Unexpected Findings of Regulatory Factor X6 Gene Mutation and Severe Hepatic Macrovesicular Steatosis in a Neonate with Congenital Left Ventricle Diverticulum: A Case Report.

Herein we present a case of a neonate with congenital left ventricular diverticulum (LVD), a rare anomaly, with an unusual course and unexpected findings. The neonate was born at 35 weeks in Namazi Hospital (Shiraz, Iran) and presented with a pulsatile umbilical mass immediately after birth. Based on multiple imaging modalities, the presence of a connection between the left ventricular apex and the umbilicus was confirmed. Percutaneous closure of LVD was unsuccessful. The patient's clinical course deteriorated after developing sepsis and multiorgan failure. The patient passed away before any corrective surgery could be performed. Unexpected findings in post-mortem evaluation were severe hepatic macrovesicular steatosis (suggestive of metabolic liver disease) and regulatory factor X6 (RFX6) heterozygous missense mutation in whole-exome sequencing.

Age-adjusted Reference Values and Z-scores of the Coronary Sinus Diameter among Healthy Children and Adolescents in Southern Iran.

BACKGROUND: Coronary sinus dimension is an important factor for diagnosing some types of cyanosis as well as congenital heart diseases and insertion of some devices into the coronary sinus if required. This study was designed to access the diameter and Z-score of the coronary sinus among children under 18 years of age. METHODS: In this cross-sectional study on 95 individuals, the coronary sinus diameter was measured by transthoracic echocardiography in the four-chamber view at the connection to the right atrium, middle part, and distal end. The linear regression equation was utilized to determine age-adjust reference values, Z-scores, and the relationship between the individuals' coronary sinus diameter age, height, and body surface area. The study was conducted from March to July 2020 in Namazi hospital clinic of Shiraz University of Medical Sciences, Shiraz, Iran. RESULTS: The mean age of the persons who entered this study was 5.87±4.25 years. The mean coronary sinus diameter was 4.91±1.29 mm at the site of connection to the right atrium, 4.50±1.44 mm at the middle part, and 3.74±1.32 mm at the distal end. Coronary sinus diameter correlates positively with the participants' age, weight, height, and body surface area (P<0.001). CONCLUSION: Coronary sinus diameter significantly correlates with the age, height, and body surface area of the cases. These features are useful in diagnosing some congenital heart diseases and insertion of suitable devices through it.

Introducing Copula as a Novel Statistical Method in Psychological Analysis.

During the past decades, the relationship between various psychological parameters had been studied in detail. However, the dependency structure of correlated parameters was rarely investigated. Knowing the dependence structure helps in finding the probability matrix of the interaction between the parameters. In this research, a novel approach was introduced in psychological analysis using copula functions. For this purpose, the self-esteem and anxiety of 141 university students in Iran were extracted using the Coopersmith Self-esteem Inventory and the Zang Anxiety Scale. Then the dependence structure of self-esteem and anxiety were established using copula functions. The Frank copula achieved the best fit for the joint variables of self-esteem and anxiety. Finally, the probability matrix of different classes of anxiety, taking into account self-esteem classes, was extracted. The results indicated that poor self-esteem leads to severe or very severe anxiety, with more than 98% probability, while strong self-esteem may lead to normal and mild anxiety, with about 80% probability. It can be concluded that the method was promising, and that copula functions can open a window to the dependence structure analysis of psychological parameters.

Seed's morphology-induced core-shell composite particles by seeded emulsion polymerization for drug delivery.

Cross-liked poly(2-hydroxyethyl methacrylate-co-methacrylic acid) seeds with different morphologies such as cauliflower-like, lobed spherical, and spherical were used in seeded emulsion polymerization (SEP) of 2-(dimethylamino)ethyl methacrylate (DMAEMA), 2-hydroxyethyl methacrylate (HEMA), and methacrylic acid (MAA). The morphological structure of produced composite particles was observed using field emission scanning electron microscopy (FE-SEM). The origin of the formation of different morphologies was discussed using various thermodynamic parameters such as solubility parameters and intermolecular forces between polymeric components involved. Also, the effect of the morphology of seed particles on the resultant structures was investigated. Results showed that morphology of fabricated composite particles is induced from morphology of seed particles with larger sizes. Finally, the fabricated composite particles were utilized in the controlled release of DOX. The effect of morphological changes of synthesized composite particles on the cumulative release behavior at acidic environment indicated the pH-sensitive nature of drug release through carriers. The particles with PDMAEMA shell showed the highest release of DOX at pH = 7.4 whereas PMAA shells displayed the least cumulative release. Inversely, the lowest cumulative release at pH = 1.2 was shown by PDMAEMA-coated carriers. Moreover, particles with spherical morphology had better drug release than cauliflower-like ones originated from smart nature of carriers.

Management of Persistent Pulmonary Hypertension After Correction of Congenital Heart Defect with Autologous Marrow-Derived Mononuclear Stem Cell Injection into the Pulmonary Artery: A Pilot Study.

Pulmonary arterial hypertension (PAH) related to left-to-right shunt can progress to Eisenmenger syndrome, a serious and fatal disease that is not yet curable. This pilot study considered stem cell injection as a new treatment modality in persistent pulmonary hypertension after the correction of a congenital heart defect. Three patients with persistent pulmonary hypertension after ventricular septal defect repair were included in this pilot study for a clinical trial. Mononuclear stem cells derived from patients' bone marrow specimens were injected into the right and left pulmonary arteries via cardiac catheterization. The patients were followed over a 6-month period, with six-minute walk test, echocardiography and repeated angiography performed in the sixth month after treatment. The results of the study showed improvement of 40 m, 280 m and 100 m in 6-minute walk distance in patients 1 to 3, respectively. The peak PR gradient decreased 2, 5 and 9 mmHg by echocardiography, and mean PA pressure decreased 21, 22 and 9 mmHg by catheterization in patients 1 to 3, respectively. Pulmonary artery resistance decreased 4, 4.5 and 1.3 Wood units after 6 months of stem cell therapy in the three patients. No short-term complications were detected in this pilot trial, and all patients tolerated the procedure without any complications. Intrapulmonary artery injection of stem cells may have a role in the treatment of persistent PAH secondary to congenital heart disease. This procedure is feasible, with no significant complications, and this study can be considered as a platform for larger studies.

