Iranian Journal of Neonatology
Volume:15 Issue: 2, Spring 2024

Palliative care is an interdisciplinary approach to improves the quality of life of patients and their families who are facing challenges associated with life-threatening illness, whether physical, psychological, social or spiritual. The quality of life of caregivers improves as well. Each year, an estimated 56.8 million people, including 25.7 million in the last year of life, are in need of palliative care. Worldwide, only about 14% of people who need palliative care currently receive it (1). "End of life care" (ELC) is a part of "palliative care". Palliative care is provided at every stage of the treatment process, but ELC is done only for the end of life. The aim of ELC is improving the quality of life and providing conditions for a comfortable and dignified death. Pain control is the most important part of palliative care and should be controlled within 15 minutes after pain is discovered. Then, it is recommended that you assess pain at least every 3 hours with your department's guidelines (2). The first month of life has the highest risk of dying (NMR), with an average global rate of 17 deaths per 1,000 live births in 2022, down by 53 per cent from 37 deaths per 1,000 live births in 1990. Mortality rate after the first month and before reaching age 1 (IMR) was estimated at 11 deaths per 1,000 and Mortality rate after reaching age 1 and before reaching age 5 (Under 5MR) was estimated at 9 deaths per 1,000 in 2022. Globally, 2.3 million children died in the first month of life in 2022 – approximately 6,300 neonatal deaths every day (3, 4). Also in the United States, the daily cost of NICU is over $3,500 per infant. An extended stay typically costs up to $1 million. This fee does not include the cost of caring for a severely disabled child after discharge from the hospital (5). Despite the high mortality and morbidity of newborns in the world, in a cross-sectional study, through a survey (400 neonatal nurses), from 11 countries: 48% of them were not trained for ELC and only 67 nurses (17%) stated that their NICU unit has a protocol for performing ELC (6). Certainly, the situation of palliative care in developing countries is more challenging (7, 8). We need to formulate and train comprehensive protocols for palliative and end of life care, respecting the cultural and religious issues of these countries (9). Until then, we need to emphasis on pain control in infants with life-limiting disease.

Preterm delivery is a risk factor for increased respiratory morbidity in early childhood. This study aimed to quantify respiratory morbidity in preterm survivors, comparing incidence rates among different gestational ages, birth weighst, and current age groups. Additionally, we sought to evaluate variations in respiratory outcomes between groups with and without bronchopulmonary dysplasia (BPD).
Our study included 89 prematurely born children who were receiving palivizumab prophylaxis for respiratory syncytial virus infection. We categorized the patients based on four criteria: 1) current age of less and more than 1 year, 2) gestational age (GA) of less and more than 30 weeks, 3) birth weight (BW) of less than 1000 g, between 1000 g and 1500 g, and more than 1500 g, 4) with and without BPD. We compared these groups in terms of respiratory morbidity and respiratory therapy.
The average age was 11.9 months, the average GA was 28.9 weeks and the average BW was 1202.4 g. According to the 28-day definition of BPD 75.3% patients had BPD. Around one-third (35.9%) of patients experienced wheezing episodes, 7.8% had pneumonia, 10.1% were hospitalized due to respiratory exacerbation and just 1.1% had RSV infection. There were no statistically significant differences between the different age, GA, BW, or BPD/non-BPD groups in the number of hospitalizations or pneumonia. On the other hand, children older than 12 months and children with BPD had significantly more wheezing episodes. Fifty-nine (66.3%) patients had been receiving inhaled corticosteroids (ICS), all of whom had BPD.
Prematurely-born children receiving palivizumab had significant respiratory morbidity, but majority did not have RSV infection. Further clinical studies are necessary to improve our understanding of the role of ICS in patients with established BPD.

Our study aimed to compare the twice-weekly and three-time weekly prophylactic fluconazole regimens among low birth weight premature neonates.
Premature neonates (40 cases) were divided into two groups and received two and three-times-weekly fluconazole regimens for three weeks. Surveillance of fungal colonization was performed before treatment and twice weekly.
Fungal colonization occurred in 11 infants in group A and 4 infants in group B (55% vs. 20%, p=0.022). In both groups, the rectum was the most common site of colonization. Candida glabrata, Candida orthopsilosis and, Candida albicans were the most commonly isolated species in both groups. There were no significant differences between the two groups regarding birth weight, gestational age, and other baseline risk factors for fungal colonization.
In this study, the three-times-weekly administration of prophylactic fluconazole appears to be more effective in preventing fungal colonization than the twice-weekly regimen among premature neonates.

