Hypoglycemia In Children Research Articles

Decreasing Trends in Mean HbA1c Are Not Associated With Increasing Rates of Severe Hypoglycemia in Children: A Longitudinal Analysis of Two Contemporary Population-Based Pediatric Type 1 Diabetes Registries From Australia and Germany/Austria Between 1995 and 2016.

To investigate temporal trends in glycemic control and severe hypoglycemia rates for pediatric patients with type 1 diabetes from 1995 to 2016 by analyzing data from the longitudinal, prospective, population-based German/Austrian (Diabetes Patient History Documentation [DPV]) and Western Australian (Western Australian Children's Diabetes Database [WACDD]) diabetes registries. Patients diagnosed with type 1 diabetes aged <15 years were identified from the DPV (N = 59,883) and WACDD (N = 2,595) registries and data extracted for all clinic visits occurring between 1995 and 2016, inclusive. Mean HbA1c and severe hypoglycemia (self-reported loss of consciousness/convulsion) rates were calculated per 100 patient-years. Between 1995 and 2016, the annual mean HbA1c decreased from 8.3 to 7.8% in the DPV cohort and from 9.2 to 8.3% in the WACDD cohort. Over the same period, the severe hypoglycemia rate decreased by an annual average of 2% (relative risk 0.983 [95% CI 0.981, 0.986]) in the DPV cohort and 6% (relative risk 0.935 [95% CI 0.934, 0.937]) in the WACDD cohort. Concomitant decreasing trends in both HbA1c and severe hypoglycemia rates were observed in boys and girls, all age-groups, and injection therapy/pump regimen groups. Over the past two decades, there have been concurrent improvements in HbA1c and decreasing severe hypoglycemia rates in two contemporary, longitudinal, population-based pediatric cohorts of type 1 diabetes. Translation of these data into clinical practice and patient education may reduce fear of hypoglycemia and enable better glycemic control.

SUN-LB034 A Rare Cause of Hyperinsulinemic Hypoglycemia in Adulthood: A Case Report and Review of the Literature

Introduction: Post-prandial hypoglycaemia (PPH) after consumption of protein is a vanishingly rare condition. Congenital hyperinsulinism is the most common cause of persistent hypoglycemia in infants and children, although it is uncommon to be diagnosed in adulthood. Dominant activation of glutamate dehydrogenase, encoded for by the GLUD1 gene, causes the hyperinsulinemia/hyperammonemia syndrome (HHS). Within a family known to be affected by HHS, the first presentation in adulthood, is intriguing. Clinical case: A 32-year old male presented with new-onset generalised non-convulsive seizures and family history of genetically proven HHS in his mother, two siblings and two of their offspring. A heterozygous missense mutation (R269H) in exon 7 of the GLUD1 gene was identified several years ago in this family. Our patient was screened clinically for hypoglycemia in childhood, but he never underwent genetic testing, as he was not considered affected. Affected family members developed confusion and hypoglycaemia following high-protein meals, which was reversed with glucose and limiting protein intake, lessened further attacks. Our patient’s school performance was poor and described feeling unwell after eating large protein meals. He was fully orientated, but his mini-mental state examination was impaired at 21/30. He was hyper-reflexic with spreading of ankle and crossed adductor reflexes. Considering his family history, we performed continuous glucose monitoring for 14 days, revealing several post-prandial and fasting hypoglycaemic episodes (as low as 1.8 mmol/L) and an HbA1c of 3.5%. HHS was proven (serum ammonia 70 µmol/L; normal less than 35 µmol/L) and genetic analysis revealed an identical mutation to the aforementioned. Diazoxide was titrated to abrogate hypoglycaemia. Genetic screening of the remaining family members will be undertaken. Clinical lessons: Among causes of PPH, HHS is rare. Missed diagnoses can have profound neurological and cognitive consequences. The intrafamilial variability is demonstrable however; it is likely that HHS was missed in our patient’s initial presentation in childhood, as seizures and intellectual disability in adulthood are exceptionally rare complications of this condition. It demonstrates that it is critical to perform genetic testing on all members of an affected family, even in those asymptomatic. Genetic diagnoses of PPH are rarely made in adulthood, as there is often a low diagnostic suspicion at such a late presentation.

