Sea-blue histiocytosis
Ann Pathol. 2023 Oct 19:S0242-6498(23)00202-X. doi: 10.1016/j.annpat.2023.10.001. Online ahead of print.ABSTRACTA 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in ...
Source: Annales de Pathologie - October 21, 2023 Category: Pathology Authors: M ónica Bronte Anaut Javier Arredondo Montero Source Type: research
Sea-blue histiocytosis
Ann Pathol. 2023 Oct 19:S0242-6498(23)00202-X. doi: 10.1016/j.annpat.2023.10.001. Online ahead of print.ABSTRACTA 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in ...
Source: Annales de Pathologie - October 21, 2023 Category: Pathology Authors: M ónica Bronte Anaut Javier Arredondo Montero Source Type: research
Sea-blue histiocytosis
Ann Pathol. 2023 Oct 19:S0242-6498(23)00202-X. doi: 10.1016/j.annpat.2023.10.001. Online ahead of print.ABSTRACTA 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in ...
Source: Annales de Pathologie - October 21, 2023 Category: Pathology Authors: M ónica Bronte Anaut Javier Arredondo Montero Source Type: research
Sea-blue histiocytosis
Ann Pathol. 2023 Oct 19:S0242-6498(23)00202-X. doi: 10.1016/j.annpat.2023.10.001. Online ahead of print.ABSTRACTA 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in ...
Source: Annales de Pathologie - October 21, 2023 Category: Pathology Authors: M ónica Bronte Anaut Javier Arredondo Montero Source Type: research
Neonatal Glycogen Storage Disease Type IA: A Rare Presentation
Endocr Metab Immune Disord Drug Targets. 2023 Oct 19. doi: 10.2174/0118715303278622231006102118. Online ahead of print.ABSTRACTGlucose homeostasis is essential for energy production and the central nervous system function, depending on glycogen metabolism. Glycogen storage diseases (GSD) are caused by enzymatic defects of the glycogen degradation and mainly involve the liver since the inhibition of hepatic glycogen breakdown results in its excessive storage and hepatomegaly. Other findings are hypoglycemia and hyperlactatemia and consequent neurological symptoms. GSD Type Ia is a severe disease with clinical manifestations...
Source: Endocrine, Metabolic and Immune Disorders Drug Targets - October 20, 2023 Category: Drugs & Pharmacology Authors: Joana Tenente Teresa Campos Carla Vasconcelos Helena Santos Marisa Carvalho Altina Ramos Laura Vilarinho Esmeralda Rodrigues Elisa Le ão Teles Source Type: research
Neonatal Glycogen Storage Disease Type IA: A Rare Presentation
Endocr Metab Immune Disord Drug Targets. 2023 Oct 19. doi: 10.2174/0118715303278622231006102118. Online ahead of print.ABSTRACTGlucose homeostasis is essential for energy production and the central nervous system function, depending on glycogen metabolism. Glycogen storage diseases (GSD) are caused by enzymatic defects of the glycogen degradation and mainly involve the liver since the inhibition of hepatic glycogen breakdown results in its excessive storage and hepatomegaly. Other findings are hypoglycemia and hyperlactatemia and consequent neurological symptoms. GSD Type Ia is a severe disease with clinical manifestations...
Source: Endocrine, Metabolic and Immune Disorders Drug Targets - October 20, 2023 Category: Endocrinology Authors: Joana Tenente Teresa Campos Carla Vasconcelos Helena Santos Marisa Carvalho Altina Ramos Laura Vilarinho Esmeralda Rodrigues Elisa Le ão Teles Source Type: research
Neonatal Glycogen Storage Disease Type IA: A Rare Presentation
Endocr Metab Immune Disord Drug Targets. 2023 Oct 19. doi: 10.2174/0118715303278622231006102118. Online ahead of print.ABSTRACTGlucose homeostasis is essential for energy production and the central nervous system function, depending on glycogen metabolism. Glycogen storage diseases (GSD) are caused by enzymatic defects of the glycogen degradation and mainly involve the liver since the inhibition of hepatic glycogen breakdown results in its excessive storage and hepatomegaly. Other findings are hypoglycemia and hyperlactatemia and consequent neurological symptoms. GSD Type Ia is a severe disease with clinical manifestations...
Source: Endocrine, Metabolic and Immune Disorders Drug Targets - October 20, 2023 Category: Drugs & Pharmacology Authors: Joana Tenente Teresa Campos Carla Vasconcelos Helena Santos Marisa Carvalho Altina Ramos Laura Vilarinho Esmeralda Rodrigues Elisa Le ão Teles Source Type: research
Neonatal Glycogen Storage Disease Type IA: A Rare Presentation
Endocr Metab Immune Disord Drug Targets. 2023 Oct 19. doi: 10.2174/0118715303278622231006102118. Online ahead of print.ABSTRACTGlucose homeostasis is essential for energy production and the central nervous system function, depending on glycogen metabolism. Glycogen storage diseases (GSD) are caused by enzymatic defects of the glycogen degradation and mainly involve the liver since the inhibition of hepatic glycogen breakdown results in its excessive storage and hepatomegaly. Other findings are hypoglycemia and hyperlactatemia and consequent neurological symptoms. GSD Type Ia is a severe disease with clinical manifestations...
