Prevalence, risk factors, and outcomes of acute kidney injury in a pediatric cardiac intensive care unit: A cross-sectional study.

Background and Aims: Acute kidney injury (AKI) is a common complication in pediatric cardiac intensive care unit (CICU). This study aims to identify the prevalence, risk factors, and outcomes of AKI in pediatrics admitted to a CICU unit of a tertiary hospital. Methods: We retrospectively gathered the data of 253 randomly selected patients admitted to the CICU unit from March 2018 to March 2022. Data were collected from EHRs. We used the Kidney Disease Improving Global Outcomes (KDIGO) criteria for identifying AKI in patients. Results: Overall, AKI prevalence was 22.9% in our population. In the multivariable analysis, vancomycin intake (odds ratio [OR]: 2.109, 95% confidence interval [CI]: 1.15-3.84), angiography (OR: 4.38, 95% CI: 1.28-14.93), and mechanical ventilation (OR: 2.08, 95% CI: 1.02-4.23) were independent risk factors of AKI development and patients with AKI had a higher in-hospital mortality rate (OR: 5.81, 95% CI: 2.55-13.19), higher need for cardiopulmonary resuscitation (OR: 3.08, 95% CI: 1.17-8.09), and longer ICU length of stay (OR: 6.49, 95% CI: 3.31-9.67). Furthermore, furosemide administration was associated with lower risk of developing AKI (OR: 0.52, 95% CI: 0.27-0.97). Conclusion: AKI is common and is associated with worse outcomes in patients with congenital heart disease. Our results emphasize the importance of early identification and monitoring of AKI in the pediatric CICU setting.

Multisystem inflammatory syndrome in children and Kawasaki disease; comparison of their clinical findings and one-year follow-up-a cross-sectional study.

BACKGROUND: Studies on Multisystem Inflammatory Syndrome in Children (MIS-C) and Kawasaki Disease (KD) have yielded inconsistent results and are lacking in Asian and African countries. This study aimed to compare the laboratory and clinical features, short-term outcomes, and one-year follow-ups of a large cohort of MIS-C and KD patients. METHODS: Data from 176 MIS-C and 56 KD patients admitted to Tehran Children's Medical Center between January 2021 and January 2022 were collected. Patients were followed up until January 2023. RESULTS: While lymphopenia and thrombocytopenia were more prevalent in MIS-C (73.2% vs. 20% in KD, p < 0.001), KD patients exhibited a higher median white blood cell count and prevalence of anemia, along with higher fibrinogen and erythrocyte sedimentation rate levels (p < 0.001, p < 0.001, p = 0.005, p < 0.001, respectively). MIS-C patients also exhibited lower ejection fraction, a greater occurrence of pericardial effusion, and a higher incidence of coronary aneurysms and ectasia, and ascites. Echocardiography after seven days of treatment showed a reduction in pathologies for both groups, but it was significant only for MIS-C. After one year, coronary artery abnormalities remained in only six cases. CONCLUSIONS: In conclusion, this study highlights differences between MIS-C and KD, including laboratory indices as well as echocardiographic and abdominal ultrasound findings. These findings contribute valuable data on Iranian patients to the existing literature on this topic and have significant implications for accurate diagnosis and improved management of pediatric patients presenting with these conditions.

The role of nailfold capillaroscopy in pediatric patients with Kawasaki disease / El papel de la capilaroscopia periungueal pacientes pediátricos con la enfermedad de Kawasaki

