Botanical extract combined with minoxidil improve hidrotic ectodermal dysplasia caused by p.G11R mutations: a case report.

PURPOSE: We aim to explore a potential treatment strategy for hair loss. MATERIALS AND METHODS: A male 6-year-old child was diagnosed with hidrotic ectodermal dysplasia 2 (HED2) caused by GJB6 (p.G11R) mutations. He presented at our clinic with diffuse thinning and fine and brittle hair since birth. Additionally, the child exhibited abnormal development of teeth, fingernails, and toenails. The condition of the child's hair had not improved significantly with age. He was treated with botanical extracts combined with Minoxidil. RESULTS: After one and a half months of treatment, the patient showed remarkable hair growth. CONCLUSIONS: Our team has previously used botanical extracts in combination for the treatment of autosomal recessive wooly hair in children. In the present case, treatment with botanical extract combined with minoxidil was found to be equally efficacious. This case report provides valuable information for future studies on the use of botanical extracts in treating hair loss, as well as a safe and effective potential treatment strategy for children with congenital alopecia.

Congenital anomalies in pregnancies with overt and pregestational type 2 diabetes: a gray portrayal from a cohort in Brazil.

OBJECTIVE: To describe the frequency and types of congenital anomalies and associated risk factors in Brazilian women with type 2 diabetes. METHODS: In this retrospective cohort study between 2005 and 2021, we included all pregnant participants with type 2 diabetes from the two major public hospitals in southern Brazil. We collected data from the electronic hospital records. Congenital anomalies were classified by the 10th revised International Classification of Diseases, Q chapter, enhanced by the EUROCAT registry classification, and categorized by type and gravity. We used multiple Poisson regression with robust estimates to estimate risks. RESULTS: Among 648 participants, we excluded 19, and 62 were lost to follow-up; therefore, we included 567 participants. Overt diabetes arose in 191 participants (33.7%, 95% CI 30.0% - 38.0%). Less than 20% of the participants supplemented folate. Congenital anomalies occurred in 78 neonates (13.8%, CI 11.0 - 16.9%), 73 babies (93.6%) presented major anomalies, and 20 (10.5%) cases occurred in participants with overt diabetes. Cardiac anomalies were the most frequent (43 isolated and 12 combined). Pre-eclampsia was associated with an increased risk in the analyses including all women (adjusted RR 1.87 (95% CI 1.23-2.85), p = 0.003), but not in analyses including only women with an HbA1c measured up to the 14th gestational age. HbA1c, either measured at any time in pregnancy (adjusted RR 1.21 (95% CI 1.10-1.33), p < 0.001) or up to the first 14 weeks (adjusted RR 1.22, 95% CI 1.10-1.35, p < 0.001) was the only sustained risk factor. Risk factors such as maternal age, obesity, diabetes diagnosis, or use of antidiabetic medications were not associated with congenital anomalies. CONCLUSION: We found a high frequency of congenital anomalies associated with poor maternal glycemic control and revealed an almost universal lack of preconception care. An urgent call to action is mandatory for the reversal of this gray scenario.

True uterus didelphys in she-camel: a case report and review of literature.

Background: Uterus didelphys is a rare congenital anomaly of the female reproductive tract characterized by a divided uterine cervix and body. It occurs due to abnormal development of the paramesonephric (Müllerian) duct. Different forms of uterus didelphys have been reported in several animal species, including bovine, equine, ewe, goat, swine, and bitch. However, there is no previous report that has documented a completely divided female genital tract in she-camel. Moreover, there is a lack of literature regarding this anomaly in animals. Therefore, the present study reports, for the first time, a rare case of a completely divided female genital tract in a she-camel. In addition, the existing relevant literature on uterus didelphys in different animal species is reviewed. Case presentation: A female reproductive tract of she-camel, approximately 10 years old, with a history of previous successful pregnancy, was brought to the anatomy department following the slaughtering of the animal. Initial examination revealed a normal reproductive tract consisting of two ovaries, two fallopian tubes, a uterus, and a vagina. A closer examination revealed a completely divided vagina, with an external os opened into each part of the vagina, as well as a divided uterine body and cervix. Intrauterine infusion of saline through one external os confirmed complete separation of uterine body and cervix. Conclusion: To the authors' knowledge, this is the first reported case of a completely divided female genital tract in a she-camel. This review summarizes the previous reports about uterus didelphys in farm animals.