MON-441 Copeptin Response to Acute Hypoglycemia in Children and Adolescents

Introduction: Copeptin is a surrogate biomarker of vasopressin (AVP) and a well established stress marker in different acute clinical scenarios. Copeptin stimulation by acute hypoglycemia has been shown in the adult population and accurately identifies patients with posterior pituitary dysfunction. The role of AVP in the acute stress response remains to be defined and an interaction between AVP secretion and hypothalamic-pituitary-adrenal (HPA) axis activation in response to hypoglycemia has been suggested, requiring further confirmation. Objectives: The aims of this study were to evaluate copeptin response to acute hypoglycemia in children and adolescents and to assess its potential association with other parameters of the counter-regulatory and inflammatory responses. Methods: This is a prospective single-center study involving 77 children and adolescents (mean age; 11.2 ± 3.1years; 58 males/19 females) undergoing insulin-tolerance test. Results: Baseline plasma copeptin levels (median: 5.2pmol/L, IQR: 3.9-7.7pmol/L) increased significantly after hypoglycemia (median: 9.7pmol/L, IQR: 5.8-14.3pmol/L) (P<0.0001). Counter-regulatory hormones - ACTH, cortisol, growth hormone, prolactin, adrenaline and noradrenaline - and inflammatory markers - IL-1β, IL-6 and TNF - also significantly increased after hypoglycemia (P<0.0001 for all measurements). Copeptin increment correlated significantly with the maximal increases of ACTH (rs:0.30; P: 0.010), cortisol (rs:0.33; P:0.003), prolactin (rs:0.25; P:0.03) and IL-6 (rs:0.35; P:0.008), and with BMI-SDS (rs:-0.28, P:0.01). In stepwise multivariate regression analysis, prolactin was the only independent variable that correlated with copeptin increment (P:0.009). Conclusion: Acute hypoglycemia elicits a significant copeptin response in children and adolescents, confirming the role of copeptin as an acute stress marker in the pediatric population. Interactions between stress-induced AVP/copeptin activation and the HPA axis, IL-6 inflammatory response and BMI were suggested by univariate analyses, although not confirmed by the multivariate analysis. Nevertheless, our data indicate that AVP/copeptin secretion during hypoglycemic stress may be linked to prolactin co-activation as prolactin maximal increase was the only variable independently related to copeptin response, reinforcing the concept that AVP/copeptin might act as a prolactin releasing factor in humans.

50 Years Ago in The Journal of Pediatrics: Gluconeogenesis and Insulin in the Ketotic Variety of Childhood Hypoglycemia and in Control Children

50 Years Ago in The Journal of Pediatrics: Gluconeogenesis and Insulin in the Ketotic Variety of Childhood Hypoglycemia and in Control Children

Clinical Diversity in Focal Congenital Hyperinsulinism in Infancy Correlates With Histological Heterogeneity of Islet Cell Lesions.

Background: Congenital Hyperinsulinism (CHI) is an important cause of severe and persistent hypoglycaemia in infancy and childhood. The focal form (CHI-F) of CHI can be potentially cured by pancreatic lesionectomy. While diagnostic characteristics of CHI-F pancreatic histopathology are well-recognized, correlation with clinical phenotype has not been established.Aims: We aimed to correlate the diversity in clinical profiles of patients with islet cell organization in CHI-F pancreatic tissue.Methods: Clinical datasets were obtained from 25 patients with CHI-F due to ABCC8/KCNJ11 mutations. 18F-DOPA PET-CT was used to localize focal lesions prior to surgery. Immunohistochemistry was used to support protein expression studies.Results: In 28% (n = 7) of patient tissues focal lesions were amorphous and projected into adjoining normal pancreatic tissue without clear delineation from normal tissue. In these cases, severe hypoglycaemia was detected within, on average, 2.8 ± 0.8 (range 1–7) days following birth. By contrast, in 72% (n = 18) of tissues focal lesions were encapsulated within a defined matrix capsule. In this group, the onset of severe hypoglycaemia was generally delayed; on average 46.6 ± 14.3 (range 1–180) days following birth. For patients with encapsulated lesions and later-onset hypoglycaemia, we found that surgical procedures were curative and less complex.Conclusion: CHI-F is associated with heterogeneity in the organization of focal lesions, which correlates well with clinical presentation and surgical outcomes.

Therapies and outcomes of congenital hyperinsulinism-induced hypoglycaemia.

