AJP ReportsPub Date : 2021-04-01Epub Date: 2021-06-23DOI: 10.1055/s-0041-1731057
Sara Madureira Gomes, Rita Pissarra Teixeira, Gustavo Rocha, Paulo Soares, Hercilia Guimaraes, Paulo Santos, Joana Jardim, João Luís Barreira, Helena Pinto
{"title":"Neonatal Atypical Hemolytic Uremic Syndrome in the Eculizumab Era.","authors":"Sara Madureira Gomes, Rita Pissarra Teixeira, Gustavo Rocha, Paulo Soares, Hercilia Guimaraes, Paulo Santos, Joana Jardim, João Luís Barreira, Helena Pinto","doi":"10.1055/s-0041-1731057","DOIUrl":"https://doi.org/10.1055/s-0041-1731057","url":null,"abstract":"<p><p>The atypical hemolytic uremic syndrome (aHUS) in the newborn is a rare disease, with high morbidity. Eculizumab, considered a first-line drug in older children, is not approved in neonates and in children weighing less than 5 kg. We present a 5-day-old female newborn, born at 36 weeks' twin gestation, by emergency cesarean section due to cord prolapse, with birth weight of 2,035 g and Apgar score of 7/7/7, who develops microangiopathic hemolytic anemia, thrombocytopenia, and progressive acute renal failure. In day 5, after diagnosis of aHUS, a daily infusion of fresh frozen plasma begins, with improvement of thrombocytopenia and very slight improvement in renal function. The etiologic study (congenital infection, Shiga toxin, ADAMTS13 activity, directed metabolic study) was normal. C3c was slightly decreased. On day 16 for maintenance of anemia and severe renal failure, she started 300 mg/dose eculizumab. Anemia resolves in 10 weeks and creatinine has normal values after 13 weeks of treatment. The genetic study was normal. In this case, eculizumab is effective in controlling microangiopathy and in the recovery of renal function. Diagnosis of neonatal aHUS can be challenging because of phenotypic heterogeneity and potential overlap with other manifestations that may confound it, such as perinatal asphyxia or sepsis/disseminated intravascular coagulation.</p>","PeriodicalId":7645,"journal":{"name":"AJP Reports","volume":"11 2","pages":"e95-e98"},"PeriodicalIF":0.9,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0041-1731057","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39032471","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
AJP ReportsPub Date : 2021-04-01Epub Date: 2021-06-23DOI: 10.1055/s-0041-1731315
Aashika Janwadkar, Shirley Louis, Sheri L Nemerofsky
{"title":"Acute Otitis Media in an Extremely Preterm Infant.","authors":"Aashika Janwadkar, Shirley Louis, Sheri L Nemerofsky","doi":"10.1055/s-0041-1731315","DOIUrl":"https://doi.org/10.1055/s-0041-1731315","url":null,"abstract":"<p><p>There are a limited number of documented cases of acute otitis media (AOM) in preterm infants while hospitalized in the neonatal intensive care unit (NICU). We present a case of a former 26 weeks old infant who at 29 weeks, 6 days postmenstrual age presented with acute signs and symptoms of systemic sepsis subsequently found to be secondary to AOM with purulent ear drainage. The patient received a septic evaluation, including urine, blood, and cerebrospinal fluid studies. Treatment included intravenous antibiotics with full resolution of symptoms. AOM in extreme preterm infants is multifactorial, with leading causes that include prematurity, the use of oropharyngeal and nasogastric tube placement and endotracheal intubation, eustachian tube dysfunction, and a distinct immune response. To our knowledge, there is not another published case of AOM of a preterm baby while in the NICU.</p>","PeriodicalId":7645,"journal":{"name":"AJP Reports","volume":"11 2","pages":"e99-e101"},"PeriodicalIF":0.9,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0041-1731315","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39032472","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
AJP ReportsPub Date : 2021-04-01Epub Date: 2021-06-16DOI: 10.1055/s-0041-1728783
Benjamin R Harding, Farha Vora
