[Costello syndrome associated to a neuroblastoma. Presentation of a case]. / Síndrome de Costello asociado a neuroblastoma. Presentación de un caso.

We present the case of a newborn with Costello syndrome who died due to heart arrhythmia. In the autopsy, a neuroblastoma was found. The male patient was born at term. During the first hours of life, he developed severe respiratory failure requiring mechanical ventilation. Phenotypic features included cranial and facial dysmorphia, short thorax, tachycardia, heart murmur, abdominal distention, hepatomegaly, short extremities, widespread petechias, diminished muscular tone, ungueal hypoplasia in toes, bilateral cryptorchidia, and generalized redundant skin. In the evolution he presented several sepsis episodes, difficulty for feeding, supraventricular arrhythmia, two heart arrests, and opisthotonos, and died at 65 days of life due to heart arrhythmia. The autopsy revealed hydrocephaly, a neuroblastoma, and a heart without anatomic alterations. Costello syndrome was diagnosed. Costello syndrome is not frequent; in this patient, the diagnosis was suspected in life and was confirmed postmortem, the topic is reviewed, the important aspect in this case is the association with a neuroblastoma.

[Acute pancreatitis secondary to typhoid fever in a preschool child]. / Pancreatitis aguda secundaria a fiebre tifoidea en un preescolar.

INTRODUCTION: A case of child with acute pancreatitis associated with typhoid fever is reported herein. CASE REPORT: A four years old child was admitted to the pediatric emergency room with an acute abdomen and fever. He was submitted to a diagnostic laparotomy and mesenteric adenitis was the only finding. During the postoperative period, he persisted with abdominal pain and fever. Laboratory studies and abdominal ultrasonography revealed an acute pancreatitis. Also, Salmonella typhi was isolated from blood and stool cultures. Treatment for pancreatitis included nasogastric catheter and total parenteral nutrition. Typhoid fever was treated with ampicillin. Thereafter, his clinical course was uneventful and he was discharged 13 days after being admitted. CONCLUSIONS: Acute pancreatitis has been associated with typhoid fever. To the best of our knowledge, this is the youngest patient reported in the literature with this association. Possible pathophysiologic mechanisms are reviewed.

[Perinatal risk factors for mortality in the high-risk newborn]. / Factores de riesgo perinatal para mortalidad en el neonato de alto riesgo.

This study was to determine the risk factors for mortality in a cohort of neonates in a neonatal intensive care unit. Comparative study of the perinatal antecedents between lives and deaths, calculating risk factors for mortality by the chi square test, with Mantel and Haenzel corrected and bivariate analysis. We studied 425 neonates with, weight and age of gestation 1640 +/- 359 gr. and 32 +/- 2 weeks (X and SD), all required assisted mechanical ventilation during 10 +/- 12 days, and stayed in NICU 13 +/- 12 days. The risk factor for mortality were (p < 0.05); gesta IV, < 1 abortion previously, premature rupture of placenta, premature rupture of membranes with chorioamnionitis, pelvic delivery for vaginal via, age of gestation < 28 weeks, birth weight < 1,000 gr., septic shock, metabolic acidosis, acute renal failure, neonatal necrotizing enterocolitis and air leak. In this group of high risk neonates, for the prenatal, neonatal and postnatal antecedents, we find 12 risk factors for mortality; four attributed to the pregnancy, on to the delivery and seven for problems of the neonate.

[Evaluation of a newborn in a critical state, son of a mother with preeclampsia/eclampsia (pregnancy toxemia)]. / Evaluación del neonato en estado crítico, hijo de madre con preeclampsia/eclampsia (toxemia del embarazo).

The objective was to determine clinical characteristics and evolution of the neonate in critical status, son of mother with preeclampsia/eclampsia, in a neonatal intensive care unit (NICU). And looking for differences between the son of mother with preeclampsia and the mother with eclampsia. Revision of 425 charts of discharged neonates of a NICU. Infants of mother with preeclampsia/eclampsia were included. Was done the variables by means of descriptive statistics are analyzed, and t Student and chi square for the comparative study. 88 neonates were included, 46 of preeclampsia and 42 of eclampsia. Their characteristics were (X +/- SD): Maternal age 25 +/- 4 years, birth weight 1587 +/- 601 grams, gestational age 32 +/- 3 weeks. Apgar to the one minute 5 +/- 1. Apgar to the five minutes 6 +/- 1. All required mechanical ventilation during 8 +/- 9 days, NICU stay 12 +/- 10 days. Predominated masculine sex 63.2%, and with mortality 21.8% average. In the comparative study, preeclampsia vs eclampsia, of the same variables we found significant difference in; birth weight 1858 +/- 654 vs 1340 +/- 422 grams; gestational age 33 +/- 3 vs 31 +/- 2 weeks, and in nosocomial pneumonia 7 vs 33. Most of these neonates were preterm, with Apgar low, prevailed masculine sex, all required mechanic ventilation with stay of 12 days average in the NICU, mortality was low. And the son of mother with eclampsia presented more pneumonia nosocomial.

[Development of high risk infants following premature membrane rupture in a neonatal intensive care unit]. / Evolución del neonato de alto riesgo con antecedente de ruptura prematura de membranas en una unidad de cuidados intensivos neonatales.