Congenital hyperinsulinism is a rare disease, but is the most frequent cause of persistent and severe hypoglycaemia in early childhood. Hypoglycaemia caused by excessive and dysregulated insulin secretion (hyperinsulinism) from disordered pancreatic β cells can often lead to irreversible brain damage with lifelong neurodisability. Although congenital hyperinsulinism has a genetic cause in a significant proportion (40%) of children, often being the result of mutations in the genes encoding the KATP channel (ABCC8 and KCNJ11), not all children have severe and persistent forms of the disease. In approximately half of those without a genetic mutation, hyperinsulinism may resolve, although timescales are unpredictable. From a histopathology perspective, congenital hyperinsulinism is broadly grouped into diffuse and focal forms, with surgical lesionectomy being the preferred choice of treatment in the latter. In contrast, in diffuse congenital hyperinsulinism, medical treatment is the best option if conservative management is safe and effective. In such cases, children receiving treatment with drugs, such as diazoxide and octreotide, should be monitored for side effects and for signs of reduction in disease severity. If hypoglycaemia is not safely managed by medical therapy, subtotal pancreatectomy may be required; however, persistent hypoglycaemia may continue after surgery and diabetes is an inevitable consequence in later life. It is important to recognize the negative cognitive impact of early‐life hypoglycaemia which affects half of all children with congenital hyperinsulinism. Treatment options should be individualized to the child/young person with congenital hyperinsulinism, with full discussion regarding efficacy, side effects, outcomes and later life impact.

Persistent Hypoglycemia in Children: Targeted Gene Panel Improves the Diagnosis of Hypoglycemia Due to Inborn Errors of Metabolism

To evaluate the role of next generation sequencing in genetic diagnosis of pediatric patients with persistent hypoglycemia. Sixty-four patients investigated through an extensive workup were divided in 3 diagnostic classes based on the likelihood of a genetic diagnosis: (1) single candidate gene (9/64); (2) multiple candidate genes (43/64); and (3) no candidate gene (12/64). Subsequently, patients were tested through a custom gene panel of 65 targeted genes, which included 5 disease categories: (1) hyperinsulinemic hypoglycemia, (2) fatty acid-oxidation defects and ketogenesis defects, (3) ketolysis defects, (4) glycogen storage diseases and other disorders of carbohydrate metabolism, and (5) mitochondrial disorders. Molecular data were compared with clinical and biochemical data. A proven diagnosis was obtained in 78% of patients with suspicion for a single candidate gene, in 49% with multiple candidate genes, and in 33% with no candidate gene. The diagnostic yield was 48% for hyperinsulinemic hypoglycemia, 66% per fatty acid-oxidation and ketogenesis defects, 59% for glycogen storage diseases and other carbohydrate disorders, and 67% for mitochondrial disorders. This approach provided a diagnosis in ~50% of patients in whom clinical and laboratory evaluation did not allow identification of a single candidate gene and a diagnosis was established in 33% of patients belonging to the no candidate gene class. Next generation sequencing technique is cost-effective compared with Sanger sequencing of multiple genes and represents a powerful tool for the diagnosis of inborn errors of metabolism presenting with persistent hypoglycemia.

Approach to hypoglycaemia in childhood

Approach to hypoglycaemia in childhood

Novel homozygous PCK1 mutation causing cytosolic phosphoenolpyruvate carboxykinase deficiency presenting as childhood hypoglycemia, an abnormal pattern of urine metabolites and liver dysfunction.

Clinical and laboratory data were collected from three Finnish patients including a sibling pair and another unrelated child with unexplained childhood hypoglycemia. Transient elevation of alanine transaminase, lactate and tricarboxylic acid cycle intermediates, especially fumarate, were noticed in urine organic acid analysis. Exome sequencing was performed for the patients and their parents. A novel homozygous PCK1 c.925G>A (p.G309R) mutation was detected in all affected individuals. COS-1 cells transfected with mutant PCK1 transcripts were used to study the pathogenic nature of the detected variant. The COS-1 transfected cells showed the mutant gene to be incapable of producing a normally functioning cytosolic phosphoenolpyruvate carboxykinase (PEPCK) enzyme. This report further delineates the clinical phenotype of isolated cytosolic PEPCK deficiency and offers a metabolic pattern helping to recognize these patients. Cytosolic PEPCK deficiency should be considered in the differential diagnosis of children presenting with hypoglycemia, hepatic dysfunction and elevated tricarboxylic acid intermediates in urinary organic acid analysis.