{"title":"Report of a Confirmed SARS-CoV-2 Positive Newborn after Delivery Despite Negative SARS-CoV-2 Testing on Both Parents.","authors":"Benjamin R Harding, Farha Vora","doi":"10.1055/s-0041-1728783","DOIUrl":"https://doi.org/10.1055/s-0041-1728783","url":null,"abstract":"<p><p>We present a case of a term infant born to an asymptomatic mother at a community hospital who required transfer to a local neonatal intensive care unit (NICU) immediately after birth for respiratory distress. The infant was tested for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) at 24 hours of life by reverse transcription polymerase chain reaction (RT-PCR) testing due to the absence of prenatal maternal COVID-19 testing and was found to be positive for SARS-CoV-2 at that time. A second RT-PCR test was obtained on the infant on day of life (DOL) 4 and was also positive, confirming an accurate diagnosis of COVID-19 disease in the infant. Both the mother and father remained asymptomatic and concomitantly tested negative for SARS-CoV-2 on two separate occasions. The infant subsequently clinically improved and was discharged without any complications. This case raises the potential concern for two unreported newborn issues related to COVID-19. First, the potential unreliability of negative maternal COVID-19 testing surrounding the time of delivery as it relates to routine newborn testing and isolation needs, and second, if the negative material testing was accurate, this raises the concern for a potential case of nosocomial COVID-19 infection within the first 24 hours of life.</p>","PeriodicalId":7645,"journal":{"name":"AJP Reports","volume":"11 2","pages":"e80-e83"},"PeriodicalIF":0.9,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0041-1728783","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39250273","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
AJP ReportsPub Date : 2021-04-01Epub Date: 2021-08-19DOI: 10.1055/s-0041-1732407
Betty Pham, Anne Denslow, Michel Mikhael, Jina Lim
{"title":"A Case of an Extremely Low Birth Weight Infant with <i>Morganella morganii</i> Bacteremia and Peritonitis.","authors":"Betty Pham, Anne Denslow, Michel Mikhael, Jina Lim","doi":"10.1055/s-0041-1732407","DOIUrl":"https://doi.org/10.1055/s-0041-1732407","url":null,"abstract":"<p><p>We describe a case of late onset <i>Morganella morganii</i> sepsis in an extremely low birth weight male neonate born at 23 and 4/7 weeks gestational age to a 30-year-old primigravid mother due to preterm labor. The mother was otherwise healthy with an unremarkable prenatal course. She received steroids and ampicillin prior to delivery. While initial blood cultures were negative, at day of life 4, the neonate developed signs of sepsis with leukocytosis and bandemia, and subsequent blood culture demonstrated growth of <i>M. morganii</i> . The patient then had spontaneous intestinal perforation on day of life 8 with peritoneal cultures growing <i>M. morganii</i> . The infant responded to standard therapy and survived to discharge, with few mild developmental delays upon outpatient follow-up. While <i>M. morganii</i> has been demonstrated in the neonatal population, it generally causes early onset sepsis and is associated with high mortality in preterm neonates. Here, we present this case of late onset neonatal sepsis with <i>M. morganii</i> complicated by spontaneous intestinal perforation, with survival in a 23 weeks gestation infant.</p>","PeriodicalId":7645,"journal":{"name":"AJP Reports","volume":"11 2","pages":"e113-e118"},"PeriodicalIF":0.9,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b1/fd/10-1055-s-0041-1732407.PMC8376401.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39348472","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
AJP ReportsPub Date : 2021-04-01Epub Date: 2021-06-23DOI: 10.1055/s-0041-1728782
Gloria Akuamoah-Boateng, Raymond C Stetson, Bethany D Kaemingk, David A Bieber, Jane E Brumbaugh
{"title":"Profound Hypotonia and Respiratory Failure due to Suspected Nemaline Myopathy in a Preterm Infant.","authors":"Gloria Akuamoah-Boateng, Raymond C Stetson, Bethany D Kaemingk, David A Bieber, Jane E Brumbaugh","doi":"10.1055/s-0041-1728782","DOIUrl":"https://doi.org/10.1055/s-0041-1728782","url":null,"abstract":"<p><p>Congenital myopathies, such as nemaline myopathy, may present with hypotonia and respiratory failure in the neonatal period. Respiratory function can be further compromised in affected infants by the development of chylous effusions. We present the case of a preterm male infant born at 32 <sup>6/7</sup> weeks' gestation, who was profoundly hypotonic and required intubation at birth. His clinical course progressed from acute to chronic respiratory failure with mechanical ventilation dependence. He developed bilateral chylous pleural effusions during the newborn period. Whole exome sequencing identified an <i>ACTA1</i> gene mutation leading to the presumed diagnosis of nemaline myopathy. This case highlights the need to include congenital myopathies in the differential for a preterm newborn with hypotonia and respiratory failure.