European journal of pediatrics
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Case Reports
An unusual cause of gastric perforation in childhood: trichobezoar (Rapunzel syndrome). A case report.
Trichobezoars are hair balls found in the stomach and formed following trichitillomania and trichopaghia. Rapunzel syndrome is a rare condition in which the presence of giant trichobezoars causes mechanical obstruction. To date, only two cases of stomach perforation caused by trichobezoars have been reported among pediatric patients. ⋯ This trichobezoar mass was totally excised by expanding the perforation area. Conclusion This is the reported third case of gastric perforation caused by trichobezoar in a pediatric patient. Among acute abdominal cases, gastric perforation should remain a possibility in differential diagnosis.
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This review summarizes current knowledge on vitamin K for the paediatrician. Vitamin K is a fat-soluble vitamin, present in plants as phylloquinone and produced by bacteria as menaquinone. It is acting as a co-factor for gamma-glutamyl carboxylase. This enzyme is responsible for post-translational modification of some glutamate side chains to gamma-carboxyglutamate. The majority of gamma-carboxylated proteins function in blood coagulation; others play a role in calcium homeostasis. ⋯ Further work is needed before firm recommendations can be made regarding dose in preterm infants and in patients with fat malabsorption/cholestasis or regarding the role of vitamin K in the prevention of osteoporosis.
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Review Case Reports
Acute paraplegia due to spinal arteriovenous fistula in two patients with hereditary hemorrhagic telangiectasia.
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by recurrent epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations (AVM). Of these, spinal AVM is a rare manifestation that concerns mainly children. ⋯ The prognosis of spinal AVM mainly depends on the presence or not of medullar signs and symptoms and on the delay before treatment. In conclusion, any child with a family history of HHT should be considered at risk for spinal AVM in order to improve management of such complications and to decrease the risk of neurological sequellae.
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We report the case of a 13-year-old boy with Costello syndrome and chronic constipation who received phosphate-containing Fleet-pediatric enemas at regular intervals. The day before admission he was given four enemas for severe constipation. Within 24 h the boy had developed severe hyperphosphatemia (phosphate 17.75 mmol/L), hypertonic dehydration (sodium 171 mmol/L) and severe hypocalcemia (calcium 0.56 mmol/L). ⋯ Children with developmental delay often display bowel dysfunction and chronic constipation and are therefore high-risk patients. The accepted opinion-that these enemas are not absorbed and therefore systemically inactive-is not true. Physicians should be aware of the potentially lethal complications of this treatment, which is part of everyday clinical practice.
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A 4-month-old boy was admitted for having diffuse eruption in the perianal region, legs, trunk hands, and face with failure to thrive, edema, hypoalbuminemia, and anemia. The patient was thought to have acrodermatitis enteropathica-like eruption due to malabsorption. The eruption completely resolved with enzyme supplement and proper nutrition and skin care.