Background: Nailfold capillaroscopy has been used as a non-invasive diagnostic method for microvasculature evaluation in various rheumatological disorders. The present study aimed to determine the utility of nailfold capillaroscopy in the diagnosis of Kawasaki Disease (KD). Method: In this case–control study nailfold capillaroscopy was performed in 31 patients with KD and 30 healthy controls. All nailfold images were evaluated for capillary distribution and capillary morphology such as enlargement, tortuosity, and dilatation of the capillaries. Result: Abnormal capillaroscopic diameter was identified in 21 patients from the KD group and 4 patients in the control group. The most common abnormality in capillary diameter was irregular dilatation in 11 (35.4%) KD patients and in 4 people (13.3%) in the control group. Distortions of the normal capillary architecture was commonly seen in the KD group (n=8). A positive correlation was observed between coronary involvement and abnormal capillaroscopic results (r=.65, P<.03). The sensitivity and specificity of capillaroscopy for the diagnosis of KD were 84.0% (95%CI: 63.9–95.5%) and 72.2% (95%CI: 54.8–85.8%), respectively. The PPV and NPV of capillaroscopy for KD were 67.7% (95%CI: 48.6–83.3) and 86.7% (95% CI: 69.3–96.2), respectively. Conclusion: Capillary alterations are more common in KD patients compared to control group. Thus, nailfold capillaroscopy can be useful in detecting these alterations. Capillaroscopy is a sensitive test for detecting capillary alternations in KD patients. It could be used as a feasible diagnostic modality for evaluating microvascular damage in KD.(AU)

Antecedentes: La capilaroscopia periungueal se ha utilizado como un método diagnóstico no-invasivo para evaluación de la microvasculatura en varios trastornos reumatológicos. El presente estudio pretendió determinar la utilidad de la capilaroscopia periungueal en el diagnóstico de la enfermedad de Kawasaki (KD). Método: En este estudio de casos y controles se realizó la capilaroscopia periungueal para 31 pacientes con KD y 30 controles sanos. Todas las imágenes de la ungueal se evaluaron para la distribución capilar y la morfología capilar, como la ampliación, la tortuosidad y la dilatación de los capilares. Resultado: Se identificó un diámetro capilaroscópico anormal en 21 pacientes del grupo KD y en 4 pacientes del grupo control. La anomalía más común en el diámetro de los capilares fue la dilatación irregular en 11 (35,4%) pacientes con KD y 4 personas (13,3%) en el grupo control. Las distorsiones de la arquitectura capilar normal se observaron comúnmente en el grupo KD (n=8). Se observó una correlación positiva entre la afectación coronaria y los resultados capilares anormales (r=0,65, p <0,03). La sensibilidad y la especificidad de una capilaroscopia para el diagnóstico de KD fueron del 84,0% (IC del 95%: 63,9-95,5%) y del 72,2% (IC del 95%: 54,8-85,8%), respectivamente. El VPP y el VAN de la capilaroscopia para KD fueron del 67,7% (IC del 95%: 48,6-83,3) y del 86,7% (IC del 95%: 69,3-96,2), respectivamente. Conclusión: Las alteraciones capilares son más frecuentes en los pacientes con KD en comparación con el grupo control. Así, la capilaroscopia periungueal puede ser útil en la detección de estas alteraciones. La capilaroscopia es una prueba sensible para detectar las alteraciones capilares en pacientes con KD. Podría utilizarse como modalidad diagnóstica factible para evaluación el daño microvascular en la KD.(AU)

The role of nailfold capillaroscopy in pediatric patients with Kawasaki disease.

BACKGROUND: Nailfold capillaroscopy has been used as a non-invasive diagnostic method for microvasculature evaluation in various rheumatological disorders. The present study aimed to determine the utility of nailfold capillaroscopy in the diagnosis of Kawasaki Disease (KD). METHOD: In this case-control study nailfold capillaroscopy was performed in 31 patients with KD and 30 healthy controls. All nailfold images were evaluated for capillary distribution and capillary morphology such as enlargement, tortuosity, and dilatation of the capillaries. RESULT: Abnormal capillaroscopic diameter was identified in 21 patients from the KD group and 4 patients in the control group. The most common abnormality in capillary diameter was irregular dilatation in 11 (35.4%) KD patients and in 4 people (13.3%) in the control group. Distortions of the normal capillary architecture was commonly seen in the KD group (n=8). A positive correlation was observed between coronary involvement and abnormal capillaroscopic results (r=.65, P<.03). The sensitivity and specificity of capillaroscopy for the diagnosis of KD were 84.0% (95%CI: 63.9-95.5%) and 72.2% (95%CI: 54.8-85.8%), respectively. The PPV and NPV of capillaroscopy for KD were 67.7% (95%CI: 48.6-83.3) and 86.7% (95% CI: 69.3-96.2), respectively. CONCLUSION: Capillary alterations are more common in KD patients compared to control group. Thus, nailfold capillaroscopy can be useful in detecting these alterations. Capillaroscopy is a sensitive test for detecting capillary alternations in KD patients. It could be used as a feasible diagnostic modality for evaluating microvascular damage in KD.

