AIM： TO study the implication of prokineticin 1 （PKI/EGVEGF） and prokineticin 2 （PK2/13v8） in hepatocellular carcinoma angiogenesis.METHODS： The gene induction of PK1/EG-VEGF and PK2/Bv8 was investigated in 10 n...AIM： TO study the implication of prokineticin 1 （PKI/EGVEGF） and prokineticin 2 （PK2/13v8） in hepatocellular carcinoma angiogenesis.METHODS： The gene induction of PK1/EG-VEGF and PK2/Bv8 was investigated in 10 normal, 28 fibrotic and 28 tumoral livers by using real time PCR. Their expression was compared to the expression of VEGF （an angiogenesis marker）, vWF （an endothelial cell marker） and to CD68 （a monocyte/macrophage marker）. Furthermore, the rnRNA levels of PK1/EG-VEGF, PK2/Bv8, prokineticin receptor 1 and 2 were evaluated by real time PCR in isolated liver cell populations. Finally, PK2/Bv8 protein was detected in normal liver paraffin sections and in isolated liver cells by immunohistochernistry and immunocytochemistry.RESULTS： PK2/Bv8 mRNA but not PK1/EG-VEGF was expressed in all types of normal liver samples examined. In the context of liver tumor development, we reported that PK2/13v8 correlates only with CD68 and showed a significant decrease in expression as the pathology evolves towards cancer. Whereas, VEGF and vWF mRNA were significantly upregulated in both fibrosis and HCC,as expected. In addition, out of all isolated liver cells examined, only Kupffer cells （liver resident macrophages） express significant levels of PK2/Bv8 and its receptors, prokineticin receptor 1 and 2.CONCLUSION： In normal liver PK2/Bv8 and its receptors were specifically expressed by Kupffer cells. PK2/Bv8 expression decreased as the liver evolves towards cancer and did not correlate with HCC angiogenesis.展开更多
AIM:To compare characteristics and outcomes of resected and nonresected main-duct and mixed intraductal papillary mucinous neoplasms of the pancreas(IPMN).METHODS:Over a 14-year period,50 patients who did not undergo ...AIM:To compare characteristics and outcomes of resected and nonresected main-duct and mixed intraductal papillary mucinous neoplasms of the pancreas(IPMN).METHODS:Over a 14-year period,50 patients who did not undergo surgery for resectable main-duct or mixed IPMN,for reasons of precluding comorbidities,age and/or refusal,were compared with 74 patients who underwent resection to assess differences in rates of survival,recurrence/occurrence of malignancy,and prognostic factors.All study participants had dilatation of the main pancreatic duct by ≥ 5 mm,with or without dilatation of the branch ducts.Some of the nonsurgical patients showed evidence of mucus upon perendoscopic retrograde cholangiopancreatography or endoscopic ultrasound and/or after fine needle aspiration.For the surgical patients,pathologic analysis of resected specimens confirmed a diagnosis of IPMN with involvement of the main pancreatic duct or of both branch ducts as well as the main pancreatic duct.Clinical and biologic follow-ups were conducted for all patients at least annually,through hospitalization or consultation every six months during the first year of follow-up,together with abdominal imaging analysis(magnetic resonance cholangiopancreatography or computed tomography) and,if necessary,endoscopic ultrasound with or without fine needle aspiration.RESULTS:The overall five-year survival rate of patients who underwent resection was significantly greater than that for the nonsurgical patients(74% vs 58%; P =0.019).The parameters of age(< 70 years) and absence of a nodule were associated with better survival(P < 0.05); however,the parameters of main pancreatic duct diameter > 10 mm,branch ductdiameter > 30 mm,or presence of extra pancreatic cancers did not significantly influence the prognosis.In the nonsurgical patients,pancreatic malignancy occurred in 36% of cases within a mean time of 33 mo(median:29 mo; range:8-141 mo).Comparison of the nonsurgical patients who experienced disease progression with those who did not progress showed no significant differences in age,sex,symptoms,subtype of IPMN,or follow-up period; only the size of the main pancreatic duct was significantly different between these two sub-groups,with the nonsurgical patients who experienced progression showing a greater diameter at the time of diagnosis(> 10 mm).CONCLUSION:Patients unfit for surgery have a 36% greater risk of developing pancreatic malignancy of the main-duct or mixed IPMN within a median of 2.5 years.展开更多
