Background

Congenital intestinal pseudo-obstruction in neonates occurs from gastrointestinal dysmotility. It usually manifests as increased gastric residue before feeding, abdominal distension, or constipation.

Objectives

We investigated the long-term outcome of congenital intestinal pseudo-obstruction in neonates.

Methods

We studied neonates with a diagnosis of congenital intestinal pseudo-obstruction who were admitted to NICU in Chung-Ang University Hospital between January 2006 and December 2018 (n=33). We excluded seven neonates because of loss to follow-up. We retrospectively analyzed their medical records and their follow-up outcomes (n=26).

Results

Twenty-six neonates were included in the study, with an average gestational age of 34.4 ± 4.2 weeks and an average birth weight of 2.3 ± 0.9 kg. Of these neonates, 18 were premature infants, and eight were term infants. Twenty neonates (76.9%) received enemas, and among these, nine neonates did not require further intervention. Furthermore, 16 neonates (61.5%) underwent surgery (ileostomy or colostomy). Repair operations were performed on average at 7.25 months after the initial surgical procedure.

Conclusion

Congenital intestinal pseudo-obstruction occurs primarily in premature infants when compared with term infants. This condition commonly develops because of immature ganglion cells. Early surgery in premature infants shows an improved outcome in growth and development.

Background

The optimal amounts of protein in critically ill infants are unknown.

Objectives

To study the effect of three different amounts of protein intake on protein balance in critically ill infants following cardiac surgery.

Methods

A randomized controlled open-label trial including infants between 1-24 months old was performed. Patients were randomized to receive: 1) standard diet (1.7 g protein/100 mL), 2) protein-enriched (2.6 g/100 mL), 3) high-protein enriched (5.1 g/100 mL). Blood and urine biochemical determinations, and nitrogen balance were performed during the first day of feeding and between the days 3 to 5.

Results

22 infants were included. Increase in total proteins, retinol-binding-protein, transferrin and nitrogen balance was higher in group 3. However, only a significant increase was observed in total protein in group 1, retinol and transferrin in group 3 and nitrogen balance in groups 1 and 2 (table). 1 patient of group 2 and 2 patients of group 3 presented urea increase higher than 80 mg/dl.

Conclusion

A higher protein intake was associated with higher increase of serum protein levels in infants following cardiac surgery. However, high-protein enriched diet could not be tolerated by some patients.

Background

Nutrition is one of the cornerstones of intensive care. Working out an individual nutritive program requires estimation of patient’s nutritive status, anthropometric parameters, daily protein needs, basal metabolic rate. Severe mechanical trauma (SMT), especially with leading brain injury, and furthermore accompanied with SIRS increases metabolic rate. On the contrary, spinal trauma with tetraplegia decreases it. Moreover, intensive care in patients with SMT includes drug-induced sleep, MLV, inotropic support, artificial hypothermia, each of these methods has impact on metabolic rate. We have to define current metabolic rate exactly to avoid hypo- or hyper alimentation.

Objectives

The aim of the study was to compare calculated and measured metabolic rate in children with SMT and to show a correspondence between metabolic rate and trauma structure, neurological status, SIRS and particularities of intensive care.

Methods

Our study includes 50 patients with SMT. Using Quarksline metabolograph we provided several metabolic rate measurements for each of them. The measured values were compared to calculated values of metabolic rate.

Results

The comparison showed that measured values were lower than calculated ones. SIRS, inotropic support lead to increase in energy needs while sedation, MLV, hypothermia and neurological deficit (such as tetraplegia or vegetative status) decreases metabolic rate. Under the influence of various factors current metabolic rate lies in a narrow range and changes quickly.

Conclusion

Indirect calorimetry seems to be an objective method of metabolic rate evaluation.

Background

Intestinal citrulline and arginine synthesis is limited in preterm infants. Arginine deficiency can contribute to the high rate of morbidity and mortality in premature births.

Objectives

Describe citrulline and arginine kinetics.