Comprehensive meta-analysis of surgical procedure for congenital diaphragmatic hernia: thoracoscopic versus open repair.

PURPOSE: Previous studies have shown a higher recurrence rate and longer operative times for thoracoscopic repair (TR) of congenital diaphragmatic hernia (CDH) compared to open repair (OR). An updated meta-analysis was conducted to re-evaluate the surgical outcomes of TR. METHODS: A comprehensive literature search comparing TR and OR in neonates was performed in accordance with the PRISMA statement (PROSPERO: CRD42020166588). RESULTS: Fourteen studies were selected for quantitative analysis, including a total of 709 patients (TR: 308 cases, OR: 401 cases). The recurrence rate was higher [Odds ratio: 4.03, 95% CI (2.21, 7.36), p < 0.001] and operative times (minutes) were longer [Mean Difference (MD): 43.96, 95% CI (24.70, 63.22), p < 0.001] for TR compared to OR. A significant reduction in the occurrence of postoperative bowel obstruction was observed in TR (5.0%) compared to OR (14.8%) [Odds ratio: 0.42, 95% CI (0.20, 0.89), p = 0.02]. CONCLUSIONS: TR remains associated with higher recurrence rates and longer operative times. However, the reduced risk of postoperative bowel obstruction suggests potential long-term benefits. This study emphasizes the importance of meticulous patient selection for TR to mitigate detrimental effects on patients with severe disease.

Congenital eyelid imbrication syndrome: A rare occurrence in Pakistan.

Congenital eyelid imbrication syndrome (CEIS) is a rare condition presenting at birth and is characterised by overriding of the upper lid on the lower lid. It is due to longer upper lid, than the lower lid. Overriding leads to spontaneous eversion of the upper lids. In our patient, examination revealed canthal tendon laxity and hyperaemia of the tarsal conjunctiva. All the rest of the structures in the eyeball and adnexa were normal. Spontaneous eversion occurred in two weeks as the upper lid grew with time. No treatment was required.

Development and evaluation of a candidate reference measurement procedure for detecting 17α-hydroxyprogesterone in dried blood spots using isotope dilution liquid chromatography tandem mass spectrometry.

17α-Hydroxyprogesterone (17α-OHP) quantification in dried blood spots (DBS) is essential for newborn screening for congenital adrenal hyperplasia (CAH), which is challenging due to its low physiological concentration. The high false-positive rates of immunoassays necessitate the development of more accurate methods. Liquid chromatography tandem mass spectrometry (LC-MS/MS) offers increased specificity and sensitivity, yet standardized procedures for 17α-OHP measurement are required for clinical application. A candidate reference measurement procedure (cRMP) using isotope dilution LC-MS/MS was developed for 17α-OHP quantification in DBS. By utilizing stable isotope-labeled D8-17α-OHP as an internal standard, the cRMP was optimized, covering sample preparation, calibration, and LC-MS/MS analysis. The method performance was validated across several parameters, including precision, accuracy, specificity, detection limits, and matrix effects. Clinical applicability was further assessed through the establishment of reference intervals for healthy newborns. The developed cRMP exhibited a linear range of 1.00 to 80.00 ng/mL for 17α-OHP, with detection and quantification limits of 0.14 ng/mL and 0.52 ng/mL, respectively. Inter- and intraday precision demonstrated coefficients of variation within 1.27 to 5.69%. The recovery rates and matrix effects were well within acceptable limits, ensuring method reliability. Clinical application showed distinct reference intervals for healthy newborns that were unaffected by sex but influenced by weight and gestational age. This method significantly enhances CAH diagnostic accuracy in newborns, providing a valuable tool for clinical laboratories and improving newborn screening program standardization and traceability.

