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  1. Article: That head lag is impressive! Infantile botulism in the NICU: a case report.

    Wardinger, Jaimie E / Darwish, Nada / Amatya, Shaili

    Maternal health, neonatology and perinatology

    2024  Volume 10, Issue 1, Page(s) 1

    Abstract: Background: Infantile botulism (IB) is a devastating and potentially life-threatening neuromuscular disorder resulting from intestinal colonization by Clostridium botulinum and the resultant toxin production. It can present with constipation, descending ...

    Abstract Background: Infantile botulism (IB) is a devastating and potentially life-threatening neuromuscular disorder resulting from intestinal colonization by Clostridium botulinum and the resultant toxin production. It can present with constipation, descending paralysis, and, potentially, respiratory failure. Botulism is a diagnosis that is more commonly seen in the pediatric intensive care unit (PICU) or on the general pediatric wards and would not typically be managed in the neonatal intensive care unit (NICU), and therefore requires high clinical suspicion to ensure prompt diagnosis and treatment.
    Case presentation: We discuss a case where an infant from central Pennsylvania presented to a Level IV NICU rather than to the PICU for an evaluation for sepsis and was uniquely diagnosed with IB. The infant presented with poor oral feeding and reduced oral intake, hypothermia, and lethargy. His symptoms progressed into hypoxia and acute respiratory failure. Interestingly, this infant had no known exposure to honey or any other identifiable sources of botulism contact. The infant's twin brother and the other infants who attended the mother's in-home daycare remained asymptomatic. This infant was initially evaluated and managed for a potential infectious etiology. However, a diagnosis of IB was suspected, and was later confirmed through the detection of botulinum toxin in the infant's stools. A high level of suspicion allowed for timely treatment with Botulism Immune Globulin neutralizing antibodies (BabyBIG), even prior to confirmatory testing. We describe the process of obtaining BabyBIG, as well as the natural course of illness after treatment in our patient who ultimately made a complete recovery.
    Conclusions: This case highlights the importance of considering infantile botulism as a diagnostic possibility even in the absence of risk factors, and the need for vigilance in diagnosing and treating this rare but potentially life-threatening condition. With timely recognition, subsequent treatment with BabyBIG, and supportive care, infants with infantile botulism can be expected to recover completely. This information is particularly important for neonatologists providing care for infants outside the neonatal period, especially during times of high patient census and resulting overflow of pediatric admissions in the NICU.
    Language English
    Publishing date 2024-01-02
    Publishing country England
    Document type Journal Article
    ZDB-ID 2809925-4
    ISSN 2054-958X
    ISSN 2054-958X
    DOI 10.1186/s40748-023-00172-2
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: That head lag is impressive! Infantile botulism in the NICU

    Jaimie E Wardinger / Nada Darwish / Shaili Amatya

    Maternal Health, Neonatology and Perinatology, Vol 10, Iss 1, Pp 1-

    a case report

    2024  Volume 5

    Abstract: Abstract Background Infantile botulism (IB) is a devastating and potentially life-threatening neuromuscular disorder resulting from intestinal colonization by Clostridium botulinum and the resultant toxin production. It can present with constipation, ... ...

    Abstract Abstract Background Infantile botulism (IB) is a devastating and potentially life-threatening neuromuscular disorder resulting from intestinal colonization by Clostridium botulinum and the resultant toxin production. It can present with constipation, descending paralysis, and, potentially, respiratory failure. Botulism is a diagnosis that is more commonly seen in the pediatric intensive care unit (PICU) or on the general pediatric wards and would not typically be managed in the neonatal intensive care unit (NICU), and therefore requires high clinical suspicion to ensure prompt diagnosis and treatment. Case presentation We discuss a case where an infant from central Pennsylvania presented to a Level IV NICU rather than to the PICU for an evaluation for sepsis and was uniquely diagnosed with IB. The infant presented with poor oral feeding and reduced oral intake, hypothermia, and lethargy. His symptoms progressed into hypoxia and acute respiratory failure. Interestingly, this infant had no known exposure to honey or any other identifiable sources of botulism contact. The infant’s twin brother and the other infants who attended the mother’s in-home daycare remained asymptomatic. This infant was initially evaluated and managed for a potential infectious etiology. However, a diagnosis of IB was suspected, and was later confirmed through the detection of botulinum toxin in the infant’s stools. A high level of suspicion allowed for timely treatment with Botulism Immune Globulin neutralizing antibodies (BabyBIG), even prior to confirmatory testing. We describe the process of obtaining BabyBIG, as well as the natural course of illness after treatment in our patient who ultimately made a complete recovery. Conclusions This case highlights the importance of considering infantile botulism as a diagnostic possibility even in the absence of risk factors, and the need for vigilance in diagnosing and treating this rare but potentially life-threatening condition. With timely recognition, subsequent treatment with BabyBIG, and ...
    Keywords Infantile botulism ; BabyBIG ; Infant ; Sepsis ; Medicine ; R
    Subject code 610
    Language English
    Publishing date 2024-01-01T00:00:00Z
    Publisher BMC
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  3. Article ; Online: Disruption of the SP-A/SP-R210

