Comparison of two neonatal intravenous amino acid formulations in preterm infants: a multicenter study.

Two neonatal intravenous amino acid solutions (Aminosyn-PF and Troph-Amine) were compared in 44 preterm infants. The rate of weight gain, nitrogen balance, and changes in plasma aminograms were determined over 7 days to ascertain whether different outcomes could be identified for the two solutions. At study entry, the infants received a minimum infusion of 2 g amino acid/kg/d with 50 or more nonprotein kcal/kg/d. Group mean amino acid intake over the study period was approximately 2.6 g/kg/d for both groups; nonprotein caloric intake approximated 90 kcal/kg/d. Results showed no significant differences between solutions for the rate of weight gain, nitrogen balance, and nitrogen retention, which approximated intrauterine rates. The rate of weight gain averaged nearly 15 g/kg/d for both solutions. Differences between day 0 and day 7 plasma aminograms showed significant changes between solutions for histidine, lysine, methionine, phenylalanine, threonine, and glutamic acid. However, day 7 plasma aminograms for both solutions compared favorably with those from enterally fed preterm infants reported in the literature. Failure to identify significant differences for the rate of weight gain, nitrogen balance, or nitrogen retention between the two groups suggests that differences in plasma aminograms resulting from use of one solution or the other had no short-term clinical consequences in the premature infants studied.

Mural thrombi in children: potentially lethal complication of central venous hyperalimentation.

While benefits of total parenteral nutrition (TPN) are well documented in a wide range of surgical conditions, deaths of two children secondary to mural thrombi from central venous catheters underscore the potential risks of such therapy. With the proven accuracy and widespread availability of echocardiography for diagnosis of mural thrombi, routine surveillance of all patients receiving TPN via central venous catheters is recommended, if fevers are present or if Candida is isolated on blood cultures. While treatment may involve surgical intervention, most mural thrombi will respond to thrombolytic agents. New approaches to long-term venous access are needed to prevent this lethal complication.

Familial retroperitoneal fibrosis.

Retroperitoneal fibrosis is an unusual cause of obstructive uropathy in the pediatric population. The etiology is unknown although there are laboratory and clinical associations with various autoimmune diseases. Familial associations have not been reported in children. A family is reported where two siblings have idiopathic retroperitoneal fibrosis. In addition, these sisters and the father manifest clinical laboratory evidence for systemic immunologic diseases. These young girls represent the first patients to illustrate both autoimmune and familial characteristics of this disease. These cases support the concept that retroperitoneal fibrosis is a local manifestation of a systemic immune disease.

Anterior cricoid split for subglottic stenosis.

Subglottic stenosis is a common problem that often results from ventilatory support necessary in the premature infant. Previous methods of treatment include tracheostomy with dilatation of the stenosis, steroid injections, and procedures to stent the trachea. Results of these methods have been unsatisfactory because of the multiple procedures needed to obtain an adequate airway as well as the high mortality from long-term tracheostomy in infants. In the past 2 years, seven infants have undergone an anterior cricoid split for tight subglottic stenosis and airway obstruction. Of the seven patients, six were premature, five of whom required ventilatory support ranging from 4 to 30+ days. Each child presented in respiratory distress with symptoms present in five children from 1 to 11 months (mean 3.8) after birth. Bronchoscopy identified the site of obstruction in each case as subglottic, with a narrow lumen, usually less than 2.5 mm in diameter. Anterior cricoid split was performed at ages ranging from 2 to 11 months (mean 5.0). All children were extubated at 10 to 14 days and subsequently discharged home asymptomatic; none required postoperative tracheostomy. Complications developed in five children, including atelectasis, otitis media, phlebitis, and tracheocutaneous fistula in two, one of whom required operative closure. One child was rebronchoscoped at 3 weeks postoperatively for bronchospasm, which resolved on aminophylline. The subglottic trachea was normal. At follow-up ranging from 2 to 21 months (mean 8.3), no child has symptoms referrable to the subglottic region. In one patient, a brief period of respiratory distress recurred 3 months postoperatively due to tracheomalacia.(ABSTRACT TRUNCATED AT 250 WORDS)

Hazards of splenic embolization.

Perceived high operative risk for splenectomy in children with hematologic disorders and hypersplenism has led to attempts at transcatheter splenic embolization (TSE) as a possibly safer alternative. A recent experience with a child who presented with an apparent acute subcapsular hematoma after TSE and who underwent complicated emergency splenectomy prompted review of the risks and complications of TSE. Although theoretically attractive in many respects, TSE is associated with significant morbidity. The resultant diagnostic and therapeutic difficulties of this procedure can potentially complicate patient management, and the often life-threatening sequelae warrant considerable restraint in its use. In addition, TSE often fails to produce the desired, long-term hematologic response. Because of the morbidity and risk associated with TSE, primary operative intervention is advised for most children in whom splenectomy is indicated. Morbidity associated with the direct operative approach may be minimized by judicious use of blood components and by meticulous surgical technique.

Necrotizing enterocolitis in the unfed infant.

From 1974 to 1981, 139 infants with neonatal necrotizing enterocolitis (NEC) were treated at our institution. Fourteen of these infants had not been fed prior to development of NEC. The unfed infants who developed intestinal necrosis had lower birth weights, were less mature, and had lower Apgar scores. The incidence of respiratory distress syndrome (RDS) and perinatal asphyxia were significantly higher in the entire unfed group. Unfed infants generally had longstanding indwelling umbilical artery catheters. Pneumatosis intestinalis was not often seen in unfed infants, even in those who developed intestinal necrosis and perforation. Anatomic location of the disease as determined at surgery was sometimes atypical. The pathologic lesion present in the intestine of the unfed infant suggested a primarily, if not purely, ischemic etiology.