Although endotracheal intubation is an emergency procedure that is frequently performed in neonatal care, there is still uncertainty about the best method for the estimation of the optimal depth of the endotracheal tube (ETT) in neonates. We aimed to compare two different methods of estimation of ETT insertion depth in neonates.
This study was conducted on 120 neonates needing intubation. The ETT insertion depth was estimated in 60 neonates according to the oro-helical length (OHL) method and it was estimated in the other 60 neonates according to the Tochen’s formula method. Both groups were compared in correct estimation of the proper ETT position in chest radiography.
The incidence of ETT malposition did not differ significantly between the OHL group (31.7 %) and the Tochen’s formula group (45 %) (P > 0.05). There was also no significant difference in chest expansion in both groups in chest X ray.  Two neonates in the OHL group developed air leaks (3.3 %) versus 3 neonates in Tochen’s formula group (5 %) with no significant difference (P > 0.05). Furthermore, there was no significant difference between both methods regarding the incidence of accidental extubation, There was a positive correlation between the estimated ETT insertion depth by OHL measurement and weight and gestational age (p < 0.001).
The OHL measurement can be used as an alternative to Tochen’s formula for estimation of the optimal ETT insertion depth especially in emergency situations when the weight of the baby is not known.

Altered concentrations of cerebrospinal fluid (CSF) and blood amino acids may be related to epilepsy or the severity of the seizure. In the present study, we assessed the concentrations of amino acids in CSF and plasma in neonates with refractory seizures.

In this prospective cohort study, 27 neonates aged 1 to 56 days with refractory seizures were included. Blood and CSF samples were collected from each neonate within 24 hours after receiving 2nd antiseizure medications. All plasma and CSF samples were sent to the laboratory to measure amino acid concentrations. The associations between CSF and plasma amino acid levels with different variables were evaluated.

Except for leucine (P=0.15) and isoleucine (P=0.07), the levels of all amino acids were significantly higher in plasma than CSF. Significant associations were observed between types of seizure and plasma citrulline (P=0.008) and leucine concentrations (P=0.04). The mean of CSF glutamic acid was also statistically different among neonates with different EEG results (P=0.02).

Our findings indicate that several plasma and CSF amino acids could be candidate biomarkers for neonatal refractory seizures. Further studies with larger sample size are to confirm our findings.

The objective of our study to analyze the data of the prenatal diagnosis of congenital malformations at the maternity and reproductive health hospital "Les Orangers" in Rabat and to identify the main anomalies detected, their percentage and their short-term outcome.

This is a cross-sectional study conducted at the maternity and reproductive health hospital "Les Orangers" in Rabat, Morocco. The data was collected and reported on pre-established sheets and on the register of malformations of the hospital.

A total of 245 cases of congenital malformations comprising 470 types of congenital malformations were recorded out of a total of 43,923 births over a period of five and a half years, giving a prevalence of 5.58 per thousand.Prenatal diagnosis was made in a third of cases (33%), essentially during the 2nd-3rd trimester of pregnancy. The anomalies revealed by this antenatal diagnosis were dominated by urinary malformations in 70% and central nervous system anomalies in 67%, followed by other types of congenital anomalies in less than 40% of cases, while genetic problems were detected in 2.5%; this rate is underestimated since chromosomal abnormalities sometimes appear as syndromes, so that in some diseases, genetic changes are not separated from other abnormalities, and since more than half (50.7%) of cases presenting polymalformative syndromes not survived 77% of cases i.e. (48.5% of deaths and 28.5% of FDIU) and an etiological study was not carried out.

Antenatal ultrasound allows early detection and monitoring of the evolution of congenital malformations, and thus the possibility of ensuring early and adequate management of these anomalies from birth. In our context, it is necessary to develop a prenatal screening program for congenital anomalies and a network of reference centers for the management of these anomalies in order to improve their prognosis.

Studies have indicated contradictory results concerning the impact of protein-based diets on very low birth weight neonates. Hence, this study explored the impacts of various calorie and protein diets on the growth of very low birth weight neonate during 15 days.
This study was carried out on 44 neonates with very low birth weight, selected regarding the inclusion criteria. They were assigned into two clusters based on their birth weight. Each cluster included a control group receiving the standard diet formula, and an experimental group with neonates weighing 1000-1500gr who followed a high-energy diet with 4gr protein and those weighing less than 1000gr who received a high-energy diet with 4.2gr protein. Finally, the neonates' weight, height, and head circumference were measured every 3 days for 15 days.
According to the findings, the mean weight of  the neonates who weighed between 1000 and 1500gr was higher in the intervention group from the third day after initiating the diet, though no significant difference was observed. Similarly, the mean weight of intervention group in the second cluster was higher than the neonates in the control group from the third day with a statistically significant difference on the 15th day. Moreover, no significant difference was found in other measurements between groups.
The results demonstrated the effect of a higher weight of protein intake group on the 15th day. Hence, it recommends providing higher protein intake formula to lower infant birth weight for better growth.

