Brown-Vialetto-Van Laere syndrome.

Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurodegenerative disorder of childhood. According to the previous reports, it has various primary signs and symptoms. Because of the simple treatment with riboflavin supplementation, it is important to have suspicious to this disease and begin treatment even before genetic test confirm. We report a five-year-old girl with BVVLS that manifest with hearing problems, first. There was obvious improvement in her disease clinical signs with riboflavin supplementation treatment.

Internal validation and evaluation of the predictive performance of models based on the PRISM-3 (Pediatric Risk of Mortality) and PIM-3 (Pediatric Index of Mortality) scoring systems for predicting mortality in Pediatric Intensive Care Units (PICUs).

PURPOSE: The study was aimed to assess the prognostic power The Pediatric Risk of Mortality-3 (PRISM-3) and the Pediatric Index of Mortality-3 (PIM-3) to predict in-hospital mortality in a sample of patients admitted to the PICUs. DESIGN AND METHODS: The study was performed to include all children younger than 18 years of age admitted to receive critical care in two hospitals, Mashhad, northeast of Iran from December 2017 to November 2018. The predictive performance was quantified in terms of the overall performance by measuring the Brier Score (BS) and standardized mortality ratio (SMR), discrimination by assessing the AUC, and calibration by applying the Hosmer-Lemeshow test. RESULTS: A total of 2446 patients with the median age of 4.2 months (56% male) were included in the study. The PICU and in-hospital mortality were 12.4 and 16.14%, respectively. The BS of the PRISM-3 and PIM-3 was 0.088 and 0.093 for PICU mortality and 0.108 and 0.113 for in-hospital mortality. For the entire sample, the SMR of the PRISM-3 and PIM-3 were 1.34 and 1.37 for PICU mortality and 1.73 and 1.78 for in-hospital mortality, respectively. The PRISM-3 demonstrated significantly higher discrimination power in comparison with the PIM-3 (AUC = 0.829 vs 0.745) for in-hospital mortality. (AUC = 0.779 vs 0.739) for in-hospital mortality. The HL test revealed poor calibration for both models in both outcomes. CONCLUSIONS: The performance measures of PRISM-3 were better than PIM-3 in both PICU and in-hospital mortality. However, further recalibration and modification studies are required to improve the predictive power to a clinically acceptable level before daily clinical use. PRACTICE IMPLICATIONS: The calibration of the PRISM-3 model is more satisfactory than PIM-3, however both models have fair discrimination power.

The effect of head positioning on brain tissue oxygenation in preterm infants: a randomized clinical trial study.

BACKGROUND: CNS injury in preterm infants is still one of the leading causes of mortality and morbidity. Routine care events might affect the perfusion and cerebral oxygenation of preterm infants. Although positioning the infant's head in a particular condition to improve brain oxygenation is included in many institutions, there is no robust clinical evidence to support this intervention's effectiveness. OBJECTIVE: The present study aimed to determine the effect of head positioning on brain tissue oxygenation in preterm infants. METHODS: This study is a randomized clinical trial. In the first 48 h after birth, 39 infants who met the study inclusion criteria underwent head positioning intervention. In this case, the infants were placed in the supine position, and every 2 h, the head position was changed continuously to one of six randomized modes [using random modes generated by SPSS]. During each head positioning, brain tissue oxygenation was recorded by NIRS. RESULTS: The findings showed a significant difference in brain tissue oxygen saturation among these positions (P < 0.001). Dunn's test showed that the brain tissue oxygen saturation in the third position (head rotates 45-60 degrees from the midline to the right and the head of the bed is zero degrees) was significantly lower than the baseline (P = 0.029; Mean difference = 2.3). Also, in the third position, compared to the first position (P = 0.002; Mean difference = 1.9) and compared to the fourth position (P = 0.003; Mean difference = -2.1), and in the second position compared to the first position (P = 0.046; Mean difference = 1.3), the brain tissue oxygen saturation of the infants was lower. CONCLUSION: Based on the results of the present study, head positioning was effective on brain tissue oxygenation in preterm infants in the first 48 h after birth; Therefore, it is recommended when possible, not to rotate the infant's head during the first 48 h after birth while the head of the bed is at 0°.

The effects of implementation of a stepwise algorithmic protocol for nutrition care process in gastro-intestinal surgical children in Pediatric Intensive Care Unit (PICU).

