Sustained normoglycemia in newly diagnosed type I diabetic subjects. Short-term effects and one-year follow-up.

The impact on remission of normalizing blood glucose levels immediately after diagnosis of type I diabetes was studied in 14 adolescents. Accordingly, in this randomized prospective primary intervention study, 7 of the subjects (i.v. group) received insulin by continuous intravenous (i.v.) infusion via a portable preprogrammed system for 28-62 days and 7 (s.c. group) received conventional subcutaneous (s.c.) therapy. Before therapy, the two groups did not differ significantly with respect to glycosylated hemoglobin, fasting plasma C-peptide, or 24-h urinary C-peptide excretion. During the infusion period, the overall mean fasting plasma glucose (FPG) concentration for the i.v. group was 84 mg/dl with a mean coefficient of variation of 18 +/- 4% (mean +/- SD). During the comparable period for the s.c. group, the mean FPG was 253 mg/dl with a coefficient of variation of 30 +/- 20%. Twenty-four-hour urinary glucose excretions for the two groups were 0.29 +/- 0.06 (mean +/- SEM) and 59 +/- 11 g/day, respectively. Daily insulin requirements in the i.v. group decreased from 1.47 +/- 0.19 U/kg body wt/day at the start to 0.47 +/- 0.10 U/kg/day at the end of the infusion period. Notably, 10-25 days after the infusion period, 5 of 7 subjects experienced a further decrease to a low of 0.27 +/- 0.01 U/kg/day. The mean peak and low requirements in the s.c. group were 0.71 +/- 0.15 and 0.33 +/- 0.13 U/kg/day, respectively, with the only peak requirements being significantly different (P less than 0.01). No patient was able to discontinue insulin therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Evaluation of doubly labeled water for measuring energy expenditure during changing nutrition.

Change in abundance of 2H and 18O in dietary water during a doubly labeled water energy period may introduce error into the calculated carbon dioxide production rate (RCO2). To examine the accuracy of 2H2(18)O during changing nutritional regimens, we compared 2H2(18)O and periodic open-circuit respiratory gas exchange (RGE) in postsurgical infants who were changing from parenteral to alternative parenteral and/or oral nutrition. The two methods were compared before and after correction for shifts in isotopic abundance of the infant water pools during the energy-expenditure period. Baseline corrections were predicted using the difference between abundances of the initial body water and final nutrient solutions. Before isotopic correction, 2H2(18)O underestimated RCO2 in eight subjects by 11.8 +/- 20.1% (mean +/- SD). After correction, agreement between the two methods improved; the underestimate was then -8.7 +/- 12.9%. To obtain maximum precision of 2H2(18)O, subjects should be maintained on the same nutritional regimen before and during the study unless valid correction formulae are used.

Balloon embolization of a bleeding gastroduodenal artery in a 1-year-old child.

Severe stress can produce ulceration and uncontrollable hemorrhage in the pediatric age group. Modern angiographic techniques, using embolization of a Silastic balloon to occlude a visualized bleeding vessel, can successfully control hemorrhage and avoid surgical intervention and its resultant disordered gastric physiology. Recently this technique was used to control hemorrhage from the gastroduodenal artery in a 1-year-old child with severe respiratory sepsis. This is the youngest reported child in whom the technique has been used to control bleeding from this vessel.

Lymphoscintigraphy for cystic hygroma.

We present the case of an infant with a cystic hygroma investigated by lymphoscintigraphy. The source of lymph flowing into the hygroma was identified as were some ectatic lymphatics feeding it. Lymph flow was very rapid. The implications for therapy are discussed. Lymphoscintigraphy can assess many aspects of lymph flow not accessible by other means.

Wide gap esophageal atresia with inaccessible upper pouch.

Circular myotomy of the proximal esophageal pouch has allowed end-to-end esophagoesophagostomy in infants with wide gap esophageal atresia. Occasionally, the proximal pouch ends so high in the thorax that the circular myotomy is technically impossible under conventional means. Exteriorization, mobilization, and myotomy of the proximal esophageal pouch via a separate cervical incision has allowed us to overcome this limitation of an inaccessible upper pouch and has permitted esophagoesophagostomy. This technique has been performed successfully in two infants with good results and no morbidity over a follow-up period of 1 1/2 to 3 1/2 years.

A new model of tracheal stenosis and its repair with free periosteal grafts.

