For the fifth edition of this two-volume medical text on pediatric gastrointestinal disease Professor Allan Walker has handed over lead editorship duties to ...

Walker's pediatric gastrointestinal disease

The pathophysiology of pancreatic autodigestion is poorly understood. Pancreatitis affects all age groups, and the diagnosis is sometimes missed when serum amylase and lipase activities are not measured in the child with abdominal pain. Acute pancreatitis in children has become a more commonly seen condition and the causes have varied. Laboratory and radiological studies play an important role in determining the diagnosis and prognosis. Family history is important in the diagnosis of idiopathic hereditary pancreatitis. Most acute episodes resolve with supportive care, but the mortality in acute pancreatitis is currently about 15% (Hadorn et al., 1980). Endoscopic retrograde cholangiopancreatography or an endoscopic retrograde pancreatogram may be necessary to investigate relapses of pancreatitis. Chronic pancreatitis can be a life-threatening condition requiring lifetime medical management.

Pancreatitis in children.

Purpose: To report a pediatric case of optic neuritis with subsequent development of central retinal vein occlusion (CRVO). Methods: A case and its findings were analyzed. Results: A 16-year-old boy presented with painful vision loss in the left eye, an afferent pupillary defect, and optic disc edema. Magnetic resonance imaging showed optic nerve enhancement and contrast-enhancing cerebral white-matter lesions, consistent with optic neuritis and demyelinating disease. He received intravenous methylprednisolone followed by a prednisone taper. At the 3-week follow-up, the visual acuity (VA) in the left eye had worsened and fundoscopic examination showed a new CRVO. A hypercoagulable workup showed antiphospholipid syndrome, which was treated with warfarin. He received intravitreal antivascular endothelial growth factor treatment with subsequent improvement in VA and resolution of the macular edema. Conclusions: This case describes an unusual mechanism for CRVO via a combination of optic disc edema from optic neuritis and hypercoagulability from antiphospholipid syndrome. It is important to recognize this complication of optic disc edema and the necessary workup for a pediatric CRVO.

Pediatric Central Retinal Vein Occlusion Secondary to Concurrent Mechanisms of Optic Neuritis and Antiphospholipid Syndrome

The article describes a clinical case of bilateral occlusion of retinal vessels in a patient with Waldenstrom's disease - a rare lymphoplasmocytic tumor of the bone marrow characterized by a complex of syndromes, among which the syndrome of blood hyperviscosity dominates. Comprehensive clinical, instrumental and laboratory examinations revealed that besides the syndrome of blood hyperviscosity the patient also had loci of cerebral ischemia (according to magnetic resonance imaging), ocular hypoperfusion with severe deficiency of retinal and choroidal blood flow (according to Doppler methods) indicating the presence of ocular ischemic syndrome. Since bilateral occlusion of retinal vessels without concomitant vascular and/or systemic pathology is rare, patients with such diagnosis should be referred to a hematologist.

[Bilateral occlusion of retinal vessels in Waldenstrom's disease (a case study)]

潰瘍性大腸炎と抗リン脂質抗体症候群を併発し，脳静脈洞血栓症を発症した1 例

antiphospholipid antibodies in assays repeated 12 weeks apart, along with a significant thrombotic event (his bilateral CRVO) P ro-inflammatory conditions such as inflammatory bowel disease (IBD) may lead to a prothrombotic state. Coagulation abnormalities in ulcerative colitis (UC) or Crohn’s disease (CD) are associated with increased concentrations of factor V, factor VIII, and fibrinogen or deficiency of protein C/S or antithrombin III. However, there are myriad patients with IBD that have had arterial and/or venous thrombosis formation despite normal coagulation factors (1). Vascular complications of UC and CD include deep vein thrombosis, cerebral vein thrombosis, and as well as central retinal vein occlusion (CRVO). While the retinal vein is an unusual site for thrombosis, there have been a few case reports describing unilateral CRVO in patients with IBD. Here we describe a unique case of a UC patient with visual complaints found to have bilateral central retinal vein occlusion. Further workup led to an additional diagnosis of antiphospholipid syndrome (APS). The importance of prompt recognition and treatment to prevent visual loss is further discussed.

Bilateral Central Retinal Vein Occlusion in a patient with Ulcerative Colitis and Antiphospholipid Antibody Syndrome.

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Bilateral Central Retinal Vein Occlusion in a patient with Ulcerative Colitis and Antiphospholipid Antibody Syndrome.