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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.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.The Hospital for Sick Children, Toronto, Ontario.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.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.

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.

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.

Aortopexy is the more extended treatment for severe tracheomalacia, when it fails, reintervention and other procedures are necessary. We present our experience in the treatment of this pathology.Tracheomalacia, bronchomalacia and tracheobroncomalacia cases during a twelve year period (1983-1995) were reviewed (type, age, symptoms, surgical procedures and results were collected).Nineteen patients (9 males, 10 females) presented tracheobronchomalacia in our Hospital. Diagnosis was made by bronchoscopy in all of them: nine cases of tracheomalacia, five bronchomalacias and five suprastomal malacias were found. Mean diagnosis age was twenty four months (newborn-twelve years). Ten patients were managed nonoperatively, five with anterior cricoideal suspension and two cases were treated by aortopexy; one of them, after 2 months of respiratory stridor and recurrent respiratory arrest, needed an endoscopically placed intraluminal expandable Palmaz Prothesis. This prothesis was removed two years later. In this moment, the patient is doing well without stridor or respiratory symptoms.

Between 1974 and 1988, 86 newborns with perforated necrotizing enterocolitis (NEC) were treated by either laparotomy (usually involving a bowel resection and a temporary stoma) or a peritoneal drain under local anesthesia. The survival of babies in the laparotomy group was 57% versus 59% in the drained group. However, for neonates less than 1,000 g survival in the drained group was 69% compared to 22% for the laparotomy group (P.01). As the weight of the babies increased over 1,000 g, the survival in the laparotomy group increased to 67%. There was no significant increase in survival in infants over 1,500 g. The highest neonatal mortality risk is generally found among babies weighing less than 1,000 g at birth with a gestational age of less than 30 weeks. This risk increases even more when perforated NEC is added to the prematurity. With the use of peritoneal drainage, survival in this group can approach that of larger neonates.

Summary 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 endorgan 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 preoperative diagnosis. The usefulness of pre-operative localization requires further research.

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 level1%) 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 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.

Pediatric malignant pheochromocytomas are very rare tumors, and no institution has more than one or two of these problem cases. The authors report on two children with such tumors, over a 9-year period, from two hospitals. In 1984, D.B. (14 years of age) presented with symptoms and signs of extradural metastasis from a right adrenal primary; he also had lung and bone metastases. After spinal decompression almost 4 years. He has remained well and is in remission 6 years later. In 1987, G.R. (13 years of age) presented with a larger right adrenal malignant pheochromocytoma invading surrounding structures; he also had liver metastasis. Preoperative chemotherapy did not shrink the tumor much; it was grossly resected, and there were many postoperative problems. In 1990, bone metastases developed, for which radiotherapy and chemotherapy were used. Three years later, the metastases have not disappeared; he remains on chemotherapy, and his liver function is borderline. From our small experience as well as a literature review, it appears that surgical excision remains the treatment of choice for the pediatric malignant phoechromocytoma. Unresectable tumors may be rendered resectable by intensive chemotherapy (similar to that used for neuroblastoma); adjuvant chemotherapy should be used for residual disease after surgery and for metastatic disease.

The purpose of this study was to assess the accuracy of CT for determining surgical resectability of hepatoblastoma before and after chemotherapy.Preoperative and postoperative CT scans of 15 children with surgically confirmed hepatoblastoma were retrospectively reviewed and correlated with surgical findings and pathologic analysis.Before chemotherapy, 12 of the 15 children had unresectable tumors because of bilobar involvement, metastatic disease, or vascular extension. After chemotherapy, tumor volumes decreased by 20-98%, with increased areas of low attenuation and calcification. Periportal areas of low attenuation (four of nine), abdominal adenopathy (four of four), and lung nodules (four of six) disappeared after chemotherapy. On the basis of CT and clinical findings, all tumors were thought to be resectable. Surgical correlation showed that the extent of hepatic tumor had been overestimated on immediate preoperative CT scans in three children (tumor was staged as bilobar, but only single-lobe resection was required). Correlation between areas of low attenuation in the tumor and necrosis in pathologic specimens was poor. Tumor invasion of periportal lymphatics was seen in one of five children with periportal areas of low attenuation. Postoperatively, five of 13 children had CT abnormalities, three at the resection margins with calcification, low attenuation, or both. Hepatic or abdominal disease has not recurred, and 12 of the 14 children who survived surgery are well. One child who had abnormal findings on preoperative chest CT died of pulmonary metastases. Another in whom chemotherapy was limited because of toxic effects died after tumor recurred in the liver.Pretreatment CT scans cannot be used to predict ultimate resectability of hepatic tumors; nor are preoperative scans always accurate for judging exact lobar involvement. Absence of disease as shown on CT scans correlated with absence both at surgery and at follow-up. Postoperative hepatic changes are common and do not necessarily reflect recurrent or residual tumor.

