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Most patients with cystic fibrosis require oral administration of pancreatic enzymes to treat pancreatic insufficiency. Recent use of higher-strength enzyme preparations in large doses has been found to be associated with fibrotic strictures of the colon. We report a case of pancolonic fibrosis due to pancreatic enzyme use.

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One to two percent of patients who have choledochal cysts present with cyst rupture and bile peritonitis. Reported cases have been managed with external drainage of the cyst followed by a second procedure to excise the cyst and reconstruct the biliary tract. The authors report two cases of ruptured choledochal cyst treated with primary cyst excision and biliary drainage. The satisfactory outcome of these patients suggests that this is the preferred management.

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The authors report the case of an 8-year-old boy who had isolated microgastria. The patient had required lifelong jejunal tube feeding because of inadequate gastric volume and intractable gastroesophageal reflux. Gastric augmentation was performed with a Roux-en-Y jejunal reservoir (Hunt-Lawrence pouch), with good results.

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Platelet-activating factor (PAF) is an important endogenous mediator of neonatal necrotizing enterocolitis (NEC). Injection of PAF into weanling and adult rats causes ischemic bowel necrosis that is morphologically similar to NEC. The purpose of this study was to adapt the PAF model of intestinal injury to the suckling rat and to attempt to alter susceptibility to PAF-induced bowel necrosis by early weaning and formula feeds. At ages 15 to 20 days, rat pups were selected to be weaned to either formula or 5% dextrose or to nurse ad lib (total n = 54). At ages 16, 18, 20, 21, 23, or 25 days of life, animals received PAF (50 micrograms/kg) and endotoxin (1 mg/kg) by intraperitoneal injection. Animals were sacrificed 2 h after injection. Intestinal samples were submitted to be graded by a pathologist in a blinded fashion. Injury scores ranged from 0 to 10, based on the percentage of villous necrosis. Prior to age 20 days, minimal histologic injury was present (mean scores on days 16, 18 = 1.7 +/- 0.9, 1.7 +/- 0.6). Combined injury scores for weaned and nursed animals on days 20 and 23 were significantly greater than on days 16 and 18 (p = .0001). Histologic injury in the dextrose group was significantly less than the formula-fed group on day 21 and greater on day 25. Suckling rats showed resistance to PAF-induced bowel necrosis prior to 20 days of age, during the middle of the weaning period. Early weaning to formula did not alter susceptibility to injury, which suggests that PAF-acetylhydrolase from breast milk does not confer this resistance to PAF.

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UNLABELLED: Necrotizing fasciitis (NF) is a bacterial infection of the soft tissues with a fulminant course and a high mortality rate. The authors performed a review to define the diagnosis, bacteriology, and management of NF in the pediatric population. This report of 20 cases treated over 18 years represents the largest reported pediatric experience. These infections were attributable to secondary infection of varicella lesions (5), omphalitis (4), extremity lesions (4), perineal infections (3), head and neck lesions (2), inguinal herniorrhapy (1), and breast abscess (1). Nineteen of the 20 children were healthy, without chronic disease or immunosuppression. All patients presented with an altered sensorium and signs of systemic toxicity. Fever (40%), tachycardia (70%), and abnormal white blood cell count (50%) were not uniformly present. There was marked tissue edema in all patients, with a characteristic peau d'orange appearance in 18. Seven infections were caused by streptococcus; the remainder were polymicrobial, involving multiple aerobes and anaerobes. Initial gram stain was of limited utility; in 14 of 19 cases the result was negative or showed only one of many organisms present. Fifteen patients survived and five died. All survivors underwent aggressive surgical debridement within 3 hours of admission. The survivors required of a mean of 3.8 operations. Fascial excision of up to 35% of total body surface area was required. One patient required amputation, two had colostomies, and six required extensive skin grafting for reconstruction. All five patients who died had delayed initial management. CONCLUSION: NF is a serious cause of death in previously healthy children. The diagnosis should be considered in the presence of any soft tissue infection presenting with signs of toxicity and marked wound edema, even in the absence of fever or abnormal white blood cell count. Immediate surgical debridement and coverage with penicillin, an aminoglycoside, and metronidazole are essential. Subsequent changes in antibiotics should be based on culture data because gram stain results are not reliable. More than one operation is required in almost all cases.

