Neonatal transient pseudohypoparathyroidism: could it be included among inactivating parathyroid hormone (PTH)/PTH-related protein signalling disorders?

We report a case of transient pseudohypoparathyroidism in a full-term newborn that presented at 20 hours of life with hypocalcemic seizures, hyperphosphatemia and raised parathormone levels. The diagnosis of pseudohypoparathyroidism was made according to biochemical investigations. The infant was treated with calcium supplementation and vitamin D analog therapy, and he remained stable and symptom-free with normal serum biochemistries during follow-up. We suggest that transient pseudohypoparathyroidism of the newborn (ntPHP) might be included among inactivating parathyroid hormone (PTH)/PTH-related protein signaling disorders as defined by the classification schema recently proposed by the European Pseudohypoparathyroidism Network. To the best of our knowledge, this is the first report in which the new classification has been applied to a case of ntPHP.

The Long-Term Efficacy of a Galactagogue Containing Sylimarin-Phosphatidylserine and Galega on Milk Production of Mothers of Preterm Infants.

AIMS: To investigate the efficacy of a galactagogue, containing Sylimarin-phosphatidylserine (SILITIDIL) and galega consumed in the first month after delivery by mothers of preterm infants, in maintaining milk production during the first 3-6 months after delivery. MATERIALS AND METHODS: Mothers of infants born at gestational age (GA) between 27 and 32 weeks, enrolled in our previous prospective, double-blind, randomized trial and randomly allocated to receive either the galactagogue (GG) or a placebo (PG), were asked about their milk production at 3 and 6 months after delivery. RESULTS: Of the 100 mothers involved in this study, 45 of GG and 44 of PG responded comprehensively to the questions asked. At the third month after delivery, exclusive human milk administration was reported by 22 mothers of GG and 12 mothers of PG (p < 0.05), whereas 29 mothers of GG and 18 mothers of PG were able to administer >50% of the amount of milk assumed. At the sixth month of life, only eight infants received exclusive human milk (six and two of GG and PG, respectively), and the data are not sufficient for a meaningful clinical evaluation. CONCLUSIONS: It is assumed that a galactagogue during the first month after delivery improves human milk administration to preterm neonates after discharge and for the first 3 months of life.

BiliCheck vs JM-103 in identifying neonates not at risk of hyperbilirubinaemia.

BACKGROUND: Transcutaneous bilirubinometry is widely used to predict hyperbilirubinemia by using several devices. The aim of this study was to compare the predictive ability of BiliCheck vs JM-103 in identifying neonates not at risk of significant hyperbilirubinemia, putting the data obtained with the two instruments on our transcutaneous bilirubin nomogram built with the BiliCheck. METHODS: Transcutaneous bilirubin (TcB) measurement was performed when jaundice appeared in newborn babies and/or just before discharge from the hospital. It was performed at the forehead with the two instruments within 5 minutes by two experienced neonatologists, each one blind to the value obtained by the other. Blood samples were drawn to obtain total serum bilirubin (TSB) levels soon after TcB measurements. RESULTS: A total of 627 paired-sample measurements were obtained from 298 newborn babies. Out of the total population studied, 16 newborn babies (5.4%) showed significant hyperbilirubinemia defined as TSB value >17 mg/dL, or as need for phototherapy treatment according to the AAP guidelines. TcB measurements showed false negative results in the first 60 hours of life using both devices. After the 60th hour of life, TcB measurements using both devices successfully predicted newborn babies not at risk of significant hyperbilirubinemia, being the JM-103 more reliable than BC because of fewer false positive results. CONCLUSIONS: Our study shows that both BC and JM-103 can exclude subsequent significant hyperbilirubinemia when the measurements are performed after the 60th hour of life. Nevertheless, the transcutaneous pre-discharge screening should be considered only as the first step, and it has to be followed by a follow-up through the first days after discharge.

Validation of transcutaneous bilirubin nomogram in identifying neonates not at risk of hyperbilirubinaemia: a prospective, observational, multicenter study.

BACKGROUND: Transcutaneous bilirubin (TcB) measurement is widely used as screening for neonatal hyperbilirubinaemia. AIMS: To prospectively validate TcB measurement using hour-specific nomogram in identifying newborn infants not at risk for severe hyperbilirubinaemia. STUDY DESIGN: prospective, observational, multicenter. SUBJECTS: 2167 term and late preterm infants born in 5 neonatal units in the Lazio region of Italy. METHODS: All neonates had simultaneous TcB and total serum bilirubin (TSB) measurements, when jaundice appeared and/or before hospital discharge. TcB and TSB values were plotted on a percentile-based hour-specific transcutaneous nomogram previously developed, to identify the safe percentile able to predict subsequent significant hyperbilirubinaemia defined as serum bilirubin >17 mg/dL or need for phototherapy. RESULTS: Fifty-five babies (2.5%) developed significant hyperbilirubinaemia. The 50th percentile of our nomogram was able to identify all babies who were at risk of significant hyperbilirubinaemia, but with a high false positive rate. Using the 75th percentile, two false negatives reduced sensitivity in the first 48 hours but we were able to detect all babies at risk after the 48th hour of age. CONCLUSIONS: This study demonstrates that the 75th percentile of our TcB nomogram is able to exclude any subsequent severe hyperbilirubinaemia from 48 h of life ahead.

