular dilation with systolic dysfunction,and the left ventricular ejection fraction was only 38%with severely elevated brain natriuretic peptide.She received a combination of dexamethasone and thalidomide for 1 mo,but her symptoms did not improve.Therefore,we added a two-per-week bortezomib injection.After 2 wk,the patient’s heart function had improved significantly.CONCLUSION This case provides information about the treatment of POEMS syndrome with complications and highlights the challenges of developing a standardized treatment.展开更多
A 12-year-old cat, weighting 7.4 kg, suffering from fatigue, exercise intolerance, polyphagia, polyuria and polydipsia was presented. Obesity, a massive head, inferior prognathia and widened inter-dental spaces were n...A 12-year-old cat, weighting 7.4 kg, suffering from fatigue, exercise intolerance, polyphagia, polyuria and polydipsia was presented. Obesity, a massive head, inferior prognathia and widened inter-dental spaces were noted on examination. Radiographic surveys showed organs enlargement. Labaratory results revealed hyperglycemia and glycosuria. Based on fasting hyperglycemia, concurrent hyperglycemia and glycosuria, diagnosis of diabetes mellitus was made. However, according to the poor diabetic regulation, clinical signs and the absence of other diseases, a tentative diagnosis of acromegaly was confirmed by increased plasma levels of growth hormone. Managing diabetes mellitus with increasing doses of insulin was the only possible therapeutic strategy.展开更多
POLYNEUROPATHY, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes （POEMS） syndrome is a rare paraneoplastic syndrome characterized by the presence of a monoclonal plasma cell disorder, peripheral...POLYNEUROPATHY, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes （POEMS） syndrome is a rare paraneoplastic syndrome characterized by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and typical skin abnormalities. The cause of POEMS syndrome is unknown.1 Although numerous cases with POEMS syndrome have been reported, the POEMS syndrome patients with malignant tumor were seldom reported. Here, we present a rare case with breast cancer accompanied by POEMS syndrome.展开更多
文摘BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,skin changes(POEMS)syndrome is a rare paraneoplastic syndrome that encompass multiple systems.The most common clinical symptoms of POEMS syndrome are pro-gressive sensorimotor polyneuropathy,organ enlargement,endocrine disorders,darkening skin,a monoclonal plasma cell proliferative disorder,and lymph node hyperplasia.The organomegaly consists of hepatosplenomegaly and/or lym-phadenopathy;cases of cardiomyopathy are rare.Diagnoses are often delayed because of the atypical nature of the syndrome,exposing patients to possibly severe disability.Therefore,identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients.lenalidomide and dexamethasone.CONCLUSION When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin,the POEMS syndrome is the most possible diagnosis.
文摘BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor.The clinical manifestations of POEMS syndrome are diverse.Due to the insidious onset and lack of specific early-stage manifestations,POEMS syndrome is easily misdiagnosed or never diagnosed,leading to delayed treatment.Neurological symptoms are usually the first clinical manifestation,while ascites is a rare symptom in patients with POEMS syndrome.CASE SUMMARY A female patient presented with unexplained ascites as an initial symptom,which is a rare early-stage manifestation of the condition.After 1 year,the patient gradually developed progressive renal impairment,anemia,polyserosal effusion,edema,swollen lymph nodes on the neck,armpits,and groin,and decreased muscle strength of the lower extremities.The patient was eventually diagnosed with POEMS syndrome after multidisciplinary team discussion.Treatment comprised bortezomib+dexamethasone,continuous renal replacement therapy,chest and abdominal closed drainage,transfusions of erythrocytes and platelets,and other symptomatic and supportive treatments.The patient’s condition initially improved after treatment.However,then her symptoms worsened,and she succumbed to the illness and died.CONCLUSION Ascites is a potential early manifestation of POEMS syndrome,and this diagnosis should be considered for patients with unexplained ascites.Furthermore,multidisciplinary team discussion is helpful in diagnosing POEMS syndrome.