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%.

A series of 18 children suffering from persistent idiopathic neonatal hypoglycemia (PINH) is reported. Medical and surgical managements are described in detail. All patients subjected to surgery had failed medical treatment. These patients were divided into two groups: 1) 85% pancreatectomy leaving the uncinate process in situ, and 2) 95% pancreatectomy leaving a small rim of pancreatic tissue along the duodenum and the common bile duct. The spleen was preserved in all cases. Two out of 5 children of group 1 required further resection of the pancreas for persistent hypoglycemia and were converted to 95% pancreatectomy. Since 1981 95% pancreatectomy was exclusively employed. Only one patient required insulin for 3 weeks postoperatively. Histo-pathology and immunohistochemistry revealed islet cell dysplasia and islet cell nuclear hypertrophy in the majority of cases. 35% of the patients had focal adenomatosis. Better control of hypoglycemia is achieved by primary 95% pancreatectomy and, thus, 95% pancreatectomy is recommended as the initial procedure in the treatment of PINH.

A series of 18 children suffering from persistent idiopathic neonatal hypoglycemia (PINH) is reported. Medical and surgical managements are described in detail. All patients subjected to surgery had failed medical treatment. These patients were divided into two groups: 1) 85% pancreatectomy leaving the uncinate process in situ, and 2) 95% pancreatectomy leaving a small rim of pancreatic tissue along the duodenum and the common bile duct. The spleen was preserved in all cases. Two out of 5 children of group 1 required further resection of the pancreas for persistent hypoglycemia and were converted to 95% pancreatectomy. Since 1981 95% pancreatectomy was exclusively employed. Only one patient required insulin for 3 weeks postoperatively. Histopathology and immunohistochemistry revealed islet cell dysplasia and islet cell nuclear hypertrophy in the majority of cases, 35% of the patients had focal adenomatosis. Better control of hypoglycemia is achieved by primary 95% pancreatectomy and, thus, 95% pancreatectomy is recommended as the initial procedure in the treatment of PINH.

Antireflux surgery was performed in 234 children over a 5-year period; 153 were neurologically impaired (NI) and 81 were neurologically normal (NN). Initial presentation, demographic data, and type of antireflux operation were similar in the two groups. Eighty-six percent of the NI group versus 30% of the NN group had gastrostomy tubes placed. The incidence of late postoperative complications was 26% in the NI group and 12% in the NN group (P less than .01). During the late postoperative period, NI children underwent reoperation four times as frequently as NN children (19% v 5%, respectively; P less than .01). Wrap herniation accounted for 38% of complications and 59% of reoperations in the late postoperative period. Mortality due to aspiration occurred in 9% of the NI group versus 1% of the NN group. Combined failure rate (reoperation plus aspiration-induced deaths) was 28% in NI and 6% in NN (P less than .01). We conclude that neurological status is the major predictor of operative success and that wrap herniation due to crural disruption is the most common cause of operative failure.

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.

Change in abundance of /sup 2/H and /sup 18/O in dietary water during a doubly labeled water energy period may introduce error into the calculated carbon dioxide production rate (RCO/sub 2/). To examine the accuracy of /sup 2/H/sub 2/(/sup 18/)O during changing nutritional regimens, we compared /sup 2/H/sub 2/(/sup 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, /sup 2/H/sub 2/(/sup 18/)O underestimated RCO/sub 2/ 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 /sup 2/H/sub 2/(/sup 18/)O, subjects should be maintained on the same nutritional regimen before and during the study unless valid correction formulae are used.

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.