AIM: To identify risk factors associated with survival in patients retransplanted for hepatitis C virus(HCV) recurrence and to apply a survival score to this population.METHODS: We retrospectively identified 108 patie...AIM: To identify risk factors associated with survival in patients retransplanted for hepatitis C virus(HCV) recurrence and to apply a survival score to this population.METHODS: We retrospectively identified 108 patients retransplanted for HCV recurrence in eight European liver transplantation centers(seven in France, one in Spain). Data collection comprised clinical and laboratory variables, including virological and antiviral treatment data. We then analyzed the factors associated with survival in this population. A recently published score that predicts survival in retransplantation in patients with hepatitis C was applied. Because there are currently no uniform recommendations regarding selection of the best candidates for retransplantation in this setting, we also described the clinical characteristics of 164 patients not retransplanted, with F3, F4, or fibrosing cholestatic hepatitis(FCH) post-first graft presenting with hepatic decompensation. RESULTS: Overall retransplantation patient survival rates were 55%, 47%, and 43% at 3, 5, and 10 years, respectively. Patients who were retransplanted for advanced cirrhosis had survival rates of 59%, 52%, and 49% at 3, 5, and 10 years, while those retransplanted for FCH had survival rates of 34%, 29%, and 11%, respectively. Under multivariate analysis, and adjusting for the center effect and the occurrence of FCH, factors associated with better survival after retransplantation were: negative HCV viremia before retransplantation, antiviral therapy after retransplantation, non-genotype 1, a Model for End-stage Liver Disease(MELD) score < 25 when replaced on the waiting list, and a retransplantation donor age < 60 years. Although the numbers were small, in the context of the new antivirals era, we showed that outcomes in patientswho underwent retransplantation with undetectable HCV viremia did not depend on donor age and MELD score. The Andrés score was applied to 102 patients for whom all score variables were available, producing a mean score of 43.4(SD = 6.6). Survival rates after the date of the first decompensation post-first liver transplantation(LT1) in the liver retransplantation(re LT) group(94 patients decompensated) at 3, 5, and 10 years were 62%, 59%, and 51%, respectively, among 78 retransplanted individuals with advanced cirrhosis, and 42%, 32%, and 16% among 16 retransplanted individuals with FCH. In the non-re LT group with hepatic decompensation, survival rates were 27%, 18%, and 9% at 3, 5, and 10 years, respectively(P < 0.0001). Compared with non-retransplanted patients, retransplanted patients were younger at LT1(mean age 48 ± 8 years compared to 53 ± 9 years in the no re LT group, P < 0.0001), less likely to have human immunodeficiency virus(HIV) co-infection(4% vs 14% among no re LT patients, P = 0.005), more likely to have received corticosteroid bolus therapy after LT1(25% in re LT vs 12% in the no re LT group, P = 0.01), and more likely to have presented with sustained virological response(SVR) after the first transplantation(20% in the re LT group vs 7% in the no re LT group, P = 0.028).CONCLUSION: Antiviral therapy before and after retransplantation had a substantial impact on survival in the context of retransplantation for HCV recurrence, and with the new direct-acting antivirals now available, outcomes should be even better in the future.展开更多
In children, chronic cervicofacial ulceration related to dental infection is rare. Thus the diagnosis is often late and the treatment is consequently delayed. We report 2 new cases. Cases report.-A 13-year-old boy pre...In children, chronic cervicofacial ulceration related to dental infection is rare. Thus the diagnosis is often late and the treatment is consequently delayed. We report 2 new cases. Cases report.-A 13-year-old boy presented with a 1-year history of chronic and suppurative ulceration on the right cheek. Culture was positive for actinomycetes. In spite of a prolonged and miscellaneous antibiotherapy, the lesion recured. The ulceration healed after the eradication of infection on a right superior molar. A 12-year-old girl presented with a right sub-mandibular ulceration, which appeared 3 months before. This lesion did not respond to penicillinotherapy given during 3 months. An infection on a right inferior molar was diagnosed on a tomodensitometry. 3 months after the tooth extraction, the ulceration healed without recurrence. Conclusion.-These cases emphasize the interest to look for a dental infection at the origin of chronic cervicofacial lesion.展开更多