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Traumatic solid organ injuries are easily recognizable on computed tomography (CT) scans and usually are treated nonoperatively. Small bowel injuries may be difficult to diagnose and require prompt operation. This study was done to assess the role of clinical examination versus diagnostic tests in evaluating these injuries. The medical records of all pediatric (< or = 18 years old) patients treated at a pediatric trauma center from 1984 to 1995 were reviewed. Statistical analysis was performed using SAS software, with P values of less than .05 considered significant. Small bowel injury occurred in 48 patients (21 blunt, 27 penetrating). Most blunt injuries were automobile-related (11 patients) or attributable to recreational activities (4) or bicycle accidents (2). Penetrating injuries were primarily caused by assaults with guns (21) or knives (4). All conscious patients with small bowel injury had abnormal physical examination findings at the time of presentation. Nineteen patients had generalized peritonitis, and 14 had localized abdominal tenderness. The serum amylase level was abnormal in 2 of 18 cases. Abdominal CT scans were obtained in six patients and showed the injury in only three. Peritoneal lavage (DPL), performed in 10 patients, led to the diagnosis in five. There was no significant difference in the complication rate (30%) between patients operated on immediately because of a diagnostic test result and those operated on later, after a period of clinical observation (P = 1.0, Fisher's Exact test). Associated injuries were common (60%) among both blunt and penetrating cases. In this nonoperative era of pediatric trauma care, small bowel injury is best diagnosed clinically. The physical examination is 100% sensitive in the conscious patient, and specificity is achieved by serial examination. Serum amylase, CT scan, and DPL are not reliable diagnostic tests to exclude these injuries. Patients can be observed until physical findings suggest bowel injury without increased morbidity. Associated injuries are common; thus, patients are best treated where multidisciplinary support is available.

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Necrotizing enterocolitis (NEC) develops primarily after the onset of enteral feeds in the premature infant. The purpose of this study was to evaluate the influence of intestinal luminal nutrients on histologic injury and the oxidant response in a rat model of NEC. On postnatal Days 10 and 35, Sprague-Dawley rats (total n = 81) underwent abdominal laparotomy. A control group received sham-injury only. The ischemia groups received a single intraluminal injection of 0.25 ml (Day 10) or 1.0 ml (Day 35) of lactose (8.6 g/dl), casein (2.2 g/dl), corn oil (4.4 g/dl), or infant formula (Similac; 20 g/dl). After injection of the nutrient solutions, ischemia groups underwent mesenteric occlusion for 1 hr and intraluminal injection of platelet-activating factor (50 microgram/kg). Necropsies were performed after 6 hr or at demise. Intestinal samples were taken for histology, total glutathione (GSH; an antioxidant), and conjugated dienes (a lipid peroxidation product). Histologic injury was scored from 0 (normal) to 5 (transmural necrosis). Microscopic injury scores in the oil group were significantly higher than the casein group (P < 0.05) and trended toward being higher in the formula group (P = 0.085) at age 10 days. Total GSH activity was significantly higher in the sham groups than all ischemia groups on Day 10 (P < 0.001) and than the corn oil group on Day 35 (P < 0.05). GSH activity did not differ among ischemia groups. Conjugated diene concentrations were significantly higher in the casein group than the lactose and sham groups at age 10 days (P < 0.05) only. We conclude that intraluminal lipids may augment intestinal ischemic injury in the newborn (age 10 days) but not the weanling rat. While oxygen-free radicals were present during injury, lipid peroxidation from oxygen radicals was not responsible for this increase in histologic injury.

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Focal dermal hypoplasia is a rare, X-linked dominant syndrome characterized by dysplasia of the skin, skeleton, and central nervous system. We report an infant who was born with severe focal dermal hypoplasia and an epigastric hernia. Operative timing and approach to abdominal wall defects in the presence of severe cutaneous dysplasia are discussed.