Methods

prospective observational study in patients born <1500 g. Citrulline and arginine (dried blood on paper, analysis by tandem mass spectrometry), and other analytical-clinical data collected at 3 and 15 days.

Results

58 patients: 29.6±2.5 weeks, 1210±346 grams. Only two cases developed necrotizing enterocolitis, two died and 46.6%developed sepsis. Initial citrulline (18.5±6.6 mcmol/L) falls significantly (p<0.05) at 15 days of age (14.2±4.5). Initial arginine (21.1±14.2 mcmol/L) falls significantly (p<0.05) at 15 days of life (14.0±6.5). Decrease in citrulline in first 15 days correlates with arginine (R 0.63, p>0.001). Greater basal citrulline correlates with lower gestational age (p 0.044), higher frequency of sepsis (p 0.037), more delay in initiating (p<0.001) and achieving complete enteral nutrition (p 0.026) and longer duration of parenteral nutrition (p 0,02). The highest arginine decrease at 15 days of age correlates with a higher frequency of necrotizing enterocolitis (p<0.001), sensitivity 98.1% and specificity 100% for cut-off point of 6.71 mcmol/L.

Conclusion

Arginine and citrulline decrease in first days of life in preterm infants, with a relationship between greater basal citrulline and worse digestive tolerance and between the greater decrease with more nosocomial sepsis. Citrulline levels are related to age and can serve as reference values, which facilitates the evaluation of compromised intestinal function in preterm infants with severe gastrointestinal problems. The greatest decreases in arginine are indicative of digestive complications.

Background

Carnitine availability is especially important during the immediate postnatal period.

Objectives

to characterize carnitine status in preterm infants <1500 g and to determine if not orally-fed low birth weight babies are at risk of carnitine deficiency.

Methods

Prospective observational study in patients born <1500 g. Free carnitine levels, and analytical-clinical data collected at 3 and 15 days.

Results

58 patients included, gestational age 29.6±2.5 weeks, 1210±346 grams. Two cases developed necrotizing enterocolitis and two died. Sepsis in 46.6%. Basal carnitine is 25.6±12.3 mcmol/L and falls significantly (p 0.011) at 15 days of age (21.0±8.9). This fall is 32.4% in cases that develop nosocomial sepsis compared to 5.4% in those who do not (p 0.022). Basal carnitine inversely correlates with the days of mechanical ventilation. Carnitine at 15 days of age rises more in patients of older gestational age and is inversely proportional to the days of invasive mechanical ventilation, the days of enteral initiation (p 0.016), the days with complete enteral nutrition (p 0.023) and duration of parenteral nutrition (p 0.014). Carnitine at 15 days of life is significantly lower in those who develop nosocomial sepsis (18.75±9.6 vs 22.9±8.0, p 0.016).

Conclusion

premature babies are born with a limited amount of carnitine and are not able to synthesize enough to maintain blood levels. Carnitine deficiency can occur despite parenteral nutrition, during the first two weeks of life. More research is needed into the metabolic consequences secondary to poor carnitine intake in premature infants before considering the administration of carnitine supplements.

Background

Nutritional support during pediatric critical illness is a fundamental goal of critical cares, which requires careful assesment and prescription. However, no nutritional protocols exists in Spain. In order to elaborate them, it is important to know first-hand about nutritional support practices in PICUs across the country.

Objectives

To review the nutritional support of children admitted to Spanish Pediatric Intensive Care Units (PICUs).

Methods

Prospective cross-sectional multicenter study performed in 13 Spanish PICUs. Children who received artificial nutrition (enteral or parenteral) were included. Patient and feeding characteristics during their admission were collected.

