Carnitine deficiency of skeletal muscle: report of a treated case.

We studied a 10-year-old girl with an insidious muscle disease beginning at age 7. Muscle biopsy showed that the majority of type I fibers were vacuolated and contained lipid excess. Carnitine deficiency was found in skeletal muscle. The patient was treated with 3.0 gm L-carnitine per day and with a medium-chain triglyceride diet. She showed a rapid improvement and recovery of strength. A muscle biopsy 8 months later showed a decreased lipid content. Oral carnitine replacement represents an effective treatment for the disease.

Effect of infused hypothalamic phospholipids on the maturation of foetal rabbit lungs.

The effect of hypothalamic phospholipids on the maturation of foetal lung was investigated in a series of experiments in rabbits. Sixteen pregnant does were injected with hypothalamic phospholipids (2 mg day-1 kg-1) from day 21 of gestation. A second group of 16 does was injected with saline, and served as controls. The foetuses were delivered at gestational ages between 27 and 28 days by means of caesarean section under intravenous pentothal anaesthesia. Foetuses born from treated mothers were more active, breathed better, and their lungs expanded to a greater extent compared with the foetuses born to control mothers. In the animals of treated mothers there was, on electron microscopy, evidence of accelerated maturation of alveolar epithelial cells.

Continuous peritoneal dialysis in newborns.

OBJECTIVES: To report the complications and outcome of 10 newborns affected by acute renal failure (ARF), treated by continuous peritoneal dialysis (CPD). DESIGN: All newborns admitted for tertiary treatment to the Neonatal Intensive Care Unit of the University of Padova, who underwent CPD between February 1986 and December 1990, were analyzed retrospectively. PATIENTS: Ten newborns (mean weight 2077 g, range 540-4930 g) received CPD, 6 of whom were preterm. All the survivors completed the study. INTERVENTIONS: A number 9, 5 French Tenckhoff catheter was used, and a closed circuit was created by means of a modified continuous ambulatory peritoneal dialysis (CAPD) technique. The mean duration of dialytic therapy was 7 days. RESULTS: At the end of the dialytic period, 7 of the 10 patients had normal serum potassium and sodium values. CPD produced two different types of complications: leakage of the dialytic fluid in very low weight newborns and one episode of peritonitis during a chronic dialysis treatment. Six died of severe respiratory failure (in no case, however, was this attributable to ARF or CPD procedure). All but one of the survivors regained normal renal function. The only exception necessitated a kidney transplant. CONCLUSION: We believe that this technique, although invasive, improves the outcome of both preterm and low birth weight newborns affected by ARF.

[A case of neonatal necrotizing enterocolitis due to rotavirus]. / Un caso di enterocolite necrotizzante neonatale da rotavirus.

Recently the role of rotavirus in the etiopathogenesis of neonatal hemorrhagic gastroenteritis and necrotizing enterocolitis has been underlined. We report a case of NEC caused by rotavirus, that occurred in a term neonate born from a drug abusing mother, during an outbreak of rotavirus-associated which appeared with gastrointestinal bleeding and mild systemic signs in comparison with marked radiologic findings, rapidly evolved from the fourth day of life, after the normalization of abdominal roentgenogram.

[Treatment of necrotizing enteritis using peritoneal drainage. Presentation of a clinical case (author's transl)]. / Trattamento mediante drenaggi peritoneali dell'enterite necrotizzante. Presentazione di un caso clinico.

Necrotizing Enterocolitis (NEC) of the new-born is a serious syndrome characterized by bilious vomiting, gastric retention, abdominal distention and bloody stools. Furthermore, the general condition of the neonate is frequently compromised. The pathogenesis is multi-factorial; however, most authors state that the primary cause of this syndrome is due to ischemia of the intestinal wall. The most serious complication in babies with NEC is perforation of the necrotic bowel, a condition which must always be treated surgically. We present a case of NEC that has a particularly interesting clinical course and, as well, an interesting approach to treatment, which involved the placement of a peritoneal drain. This drain was subsequently utilized for peritoneal lavage once the diagnosis of NEC was confirmed, because we felt that the patient's general condition was so serious that he could not tolerate surgery at this time.

[Skin lesions caused by oxygen saturation monitor probe: pathogenetic considerations concerning 2 neonatal cases]. / Lesioni cutanee da saturimetro: considerazioni patogenetiche su due casi neonatali.

Two cases of skin injury by a pulse oximeter probe are reported. The Authors hypothesize the local pressure as pathogenetic mechanism of the skin lesion and emphasize the importance of a careful management of critically ill patients during non-invasive monitoring.