    Guan, Zhiwei / Worth, Brandon / Umstead, Todd M / Amatya, Shaili / Booth, Jennifer / Chroneos, Zissis C

    American journal of physiology. Lung cellular and molecular physiology

    2024  Volume 326, Issue 4, Page(s) L508–L513

    Abstract: Prolonged labor can lead to infection, fetal distress, asphyxia, and life-threatening harm to both the mother and the baby. Surfactant protein A (SP-A) was shown to contribute to the maintenance of pregnancy and timing of term labor. SP-A modulates the ... ...

    Abstract Prolonged labor can lead to infection, fetal distress, asphyxia, and life-threatening harm to both the mother and the baby. Surfactant protein A (SP-A) was shown to contribute to the maintenance of pregnancy and timing of term labor. SP-A modulates the stoichiometric expression of the SP-R210
    MeSH term(s) Female ; Pregnancy ; Animals ; Mice ; Lipopolysaccharides/adverse effects ; Pulmonary Surfactant-Associated Protein A/genetics ; Pulmonary Surfactant-Associated Protein A/metabolism ; Parturition/metabolism ; Fetus/metabolism ; Inflammation/metabolism
    Chemical Substances Lipopolysaccharides ; Pulmonary Surfactant-Associated Protein A
    Language English
    Publishing date 2024-02-13
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1013184-x
    ISSN 1522-1504 ; 1040-0605
    ISSN (online) 1522-1504
    ISSN 1040-0605
    DOI 10.1152/ajplung.00383.2023
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article: Imaging NAD(H) Redox Alterations in Cryopreserved Alveolar Macrophages from Ozone-Exposed Mice and the Impact of Nutrient Starvation during Long Lag Times.

    Xu, He N / Floros, Joanna / Li, Lin Z / Amatya, Shaili

    Antioxidants (Basel, Switzerland)

    2021  Volume 10, Issue 5

    Abstract: Employing the optical redox imaging technique, we previously identified a significant redox shift of nicotinamide adenine dinucleotide (NAD and the reduced form NADH) in freshly isolated alveolar macrophages (AM) from ozone-exposed mice. The goal here ... ...

    Abstract Employing the optical redox imaging technique, we previously identified a significant redox shift of nicotinamide adenine dinucleotide (NAD and the reduced form NADH) in freshly isolated alveolar macrophages (AM) from ozone-exposed mice. The goal here was twofold: (a) to determine the NAD(H) redox shift in cryopreserved AM isolated from ozone-exposed mice and (b) to investigate whether there is a difference in the redox status between cryopreserved and freshly isolated AM. We found: (i) AM from ozone-exposed mice were in a more oxidized redox state compared to that from filtered air (FA)-exposed mice, consistent with the results obtained from freshly isolated mouse AM; (ii) under FA exposure, there was no significant NAD(H) redox difference between fresh AM that had been placed on ice for 2.5 h and cryopreserved AM; however, under ozone exposure, fresh AM were more oxidized than cryopreserved AM; (iii) via the use of nutrient starvation and replenishment and H
    Language English
    Publishing date 2021-05-12
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2704216-9
    ISSN 2076-3921
    ISSN 2076-3921
    DOI 10.3390/antiox10050767
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article: Single Nucleotide Polymorphisms Interactions of the Surfactant Protein Genes Associated With Respiratory Distress Syndrome Susceptibility in Preterm Infants.

    Amatya, Shaili / Ye, Meixia / Yang, Lili / Gandhi, Chintan K / Wu, Rongling / Nagourney, Beth / Floros, Joanna

    Frontiers in pediatrics

    2021  Volume 9, Page(s) 682160

    Abstract: Background: ...

    Abstract Background:
    Language English
    Publishing date 2021-10-04
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2021.682160
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Hedgehog signaling pathway gene variant influences bronchopulmonary dysplasia in extremely low birth weight infants.

    Amatya, Shaili / Rajbhandari, Sharina / Pradhan, Sandeep / Trinh, Van / Paudel, Umesh / Parton, Lance A

    World journal of pediatrics : WJP

    2021  Volume 17, Issue 3, Page(s) 298–304

    Abstract: Background: Genome wide association study identified hedgehog interacting protein gene (HHIP) variants with chronic obstructive pulmonary disease and asthma. Loss of HHIP, a key regulator of the hedgehog signaling pathway, leads to impaired lung ... ...

