Cebocephalus associated with trisomy 13-15 mosaicism.

A case of cebocephalus occurred in association with a 10% mosaicism of trisomy 13-15. Both parents of the affected child had normal karyotypes. Cebocephalus has been associated with abnormal chromosomes such as a deletion of the short arm of chromosome 18 as well as the trisomy 13-15.

Should fewer premature infants be screened for retinopathy of prematurity in the managed care era?

OBJECTIVE: To determine appropriate upper limits for gestational age and birth weight when screening infants for retinopathy of prematurity (ROP). DESIGN: Retrospective survey. SETTING: Tertiary neonatal intensive care nursery. PATIENTS: Seven hundred seven infants born July 1, 1990 to June 30, 1996 and screened for ROP according to the 1988 to 1996 American Academy of Pediatrics guidelines. OUTCOME MEASURES: Maximum stage of ROP with respect to birth weight and gestational age. RESULTS: No ROP more than Stage 1 was observed in infants with gestational ages >/=32 weeks or birth weights >/=1500 g. All cases of threshold and Stage 4 ROP were confined to infants with gestational ages 1500 g or gestational ages >28 weeks. If ROP screening is limited to infants with birth weights of

New postnatal growth grids for very low birth weight infants.

OBJECTIVE: To generate contemporary postnatal growth curves for hospitalized very low birth weight infants. DESIGN: Retrospective survey. SETTING: Tertiary intensive care nursery. PATIENTS: All surviving singleton, appropriate-for-gestational age infants with birth weight < or = 1500 g, born January 1, 1987, to May 31, 1991, who did not develop necrotizing enterocolitis (N = 205). MEASUREMENTS AND RESULTS: Macronutrient intakes and body weights were recorded daily, with crown-heel length and occipital-frontal head circumference recorded weekly up to 105 days of age or hospital discharge, whichever occurred first. Growth curves were generated for four birth weight ranges: 501 through 750, 751 through 1000, 1001 through 1250, and 1251 through 1500 g. Compared to previously published growth curves, the current infants regained birth weight more quickly and exhibited larger average daily weight gains. These differences were most apparent in infants of lowest birth weight. CONCLUSIONS: The "premature growth grid" constructed by Dancis et al more than 40 years ago may no longer be a useful standard of early postnatal growth for present-day very low birth weight, appropriate-for-gestational-age infants. The new weight curves are a more accurate reflection of current in-hospital growth trends, especially for infants weighing < or = 1000 g at birth.

Unusual syndrome among premature infants: association with a new intravenous vitamin E product.

Eight small, premature infants developed an unusual symptom complex of pulmonary deterioration, thrombocytopenia, liver failure, ascites, and renal failure. Five infants died; the health of the other three infants improved and they were discharged from the hospital. This unusual syndrome occurred after introduction of a new intravenous vitamin E product (E-Ferol, alpha-tocopherol acetate) for routine use in the intensive care nursery. Even though no definite conclusion was reached as to its cause, the administration of this intravenous vitamin E product appears to be a significant risk factor.

Release of benzalkonium chloride from a heparin-bonded umbilical catheter with resultant factitious hypernatremia and hyperkalemia.

Elevated serum sodium and potassium levels were recently observed when sampled through a heparin-bonded umbilical catheter and measured with certain ion-selective electrodes. The cationic surfactant, benzalkonium chloride (BZC), is known to falsely elevate those cations in serum. Inasmuch as most heparin-bonded umbilical catheters use BZC during the bonding process, an in vitro study was performed to estimate the quantity of BZC released and the duration of sodium and potassium elevations during pooled sera infusion. Three heparin-bonded umbilical catheters and 3 silastic umbilical catheters were first flushed with 0.3 mL of normal saline and then perfused with pooled sera (sodium, 142 mEq/L; potassium, 4.6 mEq/L) at 2.5 microL/h. Effluent samples were collected from 0 to 8 hours and analyzed by ion-selective electrodes. Elevated serum sodium concentrations from 160 to greater than or equal to 250 mEq/L and potassium concentrations from 6.0 to greater than or equal to 9.6 mEq/L were observed. The BZC concentration in the catheter effluent was measured by mass spectrometry, with peak values of 10 micrograms/mL detected by this method. When varying concentrations of BZC (1 to 100 micrograms/mL) were added directly to pooled serum, a dose-dependent increase in serum sodium was observed. These data demonstrate that BZC released from heparin-bonded umbilical catheters elevates serum electrolyte values measured with some ion-selective electrodes. The observed increase in sodium and potassium concentrations may lead to clinical errors in management. Benzalkonium chloride is associated with myriad of linical symptomatology. Whether this amount of BZC is toxic in the small premature neonate is presently unknown.

