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1型神经纤维瘤病患儿的腹部偏头痛:病例系列及对NF1胃肠道受累情况的综述

Abdominal migraine in children with neurofibromatosis type 1: a case series and review of gastrointestinal involvement in NF1.

作者信息

Heuschkel R, Kim S, Korf B, Schneider G, Bousvaros A

机构信息

Division of Gastroenterology, Children's Hospital, Partners Center for Human Genetics, Boston, Massachusetts 02115, USA.

出版信息

J Pediatr Gastroenterol Nutr. 2001 Aug;33(2):149-54. doi: 10.1097/00005176-200108000-00010.

Abstract

BACKGROUND

Symptomatic involvement of the gastrointestinal tract in children with neurofibromatosis type 1 (NF1) is rare. Most reported complications in adults are caused by the presence of neurofibromas in the stomach, small bowel, or mesentery. In contrast, abdominal pain in children with NF1 may be the result of nonanatomic causes, such as migraine. There are no previous reports of an association between abdominal migraine and NF1.

METHODS

Children with abdominal migraine were identified from a group of children with NF1, all of whom had been followed up for a minimum of 3 years. Medical records of cases were reviewed independently by two authors. MEDLINE was searched via PubMed for all reports of children with NF1 and any associated gastrointestinal involvement.

RESULTS

Six children with NF1 and intermittent, episodic, severe abdominal pain are reported. Investigations for obstructive or inflammatory causes of abdominal pain were negative. All patients had previously been diagnosed with migraine headaches by a neurologist. In five of the six patients, propranolol (10-15 mg three times daily) resulted in relief of their abdominal pain within days of starting therapy. Our review identified 24 children in the medical literature with gastrointestinal complications of NF1, mostly secondary to visceral neurofibromas. In almost all of these cases, clinical examination and simple radiologic investigations led to the definitive diagnosis. There were no reports of abdominal migraine complicating NF1.

CONCLUSIONS

Abdominal pain secondary to migraine is an unrecognized cause of abdominal pain in children with NF1 and may be more common than anatomic causes of abdominal pain in children with NF1. In children with NF1 and severe recurrent abdominal pain in whom an evaluation for anatomic lesions is negative, a trial of migraine therapy may be indicated.

摘要

背景

1型神经纤维瘤病(NF1)患儿出现胃肠道症状较为罕见。成人中报道的大多数并发症是由胃、小肠或肠系膜中的神经纤维瘤引起的。相比之下,NF1患儿的腹痛可能是非解剖学原因导致的,如偏头痛。此前尚无关于腹型偏头痛与NF1之间关联的报道。

方法

从一组NF1患儿中识别出腹型偏头痛患儿,所有患儿均至少随访了3年。两位作者独立查阅病例的医疗记录。通过PubMed在MEDLINE中检索所有关于NF1患儿及任何相关胃肠道受累情况的报告。

结果

报告了6例患有NF1且有间歇性、发作性、严重腹痛的患儿。对腹痛的梗阻性或炎症性原因的检查均为阴性。所有患儿此前均由神经科医生诊断为偏头痛。6例患儿中有5例,普萘洛尔(每日3次,每次10 - 15毫克)在开始治疗数天内即可缓解腹痛。我们的综述在医学文献中发现了24例患有NF1胃肠道并发症的患儿,大多继发于内脏神经纤维瘤。在几乎所有这些病例中,临床检查和简单的影像学检查即可明确诊断。尚无NF1并发腹型偏头痛的报告。

结论

偏头痛继发的腹痛是NF1患儿腹痛未被认识的原因,可能比NF1患儿腹痛的解剖学原因更常见。对于NF1且有严重复发性腹痛但解剖学病变评估为阴性的患儿,可能需要进行偏头痛治疗试验。

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