A novel mutation in the ALS2 gene in an iranian kurdish family with juvenile amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a rare disorder that affects both upper and lower motor neurons. Mutations in Alsin Rho Guanine Nucleotide Exchange Factor (ALS2) correlates with three similar but distinctive syndromes, including the juvenile form of ALS. An Iranian Kurdish family was involved in this study and all members were evaluated with relevant clinical guidelines. Whole exome sequencing and sanger sequencing were applied to all family members to undermine the possible genetic factors. A substitution c. 2110 C>T (p. Arg704X) identified in the ALS2 gene. Bioinformatics analysis indicated the mutation is located in the well-conserved and functional domain of the protein. This study recognized a novel mutation in the ALS2 gene in a proband with the juvenile form of ALS. To our knowledge, this is the first identified ALS2 mutation among the Iranian population.

Intravenous Methylprednisolone Pulse Therapy Versus Intravenous Immunoglobulin in the Prevention of Coronary Artery Disease in Children with Kawasaki Disease: A Randomized Controlled Trial.

BACKGROUND: Kawasaki disease (KD) is often complicated by coronary artery lesion (CAL), including dilatation or aneurysms. Intravenous immunoglobulin (IVIG) is used with aspirin to prevent CAL in KD. OBJECTIVE: Given that the primary treatment for other vasculitis is the use of corticosteroids, this study has been performed to evaluate the effect of intravenous methylprednisolone pulse (IVMP) therapy in preventing CAL in KD. METHOD: A randomized, single-blind clinical trial was conducted on 40 KD patients aged six months to five years. Patients were randomized into two groups according to the main treatment plan in addition to aspirin: case group (IVMP for three consecutive days and then oral prednisolone for three days) and control group (intravenous immunoglobulin 2 g/kg). Echocardiography was performed for all children at least three times, during the acute phase, two weeks, and two months later. RESULTS: Data analysis at the end of the study was done on 40 patients (20 patients in each group). There were no significant differences in age and sex distribution, mean fever, and acute phase duration, as well as baseline echocardiography in the two groups. The frequency of CAL was 20% in the case group and 45% in the control group, after two weeks (p<0.05), but there was no significant difference between two groups in types of coronary artery lesion after two weeks and the frequency and severity of CAL after two months. CONCLUSION: IVMP as initial line therapy effectively control systemic and vascular inflammation and decrease coronary artery damage in KD.

Whole Exome Sequencing Identified the Causative Mutation in a 4-Year-Old Female with Mulibrey Nanism: A Case Report.

Mulibrey Nanism is a rare multisystem disorder inherited in an autosomal recessive manner caused by mutations in the TRIM37 gene. Most of the reported cases are from Finland, but this condition has rarely occurred in other countries. Although the clinical diagnosis of Mulibrey nanism is a challenge during the first months of life, the disease can be suspected clinically due to the distinctive features of the patients. A 4-year-old female with pneumonia, cardiomyopathy, growth retardation, peripheral edema, and characteristic craniofacial features was referred to Tehran Hope Generation Foundation Genetic diagnosis Center, in October 2021. Genomic DNA was isolated from peripheral blood samples of the patient and her parents and Whole exome sequencing was performed for the patient. Whole exome sequencing revealed a homozygous G>A splice site variant (TRIM37; c.370-1G>A). Sanger sequencing confirmed the segregation of the variant with phenotype in this family. Whole exome sequencing can be helpful in the diagnosis of the patients suspecting to Mulibrey nanism and lacking sufficient clinical presentation according to the diagnostic algorithm.

Hematological indices in pediatric patients with acyanotic congenital heart disease: a cross-sectional study of 248 patients.

