Anesthetic management of a neonate with congenital diaphragmatic hernia under high-frequency oscillatory ventilation.

Perioperative management of a neonate with congenital diaphragmatic hernia (CDH) is challenging because of pulmonary hypoplasia, pulmonary hypertension, and respiratory insufficiency. In this report, we present our intra-operative experience in a 4-days old and 3070 grams CDH neonate. He was admitted to neonatal intensive care unit and intubated due to severe respiratory insufficiency. He showed signs of severe pulmonary hypoplasia and his echocardiography revealed a cardiac dextroversion. The patient was relatively stabilized after four days under combined high-frequency oscillatory ventilation (HFOV) and inhaled nitric oxide (iNO). A corrective surgical intervention was sustained with dopamine, dobutamine, fentanyl and midazolam infusions. Ventilator settings were: 9 cmH2O MAP; 15-Hz frequency; 30 cmH2O amplitude and 55% FiO2. Venous-blood gas analysis indicated pH:7.38 pO2:36.2, pCO2:39.2 with SpO2:98%. We believe that HFOV and iNO combination is an effective alternative for the anesthetic management of CDH cases as it provides better gas exchange and less volutrauma.

Novel homozygous inactivating mutation of the calcium-sensing receptor gene (CASR) in neonatal severe hyperparathyroidism-lack of effect of cinacalcet.

BACKGROUND: NSHPT is a life-threatening disorder caused by homozygous inactivating calcium-sensing receptor (CASR) mutations. In some cases, the CaSR allosteric activator, cinacalcet, may reduce serum PTH and calcium levels, but surgery is the treatment of choice. OBJECTIVE: To describe a case of NSHPT unresponsive to cinacalcet. PATIENT AND RESULTS: A 23-day-old girl was admitted with hypercalcemia, hypotonia, bell-shaped chest and respiratory distress. The parents were first-degree cousins once removed. Serum Ca was 4.75 mmol/l (N: 2.10-2.62), P: 0.83 mmol/l (1.55-2.64), PTH: 1096 pg/ml (9-52) and urinary Ca/Cr ratio: 0.5mg/mg. First, calcitonin was given (10 IU/kg × 4/day), and then 2 days later, pamidronate (0.5mg/kg) for 2 days. Doses of cinacalcet were given daily from day 28 of life starting at 30 mg/m2 and increasing to 90 mg/m2 on day 43. On day 33, 6 days after pamidronate, serum Ca levels had fallen to 2.5 mmol/l but, thereafter, rose to 5 mmol/l despite the cinacalcet. Total parathyroidectomy was performed at day 45. Hungry bone disease after surgery required daily Ca replacement and calcitriol for 18 days. At 3 months, the girl was mildly hypercalcemic, with no supplementation, and at 6 months, she developed hypocalcemia and has since been maintained on Ca and calcitriol. By CASR mutation analysis, the infant was homozygous and both parents heterozygous for a deletion-frameshift mutation. CONCLUSION: The predicted nonfunctional CaSR is consistent with lack of response to cinacalcet, but total parathyroidectomy was successful. An empiric trial of the drug and/or prompt mutation testing should help minimize the period of unnecessary pharmacotherapy.

Acute peritoneal dialysis in neonates with acute kidney injury and hypernatremic dehydration.

OBJECTIVE: We aimed to evaluate the efficacy of acute peritoneal dialysis (PD) and clinical outcomes in neonates with acute kidney injury (AKI) and hypernatremic dehydration. ♢ METHODS: The medical records of 15 neonates with AKI and hypernatremic dehydration who were treated with acute PD were reviewed. The diagnoses were AKI with hypernatremic dehydration with or without sepsis in 13 patients and AKI with hypernatremia and congenital nephropathy in 2 patients. The main indications for PD were AKI with some combination of oligoanuria, azotemia, hyperuricemia, and metabolic acidosis unresponsive to initial intensive medical treatment. ♢ RESULTS: The mean age of the patients at dialysis initiation was 11.9 ± 9 days, and the mean duration of PD was 6.36 ± 4.8 days. In 7 patients (46.7%), hypotension required the use of vasopressors, and in 6 patients (40%), mechanical ventilation was required. Peritoneal dialysis-related complications occurred in 7 patients (46.7%), the most common being catheter malfunction (n = 6). Four episodes of peritonitis occurred in the 15 patients (26.7%), 2 episodes in patients with congenital renal disease and 2 episodes in patients with sepsis and multiorgan failure, who did not survive. Congenital renal disease, septicemia, and the need for mechanical ventilation were important factors influencing patient survival. All patients with no pre-existing renal disease or sepsis recovered their renal function and survived. ♢ CONCLUSIONS: In neonates with AKI and hypernatremic dehydration, PD is safe and successful, and in patients without congenital renal disease or sepsis, the prognosis is good. Peritoneal dialysis should be the treatment of choice in neonates with AKI and hypernatremic dehydration who do not respond to appropriate medical treatment.

Congenital diaphragmatic hernia: a 4-year experience in a single centre.

BACKGROUND: This study aimed to evaluate congenital diaphragmatic hernia (CDH) patients in our department during a 4-year period. PATIENTS AND METHODS: A retrospective study of 10 cases of CDH patients managed in the Neonatology and Pediatric Surgery Units of Goztepe Teaching Hospital from 2000 to 2004. RESULTS: The mean birth weight of the patients was 2,600 g and the mean gestational age was 37.6 weeks. The mean admission time for the patients was 10.4 h. The mortality rate was 50%, comprising two deaths before operation and one during operation. The surviving infants followed for 1 year are free of any symptoms and complications. CONCLUSION: Conventional ventilation is an other treatment modality, especially in order to gain time before surgical operation for newborns with CDH.