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The human premature newborn is susceptible to necrotizing enterocolitis (NEC) in the first 1 to 3 weeks of life, a time when the gastrointestinal tract is structurally and functionally premature. Studies of NEC are hampered by the lack of a standard, reproducible model in newborn animals. The purpose of this study was to produce a model for intestinal ischemic injury in newborn rats. On Days 14, 18, 22, and 26 of life, newborn rats (10/day) were subjected to 1 hr of superior mesenteric artery occlusion with a microaneurysm clip. Platelet activating factor (PAF, 50 micrograms/kg) was injected into the lumen of the proximal small intestine after occlusion was initiated. Control animals (10/day) underwent sham laparotomy on Days 14, 18, 22, and 26. Animals were autopsied upon demise (7.6 +/- 0.7 hr) or at 24 hr. The intestine was inspected for gross ischemic changes and samples were taken for histology and myeloperoxidase (MPO, an index of neutrophil infiltration). Ischemic injury was graded in a blinded fashion, by a pathologist, using a scale from 0 to 4 (0, no injury; 4, full-thickness necrosis). All animals in the experimental groups had evidence of histologic injury (mean +/- SEM) on Days 14 (1.0 +/- 0.0), 18 (2.5 +/- 0.5), 22 (3.6 +/- 0.3), and 26 (3.1 +/- 0.5). The sham-operated control animals had no injury (P < 0.0001). MPO levels (U/g protein) on Days 18 (27.2 +/- 1.7 vs 13.9 +/- 2.3), 22 (40.9 +/- 5.4 vs 7.6 +/- 0.8), and 26 (29.3 +/- 4.4 vs 7.6 +/- 1.0) were significantly higher in experimental groups vs controls (P < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

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When traumatic abdominal injury and acute appendicitis occur within a short period of time, the diagnosis can be difficult and may result in a delay in treatment of the appendicitis. We report the case of a four-year-old boy with documented intraabdominal injury from a motor vehicle crash who developed appendicitis while hospitalized and recovering from his injuries.

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Premature infants are susceptible to intestinal ischemia during the newborn period when their intestinal tracts are functionally and structurally immature. Studies have shown that exogenous glucocorticoids hasten intestinal maturation. We investigated the effects of hydrocortisone on platelet activating factor (PAF)-induced intestinal ischemia in the neonatal rat. On Postnatal Days 7-11, Sprague-Dawley rats were given intraperitoneal (ip) injections of either saline (SAL) or hydrocortisone (HC; 50 mg/kg total). On Day 12, rats were injected with either PAF (2 micrograms/kg) or an equal volume of saline. After 2 hr the rats were sacrificed and sections were taken for histology. The remaining intestine was analyzed for maltase, lactase, myeloperoxidase (MPO), and xanthine oxidase (XO). Experimental groups were as follows: SAL (N = 8), received saline only; SAL+PAF (N = 8), received saline plus PAF; HC (N = 3), received hydrocortisone+saline; and HC+PAF (N = 5), received hydrocortisone plus PAF. XO was significantly decreased (P < 0.001) in the hydrocortisone-treated groups (HC + SAL = 16.36 +/- 18.42 units/g protein, HC + PAF = 17.33 +/- 9.06 units/g protein) vs the controls (SAL only = 108.90 +/- 20.24 units g/protein, SAL + PAF = 145.77 21.28 units/g protein). MPO was not significantly elevated in SAL + PAF (4.60 +/- 0.95 units/g protein) vs HC + PAF (2.18 +/- 0.80 units/g protein) in this study. Maltase was significantly elevated (P < 0.001) in the HC + PAF (241.46 +/- 40.6 mole/min/g protein) and HC + SAL (152.78 +/- 16.35 mole/min/g protein) vs saline only (28.35 +/- 5.77 mole/min/g protein and SAL + PAF (37.29 +/- 8.70 mole/min/g protein. Animals (7/8) in the SAL + PAF group developed ischemia by inspection and histologic exam.(ABSTRACT TRUNCATED AT 250 WORDS)