Objectives. -To know nosocomial infections (NI) rates among newborns and hospitalised children according to age and units of hospitalisation, Methods. -In 2001, the Comitétechnique des infections nosocomiales (CT...Objectives. -To know nosocomial infections (NI) rates among newborns and hospitalised children according to age and units of hospitalisation, Methods. -In 2001, the Comitétechnique des infections nosocomiales (CTIN) initiated a one-day prevalence survey on an arranged date. It was conducted by the 5 centres de coordination de lutte contre les infections nosocomiales (C.CLIN). Paediatric data only were analysed dividing them into 2 groups: newborns (NB) up to 28 days old, whether in maternity, neonatology or intensive care units, and children (CH) from 29 days to 18 years old with separate analysis for surgery, cancer units and intermediate care and rehabilitation centres (ICRC). Results. -The survey included 21 596 children under 18 years old: 9136 NB and 12,460 CH, i.e. 7.1%of the total hospital population on that one given day. A total of 2.6%of the patients were infected, with 1.2%of NB and 3.3%of CH. There were 1.09 infections per patient. 456 different microorganisms were responsible for 562 infections, i.e. 81.1%: 18,6%Staphylococcus aureus of which 26.7%were methicillin resistant (MRSA), 21.9%Staphylococcus epidermis, 21.9%enterobacteries, 10.3%Pseudomonas aeruginosa, 4.4%Candida albicans, and 4.2%rotavirus. NB and CH infection factors were studied separately. Selective criteria for hospitalised NB were: gender: male(P < 0.05), level of gravity (P < 0.05), surgery (P < 0.001), catheter (P < 0.01) and hospital type (P < 0.01). For EN, criteria were severity score (P < 0.01), surgery (P < 0.001), catheter (P < 0.01), and hospital type (P < 0.01). The rate for patients hospitalised in cancer units was 13.2%with no significant factors found. The rate of infected patients in intensive care units was 7.8%for NB and 16.2%for CH, with 9.6%neonatal, 30.8%heart condition, 26.3%non-specialised units and 14.2%children units. ICRC infection rate was 4.5%while surgery units had a rate of 1.9%with 2.4%orthopaedic, 0.7%children units, 2.0%non-specialised units and 8.1%neurosurgery. Discussion. -The 2001 NI level showed a significant fall from 3.5%in the 1996 survey (all ages) to 2.4%. However, including newborns from private maternity units into the survey may have partly lowered this level. MRSA levels have also dropped from 46%in 1996 to 27%in 2001. It is difficult to compare international data as definitions vary. Furthermore, it should be possible to separate newborns from older children as well as specifying the type of units involved. Infected patients levels are generally lower in the French surveys, no matter the age group or units studied than in other surveys. Conclusion. -This survey has enabled the monitoring of the children population in maternity units, on a given day, in France. It showed that the rate of NI in the paediatric population seems low compared to previous data or other countries’data. Efforts need to be made in order to lower it further. Incidence surveillance method is preferable to single out which risk factors can be acted upon. Standardized national protocols are offered to maternity units and should soon be available for newborns units as well as older children units.展开更多