Balloon dilation of congenital perforated duodenal web in newborns: Evaluation of short and long-term results.

BACKGROUND: Incomplete congenital duodenal obstruction (ICDO) is caused by a congenitally perforated duodenal web (CPDW). Currently, only six cases of balloon dilatation of the PDW in newborns have been described. AIM: To present our experience of balloon dilatation of a perforated duodenal membrane in newborns with ICDO. METHODS: Five newborns who underwent balloon dilatation of the CPDW along a preinstalled guidewire between 2021 and 2023 were included. Nineteen newborns diagnosed with ICDO who underwent laparotomy were included in the control group. RESULTS: In all cases, good anatomical and clinical results were obtained. In three cases, a follow-up study was conducted after 1 year. The average time to start enteral feeding per os was significantly earlier in the study group (4.4 d) than in the laparotomic group (21.2 days; P < 0.0001). The time spent by patients in the intensive care unit and hospital after balloon dilatation was also significantly shorter. We determined the selection criteria for possible and effective CPDW balloon dilatation in newborns as follows: (1) Presence of dynamic radiographic signs of the passage of a radiopaque substance beyond the zone of narrowing or radiographic signs of pneumatisation of the duodenum and small bowel distal to the web; (2) presence of endoscopic signs of CPDW; (3) successful cannulation with a guidewire performed parallel to the endoscope, with holes in the congenital duodenal web; and (4) successful positioning of the balloon performed along a freestanding guidewire on the web. CONCLUSION: Strictly following selection criteria for newborns with ICDO caused by CPDW ensures that endoscopic balloon dilatation using a pre-installed guidewire is safe and effective and shows good 1-year follow-up results.

Amniotic fluid-derived small extracellular vesicles for predicting postnatal severe outcome of congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a life-threatening condition with high morbidity and mortality rates. The survival rate of neonates with severe CDH is reportedly only 10%-15%. However, prenatal prediction of severe cases is difficult, and the discovery of new predictive markers is an urgent issue. In this study, we focused on microRNAs (miRNAs) in amniotic fluid-derived small EVs (AF-sEVs). We identified four miRNAs (hsa-miR-127-3p, hsa-miR-363-3p, hsa-miR-493-5p, and hsa-miR-615-3p) with AUC > 0.8 to classify good prognosis group and poor prognosis group in human study. The AUC for hsa-miR-127-3p and hsa-miR-615-3p, for predicting the poor prognosis, were 0.93 and 0.91, respectively. In addition, in the in vivo study, the miRNA profiles of the lung tissues of CDH rats were different from those of control rats. Additionally, two elevated miRNAs (rno-miR-215-5p and rno-miR-148a-3p) in the lung tissues of CDH rats were increased in the AF-sEVs of CDH rats. Our results suggest that severe CDH neonates can be predicted prenatally with high accuracy using miRNAs contained in AF-sEVs. Furthermore, miRNA profile changes in AF-sEVs reflected the lung status in CDH. Our findings may contribute to the development of advanced perinatal care for patients with CDH.

Slipped capital femoral epiphysis in an adolescent with congenital adrenal hyperplasia: A case report.

In previous reports, hypothyroidism, hypopituitrism, and hypogonadism were common endocrine causes of SCFE, but this is the first time that congenital adrenal hyperplasia has been observed. As such, patients who have undergone long-term endocrine treatment for congenital adrenal hyperplasia could potentially be subjected to a higher risk for SCFE.

A Rare Case of Left Main Coronary Artery Coronary Sinus Fistula in an 85-Year-Old Female.