Previous studies show that the doubly labeled water method is accurate for measuring energy expenditure in the adult human. To validate this method in infants, carbon dioxide production rate and energy expenditure were measured for 5 to 6 days by doubly labeled water (DLW) and periodic open circuit respiratory gas exchange (RGE) in 10 blinded studies in nine infants following abdominal surgery. Infants were maintained on consistent oral or parenteral nutrition prior to and during study. This avoided diet-related changes in baseline isotopic enrichment of body water which could theoretically contribute to significant errors in calculation of carbon dioxide production rate. For DLW, insensible water loss was assumed to be proportional to respiratory volume and body surface area, where the former was predicted from carbon dioxide production rate. Insensible water loss thus calculated averaged 18% of water turnover. Rates of carbon dioxide production measured by DLW were not significantly different from that of RGE (10.4 +/- 1.1 and 10.5 +/- 0.9 l/kg/day, mean +/- SD, respectively). Energy expenditure was calculated using respiratory quotients from dietary intake (DLW:DIET) and RGE (DLW:RGE) data. There was no significant difference between energy expenditure determined by DLW (DLW:DIET and DLW:RGE) and that measured by RGE (58.5 +/- 6.1, 56.8 +/- 6.1, and 57.3 +/- 5.1 kcal/kg/day, mean +/- SD, respectively). Rate of carbon dioxide production, DLW:diet, and DLW:RGE calculated by DLW differed from corresponding RGE values by -0.9 +/- 6.2, -1.1 +/- 6.1, and 1.6 +/- 6.2%, mean +/- SD, respectively. These findings demonstrate the validity of the doubly labeled water method for determining energy expenditure in infants without concurrent water balance studies.

The combination of isotope scanning and angiography is extremely valuable in the preoperative and postoperative evaluation of abdominal diseases, particularly hepatic disease. This technique can accurately detect, show the extent of and in many instances predict the nature of various hepatic abnormalities.Armed with this information, the pediatrician or surgeon is able to best decide whether surgery, irradiation or medical management are indicated.

Evaluation of 115 children who had irradiation to the liver reveals an increase in abnormality of the parameters studied when large volumes of liver are irradiated or when the liver is irradiated following resection. To some extent, this is dependent on dose but abnormalities have been observed at doses below 2,499 rads and as low as 1,200 rads. Possibly, this may be related to the increased sensitivity of the younger child who generally received these lower dosages.Although evaluation in the chronic phase shows no significant difference of abnormality of age as related to dose, there does appear to be a slight trend in the acute phase of irradiation to implicate sensitivity of children under 1 year.

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.

Studies aimed at quantifying the benefits of infused rather than injected insulin have been impeded by the unavailability of suitable methods for continuous vascular access. We have developed indwelling catheters suitable for the long-term blood sampling as well as insulin infusion. A typical catheter consists of a silicone rubber tube, which terminates with a blunt-point needle. Internal or external reinforcement as well as a removable strain relief fitting protect the area of insertion of the needle. Anchoring of the implanted extravascular portion of the catheter is important and is achieved by special grommets that prevent motion without obstructing the lumen. Methods of catheter assembly as well as implantation in the jugular and portal veins of dogs and jugular veins of humans are described. A total of 34 blood sampling catheters were implanted in 26 beagle dogs: 74% were uncomplicated with a duration of implantation of 18–576 days while 15% and 11%, respectively, failed due to infection or physical damage after an average of 200 days. Thirteen portal infusion catheters were implanted: 62% were functional a mean of 100 days (14–166 days) after implantation while 35% became covered with an endothelial sleeve and had to be removed. Thirty-two peripheral infusion catheters were implanted in dogs: one infection occurred, two catheters were physically damaged, and three were plugged. There were no catheter-related complications in the five adolescent diabetic subjects with such jugular venous catheters studied for an average of 19.5 days and three adult diabetic subjects in whom catheters remained patent for 150–270 days. It is the inherent characteristics of the silicone rubber material as well as the new methods of catheter construction and implantation that virtually eliminate catheter-related problems and that now provide an ideal route of access for long-term intravascular insulin infusion in diabetes mellitus.

Maldescent is known to increase the risk of cancer of the testis. However, the effect of orchiopexy on reducing tumour risk and altering tumour type or stage at diagnosis is not established. The authors review the records of patients with testicular tumours seen for seminoma (1958 to 1975) and nonseminoma (1958 to 1970). Of 646 patients, 53 (8.2%) had a history of testicular maldescent; in 42 (79%) the maldescent was unilateral and in 6 of these, the tumour developed in the normally descended testis. Twenty patients had successful orchiopexy at a median age of 14 years (range from 6 to 35 years). Tumours found in the 53 patients were seminoma (38 patients), teratoma (7 patients), embryonal carcinoma (6 patients), teratocarcinoma (1 patient) and choriocarcinoma (1 patient). Average age at the time of diagnosis of seminoma was 38 years and of nonseminoma 31 years. Average age was lower at the time of diagnosis of the tumour for those who had successful orchiopexy than for those who did not. Earlier stage nonseminomas were found in patients who had an orchiopexy. Twenty-five (66%) of the 38 seminomas occurred in testes located outside the scrotum, whereas only 6 (40%) of 15 nonseminomas occurred in a nonscotal position, suggesting that persistent maldescent favours seminoma over nonseminoma. In this retrospective review, no statement can be made about the effect of orchiopexy on tumour risk. However, this procedure appears to lead to an earlier diagnosis and may influence the type of tumour that subsequently develops.