Developmental Outcomes of Preterm Infants With Neonatal Hypoglycemia.

Neonatal hypoglycemia has been associated with abnormalities on brain imaging and a spectrum of developmental delays, although historical and recent studies show conflicting results. We compared the cognitive, academic, and behavioral outcomes of preterm infants with neonatal hypoglycemia with those of normoglycemic controls at 3 to 18 years of age. A secondary analysis of data from the Infant Health and Development Program, a national, multisite, randomized controlled longitudinal intervention study of long-term health and developmental outcomes in preterm infants. Of the 985 infants enrolled in the Infant Health and Development Program, 745 infants had glucose levels recorded. Infants were stratified into 4 groups by glucose level. By using standardized cognitive, academic, and behavioral assessments performed at 3, 8, and 18 years of age, we compared groups after adjusting for intervention status, birth weight, gestational age, sex, severity of neonatal course, race, maternal education, and maternal preconception weight. No significant differences were observed in cognitive or academic skills between the control and effected groups at any age. Participants with more severe neonatal hypoglycemia reported fewer problem behaviors at age 18 than those without hypoglycemia. No significant differences in intellectual or academic achievement were found between preterm infants with and without hypoglycemia. A statistical difference was found in behavior at age 18, with hypoglycemic children showing fewer problematic behaviors than normoglycemic children. This difference was not clinically meaningful. Using extended outcomes, our results are consistent with previous studies that found no significant neurodevelopmental outcomes associated with neonatal hypoglycemia in preterm-born children.

Management of Hypoglycemia in Children and Adolescents with Type 1 Diabetes Mellitus.

Hypoglycemia and fear of hypoglycemia limit appropriate glycemic control in many children and adolescents with type 1 diabetes. Traditional approaches to the prevention of hypoglycemia including patient education about modifiable risk factors for hypoglycemia (changes in insulin, diet, and exercise) and frequency of self glucose monitoring remain important for hypoglycemia prevention. Continuous glucose monitoring systems with or without a partial closed-loop control of insulin infusion have been very useful in the prevention of hypoglycemia. Oral carbohydrate and parenteral glucagon continue to be the mainstays of hypoglycemia treatment. In the future, we can look forward to regulatory approval of closed-loop insulin delivery and glucose monitoring systems to facilitate euglycemia, as well as glucagon administered by the intranasal route to treat hypoglycemia.

Images from 18F-DOPA Scan in Congenital Hyperinsulinism: Not Always a Clue for Diagnosis.

Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycaemia in childhood (Horm Res 70:65-72, 2008; J Clin Endocr Metab 93:869-875, 2008). 18-Fluoro-L-dihydroxy-phenylalanine (18F-DOPA) positron emission tomography (PET) can detect areas of increased activity in the pancreas and may differentiate focal from diffuse CHI (J Clin Endocr Metab 93:869-875, 2008; Radiology 253:216-222, 2009). We here report the case of a girl who complained of recurrent episodes of severe hypoglycaemia despite previous partial pancreatectomy. To evaluate the need for additional surgical intervention, we performed 18F-DOPA PET/computed tomography (CT), which showed a focal lesion corresponding to the anatomical region of the pancreatic tail. On the other hand, abdominal magnetic resonance imaging (MRI) clearly demonstrated that the 18F-DOPA uptake was in a loop of bowel occupying the previous surgical bed. Our case highlights that bowel uptake can be a possible pitfall in the interpretation of 18F-DOPA PET/CT in children affected by CHI, suggesting that when 18F-DOPA PET/CT results do not fit the clinical picture, magnetic resonance imaging (MRI) may allow a more accurate correlation of the radiotracer activity with the underlying anatomical or pathological structure.

Fear of Hypoglycemia in Children and Adolescents and Their Parents with Type 1 Diabetes.

Hypoglycemia is a frequent occurrence in children and adolescents with type 1 diabetes. A variety of efforts have been made to standardize the definition of hypoglycemia and to define one of its most significant psychosocial consequences-fear of hypoglycemia (FOH). In addition to documenting the experience of FOH in children and adolescents type 1 diabetes and their parents, studies have investigated the relations between FOH and glycemic control and diabetes technology use. This review provides a summary of the recent FOH literature as it applies to pediatric type 1 diabetes.

Systemic primary carnitine deficiency with hypoglycemic encephalopathy.

