Pediatric Cardiology Fellowship Standards for Training in Exercise Medicine and Curriculum Outline.

Over the past 2 decades, fundamentals of exercise medicine, including clinical exercise testing, assessment and promotion of physical activity, exercise prescription, and supervised exercise training/rehabilitation programming have demonstrated considerable clinical value in the management of children and adolescents with congenital and acquired heart disease. Although the principles of exercise medicine have become an integral component in pediatric cardiology, there are no standardized training recommendations for exercise physiology during pediatric cardiology fellowship at this time. Thus, the Pediatric Cardiology Exercise Medicine Curriculum Committee (PCEMCC) was formed to establish core and advanced exercise physiology training recommendations for pediatric cardiology trainees. The PCEMCC includes a diverse group of pediatric cardiologists, exercise physiologists, and fellowship program directors. The expert consensus training recommendations are by no means a mandate and are summarized herein, including suggestions for achieving the minimum knowledge and training needed for general pediatric cardiology practice.

Absence of mutations in superoxide dismutase and catalase genes in patients with Parkinson's disease.

BACKGROUND: Parkinson's disease (PD) is an adult-onset, neurodegenerative disorder characterized by a selective loss of the dopaminergic cells of the substantia nigra and by progressive motor decline. Studies have shown aberrant oxidative stress metabolism within the substantia nigra and other dopaminergic regions of the brain in patients with PD. OBJECTIVE: To screen the genes of three free radical detoxifying enzymes--copper/zinc superoxide dismutase, manganese superoxide dismutase, and catalase--for mutations in patients with PD. PATIENTS AND METHODS: A total of 107 unrelated patients with PD from two PD populations (familial and sporadic) were screened for mutations in the genes of copper/zinc superoxide dismutase, manganese superoxide dismutase, and catalase by single-strand conformation analysis. The diagnosis of PD was based on the clinical observations of resting tremor, rigidity, and bradykinesia. RESULTS: No mutations were identified. However, we did identify an amino acid substitution (glycine to aspartic acid) in exon 9 of the catalase gene in one patient; decreased red blood cell catalase activity was observed in this patient. CONCLUSION: Parkinson's disease is not caused by mutations in the genes of these three detoxifying enzymes. The exon 9 variant in the catalase gene in the one family with PD is most likely a silent mutation and not the genetic cause of PD in this family.

Complications of primary repair of colon injury: literature review of 2,964 cases.

OBJECTIVE: To evaluate the safety and efficacy of primary repair of colon injuries. MATERIALS AND METHODS: A literature review was made of 35 publications containing 5,400 colon injuries in retrospective and prospective studies. RESULTS: There were 62 (2.4%) failures in 2,627 primary repairs based on surgeon discretion or absence of risk factors. In prospective series of 337 patients repaired without exclusionary criteria, there were 4 (1.2%) suture line failures (P = not significant). In prospective randomized trials without exclusionary criteria, 127 primary repairs had less morbidity compared with 109 diverted patients (P <0.02). The leak rate after resection and anastomosis (5.5%) is greater than after simple suture of perforation (1.4%; P <0.001). The 66 colon repair leaks were treated by conversion to colostomy or led to fistulae that usually healed spontaneously. A preponderance of failed repairs occurred in the setting of multiple injuries or comorbid conditions. CONCLUSIONS: Penetrating and blunt colon injuries in civilian practice are safely managed by primary repair, but colostomy may still be advised in selected cases.

Laparoscopic Swenson pull-through: a comparison with the open procedure.