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 < 0.01). No patient was able to discontinue insulin therapy. The fasting plasma C-peptide level rose significantly (P < 0.05) by 1 mo in the i.v. group, but only achieved similar levels by 4 mo in the s.c. group. In the i.v. group, 24-h urinary C-peptide excretion decreased during the first week of therapy, rose through the infusion period, and finally peaked at 60 ± 14 μg day 3–30 days later. In the s.c group, the urinary C-peptide excretion gradually increased reaching a significantly lower peak (P < 0.05) of 36 ± 8 μg day at 113 ± 38 days. Glycosylated hemoglobin levels declined significantly only in the i.v. group with 4 subjects achieving normal levels. At 1 mo after diagnosis, the i.v. group had significantly higher (P < 0.05) fasting plasma C-peptide levels and lower (P < 0.005) glycosylated hemoglobin levels than did the s.c. group. Inspite of the higher peak urinary C-peptide excretion for the i.v. group, 4 and 12 mo after diagnosis the groups did not differ in mean 24-h urinary C-peptide excretion, fasting plasma C-peptide, insulin requirements, or glycosylated hemoglobin. We conclude that sustained strict glycemie control within normal limits at diagnosis of type I diabetes does not of itself augment the remission phase.

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.

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.

Injury patterns and use of passenger restraints were studied in 91 children injured while riding in motor vehicles and admitted to The Hospital for Sick Children, Toronto, from June 1984 through December 1985. Of theses, 44 had used restraints and 38 had not. Nine were excluded from the study because restraint use could not be determined. Age and sex distributions were closely matched in both study groups. The no-restraint group had more massive head injuries, thoracic injuries, and liver and spleen injuries than the restraint group. Classic "seatbelt syndrome" injuries were seen in the seatbelt group. The overall Injury Severity Scores were not significantly different between the groups. Despite mandatory legislation, many children do not use restraints, and many who do still suffer severe or fatal injuries. We conclude that better compliance with existing passenger restraint laws and more effective restraint systems are needed.

Segmental infarction of the greater omentum is an unusual cause of acute abdominal pain in children. Over 30 years at the Hospital for Sick Children in Toronto, this entity was encountered nine times. All children complained of right lower quadrant pain. They were tender in that area and were thought to have acute appendicitis. At operation, the appendix was found to be normal in all and an area of infarcted omentum was identified as the cause of the acute illness. Torsion of the infarcted omentum was noted in four cases. Excision of the omentum was curative.

Devices and methods for long-term intravenous (i.v.) insulin infusion in ambulatory patients have recently been developed. However, to date optimal waveforms for open-loop insulin administration in severely insulin deficient patients have not been defined. We studied 7 nonobese uncomplicated type 1 diabetics aged 16.5 ± 0.2 yr who received insulin continuously for 14–23 days by central venous infusion. A pattern of i.v. insulin administration was developed and eventually refined to include a constant basal infusion rate augmented at meal time by a series of 3 pulses. The first pulse lasted 10–15 min with an infusion rate 1.5–2 times the basal rate. The second pulse was 60–100 min in duration with a rate 4–6 times the basal infusion rate. The third pulse lasted 40–100 min with an infusion rate 1.5–3 times the basal rate. Accompanying the evolution of this waveform, the mean fasting plasma glucose fell from 262 ± 40 mg/dl on conventional therapy to 92 ± 9 mg/dl during infusion with normal meal excusions (mean a.c. glucose 80 ± 3 mg/dl with a rise of 35 ± 3 mg/dl at 41 ± 3 min). Mean urinary glucose excretion decreased from 55 before to 1.9 g/day during the infusion period. Fasting serum cholesterol and triglycerides dropped from 220 ± 28 and 281 ± 94 to 138 ± 13 and 115 ± 24 mg/dl, respectively.Mean HbA, declined from 14.8% before to 10.7% at the conclusion of the infusion period. All indices of control showed improvement significant at P < 0.05. There were no catheter-related complications and the system was well accepted by all subjects. These studies confirm that continuous central venous insulin infusion with a relatively simple waveform is feasible for the prolonged normalization of glycemia and other indices of metabolic control in ambulatory adolescent diabetics.