Coronary arteriovenous fistulas (CAVFs) are congenital or acquired communications between the coronary arteries and coronary venous system, and they can also include other cardiac structures or vasculature. We discuss a case of a large fistula between the left main coronary artery and the right atrium in a geriatric patient with a history of gastrointestinal arteriovenous malformations (AVM). The occurrence of CAVFs, an uncommon cardiac irregularity, is particularly infrequent among older adults. Typically, it is discovered by chance when investigating symptoms such as shortness of breath or chest pain, where coronary angiography is necessary to determine the most effective treatment strategy. This case highlights the possible utility of evaluating CAVFs in patients with a history of gastrointestinal AVM who similarly present with clinical symptoms of high-output heart failure. Once identified, this could simplify the treatment approach and improve communication between healthcare providers to minimize the risk of harm to the patient.

Anesthetic Challenges in a Pregnancy Case With Congenital Kyphoscoliosis.

Kyphoscoliosis is a well-known spinal deformity. The abnormal curvature in both the coronal and sagittal planes presents unique challenges during pregnancy. This case discusses the management of a 27-year-old primigravida with thoracolumbar kyphoscoliosis, who underwent an emergency cesarean section at 39.3 weeks of gestation. An interdisciplinary team consisting of an obstetrician, pulmonologist, orthopedic surgeon, anesthesiologist, and physiotherapist collaborated in her care. In such cases, successful outcomes require a tailored approach that prioritizes maternal-fetal well-being and minimizes potential complications associated with complex spinal deformity during pregnancy and childbirth.

Surgical Management of Bilateral Single-System Ectopic Ureters (BSSEUs) in a Pediatric Patient: A Report of a Rare Case and Literature Review.

Bilateral single-system ectopic ureters (BSSEUs) are among the rarest entities encountered in pediatric urology. A BSSEU occurs when the ureteric buds originate cranially from the mesonephric ducts, causing a delay in their integration into the urogenital sinus. It presents as continuous incontinence in females, whereas symptoms like infection and discomfort are present in males. We describe a case involving a BSSEU opening into the vagina and urethra, with the patient experiencing continuous urinary incontinence, and its diagnosis and management. Here, We discuss a rare case of a four-year-old girl exhibiting continuous urinary incontinence or dribbling associated with recurrent urinary tract infections (UTIs) attributed to bilateral ectopic ureters. Imaging modalities, including contrast-enhanced computed tomography(CECT) and MRI, revealed the presence of BSSEUs accompanied by hydroureteronephrosis. The condition was managed with prompt surgical intervention involving bilateral ureteric reimplantation. Subsequent to the procedure, the patient experienced a significant improvement in continence mechanism and bladder capacity, obviating the requirement of urinary diversion procedure appendicovesicostomy or bladder neck reconstruction. Notably, while BSSEUs are an uncommon presentation, their timely and appropriate management is paramount in preventing potential renal damage. This case underscores the significance of vigilant monitoring and proactive intervention in addressing such complex urological anomalies in pediatric patients.

Serious Concern of Congenital Zika Syndrome (CZS) in India: A Narrative Review.

Congenital Zika syndrome (CZS) is a major concern in India and highlights the multifaceted challenges posed by the Zika virus (ZIKV). The alarming increase in CZS cases in India, a condition that has serious effects on both public health and newborns, has raised concerns. This review highlights the importance of raising concern and awareness and taking preventive measures by studying the epidemiology, clinical symptoms, and potential long-term consequences of CZS. The review also contributes to worldwide research and information sharing to improve the understanding and prevention of CZS. As India deals with the changing nature of CZS, this thorough review is an important tool for policymakers, health workers, and researchers to understand what is happening now, plan for what to do in the future, and work together as a team, using medical knowledge, community involvement, and study projects to protect newborns' health and reduce the public health impact of these syndromes.

Polyorchidism in a young Sprague-Dawley rat.