Background: Primary cutaneous cribriform carcinoma (PCCC) is a rare apocrine tumour occurring in middle-aged people. This neoplasm is often located on the limbs. The histopathological diagnosis is difficult, mainly be...Background: Primary cutaneous cribriform carcinoma (PCCC) is a rare apocrine tumour occurring in middle-aged people. This neoplasm is often located on the limbs. The histopathological diagnosis is difficult, mainly because this tumour is exceptional. We, in this study, report a patient with PCCC. Case report: The patient was a 37-year-old man who presented with a nodule of the left knee. Results: Histopathologic findings showed an asymmetrical deep dermal tumour with a cribriform pattern. The aggregations of neoplastic cells were interconnected and varied in size and shape. The cells were arranged in solid nests or tubular stru ctures. In the lumina of tubules, some papillary protrusion of basophilic cells was seen. The ductal elements were lined by cuboidal or cylindric cells with ima ges of decapitation secretion. The nuclei of the neoplastic cells were pleomorph ic. A wide excision was performed with sentinel inguinal node biopsy. After a 2 -year follow-up, neither persistence at the local site nor metastasis was obse rved. Conclusions: Clinical and pathological features of PCCCare reviewed. Diffe rential diagnoses, including cutaneous metastasis of adenocarcinoma, adenoid bas al cell carcinoma and adenoid cystic carcinoma, are discussed.展开更多
Background. DisuloneTM （dapsone +iron oxalate） is a sulfone used in the treatment of numerous skin diseases. We report two cases of hepatosiderosis secondary to long-term administration of DisuloneTM. Patients and m...Background. DisuloneTM （dapsone +iron oxalate） is a sulfone used in the treatment of numerous skin diseases. We report two cases of hepatosiderosis secondary to long-term administration of DisuloneTM. Patients and methods. Case n<sub>1. A 51-year-old manwas treated with DisuloneTM for a neutrophilic skin disease. After 17 years of treatment, elevated serumferritin and free iron with hemolysis were found. Liver biopsy confirmed hepatosiderosis. A diagnosis of genetic hemochromatosis was ruled out by the absence of C282Y mutation of the HFE gene. Case n<sub>2. A 52-year-old man receiving DisuloneTM for dermatitis herpetiformis for 25 years presented elevated serumferritin and free iron with hemolysis. Hepatic iron overload was confirmed by liver biopsy. The absence of C282Y mutation （HFE gene）-ruled out a diagnosis of genetic hemochromatosis. Discussion. In our two cases, hepatosiderosis was noted after long-term administration of DisuloneTM. This complication has been reported only rarely. In murine models, a relationship was found between prolonged administration of dapsone and hepatic iron overload as revealed by hemolysis. Although it is difficult to extrapolate this relationship to humans with any certainty, our patients had also chronic hemolysis and iron overload secondary to administration of DisuloneTM. Moreover in France, dapsone is marketed in combination with iron oxalate, with the attendant risk of iron overload. These cases raise the question of the need for serum ferritin analysis during DisuloneTM therapy.展开更多
基金INSERM,the Ministère de l'Education Nationale de la Recherche et de la Technologie,the Région Bretagne.No.2079
文摘AIM： TO study the implication of prokineticin 1 （PKI/EGVEGF） and prokineticin 2 （PK2/13v8） in hepatocellular carcinoma angiogenesis.METHODS： The gene induction of PK1/EG-VEGF and PK2/Bv8 was investigated in 10 normal, 28 fibrotic and 28 tumoral livers by using real time PCR. Their expression was compared to the expression of VEGF （an angiogenesis marker）, vWF （an endothelial cell marker） and to CD68 （a monocyte/macrophage marker）. Furthermore, the rnRNA levels of PK1/EG-VEGF, PK2/Bv8, prokineticin receptor 1 and 2 were evaluated by real time PCR in isolated liver cell populations. Finally, PK2/Bv8 protein was detected in normal liver paraffin sections and in isolated liver cells by immunohistochernistry and immunocytochemistry.RESULTS： PK2/Bv8 mRNA but not PK1/EG-VEGF was expressed in all types of normal liver samples examined. In the context of liver tumor development, we reported that PK2/13v8 correlates only with CD68 and showed a significant decrease in expression as the pathology evolves towards cancer. Whereas, VEGF and vWF mRNA were significantly upregulated in both fibrosis and HCC,as expected. In addition, out of all isolated liver cells examined, only Kupffer cells （liver resident macrophages） express significant levels of PK2/Bv8 and its receptors, prokineticin receptor 1 and 2.CONCLUSION： In normal liver PK2/Bv8 and its receptors were specifically expressed by Kupffer cells. PK2/Bv8 expression decreased as the liver evolves towards cancer and did not correlate with HCC angiogenesis.
