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Case Report
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| A preterm very low birth weight male neonate with refractory hypoglycemia and hyperinsulinemia and hyperammonemia: A rare case report | ||||||
| Jillalla Narsing Rao1, Swathi Chacham2, Uppin Narayan Reddy3, Janampally Ravikiran4, Mohd Ahmeedulla Khan4, akkampudi Nagasravani4 | ||||||
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1MD Pediatrics, Professor, Department of Pediatrics, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India.
2MD Pediatrics, DM Neonatology(from PGIMER, Chandigarh), Assistant Professor, Department of Pediatrics, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India. 3MD Pediatrics, Professor and Head, Department of Pediatrics, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India. 4MBBS, (MD, Pediatrics), Junior Resident, Department of Pediatrics, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India. | ||||||
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| How to cite this article |
| Rao JN, Chacham S, Reddy UN, Ravikiran J, Khan MA, Nagasravani J. A preterm very low birth weight male neonate with refractory hypoglycemia and hyperinsulinemia and hyperammonemia: A rare case report. Int J Case Rep Images 2015;6(5):272–275. |
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Abstract
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Introduction:
Hypoglycemia is an important metabolic complication in neonates, more so in newborns with perinatal risk factors. Physiological immaturity of gluconeogenesis, lipolysis coupled with hyperinsulinemia contributes to hypoglycemia in small for gestational age (SGA) neonates. Persistent hyperinsulinemic hypoglycemia of infancy (PHHI), a hyperinsulinemic condition is an important differential diagnosis for intractable and refractory hypoglycemia. Hyperinsulinemic-hyperammonemia (HI/HA) syndrome, a rare autosomal dominantly inherited disorder, is the second most common cause for hyperinsulinemic-hypoglycemia in infancy. Both symptomatic as well as asymptomatic hypoglycemia involves the occipital cortex leading to cortical blindness, necessitating early etiological diagnosis and prompt intervention. We report a preterm male neonate with rare manifestations of refractory hypoglycemia, hyperinsulinemia and hyperammonemia.
Case Report: A 30 weeks, 1300 grams male neonate, born by C-section had respiratory distress, requiring mechanical ventilation (MV) for 10 days. On 11th day of life, neonate developed recurrent apneic episodes along with jitteriness and seizures. Initial evaluation revealed low blood sugar levels which persisted despite high glucose infusion rate (GIR 12 mg/kg/min). There was hyperammonemia (serum NH3 levels 273 µg/dL) along with hyperinsulinemia. However, the serum cortisol, thyroid, growth hormone levels and blood lactate were normal. Similarly, metabolic screening for inborn errors of metabolism (IEM) was normal. Abdominal imaging with ultrasound and contrast-enhanced computed tomography (CT) scan did not reveal pancreatic hyperplasia. Persistent hypoglycemia, hyperinsulinemia along with hyperammonemia could suggest hyperammonemic hyperinsulinemic syndrome in this neonate. The infant responded to oral diazoxide. Conclusion: We report a preterm, very low birth weight (VLBW) male neonate with refractory hypoglycemia and hyperinsulinemia and hyperammonemia, which responded to diazoxide. | |
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Keywords:
Hyperammonemia, Hyperinsulinemic, Hypoglycemia, Persistent hyperinsulinemic hypoglycemia of infancy, Prematurity
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Author Contributions
Jillalla Narsing Rao – Substantial contributions to conception and design, Acquisition of data, Analysis & interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published Swathi Chacham – Substantial contributions to conception and design, Acquisition of data, Analysis & interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published Uppin Narayan Reddy – Substantial contributions to conception and design, Acquisition of data, Analysis& interpretation of date, Revising it critically for important intellectual content, Final approval of the version to be published Janampally ravikiran – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published Mohd Ahmeedulla Khan – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published Jakkampudi Nagasravani – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published |
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Guarantor of submission
The corresponding author is the guarantor of submission. |
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Source of support
None |
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Conflict of interest
Authors declare no conflict of interest. |
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Copyright
© 2015 Jillalla Narsing Rao et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information. |
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