The treatment effect of diazoxide on 44 patients with congenital hyperinsulinism

Hu, Shuwen; Xu, Zhou; Yan, Jie; Liu, Min; Sun, Bing; Li, Wenjing; Sang, Yanmei

doi:10.1515/jpem-2012-0224

Cited by 20 publications

(19 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The incidence of the common adverse effects of diazoxide, like hyponatremia and fluid retention, was not different between the two groups and none of the infants in our study had the other reported adverse effects of diazoxide, such as necrotising enterocolitis, hypertrichosis, refractory hypotension, ketoacidosis and neutropenia. The similar safety profile of diazoxide has been demonstrated by various authors . In our study, the infants showed a good cortisol response at the time of hypoglycaemia and thus demonstrated a relatively adequate counter‐regulatory hormonal response in SGA infants with hypoglycaemia, similar to a previous study from the same unit .…”

Section: Discussionsupporting

confidence: 90%

Randomised controlled trial of diazoxide for small for gestational age neonates with hyperinsulinaemic hypoglycaemia provided early hypoglycaemic control without adverse effects

et al. 2018

View full text Add to dashboard Cite

show abstract

Section: Discussionsupporting

confidence: 90%

Randomised controlled trial of diazoxide for small for gestational age neonates with hyperinsulinaemic hypoglycaemia provided early hypoglycaemic control without adverse effects

et al. 2018

View full text Add to dashboard Cite

show abstract

“…The conservative treatment constitutes primarily of diazoxide, or octreotide, as first choice, but a significant number of the patients are unresponsive or only partially responsive to medical treatment . Alternatively, continuous glucagon, sirolimus or nifedipine among others have been used, however with variable success .…”

Section: Introductionmentioning

confidence: 99%

The difficult management of persistent, non‐focal congenital hyperinsulinism: A retrospective review from a single, tertiary center

Rasmussen

Меликян²,

Globa³

et al. 2020

Pediatr Diabetes

View full text Add to dashboard Cite

Background/Objective: Congenital hyperinsulinism (CHI) is a rare, heterogeneous disease with transient or persistent hypoglycemia. Histologically, focal, diffuse, and atypical forms of CHI exist, and at least 11 disease-causing genes have been identified. Methods:We retrospectively evaluated the treatment and outcome of a cohort of 40 patients with non-focal, persistent CHI admitted to the International Hyperinsu-Results: Twenty-two patients (55%) could not be managed with medical monotherapy (diazoxide or octreotide) and six (15%) patients developed severe potential side effects to medication. Surgery was performed in 17 (43%) patients with resection of 66% to 98% of the pancreas. Surgically treated patients had more frequently K ATP -channel gene mutations (surgical treatment 12/17 vs conservative treatment 6/23, P = .013), highly severe disease (15/17 vs 13/23, P = .025) and clinical onset <30 days of age (15/17 vs 10/23, P = .004).At last follow-up at median 5.3 (range: 0.3-31.3) years of age, 31/40 (78%) patients still received medical treatment, including 12/17 (71%) after surgery. One patient developed diabetes after a 98% pancreatic resection. Problematic treatment status was seen in 7/40 (18%). Only 8 (20%) had clinical remission (three spontaneous, five after pancreatic surgery). Neurodevelopmental impairment (n = 12, 30%) was marginally associated with disease severity (P = .059). Conclusions: Persistent, non-focal CHI remains difficult to manage. Neurological impairment in 30% suggests a frequent failure of prompt and adequate treatment. A high rate of problematic treatment status at follow-up demonstrates an urgent need for new medical treatment modalities. K E Y W O R D S congenital hyperinsulinism, genetic mutations, hypoglycemia, surgery, treatment

show abstract

“…Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in neonates and infants. [1][2][3][4] Immediate evaluation and early diagnosis of CHI are important for initiating the appropriate treatment because of the increased risk of permanent brain injury secondary to the metabolic actions of insulin. 2 Most cases of CHI may persist for the first week/month of life before improving spontaneously.…”

Section: Introductionmentioning

confidence: 99%

“…5 Diazoxide is a benzothiadiazine derivative, a peripheral vasodilator mainly used for hypertensive emergencies. [2][3][4]6 The agent increases membrane permeability to potassium ions in the vascular smooth muscle, thereby stabilizing the membrane action potential and preventing vascular smooth muscle contraction. 6 It also inhibits insulin release by interacting with adenosine triphosphate-sensitive potassium channels of pancreatic islet β-cells.…”

Section: Introductionmentioning

confidence: 99%

Cerebral Insufficiency Caused by Diazoxide in a Premature Neonate with Congenital Hyperinsulinism

et al. 2019

View full text Add to dashboard Cite

Diazoxide is a peripheral vasodilator that has been used for intravenous treatment of hypertensive emergencies. However, it is currently used mainly for hyperinsulinemic hypoglycemia in lower dose orally, and its major side effects are edema and pulmonary hypertension. Herein, we report the first association between periventricular leukomalacia (PVL) and intractable hypotension due to diazoxide. A Japanese female premature infant showed hypoglycemia concomitant with hyperinsulinemia. She was diagnosed with congenital hyperinsulinism, and oral diazoxide was started. Six days after starting diazoxide, she suddenly showed peripheral coldness, oliguria, and severe hypotension. The hypotension was refractory to general vasopressor therapies and persisted even after the discontinuation of diazoxide. Cranial echography showed periventricular echodensities followed by cystic PVL. Low-dose vasopressin effectively treated the hypotension. This single case reminds us the serious adverse events of diazoxide that have been forgotten, especially in premature neonates.

show abstract

The treatment effect of diazoxide on 44 patients with congenital hyperinsulinism

Abstract: Diazoxide is a first-line treatment of CHI with high safety. Most of the patients with CHI were responsive to diazoxide treatment. Diazoxide should be used as a trial immediately after CHI diagnosis was made.

Cited by 20 publications

References 0 publications

Randomised controlled trial of diazoxide for small for gestational age neonates with hyperinsulinaemic hypoglycaemia provided early hypoglycaemic control without adverse effects

Randomised controlled trial of diazoxide for small for gestational age neonates with hyperinsulinaemic hypoglycaemia provided early hypoglycaemic control without adverse effects

The difficult management of persistent, non‐focal congenital hyperinsulinism: A retrospective review from a single, tertiary center

Cerebral Insufficiency Caused by Diazoxide in a Premature Neonate with Congenital Hyperinsulinism

Contact Info

Product

Resources

About