BACKGROUND In Wilson disease lack of biliary copper excretion causes hepatocellular injury by accumulation of free toxic copper.Its overspill to serum accounts for neuronal damage as second common manifestation.Therap...BACKGROUND In Wilson disease lack of biliary copper excretion causes hepatocellular injury by accumulation of free toxic copper.Its overspill to serum accounts for neuronal damage as second common manifestation.Therapy with copper chelators or zinc targets the removal of this free copper.However,in some patients liver disease persists for unknown reason despite normalized free copper.The discovery of a hyperimmunity as a contributing pathogenetic factor was discovered in this case report with implication also for other liver diseases.CASE SUMMARY A 9-year-old girl was diagnosed in August 2009 by family screening of having asymptomatic Wilson disease with elevated transaminases.Already at time of diagnosis antinuclear antibodies(ANA)were elevated without hyperimmunoglobulinemia(immunoglobulin G,IgG).After one year of therapy with Dpenicillamine transaminases normalized together with free serum copper.Under continuous therapy with copper chelators free copper remained normal until today,whereas transaminases raised to alanine aminotransferase values of 571 U/L in December 2019.For hyperimmunity a tentative steroid course on top of Dpenicillamine improved transaminases.Thus,hyperimmunity may have impact on liver inflammation after control of the metabolic disturbance.A retrospective cohort study confirmed the common association of elevated transaminases with ANA,but no IgG elevation.CONCLUSION This hyperimmune-triggered condition may represent a new entity which per se or on top of other liver diseases induces liver inflammation responsive to steroids.展开更多
Germ cell tumours (GCTs) of the ovary are rare, comprising approximately 20% of all ovarian tumors with the malignant variant accounting for less than 5% of all ovarian neoplasms. Malignant mixed germ cell tumours are...Germ cell tumours (GCTs) of the ovary are rare, comprising approximately 20% of all ovarian tumors with the malignant variant accounting for less than 5% of all ovarian neoplasms. Malignant mixed germ cell tumours are rarer still with dysgerminoma & yolk sac tumour being the most common components with three component variants being categorised in the rarest of rare varieties. Bilateral dysgerminomas occur in 15% of germ cell neoplasms but rarely present with premature ovarian failure. We present the case of an adolescent girl with a short history of abdominal pain and distension with amenorrhoea for a year. Clinical and radio ogical examination revealed a pelvic/adnexal mass with elevation of tumour markers and she had to undergo a staging laparotomy with bilateral salpingo-oophorectomy. Histopatholgy examination was reported as malignant mixed germ cell tumour of left ovary, predominantly with immature teratoma and minor components of yolk-sac tumour and dysgerminoma and right ovary with dysgerminoma. In view of mixed germ cell disease, she was planned for adjuvant chemotherapy.展开更多
There have been many chapters written about macrophage polarization.These chapters generally focus on the role of macrophages in orchestrating immune responses by highlighting the T-cell-derived cytokines that shape t...There have been many chapters written about macrophage polarization.These chapters generally focus on the role of macrophages in orchestrating immune responses by highlighting the T-cell-derived cytokines that shape these polarizing responses.This bias toward immunity is understandable,given the importance of macrophages to host defense.However,macrophages are ubiquitous and are involved in many different cellular processes,and describing them as immune cells is undoubtedly an oversimplification.It disregards their important roles in development,tissue remodeling,wound healing,angiogenesis,and metabolism,to name just a few processes.In this chapter,we propose that macrophages function as transducers in the body.According to Wikipedia,WA transducer is a device that converts energy from one form to another.^The word transducer is a term used to describe both the"sensor,which can interpret a wide range of energy forms,and the"actuator,which can switch voltages or currents to affect the environment.Macrophages are able to sense a seemingly endless variety of inputs from their environment and transduce these inputs into a variety of different response outcomes.Thus,rather than functioning as immune cells,they should be considered more broadly as cellular transducers that interpret microenvironmental changes and actuate vital tissue responses.In this chapter,we will describe some of the sensory stimuli that macrophages perceive and the responses they make to these stimuli to achieve their prime directive,which is the maintenance of homeostasis.展开更多
文摘BACKGROUND In Wilson disease lack of biliary copper excretion causes hepatocellular injury by accumulation of free toxic copper.Its overspill to serum accounts for neuronal damage as second common manifestation.Therapy with copper chelators or zinc targets the removal of this free copper.However,in some patients liver disease persists for unknown reason despite normalized free copper.The discovery of a hyperimmunity as a contributing pathogenetic factor was discovered in this case report with implication also for other liver diseases.CASE SUMMARY A 9-year-old girl was diagnosed in August 2009 by family screening of having asymptomatic Wilson disease with elevated transaminases.Already at time of diagnosis antinuclear antibodies(ANA)were elevated without hyperimmunoglobulinemia(immunoglobulin G,IgG).After one year of therapy with Dpenicillamine transaminases normalized together with free serum copper.Under continuous therapy with copper chelators free copper remained normal until today,whereas transaminases raised to alanine aminotransferase values of 571 U/L in December 2019.For hyperimmunity a tentative steroid course on top of Dpenicillamine improved transaminases.Thus,hyperimmunity may have impact on liver inflammation after control of the metabolic disturbance.A retrospective cohort study confirmed the common association of elevated transaminases with ANA,but no IgG elevation.CONCLUSION This hyperimmune-triggered condition may represent a new entity which per se or on top of other liver diseases induces liver inflammation responsive to steroids.
文摘Germ cell tumours (GCTs) of the ovary are rare, comprising approximately 20% of all ovarian tumors with the malignant variant accounting for less than 5% of all ovarian neoplasms. Malignant mixed germ cell tumours are rarer still with dysgerminoma & yolk sac tumour being the most common components with three component variants being categorised in the rarest of rare varieties. Bilateral dysgerminomas occur in 15% of germ cell neoplasms but rarely present with premature ovarian failure. We present the case of an adolescent girl with a short history of abdominal pain and distension with amenorrhoea for a year. Clinical and radio ogical examination revealed a pelvic/adnexal mass with elevation of tumour markers and she had to undergo a staging laparotomy with bilateral salpingo-oophorectomy. Histopatholgy examination was reported as malignant mixed germ cell tumour of left ovary, predominantly with immature teratoma and minor components of yolk-sac tumour and dysgerminoma and right ovary with dysgerminoma. In view of mixed germ cell disease, she was planned for adjuvant chemotherapy.
文摘There have been many chapters written about macrophage polarization.These chapters generally focus on the role of macrophages in orchestrating immune responses by highlighting the T-cell-derived cytokines that shape these polarizing responses.This bias toward immunity is understandable,given the importance of macrophages to host defense.However,macrophages are ubiquitous and are involved in many different cellular processes,and describing them as immune cells is undoubtedly an oversimplification.It disregards their important roles in development,tissue remodeling,wound healing,angiogenesis,and metabolism,to name just a few processes.In this chapter,we propose that macrophages function as transducers in the body.According to Wikipedia,WA transducer is a device that converts energy from one form to another.^The word transducer is a term used to describe both the"sensor,which can interpret a wide range of energy forms,and the"actuator,which can switch voltages or currents to affect the environment.Macrophages are able to sense a seemingly endless variety of inputs from their environment and transduce these inputs into a variety of different response outcomes.Thus,rather than functioning as immune cells,they should be considered more broadly as cellular transducers that interpret microenvironmental changes and actuate vital tissue responses.In this chapter,we will describe some of the sensory stimuli that macrophages perceive and the responses they make to these stimuli to achieve their prime directive,which is the maintenance of homeostasis.