Results

Eigthy four children were included (54% males), 47.6% were younger than one year old. The most frequent diagnosis was cardiopathy (28.6%), followed by respiratory (22.6%) and neurological diseases (11.9%). Enteral nutrition was administered to 82% of the patients. Early enteral nutrition (<48 hours) was prescribed in 39.3% of the patients, being started in the first 24 hours of admission in 17.9%. Mean time to start enteral feeds was 1.1±0.93 days. Enteral nutrition was administered by nasogastric tube, continuous in 50% and discontinuous in 27.4% of patients, and by transpyloric tube in 21.4%. In total, 18 patients received parenteral nutrition. Mean enteral calorie intake was 43.4(IQR 35.2) Kcal/Kg/day and, attending to the maximum calorie intake, the mean was 83.3(IQR 20.4) Kcal/Kg/day.

Conclusion

Most patients received enteral nutrition during admission but only 39% of them had early enteral feeding. The most frequent way of enteral feeding was by nasogastric tube. Mean enteral calorie intake did not achieved the international recommendations.

Background

In general, most neonates will be checked for neonatal screening test about 3 to 7 days after birth.

Objectives

We examined the factors affecting the false positive results in the accidental neonatal screening test (NST) within 3 days after birth and tried to find out the most relevant factors.

Methods

From January 2011 to December 2016, we reviewed the electronic medical records of 142 patients with a gestational age of 34 weeks or more who had false positives at the first NST. NST was performed by tandem mass spectrometry. We collected information on gestational age, birth weight, delivery method, Apgar score at the time of birth. Postnatal exam time, body weight, feeding volume and parenteral nutrition, antibiotics, and steroid administration were also investigated at the time of NST.

Results

In the first NST, there were 9 cases of galactosemia, 27 cases of congenital hypothyroidism, 102 cases of congenital adrenal hyperplasia and 1 cases of homocystinuria. Gender, birth weight, and delivery method were statistically significant (P<0.05) at the time of birth using Pearson chi-square method. There were significant differences in body weight, feeding volume, amino acid and lipid administration at the time of NST (P<0.05). In binary logistic regression analysis, the gender, mode of delivery, and the body weight seemed significant statistically.

Conclusion

We could not identify the major factors of the false positive results in the NST performed within 3 days after birth. The first NST after birth should be performed at the time of outpatient department or after precise information collection.

Background

Nutrition support in Pediatric Intensive Care Units (PICUs) requires careful assesment and prescription because it can affect patients’ outcome.

Objectives

To review the nutritional status of children admitted to Spanish PICUs.

Methods

Prospective cross-sectional multicenter study performed in 13 Spanish PICUs. Children who received artificial nutrition (enteral or parenteral) were included. Anthropometric data, feeding characteristics and digestive complications during their admission were collected. Nutritional status was determined by Waterloo index.

Results

Eigthy four children were included. On admission, 42.3% of the patients suffered from acute malnutrition, 37.3% chronic malnutrition and 12.8% overnutrition. 17.9% of the children did not received any enteral nutrition, this percentage was higher in youngest patients (p=0.038). Mean enteral calorie intake was 43.4(IQR 35.2) Kcal/Kg/day and, the mean of maximum calorie intake was 83.3(IQR 20.4) Kcal/Kg/day. Only 39.3% of the patients received early enteral nutrition (<48 hours). Significant correlations were observed between days to onset of enteral nutrition and PRIMSIII (r= 0.33, p=0.006), maximum calorie intake (r= -0.25, p=0.04), and age (r= 0.35, p=0.03). There was a correlation between mean calorie intake and days on mechanical ventilation (r=0.31, p=0.02). The most frequent complication was vomiting (64%) and abdominal distension (15.5%). Enteral nutrition was not associated to severe digestive complications.

Conclusion

A high percentage of critically ill children are malnourished on admission. Only 39.3% of patients had early enteral feeding. Delayed enteral nutrition is related to higher PRIMSIII, less maximum calorie intake and younger patients. The most frequent complication was vomiting, no serious digestive complications were detected.

Background

Phenylketonuria (PKU) is the most prevalent disorder caused by an inborn error in aminoacid metabolism, increase in blood phenylalanine concentrations. If untreated, PKU leads to profound and irreversible mental disability.