Malignant hyperthermia. Current concepts.

At least three myopathies have been associated with malignant hyperthemia (MH). The clinical manifestations of MH are variable and depend on the nature of the underlying myopathy and the anesthetic agents administered. Unless muscle relaxants are used, fever and muscle rigidity may be delayed at onset. Tachycardia and tachypnea are often the earliest manifestations and can occur immediately or several hours into a surgical procedure. Life-threatening cardiac arrhythmias may result from hyperkalemia and acidosis. A hyperthermic reaction developed in an 8-year-old boy with a family history of Duchenne's muscular dystrophy one hour after induction of anesthesia. Temperature elevation and muscle rigidity were minor components of the condition. Determination of arterial blood gas concentrations and the serum potassium level established the diagnosis and enabled the start of lifesaving therapy.

Parenteral 20% safflower oil emulsion safety and effectiveness as a caloric source in newborn infants.

Five surgical infants, ages 3 to 30 days, were studied while receiving peripheral total parenteral nutrition for a period of 2 weeks. By a randomized sequence, either 10 or 20% safflower oil emulsion was infused the first week, followed by the alternate solution during the second week. Caloric intake was 80 to 100 cal/kg/day, of which lipids comprised one-third to one-half. No adverse side effects were noted. Eosinophilia developed in three patients, but otherwise no changes in hematologic and biochemical parameters, including liver enzymes, were noted. Weight gain averaged 11.1 g/kg/day during the study period and was similar for the 10 and 20% solutions. Intravenous fluid intake averaged 21 ml/kg/day less during the week of the patients received 20% fluid emulsions. The 20% safflower oil emulsion proved comparable to the 10% solution in both safety and efficacy and enabled significant reduction of fluid intake while maintaining infused caloric levels.

Inguinal hernia and hydrocele in infants and children.

Forty senior pediatric surgeons were surveyed regarding difficult decisions in the management of inguinal hernia. Areas covered were diagnosis, surgical techniques, hydrocele, incarceration, the contralateral side of a clinically apparent inguinal hernia, and inguinal hernia in the premature baby. The lack of agreement on many questions indicates that more than one approach may be effective in managing problems associated with hernia and that rigid policies are unwarranted.

A congenital abnormality in the arrangement of muscle bundles in a segment of the distal ileum, producing obstruction: a variant of the so-called "giant Meckel's diverticulum".

This 4-year-old male child was diagnosed at birth as having several minor congenital anomalies. X-rays taken during the first year of his life showed a single, massively dilated loop of bowel in the upper abdomen, not appreciated at the time. In 1980 he was admitted to Jackson Memorial Hospital with the diagnosis of small bowel obstruction. An upper gastrointestinal series showed one tremendously dilated loop of distal ileum. At laparotomy, the patient was found to have extreme segmental dilatation of one loop of distal ileum which ended abruptly; there was no evident external cause for obstruction. The resected loop contained in excess of 200 cc of watery brown liquid. The mucosal folds and underlying smooth muscle bundles, in the dilated portion only, were not arranged circumferentially but rather in a distinctive finger-print-like pattern with trifurcations, whorls, and intricate interdigitations which had probably produced contractions of a circus type rather than normal peristaltic waves. We have been able to find only three reports in the literature in which, as was the case here, the so-called "giant Meckel's diverticulum" presented as a single tremendously dilated segment of ileum, sharply demarcated at its distal end. In none of them is there any description of the orientation of muscle bundles. We believe that the abnormal arrangement of smooth muscle in the muscular coat in this specimen, and perhaps in the others, probably represents the underlying cause for the extreme localized dilatation.

A mixed hamartoma of the liver: light and electron microscopy.

The gross, microscopic, and ultrastructural features of a mixed liver hamartoma occurring in a three month old infant are reported. The differentiation from two solid liver masses, focal nodular hyperplasia and liver cell adenoma is emphasized. Mesenchymal hamartomas, though usually cystic rather than solid masses, share the histologic feature of fibroductular tissue with mixed liver hamartomas and focal nodular hyperplasia. Only the mixed liver hamartoma has extremely broad fields of ductules and an embryonic type of hepatocyte at the ultrastructural level. These and other hepatic lesions show morphologic evidence of transformation of liver cells into biliary epithelial cells in association with vascular connective tissue.

Gastrointestinal hemorrhage. An unusual complication of total anomalous pulmonary venous drainage.

A newborn boy had exsanguinating gastrointestinal hemorrhage in in the first week of life secondary to an unusual form of infradiaphragmatic pulmonary venous drainage. Autopsy and postmortem angiograms demonstrated huge esophageal varices as the course of the uncontrolled bleeding. The occurrence of massive gastrointestinal hemorrhage should be considered to be a rare but possibly lethal complication in patients with total anomalous pulmonary venous drainage (TAPVD) type III and IV.

Perforated appendicitis: current experience in a Childrens Hospital.

A review of 89 consecutive cases of perforated appendicitis recently treated surgically at Childrens Hospital of Los Angeles revealed no mortality and a complication rate of 17%. Significant factors in this low morbidity are: adequate preoperative resuscitation, routine administration of broad spectrum antibiotics pre and postoperatively, and attention to surgical detail. Anaerobic organisms were invariably present in cultures of the peritoneal fluid taken at operation. Anaerobes were also present in the blood in all 5 patients having positive blood cultures and were frequently pathogens whenever postoperative infectious complications occurred. The use of antibiotics effective against anaerobic organisms was common in this series and produced no morbidity.