文摘Background:The detection of polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes (POEMS) syndrome at early stage is challenging for neurologists.Since polyneuropathy could be the first manifestation,it could be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP).The present study aimed to determine the clinical and electrophysiological features of POEMS syndrome to distinguish from CIDP.Methods:The data of a group of patients with POEMS (n =17) and patients with CIDP (n =17) in Zhongshan Hospital Fudan University from January 2015 to September 2017 were analyzed in this retrospective study.The clinical features,neurological symptoms,and electrophysiological findings were compared between the two groups.Results:Clinically,patients with POEMS demonstrated significantly more neuropathic pain in the lower extremities than patients with CIDP (58.8 % vs.11.8 %,P =0.01).Multisystem features like edema,skin change,organomegaly,and thrombocytosis were also pointed towards the diagnosis of POEMS syndrome.Electrophysiologically,terminal latency index (TLI) was significantly higher in patients with POEMS than that in patients with CIDP (median nerve:0.39 [0.17-0.52] vs.0.30 (0.07-0.69),Z =-2.413,P =0.016;ulnar nerve:0.55 [0.23-0.78] vs.0.42 [0.12-0.70],Z =-2.034,P =0.042).Patients with POEMS demonstrated a higher frequency of absent compound muscle action potential of the tibial nerve (52.9% vs.17.6%,P =0.031),less conduction block (ulnar nerve:0 vs.35.3%,P =0.018),and less temporal dispersion (median nerve:17.6% vs.58.8%,P =0.032) than CIDP group.The combination of positive serum monoclonal protein and high TLI (if either one or both were present) discriminated POEMS from CIDP with a sensitivity of 94.1% and 47.1% and specificity of 76.5% and 100.0%,respectively.Conclusions:POEMS syndrome could be distinguished from CIDP through typical clinical and electrophysiological characteristics in practice.The combination of serum monoclonal protein and high TLI might raise the sensitivity of de
文摘AIM:To assess the feasibility of volumetric intensity-modulated arc radiotherapy (VMAT) in patients with limited polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. METHODS:A 70-year-old male with histologically confirmed osteosclerotic myeloma was treated in our department in July 2010 with VMAT. Fourty-six Gray in 23 fractions were given on three bone lesions. Doses delivered to target volume and critical organs were compared with a tridimensional conformal radiotherapy (3D-RT) plan. Treatment was well tolerated without any side effects.RESULTS:VMAT improved dose homogeneity within the target volume, as compared to 3D-RT (standard deviations:2.9 Gy and 1.6 Gy for 3D and VMAT, respectively). VMAT resulted in a better sparing of critical organs. Dose delivered to 20% of organ volume (D20) was reduced from 22 Gy (3D-RT) to 15 Gy (VMAT) for small bowel, from 24 Gy (3D-RT) to 17 Gy (VMAT) for bladder and from 47 Gy (3D-RT) to 3 Gy (VMAT) for spinal cord. Volumes of critical organs that received at least 20 Gy (V20) were decreased by the use of VMAT, as compared to 3D-RT (V20 bladder:10% vs 99%; V20 small bowel:6% vs 21%). One year after treatment completion, no tumor progression has been reported. CONCLUSION:VMAT improved dose distribution as compared to 3D-RT for limited osteosclerotic myeloma, with better saving of critical organs.
文摘Solitary pituitary metastasis is a rare phenomenon in human neoplasms. We report a case of lung cancer with the initial manifestation of endocrinopathy resulting from pituitary metastasis. The patient's initial diagnosis was a poorly differentiated carcinoma, however, morbid anatomy revealed a definite diagnosis of large cell neuroendocrine carcinoma(LCNEC). Clinical physicians should be aware of potential initial manifestations such as endocrine abnormalities including panhypopituitarism and diabetes insipidus due to solitary pituitary metastasis. This case demonstrates that an endocrine abnormality such as panhypopituitarism could be an initial manifestation of LCNEC.