Background: Congenital heart disease CHD is a significant cause of mortality and morbidity in children worldwide. Patients with congenital heart disease may develop hematological problems, including thrombocytopenia and neutropenia. In addition, several studies indicate the higher frailty of patients with CHDs to infections and malignancies. Nevertheless, the mechanisms of immune system changes in these patients have remained in the shadow of uncertainty. Moreover, very few studies have worked on cytopenia in CHD. This study has assessed the frequency of thrombocytopenia, neutropenia, lymphopenia, and anemia in pediatric patients with acyanotic congenital heart disease ACHD prior to open-heart surgery. Methods: This cross-sectional study was handled in the Pediatric Cardiology Clinic, Tehran University of Medical Sciences, during pre-operation visits from 2014 till 2019. Two hundred forty-eight children and adolescents with acyanotic congenital heart disease before open-heart surgery met the criteria to enter the study. Results: A total of 191 (76.7%) patients with Ventricular Septal Defects (VSD), 37 (14.85%) patients with Atrial Septal Defects (ASD), and 20 (8.11%) patients with Patent Ductus Arteriosus (PDA) were enrolled in this study. The median age was 23.87 months. Thrombocytopenia and neutropenia were found, respectively, in 3 (1.2) and 23 (9.2%) patients. Hemoglobin level and lymphocyte count were significantly lower in patients with neutropenia than patients with normal neutrophil count (P value = 0.024 and P value = 0.000). Significant positive correlations were found between neutropenia and anemia. There were no correlations between neutrophil count and Platelets. Also, anemia was found in 48 patients (19.3%). The study also found a statistically significant correlation between the co-existence of VSD and neutropenia in the patients (P value = 0.000). Conclusion: Although most were mildly neutropenic, there was a significant correlation between neutropenia and Ventricular Septal Defect compared to PDA and ASD groups. Regarding the importance of neutropenia to affect the prognosis of congenital heart defects in infections, it is important to consider further studies on the status of immune system function in these patients.

COVID-19: A New Horizon in Congenital Heart Diseases.

Objective: Previous studies have demonstrated that both children and adult patients with a history of congenital heart disease (CHD) are at high risk for coronavirus disease 2019 (COVID-19) infection. This study investigates the status of COVID-19 infection among children undergoing surgical repair within the past 2 years. Methods: All alive patients operated on in a tertiary referral center between March 2018 and March 2020 were recruited in the present study. Detailed demographics, past medical and surgical history, and physical examination were reviewed for each patient. During the COVID-19 pandemic, data regarding the patient's status were collected by telephone survey from April 15 to April 30, 2020. Results: A total number of 210 patients are analyzed in this study. Participants' median age was 21.59 months [interquartile range (IQR) = 12-54.67], and 125 (59.5%) were female. The median interval between surgery and COVID-19 assessment was 305 days (IQR = 215-400). In addition, 67 (32%) patients used angiotensin receptor blocker (ARB)/angiotensin-converting enzyme (ACE) inhibitor (spironolactone and/or captopril). Sixteen patients (7.6%) were symptomatic and had positive chest CT results and/or RT-PCR compared to the previously reported prevalence of COVID-19 among the pediatric population (2.4% of children with <18 years of age); the prevalence of COVID-19 among the patients operated on due to CHD in the present study was significantly higher (p = 0.00012). Two patients were admitted to the intensive care unit (ICU); one patient was discharged 2 weeks later with acceptable status, and one patient died 2 days after ICU admission due to cardiac and respiratory arrest and myocarditis. The complexity of the underlying cardiac disorders was not different between patients with low risk (p = 0.522), suspicious patients (p = 0.920), and patients positive for COVID-19 (p = 0.234). The ARB/ACE inhibitor consumption was not associated with the COVID-19 infection [p = 0.527, crude odds ratio (OR) = 1.407, 95% CI = 0.489-4.052]. Conclusion: Children with a history of previous CHD surgery are more susceptible to infections, especially those infections with pulmonary involvements, as the lung involvement could cause worsening of the patient's condition by aggravating pulmonary hypertension. The results of the current study indicate that these patients are more prone to COVID-19 infection compared to the healthy children population.