    Abstract Background: Genome wide association study identified hedgehog interacting protein gene (HHIP) variants with chronic obstructive pulmonary disease and asthma. Loss of HHIP, a key regulator of the hedgehog signaling pathway, leads to impaired lung morphogenesis and lethality in animal models, through unimpeded sonic hedgehog expression blocking mesenchymal-expressed fibroblast growth factor 10 (FGF10). Since bronchopulmonary dysplasia (BPD) is also associated with altered lung development and worsens with stimuli including mechanical ventilation, reactive oxygen species, and inflammation, HHIP and FGF10 may be candidate genes.
    Methods: This was an observational, cohort study including extremely low birth weight infants that who developed BPD and those who did not. DNA was isolated from buccal swabs and subjected to allelic discrimination, using specific HHIP and FGF10 probes. Protein levels were measured in tracheal aspirates. Student's t test, Chi-square, Z test and logistic regression were used.
    Results: Demographic characteristics did not differ except that birth weight (715 ± 153 vs. 835 ± 132 g) and gestational age (25 vs. 26 weeks) were less in babies with BPD. HHIP variant rs13147758 (GG genotype) was found to be independently protective for BPD (odds ratio 0.35, 95% confidence interval 0.15-0.82, P = - 0.02). Early airway HHIP protein levels were increased in infants with BPD compared to those without [median (interquartile range) 130.6 (55.6-297.0) and 41.2 (22.1-145.6) pg/mL, respectively; P = 0.05]. The FGF10 single nucleotide polymorphisms were not associated with BPD.
    Conclusion: HHIP, as a regulator of lung bud formation, affects BPD susceptibility, and may be valuable in understanding the specific mechanisms for this disease as well as for identifying therapeutic targets in the era of personalized medicine.
    MeSH term(s) Birth Weight ; Bronchopulmonary Dysplasia/genetics ; Cohort Studies ; Genome-Wide Association Study ; Gestational Age ; Hedgehog Proteins/genetics ; Humans ; Infant, Extremely Low Birth Weight ; Infant, Newborn
    Chemical Substances Hedgehog Proteins
    Language English
    Publishing date 2021-04-16
    Publishing country Switzerland
    Document type Journal Article ; Observational Study
    ZDB-ID 2236681-7
    ISSN 1867-0687 ; 1708-8569
    ISSN (online) 1867-0687
    ISSN 1708-8569
    DOI 10.1007/s12519-021-00427-y
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article: Imaging NAD(H) Redox Alterations in Cryopreserved Alveolar Macrophages from Ozone-Exposed Mice and the Impact of Nutrient Starvation during Long Lag Times

    Xu, He N / Floros, Joanna / Li, Lin Z / Amatya, Shaili

    Antioxidants. 2021 May 12, v. 10, no. 5

    2021  

    Abstract: Employing the optical redox imaging technique, we previously identified a significant redox shift of nicotinamide adenine dinucleotide (NAD and the reduced form NADH) in freshly isolated alveolar macrophages (AM) from ozone-exposed mice. The goal here ... ...

    Abstract Employing the optical redox imaging technique, we previously identified a significant redox shift of nicotinamide adenine dinucleotide (NAD and the reduced form NADH) in freshly isolated alveolar macrophages (AM) from ozone-exposed mice. The goal here was twofold: (a) to determine the NAD(H) redox shift in cryopreserved AM isolated from ozone-exposed mice and (b) to investigate whether there is a difference in the redox status between cryopreserved and freshly isolated AM. We found: (i) AM from ozone-exposed mice were in a more oxidized redox state compared to that from filtered air (FA)-exposed mice, consistent with the results obtained from freshly isolated mouse AM; (ii) under FA exposure, there was no significant NAD(H) redox difference between fresh AM that had been placed on ice for 2.5 h and cryopreserved AM; however, under ozone exposure, fresh AM were more oxidized than cryopreserved AM; (iii) via the use of nutrient starvation and replenishment and H₂O₂-induced oxidative stress of an AM cell line, we showed that this redox difference between cryopreserved and freshly isolated AM is likely the result of the double “hit”, i.e., the ozone-induced oxidative stress plus nutrient starvation that prevented freshly isolated AM from a full recovery after being on ice for a prolonged time period. The cryopreservation technique we developed eliminates/minimizes the effects of oxidative stress and nutrient starvation on cells. This method can be adopted to preserve lung macrophages from animal models or clinical patients for further investigations.
    Keywords NAD (coenzyme) ; air ; cell lines ; cryopreservation ; ice ; lungs ; macrophages ; mice ; oxidation ; oxidative stress ; ozone ; starvation
    Language English
    Dates of publication 2021-0512
    Publishing place Multidisciplinary Digital Publishing Institute
    Document type Article
    Note NAL-AP-2-clean
    ZDB-ID 2704216-9
    ISSN 2076-3921
    ISSN 2076-3921
    DOI 10.3390/antiox10050767
    Database NAL-Catalogue (AGRICOLA)

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  8. Article: Sex and SP-A2 Dependent NAD(H) Redox Alterations in Mouse Alveolar Macrophages in Response to Ozone Exposure: Potential Implications for COVID-19.