This study was performed to compare the standard open Swenson pull-through (OSP) with the laparoscopic Swenson pull-through (LSP) for Hirschsprung's disease. The Swenson pull-through was performed on eight patients with a rectosigmoid transition zone, during a 14-month period, using one camera port and three working ports. The results were compared with those of 10 patients with a similar lesion treated by the open procedure during an overlapping 19-month period. One laparoscopic procedure was converted to the open version because of technical difficulties. Both methods had a hand-sewn anastomosis approximately 1 cm above the pectinate line. The preoperative variables of age, weight, incidence of colostomy, and incidence of Down's syndrome were similar for the two groups. The operating time for LSP was similar to that for OSP (4 hours 42 minutes v 4 hours 37 minutes, respectively: P = NS). Postoperatively, the laparoscopic group had a shorter hospital stay (5.25 v 8.8 days; P < .05) and had a shorter period until the start of oral intake (2.75 v 5 days; P < .05). The requirement for narcotic pain medication was similar (12.6 v 12.8 doses; P = NS). Early postoperative complications were more common in the open group (3 wound infections, 1 prolonged ileus, and 1 anastomotic leak). No complications occurred in the laparoscopic group. Late postoperative follow-up was too short to compare functional results. The authors conclude that the Swenson pull-through can be performed safely with the laparoscope, with reduced morbidity.

The feasibility of laparoscopic swenson pull-through.

The Swenson abdominoperineal pull-through is the authors' preferred treatment for children with Hirschsprung's disease. The present study was performed to determine whether this procedure's principles could be applied to laparoscopic techniques. The operations were performed in 13 mongrel dogs--first to optimize techniques, and second to observe postoperative function in the surviving animals. The authors found that the dog is a good model in which to study this procedure, and it appears possible to perform a satisfactory Swenson pull-through with the laparoscope.

The treatment of complicated appendicitis in children using peritoneal drainage: results from a public hospital.

During the 2-year period from January 1, 1987 to December 31, 1988, 656 emergency appendectomies were performed on the Pediatric Surgery Service at the Los Angeles County-USC Medical Center. Of these, 398 patients were 12 years of age or less, and 227 appendices (57%) were perforated or gangrenous. The records of 167 of those patients with perforated or gangrenous appendices, treated by a standardized protocol are summarized. The protocol included perioperative antibiotics of gentamicin and clindamycin, appendectomy through a muscle-splitting incision, irrigation of the peritoneal cavity with saline, and peritoneal drainage through the lateral aspect of the wound with skin closure. There was no mortality, and the major complication rate was 8%, with 3% developing intraabdominal abscesses and 5% with bowel obstructions. The minor complication rate was 11%, and included prolonged ileus and prolonged fever, with no wound infections. The average hospital stay was 8.7 days. Our experience suggests that the adopted protocol is reliable for preventing wound infections without increasing the rate of intraabdominal abscesses in an innercity population with particularly advanced stages of appendicitis.

Laparoscopy improves outcomes for pediatric splenectomy.

BACKGROUND: Pediatric laparoscopic splenectomy is a relatively new surgical procedure with a limited number of reports comparing its outcomes to that of the open procedure. The authors have minimized the invasiveness of our procedure by using only three ports and have described the technique as well as compared it with the open method. METHODS: A retrospective review of seven laparoscopic splenectomies (LS) using a three port technique were compared with seven open splenectomies (OS) performed for similar indications at a single children's hospital. RESULTS: The average age in the LS group was 8.7 years compared with 8.9 years for OS, (P value not significant), and the average weights were also similar. The indications for splenectomy were hereditary spherocytosis, idiopathic thrombocytopenic purpura, sickle cell anemia, and splenic cyst. All splenectomies were performed safely with an average estimated blood loss of 41 mL for LS and 34 mL for OS (P value not significant). Operative time averaged 147 minutes for LS and 86 minutes for OS (P < .05). LS patients recovered more rapidly and were discharged home on a median of postoperative day (POD) 2 versus POD 4 for OS (P < .05). LS patients received significantly less total amount of intravenous pain medication with an average of 0.18 mg/kg of morphine sulfate versus 0.8 mg/kg for OS (P< .05). Total hospital charges were higher for LS with an average of $10,899 versus $8,275 for OS (P < .05). CONCLUSIONS: Laparoscopic splenectomy currently is a safe procedure, offering better cosmesis, much less pain, and a shorter hospital stay compared with the traditional open procedure. The more sophisticated equipment and time needed to carry out the procedure led to a modestly increased hospital cost.

The effect of cholecystokinin-octapeptide on the hepatobiliary dysfunction caused by total parenteral nutrition.