Japanese Kawasaki Disease Scoring Systems: Are they Applicable to the Iranian Population?

BACKGROUND: Kawasaki disease (KD) is the most frequent cause of coronary artery aneurysm (CAA) in children. This study tried to evaluate the accuracy of different KD scores developed for prediction of CAA, in an Iranian population. METHODS: This is a cross-sectional retrospective investigation on pediatric patients with a diagnosis of KD. Clinical manifestations, laboratory, and echocardiographic data were recorded. Five Kawasaki scores, including Kobayashi, Egami, Sano, Nakano, and Harada, were assessed and analyzed in relation to CAA and intravenous immunoglobulin (IVIG) resistance. RESULTS: During five years, we recruited 121 cases of KD under 13 years of age. The rates of CAA and IVIG resistance were 16.5%, and 13.2% respectively. The IVIG resistance group was significantly younger than responder patients. All five scores had low sensitivity in predicting CAA or IVIG resistant cases; the highest sensitivity pertained to the Harada score with 50% sensitivity and 59% specificity (the area under the curve: 0.545, with a 95% confidence interval: 0.423 to 0.667) in predicting CAA, which is lower than the usual acceptable criteria for a screening test. The specificity of all other scores were more than 85% in predicting CAA or IVIG resistance. Gender, fever before therapy and laboratory data showed no significant difference between the groups. CONCLUSION: The Kobayashi, Egami, Sano, Nakano and Harada scores have limited usefulness in the Iranian population to predict high risk patients for coronary artery involvement or IVIG resistance; in our study, age under one year was a risk factor for IVIG resistance.

Fabricating cauliflower-like and dumbbell-like Janus particles: Loading and simultaneous release of DOX and ibuprofen.

Most recent studies on Janus particles have concentrated on amphiphilic Janus particles. Herein, a facile and efficient method based on polymerization-induced phase separation is presented to fabricate Janus particles with different morphologies. Theses novel hydrophilic Janus particles with two distinct hydrophilic domains have been prepared by combination of distillation precipitation polymerization (DPP) and seeded emulsion polymerization. Crosslinked poly(2-hydroxyehtyl methacrylate) (PHEMA) cores were synthesized via DPP and used as seed particles. Also, methacrylic acid (MAA) was used as second hydrophilic monomer. Effect of feeding approach, and reaction medium were investigated on the morphology of fabricated composite particles. Different morphologies such as cauliflower-like, dumbbell-like, and core-shell toward cauliflower-like structures were obtained by varying feeding approach and polymerization medium. The morphology of resultant particles was observed by means of FE-SEM and TEM images and their particle sizes were confirmed by DLS. Fabricated composite particles were used to simultaneously carry both DOX and ibuprofen. Drug release studies showed that Janus particles can be used in loading and release of two drugs simultaneously. However, release behaviors of DOX and ibuprofen were different at different pH values. Also, release behavior was significantly affected by the structure of composite particles.

Investigation of different core-shell toward Janus morphologies by variation of surfactant and feeding composition: A study on the kinetics of DOX release.

Composite particles with two individual hydrophilic parts were synthesized via seeded emulsion polymerization. As first part, nearly-monodisperse ethylene glycol dimethacrylate (EGDMA)-crosslinked poly(acrylic acid) (PAA) particles were synthesized by distillation precipitation polymerization (DPP). These particles were used as seeds in emulsion polymerization of 2-(dimethylamino)ethyl methacrylate (DMAEMA). Effects of type of surfactant, monomers/seed weight ratio and amount of shell crosslinker on the synthesized composite particles' morphology were studied. Different morphologies consisting of core-shell, Janus type, raspberry-like and porous core-shell structures were investigated by variations of polymerization parameters. Different structures were chosen as drug carriers and subjected to DOX loading and release system. Results showed that amount of drug loading and extent of release were strongly dependent on the structure of carriers whereas for all carriers, DOX was released more rapid. Kinetics of release was evaluated by different mathematical models to investigate the release mechanism through composite particles. Results showed that only Korsmeyer-Peppas model fitted the drug release data and other ones were inappropriate in this field.

Simultaneous two drugs release form Janus particles prepared via polymerization-induced phase separation approach.

Seeded emulsion polymerization of 2-dimethylaminoethylamino methacrylate (DMAEMA) was carried out using monodispersed poly(2-hydroxyehtyl methacrylate) (PHEMA) seeds to produce Janus particles. Three feeding approaches were used comprising one together, rest and continuous feeding methods to investigate different morphologies. However, FE-SEM results showed that all feeding approaches yielded dumbbell-like Janus particles. Furthermore, snowman-like Janus particles were obtained via seeded distillation precipitation polymerization (DPP). It is shown that minimizing the total interfacial free energy alongside difference in solubility parameters of Janus domains are responsible for obtained morphologies. Two different morphologies (dumbbell-like and snowman-like) were chosen as carriers of ibuprofen and DOX simultaneously. Also, simultaneous release of two drugs were investigated in different conditions. Dumbbell-like Janus particles showed higher ibuprofen loading whereas DOX was more loaded onto snowman-like Janus particles. Also, DOX was released more rapidly through Janus particles at different pH values and both types of Janus particles showed similar drugs release behaviors.