文摘AIM:To compare characteristics and outcomes of resected and nonresected main-duct and mixed intraductal papillary mucinous neoplasms of the pancreas(IPMN).METHODS:Over a 14-year period,50 patients who did not undergo surgery for resectable main-duct or mixed IPMN,for reasons of precluding comorbidities,age and/or refusal,were compared with 74 patients who underwent resection to assess differences in rates of survival,recurrence/occurrence of malignancy,and prognostic factors.All study participants had dilatation of the main pancreatic duct by ≥ 5 mm,with or without dilatation of the branch ducts.Some of the nonsurgical patients showed evidence of mucus upon perendoscopic retrograde cholangiopancreatography or endoscopic ultrasound and/or after fine needle aspiration.For the surgical patients,pathologic analysis of resected specimens confirmed a diagnosis of IPMN with involvement of the main pancreatic duct or of both branch ducts as well as the main pancreatic duct.Clinical and biologic follow-ups were conducted for all patients at least annually,through hospitalization or consultation every six months during the first year of follow-up,together with abdominal imaging analysis(magnetic resonance cholangiopancreatography or computed tomography) and,if necessary,endoscopic ultrasound with or without fine needle aspiration.RESULTS:The overall five-year survival rate of patients who underwent resection was significantly greater than that for the nonsurgical patients(74% vs 58%; P =0.019).The parameters of age(< 70 years) and absence of a nodule were associated with better survival(P < 0.05); however,the parameters of main pancreatic duct diameter > 10 mm,branch ductdiameter > 30 mm,or presence of extra pancreatic cancers did not significantly influence the prognosis.In the nonsurgical patients,pancreatic malignancy occurred in 36% of cases within a mean time of 33 mo(median:29 mo; range:8-141 mo).Comparison of the nonsurgical patients who experienced disease progression with those who did not progress showed no significant differences in age,sex,symptoms,subtype of IPMN,or follow-up period; only the size of the main pancreatic duct was significantly different between these two sub-groups,with the nonsurgical patients who experienced progression showing a greater diameter at the time of diagnosis(> 10 mm).CONCLUSION:Patients unfit for surgery have a 36% greater risk of developing pancreatic malignancy of the main-duct or mixed IPMN within a median of 2.5 years.
基金Supported by A research grant from Sao Paulo Research Foundation(FAPESP grant number 2012/03895-6)
文摘AIM: To identify risk factors associated with survival in patients retransplanted for hepatitis C virus(HCV) recurrence and to apply a survival score to this population.METHODS: We retrospectively identified 108 patients retransplanted for HCV recurrence in eight European liver transplantation centers(seven in France, one in Spain). Data collection comprised clinical and laboratory variables, including virological and antiviral treatment data. We then analyzed the factors associated with survival in this population. A recently published score that predicts survival in retransplantation in patients with hepatitis C was applied. Because there are currently no uniform recommendations regarding selection of the best candidates for retransplantation in this setting, we also described the clinical characteristics of 164 patients not retransplanted, with F3, F4, or fibrosing cholestatic hepatitis(FCH) post-first graft presenting with hepatic decompensation. RESULTS: Overall retransplantation patient survival rates were 55%, 47%, and 43% at 3, 5, and 10 years, respectively. Patients who were retransplanted for advanced cirrhosis had survival rates of 59%, 52%, and 49% at 3, 5, and 10 years, while those retransplanted for FCH had survival rates of 34%, 29%, and 11%, respectively. Under multivariate analysis, and adjusting for the center effect and the occurrence of FCH, factors associated with better survival after retransplantation were: negative HCV viremia before retransplantation, antiviral therapy after retransplantation, non-genotype 1, a Model for End-stage Liver Disease(MELD) score < 25 when replaced on the waiting list, and a retransplantation donor age < 60 