    Xu, He N / Lin, Zhenwu / Gandhi, Chintan K / Amatya, Shaili / Wang, Yunhua / Li, Lin Z / Floros, Joanna

    Antioxidants (Basel, Switzerland)

    2020  Volume 9, Issue 10

    Abstract: Co-enzyme nicotinamide adenine dinucleotide (NAD(H)) redox plays a key role in macrophage function. Surfactant protein (SP-) A modulates the functions of alveolar macrophages (AM) and ozone ( ... ...

    Abstract Co-enzyme nicotinamide adenine dinucleotide (NAD(H)) redox plays a key role in macrophage function. Surfactant protein (SP-) A modulates the functions of alveolar macrophages (AM) and ozone (O
    Keywords covid19
    Language English
    Publishing date 2020-09-25
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2704216-9
    ISSN 2076-3921
    ISSN 2076-3921
    DOI 10.3390/antiox9100915
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article: SNP and Haplotype Interaction Models Reveal Association of Surfactant Protein Gene Polymorphisms With Hypersensitivity Pneumonitis of Mexican Population.

    Gandhi, Chintan K / Chen, Chixiang / Amatya, Shaili / Yang, Lili / Fu, Chenqi / Zhou, Shouhao / Wu, Rongling / Buendía-Roldan, Ivette / Selman, Moisés / Pardo, Annie / Floros, Joanna

    Frontiers in medicine

    2021  Volume 7, Page(s) 588404

    Abstract: Background: ...

    Abstract Background:
    Language English
    Publishing date 2021-01-05
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2775999-4
    ISSN 2296-858X
    ISSN 2296-858X
    DOI 10.3389/fmed.2020.588404
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article: Early Salivary miRNA Expression in Extreme Low Gestational Age Newborns.

    Siddaiah, Roopa / Emery, Lucy / Stephens, Heather / Donnelly, Ann / Erkinger, Jennifer / Wisecup, Kimberly / Hicks, Steven D / Kawasawa, Yuka Imamura / Oji-Mmuo, Christiana / Amatya, Shaili / Silveyra, Patricia

    Life (Basel, Switzerland)

    2022  Volume 12, Issue 4

    Abstract: Background: MicroRNAs (miRNA) are small non-coding RNAs that regulate gene expression playing a key role in organogenesis. MiRNAs are studied in tracheal aspirates (TA) of preterm infants. However; this is difficult to obtain in infants who are not ... ...

    Abstract Background: MicroRNAs (miRNA) are small non-coding RNAs that regulate gene expression playing a key role in organogenesis. MiRNAs are studied in tracheal aspirates (TA) of preterm infants. However; this is difficult to obtain in infants who are not intubated. This study examines early salivary miRNA expression as non-invasive early biomarkers in extremely low gestational age newborns (ELGANs). Methods: Saliva was collected using DNA-genotek swabs, miRNAs were analyzed using RNA seq and RT PCR arrays. Salivary miRNA expression was compared to TA using RNA seq at 3 days of age, and longitudinal changes at 28 days of age were analyzed using RT PCR arrays in ELGANs. Results: Approximately 822 ng of RNA was extracted from saliva of 7 ELGANs; Of the 757 miRNAs isolated, 161 miRNAs had significant correlation in saliva and TA at 3 days of age (r = 0.97). Longitudinal miRNA analysis showed 29 miRNAs downregulated and 394 miRNAs upregulated at 28 days compared to 3 days of age (adjusted p < 0.1). Bioinformatic analysis (Ingenuity Pathway Analysis) of differentially expressed miRNAs identified organismal injury and abnormalities and cellular development as the top physiological system development and cellular function. Conclusion: Salivary miRNA expression are source for early biomarkers of underlying pathophysiology in ELGANs.
    Language English
    Publishing date 2022-03-30
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2662250-6
    ISSN 2075-1729
    ISSN 2075-1729
    DOI 10.3390/life12040506
    Database MEDical Literature Analysis and Retrieval System OnLINE

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