Duplicate testes lined in series were observed in the right scrotum of a 6-week-old Sprague-Dawley rat in a single-dose toxicity study. Of the two right testicles, one was spherical and less than half the size of a normal testis. The other was oval-shaped, slightly smaller than a normal testis, and possessed clear, tortuous blood vessels similar to those of a normal testis. Each right testis was grossly separated but faced the intertesticular adipose tissue and was sparsely joined by thin cord-like structures. Only one epididymis covered or encompassed the two right testes. The caput epididymis was attached to the smaller spherical testis, whereas the cauda epididymis was attached to the oval testis. Histopathological examination revealed that the smaller spherical testis on the right side and the testis on the left side were normal. The oval-shaped testis on the right exhibited markedly dilated degenerative seminiferous tubules with one to two layers of Sertoli or germ cells, and almost no spermatogenesis was observed. Multinucleated germ cells were observed in the lumen of the degenerated seminiferous tubules. The right epididymis was morphologically normal and contained few sperm in the epididymal duct of the tail. The cord-like structures between duplicate testes comprised fibrous and adipose tissues. Single efferent ductules, ectopic cartilage, and skeletal muscle tissues were buried in the adipose tissue. To our knowledge, this is the first report of spontaneous polyorchidism in a rodent.

Lissencephaly and Advanced-Stage Congenital Cytomegalovirus Infection in a Neonate.

This case report investigates the management of a 24-week-old neonate with congenital cytomegalovirus (CMV) infection and its sequelae, including severe intrauterine growth restriction, thrombocytopenia, and brain anomalies, ultimately progressing to lissencephaly. The diagnostic challenges included delayed clinical suspicion of congenital CMV, which was not identified until after delivery through CMV DNA polymerase chain reaction, and differentiating its symptoms from other potential causes of the neonate's condition. Aggressive interventions included antibiotics, antiviral therapy with ganciclovir, and supportive measures such as intubation, CPR, respiratory support, blood transfusions, and management of coagulopathy. Despite these efforts, the patient deteriorated due to progressive hypoperfusion, hypoxemic cardiorespiratory failure, and disseminated intravascular coagulopathy. Due to the poor prognosis and extent of multiorgan damage, support was withdrawn per parental consent. This case highlights the complications encountered when managing an advanced-stage neonatal CMV infection and emphasizes the importance of a multidisciplinary and holistic approach to guide diagnosis and treatment.

Coronary Cameral Fistula: A Rare Case Presenting With Non-ST-Segment Elevation Myocardial Infarction and Pulmonary Arterial Hypertension.

Coronary cameral fistulas (CCFs) are uncommon congenital or acquired anomalies characterized by abnormal connections between a coronary artery and a cardiac chamber. While often asymptomatic and incidentally detected, symptomatic presentations are rare, and symptoms may vary depending on the size and location of the fistula. We present the case of a 67-year-old female with complaints of intermittent typical cardiac chest pain and exertional dyspnea. Further evaluation revealed a CCF originating from the left anterior descending coronary artery and the left ventricle. Additionally, the patient was found to have pulmonary hypertension on right heart catheterization. This case highlights the importance of considering CCF in the differential diagnosis of chest pain, particularly in the presence of atypical symptoms and associated pulmonary hypertension (WHO Group 4). Further research is warranted to elucidate the optimal management strategies for symptomatic CCF, especially in cases complicated by pulmonary hypertension.

Congenital Myasthenic Syndromes in Belgium: Genetic and Clinical Characterization of Pediatric and Adult Patients.