years. Although the numbers were small, in the context of the new antivirals era, we showed that outcomes in patientswho underwent retransplantation with undetectable HCV viremia did not depend on donor age and MELD score. The Andrés score was applied to 102 patients for whom all score variables were available, producing a mean score of 43.4(SD = 6.6). Survival rates after the date of the first decompensation post-first liver transplantation(LT1) in the liver retransplantation(re LT) group(94 patients decompensated) at 3, 5, and 10 years were 62%, 59%, and 51%, respectively, among 78 retransplanted individuals with advanced cirrhosis, and 42%, 32%, and 16% among 16 retransplanted individuals with FCH. In the non-re LT group with hepatic decompensation, survival rates were 27%, 18%, and 9% at 3, 5, and 10 years, respectively(P < 0.0001). Compared with non-retransplanted patients, retransplanted patients were younger at LT1(mean age 48 ± 8 years compared to 53 ± 9 years in the no re LT group, P < 0.0001), less likely to have human immunodeficiency virus(HIV) co-infection(4% vs 14% among no re LT patients, P = 0.005), more likely to have received corticosteroid bolus therapy after LT1(25% in re LT vs 12% in the no re LT group, P = 0.01), and more likely to have presented with sustained virological response(SVR) after the first transplantation(20% in the re LT group vs 7% in the no re LT group, P = 0.028).CONCLUSION: Antiviral therapy before and after retransplantation had a substantial impact on survival in the context of retransplantation for HCV recurrence, and with the new direct-acting antivirals now available, outcomes should be even better in the future.
文摘In children, chronic cervicofacial ulceration related to dental infection is rare. Thus the diagnosis is often late and the treatment is consequently delayed. We report 2 new cases. Cases report.-A 13-year-old boy presented with a 1-year history of chronic and suppurative ulceration on the right cheek. Culture was positive for actinomycetes. In spite of a prolonged and miscellaneous antibiotherapy, the lesion recured. The ulceration healed after the eradication of infection on a right superior molar. A 12-year-old girl presented with a right sub-mandibular ulceration, which appeared 3 months before. This lesion did not respond to penicillinotherapy given during 3 months. An infection on a right inferior molar was diagnosed on a tomodensitometry. 3 months after the tooth extraction, the ulceration healed without recurrence. Conclusion.-These cases emphasize the interest to look for a dental infection at the origin of chronic cervicofacial lesion.
文摘Objectives. -To know nosocomial infections (NI) rates among newborns and hospitalised children according to age and units of hospitalisation, Methods. -In 2001, the Comitétechnique des infections nosocomiales (CTIN) initiated a one-day prevalence survey on an arranged date. It was conducted by the 5 centres de coordination de lutte contre les infections nosocomiales (C.CLIN). Paediatric data only were analysed dividing them into 2 groups: newborns (NB) up to 28 days old, whether in maternity, neonatology or intensive care units, and children (CH) from 29 days to 18 years old with separate analysis for surgery, cancer units and intermediate care and rehabilitation centres (ICRC). Results. -The survey included 21 596 children under 18 years old: 9136 NB and 12,460 CH, i.e. 7.1%of the total hospital population on that one given day. A total of 2.6%of the patients were infected, with 1.2%of NB and 3.3%of CH. There were 1.09 infections per patient. 456 different microorganisms were responsible for 562 infections, i.e. 81.1%: 18,6%Staphylococcus aureus of which 26.7%were methicillin resistant (MRSA), 21.9%Staphylococcus epidermis, 21.9%enterobacteries, 10.3%Pseudomonas aeruginosa, 4.4%Candida albicans, and 4.2%rotavirus. NB and CH infection factors were studied separately. Selective criteria for hospitalised NB were: gender: male(P < 0.05), level of gravity (P < 0.05), surgery (P < 0.001), catheter (P < 0.01) and hospital type (P < 0.01). For EN, criteria were severity score (P < 0.01), surgery (P < 0.001), catheter (P < 0.01), and hospital type (P < 0.01). The rate for patients hospitalised in cancer units was 13.2%with no significant