Related Experiment Video

Updated: Oct 8, 2025

Early Pathological and Magnetic Resonance Detection of Cerebral Injury Using a Rat Model of Neonatal Hypoxic Ischemic Encephalopathy
05:52

Early Pathological and Magnetic Resonance Detection of Cerebral Injury Using a Rat Model of Neonatal Hypoxic Ischemic Encephalopathy

Published on: October 28, 2022

863

Thiamine-responsive, life-threatening, pulmonary hypertensive crisis with encephalopathy in young infants: A case

Suryakanthi C1, P Keerthi Kundana2, Nihaal Reddy3

  • 1Department of Pediatric Critical Care, Rainbow Children's Hospital, Secunderabad, Telangana, India.

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society
|December 24, 2021
PubMed

Insights

Infants with severe encephalopathy and pulmonary hypertensive crisis due to Thiamine (Vitamin B1) deficiency show rapid improvement with early supplementation. This rare condition requires high clinical suspicion for prompt diagnosis and treatment.

Area of Science:

  • Pediatrics
  • Nutritional Neuroscience
  • Cardiology

Background:

  • Thiamine (Vitamin B1) deficiency (TD) is uncommon in infants but can present with severe, life-threatening symptoms.
  • A rare presentation involves severe encephalopathy and pulmonary hypertensive crisis.

Purpose of the Study:

  • To describe a rare form of infantile TD.
  • Highlight the clinical presentation and response to thiamine supplementation.

Main Methods:

  • Descriptive case series of six infants (1-3 months old).
  • Infants presented with encephalopathy and cardio-pulmonary arrest.
  • Evaluations included cardiac, neuroimaging, and metabolic assessments.

Main Results:

  • All infants exhibited severe pulmonary arterial hypertension (PAH), shock, metabolic acidosis, and encephalopathy.
  • Exclusive breastfeeding was noted in all cases.
  • Thiamine treatment led to dramatic improvements in hemodynamic and neurological function with no major deficits on follow-up.

Conclusions:

  • Early identification of this rare infantile TD is crucial.
  • A high index of suspicion is warranted to prevent mortality and morbidity.
  • Prompt thiamine supplementation can reverse severe clinical manifestations.
Abstract

Keywords:
EncephalopathyPulmonary arterial hypertension crisisThiamine deficiency

More Related Videos

Modeling Encephalopathy of Prematurity Using Prenatal Hypoxia-ischemia with Intra-amniotic Lipopolysaccharide in Rats07:36

Modeling Encephalopathy of Prematurity Using Prenatal Hypoxia-ischemia with Intra-amniotic Lipopolysaccharide in Rats

Published on: November 20, 2015

11.5K
Author Spotlight: Assessing the Feasibility of Using Amplitude-Integrated EEG During Neonatal Transport05:15

Author Spotlight: Assessing the Feasibility of Using Amplitude-Integrated EEG During Neonatal Transport

Published on: June 21, 2024

900

Related Concept Videos

Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
296
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses...
20
Teratogenicity01:07

Teratogenicity

The ability of a drug to produce structural deformations and functional abnormalities in the developing embryo or the fetus is called teratogenicity, and the drug producing this effect is known as a teratogen. Teratogenic effects include stillbirth, miscarriage, intrauterine growth restriction, and neurocognitive delay. A teratogen may affect the embryo at different stages of development, which is important in determining the type and extent of the damage. During blastocyst formation, the early...
3.0K
Drug Dosing: Infants and Children01:29

Drug Dosing: Infants and Children

Pediatric patient dosages diverge from adults due to disparities in body surface area, total body water, and extracellular fluid per kilogram of body weight. The dosing regimen considers the variations in pharmacokinetics and pharmacology across distinct age groups, encompassing preterm newborns, infants, young children, older children, and adolescents. Calculation of pediatric patient doses is predicated on determining body surface area, which exhibits a superior correlation with the child's...
24
Vitamins01:30

Vitamins

Vitamins, derived from the Latin word for life, are essential organic substances required in small quantities for optimal growth and overall well-being. Unlike other organic nutrients, vitamins don't act as sources of energy or building materials but rather facilitate these nutrients' utilization by the body. Vitamins are predominantly coenzymes, assisting enzymes in specific chemical actions, like the oxidation of glucose for energy involving B vitamins. Most vitamins are not produced...
1.1K