scholarly journals Necrotizing Enterocolitis and Respiratory Distress Syndrome as First Clinical Presentation of Mitochondrial Trifunctional Protein Deficiency

Author(s):  
Eugène F. Diekman ◽  
Carolien C. A. Boelen ◽  
Berthil H. C. M. T. Prinsen ◽  
Lodewijk IJlst ◽  
Marinus Duran ◽  
...  
Author(s):  
Anna V. Oláh ◽  
Robert G. Price ◽  
László Csáthy ◽  
Éva Országh ◽  
Éva Oláh ◽  
...  

AbstractSerum N-acetyl-β-D-glucosaminidase (NAG; EC 3.2.1.30) is a hexosaminidase and may be a predictor of vascular injury, e.g., in infant respiratory distress syndrome, pneumonia, broncho-pulmonary dysplasia and necrotizing enterocolitis. To estimate the new diagnostic prospects we have modified our urinary NAG assay. In this sensitive colorimetric micro-assay, VRA-GlcNAc was used as a substrate. In the present study the age dependence of serum NAG activity was investigated in newborn babies, infants (1–24 months), children (2–18 years) and adults (19–80 years). Serum NAG activity was found to be age-dependent; it is higher in early childhood (11–59 U/l) but decreases to a constant value at the age of 1–2 years. After the age of 2 years it is similar to adults' NAG (10–30 U/l). In pediatrics age-matched reference ranges must be taken into consideration.


Author(s):  
Dr. Samiya Al Hashmi ◽  
Dr. Jazel Manarang ◽  
Dr. Hussein Al Lawati ◽  
Dr. Mujtaba. A. Al Ajmi

Congenital surfactant deficiency is a rare condition diagnosed in newborns who present with respiratory distress at birth. We report a case of a term Omani neonate with fatal surfactant protein deficiency who was admitted to the Neonatal Intensive Care Unit (NICU)of the Royal Hospital with respiratory distress syndrome with persistent interstitial infiltrates on serial chest x-ray responsive to intermittent surfactant administration. He underwent a lung biopsy, and immunohistochemistry confirmed the diagnosis of congenital surfactant protein deficiency. However, despite aggressive treatment and supportive measures, his condition rapidly deteriorated, and he succumbed after two months of admission. This case report will highlight and review surfactant deficiency differential diagnoses, management, and complications.


2019 ◽  
Vol 10 (3) ◽  
pp. 2014-2016
Author(s):  
Ahmed Siddique A ◽  
Jagadeesan M ◽  
Mariraj I ◽  
Ramkumar M ◽  
Prasanna Karthik S1 ◽  
...  

Acute respiratory distress syndrome (ARDS) is a disease, mainly occurring in critically ill patients. The systemic spread of infections mainly causes ARDS due to the seepage of fluid in the spaces of the lung . Scrub typhus is a infection caused by . The bite of mite transmits it. Scrub typhus is frequently due to its non-specific clinical presentation and relatively low level of suspicion in treating physicians. The clinical presentation of scrub typhus is varied from fever, , rashes, headache, to pneumonia, acute respiratory distress syndrome, sepsis, central nervous system involvement. The disease is usually indistinguishable from other febrile illness like enteric fever, disease, malaria and certain viral hemorrhagic fevers. Identification of an points to the diagnosis without which the diagnosis is based on a high index of clinical suspicion. We report an interesting case of scrub typhus presenting as acute respiratory distress syndrome.


PEDIATRICS ◽  
1982 ◽  
Vol 70 (2) ◽  
pp. 329-330
Author(s):  
Molly A. Droge ◽  
Catherine V. Jewett ◽  
Frank C. Schmalstieg

The review of adult respiratory distress syndrome (ARDS) in children by Lyrene and Truog1 summarizes their experience in 15 children ranging in age from 15 months to 14 years. We wish to emphasize that this syndrome may occur in very young infants. We have cared for a previously well 6-week-old infant who had the clinical presentation, course, and ventilatory data compatible with ARDS. The infant was found dusky and gasping in her crib and was noted at a local hospital to be tachypneic and in marked respiratory distress (Pao2 was 29 mm Hg on room air).


2020 ◽  
Vol 24 (4) ◽  
pp. 611-617
Author(s):  
A. V. Bolonska ◽  
O. Yu. Sorokina

Annotation. Bronchopulmonary dysplasia and necrotizing enterocolitis have become modern problems of effective care of premature neonates. These two pathologies significantly delay the discharge of a premature baby from the hospital, lead to significant economic costs and worsen the quality of life of these patients. The aim of the study was to identify controlled predictors of bronchopulmonary dysplasia, however, in the analysis of the initial status of patients obtained useful results for the parallel treatment of necrotizing enterocolitis. The study recruited 133 neonates with a gestational age of 28–32 weeks with a diagnosis of respiratory distress syndrome on the basis of two NICU in Dnipro in the period from 2016 to 2020. According to the results of the study in the structure of treatment of premature neonates there were significant risks of bronchopulmonary dysplasia: the duration of respiratory support by mechanical ventilation, non-invasive ventilation, additional oxygenation, nebulizer therapy, and for necrotizing enterocolitis – lower fluid intake, hemoglobin level in 1, 3, 7 days of life, moderate and severe asphyxia. Some of the approaches in therapy are cross-cutting, such as the prevention of anemia in respiratory distress syndrome, fluid intake differences and intensive care methods reducing the duration of respiratory support for the prevention of late neonatal sepsis, we can create an algorithm that takes into account all the risks and enhance outcome for these patients. The perspectives for future work – research of neurological status of former premature neonates and finding out predictors of cerebral palsy.


2016 ◽  
Vol 0 (4.75) ◽  
pp. 108
Author(s):  
L.O. Maltseva ◽  
M.F. Mosentsev ◽  
D.V. Bazylenko ◽  
O.M. Bilan ◽  
L.V. Kunik

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