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1.
Harmful Algae ; 133: 102596, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38485444

ABSTRACT

OBJECTIVE: The study objective was to evaluate the association between Karenia brevis (K. brevis) exposure during pregnancy and the prevalence of biliary atresia (BA) in offspring. STUDY DESIGN: This was a hospital-based, case-control study in which cases were infants diagnosed with BA at Johns Hopkins All Children's Hospital from October 2001 to December 2019. Cases were matched 1:4 by age to controls who were randomly selected from a pool of healthy infants hospitalized during the study period for common pediatric diagnoses. Infants were excluded if they had congenital anomalies and/or were non-Florida residents. Gestational K. brevis exposure levels (cells/liter) were determined from Florida Fish and Wildlife Conservation Commission exposure data at 10- and 50 mile radii from the mother's zip code of residence. Multivariable conditional logistic regression determined odds of BA in offspring in relation to maternal gestational K. brevis exposure adjusted for infant sex, race/ethnicity, coastal residence, and seasonality. RESULTS: Of 38 cases and 152 controls, no significant inter-group differences were observed for infant race/ethnicity, season of birth, or coastal residence. Median gestational exposure at the 10 mile radius was 0 cells/liter in both groups. A greater proportion of cases had no gestational K. brevis exposure (63.2 %, n = 24) in comparison to controls (37.5 %, n = 57; p = .04) at a 10 mile radius. At a 50 mile radius, cases had a peak median exposure at 6 months of gestation compared to controls' peak at 9 months. After adjustment for sex, seasonality, race/ethnicity, and coastal residence, there was no significant association between BA and maximum K. brevis exposure per trimester of pregnancy observed at a 10- or 50 mile radius. CONCLUSION: In this matched case-control study, we observed no association between gestational K. brevis (cells/liter) exposure at a 10- or 50 mile radius from maternal zip code of residence and BA in offspring.


Subject(s)
Biliary Atresia , Dinoflagellida , Animals , Humans , Infant , Biliary Atresia/epidemiology , Case-Control Studies , Florida , Harmful Algal Bloom , Marine Toxins , Random Allocation
2.
BMC Pediatr ; 23(1): 440, 2023 09 02.
Article in English | MEDLINE | ID: mdl-37660010

ABSTRACT

BACKGROUND: Sacrococcygeal teratomas (SCTs) are the most common congenital neoplasm and often require resection soon after birth. There are rare reports of cardiac arrest during surgery due to manipulation of the tumor triggering secondary necrosis and hyperkalemia. CASE PRESENTATION: This case describes a very preterm infant with a SCT who develops spontaneous preoperative tumor lysis syndrome (TLS). The medical team utilized rasburicase and the patient underwent total gross resection at 40 h of life. CONCLUSIONS: We emphasize the importance of the early recognition and management of tumor lysis syndrome in SCT with rasburicase, aggressive management of hyperkalemia and consideration of early resection of SCTs even in the case of a very premature infant.


Subject(s)
Hyperkalemia , Infant, Premature, Diseases , Teratoma , Tumor Lysis Syndrome , Infant, Newborn , Infant , Humans , Infant, Premature , Teratoma/complications , Teratoma/surgery , Aggression , Infant, Premature, Diseases/surgery
5.
Neuromuscul Disord ; 32(2): 166-169, 2022 02.
Article in English | MEDLINE | ID: mdl-35094889

ABSTRACT

PURA syndrome is a rare, clinically heterogeneous disorder characterized by a wide spectrum of neurodevelopmental problems, and occasionally congenital heart defects, urogenital malformations, skeletal abnormalities and endocrine disorders. We describe the hospital course, diagnostic evaluations as well as neurologic and neuromuscular follow up of an infant diagnosed with PURA syndrome based on a pathogenic deletion at c.697_699 (p.Phe233del) of the PURA gene identified on whole exome sequencing. Upon initial examination, fluctuation of neuromuscular tone and reflexes were noted in conjunction with hypotonia and severe apneic episodes, suggestive of neuromuscular junction involvement. A definitive role of the neuromuscular junction has not been previously reported with PURA syndrome. The infant was started on pyridostigmine, an acetylcholinesterase inhibitor, with significant improvement in neuromuscular tone and motor movements. In addition, pyridostigmine also resulted in resolution of apneas and improved respiratory status which suggests its potential therapeutic role in patients with PURA syndrome.


Subject(s)
Channelopathies , Epilepsy , Intellectual Disability , Acetylcholinesterase/genetics , Apnea , DNA-Binding Proteins/genetics , Epilepsy/genetics , Humans , Infant , Intellectual Disability/genetics , Pyridostigmine Bromide/therapeutic use , Transcription Factors/genetics
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