文摘Diabetes mellitus is a chronic disease with a world wide distribution. Use of herbal remedies has been on increase with World Health Organization estimating that 80 percent of the world’s population presently uses some form of herbal medicine for some aspect of primary health care. Objectives of this study were therefore to determine the perceptions people with diabetes mellitus have towards herbal remedies, to determine the extent to which they use herbal remedies and also to establish whether there is any association between the perceptions people have on herbal remedies and use of herbal remedies. The study was carried out in Murang’a District, in Mathioya and Kangema Constituencies where five community health units were purposively selected to participate in the study based on their level of establishment in community health strategy. Data was collected using interview schedules. SPSS was used for data analysis. Significant findings from this study were: a significant number of the respondents (15%) were diagnosed with diabetes mellitus when already admitted in the wards prior to which period they had no idea that they were diabetic, over 86% of those interviewed were given information on diabetes management on diagnosis and they attend hospital clinics for follow-up regularly and therefore this means that the reason for seeking alternative modes of treatment is not due to lack of information on diabetes but due to other reasons, 12.4% of those interviewed admitted using herbal remedies as part of their management of diabetes. Recommendations made following the study were: the government of Kenya through Ministry of Health should encourage rigorous screening of clients and population in general for diabetes to ensure diabetes is diagnosed early and put under appropriate management and that the government of Kenya through Ministry of Health should put up a campaign educating diabetic patients on the potential dangers associated with combining herbal remedies with contemporary medicines due to their interac
文摘BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a plasma cell proliferative disorder.The syndrome is characterized by elevated plasma cells,platelets,and vascular endothelial growth factor levels.Although heart disease rarely occurs in POEMS syndrome,the death rate increases sharply after heart failure.We report a patient who initially presented with an endocrine disease and developed congestive heart failure related to POEMS syndrome 9 years later.CASE SUMMARY A 23-year-old woman with no history of menstruation and a 9-year history of type I diabetes reported feeling breathless after activities.She could not lie down and rest at night.Three months prior,she experienced pain and increased tension in her left thigh accompanied by tenderness and edema in both lower extremities.The chief complaint upon hospital admission was that blood sugar has increased for more than 9 years,pain in the left thigh,and edema in both legs for more than 2 mo.After a multisystem evaluation,she was diagnosed with POEMS syndrome.Her echocardiogram showed left ventricular dilation with systolic dysfunction,and the left ventricular ejection fraction was only 38%with severely elevated brain natriuretic peptide.She received a combination of dexamethasone and thalidomide for 1 mo,but her symptoms did not improve.Therefore,we added a two-per-week bortezomib injection.After 2 wk,the patient’s heart function had improved significantly.CONCLUSION This case provides information about the treatment of POEMS syndrome with complications and highlights the challenges of developing a standardized treatment.
文摘A 12-year-old cat, weighting 7.4 kg, suffering from fatigue, exercise intolerance, polyphagia, polyuria and polydipsia was presented. Obesity, a massive head, inferior prognathia and widened inter-dental spaces were noted on examination. Radiographic surveys showed organs enlargement. Labaratory results revealed hyperglycemia and glycosuria. Based on fasting hyperglycemia, concurrent hyperglycemia and glycosuria, diagnosis of diabetes mellitus was made. However, according to the poor diabetic regulation, clinical signs and the absence of other diseases, a tentative diagnosis of acromegaly was confirmed by increased plasma levels of growth hormone. Managing diabetes mellitus with increasing doses of insulin was the only possible therapeutic strategy.
基金Supported by a grant from the Science and Technology Planning Project of Guangdong Province,China(2013B010404023)
文摘POLYNEUROPATHY, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes （POEMS） syndrome is a rare paraneoplastic syndrome characterized by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and typical skin abnormalities. The cause of POEMS syndrome is unknown.1 Although numerous cases with POEMS syndrome have been reported, the POEMS syndrome patients with malignant tumor were seldom reported. Here, we present a rare case with breast cancer accompanied by POEMS syndrome.