Acute hypoglycemia in children is not an uncommon disease that can be encountered in the Emergency Department. Most cases of childhood hypoglycemia are caused by ketotic hypoglycemia due to missed meals. Often, hypoketotic hypoglycemia can also occur, which suggests hyperinsulinemia or a defect in fatty acid oxidation. Carnitine is essential for long chain fatty acids transfer into mitochondria for oxidation. We present a case of systemic primary carnitine deficiency who presented with seizures due to hypoketotic hypoglycemia.

Frequency of Hypoglycaemia in Children and Young People's Diabetes Clinic

Frequency of Hypoglycaemia in Children and Young People's Diabetes Clinic

Le syndrome de Beckwith-Wiedemann : que faut-il rechercher en anténatal ? À propos d’une série de 14 cas

ObjectivesBeckwith-Wiedemann syndrome (BWS) is the most common overgrowth syndrome and has an incidence of 1/13,700. The majority of the cases are diagnosed after birth. Patients with BWS have an increased risk of neonatal hypoglycemia and embryonal tumors development in childhood. We wanted to identify the ultrasound signs that must alert physicians to prepare best perinatal management strategies. MethodsWe conducted a retrospective study of a population of 14 cases of BWS diagnosed in perinatal period; four of them were detected prenatally by ultrasound. The anomalies signs described in prenatal were analyzed and compared with the clinical features of the postnatal period. ResultsThe major features reported were represented by macrosomia for 71.4% with an increase of abdominal circumference, and macroglossia for 78.6%. The minor features were various with 64% of visceromegaly (nephromegaly and/or hepatomegaly), 50% of hydramnios and for 80% of male children a genital anomaly (crytorchidism and/or hypospadias). ConclusionThis study identified some prenatal ultrasound signs that should alert the clinician to the possibility of BWS. A genetic conseling, after confirmation by molecular diagnosis, could be proposed in a near future in prenatal, and could improve postnatal management strategies for these affected children at high postnatal risk.

Hypoglycemia and Risk Factors for Death in 13 Years of Pediatric Admissions in Mozambique.

Hypoglycemia is a life-threatening complication of several diseases in childhood. We describe the prevalence and incidence of hypoglycemia among admitted Mozambican children, establishing its associated risk factors. We retrospectively reviewed clinical data of 13 years collected through an ongoing systematic morbidity surveillance in Manhiça District Hospital in rural Mozambique. Logistic regression was used to identify risk factors for hypoglycemia and death. Minimum community-based incidence rates (MCBIRs) for hypoglycemia were calculated using data from the demographic surveillance system. Of 49,089 children < 15 years hospitalized in Manhiça District Hospital, 45,573 (92.8%) had a glycemia assessment on admission. A total of 1,478 children (3.2%) presented hypoglycemia (< 3 mmol/L), of which about two-thirds (972) were with levels < 2.5 mmol/L. Independent risk factors for hypoglycemia on admission and death among hypoglycemic children included prostration, unconsciousness, edema, malnutrition, and bacteremia. Hypoglycemic children were significantly more likely to die (odds ratio [OR] = 7.11; P < 0.001), with an associated case fatality rate (CFR) of 19.3% (245/1,267). Overall MCBIR of hypoglycemia was 1.57 episodes/1,000 child years at risk (CYAR), significantly decreasing throughout the study period. Newborns showed the highest incidences (9.47 episodes/1,000 CYAR, P < 0.001). Hypoglycemia remains a hazardous condition for African children. Symptoms and signs associated to hypoglycemia should trigger the verification of glycemia and the implementation of life-saving corrective measures.

Predictive Low-Glucose Insulin Suspension Reduces Duration of Nocturnal Hypoglycemia in Children Without Increasing Ketosis.

