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Neonatal Hypoglycemia


Presentation

  • In this paper, we present two siblings with TBX19 gene mutation. The first case was investigated at the age of 2 months for severe hypoglycemia, recurrent convulsions, and prolonged cholestatic jaundice persisting since the neonatal period.[ncbi.nlm.nih.gov]
  • A case of neonatal hypoglycemia with extensive occipital cortical loss is presented.[ncbi.nlm.nih.gov]
  • An abnormality of circulating glucagon found in one child with this disorder suggested that hyperinsulinism may not be the sole hormonal imbalance present, but rather that this disease is one of generalized disturbance of islet cell function.[ncbi.nlm.nih.gov]
  • Globus pallidus injury was present in one patient who had the most severe cortical injury. CONCLUSION: We found a specific pattern of injury that correlates well with the sparse pathologic and imaging reports on neonatal hypoglycemia.[ncbi.nlm.nih.gov]
  • A wide range of rare endocrine and metabolic disorders can present with neonatal hypoglycemia, of which congenital hyperinsulinism is responsible for the most severe form of hypoglycemia.[ncbi.nlm.nih.gov]
Asymptomatic
  • However, controversy remains surrounding its definition and management especially in asymptomatic patients.[ncbi.nlm.nih.gov]
  • Fundamental issue of great professional controversy is concerning the best manner to manage asymptomatic newborns NH. Both, overtreating NH and undertreating NH are poles with significant potential disadvantages.[ncbi.nlm.nih.gov]
  • The history of severe, persistent neonatal hypoglycemia in an older sibling should lead the physician to investigate subsequent children for the presence of asymptomatic hypoglycemia.[ncbi.nlm.nih.gov]
  • Asymptomatic hypoglycemic (blood glucose level Results: Dextrose gel with feeds increased the blood glucose level in 184/250 (74%) of asymptomatic hypoglycemic infants compared to 144/248 (58%) with feeds only (p 0.01).[karger.com]
  • BACKGROUND: Transient asymptomatic neonatal hypoglycemia (TANH) is common as infants transition from their mother's energy stores to their own.[ncbi.nlm.nih.gov]
Hypothermia
  • Hypothermia 5. Hyperviscosity 6. Erythroblastosis fetalis, fetal hydrops 7. Other a. Iatrogenic causes b. Congenital cardiac malformations C. Intrauterine growth restriction D. Hyperinsulinism E. Endocrine disorders F.[aafp.org]
  • […] confused with: hypocalcemia Sepsis CNS disorders Cardiorespiratory problemsNeonatal hypoglycemia can also show no symptoms in some newborns or may be life threatening.Some observed symptoms are (these symptoms may be transient but reoccurring): Jitteriness hypothermia[en.wikipedia.org]
  • Symptoms Jittery or Tremor s Lethargic Hypotonia Apnea Hypothermia Cyanosis Seizure s Weak or high pitched cry Poor feeding V.[fpnotebook.com]
  • Neuroglycopenic signs include seizure, coma, cyanotic episodes, apnea, bradycardia or respiratory distress, and hypothermia. Listlessness, poor feeding, hypotonia, and tachypnea may occur.[merckmanuals.com]
  • Involvement Cerebral Palsy About CP Causes & Risk Factors Signs and Symptoms Diagnosis Treatments and Therapies Assistive Technology Legal Help HIE About HIE Causes & Risk Factors Signs of Fetal Distress Diagnosis Brain Imaging Treatment Therapeutic Hypothermia[abclawcenters.com]
Poor Feeding in Infants
  • A 6-day old term infant born to a non-diabetic mother was admitted to our hospital with, seizures, irritability and poor feeding. The infant's Apgar score was 8 at 1 minute and 9 at 5 minutes and weighed 3100 g.[ijri.org]
Small Head
  • In the present series, with shorter follow-up, 85% are walking by 2 years, 14% have moderate cognitive delay, one third have small head size, one third have visual abnormalities, and 27% develop later seizures.[pediatricneurologybriefs.com]
Visual Hallucination
  • Ictal manifestations of main seizures were identical to occipital lobe seizures, such as eye deviation, eye blinking, ictal vomiting, and visual hallucination.[ncbi.nlm.nih.gov]
Microphallus
  • The microphallus is a remediable manifestation of hypopituitarism.[ncbi.nlm.nih.gov]
  • A case of congenital hypopituitarism with microphallus and severe neonatal hypoglycemia is described. The results of the clinical examination and of the tests of hypothalamo-pituitary function are discussed.[ncbi.nlm.nih.gov]
Lethargy
  • Furthermore, the 3 most common clinical manifestations of the neonates with hypoglycemia were jittieriness 38%, lethargy 35%, and temperature instability 32%.[en.wikipedia.org]
  • The most common clinical manifestations can include altered level of consciousness, seizure, vomiting, unresponsiveness, and lethargy.[emedicine.medscape.com]
  • Some infants will display no obvious symptoms, while others will exhibit lethargy, apnea, a bluish hue to the skin, low body temperatures, jitters, and in severe cases, seizures.[thurswell.com]
  • Adrenergic signs include diaphoresis, tachycardia, lethargy or weakness, and shakiness. Neuroglycopenic signs include seizure, coma, cyanotic episodes, apnea, bradycardia or respiratory distress, and hypothermia.[merckmanuals.com]
  • View/Print Table TABLE 1 Clinical Signs Associated with Hypoglycemia* Changes in levels of consciousness Irritability Lethargy Stupor Apnea, cyanotic spells Coma Feeding poorly after feeding well Hypothermia Hypotonia, limpness Tremor Seizures TABLE 1[aafp.org]
Neonatal Seizures
  • Nine children with neonatal seizures had moderate or severe white matter injury and 7 had cortical involvement. At 18-month follow-up of 34 children, outcome was normal in 8, showed mild impairment in 15, moderate in 8, and severe impairment in 3.[pediatricneurologybriefs.com]
  • seizures -- Intraventricular hemorrhage -- Surgical emergencies in the newborn -- Necrotizing enterocolitis.[worldcat.org]
  • Figures and Tables - Analysis 1.5 Comparison 1 Dextrose gel versus control, Outcome 5 Neonatal seizures. Figures and Tables - Analysis 1.6 Comparison 1 Dextrose gel versus control, Outcome 6 Exclusive breast feeding after discharge (WHO definition).[cochranelibrary.com]
  • Neonatal seizure s ‐ No seizures occurred in the dextrose gel or placebo group in the one study that reported this outcome (one study, 237 infants). Therefore, the odds ratio is not estimable.[doi.org]
Apathy
  • “In approximate order of frequency there are jitteriness or tremors, apathy, episodes of cyanosis, convulsions, intermittent apneic spells or tachypnea, weak or high-pitched cry, limpness or lethargy, difficulty in feeding, and eye rolling.[cps.ca]
  • […] judgment Nonspecific dysphoria, moodiness, depression, crying, exaggerated concerns Feeling of numbness, pins and needles (paresthesia) Negativism, irritability, belligerence, combativeness, rage Personality change, emotional lability Fatigue , weakness, apathy[en.wikipedia.org]
Behavior Problem
  • Other neurologic problems included developmental delays, learning and behavior problems, hyperactivity and attention difficulties, autistic features, microcephaly and cortical blindness.[ncbi.nlm.nih.gov]

