[Neonatal buprenorphine withdrawal syndrome, what is the right therapy?]. / Sindrome di astinenza da buprenorfina in neonato, quale terapia?

Buprenorphine is a new substance now widely used in detoxification of heroin addicts. In this report we describe the case of a newborn baby who suffered from a neonatal withdrawal syndrome characterized by a severe and prolonged course. This syndrome was followed by marked symptoms of impaired neurological development and by epilepsy. The ineffectiveness of methadone in relieving withdrawal symptoms from buprenorphine and its potential dangerous effects in this case are also outlined, whereas the efficacy of therapy with phenobarbytal is stressed. We think that this case is noteworthy, since the widespread use of buprenorphine among drug addicts will probably provoke an increase in the number of cases of buprenorphine withdrawal syndrome in the next years.

[Comparison of radiology and 24-hour esophageal pH-monitoring in the diagnosis of gastro-esophageal reflux in various pediatric age groups]. / Confronto tra radiologia e pH-metria esofagea di 24 ore nella diagnosi della malattia da reflusso gastro-esofageo in diverse età pediatriche.

We retrospectively examined the 24 hour oesophageal pH study and the upper gastrointestinal (UGI) series of 67 children aged 5 weeks to 15 years. Thirty-seven were suffering from gastro-oesophageal reflux (GER) and 27 from other diseases. Nineteen of them were younger than 6 months, 22 older than 18 months and the remaining 26 ranged from 6 to 18 month old. We found a high incidence of false positive and false negative results of the UGI series in all age groups. The number of false positive results was much higher in infants younger than 6 months (67%) than in the older children studied (27% between 6 and 18 months and 14% after 18 months). On the contrary, false negative results were absent in the first 6 months of life, but their number increased with age, up to 40% in children older than 18 months. Moreover, the 24 hour ph monitoring was found to have a high sensitivity (100%) and specificity (82-100%) in all age groups. For this reason, we believe than this examination could be used as the first line of investigation for the diagnosis of GER in all children, independently of their age.

[Gastroesophageal reflux and infantile cerebral palsy: our experience and review of the literature]. / Reflusso gastro-esofageo e paralisi cerebrale infantile: nostra esperienza e revisione della letteratura.

22 infant and children, all tetraplegic from cerebral palsy were admitted to our hospital for suspected gastroesophageal reflux. This working diagnosis was confirmed in 17 of them (77%) by an upper GI series and/or 24 hour oesophageal pH monitoring. All 17 were treated with medical therapy. In only 12.5% of them gastroesophageal symptoms improved. Seven children underwent surgery with complete resolution of vomiting in 57% of cases. These data confirm the high frequency of gastroesophageal reflux in children with cerebral palsy and its poor response to medical therapy. Most of these patients require surgical treatment, which unfortunately does not always resolve this vexing problem.

Chlorpromazine disposition in relation to age in children.

The pharmacokinetics of chlorpromazine after intravenous infusion were studied in 25 children. The pharmacokinetic parameters studied are markedly different from those reported for adults. A clear relationship was demonstrated between age, serum terminal half-life (r = 0.75) and systemic clearance (r = -0.43). It appears that the pharmacokinetics of chlorpromazine are more rapid in children than in adults.

[Clinical experience with a hydrolyzed soy formula in infants with protracted enteritis and atopic eczema]. / Esperienza clinica con una formula a base di idrolisato di soia in lattanti con enterite protratta e con eczema atopico.

A lactose-free soy and beef hydrolysate based formula ("Pregomin" Milupa) was studied in 12 infants affected with protracted enteritis and in 10 infants affected with atopic eczema. After 1 month of treatment with "Pregomin" an oral provocation test was performed using intact soy protein. The aim of this latter test was to evaluate if the hydrolysed soy formula could cause allergic sensitization to soy protein. We obtained the following results in the group of infants with enteritis: good catch-up growth, malnutrition laboratory test improvement, negative allergic tests, positive oral provocation test in only one infant who had been previously fed with intact soy protein. In the group with atopic eczema we found an improvement of the eczema, negative allergic tests, 3 out of 9 positive oral provocation tests (in infants previously fed with soy protein based formulas). Our data show that "Pregomin" formula was effective both from a gastroenterological point of view and from an allergological one. Furthermore we did not observe any positive oral provocation test with soy protein in infants who had never been exposed to this type of protein. The latter result seems to demonstrate that "Pregomin" formula does not possess any allergenic properties.