Ten patients with metastatic osteosarcoma were treated at the Joint Center for Radiation Therapy, the Sidney Farber Cancer Institute, and the Children's Hospital Medical Center from 1973 to 1976. Patients were treated with an aggressive multimodality approach with included surgery, chemotherapy, and radiation therapy. Three of 10 patients are alive with no evidence of disease, five are alive, with disease, and two are dead of disease. The median survival is 12+ months. Local control data for radiation combined with high dose methotrexate in metastatic osteosarcoma is shown.

Total parenteral nutrition TPN has been used to treat or prevent malnutrition in 65 children with a variety of solid tumors and leukemia in the past 7 years. TPN was used in 58 patients with gastrointestinal complications of surgery, chemotherapy, or radiation; in 2 patients for preoperative correction of malnutrition; and in 8 who were entered into a prospective study. During TPN, general nutrition and appearance improved in all patients. Weight gain was noted in most. Despite gastrointestinal complications, which usually require the interruption of chemotherapy and radiation, treatment could be continued at full dose in 31 children with nutritional support by TPN. TPN was discontinued in 6 patients when blood cultures became positive. Sepsis was treated successfully by removal of the central venous catheter in all 6 and administration of antibiotics in 3. TPN appears to be safe and effective means of combating malnutrition which may occur with cancer and its therapy.

Treatment results for 32 children with Wilms' tumor seen at the JCRT, CHMC and SFCI from January 1, 1971, through December 31, 1975, are presented. Indications for and technical details of irridation are discussed. The uniform efficacy of irradiation in preventing local relapse in all patients presenting with unilateral disease despite tumor spill in nine is stressed. Actuarial survival of nearly 80% of all patients seen and followed for more than 2 years has been achieved. Eight of 14 patients with documented pulmonary relapse have been controlled with irradiation, chemotherapy and surgery. The importance of an aggressive multidisciplinary approach to this tumor is stressed.

Acute and chronic starvation is often associated with childhood cancer. Total parenteral nutrition (TPN) with 20% glucose and 3.0% amino acids, and minerals and vitamins was instituted to treat or prevent malnutrition in 41 children with cancer, ages three months to 18 years. TPN was required for anorexia, vomiting and diarrhea associated with anti-cancer therapy in 33 patients for intestinal complications or surgery in nine, and for preoperative correction of malnutrition in two. During TPN, general nutrition and appearance improved in all patients. Weight gain was noted in most. Despite gastrointestinal complications which usually require the interruption of chemotherapy and irradiation, in 21 children treatment could be continued at full dose with nutritional support by TPN. TPN was discontinued in six patients when blood cultures became positive. Sepsis was treated successfully by removal of the central venous catheter in all six and administration of antibiotics in three. No metabolic complications were noted. TPN appears to be a safe and effective means of combating the malnutrition which may occur with cancer and its therapy.

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.

Forty children with localized resectable intestinal non-Hodgkin's lymphoma were seen between 1948 and 1974. Survival was related to the extent of disease at presentation and to therapy. No deaths occurred after 15 months and no recurrences occurred after 13 months after diagnosis. Six of eight stage IE patients (75%) and nine of 29 stage IIE patients (31%) have survived a minimum of 2 years; one of the three stage IVE patients has survived 17 years. Unfavorable prognostic findings at surgery were serosal involvement, presence of tumor at the surgical margins, mesenteric nodal involvement, and the presence of abdominal fluid or blood. Paraortic nodal involvement or multiple primary foci were universally fatal. Eight of 11 patients (73%) treated with surgery and whole abdominal irradiation (greater than or equal 2000 rad) with or without chemotherapy have survived. Seven of 18 patients (39%) treated with surgery and low dose chemotherapy have survived. One of seven patients treated with surgery and localized or low dose radiation therapy with or without chemotherapy has survived. Four patients treated with surgery alone died. Bone marrow and central nervous system involvement occurred after previous disease relapse elsewhere.

Four patients are described who have manifested unusual reactions to combined irradiation and chemotherapy when irradiation of the liver was involved. Two had had partial resection of the liver and irradiation of all of the remaining liver tissue; each of the others, with an intact liver, may have had compromise of liver function by infection and/or by lower dose irradiation of the left lobe of liver.We suggest that irradiation of the liver, especially in patients who receive chemotherapeutic agents which may require liver for detoxification or excretion, be very carefully administered, even at a minimal dose. Any underlying compromise of the liver must be evaluated carefully. In each patient, hemoglobin levels were normal throughout the clinical course.