The development of 77 neonates, with maternal premature membranes rupture in a neonatal intensive care unit was reviewed. Clinical characteristics, evolution, complications and mortality, as well as the risk factors for mortality were reviewed. The incidence was 25.8% in 298 neonates discharged, maternal age 25 + 6 years (X and standard deviation), number of gestations 2 + 1, maternal infection in 11 (14.2%), chorioamnioitis in 18 (23.3%), the ruptured membranes time was > 24 hours in 48%. Vaginal birth were 37.7%, and cesarean section 59.7%. Males predominated with 61%, gestational age 31 + 2 weeks, birth weight 1577 + 530. The three main morbidity causes were nosocomial pneumonia (44.1%), respiratory distress syndrome (39%), and intrauterine pneumonia (33.8%). By clinic and laboratory 3.8% neonates had septicemia, but only eight had some positive culture. Disease during pregnancy, vaginal birth, prematurity and neonatal sepsis were risk factors for mortality. Incidence of infants with PROM and neonatal sepsis is higher than majority of other reports. All premature neonates with PROM more; disease during pregnancy, vaginal birth, or neonatal sepsis, have high risk for mortality, then they need specially cares.

[Esophageal perforation in premature newborn infants. Presentation of 8 cases]. / Perforación de esófago en recién nacidos prematuros. Presentación de ocho casos.

We report eight newborn with esophageal perforation. They were preterm infants and received mechanical ventilation for respiratory problem. Six were associated with pneumothorax, three of they show aberrant way of an orogastric tube, and another three show milk fluid in the pleural tube installed for pneumothorax. The management was fasting during ten days, parenteral nutrition, wide spectrum antibiotics, and prevention of complications; surgery treatment is not the first of all recommended at present.

[Risk factors in neonatal necrotizing enterocolitis]. / Factores de riesgo en la enterocolitis necrosante neonatal.

In this case controls prospective study, risk factors for necrotizing enterocolitis from a neonatal intensive care unit, were investigated. In 34 cases and 69 controls, were found; the fetal distress, Apgar score < or = 5 at one minute, < or = 6 at five minutes, gestational age < or = 34 weeks and birth weight < or = 1500 g, like risk factors for necrotizing enterocolitis. The investigation of a new risk factor, abdominal distention with plentiful intestinal gas documented in X ray at initial hours of life, by energetic reanimation at birth, showed that it is not a risk factor.

[Multisystemic impact in the full-term newborn with perinatal asphyxia]. / Repercusión multisistémica en el recién nacido a término con asfixia perinatal.

We studied 30 full-term newborn infants, admitted to the newborn intensive care unit of "Manuel Gea González" General Hospital between January 1, 1989 and December 31, 1990. All infants had 5-minute Apgar score of 5 or less. The disturbances related to: brain, heart, liver, bowel, coagulation system, calcium and glucose homeostasis were investigate in all cases. Of the 30 infants, 29 (97%) had developed 1 to 7 abnormalities in the systems studied. Most of them had from 2 to 5 abnormalities (77%). The commonest disturbance was brain involvement in 22 cases (73%); metabolic and coagulation changes had also been observed, while heart disturbances were less common. There was no significant difference between the intensity and number of systemic abnormalities observed in these infants and the alterations seen during pregnancy, labor, delivery, administration of anesthesia and Apgar score. These observations suggest a striking relationship between asphyxia in the newborn infant and the development of systemic disturbances.

[Response to the treatment with indomethacin in persistent ductus arteriosus]. / Respuesta al tratamiento con indometacina en la persistencia del conducto arterioso.

The clinical charts of 46 newborn babies with persistent arterial duct, hospitalized in the Intensive Care Unit were reviewed. The diagnosis was made, on the average, after the fifth day of life, finding a precordial murmur in 75%, hyperdynamic precordium in 48%, and outstanding pulses in 22%. Radiologically, cardiomegaly was seen in 50% of the cases, an increase in the flow of the pulmonary artery in 41%, and no abnormalities 35%. Using echocardiography, the left atrium/aorta relation was found to be 1.4 on the average. Of the 25 patients who received indomethacin to close the conducts arteriosum, 21 (84%) responded favorably. The mortality rate was 43%. A comparative study was done between those children who were given the indomethacin and those who weren't, finding a significant difference in favor of those who did receive the medication when seeing the closure of the conduct and their survival rate.

[Transient myocardial ischemia in newborn babies with perinatal asphyxia (hypoxic cardiomyopathy)]. / Isquemia miocárdica transitoria en el recién nacido con asfixia perinatal (miocardiopatía hipóxica).

A prospective study in 76 newborn with perinatal asphyxia searching for myocardial ischemia was carried out. The disease was found in 51% of the patients. With electrocardiogram, myocardial enzymes, X ray and clinical manifestations the diagnosis was elaborated. No difference in the sex was present, the mean of gestational age was 35 weeks, and with mean birth weight 2,216 g, respiratory distress was present in all the people; only 20.5% developed heart failure and two had heart murmurs; 61.5% showed cardiomegaly. The creatine kinase MB isoenzyme at twelve hours after birth was raised in most of the patients. Respiratory distress syndrome was the principal diagnosis in 38%; hypoxic ischemic encephalopathy and peri-intraventricular hemorrhage was present in 50 and 33% of the patients, respectively. Mortality rate was 33%. Also a comparative study in the infants with and without myocardial ischemia was carried out appearing significative difference in: 1. Cardiomegaly, 2. Hypoxic-ischemic encephalopathy and 3. Creatine kinase MB isoenzyme.