Diagnostic accuracy of pH monitoring in gastro-oesophageal reflux.

One hundred and eleven children admitted with suspected gastro-oesophageal reflux were studied, with 24 hour oesophageal pH monitoring as the first line of investigation. Barium swallow examination, or oesophagoscopy, or both, were carried out only in children with abnormal pH, who subsequently had a trial of 1-12 months medical treatment. All patients were followed up for eight months to two years. A final diagnosis of gastro-oesophageal reflux was made in 41 patients, in all of whom the pH study was abnormal (100% sensitivity). The final diagnosis was different in 70 patients; 66 of these had a normal pH (94% specificity). All children with gastro-oesophageal reflux were treated with drugs. All those with a percentage reflux time of more than 27 and more than 20 episodes of reflux lasting more than 5 minutes failed to improve and needed operation. We conclude that monitoring of the oesophageal pH should be the first line of investigation in patients with gastro-oesophageal reflux and should be used together with clinical data and other investigations, to identify those children who will need operation.

Recurrent abdominal pain in children: a long term follow-up.

A long-term follow-up study (minimum of 10 years) of 16 children admitted for recurrent abdominal pain revealed that in 50% of the sample the disturbance disappeared completely, persisted in 25% and in the remaining 25% other painful symptoms developed. The variables that characterized the poor outcome group were: belonging to a "painful family", many surgical procedures, low educational level and social class and a low score in the Q3 factor of the Sixteen Personality Factor Questionnaire (spare capacity to control emotions).

Carnitine and the premature.

After birth, the main energy fuel for the newborn is constituted by fat. Carnitine is necessary for the beta-oxidation of long chain fatty acids at the mitochondrial level, and seems also to have a role in the metabolism of the branched-chain amino acids, in ammonia detoxification, and in urea production. Colostrum is particularly rich in carnitine whereas semi-elemental formulae and soy-based formulae contain little or no carnitine. Since the newborn has a low capacity for carnitine biosynthesis, it seems useful to administer L-carnitine to infants on total parenteral nutrition, soy-based or semi-elemental formulae.

Effect of carnitine on lipid metabolism in the neonate. II. Carnitine addition to lipid infusion during prolonged total parenteral nutrition.

The effect of carnitine administration on lipid metabolism and carnitine and acylcarnitine plasma values of newborn infants, given total parenteral nutrition for the first 7 days of life, was studied during a 4-hour infusion of Intralipid. An increase in plasma concentrations of total carnitine, free carnitine, and short-chain and long-chain acylcarnitine was found, but no significant change in triglycerides, free fatty acids, glycerol, or beta-hydroxybutyrate plasma values was noted, as compared with values obtained without carnitine administration. Moreover, the low free carnitine and short-chain and long-chain acylcarnitine plasma levels found in newborn infants after 7 days of total parenteral nutrition did not seem to impair the utilization of infused lipids. The results support the concept that the relation between the carnitine pool and lipid metabolism can be influenced by intravenous glucose infusion. Low carnitine plasma concentrations do not necessarily signify a depletion of body carnitine, and sufficient tissue carnitine concentrations can probably maintain good lipid utilization for an extended period.

Effect of carnitine on lipid metabolism in the newborn. I. Carnitine supplementation during total parenteral nutrition in the first 48 hours of life.