[Resuscitation in the delivery room: experience in 3 Veneto Centers]. / La rianimazione in sala parto: esperienza in 3 Centri del Veneto.

On the basis of the classical USA guidelines for neonatal resuscitation, we examined the personnel activity in delivery room in 3 Veneto's Centres of different level (level I, II, III). Totally, we studied 3492 neonates in the period January, 1-December, 31, 1994. Three hundred and seven (8.7%) of them needed resuscitation at birth; respectively 5.6%, 4.6% and 14.2% in the level I, II and III Centres. The management of the first neonatal resuscitation's step was similar in the 3 studied Hospitals, while the second phases (ventilation) was different among the Centres. In fact, in the level I and II Hospitals the most part of the neonates were treated by ventilation bag (81.8% and 74.6%, respectively), while only a little part of them received endotracheal intubation (18.2% and 25.4%, respectively). In the level III Centre, endotracheal intubation (87.4%) was more frequently used respect to ventilation bag (12.6%). The third phases, chest compressions, was performed in many resuscitated infants in the level I (54.5%) and II (22.8%) Hospitals, while no infant needed it in the 3th Centre. The last step, drug and fluid administration, interested few patients in every Centre. Furthermore, the physicians of the 3 examined Institutions followed no protocol for neonatal resuscitation. The differences in neonatal resuscitation policy among the 3 studied Centres demonstrate the absence of a protocol and an educational program for the personnel. Theoretical and practical guidelines for correct neonatal resuscitation have to be implemented in our Region.

Prenatal theophylline and necrotizing enterocolitis in premature newborn infants.

To determine whether prenatal theophylline therapy would increase the incidence of neonatal necrotizing enterocolitis (NEC) we studied bowel dysfunction in 59 consecutive premature infants (g.s. < 34 weeks), whose mothers were treated with theophylline as a tocolytic during the last trimester, or as surfactant synthesis inductor, for at least three days prior to premature labor (Group A). As case-control we considered the premature, matched for gestational age born immediately before, and whose was untreated with theophylline (Group B). NEC occurred in one patient from group A during the second postnatal week, and surgery was performed. First passage of meconium and start of enteral feeding were comparable in groups A and B, while gastric residuals lasting more than 4 days were found statistically increased (p < 0.03) in antenatally treated group A prematures. Furthermore, 18 out of 49 prematures postnatally treated with theophylline had gastric residuals (36%) with respect to 5 out of 69 untreated (7%) (p < 0.001). Also the premature infants treated ante and postnatally with theophylline showed a statistically significant increase of lasting gastric residuals with respect to the untreated, 13/16 vs 5/7, respectively (p < 0.03). Antenatal theophylline administered to high risk mothers, when maternal diseases do not allow the use of steroids, does not appear to later increase the risk of NEC in premature infants, and provides a chance to avoid the risks related to premature birth. Inhibitory activity on gut motility and gastric irritability are only detectable during the first postnatal days, enhanced by gut immaturity of preterm infants.

[The micro-ESR with the capillary tube inclined to 45 degrees in the "sepsis screen" of neonatal infection due to beta-hemolytic B-group Streptococcus]. / MicroVES con capillare inclinato a 45 gradi nello "sepsis screen" dell'infezione neonatale da streptococco beta emolitico gruppo B.

The authors describe the advantages of a new method to determinate the erythrocyte sedimentation rate (micro-ESR) during neonatal GBS infections. They utilize a capillary tube placed at a 45 degrees angle and have the results of this test only after 15 minutes. The micro-ESR is proposed as a simple and quick method of sepsis screen in term and preterm newborns.

[Follow-up of the preterm child at 1 year of correct age: assessment of development risk]. / Follow-up del bambino nato pretermine a 1 anno di età corretta: valutazione del rischio di sviluppo.