Source: Endocrine, Metabolic and Immune Disorders Drug Targets - October 20, 2023 Category: Endocrinology Authors: Joana Tenente Teresa Campos Carla Vasconcelos Helena Santos Marisa Carvalho Altina Ramos Laura Vilarinho Esmeralda Rodrigues Elisa Le ão Teles Source Type: research
Neonatal Glycogen Storage Disease Type IA: A Rare Presentation
Endocr Metab Immune Disord Drug Targets. 2023 Oct 19. doi: 10.2174/0118715303278622231006102118. Online ahead of print.ABSTRACTGlucose homeostasis is essential for energy production and the central nervous system function, depending on glycogen metabolism. Glycogen storage diseases (GSD) are caused by enzymatic defects of the glycogen degradation and mainly involve the liver since the inhibition of hepatic glycogen breakdown results in its excessive storage and hepatomegaly. Other findings are hypoglycemia and hyperlactatemia and consequent neurological symptoms. GSD Type Ia is a severe disease with clinical manifestations...
Source: Endocrine, Metabolic and Immune Disorders Drug Targets - October 20, 2023 Category: Drugs & Pharmacology Authors: Joana Tenente Teresa Campos Carla Vasconcelos Helena Santos Marisa Carvalho Altina Ramos Laura Vilarinho Esmeralda Rodrigues Elisa Le ão Teles Source Type: research
Neonatal Glycogen Storage Disease Type IA: A Rare Presentation
Endocr Metab Immune Disord Drug Targets. 2023 Oct 19. doi: 10.2174/0118715303278622231006102118. Online ahead of print.ABSTRACTGlucose homeostasis is essential for energy production and the central nervous system function, depending on glycogen metabolism. Glycogen storage diseases (GSD) are caused by enzymatic defects of the glycogen degradation and mainly involve the liver since the inhibition of hepatic glycogen breakdown results in its excessive storage and hepatomegaly. Other findings are hypoglycemia and hyperlactatemia and consequent neurological symptoms. GSD Type Ia is a severe disease with clinical manifestations...
Source: Endocrine, Metabolic and Immune Disorders Drug Targets - October 20, 2023 Category: Endocrinology Authors: Joana Tenente Teresa Campos Carla Vasconcelos Helena Santos Marisa Carvalho Altina Ramos Laura Vilarinho Esmeralda Rodrigues Elisa Le ão Teles Source Type: research
Kawasaki disease or polyarteritis nodosa: coronary involvement, a diagnostic conundrum
AbstractPolyarteritis nodosa (PAN) is a medium-vessel vasculitis presenting with cutaneous and multisystem involvement with considerable morbidity. The necrotizing vasculitis in PAN typically involves renal, celiac, and mesenteric vascular beds. Coronary artery involvement is a characteristic feature of Kawasaki disease, another medium-vessel vasculitis; however, it has been rarely reported with PAN. Here, we present 2 cases with PAN involving coronaries mimicking Kawasaki disease. A 3.5-year-old boy with classical features of Kawasaki disease with giant coronary aneurysm refractory to IVIg, methylprednisolone, infliximab ...
Source: Rheumatology International - October 19, 2023 Category: Rheumatology Source Type: research
Clinical and laboratory features associated with macrophage activation syndrome in Still ’s disease: data from the international AIDA Network Still’s Disease Registry
AbstractTo characterize clinical and laboratory signs of patients with Still ’s disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. Patients with Still’s disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still’s Disease Registry. Cli nical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still’s...
Source: Internal and Emergency Medicine - October 12, 2023 Category: Emergency Medicine Source Type: research
Epidemiological and clinical characteristics of 66 Tunisian Sickle cell syndrome patients
Conclusion: S/C patients present the best tolerated form and were the least affected by chronic complications and therefore can lead an almost normal life.Keywords: Acute complications; chronic complications; sickle cell syndrome; steady state. (Source: African Health Sciences)
Source: African Health Sciences - October 11, 2023 Category: African Health Authors: Ahlem Sahli, Faida Ouali, Rym Dabboubi, Sondess Hadj Fredj, Nabila Meddeb, Naila Mzoughi, Taieb Messaoud Source Type: research
An underrecognized cause of hepatomegaly in uncontrolled type I diabetes
Acta Gastroenterol Belg. 2023 Jul-Sep;86(3):507-508. doi: 10.51821/86.3.12257.NO ABSTRACTPMID:37814573 | DOI:10.51821/86.3.12257 (Source: Acta Gastro-Enterologica Belgica)
Source: Acta Gastro-Enterologica Belgica - October 10, 2023 Category: Gastroenterology Authors: K Ferdinande J Decaestecker L Seynhaeve E Steenkiste C De Vloo Source Type: research