PURPOSE: Patients on total parenteral nutrition (TPN) commonly have hepatobiliary dysfunction. Interruption of the enterohepatic circulation (EHC) and gallbladder stasis are part of the pathogenesis. Cholecystokinin-octapeptide (CCK-OP), by emptying the gallbladder, stimulates the EHC. This study was performed to determine whether daily CCK-OP infusions can ameliorate the hepatobiliary dysfunction caused by TPN. METHODS: Rabbits maintained on a standard TPN for 12 days were divided into two groups. One group (n = 6) received daily intravenous doses of CCK-OP, and the other (n = 13) received TPN only. A lab-chow-fed (LCF) group (n = 8) served as controls. The authors studied bile flow and bile acid secretion rates, sulfobromophthalein (BSP) secretion, gallbladder emptying in response to CCK-OP, and liver histology. RESULTS: The LCF group had a bile flow of 82.3 microL/kg/min; that for the TPN-only group was 45.7 microL/kg/min (P < .001). The daily CCK-OP group did not improve more than the TPN-only group, with a bile flow of 45.8 microL/kg/min (P = NS). Bile acid secretion was 0.64 mumol/kg/min for the LCF group, 0.46 for the TPN-only group (P = NS), and 0.46 for the daily CCK-OP group (P = NS). TPN impaired the ability of the gallbladder to empty, and this was restored with daily CCK-OP. In the LCF group, the mean BSP secretion was 81.7% of a 5-mg/kg bolus within 60 minutes, compared with 72.5% in the daily CCK-OP group (P = NS) and 63.5% in the TPN-only group (P < .01). Histological examination of the liver showed that daily CCK-OP produced less periportal inflammation and fibrosis, although all TPN groups had hepatocyte damage in the centrilobular area. CONCLUSION: Stimulation of the EHC with daily CCK-OP infusions during TPN decreased periportal inflammation and fibrosis, maintained gallbladder emptying capacity, and improved organic anion (BSP) secretion, although bile flow and bile acid secretion were not improved, and hepatocyte damage persisted.

Facial asymmetry.

A case of monostotic, mandibular fibrous dysplasia has been described in a 22-year-old Amerasian male, who had facial asymmetry of approximately 5 years' duration. Foci of ill-defined sclerotic, pagetoid calcifications were seen on routine film. The CT scans demonstrated bone facial and slight lingual expansion of bone with thinning of the cortex. A three-dimensional, enhanced CT film showed that this lesion was greater than 4 cm. The clinical, radiologic, and microscopic features of fibrous dysplasia have been described.

Cardiac rehabilitation improves heart rate recovery following peak exercise in children with repaired congenital heart disease.

We assessed heart rate (HR) recovery following peak exercise before and after a 12-week cardiac rehabilitation program in 14 children, 12.1+/-1.8 years of age, with repaired complex congenital heart disease (CHD; 11 with Fontan surgery) and impaired exercise performance. Exercise testing using bicycle ergometry was performed at baseline, after completion of the rehab program and 1.0+/-0.2 years after the baseline test. These data were compared to HR recovery in 15 controls (age, 12.7+/-2.4 years) with CHD (13 with Fontan surgery) with two serial exercise tests at an interval of 1.1+/- 0.3 years. There was no change in peak HR between the two serial tests in either group. Peak VO2 improved in the rehab group (26.3+/-9.6 ml/kg/min at baseline vs 30.9+/-9.6 ml/kg/min after rehab, p=0.01) but remained unchanged in controls on serial testing. One-minute HR recovery (in beats per minute) improved significantly following completion of the rehab program (27+/-15 at baseline vs 40+/-23 after rehab, p=0.01). Partial improvement in 1-minute HR recovery in the rehab group persisted 1 year later (1-minute HR recovery, 35+/-19; p=0.1 compared to baseline). There was no change in 1-minute HR recovery over time in the control group (37+/-16 vs 40+/-13, p = not significant). In conclusion, HR recovery following peak exercise improves in children with CHD after participation in a cardiac rehab program.