Longitudinal follow-up of preterm neonates after discharge from Intensive Care satisfies the family's need for support and ensures an ongoing link with the hospital concerned, but must be based on a specific, sensitive and predictive screening program. We report a prospective cohort study in a tertiary neonatal intensive care unit (NICU) using the Perinatal Risk Inventory (PERI)1, at discharge. Of 87 consecutive newborn referred to the tertiary NICU at the Pediatrics Department of Padua University in 1993 (gestational age > or = 25 weeks, weight at birth < 1500 g), 65 (75%) survived: 54 cases complied with the Fitzhardinge '80 criteria and were followed up to at least 1 year (corrected age). A further 18 neonates with neonatal cerebral problems were also considered. The PERI at discharge was combined with neonatological and neuro-evolutional evaluation and examination of neuro-sensorial development indicators at the corrected ages of 0, 3, 6 and 12 months. PERI scores were: < or = 7 in 36; 8-11 in 22; > 11 in 14. The cut off point at 7 to 11 increases the sensitivity, the specificity and the predictive values of the test. At 1 year (corrected age), 6 patients (8.3%) had a diagnosis of spastic diplegia and 2 (2.7%) had spastic hemiplegia. The relative risk of cerebral paralysis was 4.5 in the neonates with IVH 4 degrees, odds ratio 6.7, and 3.6 in the 16 patients with bronchopulmonary dysplasia (BPD), odds ratio 4.7. 72 patients were selected for follow-up by the Fitzhardinge '80 criteria, whereas with the PERI (cut off point 7) only 44 neonates would have been selected. This confirms the need for new criteria in recruiting preterm neonates for longitudinal follow-up to quantify neurological risks, particularly in view of the socioeconomic impact of the problem.

[Clinical aspects and diagnosis of neonatal infections caused by group B beta-hemolytic Streptococcus]. / Clinica e diagnosi dell'infezione neonatale da streptococco beta-emolitico di gruppo B.

Early-onset infection findings caused by Group B Streptococcus occur within 24 hours of birth (60 per cent of cases) but they may appear anytime during the first 5 days of life. In our experience early-onset infection affects both preterm and term neonates. The Authors report the usual clinical signs described for bacterial infections. Unusual findings are also reported: among 34 infants with early-onset infection, the congenital diaphragmatic hernia was associated with GBS septicemia in two neonates; beads of perspiration were the first only clinical finding in one neonate too. Two cases of late-onset infection are also reported.

Spontaneous intraventricular haemorrhage in utero.

The authors report three cases of unexplained prenatal intraventricular haemorrhage (IVH) in three term infants. In the first two cases the suspected diagnosis of prenatal IVH was made a few hours after delivery, in accordance with the ultrasonographic feature of clots in the ventricles, whereas in the third case prenatal ultrasonography was suggestive of hydrocephalus with intraventricular clots.

Postnatal changes in blood S-adenosylmethionine concentration. Some observations in hyaline membrane disease.

The blood concentration of S-adenosylmethionine in 18 premature neonates affected by hyaline membrane disease did not differ from that found in a control group of healthy premature newborns. However, these values are higher than those found in full-term infants: this fact suggests that the methionine cycle is active in both the fetus and premature neonate with consequent conservation of homocysteine sulfur. These data provide ancillary evidence in support of the hypothesis that in premature babies cyst(e)ine is an essential amino acid. The concentration of S-adenosylmethionine reaches its lowest level 30 days after birth and then stabilizes at the age of 2--5 months with blood levels similar to those of the normal adult.

Oxygen saturation in premature neonates with bronchopulmonary dysplasia in a hammock.

Premature neonates with bronchopulmonary dysplasia (BPD) frequently present borderline hypoxemia and the risk for oxygen desaturation may increase in relation to the posture. Our aim was to see if infants with BPD experience severe hypoxemia (SaO2 < 85%) in a hammock, a 'containing' posture considered advantageous to neuromotor and relational development of the preterm. Fifteen pulse oximetry recordings (Ohmeda B105 3760 Pulse Oximeter) were obtained in 15 subjects (range of gestational age and postnatal age 27-30 and 33-48 weeks, respectively; range of birth weight and body weight at entrance to the study 0.64-1.35 and 0.97-2.24 kg, respectively) before, during and after placement in a hammock; each testing period lasted 15 min, and each baby served as his or her own control. BPD preterm infants were receiving oxygen therapy by continuous flow standard nasal cannulas (FiO2 > 25%, < 40%). The analysis of the data, that have a rough gaussian distribution, indicates a worsening of SaO2 in the hammock position. In fact, mean +/- SEM, median and range of the SaO2 values in pre- and posthammock position are comparable, but are significantly different at 99.9% confidence level (CL) in prehammock vs. hammock posture and at 98% CL in posthammock vs. hammock posture. Moreover, the percent of time with SaO2 < 85% during the periods recorded increased about 10 +/- 5% in a hammock (24 +/- 4%), in comparison to pre- (14 +/- 3%) and posthammock position (15 +/- 3%). These results suggest that oxygen-dependent BPD preterm infants in the hammock posture may experience severe hypoxemia that in part limits the possible advantages of the 'containment'.

Methylxanthines increase renal calcium excretion in preterm infants.