Illness with fatalities in premature infants: association with an intravenous vitamin E preparation, E-Ferol.

Three clusters of an unusual syndrome in premature infants were investigated in three intensive care nurseries in 1984. A retrospective cohort study of 68 infants weighing less than or equal to 1,250 g at birth and surviving at least 72 hours revealed that in 13 infants ascites developed and in four at least two of the following abnormal laboratory values were found within a seven-day period: serum direct bilirubin greater than or equal to 2 mg/dL, blood urea nitrogen greater than or equal to 40 mg/dL or serum creatinine greater than or equal to 2 mg/dL, and platelet count less than or equal to 60,000/microL. All cases occurred after the introduction and use of intravenous E-Ferol, a vitamin E preparation that was new on the market when the clusters were reported. All 17 case infants but only 23 of 51 (45%) noncase infants received E-Ferol (P less than .0001). Case and noncase infants were similar with respect to other complications and to receipt of medications and parenteral nutrition. A dose-response relationship was found; cases occurred in infants receiving E-Ferol dosages of greater than 20 U/kg/d. Case infants who had higher daily doses of E-Ferol had a shorter latency. No new cases were reported after use of E-Ferol was stopped. Results of these investigations led to a nationwide recall of intravenous E-Ferol.

Intraventricular haemorrhage in preterm infants: evidence of suppressed fibrinolysis.

Intraventricular hemorrhage (IVH) in premature infants may be related to the immaturity of the vascular bed in the germinal matrix. We measured six hemostatic parameters whose alterations may represent an additional risk factor for IVH in preterm infants. On postnatal day 1 there were differences between plasminogen activator inhibitor-1 (PAI-1) activity and antigen, of both full-term and preterm infants with and without IVH (P < 0.05). Preterms with IVH were different to both full-terms and preterms without IVH. No difference was observed in plasma concentrations of fibrinogen, plasminogen and von Willebrand factor. Plasma concentrations of antithrombin III were significantly higher in full-term infants than in preterm infants. The difference between the platelet counts of preterm infants with and without IVH was not significant (P > 0.05). Elevation of crosslinked fibrin degradation products (XDP), determined by the SimpliRED D-dimer test, correlated in four out of five premature infants with the diagnosis of IVH by ultrasonography. No elevation of D-dimer XDP was observed in premature infants without IVH (11/12) and full-term infants (6/6). In conclusion, a hypercoagulable state, indicated by a rise in D-dimer XDP, may be initiated by some types of trauma to fragile blood vessels of the preterm infants who develop IVH. This hypercoagulability is further exacerbated by the increased release of PAI-1 leading to suppressed fibrinolysis.

Plasma carnitine alterations in premature infants receiving various nutritional regimes.

Plasma carnitine, carnitine esters, and triglyceride concentrations were determined in 36 appropriate-forgestational-age (AGA) infants at various stages of prematurity throughout hospitalization to determine the effect of a carnitine-free and carnitine-containing diet on plasma carnitine and triglyceride concentrations. The infants were entered into one of three experimental groups based on birth weight: group I less than 1.0 kg; group II 1.0-1.51 kg; and group III 1.52-2.5 kg. Throughout the study subjects were placed on appropriate nutritional regimes which included hyperalimentation (HA), intravenous (iv) fat emulsion (Intralipid), Portagen, Enfamil-24 Premature Formula, Enfamil-20, and breastmilk. Blood samples were drawn from each infant at birth, days 1-5,7 then weekly, also before and after each nutritional intervention to determine carnitine and triglyceride concentrations. Results showed that plasma total carnitine and nonesterified carnitine decreased in all groups when the infants were maintained on a carnitine-free diet (HA, Intralipid, Portagen). In general, the carnitine levels continued to decrease until a carnitine-containing diet was initiated. Once a carnitine-containing diet was begun, plasma total carnitine (TC) and nonesterified carnitine (NEC) levels increased at fairly similar rates in all groups. However, an inverse relationship between carnitine and triglyceride (TG) concentrations were not seen in these infants. This would indicate that most premature infants require exogenous carnitine to maintain the plasma concentration of carnitine. However, a decreased concentration of plasma carnitine was not correlated with an elevated TG level under the conditions of this study.

Altered platelet-activating factor levels and acetylhydrolase activities are associated with increasing severity of bronchopulmonary dysplasia.