BACKGROUND: Congenital myasthenic syndromes (CMS) are a group of genetic disorders characterized by impaired neuromuscular transmission. CMS typically present at a young age with fatigable muscle weakness, often with an abnormal response after repetitive nerve stimulation (RNS). Pharmacologic treatment can improve symptoms, depending on the underlying defect. Prevalence is likely underestimated. This study reports on patients with CMS followed in Belgium in 2022. METHODS: Data were gathered retrospectively from the medical charts. Only likely pathogenic and pathogenic variants were included in the analysis. RESULTS: We identified 37 patients, resulting in an estimated prevalence of 3.19 per 1,000,000. The patients harbored pathogenic variants in CHRNE, RAPSN, DOK7, PREPL, CHRNB1, CHRNG, COLQ, MUSK, CHRND, GFPT1, and GMPPB. CHRNE was the most commonly affected gene. Most patients showed disease onset at birth, during infancy, or during childhood. Symptom onset was at adult age in seven patients, caused by variants in CHRNE, DOK7, MUSK, CHRND, and GMPPB. Severity and distribution of weakness varied, as did the presence of respiratory involvement, feeding problems, and extraneuromuscular manifestations. RNS was performed in 23 patients of whom 18 demonstrated a pathologic decrement. Most treatment responses were predictable based on the genotype. CONCLUSIONS: This is the first pooled characterization of patients with CMS in Belgium. We broaden the phenotypical spectrum of pathogenic variants in CHRNE with adult-onset CMS. Systematically documenting larger cohorts of patients with CMS can aid in better clinical characterization and earlier recognition of this rare disease. We emphasize the importance of establishing a molecular genetic diagnosis to tailor treatment choices.

The importance of the 'uterine factor' in recurrent pregnancy loss: a retrospective cohort study on women screened through 3D transvaginal ultrasound.

STUDY QUESTION: What is the prevalence of congenital and acquired anomalies of the uterus in women with recurrent pregnancy loss (RPL) of unknown etiology examined using 3D transvaginal ultrasound (US)? SUMMARY ANSWER: Depending on the adopted diagnostic criteria, the prevalence of partial septate uterus varies between 7% and 14% and a T-shaped uterus is 3% or 4%, while adenomyosis is 23%, at least one of type 0, type 1 or type 2 myoma is 4%, and at least one endometrial polyp is 4%. WHAT IS KNOWN ALREADY: ESHRE and the Royal College of Obstetricians and Gynaecologists guidelines on RPL recommend the adoption of the 3D transvaginal US to evaluate the 'uterine factor'. Nevertheless, there are no published studies reporting the prevalence of both congenital and acquired uterine anomalies as assessed by 3D transvaginal US and diagnosed according to the criteria proposed by the most authoritative panels of experts in a cohort of women with RPL. STUDY DESIGN, SIZE, DURATION: This was a retrospective cohort study including 442 women with at least two previous first-trimester spontaneous pregnancy losses (i.e. non-viable intrauterine pregnancies), who referred to the obstetrics and gynecology unit of two university hospitals between July 2020 and July 2023. PARTICIPANTS/MATERIALS, SETTING, METHODS: Records of eligible women were reviewed. Women could be included in the study if: they were between 25 and 42 years old; they had no relevant comorbidities; they were not affected by infertility, and they had never undergone ART; they and their partner tested negative to a comprehensive RPL diagnostic work-up; and they had never undergone metroplasty, myomectomy, minimally invasive treatments for uterine fibroids or adenomyomectomy. Expert sonographers independently re-analyzed the stored 2- and 3D transvaginal US images of all included patients. Congenital uterine anomalies (CUAs) were reported according to the American Society for Reproductive Medicine (ASRM) 2021, the ESHRE/European Society for Gynaecological Endoscopy (ESGE) and the Congenital Uterine Malformation by Experts (CUME) criteria. Acquired uterine anomalies were reported according to the International Federation of Gynecology and Obstetrics (FIGO) and the Morphological Uterus Sonographic Assessment (MUSA) criteria. MAIN RESULTS AND THE ROLE OF CHANCE: The partial septate uterus was diagnosed in 60 (14%; 95% CI: 11-17%), 29 (7%; 95% CI: 5-9%), and 47 (11%; 95% CI: 8-14%) subjects, according to the ESHRE/ESGE, the ASRM 2021, and the CUME criteria, respectively. The T-shaped uterus was diagnosed in 19 women (4%; 95% CI: 3-7%) according to the ESHRE/ESGE criteria and in 13 women (3%; 95% CI: 2-5%) according to the CUME criteria. The borderline T-shaped uterus (diagnosed when two out of three CUME criteria for T-shaped uterus were met) was observed in 16 women (4%; 95% CI: 2-6%). At least one of FIGO type 0, type 1, or type 2 myoma was detected in 4% of included subjects (95% CI: 3-6%). Adenomyosis was detected in 100 women (23%; 95% CI: 19-27%) and was significantly more prevalent in women with primary RPL and in those with three or more pregnancy losses. At least one endometrial polyp was detected in 4% of enrolled women (95% CI: 3-7%). LIMITATIONS, REASONS FOR CAUTION: The absence of a control group prevented us from investigating the presence of an association between both congenital and acquired uterine anomalies and RPL. Second, the presence as well as the absence of both congenital and acquired uterine anomalies detected by 3D US was not confirmed by hysteroscopy. Finally, the results of the present study inevitably suffer from the intrinsic limitations of the adopted classification systems. WIDER IMPLICATIONS OF THE FINDINGS: The prevalence of CUAs in women with RPL varies depending on the classification system used. For reasons of clarity, the US reports should always state the name of the uterine anomaly as well as the adopted classification and diagnostic criteria. Adenomyosis seems to be associated with more severe forms of RPL. The prevalence rates estimated by our study as well as the replicability of the adopted diagnostic criteria provide a basis for the design and sample size calculation of prospective studies. STUDY FUNDING/COMPETING INTEREST(S): No specific funding was used. The authors have no conflicts of interest to declare. TRIAL REGISTRATION NUMBER: N/A.