To determine the effect of a short course of methylxanthines on renal function and on urinary calcium excretion, 20 premature neonates affected by apnea or moderate respiratory distress syndrome were randomly assigned to either a theophylline treatment or to a caffeine treatment group. The protocol included a 24-hour pretreatment study period (I) and a subsequent 24-hour period (II) following 5 days of theophylline (loading dose 5 mg/kg i.v., maintenance dose 2.5 mg/kg/12 h) or caffeine (loading dose 10 mg/kg i.v., maintenance dose 2.5 mg/kg/12 h) administration. Pre- and postxanthine treatment serum sodium, potassium, calcium and phosphorus remained stable, while serum creatinine decreased significantly (p < 0.05). Furthermore, from period I to period II, sodium urine excretion, fractional Na excretion and creatinine clearance remained statistically comparable in both study groups, along with a significant increase (p < 0.05) in calciuria, urinary Ca/creatinine and urinary Ca/Na. Predose caffeine and theophylline serum levels, assessed on the 5th day of treatment, were 12.8 +/- 1.8 and 7.9 +/- 1.7 micrograms/ml, respectively. Compared to control healthy untreated prematures, the studied premature infants showed a statistically significant increase in urine calcium excretion (10- to 15-fold), which was more evident in the theophylline group. Our data suggest further investigation to determine the long-term renal effects of methylxanthines in premature neonates, to improve assessment of the risk of nephrocalcinosis and osteopenia, in particular in association with various diuretic therapies.

Early screening and treatment of "silent" patent ductus arteriosus in prematures with RDS.

From January 1987 to December 1989, prematures with RDS weighing 1750 g or less admitted to the Neonatal Intensive Care Unite (NICU) were submitted from the third postnatal day to serial two-dimensional and pulsed Doppler (ATL MK 600) echocardiographic evaluation for "silent" patent ductus arteriosus (PDA). PDA was diagnosed in 36/175 prematures with RDS (20.5%). Thirty patients had indomethacin treatment and the PDA closed completely in 27 (90%); five needed a second course of indomethacin, that was effective in two (40%). Four RDS patients (4/36-11%) already weaned from the respirator, needed supplemental oxygen. The three non-responders and six other prematures with counterindications to the drug underwent surgical ligation (25%). As historical control, we retrospectively evaluated the population of preterm infants with RDS weighing less than or equal to 1750 g treated for hemodynamically significant PDA during three previous years before the screening protocol; in this group the echocardiographic and Doppler evaluations were done when congestive heart failure and pulmonary edema were clinically evident. In comparison, we found a reduced incidence of prematures with RDS treated for PDA, 7/120 (5.8%) a higher age at medical treatment (9 +/- 1.4 vs 4.4 - 2.3 days) and a larger prevalence of patients underwent ductal ligation (71.4%). These data show that early screening and treatment of "silent" PDA may result advantageous to improve the efficacy (90%) of indomethacin, in a critical time for the recovery of RDS, and furthermore decreasing the need of surgical ligation.

Esophageal perforation in the newborn.

Two cases of esophageal perforation in the newborn are reported. The first case underwent surgery after suspected diagnosis of a traumatic perforation of a superior atresic esophageal stump. The second case was treated with supportive therapy. Both cases were cured and discharged in good health. The possible mechanisms of spontaneous and iatrogenic perforation are discussed.

Effect of high doses of human recombinant erythropoietin on the need for blood transfusions in preterm infants.

To determine whether prophylactic treatment with recombinant human erythropoietin (rHuEPO) and iron would reduce the need for blood transfusions, we randomly assigned 22 premature infants with gestational ages less than or equal to 32 weeks and birth weights less than or equal to 1.75 kg to receive rHuEPO, 400 IU/kg three times a week, plus iron, 20 mg/wk intravenously, from the second day of life (11 infants), or no rHuEPO and no iron (11 infants). The two groups had similar birth weights and clinical variables. The treated infants required fewer blood transfusions (0.8 +/- 1.5 vs 3.1 +/- 2.1; p = 0.01) and less volume of packed erythrocytes (14.2 +/- 25.9 vs 48.4 +/- 34.0 ml/kg; p = 0.02). The amounts of blood sampled were not different (19.5 +/- 21.1 vs 27.8 +/- 19.1 ml/kg; p = 0.35). Reticulocyte and hematocrit values were higher in the treated group (4.46% +/- 0.8% vs 1.49% +/- 1.1% (p = 0.0001) and 48.1% +/- 7.3% vs 43.8% +/- 4.7% (p = 0.004), respectively). No side effects of either rHuEPO or intravenously administered iron were noted. These data indicate that rHuEPO, in combination with iron supplementation, is effective in reducing the need for blood transfusions in the premature infant. More information is needed on dosage, timing, and iron and vitamin supplementation.