Lipid inflammatory mediators are thought to play an important role in the pathogenesis of neonatal lung injury and bronchopulmonary dysplasia (BPD). Because preliminary studies from the intensive care nursery of the University of Tennessee Medical Center, Knoxville, revealed linear increased in blood platelet-activating factor (PAF) levels in very low birthweight infants developing chronic lung disease and lower cord blood PAF acetylhydrolase activities in premature infants, it was theorized that altered platelet-activating factor levels and PAF acetylhydrolase activities are associated with increasing severity of BPD. Platelet-activating factor levels (blood and tracheal lavage) and PAF acetylhydrolase activities (blood and tracheal lavage) were measured over days 1 to 2, 3 to 5 and 6 to 7 in 16 ventilated infants and weekly in 9 infants with bronchopulmonary dysplasia. Platelet-activating factor values were normalized per nanogram of lavage blood urea nitrogen. Severity of bronchopulmonary dysplasia was estimated using the scoring system developed by Toce. Mean blood and lavage PAF levels and PAF acetylhydrolase activities were compared in infants developing bronchopulmonary dysplasia with those without the disease over the first seven days of life. Infants developing chronic lung disease were significantly smaller and of younger gestational age. In infants with bronchopulmonary dysplasia, higher PAF levels in blood were seen on days 3 to 5, along with increased lavage acetylhydrolase activities on days 1 to 2. Increased levels of PAF in lavage on days 3 to 5 were associated with increasing severity of bronchopulmonary dysplasia. Altered blood and lavage platelet-activating factor levels and PAF acetylhydrolase activities appear to be associated with the pathogenesis and severity of bronchopulmonary dysplasia.

Improved fluid management utilizing humidified incubators in extremely low birth weight infants.

OBJECTIVE: To compare fluid and electrolyte management in extremely low birth weight (ELBW) infants nursed in humidified versus nonhumidified incubators. STUDY DESIGN: Setting--tertiary intensive care nursery. Subjects--all infants with birth weight < 1000 g admitted 1/95 to 1/99 who were treated with incubators and survived for > 96 hours (N = 155). Intervention--retrospective comparison of daily weights, fluid intakes, urine outputs, and serum electrolytes between group 1 (n = 70, nonhumidified incubators, born 1/95 to 1/97) and group 2 (n = 85, humidified incubators, born 1/97 to 1/99) over the first 4 days after birth. RESULTS: Despite similar daily weight losses between groups, group 1 infants received higher fluid intakes, had lower urine outputs, and had a higher incidence of hypernatremia, hyperkalemia, and azotemia (p < 0.05). Although no differences in mortality or the incidence of patent ductus arteriosus, bronchopulmonary dysplasia, or the overall rate of nosocomial infections were observed, the proportion of gram-negative isolates increased significantly (62%, p < 0.05) following the introduction of humidified incubators. CONCLUSIONS: ELBW weight infants nursed in humidified incubators have lower fluid requirements, improved electrolyte balance, and higher urine outputs during the first 4 days after birth compared to those nursed in nonhumidified incubators.

Congenital mesoblastic nephroma.

Neonatal renal neoplasms are rare: most are congenital mesoblastic nephroma. In the past this neoplasm has been confused with Wilms' tumor, which in part may account for some of the more favorable prognosis ascribed to Wilms' tumor in children less than 1 yr of age. Congenital mesoblastic nephroma is usually a benign tumor. The recommended treatment is surgical excision. This is a report of two cases of congenital mesoblastic nephroma in infants who were treated successfully by nephrectomy.

[Studies on the incidence and quality of root fillings. Evaluation by orthopantomograms]. / Untersuchung zur Häufigkeit und qualität von Wurzelfüllungen. Eine Auswertung von Orthopantomogrammen.

Incidence, distribution and quality of endodontic treatment were investigated using panoramic radiographs of 200 patients of the Department of Periodontology. The results were compared to those of similar epidemiological investigations published in the recent literature. 3.2% of all teeth showed to be root-canal-fillings, which is less than in any other investigation. 87% of all root-canal-fillings ended more than 2 mm from the apex and more than 60% exhibited insufficient obturation. Periapical lesions were detected in 60% of all root-canal-fillings teeth.

[The orthodontic findings in patients with unilateral Angle Class II]. / Kieferorthopädische Befunde bei Patienten mit einseitiger Angle-Klasse II.

In this study the original orthodontic documentation of 23 patients (twelve girls and eleven boys, average age 12.7 years) with one side Angle class II subdivision were analyzed. Computer assisted measurements were obtained from dental models and from posteroanterior and lateral oblique cephalometric radiographs of each patient. This results of this research indicate that the asymmetric occlusion was caused by dental as well as by skeletal components. In addition to an asymmetric position of the upper first molars, a lateral position of the whole mandible was established.

