Diagnosis and management of Sandifer syndrome in children with intractable neurological symptoms.

Sandifer syndrome is a rare complication of gastro-oesophageal reflux disease (GERD) when a patient presents with extraoesophageal symptoms, typically neurological. The aim of this study was to review the existing literature and describe a typical presentation and most appropriate investigations and management for the Sandifer syndrome. A comprehensive literature search was performed via PubMed, Cochrane Library and NHS Evidence databases. Twenty-seven cases and observational studies were identified. The literature demonstrates that presenting symptoms of Sandifer's may include any combination of abnormal movements and/or positioning of head, neck, trunk and upper limbs, seizure-like episodes, ocular symptoms, irritability, developmental and growth delay and iron-deficiency anaemia. A 24-h oesophageal pH monitoring was positive in all the cases of Sandifer's where it was performed, while upper GI endoscopy ± biopsy and barium swallow were diagnostic only in a subset of cases. Successful treatment of the underlying gastro-oesophageal pathology led to a complete or near-complete resolution of the neurological symptoms in all of the cases.Conclusion: It is evident from the literature that many patients with Sandifer syndrome were originally misdiagnosed with various neuropsychiatric diagnoses that led to unnecessary testing and ineffective medications with significant side effects. Earlier diagnosis of Sandifer's would have allowed to avoid them.What is Known:• Sandifer syndrome is a rare complication of gastro-oesophageal reflux disease (GERD) when a patient presents with extraoesophageal symptoms, typically neurological.• It may be difficult to recognise due to its non-specific presentation and lack of gastrointestinal symptoms.What is New:• Based on the review of 44 clinical cases of suspected Sandifer syndrome, the clinical picture was clarified: the presenting symptoms of Sandifer's may include any combination of abnormal movements and/or positioning of head, neck, trunk and upper limbs, seizure-like episodes, ocular symptoms, irritability, developmental and growth delay and iron-deficiency anaemia.• Successful treatment of the underlying gastro-oesophageal pathology led to a complete or near-complete resolution of the neurological symptoms in all of the reviewed cases.

Argatroban to achieve therapeutic anticoagulation in two patients with acute thrombosis and heparin resistance.

: We present two cases where argatroban was successfully used in patients with acute thrombosis requiring anticoagulant treatment where heparin resistance with unfractionated heparin had been encountered. The first case was a woman with abdominal arterial thrombosis, of unknown cause, treated with therapeutic low molecular weight heparin that developed pulmonary embolism despite therapeutic anticoagulation (and had evidence of heparin resistance on anti-Xa monitoring). The second patient had provoked abdominal arterial thrombosis from sepsis and could not attain therapeutic anticoagulation with intravenous unfractionated heparin. In both cases therapeutic anticoagulation was achieved with the use of argatroban, as a temporizing measure to treat the acute thrombotic event. Conventionally, argatroban has been described for use in heparin-induced thrombocytopenia. The use of argatroban is briefly discussed, especially in the context of heparin resistance where anticoagulation can be challenging. Further research using argatroban in heparin resistant patients could be justified.