Efficacy of "high-intensity" blue-light and "standard" daylight phototherapy for non-haemolytic hyperbilirubinaemia

We report our clinical experience with phototherapy in 3802 infants; 3629 were exposed to "standard" daylight phototherapy and 173 to "high-intensity" blue-light phototherapy. High-intensity blue-light phototherapy was twice as effective as standard daylight phototherapy in decreasing bilirubin concentrations. No failures occurred with high-intensity phototherapy compared with an overall failure rate of 1.84/1000 with daylight lamps; these cases were transferred to high-intensity phototherapy with prompt response. Rebound after cessation of phototherapy was greater in those exposed to high-intensity blue light with a significantly greater number requiring a second exposure. However, the incidence was still low. No third exposure was required in any infant. Nursing of infants under high-intensity blue light was more difficult and inconvenient as was clinical monitoring. The light also caused more stress on the nursing and medical personnel. However, the infants tolerated both types of phototherapy equally well. High-intensity blue-light phototherapy would seem to be the treatment of choice for infants with rapidly increasing or very high bilirubin levels, as well as in those not responding adequately to daylight phototherapy.     

Jaundice

Jaundice is a yellow discoloration of body tissues usually observed in the skin secondary to excess bilirubin in the serum. This occurs due to imbalance between production and clearance of bilirubin. It is important to understand the pathophysiology of jaundice to understand its aetiology. Careful history and physical examination, together with appropriate urine and blood tests will guide further investigations to obtain an appropriate diagnosis. It is important to assess and address associated sepsis, dehydration, electrolyte imbalance and clotting abnormality to reduce morbidity prior to instituting any treatment plan. This often involves specialists from the multidisciplinary team.     

The effect of prebiotics in the management of neonatal hyperbilirubinaemia

Background:  Breast milk oligosaccharides such as galacto-oligosaccharides (scGOS) and fructo-oligosaccharides (lcFOS) can influence the intestinal microbial flora. The latter, in turn, can modulate several intestinal and extraintestinal functions, including bilirubin metabolism. Supplementing infant formula with a prebiotic mixture might then be a novel and safe intervention to manage mild neonatal hyperbilirubinaemia.
Aim:  To investigate the effect of dietary supplementation with prebiotics on moderate hyperbilirubinaemia in healthy, term infants.
Methods:  A prospective, double-blind, clinical trial was performed on seventy-six consecutive newborns who were randomly assigned to receive a formula containing 0.8 g/dL of a mixture from scGOS and lcFOS (ratio 9:1), or maltodextrines as placebo for 28 days. Bilirubin levels were determined by the transcutaneous bilirubin measurement within 2 h after birth (T1), at 24, 48 and 72 h and at 5, 7, 10 and 28 days of life. The number of stool per day was also recorded.
Results:  Neonates receiving prebiotics showed a larger number of stools over all the duration of dietary intervention compared to that of those on placebo (Repeated Measures ANOVA p < 0.001; day 28 3.4 ± 0.0.9 vs 1.7 ± 0.9, respectively; Dunn test p < 0.05). Neonates whose formula was supplemented with prebiotics showed a lower transcutaneous bilirubin that was statistically significant from 72 h of life (5.46 ± 1.6 vs 7.07 ± 2.49, post hoc Dunn test, p < 0.05) throughout the duration of the dietary intervention (day 28 2.41 ± 0.4 vs 2.85 ± 0.5, post hoc Dunn test, p < 0.05).
Conclusion:  The addition of prebiotics to standard infant diet might represent a novel strategy to help control neonatal hyperbilirubinaemia.     

Biochemical and clinical observations in four patients with fructose-1,6-diphosphatase deficiency

Three boys and one girl suffering from inherited fructose-1,6-diphosphatase (FDPase) deficiency are reported. All four patients had less than 25% residual hepatic FDPase activity. While in two out of three patients the enzyme deficiency was also expressed in leucocytes, one patient had a normal enzyme activity. Remarkably, three patients had pronounced neonatal hyperbilirubinaemia requiring exchange transfusion.     

Comparison of the efficacy of conventional special blue light phototherapy and fiberoptic phototherapy in the management of neonatal hyperbilirubinaemia

The efficacy and usefulness of two types of phototherapy differing in the source, wavelength and irradiance of the light, conventional phototherapy consisting of special blue light and fiberoptic phototherapy, were compared in a relatively larger series of term newborns with non-haemolytic and more significant hyperbilirubinaemia than those in previous studies. In total, 108 newborns were allocated sequentially to receive either conventional phototherapy consisting of five special blue lamps or fiberoptic phototherapy. The average spectral irradiance measured at the skin surface level of newborns during the study period was significantly greater in the conventional phototherapy group. The special blue lamp of the conventional phototherapy unit had an emission spectrum almost identical to the bilirubin absorption spectrum, whereas the tungsten-halogen lamp of the fiberoptic phototherapy had a broad emission through the blue and green wavelengths (mainly in the green spectrum). Phototherapy was more effective in the conventional phototherapy group; the duration of exposure to phototherapy (h) was significantly shorter, and the overall bilirubin decline rate (as micromol/l/h and %/h) was significantly greater in the conventional phototherapy group. According to the nursing personnel, fiberoptic phototherapy was more comfortable than the conventional phototherapy frame because of the easier accessibility and handling of the infants during phototherapy. They complained of giddiness, nausea, glare, temporary blurring of vision and difficulty in detecting the skin colour changes of newborns with the blue light of the conventional phototherapy unit. Conventional phototherapy consisting of special blue fluorescent lamps with approximately twofold higher irradiance and an emission spectrum almost identical to the bilirubin absorption spectrum is preferable to fiberoptic phototherapy in the standard treatment of term newborns with non-haemolytic hyperbilirubinaemia.     