Does Ibuprofen increase neonatal hyperbilirubinemia?

OBJECTIVE: The aim of this study was to investigate whether ibuprofen exposure was associated with increased hyperbilirubinemia in preterm infants. METHODS: Since 2000, ibuprofen has been administered to all infants at <30 weeks of gestation who are admitted to our unit, to prevent patent ductus arteriosus. We retrospectively compared data for 418 infants subjected to ibuprofen prophylaxis (2000-2007) and 288 infants not exposed to ibuprofen (1993-1999). RESULTS: The ibuprofen group had a significantly higher peak total serum bilirubin level (9.0 +/- 2.5 mg/dL vs 7.3 +/- 3.3 mg/dL), more need for phototherapy (398 infants [95%] vs 254 infants [87.6%]), and a longer phototherapy duration (94.3 +/- 43.6 hours vs 87.2 +/- 38.6 hours). Groups did not differ with respect to gestational age, birth weight, gender ratio, glucose-6-phosphate dehydrogenase deficiency incidence, or hypoalbuminemia (<2.5 g/dL) incidence. Hemolytic isoimmunization was diagnosed with similar incidences (no-ibuprofen group: 7 of 288 infants; ibuprofen group: 8 of 418 infants). The rates of exchange-transfusion also were similar between the groups (no-ibuprofen group: 14 infants [4.8%]; ibuprofen group: 19 infants [4.5%]). CONCLUSIONS: Ibuprofen administration was associated with higher peak total serum bilirubin levels, and the more-pronounced hyperbilirubinemia led to longer phototherapy. The potential role of competition between ibuprofen and bilirubin in the hepatic glucuronidation pathway is discussed.

Skin bilirubin measurement during phototherapy in preterm and term newborn infants.

BACKGROUND: The few existing studies evaluating the reliability of transcutaneous bilirubin monitoring during phototherapy gave controversial results. AIMS: To evaluate the accuracy of transcutaneous bilirubin measurement in a large population of newborn infants, during phototherapy. STUDY DESIGN AND METHODS: Total serum bilirubin and transcutaneous bilirubin on patched and unpatched skin areas were simultaneously measured in newborn infants undergoing phototherapy. Transcutaneous measurements were performed with a multiwavelength transcutaneous bilirubinometer (Respironics BiliCheck). The Passing-Bablok regression and the Bland-Altman plot were used to estimate the relationship between serum and transcutaneous bilirubin. RESULTS: We studied 364 newborn infants with a mean (SD) gestational age of 34.6 (3) weeks and a mean birth weight of 2371 (805) grams. Total serum bilirubin, patched transcutaneous bilirubin and unpatched transcutaneous bilirubin were similar before phototherapy. After 52 (33) hours of phototherapy, the difference between serum bilirubin and patched transcutaneous bilirubin was 0.2 (3.1) mg/dL (not significant) while the difference between serum bilirubin and unpatched transcutaneous bilirubin was 3.2 (3.0) mg/dL (p<0.001). Statistical analysis showed a good agreement between serum bilirubin and patched transcutaneous bilirubin, while unpatched transcutaneous bilirubin underestimates serum levels. The difference between patched and unpatched values was significantly lower in preterm than in term infants (2.8 mg/dL vs. 3.6 mg/dL; p<0.001). CONCLUSION: BiliCheck can be safely used for the evaluation of bilirubin levels in newborn infants under phototherapy. Its reliability on patched skin of the forehead is high enough to consistently reduce blood draws and to ascertain when to discontinue phototherapy. Because of the individual variance, any clinical decision has to be taken on the basis of the transcutaneous bilirubin trend more than on a single value.

Which phototherapy system is most effective in lowering serum bilirubin in very preterm infants?

OBJECTIVE: To compare the effectiveness of various phototherapy systems in lowering serum bilirubin levels in preterm infants. METHODS: This randomized clinical trial enrolled 140 preterm infants with gestational age < or =30 weeks and presenting nonhemolytic hyperbilirubinemia. When total serum bilirubin level reached 6.0 mg/dl (102.6 micromol/l), eligible infants were randomly assigned to four study groups: conventional, fiberoptic Wallaby, fiberoptic Biliblanket, and combined phototherapy. Efficacy was assessed by comparing highest serum bilirubin levels, duration of treatment, and number of infants requiring exchange transfusion. RESULTS: Our results confirm that fiberoptic phototherapy, both Wallaby and Biliblanket, had the same effectiveness of conventional phototherapy. The best results have been obtained using combined phototherapy, which allowed to reach lower serum bilirubin levels, a shorter duration of treatment and a significant reduction of exchange transfusions. CONCLUSION: Our data suggest that combined phototherapy should be the method of choice in treating hyperbilirubinemia in very preterm infants.