</p>","PeriodicalId":7645,"journal":{"name":"AJP Reports","volume":"11 2","pages":"e91-e94"},"PeriodicalIF":0.9,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0041-1728782","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39032469","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
AJP ReportsPub Date : 2021-04-01Epub Date: 2021-06-30DOI: 10.1055/s-0041-1731311
Veronica Mugarab-Samedi, Michelle D Ryan, Essa Hamdan Al Awad, Adel Elsharkawy
{"title":"The Effect of Prenatal and Postnatal Treatment with Intravenous Immunoglobulin on Severity of Neonatal Hemochromatosis: The Tale of Two Brothers (Case Report).","authors":"Veronica Mugarab-Samedi, Michelle D Ryan, Essa Hamdan Al Awad, Adel Elsharkawy","doi":"10.1055/s-0041-1731311","DOIUrl":"https://doi.org/10.1055/s-0041-1731311","url":null,"abstract":"<p><p><b>Background</b> Neonatal hemochromatosis (NH) is a rare condition that was the main reason for liver transplantation in infants. With the realization that NH results from the fetal complement-mediated liver injury, intravenous immunoglobulins (IVIG) were successfully introduced for the treatment. <b>Case Presentation</b> We present two cases of NH from the same family to illustrate the role of antenatal treatment with IVIG in alleviation and possible prevention of this serious morbidity. <b>Conclusion</b> A prenatal treatment and early postnatal administration of IVIG are effective ways to manage NH that help to reduce the severity of the symptoms, prevent liver failure, and avoid the need for liver transplantation.</p>","PeriodicalId":7645,"journal":{"name":"AJP Reports","volume":"11 2","pages":"e102-e104"},"PeriodicalIF":0.9,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0041-1731311","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39149945","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
AJP ReportsPub Date : 2021-03-01Epub Date: 2021-05-27DOI: 10.1055/s-0041-1727287
Rishika P Sakaria, Roya Mostafavi, Stephen Miller, Jewell C Ward, Eniko K Pivnick, Ajay J Talati
{"title":"Kagami-Ogata Syndrome: Case Series and Review of Literature.","authors":"Rishika P Sakaria, Roya Mostafavi, Stephen Miller, Jewell C Ward, Eniko K Pivnick, Ajay J Talati","doi":"10.1055/s-0041-1727287","DOIUrl":"https://doi.org/10.1055/s-0041-1727287","url":null,"abstract":"<p><p>Kagami-Ogata syndrome (KOS) (OMIM #608149) is a genetic imprinting disorder affecting chromosome 14 that results in a characteristic phenotype consisting of typical facial features, skeletal abnormalities including rib abnormalities described as \"coat hanger ribs,\" respiratory distress, abdominal wall defects, polyhydramnios, and developmental delay. First identified by Wang et al in 1991, over 80 cases of KOS have been reported in the literature. KOS, however, continues to remain a rare and potentially underdiagnosed disorder. In this report, we describe two unrelated male infants with differing initial presentations who were both found to have the characteristic \"coat hanger\" rib appearance on chest X-ray, raising suspicion for KOS. Molecular testing confirmed KOS in each case. In addition to these new cases, we reviewed the existing cases reported in literature. Presence of polyhydramnios, small thorax, curved ribs, and abdominal wall defects must alert the perinatologist toward the possibility of KOS to facilitate appropriate molecular testing. The overall prognosis of KOS remains poor. Early diagnosis allows for counseling by a multidisciplinary team and enables parents to make informed decisions regarding both pregnancy management and postnatal care.