Comparison of two doses of breast milk and sucrose during neonatal heel prick.

BACKGROUND: The aim of the present study was to test analgesic effects of double- versus single-dose breast milk and compare this effect with efficacy of double- versus single-dose sucrose in a group of healthy term newborns during heel prick blood sampling. METHODS: Healthy newborns (n= 142) were consecutively allocated to one of the six groups: group 1, single-dose breast milk; group 2, single-dose sterile water; group 3, single-dose 12.5% sucrose; group 4, two doses breast milk; group 5, two doses sterile water; and group 6, two doses 12.5% sucrose before the heel prick. The medians for crying time and the pain scores according to the neonatal facial coding system were recorded. RESULTS: Crying times were 117 s, 126 s, 82 s, 128 s, 117 s, and 95 s in groups 1-6, respectively (P= 0.053). The mean pain scores were 4.60, 5.82, 3.91, 4.94, 5, and 4.05 in groups 1-6, respectively (P= 0.068). There was a significant difference between the groups for mean pain scores at 1 min and 3 min. There was a significant difference between the single-dose sucrose group and single-dose sterile water group at 1 min (P= 0.002). The babies in the sucrose group were active awake, whereas the ones in the breast milk group were asleep before heel prick. CONCLUSION: Two doses of sucrose solution were not superior to single-dose sucrose. Neither single nor double doses of breast milk were effective in relieving pain in neonates. Two milliliters breast milk does not reduce response to pain during minor painful procedures in term neonates even when two doses have been given. Further studies are needed.

Quantitative ultrasound and biochemical parameters for the assessment of osteopenia in preterm infants.

OBJECTIVE: Our study aimed to evaluate the feasibility of quantitative ultrasound (QUS) evaluation in osteopenia of prematurity and to compare the results to biochemical parameters. METHODS: QUS assessment of bone was performed at the end of the first postnatal week and at term-corrected age (CA) in premature infants (N = 30) and within the first week in full-term infants (N = 25). On the same day of measurement of QUS, the serum calcium, phosphorus (inorganic), and alkaline phosphatase (ALP) activity were measured in the preterm infants. RESULTS: The median of tibia z score at term-CA in premature infants was significantly lower compared to that of first postnatal week (-1 and 0.4, respectively; p < 0.0001) and it was also lower than that of term-matched controls (0.0; p = 0.001). Preterm infants at term-CA had lower weights and lengths in comparison to term infants. The median ALP value was 585 IU/L at the first postnatal week and 703 IU/L at term-CA in preterm infants (p = 0.003). The median tibia z score of infants with ALP >or=900 IU/L was significantly lower than that of the infants with ALP <900 IU/L (-1.4 vs. 0.1; p = 0.001). An inverse correlation was found between ALP levels and tibia z score at term-CA in preterm infants (rho = -0.61, p = 0.01). CONCLUSIONS: Bone density of preterm infants at term-CA was lower than that at first postnatal week. Serum ALP levels increased during the first postnatal weeks. The tibia z scores were correlated to serum ALP levels. QUS is a good screening tool for the detection of osteopenia.

Report of three cases: congenital chylothorax and treatment modalities.

Chylothorax is the most common cause of pleural effusion in the newborn. We report three patients with congenital chylothorax and discussed the clinical course and treatment options. Cases 1 and 2 with congenital chylothorax were treated by chest tube placement and total parenteral nutrition (TPN), and were fed a formula rich in medium-chain triglyceride. They were discharged home without any sequelae. Our 3rd case with chylothorax did not respond to the conventional therapies. Octreotide infusion was tried without any benefits and necessitated surgical intervention, but the infant developed chronic lung disease requiring nasal oxygen therapy until three months of age. All three patients developed complications of chylothorax treatment like chest tube dysfunction, pneumothorax, nosocomial sepsis, and cholestasis. Management of congenital chylothorax necessitates a multidisciplinary approach. Treatment options include pleural drainage, cessation of enteral feeding and initiation of TPN. Experience with octreotide treatment is limited. Surgery should be reserved for severe and refractory cases.

Sweet solutions and pacifiers for pain relief in newborn infants.

In this study we aimed to assess and compare the analgesic effects of orally administered sucrose, dextrose, dextrose or sucrose followed by a pacifier, and sterile water during minor painful procedures in neonates. One hundred thirty-eight healthy term newborn infants were enrolled in this prospective study. They received either sweet solutions or sweet solutions followed by pacifiers before the heel prick (group 1, dextrose 12.5%; group 2, dextrose 12.5% followed by a pacifier; group 3, sucrose 12.5%; group 4, sucrose 12.5% followed by a pacifier; and group 5, sterile water). The median values for crying time and the pain scores performed according to the neonatal facial coding system were recorded. The median crying times were 16.5, 55, 92.5, 102, and 132 seconds in groups 4, 2, 3, 1, and 5, respectively (P = .0001). The pain scores showed that babies in group 4 had significantly lower scores followed by groups 2, 3, 1, and 5 (P = .0001). Although group 4 had a lower pain score and shorter crying time than group 2, the difference was not statistically significant (P = .27 and P = .39). In conclusion, 12.5% dextrose or sucrose followed by a pacifier was found to be superior to dextrose only and sucrose only solutions in pain relief; sucrose followed by a pacifier resulted in lower pain scores and shorter crying time than dextrose when combined with a pacifier. The antinociceptive effect of sweet solutions can be enhanced with a pacifier.