factors found. The rate of infected patients in intensive care units was 7.8%for NB and 16.2%for CH, with 9.6%neonatal, 30.8%heart condition, 26.3%non-specialised units and 14.2%children units. ICRC infection rate was 4.5%while surgery units had a rate of 1.9%with 2.4%orthopaedic, 0.7%children units, 2.0%non-specialised units and 8.1%neurosurgery. Discussion. -The 2001 NI level showed a significant fall from 3.5%in the 1996 survey (all ages) to 2.4%. However, including newborns from private maternity units into the survey may have partly lowered this level. MRSA levels have also dropped from 46%in 1996 to 27%in 2001. It is difficult to compare international data as definitions vary. Furthermore, it should be possible to separate newborns from older children as well as specifying the type of units involved. Infected patients levels are generally lower in the French surveys, no matter the age group or units studied than in other surveys. Conclusion. -This survey has enabled the monitoring of the children population in maternity units, on a given day, in France. It showed that the rate of NI in the paediatric population seems low compared to previous data or other countries’data. Efforts need to be made in order to lower it further. Incidence surveillance method is preferable to single out which risk factors can be acted upon. Standardized national protocols are offered to maternity units and should soon be available for newborns units as well as older children units.
文摘Background: Primary cutaneous cribriform carcinoma (PCCC) is a rare apocrine tumour occurring in middle-aged people. This neoplasm is often located on the limbs. The histopathological diagnosis is difficult, mainly because this tumour is exceptional. We, in this study, report a patient with PCCC. Case report: The patient was a 37-year-old man who presented with a nodule of the left knee. Results: Histopathologic findings showed an asymmetrical deep dermal tumour with a cribriform pattern. The aggregations of neoplastic cells were interconnected and varied in size and shape. The cells were arranged in solid nests or tubular stru ctures. In the lumina of tubules, some papillary protrusion of basophilic cells was seen. The ductal elements were lined by cuboidal or cylindric cells with ima ges of decapitation secretion. The nuclei of the neoplastic cells were pleomorph ic. A wide excision was performed with sentinel inguinal node biopsy. After a 2 -year follow-up, neither persistence at the local site nor metastasis was obse rved. Conclusions: Clinical and pathological features of PCCCare reviewed. Diffe rential diagnoses, including cutaneous metastasis of adenocarcinoma, adenoid bas al cell carcinoma and adenoid cystic carcinoma, are discussed.
文摘Background. DisuloneTM （dapsone +iron oxalate） is a sulfone used in the treatment of numerous skin diseases. We report two cases of hepatosiderosis secondary to long-term administration of DisuloneTM. Patients and methods. Case n<sub>1. A 51-year-old manwas treated with DisuloneTM for a neutrophilic skin disease. After 17 years of treatment, elevated serumferritin and free iron with hemolysis were found. Liver biopsy confirmed hepatosiderosis. A diagnosis of genetic hemochromatosis was ruled out by the absence of C282Y mutation of the HFE gene. Case n<sub>2. A 52-year-old man receiving DisuloneTM for dermatitis herpetiformis for 25 years presented elevated serumferritin and free iron with hemolysis. Hepatic iron overload was confirmed by liver biopsy. The absence of C282Y mutation （HFE gene）-ruled out a diagnosis of genetic hemochromatosis. Discussion. In our two cases, hepatosiderosis was noted after long-term administration of DisuloneTM. This complication has been reported only rarely. In murine models, a relationship was found between prolonged administration of dapsone and hepatic iron overload as revealed by hemolysis. Although it is difficult to extrapolate this relationship to humans with any certainty, our patients had also chronic hemolysis and iron overload secondary to administration of DisuloneTM. Moreover in France, dapsone is marketed in combination with iron oxalate, with the attendant risk of iron overload. These cases raise the question of the need for serum ferritin analysis during DisuloneTM therapy.