Treatment of hyperkalemia with exchange transfusion.

Exchange transfusions using whole blood less than 24 hours old and deglycerolized red cells reconstituted with fresh-frozen plasma were performed in two premature infants for the treatment of hyperkalemia and cardiac arrhythmia. Improvement of their condition was immediate.

Responses of small intrapulmonary arteries to vasoactive compounds in the fetal and neonatal lamb: norepinephrine, epinephrine, serotonin, and potassium chloride.

Despite considerable study, the mechanisms responsible for the transition of the pulmonary circulation from the fetal to newborn life remain obscure. We compared the responses of third and fourth generation pulmonary arteries to norepinephrine, epinephrine, serotonin, and KCl from lambs 7 d preterm and 1, 7, and 21 d of age to assess differences between age and third and fourth generations of the pulmonary arterial tree during the transitional period. Preterm vessels were significantly smaller in internal diameter than all other aged vessels for both generations. Fourth generation vessel response to KCl increased with age (94 +/- 15 mg/mm2, preterm; 259 +/- 31 mg/mm2, 21 d). Third generation vessel response to KCl did not change with age (135 +/- 15 mg/mm2, preterm; 158 +/- 18 mg/mm2, 21 d). There were no differences in maximum response to norepinephrine and epinephrine between ages; however, third generation vessel response to these compounds was significantly greater (30-60% of maximum KCl response) than fourth generation vessel response (0-10%). Third and fourth generation vessels had the same maximum response to serotonin regardless of age or generation. Third generation vessels were significantly reduced in sensitivity (log molar concentration which produced 50% of the maximum response--EC50) to norepinephrine at d 1 (5.89 +/- 0.12 log molar) and 7 (5.90 +/- 0.21) compared to preterm (6.48 +/- 0.10) and 21 d of age (6.50 +/- .03).(ABSTRACT TRUNCATED AT 250 WORDS)

Maternal, cord, and neonatal carnitine correlations and lipid profiles of various birthweight infants.

The carnitine, acylcarnitine, and triglyceride concentrations were determined in the average for gestational age neonates of various birthweights and gestational ages and their corresponding maternal and umbilical cord plasma. Forty-two infants were divided into one of four groups based on birthweight: Group 1, less than 1000 gm; group II, 1001 to 1510 gm; group III, 1511 to 2500 gm; and group IV were term infants who served as the reference group. Results indicated that there was an overall decrease of total carnitine and nonesterified carnitine with advancing gestational age (r = -0.4418, p less than 0.01). Furthermore, there were three distinct phases of plasma carnitine concentrations from 24 weeks to term. The plasma carnitine profile of the neonates less than 1500 gm was strongly correlated (r = 0.73) to their maternal plasma carnitine profile and that of term neonate to their umbilical cord plasma. Acylcarnitine and triglycerides were not significantly correlated.

Periosteal reaction induced by prostaglandins.

Prostaglandin administration is the main therapy for ductal-dependent congenital heart disease prior to definitive therapy. We report a case in which tetralogy of Fallot and Noonan syndrome were diagnosed, complicated by small pulmonary arteries, which occasioned a delay in surgery. Treatment was started with prostaglandins, which were given for a total of 2 months, and a diffuse periosteal reaction occurred secondary to the administration of intravenous prostaglandin E1.

The use of primidone in neonates with theophylline-resistant apnea.

OBJECTIVE: To determine whether primidone reduced the occurrence of apnea of prematurity in neonates with apnea resistant to theophylline. DESIGN: Retrospective review. SETTING: Neonatal intensive care unit. PARTICIPANTS: Sixteen premature infants (mean age, 27.8 weeks) in whom apnea and bradycardia recurred despite therapeutic levels of theophylline. Six of the patients were receiving assisted ventilation. INTERVENTION: Administration of primidone (10 to 15 mg/kg per day) orally or by nasogastric tube at a mean age of 35 days. RESULTS: Apnea and bradycardia decreased significantly 24 to 72 hours after initiation of primidone treatment (by 68% and 69%, respectively) compared with pretreatment events. We obtained similar results after a separate analysis of the 10 patients who had been weaned from assisted ventilation before treatment with primidone. No toxic reactions were observed. CONCLUSIONS: Primidone represents a possible adjuvant therapy in theophylline-resistant apnea of prematurity. Caution is advised, because of primidone's complex pharmacologic characteristics, until there are further controlled prospective studies.