Objectives

Monitoring children with PKU is permanent challenge for parents and clinicians. Likewise, the laboratory personnel has to provide accurate and eloquent results and very important from a therapeutical point of view, as well. In addition, the laboratory monitoring has to be relatively convenient for the patient and parent in terms of collecting and sending sample to laboratory.

Methods

Monitoring children with PKU is a permanent challenge for parents and clinicians. Likewise, the laboratory personnel has to provide accurate and eloquent results and very important from a therapeutical point of view, as well. In addition, the laboratory monitoring has to be relatively convenient for the patient and parent in terms of collecting and sending sample to laboratory.

Results

The levels of Phe obtained in DBS by both FEIA and LC-MS/MS techniques were lower than those determined in plasma in most cases, the differences recorded increasing proportionally to the concentration values.

The factors influencing the results are represented by the quality of DBS sample and hematocrit value.

Table 1 shows the results which were obtained by the three used techniques.

Table 1.

Conclusion

When the Phe values obtained in DBS by LC-MS/MS or FEIA techniques reach acceptable levels of Phe based on age, it is necessary to check the Phe plasma levels by HPLC.

Background

Denosumab is an inhibitor of receptor activator of nuclear factor kappa-b ligand (RANKL) widely used in adults with giant cell tumor of bone (GCTB), with scarce experience of use in children. It produces rapid suppression of bone resorption, and hypocalcemia during treatment or rebound hypercalcemia following treatment are possible adverse effects.

Objectives

To describe serious adverse effects following denosumab therapy.

Methods

Only two cases have been reported in children with severe hypercalcemia. We report a case of a six-year-old with cervical GCTB received neoadjuvant treatment with denosumab during one year.

Results

The patient presented 5 months after denosumab cessation with a 15-days history of vomiting, headache and lose 3 Kg weight.Investigations revealed marked hypercalcemia (albumin-corrected serum calcium 16,2 mg/dL and ionized calcium 8 mg/dl) with acute kidney injure (creatinine 1,8). The patient was treated with saline hydration, loop diuretics, GCs and calcitonin (4UI/kg). Because of refractory hypercalcemia, it was necessary to use one dose of zoledronic acid (0,05 mg/kg). Calcium normalized 72 hours later.Parathyroid hormone, serum phosphate, alkaline phoshatase, 25-hydroxy vitamin D and thyroid function were normal. Denosumab-induced rebound hypercalcemia was diagnosed following exclusion of other causes.

Conclusion

Denosumab is a human monoclonal antibody, increasingly used in the treatment of bone tumors in children. At present, safety and efficacy data in children are scarce, and there is no guidance on how often periodic assessments should be carried out. Treatment with denosumab must be taken into account in hypercalcemia without any other cause, and in young people monitoring during and after antiresorptive therapy is required.

Background

The ventilatory response and cardiac performance as a physiological adaptation to hypoxia in high sea level have been known. There is uncertainty data of ventilation and hemodynamic adaptation during childhood, either in the high or sea level. The long term impact of hypoxia to the child growth still not much explored.

Objectives

To compare the difference of ventilatory response and cardiac performance between normal growth and stunting children and their relationship to the difference sea level.

Methods

Forty three children (24 boys and 21 girls) age 1-5 years took part of this sutdy, 19 children in 700 meters above sea level and 24 children in the sea level. The portable BGA analyze and non-invasive device were measured the ventilation and hemodynamic parameter. Anthropometric was used to describe the growth state of the children. Related-pairs t test were used to examine the correlation between variables.

Results

From 43 children as the subjects, 23 among them were stunting. There was a strong correlation between pH, PaCO2 and total concentration of CO2, bicarbonate concentration, index of contractility and increase of lactate level with the children in sea level and 700 meters above sea level (p < 0.05 , significantly). No statistically significant the difference of those parameters in normal growth compare to the stunting children in sea level and 700 meters above sea level.

Conclusion

The results indicate the possibility of Haldane effect as the ventilatory and hemodynamic response of adaptation to hypoxemia in children with normal growth and stunting in the difference of sea level.