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Recent evidence has suggested that the immaturity of the neonatal intestine may play a key role in the development of ischemic injury. However, relatively little data exist on the susceptibility of the neonatal intestine to ischemic injury at various ages especially in the fed versus fasted states. In this study, the levels of xanthine oxidase ([XO] an enzyme which is a known, major source of free radicals in postischemic tissue) and myeloperoxidase ([MPO] an index of tissue neutrophil infiltration) were measured in 1-, 5-, 10-, 15-, and 20-day-old Sprague-Dawley rats. Rats were divided into fed (n = 8/day) and fasted (n = 8/day) groups 4 hours prior to sacrifice. The entire small intestine was removed and divided into five segments: the duodenum, proximal jejunum, distal jejunum, proximal ileum, and distal ileum. The specimens were homogenized and assayed for XO and MPO levels. A significant increase in XO was observed in the fasted animals compared to the fed animals on all days. Peak levels in XO were observed in both groups from day 5 to 10. MPO levels were significantly higher in the fasted versus fed animals on day 1. MPO levels decreased as the animals aged. These data demonstrate dramatic differences in the levels of inflammatory enzymes of the newborn rat in the fed versus fasted states. Also, marked variations with age are seen in both XO and MPO. Whether the XO and MPO levels present at the time of ischemic insult affect severity of injury remains to be seen.

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Intravascular intrauterine transfusion allows a more sophisticated and exact approach to the management of severe Rh hemolytic disease. This technique involves direct manipulation of the fetal umbilical vessels; its hazards include umbilical cord trauma and thrombosis or emboli. The consequences of such events in utero are largely unknown. In this case necrotizing enterocolitis occurred in a full-term infant after three intrauterine intravascular transfusions.

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Injuries resulting from falls from heights still constitute a significant portion of urban trauma. At this pediatric trauma center, 70 children were admitted from 1985 to 1988 after sustaining a fall of 10 feet or greater or at least one story. The mean patient age was 5 years and 68% of the patients were boys. Seventy-eight percent of falls occurred from 2 stories or less and usually took place at or near the home. Most patients sustained a single major injury and all survived. The majority of injuries involved the head or skeleton and residual functional deficits were uncommon. The incidence of falls from heights has remained high in urban areas despite public education and building codes that require window guards and safety rails.

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Although most children who die of liver malignancies do so as the result of complications of pulmonary metastases, little has been published regarding the efficacy of surgically excising such lesions. To the 12 previously reported cases of children who have undergone excision of pulmonary metastases of hepatic tumors, are added 5, 4 with hepatoblastoma and 1 with hepatocellular carcinoma. Total excision of a primary hepatic tumor leads to survival much more frequently than does incomplete excision. No patient had metastases at diagnosis. The length of time between resection of the primary tumor and the development of pulmonary disease resistant to chemotherapy is available for 9 of the 17 children; it was under 6 months for the 2 who died but over 6 months for the 7 who survived. Postoperative alpha-fetoprotein (AFP) levels accurately predicted the development of metastases in our 5 patients. Resection of metastases benefitted the 4 whose AFP levels had declined to less than 25 ng/mL following initial chemotherapy and who underwent operation before their levels increased above 1,000 ng/mL. They are alive and free of disease 4 to 83 months following excision of their lesions. Resection did not benefit the 1 nonsurvivor whose AFP level fell only to 5,000 ng/mL before beginning to increase, eventually reaching 58,000 ng/mL at the time of operation. Incomplete resection of metastases unresponsive to chemotherapy predictably leads to death. Multiple thoracotomies were successful in achieving the long-term survival of 4 children in this series.(ABSTRACT TRUNCATED AT 250 WORDS)

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A large pericardial effusion was discovered in an asymptomatic 12-year-old boy admitted for an elective orthopedic procedure. On physical examination, heart rate was 96 and blood pressure was 130/70 without paradox. The neck veins were not distended, but heart tones were distant. Chest roentgenogram (CXR) showed an enlarged cardiac silhouette. Echocardiogram showed a massive pericardial effusion compressing the right atrium, with depressed ventricular contractility. Pericardiocentesis yielded 450 mL of chylous fluid. A percutaneous pericardial drain was placed and drained another 400 mL of chyle. Pericardial fluid reaccumulated even though the patient was on a low-fat diet, and 1 week after admission left thoracotomy was performed with partial pericardiectomy and pericardial window. There was 1 L of chyle in the pericardial sac; frozen section of the pericardium showed lymphangiectasia. Chest tube drainage diminished rapidly and the patient was discharged. Follow-up CXR at 1 week showed fluid in both pleural spaces requiring bilateral tube thoracostomies again draining chyle. Even with total parenteral nutrition (TPN), 500 mL/d of chyle drained from the pleural tubes. Right thoracotomy with ligation of the thoracic duct was performed after 1 week of TPN. Pleural drainage abruptly dropped, and there has been no reaccumulation in either the pleural spaces or pericardium at 6-month follow-up. This case dramatically supports early thoracic duct ligation and partial pericardiectomy as the treatment of choice for primary massive chylopericardium.