OBJECTIVENocturnal hypoglycemia can cause seizures and is a major impediment to tight glycemic control, especially in young children with type 1 diabetes. We conducted an in-home randomized trial to assess the efficacy and safety of a continuous glucose monitor–based overnight predictive low-glucose suspend (PLGS) system.RESEARCH DESIGN AND METHODSIn two age-groups of children with type 1 diabetes (11–14 and 4–10 years of age), a 42-night trial for each child was conducted wherein each night was assigned randomly to either having the PLGS system active (intervention night) or inactive (control night). The primary outcome was percent time <70 mg/dL overnight.RESULTSMedian time at <70 mg/dL was reduced by 54% from 10.1% on control nights to 4.6% on intervention nights (P < 0.001) in 11–14-year-olds (n = 45) and by 50% from 6.2% to 3.1% (P < 0.001) in 4–10-year-olds (n = 36). Mean overnight glucose was lower on control versus intervention nights in both age-groups (144 ± 18 vs. 152 ± 19 mg/dL [P < 0.001] and 153 ± 14 vs. 160 ± 16 mg/dL [P = 0.004], respectively). Mean morning blood glucose was 159 ± 29 vs. 176 ± 28 mg/dL (P < 0.001) in the 11–14-year-olds and 154 ± 25 vs. 158 ± 22 mg/dL (P = 0.11) in the 4–10-year-olds, respectively. No differences were found between intervention and control in either age-group in morning blood ketosis.CONCLUSIONSIn 4–14-year-olds, use of a nocturnal PLGS system can substantially reduce overnight hypoglycemia without an increase in morning ketosis, although overnight mean glucose is slightly higher.

Hemoglobin A1c Levels and risk of severe hypoglycemia in children and young adults with type 1 diabetes from Germany and Austria: a trend analysis in a cohort of 37,539 patients between 1995 and 2012.

Severe hypoglycemia is a major complication of insulin treatment in patients with type 1 diabetes, limiting full realization of glycemic control. It has been shown in the past that low levels of hemoglobin A1c (HbA1c), a marker of average plasma glucose, predict a high risk of severe hypoglycemia, but it is uncertain whether this association still exists. Based on advances in diabetes technology and pharmacotherapy, we hypothesized that the inverse association between severe hypoglycemia and HbA1c has decreased in recent years. We analyzed data of 37,539 patients with type 1 diabetes (mean age ± standard deviation 14.4 ± 3.8 y, range 1-20 y) from the DPV (Diabetes Patienten Verlaufsdokumentation) Initiative diabetes cohort prospectively documented between January 1, 1995, and December 31, 2012. The DPV cohort covers an estimated proportion of >80% of all pediatric diabetes patients in Germany and Austria. Associations of severe hypoglycemia, hypoglycemic coma, and HbA1c levels were assessed by multivariable regression analysis. From 1995 to 2012, the relative risk (RR) for severe hypoglycemia and coma per 1% HbA1c decrease declined from 1.28 (95% CI 1.19-1.37) to 1.05 (1.00-1.09) and from 1.39 (1.23-1.56) to 1.01 (0.93-1.10), respectively, corresponding to a risk reduction of 1.2% (95% CI 0.6-1.7, p<0.001) and 1.9% (0.8-2.9, p<0.001) each year, respectively. Risk reduction of severe hypoglycemia and coma was strongest in patients with HbA1c levels of 6.0%-6.9% (RR 0.96 and 0.90 each year) and 7.0%-7.9% (RR 0.96 and 0.89 each year). From 1995 to 2012, glucose monitoring frequency and the use of insulin analogs and insulin pumps increased (p<0.001). Our study was not designed to investigate the effects of different treatment modalities on hypoglycemia risk. Limitations are that associations between diabetes education and physical activity and severe hypoglycemia were not addressed in this study. The previously strong association of low HbA1c with severe hypoglycemia and coma in young individuals with type 1 diabetes has substantially decreased in the last decade, allowing achievement of near-normal glycemic control in these patients. Please see later in the article for the Editors' Summary.

A Case of 2-Month-Old Infant with Persistent Hyperinsulinemic Hypoglycemia Presenting as Atonic Seizure

Congenital hyperinsulinism is the most frequent cause of severe, persistent hypoglycemia in infancy and childhood. It is caused by an inappropriate insulin secretion from the pancreatic β-cells secondary to various genetic disorders. Recognition of this entity becomes important due to the fact that hypoglycemia is very severe and frequent and that it may lead to severe neurological damage in the infant manifesting as mental or psychomotor retardation or even a life-threatening events if not recognized and treated effectively in time. Hypoglycemias can be detected by seizures, fainting, or any other neurological symptoms in the neonatal period or later, usually within the first two years of life. Hypoglycemias must be rapidly and intensively treated to prevent severe and irreversible brain damages. Next, a treatment to prevent the recurrence of hypoglycemia must be set, which may include frequent and glucose-enriched feeding, diazoxide and octreotide. We report a case of congenital hyperinsulinemia in a 2 months old infant presenting as atonic seizure which has been treated with diazoxide.