標(biāo)題: Titlebook: Congenital Hyperinsulinism; A Practical Guide to Diva D. De León-Crutchlow,Charles A. Stanley Book 2019 Springer Nature Switzerland AG 2019 [打印本頁(yè)] 作者: ominous 時(shí)間: 2025-3-21 18:22
書(shū)目名稱(chēng)Congenital Hyperinsulinism影響因子(影響力)
作者: deforestation 時(shí)間: 2025-3-21 20:50 作者: dagger 時(shí)間: 2025-3-22 01:37
Diazoxide-Unresponsive Forms of Congenital Hyperinsulinism,ast five days . Inactivating mutations in the genes encoding the two subunits of the beta-cell ATP-sensitive potassium (KATP) channel are the most frequent cause of diazoxide-unresponsive hyperinsulinism. Children with KATPHI typically present at birth with severe hypoglycemia. Genetic testing can b作者: prick-test 時(shí)間: 2025-3-22 04:42
Syndromic Causes of Congenital Hyperinsulinism,d related to mechanisms within the pancreatic beta cell, several genetic syndromes have been linked to hyperinsulinism. These include Beckwith-Wiedemann syndrome, Sotos syndrome, Kabuki syndrome, and Turner syndrome. All of these syndromes have been noted to occur with subtle presentations with hype作者: MONY 時(shí)間: 2025-3-22 11:47
Molecular Diagnosis of Congenital Hyperinsulinism,mely genetic diagnosis is clinically important for patients since understanding the underlying genetic cause of the disease can guide the clinician in both medical and surgical management. In children who are unresponsive to medical treatment with diazoxide and thus will require pancreatectomy, a ge作者: 公社 時(shí)間: 2025-3-22 13:18 作者: 公社 時(shí)間: 2025-3-22 17:38
18F-DOPA PET,nagement. Over the past 20 years, it has become clear that some patients with severe disease that is unresponsive to medical therapy may benefit from pancreatectomy. One of the big challenges has been distinguishing between two histopathologic forms of HI, diffuse disease, which is not curable, and 作者: critique 時(shí)間: 2025-3-23 01:09 作者: Annotate 時(shí)間: 2025-3-23 01:37
Perioperative Management of Hyperinsulinism,rition, close attention must be paid to glucose management as the child’s glucose needs change throughout the perioperative course. A standardized approach to their perioperative care is essential to ensure an optimal recovery.作者: 非秘密 時(shí)間: 2025-3-23 06:57 作者: Legend 時(shí)間: 2025-3-23 10:47
Management of Diabetes and Pancreatic Insufficiency After Pancreatectomy,occur. Initially after surgery, patients may experience persistent hypoglycemia, persistent diabetes mellitus, or transient diabetes followed by hypoglycemia. Insulin is indicated in patients with hyperglycemia in the immediate post-op period to maintain normal glucose levels. For persistent diabete作者: babble 時(shí)間: 2025-3-23 16:23 作者: 慢跑鞋 時(shí)間: 2025-3-23 21:56
Neurodevelopmental Outcomes,evelopmental abnormalities (26–48%) and seizures (13–29%) in the hyperinsulinism population. Individuals are at risk of poor neurodevelopmental outcomes regardless of the duration of the hyperinsulinism or treatment modality. All children with hyperinsulinism require screening for developmental issu作者: 特征 時(shí)間: 2025-3-24 02:15 作者: tenosynovitis 時(shí)間: 2025-3-24 02:57
Diazoxide-Responsive Forms of Congenital Hyperinsulinism,xide to avoid fluid overload and pulmonary hypertension. Genetic forms of diazoxide-responsive hyperinsulinism include a distinctive form caused by dominant activating mutations in glutamate dehydrogenase (GDH). Leucine-induced and fasting hypoglycemia, mild hyperammonemia, and neurologic abnormalit作者: 占線 時(shí)間: 2025-3-24 09:19
Diazoxide-Unresponsive Forms of Congenital Hyperinsulinism,etic etiology. The overall goal in the management of infants with diazoxide-unresponsive HI is to identify those with focal HI and to find an effective treatment regimen for those that cannot be cured by surgery (diffuse HI).作者: Cardiac-Output 時(shí)間: 2025-3-24 13:21 作者: Aboveboard 時(shí)間: 2025-3-24 17:30
Management of Diabetes and Pancreatic Insufficiency After Pancreatectomy,e manner, exocrine pancreatic insufficiency may have a delayed onset after a subtotal pancreatectomy, and symptoms often arise only after nutritional sequela have occurred. Thus, careful screening for exocrine insufficiency with stool studies and labs should occur at baseline and at regular interval作者: 逃避責(zé)任 時(shí)間: 2025-3-24 19:58
https://doi.org/10.1007/978-3-322-81157-8xide to avoid fluid overload and pulmonary hypertension. Genetic forms of diazoxide-responsive hyperinsulinism include a distinctive form caused by dominant activating mutations in glutamate dehydrogenase (GDH). Leucine-induced and fasting hypoglycemia, mild hyperammonemia, and neurologic abnormalit作者: OGLE 時(shí)間: 2025-3-25 01:21 作者: TAP 時(shí)間: 2025-3-25 06:37 作者: 巫婆 時(shí)間: 2025-3-25 09:34