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Necrotizing enterocolitis is the most common gastrointestinal emergency in the newborn. The syndrome strikes premature infants during the first 2 weeks of life. Abdominal distention, lethargy, and feeding intolerance are early signs of NEC that may progress to gastrointestinal bleeding and hemodynamic instability. The radiographic hallmark of NEC is pneumatosis intestinalis (air in the bowel wall). The ileum and colon are the usual sites of crepitant intestinal necrosis, leading frequently to perforation. In spite of appropriate medical therapy, about half of the infants with NEC develop intestinal gangrene or perforation and require surgery, consisting of bowel resection and enterostomy formation. The most common late complication, intestinal stricture, occurs in 15 to 35 per cent of recovered infants. Overall mortality from NEC ranges from 20 to 40 per cent. The etiology of NEC is poorly understood and is considered to be multifactorial, related to ischemia, bacterial colonization, and formula feedings in a susceptible infant. Future progress in the treatment of NEC may be achieved by earlier detection of necrosis, modification of gastrointestinal flora, or by bolstering the deficient gastrointestinal immune mechanisms of the premature neonate.

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To investigate whether radiographic findings could be used to predict pan-necrosis (necrosis of 75% or more of the jejunum, ileum, and colon), we reviewed the serial abdominal radiographs and clinical records of 147 infants with necrotizing enterocolitis. Pan-necrosis occurred in 29 infants (20%) and was always fatal. The presence and degree of pneumatosis intestinalis, pneumoperitoneum and portal venous gas were studied. Pan-necrosis occurred least frequently in infants with mild or moderate pneumatosis intestinalis (8% and 12%, respectively), and with moderate frequency in those with pneumoperitoneum (33%). Pan-necrosis was found most frequently in infants with severe pneumatosis intestinalis (56%), portal venous gas (61%), or the combination of severe pneumatosis intestinalis and portal venous gas (79%). The mortality rates in these three groups were 63%, 65%, and 86%, respectively. Thus, infants with the worst outcome (those who develop pan-necrosis) can be identified on plain films by detection of severe pneumatosis intestinalis and portal venous gas.

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Resection and enterostomy are the standard operative procedures for necrotizing enterocolitis (NEC). In order to compare the results of two different methods of enterostomy, a study was carried out in 100 infants with NEC who underwent enterostomy formation and closure. A single surgeon at each of the two collaborating institutions conducted the majority of operations. Level of enterostomy was jejunum in 10, ileum in 75, and colon in 15. Type of enterostomy was separate stomas (usually brought out side by side) in 50, Mikulicz enterostomy in 39, single stoma with Hartmann's pouch in 10, and loop colostomy in 1. Complications of enterostomy formation occurred in 24 infants (24%). When infants with separate stomas were compared with those with the Mikulicz enterostomy, there was no difference in the rate of stomal or wound complications. The separate stomas had a higher rate of stricture formation in the distal bowel (36% v 18%), which may be accounted for by earlier reestablishment of intestinal continuity in the Mikulicz group. Both methods exteriorized the bowel ends close to one another, which was advantageous because subsequent closure was usually performed without a formal laparotomy. After enterostomy closure, 17 (17%) infants had complications. There was no difference in complication rate between early (before 3 months or under 2.5 kg) v late closure, or between closure of the Mikulicz enterostomy v separate stomas (although the Mikulicz enterostomy closure was accomplished more rapidly than closure of separate stomas). Morbidity was unrelated to level of enterostomy, type of enterostomy, maturing the stoma, bringing it through a separate incision, or age or weight of the infant at closure.(ABSTRACT TRUNCATED AT 250 WORDS)

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