</p>","PeriodicalId":7645,"journal":{"name":"AJP Reports","volume":"11 2","pages":"e65-e75"},"PeriodicalIF":0.9,"publicationDate":"2021-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0041-1727287","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38953807","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Severe Consumptive Coagulopathy in an Extremely-Low-Birth-Weight Infant with Intra-Abdominal Umbilical Vein Varix: A Case Report.","authors":"Mitsuhiro Haga, Kanako Itoh, Tsuguhiro Horikoshi, Fumihiko Namba, Kazuhiko Kabe","doi":"10.1055/s-0041-1727288","DOIUrl":"https://doi.org/10.1055/s-0041-1727288","url":null,"abstract":"<p><p>Recent studies have shown favorable outcomes for intra-abdominal umbilical vein varices (IUVVs) in term neonates who have no other complications. Little is known, however, about the prognosis of IUVVs in preterm neonates. We encountered a case of IUVV in an extremely low-birth-weight infant who developed severe consumptive coagulopathy after birth. The patient's coagulation test normalized as the varix spontaneously obstructed. Although life-threatening hemorrhagic complications were avoided, a cerebellum hemorrhage was found in the brain magnetic resonance imaging at the term-equivalent age. In a literature survey, coagulopathy was reported in 4 out of 15 infants with IUVVs born before 34 weeks of gestation, including our present case. Preterm infants with IUVVs may develop coagulopathy because of the prematurity of their coagulation-fibrinolysis systems. Attention should be given to the coagulation status of preterm neonates with IUVVs.</p>","PeriodicalId":7645,"journal":{"name":"AJP Reports","volume":"11 2","pages":"e76-e79"},"PeriodicalIF":0.9,"publicationDate":"2021-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0041-1727288","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38953808","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
AJP ReportsPub Date : 2021-03-01Epub Date: 2021-05-27DOI: 10.1055/s-0041-1727257
Jelle W Raats, Lievay van Dam, Pieter J van Doormaal, Marjoleine van Hengel-Jacobs, Hester Langeveld-Benders
{"title":"Neonatal Rupture of the Spleen: Successful Treatment with Splenic Artery Embolization.","authors":"Jelle W Raats, Lievay van Dam, Pieter J van Doormaal, Marjoleine van Hengel-Jacobs, Hester Langeveld-Benders","doi":"10.1055/s-0041-1727257","DOIUrl":"https://doi.org/10.1055/s-0041-1727257","url":null,"abstract":"<p><p>Neonatal intra-abdominal hemorrhage has been rarely reported in the literature. We report a case of splenic injury in a neonate, highlighting the importance of a high-index suspicion in early recognition of this rare and potentially fatal injury. We report the first case of a neonate who had a splenic rupture and underwent successful endovascular treatment.</p>","PeriodicalId":7645,"journal":{"name":"AJP Reports","volume":"11 2","pages":"e58-e60"},"PeriodicalIF":0.9,"publicationDate":"2021-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0041-1727257","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38953805","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
AJP ReportsPub Date : 2021-03-01Epub Date: 2021-05-27DOI: 10.1055/s-0041-1727261
Mahmoud Ali, Lea Mallett, Greg Miller
{"title":"Refractory Pulmonary Interstitial Emphysema in Extreme Premature Newborn.","authors":"Mahmoud Ali, Lea Mallett, Greg Miller","doi":"10.1055/s-0041-1727261","DOIUrl":"https://doi.org/10.1055/s-0041-1727261","url":null,"abstract":"<p><p>Pulmonary interstitial emphysema (PIE) occurs when air leaks into the pulmonary interstitium due to overdistension of distal airways, it occurs mainly in neonates with respiratory distress syndrome who need positive pressure ventilation but has also been reported in spontaneously breathing infants. Herein, we report on an extremely low birth weight infant with severe persistent PIE, while on invasive mechanical ventilation (high-frequency oscillatory ventilation, high-frequency jet ventilation, and neurally adjust ventilator assist) managed successfully with 2 weeks of selective right lung ventilation after failure of more conservative measures, including shorter periods of right mainstem intubation, before the prolonged trial that was successful.</p>","PeriodicalId":7645,"journal":{"name":"AJP Reports","volume":"11 2","pages":"e61-e64"},"PeriodicalIF":0.9,"publicationDate":"2021-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0041-1727261","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38953806","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}