D-PENICILLAMINE, A NON-BILIRUBIN-DISPLACING DRUG IN NEONATAL JAUNDICE

Abstract. Brodersen, R., Lakatos, L. and Karmazsin, L. (Institute of Medical Biochemistry, University of Aarhus, Denmark, and Pediatric Clinic, University Medical School, Debrecen, Hungary). D-penicillamine, a non-bilirubin-displacing drug in neonatal jaundice. Acta Paediatr Scand, 69:31, 1980.—D-penicillamine, a drug used clinically for the treatment of neonatal hyperbilirubinaemia, was tested for interference with the binding of bilirubin to human serum albumin by three methods: 1) The peroxidase technique, investigating the effect of D-penicillamine on the equilibrium concentration of unbound bilirubin in a solution containing a molar excess of albumin; 2) the MADDS method, measuring the concentration of vacant bilirubin binding site on albumin in a solution of pure albumin, or infant blood serum, with added D-penicillamine; and 3) injection of D-penicillamine into Gunn rats and determination of any decrease of plasma biiirubin which would be caused by displacement of the pigment. Results were negative in all cases. Quantitatively, the doses of D-penicillamine used clinically cannot displace bilirubin from its binding to albumin. The ameliorating effect on hyperbilirubinaemia in the newborn must be due to some other mechanism     

EFFECT OF PHOTOTHERAPY AND EXCHANGE TRANSFUSION ON PRIMARY BILE ACIDS IN THE SERUM OF HYPERBILIRUBINAEMIC NEWBORNS

ABSTRACT. Serum primary bile acid (cholic (CA) and chenodeoxycholic (CDCA) acid) concentrations were measured in 14 preterm and 11 full-term hyperbilirubinaemic newborns at the beginning and end of, and 24 and 72 hours following phototherapy. Only in the preterm newborns with gestational ages of 35-38 weeks there was a significant decrease of mean serum bile acid concentrations which could be shown 72 hours after the beginning of phototherapy. It can be hypothesized that the decrease was a result of a direct effect of light on the excretory liver function. Serum CA and CDCA concentrations were also measured in 5 hyperbilirubinaemic newborns at the beginning and end, and 24, 48 and 72 hours after the end of exchange transfusion. Exchange transfusion caused a clear immediate decrease in the mean serum primary bile acid concentrations. However, on day 2 after exchange transfusion the mean serum concentration of CA was about 150% and that of CDCA about 110% of the initial values. The most hyperbilirubinaemic newborns had extremely high primary bile acid serum concentrations before therapy. As bile acids compete with bilirubin for albumin binding it should be considered whether high bile acids in the serum of hyperbilirubinaemic newborns presuppose exchange transfusions.     

Transcutaneous bilirubin – comparing the accuracy of BiliChek® and JM 103® in a regional postnatal unit

Objective.?Transcutaneous bilirubin (TcB) has the potential to reduce serum bilirubin sampling. During a recent survey on the use of TcB in postnatal units in the Republic of Ireland, we identified that only 58% of the 19 units were using TcB and that only two devices were in use, the BiliChek^® and JM 103^®. We aimed to evaluate and compare these two devices in a regional postnatal unit.

Methods.?To evaluate and compare the accuracy of the BiliChek^® and JM 103^®, we studied simultaneous TcB and total serum bilirubin (TSB) measurements from a population of jaundiced term and near term infants. We evaluated each device with regard to correlation with TSB and potential to safely reduce serum bilirubin testing.

Results.?Both TcB devices strongly correlated with TSB (r?=?0.88 for BiliChek^® and r?=?0.70 for JM 103^®). The BiliChek^® and JM 103^® were accurate up to cut-off values of 200 μmol/L and 180?μmol/L, respectively. Using Bhutani's nomogram, 100% sensitivity was achieved using the 75th percentile for BiliChek^® and the 40th percentile for JM 103^®.

Conclusion.?Both TcB devices correlated closely with moderately increased TSB levels and are suitable screening tools to identify jaundiced infants that require a serum bilirubin, with upper limit cut-off values. Both devices reduced the need for TSB levels. We found the BiliChek^® slightly more accurate than the JM 103^® for our study population. TcB however, is not in widespread use.     

Cholelithiasis and Gilbert's syndrome in homozygous beta-thalassaemia

Cholelithiasis has been reported with a variable incidence in homozygous beta-thalassaemia, the reasons for which have only partially been defined. Disease-associated factors or specific modifier genes may be implicated. We assessed the prevalence of cholelithiasis and the effect of co-inherited Gilbert's syndrome genotype on its development in 261 thalassaemia major (TM) and 35 thalassaemia intermedia (TI) patients. Cholelithiasis was found in 20.3% of TM and in 57.1% of TI patients. Its incidence was higher (P < 0.05) in patients homozygous for the (TA7) motif in the promoter of the UGT1-A1 gene, the genotype associated with Gilbert's syndrome, which seems to be a risk factor for the development of gallstones in TM and TI patients.