Critical Event Checklists for Simulated In-Hospital Dysrhythmias in Children with Heart Disease.

Children with heart disease are at increased risk of unstable dysrhythmias and in-hospital cardiac arrest (IHCA). Clinician adherence to lifesaving processes of care is an important contributor to improving patient outcomes. This study evaluated whether critical event checklists improve adherence to lifesaving processes during simulated acute events secondary to unstable dysrhythmias. A randomized controlled trial was conducted in a cardiac ward in a tertiary care, academic children's hospital. Unannounced simulated emergencies involving dysrhythmias in pediatric patients with underlying cardiac disease were conducted weekly. Responders were pediatric and anesthesiology residents, respiratory therapists, and bedside registered nurses. Six teams were randomized into two groups-three received checklists (intervention) and three did not (control). Each team participated in four simulated scenarios over a 4-week pediatric cardiology rotation. Participants received a brief slideshow presentation, which included a checklist orientation, at the start of their rotation. Simulations were video and audio recorded and those with three or more participants were included for analysis. The primary outcome was team adherence to lifesaving processes, expressed as the percentage of completed critical management steps. Secondary outcomes included participant perceptions of the checklist usefulness in identifying and managing dysrhythmias. We used generalized estimating equations (GEE) models, which accounted for clustering within groups, to evaluate the effects of the intervention. A total of 24 simulations were conducted; one of the 24 simulations was excluded due to an insufficient number of participants. In our GEE analysis, 81.21% (78.96%, 83.47%) of critical steps were completed with checklists available versus 68.06% (59.38%, 76.74%) without checklists (p = 0.004). Ninety-three percent of study participants reported that they would use the checklists during an unstable dysrhythmia of a child with underlying cardiac disease. Checklists were associated with improved adherence to lifesaving processes during simulated resuscitations for unstable pediatric dysrhythmias. These findings support the use of scenario specific checklists for the management of unstable dysrhythmias in simulations involving pediatric patients with underlying cardiac disease. Future studies should investigate whether checklists are as effective in actual pediatric in-hospital emergencies.