Persistent Hyperinsulinemic Hypoglycemia Presenting with a Rare Complication: West Syndrome

Çamurdan, Mahmut Orhun; Cinaz, Peyami; Serdaroğlu, Ayşe; Bideci, Aysun; Demirel, Fatma

doi:10.1515/jpem.2004.17.10.1465

Cited by 11 publications

(13 citation statements)

References 11 publications

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“…The latency period of infantile spasms following the onset of neonatal hypoglycemia was 2-10 months in this case series, and epileptogenic neutral circuits could be formed during this period. This finding is consistent with previous observations that the duration of latency between the onset of insult and the development of infantile spasms is variable (9,24). This temporal latency may be explained by the developmental desynchronization hypothesis, which is that an insult results in desynchronization of the development between the abnormal cortical areas and the subcortical structures.…”

Section: Discussionsupporting

confidence: 92%

“…Although hypoglycemic brain injury induces infantile spasms, few cases of patients with neonatal hypoglycemia who subsequently developed infantile spasms have been reported (9,10). The present study describes the clinical findings for 18 infants with neonatal-onset hypoglycemia who later presented with infantile spasms, which suggest that neonatal hypoglycemia is potentially associated with the subsequent occurrence and development of infantile spasms during infancy.…”

Section: Introductionmentioning

confidence: 66%

“…Neonatal hypoglycemia is the most common metabolic disorder during the neonatal period and is a recognized trigger of infantile spasms (8)(9)(10). Previous studies have found that neonatal hypoglycemia may result in hypoglycemic neurological injuries, also known as neonatal hypoglycemic brain injuries (11)(12)(13)(14)(15)(16).…”

Section: Introductionmentioning

confidence: 99%

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Neonatal hypoglycemic brain injury is a cause of infantile spasms

Yang

Zou

Wang

et al. 2016

Experimental and Therapeutic Medicine

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Abstract. Neonatal hypoglycemic brain injury is one of the causes of infantile spasms. In the present study, the clinical history and auxiliary examination results of 18 patients who developed infantile spasms several months after neonatal hypoglycemia were retrospectively analyzed. Among the 666 patients with infantile spasms admitted to two pediatric centers between January 2008 and October 2012, 18 patients developed infantile spasms after being diagnosed with neonatal hypoglycemia, defined as a whole blood glucose concentration of <2.6 mmol/l. These patients developed infantile spasms from between 2 and 10 months (mean, 4.9 months) following the diagnosis of neonatal hypoglycemia. All 18 patients had abnormal electroencephalographic findings with either classical or modified hypsarrhythmia. Upon examination using brain magnetic resonance imaging (MRI), 10 patients (55.6%) exhibited abnormalities. The MRI results principally showed a disproportional involvement of parietal and occipital cortices and sub-cortical white matter lesions. In conclusion, the results of this study indicate that neonatal hypoglycemic brain injury is associated with the subsequent development of infantile spasms.

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Section: Discussionsupporting

confidence: 92%

Section: Introductionmentioning

confidence: 66%

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Neonatal hypoglycemic brain injury is a cause of infantile spasms

Yang

Zou

Wang

et al. 2016

Experimental and Therapeutic Medicine

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“…Altı buçuk aylıkken West sendromu tanısı aldı. Hipoglisemik beyin hasarının West sendromuna neden olabileceği literatürde bildirilmektedir, ancak mekanizması tam olarak bilinmemektedir (8)(9)(10). Operasyondan 5 ay sonra da pankreatektomiye bağlı portal ven trombozu ve özofagus varisleri gelişti.…”

Section: Discussionunclassified

Treatment and Follow-up in a Case with Diazoxide Treatment-Resistant Hyperinsulinemic Hypoglycaemia

Gökşen¹,

Murat²,

Köroğlu³

et al. 2017

jpr

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GirişHiperinsülinemik hipoglisemi (HH), pankreatik β hücrelerinde kan glukozundan bağımsız olarak düzensiz insülin salınımına bağlı gelişir ve tekrarlayıcı hipoglisemiye neden olur (1,2). Yenidoğan ve süt çocuklarında ısrarlı hipogliseminin en sık sebebidir (2-4). Sporadik ve genetik nedenli HH nadir görülürken (insidansı 1/40000) akraba evliliği sık görülen yerlerde insidans 1/2500'e kadar çıkabilir (2). Hipoglisemi anında alınan kan örneklerinde hipoglisemiyle uygunsuz olarak serum insülin değeri saptanabilir düzeydedir. Tedavide temel amaç hipogliseminin kalıcı nörolojik sekel riskinden korunmak için normoglisemiyi sürdürmektir. HH'de normoglisemiyi sağlamak için yüksek dozda glukoz infüzyonu (>8 mg/kg/dk) gerekir, ancak düzensiz insülin salınımı nedeniyle tedavi oldukça karmaşıktır (1,2). Tedavi medikal, medikal tedaviye yanıtsız olanlarda da pankreatektomidir. Cerrahi sonrasında kür olabilir, HH devam edebilir veya cerrahiye ait komplikasyonlar gelişebilir.Burada, medikal tedaviye dirençli olması nedeniyle totale yakın pankreatektomi yapılan, ancak sonrasında Öz ABS TRACT Hiperinsülinemik hipoglisemi (HH) yenidoğan ve süt çocuklarında dirençli ve tekrarlayan hipogliseminin en sık görülen ve en zor yönetilen nedenidir. Burada yaşamının 1. gününde HH tanısı alan ve izlemde diazoksit yanıtsız olması nedeniyle totale yakın pankreatektomi uygulanan bir olgu sunuldu. Olguda, erken tanı ve müdahaleye rağmen, hipoglisemi ve cerrahiye ait komplikasyon gelişti. Anahtar Kelimeler: Hiperinsülinemik hipoglisemi, pankreatektomi, West sendromu Hyperinsulinemic hypoglycemia (HH) is the most common reason for persistent and recurrent hypoglycemia in the neonatal and infancy periods. We presented a case diagnosed with HH on the first day of life and who underwent near-total pancreatectomy because of the unresponsiveness to the diazoxide treatment. Despite early diagnosis and management, complications developed due to hypoglycemia and surgery.

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“…Hyperinsulinism requires aggressive treatment to prevent irreversible brain damage in infants (1,2,10) . Diazoxide is considered as a fi rst-line medication and is generally well tolerated.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension and reopening of the ductus arteriosus in an infant treated with diazoxide

Demirel

Ünal

Çetin

et al. 2011

Journal of Pediatric Endocrinology and Metabolism

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Diazoxide is the main therapeutic agent for persistent hyperinsulinemic hypoglycemia. Generally, it is tolerated well, but rarely it can cause severe life-threatening complications. We report a neonate who was treated with diazoxide for hyperinsulinemic hypoglycemia. On the 6th day of the treatment we observed sepsis-mimicking symptoms, mild pulmonary hypertension, and re-opening of the ductus arteriosus. All these fi ndings resolved dramatically shortly after discontinuation of treatment. To our knowledge, this is the fi rst reported case of re-opening of the ductus arteriosus due to diazoxide toxicity.

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Persistent Hyperinsulinemic Hypoglycemia Presenting with a Rare Complication: West Syndrome

Cited by 11 publications

References 11 publications

Neonatal hypoglycemic brain injury is a cause of infantile spasms

Neonatal hypoglycemic brain injury is a cause of infantile spasms

Treatment and Follow-up in a Case with Diazoxide Treatment-Resistant Hyperinsulinemic Hypoglycaemia

Pulmonary hypertension and reopening of the ductus arteriosus in an infant treated with diazoxide

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