INTRODUCTION: Malnutrition is known as one of the major health problems among critically ill children; optimum nutrition support is considered as a therapeutic strategy to improve clinical outcomes and minimize the length of Pediatric Intensive Care Unit (PICU) staying as well as its costs. Implementation of an algorithmic protocol can result in the upgrade of the quality of nutrition support system in PICU. METHOD: In this study, we developed a stepwise algorithmic nutrition care protocol for PICU patients in two phases as follows: a critical review of past literature and an expert discussion panel. The final structured protocol includes three following steps for the nutrition care process: 1) Initial nutrition screening and assessment, 2) Nutritional intervention and 3) Nutritional monitoring. Pre and post-implementation audits were carried out in a 23 bed medical/surgical PICU in a children's hospital affiliated to Mashhad University of Medical Sciences over two 4-week periods to evaluate the impact of the algorithm implementation. The post-implementation audit was performed by passing 12 weeks from the protocol implementation. Critically ill children aged between 1month and 10 years, and PICU length of stay>24 h who were in post gastrointestinal surgery state, were enrolled. RESULTS: Totally, 34 eligible critically ill gastrointestinal surgical children in post-implementation audit were compared with 30 patients with similar eligibility criteria in the pre-implementation audit. Notably, there were no significant differences in gender, median age, length of PICU stay, and mechanical ventilation requirement in the two audits. The comparison of our pre and post-implementation audits showed a significant increase in the proportion of energy delivery goal achievement following performing our intervention (56.7%, and 85.3%, for pre and post-implementation audits, respectively; p-value = 0.01).Additionally, time of achieving energy and protein goals were significantly decreased (5.5 vs. 3 days; p-value = 0.008 and 4 vs 3 days; p-value = 0.002, for energy and protein delivery goal achievements, respectively). CONCLUSION: The implementation of the algorithm have significantly improved the adequacy ratio of energy delivery and also decreased the time to achieve the goal in energy and protein intake among critically ill children in post-gastrointestinal state. In this regard, further prospective studies are needed for continuing the evaluation of the algorithm implementation outcomes in critically ill children with different surgical and internal underlying diseases.

COVID-19 in a child with primary antibody deficiency.

Although presentation of COVID-19 in patients with immunodeficiency could be mild, it should not be missed, while early diagnosis and appropriate treatment can survive infected patients. Because even severe infections in PID patients may be presented with few symptoms and signs, this diagnosis should be considered in those immunocompromised patients who have exacerbating preexisting symptoms.

Role of Procalcitonin in the Prognosis of Mortality in Patients Admitted to the Intensive Care Unit: A Review Study.

Background: This review study aimed to investigate the role of PCT in the prognosis of mortality among patients admitted to the intensive care units (ICU). Procalcitonin (PCT) is a polypeptide and prohormone of calcitonin. This prohormone is secreted by thyroid gland C cells in response to hypercalcemia, and Its elevated level indicates infection, especially bacterial infections, in which there is a systematic response to infection. Materials and Methods: This narrative review study was performed based on Cochrane collaboration recommendations for reviews. We reviewed all the titles and abstracts of published research articles with the following inclusion criteria studies aimed to confirm the function of a prognostic model in predicting mortality or survival, (b) mortality or survival of a specific endpoint (for example, 30 days), (c) patients admitted to intensive care units, and d) the articles written in English. The exclusion criteria of the current review included: (a) articles whose data were not specifically focused on prognosis of patients in ICU, (b) articles that did not provide sufficient information on the cause of death of patients in ICU, and (c) articles focusing on the treatment of comorbid patients with infections in ICU. The search was conducted on Google Scholar, PubMed, Magiran, ScienceDirect, and SID. Also, to search Iranian databases, including SID and Magiran, the same terms and expressions were searched. Results: Based on the findings of this review, serum levels of PCT were reported within the range of at least 5 to more than 16 ng/ml in patients admitted to ICU. The mortality rate was estimated at 5.7% to 79% in these patients. Moreover, the incidence of sepsis was reported from 13% to 77.6%. Conclusion: Serum levels of PCT as a prognostic factor may help early detection, and better classification of the poor prognoses sepsis patients and more invasive treatment of patients admitted to ICU and are at risk for mortality.

Abuse and Neglect of Healthy Newborn by Parents: A Social Problem with a Long History.

A Two-day-old girl who was found in garbage by police and transferred to hospital. She was irritable and dehydrated; also there were burn injuries around her knees and right cheek. Her weight was 3100 grams and physical examinations were normal. Opium was found in urine. Phenobarbital (4 mg/kg/day intravenous every 12 hours) was started for her irritability. After 20 days, infant was entrusted to a welfare organization with coordination of social support of hospital. The prevention of child abuse and neglect is an urgent public health concern. Home visit by welfare organization has been proposed as a promising approach to prevent health and developmental problems among children. We report this case of an abused and neglected newborn.

Clinical outcome and bowel function after surgical treatment in Hirschsprung's disease.

BACKGROUND: Bowel function has been reported to be adversely affected following surgery in cases of Hirschsprung. We retrospectively studied both the clinical outcome and bowel function status following surgery in patients diagnosed with Hirschprung's disease (HD). 161 cases, who underwent pull-through operations for HD in Sheikh Pediatric Tertiary Centre, Mashhad, Iran. The specified time bracket spanned between 2006 and 2011. MATERIALS AND METHODS: Data was extracted from Health Information System with the aim of investigating patients for both short and long-term gastrointestinal (GI) complications after surgery bases in addition to the concurrence of any associated anomalies. Three main procedures were analysed in this respect (Swenson, Duhamel and Soave). RESULTS: In a study of 96 (59%) boys and 65 (40.3%) girls, mortality rate was reported to be 15.5% (15 males and 10 females). A considerable majority of almost three fourths were detected with both early and late GI complications after surgery. The latter mainly included constipation (30.8%), incontinence (19.8%), enterocolitis (8%), diarrhea (11%) in a declining order of incidence. Down syndrome and others HD-associated anomalies were detected in 3.7% and 24.3% of cases respectively. CONCLUSIONS: Constipation and foecal incontinence were the most prevalent postoperative complications, which were reported almost as frequent in other studies. Yet, Enterocolitis, was reported slightly less in prevalence. Also mortality rates were considerably higher, compared to developed nations.