Stenosis of 50% to 80% of the cervical or thoracic tracheal lumen was created in 17 piglets by encircling the trachea with silicone rubber sheet for 3 to 19 days (median 14 days). In 13 animals thoracic tracheal stenosis was repaired by longitudinal incision and insertion of a free tibial periosteal graft into the defect. An omental pedicle graft was applied to the surface of the free periosteal graft to augment its blood supply in three of these animals. An endotracheal silicone rubber splint was left in place for 2 to 3 weeks after the operations. Four animals served as untreated controls. In 12 of 13 piglets respiratory obstruction was relieved. One repair failed because of graft necrosis. In two animals put to death 1 month and 2 months later, stenosis was absent and the grafts showed variable ossification. The luminal surface had epithelialized. In nine of 10 animals put to death 3 months after the operation, tracheal cross-sectional areas had doubled, stenosis and granulation tissue were absent, and all the grafts had ossified and epithelialized. A mild stenosis (24%) developed over a 2 mm length in one animal and the graft had not ossified. The omental pedicle graft did not improve vascularization of the free periosteal graft. These studies describe a model of tracheal stenosis that can be used to assess various methods of repair. The results indicate that tracheal stenosis can be successfully treated by incision and insertion of a free periosteal graft into the defect.

Preoperative chemotherapy in hepatoblastoma.

Since January 1986, 15 children with hepatoblastoma received three to six cycles of chemotherapy with cisplatin (20 mg/m2/day x 5 days) and doxorubicin hydrochloride (25 mg2/m2/day x 3 days) every 3 weeks before surgery. The extent of the tumor was defined by computerized tomography scan of abdomen and chest and diagnosis confirmed by percutaneous liver biopsy before therapy was started. Tumors in 10 children were considered unresectable at diagnosis because of pulmonary metastases, extensive bilobar involvement, or venous involvement. Volume of tumor reduction ranged from 35% to 95% and was independent of tumor histologic findings. After a minimum of three chemotherapy cycles, excision was undertaken when tumor volume decreased to a size at which hepatic resection was feasible and safe. Chemotherapy complications that were not serious did not delay surgery or result in tumor growth during treatment. Complete surgical excision was possible in 13 children, including 10 who had had unresectable tumors and five with pulmonary metastases. Only three resections of more than one liver lobe were required, and partial lobectomy was possible in four children. One operative death and three postoperative complications, one severe, occurred. Cyclic chemotherapy was restarted 4 to 6 weeks after surgery until a total of six courses were given. Twelve children (eight whose tumors were originally unresectable) completed treatment 3 to 56 (median, 21) months ago and have no evidence of disease. Two other children currently undergoing therapy may have residual disease. The results to date far surpass historic survival rates, which ranged about 25%.

Primary hyperparathyroidism in a paediatric hospital.

We retrospectively reviewed the presentation and management of children with primary hyperparathyroidism (PHPT) from 1973 to 1995 at a paediatric tertiary-care centre. There were 11 patients (6 females), aged 12.3-17.7 years at presentation, with sporadic PHPT confirmed by histopathology (single adenoma). Presentation consisted of renal colic, or non-specific gastrointestinal, musculoskeletal or neurological symptoms. Misdiagnosis was common until hypercalcaemia was identified, 0.5-24 months after onset of symptoms (mean 7.7 months). All patients had hypercalcaemia and low-normal serum phosphate. The parathyroid hormone (PTH) radioimmunoassay used before 1986 was elevated in 1/4 patients; the intact PTH assay used after 1986 was elevated in 7/7 patients. At presentation, six had end-organ damage: band keratopathy, renal lesions, and/or bone disease. Preoperative localization was accurate in 0/4 patients diagnosed before 1986, but 5/7 patients diagnosed after 1986: three by ultrasound or sestamibi scan alone, and two by ultrasound and technetium scan. Surgical outcome was not dependent upon the accuracy of pre-operative localization. PHPT is rare in children but usually associated with end-organ damage, presumably due to delayed diagnosis. It should be considered in the differential diagnosis of unexplained non-specific complaints. The intact PTH assay greatly assists pre-operative diagnosis. The usefulness of pre-operative localization requires further research.

Traumatic bronchial injuries in children.

Between 1975 and 1981 five children (three girls and two boys) from 3 to 11 years were treated for blunt chest trauma with major tears in two areas of the right main bronchus. All were hit or run over by motor vehicles and were in acute respiratory distress. All suffered right pneumothoraces; three did not respond to a chest tube with suction. Four of five children had subcutaneous emphysema, and two had fractured ribs on the ipsilateral side; three children also had contralateral chest injuries. Four had major extrathoracic injuries. Three children required early repair, while two needed late treatment. All five patients recovered well and have remained healthy from 5 to 10 years after injury. These cases serve as illustrations for a review of a survey of the literature.

Multidisciplinary treatment for childhood sarcoma.

Surgical extirpation of the primary tumor has traditionally been utilized as initial treatment for sarcomas in children. The present report, however, demonstrates that sarcomas are optimally treated by means of a coordianted multidisciplinary approach. The latter offers the potential for achieving improved survival and preservation of organs and limbs, particularly for structures of the head and neck, for extremities, and in the genitourinary system.

Xanthogranulomatous cholecystitis and cholecystoduodenal fistula formation associated with total parenteral nutrition in a six year old child.

