奇瓦瓦州婴幼儿医院治疗的持续性高胰岛素性低血糖症

IF 0.5 Q4 PEDIATRICS
Claudia E Peña-Varela, Nancy L Salazar-Flores, Reyna G Ramos-Porras, Karina A Martínez-Cervantes, Lilia T Avena-Portillo, Luisa B Gámez-González
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引用次数: 0

摘要

背景:婴儿期持续性高胰岛素性低血糖(PHHI)影响0.5%至5%的儿科患者。这种情况是由胰腺β细胞功能障碍引起的,导致肿瘤和高胰岛素血症,从而导致持续的低血糖。这些发作通常对常规治疗有抵抗性,需要干预以减少胰岛素产生和增加葡萄糖水平,从而恢复代谢平衡。临床病例:我们报告3例PHHI病例。第一个是一个4天大的Rarámuri患者,持续低血糖,在胰腺切除术和活检后确诊。第二个病例是一名出生3天的Rarámuri患者,持续低血糖,胰腺造影发现胰腺病变,胰腺切除术后确诊PHHI。第三例是一名三天大的女性患者,她患有低活动和持续性低血糖,在胰切除术后也被诊断为PHHI。结论:虽然临床表现和生化结果提高了诊断的高度怀疑,但组织病理学证实是必不可少的。早期诊断是预防长期后遗症的关键。PHHI在高胰岛素性低血糖的鉴别诊断中应予以考虑,如早产、宫内生长受限、低出生体重、产妇糖尿病、1型酪氨酸血症或beckwithwithwiedemann综合征。这些病例突出了PHHI的复杂性及其在Rarámuri族裔群体中的不寻常表现,强调了对文化敏感的医疗保健的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Persistent hyperinsulinemic hypoglycemia of infancy treated at the Hospital Infantil de Especialidades de Chihuahua.

Background: Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) affects between 0.5% and 5% of pediatric patients. This condition is caused by a dysfunction of pancreatic β-cells, leading to tumors and hyperinsulinism, which result in persistent hypoglycemia. These episodes are often resistant to conventional treatment and require interventions to reduce insulin production and increase glucose levels, thereby restoring metabolic balance.

Clini calcases: We present three cases of PHHI. The first is a four-day-old Rarámuri patient with persistent hypoglycemia, diagnosed after a pancreatectomy and biopsy. The second case is a three-day-old Rarámuri patient with persistent hypoglycemia and a pancreatic lesion detected by scintigraphy, with PHHI confirmed after pancreatectomy. The third case is a three-day-old female patient with hypoactivity and persistent hypoglycemia, who was also diagnosed with PHHI after a pancreatectomy.

Conclusions: While clinical presentation and biochemical findings raise high diagnostic suspicion, histopathological confirmation is essential. Early diagnosis is key to preventing long-term sequelae. PHHI should be considered in the differential diagnosis of hyperinsulinemic hypoglycemia, such as prematurity, intrauterine growth restriction, low birth weight, maternal diabetes, tyrosinemia type 1, or Beckwith-Wiedemann syndrome. These cases highlight the complexity of PHHI and its unusual presentation in the Rarámuri ethnic group, underscoring the importance of culturally sensitive medical care.

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来源期刊
CiteScore
1.60
自引率
0.00%
发文量
73
审稿时长
20 weeks
期刊介绍: The Boletín Médico del Hospital Infantil de México is a bimonthly publication edited by the Hospital Infantil de México Federico Gómez. It receives unpublished manuscripts, in English or Spanish, relating to paediatrics in the following areas: biomedicine, clinical, public health, clinical epidemology, health education and clinical ethics. Articles can be original research articles, in-depth or systematic reviews, clinical cases, clinical-pathological cases, articles about public health, letters to the editor or editorials (by invitation).
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