Workup

Fasting Hypoglycemia
  • Activating mutations of glucokinase (GCK) ( Fig. 1 ), the “glucose sensor” of the β-cell, are rare and, depending on the mutation, may cause fasting hypoglycemia in varying degrees at varying ages of life ( 8 ).[diabetes.diabetesjournals.org]
  • Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome. J Pediatr. 2001;138:383–9. PubMed CrossRef Google Scholar 16. Bahi-Buisson N, Roze E, Dionisi C, Escande F, Valayannopoulos V, Feillet F, et al.[link.springer.com]

Treatment

  • IMPLICATIONS FOR RESEARCH: There is a need for rigorous long-term studies comparing treatment thresholds and neurodevelopmental outcomes among various treatment strategies for TANH.[ncbi.nlm.nih.gov]
  • These treatments, though effective at treating hypoglycemia, interrupt exclusive breastfeeding and interfere with mother-infant bonding. Our institution developed a treatment algorithm for newborns at risk for neonatal hypoglycemia.[ncbi.nlm.nih.gov]
  • On attendance at another hospital, hydrocortisone treatment was discontinued and antiepileptic treatment was initiated because of suspected epilepsy.[ncbi.nlm.nih.gov]
  • By following a nurse-driven protocol, our institution has successfully met these three challenges in our treatment of newborns diagnosed with neonatal hypoglycemia (NH).[ncbi.nlm.nih.gov]
  • Abstract OBJECTIVE: To determine neurodevelopmental outcome at 2 years' corrected age in children randomized to treatment with dextrose gel or placebo for hypoglycemia soon after birth (The Sugar Babies Study).[ncbi.nlm.nih.gov]

