Plasma ammonia concentrations in extremely low birthweight infants in the first week after birth: secondary analysis from the ProVIDe randomized clinical trial

Cormack, Barbara; Jiang, Yannan; Harding, Jane E; Crowther, Caroline A; Lynn, Adrienne; Nair, Arun; Hewson, Michael; Meyer, Mike; Broadbent, Roland; Webster, Dianne; Glamuzina, Emma; Ryder, Bryony; Bloomfield, Frank H.

doi:10.1038/s41390-019-0730-z

Cited by 8 publications

(8 citation statements)

References 49 publications

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“…There were significant differences amongst sites for intravenous and enteral protein intake (Figure 6). Ammonia concentrations and their relationship with protein intake and postnatal age have been reported previously [38]. Three sites did not commence fortified feeds or give preterm formula until a mean of 17 days or more after birth, Figure 2A.…”

Section: Resultssupporting

confidence: 60%

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Relationships between Neonatal Nutrition and Growth to 36 Weeks’ Corrected Age in ELBW Babies–Secondary Cohort Analysis from the Provide Trial

et al. 2020

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A key modifiable factor for improving neurodevelopment in extremely low birthweight (ELBW) babies may be improving growth, especially head growth, by optimising nutrition in the early neonatal period. We aimed to investigate relationships between nutrient intakes in the 4 weeks after birth, and growth from birth to 36 weeks’ corrected age (CA) in ELBW babies. We undertook a prospective cohort study of 434 participants enrolled in a randomised controlled trial (ProVIDe) in eight New Zealand and Australian neonatal intensive care units. Macronutrient intakes from birth to 4 weeks and weight, length and head circumference measurements from birth to 36 weeks’ CA were collected. From birth to 36 weeks’ CA, the median (IQR) z-score changes were: weight −0.48 (−1.09, 0.05); length −1.16 (−1.86, −0.43), and head circumference −0.82 (−1.51, −0.19). Changes in z-score to 4 weeks and 36 weeks’ CA were correlated with protein intake. Each 1 g·Kg−1·d−1 total protein intake in week 2 was associated with 0.26 z-score increase in head circumference at 36 weeks’ CA. Both nutritional intake and change in z-scores to 36 weeks’ CA differed widely amongst sites. Correlations between nutrition and growth, and differences in these amongst sites, indicate there may be potential to improve growth with enhanced nutrition practices.

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Section: Resultssupporting

confidence: 60%

“…Each 1 g.Kg -1 .d -1 total protein in week 2 was associated with a 0.26 z-score increase in head circumference at 36 weeks' CA (Supplementary Table S1). (35,38) 39 (36,44) Data are median (IQR). Data collected on Day 0 and day of death were excluded.…”

Section: Resultsmentioning

confidence: 99%

Relationships between Neonatal Nutrition and Growth to 36 Weeks’ Corrected Age in ELBW Babies–Secondary Cohort Analysis from the Provide Trial

et al. 2020

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“…This recommended threshold is also thought to fit very low birth weight infants (<1000 g). [19] In addition, a single reference limit of 82 μmol/L for all newborns <1 week of age has been proposed. [20] Increased ammonia production or insufficient ammonia detoxification can result in neonatal hyperammonemia.…”

Section: Pathogenesis Of Thanmentioning

confidence: 99%

A glance at transient hyperammonemia of the newborn: Pathophysiology, diagnosis, and treatment: A review

Miao

Zhao

et al. 2022

Medicine

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Hyperammonemia is the excessive accumulation of ammonia in the blood, and is usually defined as a plasma level above 100 µmol/L in neonates or above 50 µmol/L in term infants, children, and adolescents. Patients with hyperammonemia usually experience life-threatening neuropsychiatric symptoms, especially newborns. It is routinely caused by inherited metabolic diseases and also by acquired disorders, such as liver failure, portosystemic shunting, gastrointestinal hemorrhage, ureterosigmoidostomy, renal tubular acidosis, hypoxic ischemic encephalopathy, infections with urea-metabolizing organisms, and some drugs. Transient hyperammonemia of the newborn (THAN) is a special type of hyperammonemia acknowledged in the field of metabolic disease as an inwell-defined or well-understood entity, which can be diagnosed only after the exclusion of genetic and acquired causes of hyperammonemia. Although the prognosis for THAN is good, timely identification and treatment are essential. Currently, THAN is underdiagnosed and much less is mentioned for early diagnosis and vigorous treatment. Herein, we present common themes that emerge from the pathogenesis, diagnosis, and management of THAN, based on current evidence. When a newborn presents with sepsis, intracranial hemorrhage, or asphyxia that cannot explain coma and seizures, doctors should always keep this disease in mind.

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“…Normal plasma ammonia levels in premature newborns, fullterm newborns, infants, and children decrease with time, but their reference values have not been well defined thus far (2)(3)(4). In this study, we used plasma ammonia ≥100 μmol/L Original Article…”

Section: Introductionmentioning

confidence: 99%

Genetic background and clinical characteristics of infantile hyperammonemia

Li¹,

Chen²,

Chen³

et al. 2023

Transl Pediatr

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Background: This study was conducted to analyze the genetic spectrum and clinical characteristics of infantile hyperammonemia. Methods:Between January 2016 and June 2020, we retrospectively enrolled infantile hyperammonemia patients with definitive genetic diagnosis at the Children's Hospital of Fudan University. Based on the age of hyperammonemia onset, patients were grouped into neonatal and post-neonatal subgroups to compare their genetic and clinical features.Results: Collectively, 136 pathogenic or likely pathogenic variants of the 33 genes were identified.Fourteen genes were reported with hyperammonemia (42%, 14/33), with SLC25A13 and MUT being the top two detected genes. In contrast, 19 genes, which have not been previously reported with hyperammonemia, were detected (58%, 19/33), in which JAG1 and ABCC8 were the most frequently mutated genes. Compared with post-neonatal hyperammonemia, neonatal patients with hyperammonemia presented with higher rates of organic acidemia (P=0.001) and fatty acid oxidation disorder (P=0.006), but a lower rate of cholestasis (P<0.001). Patients with neonatal hyperammonemia had a higher ratio of peak plasma ammonia level ≥500 μmol/L (P=0.003) and were more likely to receive precision medicine (P=0.027); however, they had a refractory clinical course (P=0.001) and poorer prognosis than the infantile group.Conclusions: There were significant differences in the genetic spectrum, clinical features, clinical course, and outcomes between infants with different hyperammonemia onset ages.

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Plasma ammonia concentrations in extremely low birthweight infants in the first week after birth: secondary analysis from the ProVIDe randomized clinical trial

Cited by 8 publications

References 49 publications

Relationships between Neonatal Nutrition and Growth to 36 Weeks’ Corrected Age in ELBW Babies–Secondary Cohort Analysis from the Provide Trial

Relationships between Neonatal Nutrition and Growth to 36 Weeks’ Corrected Age in ELBW Babies–Secondary Cohort Analysis from the Provide Trial

A glance at transient hyperammonemia of the newborn: Pathophysiology, diagnosis, and treatment: A review

Genetic background and clinical characteristics of infantile hyperammonemia

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