The effect of carnitine administration on neonatal lipid metabolism was studied during endovenous loading with Intralipid (1 g/kg body weight over a 4-hour period). During a 6-hour period the plasma level of triglycerides, glycerol, free fatty acids (FFA), beta-hydroxybutyrate (beta-OHB), and acetoacetate were monitored in a group of newborns infused with carnitine and compared with a control group infused only with Intralipid. Carnitine administration caused an increased plasma concentration of ketone bodies, probably consequent to an increased rate of FFA mitochondrial beta-oxidation. An increased plasma level of glycerol and FFA was also observed, whereas the triglyceride plasma levels were not different between the two groups. Carnitine administration in the neonatal period seems to act by increasing ketogenesis and lipolysis.

Congenital hypoplastic anaemia developed in acute megakarioblastic leukaemia. A case report.

A 14-month-old child with congenital hypoplastic anemia, in temporary remission after steroid therapy, developed an acute megakarioblastic leukemia. After complete remission due to immunosuppressive treatment, the leukemia rapidly relapsed, and the child died at 23 months of age. The rapid development of leukemia puts some doubt on the initial diagnosis.

Plasma and urine carnitine levels during development.

Plasma and urine free carnitine concentration during human development was evaluated. A positive correlation was found between plasma level and body weight in premature neonates weighting between 1.15 and 1.80 kg. In full terms newborns, the free carnitine concentration (mean +/- S.E., 31.2 +/- 2.5 nmoles/ml) is similar to that of premature babies with 33 to 36 wk of gestational age (37.5 +/- 3.1 nmoles/ml) but significantly lower than that of premature babies aged 30 to 33 wk of gestation (43.0 +/- 5.6 nmoles/ml). In the first 2 wk of life, free carnitine level showed a good correlation with age in the full-term newborns. Adult levels are reached by the end of the first 6 months. no sex related difference was observed in any of the different groups during growth. Urinary excretion of free carnitine per day is significantly lower in infants 0 to 3 years old (mean +/- S.E., 15.5 +/- 1.8 mumoles/24 hr) and in children 3 to 10 years old (115.3 +/- 11.4 mumoles/24 hr) than in subjects ranging in age from 22 to 70 years (216 +/- 20.6 mumoles/24 hr). We found no difference between day- and nighttime urinary excretion in newborns and infants.

Primary intestinal lymphangiectasia. Comparison between endoscopic and radiological findings.

Two children with primary intestinal lymphangiectasia (PIL) are presented, both of whom had been prescribed an MCT supplemented diet low in long chain fatty acids. The hypoproteinemic edema improved in one patient, but persisted in the other. Following several years of dietary therapy, both subjects were re-evaluated, and a duodenoscopy and radiography of the digestive tract were performed. The duodenoscopic procedure evidenced two types of lymphangiectatic plaques on the surface of the duodenal mucosa: one form had a diameter of less than 1 mm, while the other exceeded 3 mm. The smaller lesions were seen in the child with the more favorable clinical course, whereas both types were observed in the other patient. Radiological examination disclosed the typical anomalies of intestinal lymphangiectasia only in the subject who had not responded to the dietary regimen. These results suggest that endoscopy affords a more precise assessment of the anatomic injury and could play an important role in formulating an accurate clinical diagnosis.

[Evaluation of 2 analytical technics for determination of fecal lipids]. / Valutazione di due tecniche analitiche per la determinazione dei lipidi fecali.

The van de Kamer titrimetric method for the determination of fecal fatty acids has been compared with the colorimetric technique described by TOMASZEWSKI. Reproducibility and accuracy as well as the applicability in a clinical laboratory service are discussed. The AA. point out that van de Kamer method still remains the method of choice for diagnostic purposes.

Effect of phototherapy on hepatic excretory function in newborns as measured by bromsulphalein clearance.

BSP clearance from the plasma was studied during phototherapy in a group of jaundice newborns. This therapy did not have any influence on the plasma dye disappearance c curve. The obtained results are consistent with the hypothesis that this therapy causes the photodegradation of bilirubin, and that the increased excretion of unconjugated bilirubin is not mediated by a generalized enhancement of the hepatic output.