A unique complication of florid xanthogranulomatous cholecystitis with cholecystoduodenal fistula formation is described in a 6 yr old male. The patient, who had a short gut syndrome, had been maintained on lifelong total parenteral nutrition (TPN) following extensive neonatal ischemic bowel necrosis secondary to gastroschisis. Endoscopic duodenal mucosal biopsy demonstrated a granulomatous inflammatory infiltrate surrounding bile casts suggesting the possibility of a fistula between the biliary tract and duodenum. Additional clinical and radiological evidence of a cholecystoduodenal fistula prompted surgical intervention. At laparotomy the gallbladder was firmly bound to the duodenum by dense fibrous adhesions. Histologic examination showed xanthogranulomatous inflammation in association with fragments of bile that were present both within the gallbladder wall and within a fistulous tract in the adjacent connective tissue.

Liver tumors.

Several different malignant and benign tumors of the liver are seen in children. They pose a significant challenge for the pediatric surgeon because they reach a very large size before discovery and hepatic resection is usually necessary for cure. Surgical mortality and morbidity are still significant.

Surgical diseases of the spleen.

Recognition of the spleen's important immune function has led to a more conservative surgical approach to the spleen. This has proved to be both safe and beneficial to the patient.

Central venous access catheters in children with haemophilia.

Twenty-five central venous lines (two external 23 subcutaneous ports) were placed in 19 boys with haemophilia A (n = 17) or B (n = 2). The mean age of the boys was 4.9 years (range 0.2-15.3 years). The haemophilia was severe (factor level < 1%) in 18 boys and moderate (factor level 3%) in one. Three boys had circulating inhibitors and three were positive for human immunodeficiency virus (HIV)-1 antibody. Central venous lines were placed to facilitate intermittent factor replacement therapy (n = 6), long-term factor prophylaxis (n = 9), induction of an immune tolerance protocol (n = 2) or therapy for acquired immunodeficiency syndrome (AIDS)-related complications (n = 2). The ports remained in place for 15795 days (mean 687 days, range 11-2059 days). The frequency of port-related sepsis was 48% (11/23 ports in eight boys) or 0.7 port infections per 1000 patient days. Ports were removed from five boys with an unresolved infection (four with Staphylococcus aureus sepsis and one with Pseudomonas sp. sepsis). Other complications requiring port removal included a catheter tip placed too high in the venous system (n = 1), severe persistent pain associated with needle access of the port (n = 1) and a subclavian vein thrombosis (n = 1). Both the benefits and risks of a subcutaneous port should be considered when deciding whether to place this device in a very young child with haemophilia.

Tracheomalacia.

Tracheomalacia describes a generalized or localized weakness of the trachea that results in excessive narrowing of the tracheal lumen during expiration or whenever intrathoracic pressure increases. Severe forms of tracheomalacia are characterized by life-threatening apneic spells, inability to extubate the airways, and episodic pneumonia. Aortopexy has proved to be an effective method of treatment for most of these conditions when seen in children. When aortopexy fails to prevent tracheal obstruction, a support applied to the external circumference of the trachea can be useful to prevent airway collapse.

Endogenous hyperinsulinism: diagnosis, management, and long-term follow-up.

Persistent hyperinsulinemic hypoglycemia of the neonate (PHHN) usually presents in the neonate or infant. Although rare, the possibility of PHHN should be considered in all neonates who have unremitting hypoglycemia. Untreated hypoglycemia results in severe neurological disability or death. An inappropriately elevated serum insulin level and low serum ketone and free-fatty-acid concentrations in the presence of hypoglycemia confirm the diagnosis. Early diagnosis and aggressive medical management have reduced the morbidity associated with PHHN. Pancreatic resection is necessary for long-term control of hypoglycemia when medical therapy fails. Ninety-five percent pancreatectomy has been the procedure of choice at the author's institution and other pancreatic centers. However, a review of the authors' experience and of in the English-language literature has demonstrated a 33% failure rate, requiring further surgery or medical management. Furthermore, long-term follow-up showed that diabetes develops in most children who undergo 95% pancreatectomy. The high failure rate of 95% pancreatectomy and the ultimate development of diabetes in virtually all children suggest the alternative approaches should be considered.

Experimental trial of balloon-expandable, metallic Palmaz stent in the trachea.