Transnationale Vergesellschaftungene manner, exocrine pancreatic insufficiency may have a delayed onset after a subtotal pancreatectomy, and symptoms often arise only after nutritional sequela have occurred. Thus, careful screening for exocrine insufficiency with stool studies and labs should occur at baseline and at regular interval作者: modifier 時(shí)間: 2025-3-25 13:02
Book 2019surgical approach, and post-operative management. Complications, such as feeding problems, andlong-term outcomes, such as neurodevelopmental issues, are carefully considered in the final chapter..Practical and user-friendly, .Congenital Hyperinsulinism. is the go-to resource for pediatric endocrinol作者: V洗浴 時(shí)間: 2025-3-25 16:30 作者: cavity 時(shí)間: 2025-3-25 21:35 作者: 大酒杯 時(shí)間: 2025-3-26 04:14 作者: refine 時(shí)間: 2025-3-26 06:16
Kirsten Scheiwe,Johanna Krawietzd hormone responses during the development of fasting hypoglycemia. This is best accomplished by performing a closely monitored fasting test. The diagnosis of hyperinsulinism relies heavily on demonstrating inappropriate effects of insulin on fasting adaptation, i.e., inappropriate suppression of li作者: 倫理學(xué) 時(shí)間: 2025-3-26 11:04 作者: corpuscle 時(shí)間: 2025-3-26 15:39 作者: 存在主義 時(shí)間: 2025-3-26 19:11
,Auslagerung wirtschaftlicher T?tigkeiten,d related to mechanisms within the pancreatic beta cell, several genetic syndromes have been linked to hyperinsulinism. These include Beckwith-Wiedemann syndrome, Sotos syndrome, Kabuki syndrome, and Turner syndrome. All of these syndromes have been noted to occur with subtle presentations with hype作者: Jubilation 時(shí)間: 2025-3-26 23:36
https://doi.org/10.1007/978-3-658-15517-9mely genetic diagnosis is clinically important for patients since understanding the underlying genetic cause of the disease can guide the clinician in both medical and surgical management. In children who are unresponsive to medical treatment with diazoxide and thus will require pancreatectomy, a ge作者: 沒(méi)血色 時(shí)間: 2025-3-27 04:43 作者: anticipate 時(shí)間: 2025-3-27 06:12 作者: Diskectomy 時(shí)間: 2025-3-27 11:24 作者: 貪婪性 時(shí)間: 2025-3-27 17:33 作者: 致命 時(shí)間: 2025-3-27 20:09
Transnationale Vergesellschaftungenm have persistent hypoglycemia after near-total pancreatectomy. A comprehensive assessment of the glycemic status should be undertaken once the patient is on a stable feeding regimen for several days. This assessment includes performing a Cure Fast to demonstrate the resolution of hyperinsulinism in作者: 別名 時(shí)間: 2025-3-27 23:12 作者: figure 時(shí)間: 2025-3-28 04:41
Transnationale Vergesellschaftungenism (HI), feeding difficulties are common, and a significant proportion of infants with HI require feeding support, complicating medical management. It is important to recognize potential causes and mechanisms contributing to feeding problems and address these through sustained multidisciplinary inp作者: Preserve 時(shí)間: 2025-3-28 09:53 作者: 事與愿違 時(shí)間: 2025-3-28 14:20
Transnationale Unternehmen und GeschlechtOur multidisciplinary approach to patients with congenital hyperinsulinism can distinguish focal from diffuse disease, localize focal lesions, and permit partial pancreatectomy with cure in almost all patients. Surgery does not cure the diffuse form of congenital hyperinsulinism but can help prevent hypoglycemia and brain damage.作者: 闖入 時(shí)間: 2025-3-28 16:47 作者: Interdict 時(shí)間: 2025-3-28 19:26 作者: enhance 時(shí)間: 2025-3-29 02:40 作者: 修正案 時(shí)間: 2025-3-29 04:58
https://doi.org/10.1007/978-3-030-02961-618FDOPA PET; Beckwith Wiedemann syndrome; Congenital hyperinsulism; Diazoxide-responsive hyperinsulinis作者: 物種起源 時(shí)間: 2025-3-29 09:22
Springer Nature Switzerland AG 2019作者: 陳腐思想 時(shí)間: 2025-3-29 12:14 作者: 推崇 時(shí)間: 2025-3-29 17:28 作者: stress-response 時(shí)間: 2025-3-29 20:58 作者: TOXIN 時(shí)間: 2025-3-30 02:57 作者: 把…比做 時(shí)間: 2025-3-30 07:03 作者: Dislocation 時(shí)間: 2025-3-30 11:28
Neurodevelopmental Outcomes,es regardless of the duration of the hyperinsulinism or treatment modality. All children with hyperinsulinism require screening for developmental issues and swift intervention if abnormalities are identified.作者: 流利圓滑 時(shí)間: 2025-3-30 15:09
,Auslagerung wirtschaftlicher T?tigkeiten,rinsulinism being a clue toward diagnosis of the syndrome, and given there are additional medical management recommendations for patients with these syndromes, it is important to consider possible syndromic etiology as part of the evaluation for patients with hyperinsulinism.作者: Osteoporosis 時(shí)間: 2025-3-30 19:21 作者: Antimicrobial 時(shí)間: 2025-3-30 22:05