Prognosis

  • This study indicates neonatal hypoglycemia may cause posterior cerebral lesions, abnormal findings at neurologic examination, and symptomatic epilepsy, most frequently occipital lobe epilepsy, usually with a good prognosis, and occasionally epileptic[ncbi.nlm.nih.gov]
  • Diffusion restriction, with low apparent diffusion coefficient values, in the mesial occipital poles may indicate the prognosis for visual outcomes in acute settings after neonatal hypoglycemia.[ncbi.nlm.nih.gov]
  • (See Prognosis.) Causes of hypoglycemia in neonates differ slightly from those in older infants and children.[emedicine.medscape.com]
  • Prognosis depends on the underlying condition. Treatment is enteral feeding or IV dextrose. Neonatal hypoglycemia may be transient or persistent.[merckmanuals.com]

Etiology

  • The etiology of this pattern of injury is unclear; however, transient hyperinsulinism may be an independent risk factor. Magnetic resonance brain imaging can delineate the extent of brain injury and guide follow-up.[ncbi.nlm.nih.gov]
  • Hyperinsulinemia is a rare cause of persistent hypoglycemia, diagnosed by excluding other etiologies. Inappropriately high fasting serum insulin levels with concurrent hypoglycemia confirm the diagnosis.[ncbi.nlm.nih.gov]
  • OBJECTIVE: To investigate the frequency, etiology and consequences of neonatal hypoglycemia.[ncbi.nlm.nih.gov]
  • (See Etiology.) Causes of hypoglycemia found in all ages include gram-negative sepsis, endotoxin shock, and ingestions, including of salicylates, alcohol, hypoglycemic agents, or beta-adrenergic blocking agents.[emedicine.medscape.com]
  • Although lengths may be normal in these infants, physical features such as micropenis or cleft lip and/or palate should suggest pituitary dysfunction as the etiology of their hypoglycemia.[ncbi.nlm.nih.gov]

Epidemiology

  • The definition of neonatal hypoglycemia has been based on the following approaches (or combination of approaches): clinical symptoms (see Table 1 ), epidemiologic approach based on a range of glucose values, acute changes in metabolic and endocrine response[aafp.org]
  • Journal of Clinical Epidemiology 2005; 58: 769-776.[alliedacademies.org]
  • Goitre epidemiology: Thyroid volume, iodine excretion, thyroglobulin and thyrotropin in Germany and Sweden. Acta Endocrinol 1 12:494501. ‎[books.google.es]
  • Keith J Barrington MD (chair); Haresh Kirpalani MD; Shoo K Lee MD; Koravangattu Sankaran MD; John Van Aerde MD Liaisons: Lillian Blackmon MD, Committee on Fetus and Newborn, American Academy of Pediatrics; Catherine McCourt MD, Health Surveillance and Epidemiology[cps.ca]
Sex distribution
Age distribution

Pathophysiology

  • Hypoglycemia in Diabetes: Pathophysiology, Prevalence, and Prevention . American Diabetes Association: : Alexandria, VA, USA , 2009. 15. Lucas A , Morley R , Cole TJ . Adverse neurodevelopmental outcome of moderate neonatal hypoglycemia .[nature.com]
  • Glutamate, which is one of the excitatory amino acid neurotransmitters found only in the central nervous system, is believed to play a major role in the pathophysiology of hypoglycemic brain injury.[pedsinreview.aappublications.org]
  • Hypoglycemia: Pathophysiology, Diagnosis, and Treatment . New York: Oxford University Press. ISBN 978-0-19-511325-9 . OCLC 36188385 . Service FJ, Cryer PE, Vella A (March 2017).[en.wikipedia.org]

Prevention

  • We evaluated the effect of a national prevention guideline stratified according to mild, moderate, and severe risks of hypoglycemia.[ncbi.nlm.nih.gov]
  • It is also essential to prevent environmental factors such as cold stress that may predispose the newborn for further decreasing blood sugar.[en.wikipedia.org]
  • However, if acted upon early, these complications are preventable with mostly very simple measures.[ncbi.nlm.nih.gov]
  • We conclude that early diagnosis and treatment of neonatal hypoglycemia is crucial to prevent future neurological sequelae, especially in patients with additional perinatal risk factors.[ncbi.nlm.nih.gov]
  • Prevention of hypoglycemic brain damage by hindering prolonged and profound neonatal hypoglycemia might contribute to psychomotor development.[ncbi.nlm.nih.gov]

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