Congenital malformations of the digestive system (CMDS) are one of the major causes of newborn mortality, particularly in developing countries. This study was conducted to evaluate the prevalence, pattern, gender distribution of CMDS as well as ethnicity status among newborns in Golestan Province, northern Iran.

This descriptive and analytical study was conducted on 36,156 live births to identify all newborns with CMDS, born between March 2018 and March 2019 in Golestan Province, north of Iran. The type of CMDS according to the International Classification of Diseases (ICD-10), the gender and ethnicity status of the newborns were recorded. The prevalence rate was estimated and compared using the exact Poisson method and odds ratio with a 95% confidence interval. Data were analyzed using STATA 14.0 software.

The prevalence rate of CMDS in newborns was 2.13 per 1000 live births. The risk of CMDS was significantly higher in males compared to females (OR=1.78, 95% CI: 1.12-2.85). The most common anomaly was Ankyloglossia (19.28%). The prevalence rate of CMDS among native Fars, Turkmen and Sistani ethnic groups was 2.15 [95% CI: 1.50-2.99], 3.05 [95% CI: 2.06-4.35] and 1.48 [95% CI: 0.64-2.91] per 1000 live births, respectively.

Due to the prevalence rate of CMDS and ethnic diversity in this region, effective prevention planning, early diagnosis and thorough screening are particularly important, especially in high-risk groups.

Most infants born prematurely are admitted to the NICU for their survival, an environment that is rich in stressors that has non positive effect on the sleep and wakefulness condition. The goal of this study was evaluating the effect of the QTP intervention on the sleep status of premature neonates admitted to the NICU.
This study was semi-experimental and 62 premature babies hospitalized in NICU, who were selected by available sampling method. The tools used included demographic information questionnaire and Als sleep and wakefulness tool. Infants in two groups were evaluated in 3 stages. before, during and after the intervention in the evening shift from 15:00 to 17:00 for 60 minutes. The data was analyzed using SPSS software (version 22). A  p value of less than 0.05 was considered statistically significant. The validity of the mentioned tool was investigated according to the content method according to the study of Rajaei in 2013 under the title of investigating the sleep-wakefulness of premature infants and its relationship with demographic characteristics, and the reliability of the tool was also investigated according to the same study.
The chi-square test showed that the variable frequency of deep sleep (type A) before (P=1)and after (P=0.05) the intervention in the two groups did not have a statistically significant difference and just during the intervention has a statistically significant difference (P<0.001). Comparison of the distribution of the frequency of deep sleep (type B) before, during and after the intervention in 2 groups shows that before the intervention, deep sleep (type B)  was not observed in the two groups. However, in the intervention group, 58.1% had deep sleep during the intervention and 6.5% after the intervention. The control group did not have deep sleep (type B) in these three time periods.
Considering the effectiveness of QTP in reducing environmental stimuli and improving sleep status, implementation of these items is recommended as a standard care to reduce stress, improve growth and development of preterm infants in the nicu.

Klinefelter syndrome (KS) is the most prevalent sex-chromosome aberration and the leading genetic cause of male hypogonadism. This chromosomal anomaly results in male hypergonadotropic hypogonadism, androgen deficiency, impaired spermatogenesis, and cognitive impairment. On the other hand holoprosencephaly (HPE) is a complex developmental disorder that represents a profound malformation of the human brain, characterized by the failure of midline cleavage of the prosencephalon into the right and left hemispheres.

Case Report: 

The study presents a unique clinical scenario involving the co-occurrence of alobar holoprosencephaly (HPE) and Klinefelter syndrome (KS) in a term male fetus born to a healthy couple with uneventful prenatal ultrasound studies. While Klinefelter syndrome is known for its chromosomal aberrations, the simultaneous presence of HPE raises intriguing questions regarding the potential association between KS and cerebral malformations.

This case highlights the importance of further research to explore the underlying mechanisms and implications of this rare intersection, shedding light on previously uncharted territory in the realm of genetic and neurodevelopmental disorders.