OBJECTIVE: To determine the effect of the Palmaz stent, which has been successfully used to relieve airway obstruction in a small group of children, on the normal and operated on animal trachea. DESIGN: In this experimental trial, stents were placed bronchoscopically in the thoracic tracheae of 4 groups of 50 anesthetized cats. The cats in group 1 (adults, n = 10) and group 2 (kittens, n = 10) had normal tracheae; the cats in group 3 (adults, n = 15) underwent horizontal tracheal incision and closure; and the cats in group 4 (adults, n = 15) underwent pericardial patch tracheoplasty. In group 3 and 4, the stents were inserted in 10 cats and 5 cats served as controls. Stents were inflated to a 15-mm diameter in group 1 and an 8-mm diameter in the other groups. Half of the animals with stents were killed 5 weeks after the procedure, and the others, 10 weeks after the procedure. SETTING: The Hospital for Sick Children, Toronto, Ontario. RESULTS: In group 1, 1 animal died of tracheal perforation. A mild cough was noted in 15 of the 39 cats with stents. In group 4, 3 cats had difficulty eating and lost weight. The results of esophagoscopy excluded esophageal inflammation or obstruction in these 3 animals. The results of bronchoscopy indicated a nonobstructing rim of granulation tissue at the end of the stent in 15 of 20 cats in groups 1 and 2 and 17 of 20 cats in groups 3 and 4 and at the repair site in all animals with stents from groups 3 and 4. The results of autopsy indicated the cross-sectional area at the site of the stent was greater than normal in group 1 (P < .003) and smaller than the normal trachea at the site of the tracheoplasty in group 4 controls without stents (P < .02); however, the cross-sectional area at the tracheoplasty site with the stent was not smaller (P < .13). The results of histologic examination indicated a mild inflammatory reaction, with granulation tissue in all animals with stents, but in group 1, with overexpanded stents, the reaction was more severe, with epithelial ulceration, fibrosis, and sealed-off perforations in most animals. In group 3, the tracheae with stents had significantly more inflammatory reaction, granulation tissue, and epithelial damage than the controls without stents. CONCLUSIONS: The Palmaz stent provokes an inflammatory reaction in the normal trachea and the trachea recently operated on. With the exception of the group 1 animals with overexpanded stents, this reaction is clinically insignificant. The Palmaz stent is able to maintain a normal lumen size after pericardial tracheoplasty in cats.

Hormonal bioassay of gonadotropin-producing hepatoblastoma.

Hepatoblastoma was associated with signs of isosexual precocity in an 11-month-old boy. Circulating gonadotropic hormone levels were elevated. Gonadotropic activity was demonstrated by bioassay in extracts of primary and metastatic tumor, and in media surrounding long-term tissue culture of the primary tumor. Morphology of the endocrine glands and bioassay of the pituitary gland obtained at autopsy indicated the presence of gonadotropins and other tropic hormones despite the physiologically abnormal hormone status. These ae the first direct studies of pituitary hormone content in this syndrome.

Immaturity alters plasma lipoprotein composition of intravenously alimented newborn infants.

Plasma lipoprotein composition in infants receiving fat-free parenteral nutrition reflects the endogenous synthesis and metabolism of lipids. We studied the composition of plasma lipoproteins in 49 appropriate for gestational age newborn infants after surgery who received only glucose and amino acid solutions for 5.4 +/- 0.3 days (M +/- SE). Of the infants studied, 31 were fullterm (gestational age 39.5 +/- 0.2 weeks) and 18 premature (34.3 +/- 0.7 weeks). Plasma lipid levels (total lipids, triglycerides, free cholesterol, sterol, esters, phospholipids) did not differ between term and premature infants, but triglycerides and cholesterol were markedly lower than in young, fasting adults. The contribution of triglycerides to lipoprotein lipids was strikingly low in chylomicrons (21% vs. 90% in young fasting adults) and VLDL (34 vs 60%) and the infants had a consistently lower cholesterol content of HDL (21 mg/dl vs. 45-50 mg/dl in adults) and LDL (43 mg/dl vs. 100 mg/dl). All infantile lipoproteins were enriched with phospholipids. These results are comparable to those reported for cord plasma. In premature babies, VLDL were markedly reduced and contained less triglycerides, free and esterified cholesterol than in term infants. In contrast, HDL were increased in preterm infants and carried more phospholipids. VLDL contributed to al lesser and HDL to a greater extent to plasma lipid transport in premature infants. We conclude that in premature infants hepatic synthesis of triglycerides and cholesterol and their secretion as VLDL is reduced, which may be caused by low substrate availability or an immaturity of the synthetic pathway. In premature infants, HDL appears to play a major role in transporting plasma lipids to peripheral tissues.

APSA: a forum for progress in pediatric surgery.

The APSA scientific program has indeed been a forum for progress for a broad spectrum of medical and surgical problems affecting infants and children. Significant contributions from studies at the bedside and in the laboratory have been made by an extraordinarily large percent of APSA members from both children's and general hospitals. In addition to the many advances that apply to our own specialty, fundamental advances have been recognized and accepted by others. Developments for the future have been identified and our entire membership can be proud of the wonderful achievements of the last 20 years of APSA scientific programs. Finally, I thank you all for giving me the honor to serve as the President of this outstanding organization, which has accomplished so much already, but whose best days are yet to come.