A novel treatment approach for paediatric Gorham-Stout syndrome with chylothorax. 2011

Nicholas Brodszki, and John-Kalle Länsberg, and Michael Dictor, and Erik Gyllstedt, and Sven-Börje Ewers, and Marcus K Larsson, and Erik A Eklund
Department of Clinical Sciences, Section for Paediatrics, The BUT team, Lund University, Sweden.

OBJECTIVE To expand the treatment options in paediatric Gorham-Stout syndrome (GSS) when conventional therapy is ineffective. METHODS Two children with biopsy confirmed GSS, a rare disorder with progressive lymphangiomatosis, were treated with a combination of interferon-α-2b, low anticoagulant, low molecular weight heparin, radiotherapy and surgery. RESULTS The combined therapy resolved the symptoms in the acute phase, and both patients have since been free of symptoms for >2 years. CONCLUSIONS The successful addition of a low anticoagulant, low molecular weight heparin (tafoxiparin) to the treatment protocol in two paediatric cases of the GSS may justify the use of this approach in similar cases.

UI MeSH Term Description Entries
D008297 Male Males
D010015 Osteolysis, Essential Syndromes of bone destruction where the cause is not obvious such as neoplasia, infection, or trauma. The destruction follows various patterns: massive (Gorham disease), multicentric (HAJDU-CHENEY SYNDROME), or carpal/tarsal. Disappearing Bone Disease,Gorham Disease,Osteolysis, Idiopathic Multicentric,Osteolysis, Massive,Cystic Angiomatosis Of Bone, Diffuse,Gorham's Disease,Gorham-Stout Disease,Essential Osteolyses,Essential Osteolysis,Gorham Stout Disease,Gorhams Disease,Idiopathic Multicentric Osteolyses,Idiopathic Multicentric Osteolysis,Massive Osteolyses,Massive Osteolysis,Multicentric Osteolyses, Idiopathic,Multicentric Osteolysis, Idiopathic,Osteolyses, Essential
D011878 Radiotherapy The use of IONIZING RADIATION to treat malignant NEOPLASMS and some benign conditions. Radiotherapy, Targeted,Targeted Radiotherapy,Radiation Therapy,Radiation Therapy, Targeted,Radiation Treatment,Targeted Radiation Therapy,Radiation Therapies,Radiation Therapies, Targeted,Radiation Treatments,Radiotherapies,Radiotherapies, Targeted,Targeted Radiation Therapies,Targeted Radiotherapies,Therapies, Radiation,Therapies, Targeted Radiation,Therapy, Radiation,Therapy, Targeted Radiation,Treatment, Radiation
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D002916 Chylothorax The presence of chyle in the thoracic cavity. (Dorland, 27th ed)
D005260 Female Females
D006495 Heparin, Low-Molecular-Weight Heparin fractions with a molecular weight usually between 4000 and 6000 kD. These low-molecular-weight fractions are effective antithrombotic agents. Their administration reduces the risk of hemorrhage, they have a longer half-life, and their platelet interactions are reduced in comparison to unfractionated heparin. They also provide an effective prophylaxis against postoperative major pulmonary embolism. LMWH,Low-Molecular-Weight Heparin,Low Molecular Weight Heparin,Heparin, Low Molecular Weight
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000925 Anticoagulants Agents that prevent BLOOD CLOTTING. Anticoagulant Agent,Anticoagulant Drug,Anticoagulant,Anticoagulant Agents,Anticoagulant Drugs,Anticoagulation Agents,Indirect Thrombin Inhibitors,Agent, Anticoagulant,Agents, Anticoagulant,Agents, Anticoagulation,Drug, Anticoagulant,Drugs, Anticoagulant,Inhibitors, Indirect Thrombin,Thrombin Inhibitors, Indirect
D013514 Surgical Procedures, Operative Operations carried out for the correction of deformities and defects, repair of injuries, and diagnosis and cure of certain diseases. (Taber, 18th ed.). Surgical Procedures,Ghost Surgery,Operative Procedures,Operative Surgical Procedure,Operative Surgical Procedures,Procedure, Operative Surgical,Procedures, Operative Surgical,Surgery, Ghost,Surgical Procedure, Operative,Operative Procedure,Procedure, Operative,Procedure, Surgical,Procedures, Operative,Procedures, Surgical,Surgical Procedure

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