Pediatric laryngeal papillomatosis: our experience (three cases)

Three cases of juvenile laryngeal papillomatosis were treated at our hospital between 1984 and 1994. Papillomas were excised in all patients because of upper airway obstruction. Two were given alpha interferon as complementary treatment. Two patients are asymptomatic after years of follow-up and one is still taking alpha interferon.     

Pediatric annular pancreas: twenty years' experience

Characterization of pediatric annular pancreas is provided by this analysis of 24 cases (22 neonates, 1 infant, 1 child). Salient observations include: (1) Presentation is affected by the degree of duodenal obstruction at birth and by coexistent anomalies. (2) Polyhydramnios usually accompanies complete high intestinal obstruction by annular pancreas. (3) Primary biliary interruption was not encountered and jaundice was not unusually prevalent. (4) There is a high incidence of associated anomalies. (5) Duodenal bypass by duodenoenterostomy was employed with excellent results.     

Successful extracorporeal membrane oxygenation (ECMO) support for fulminant community-acquired pneumococcal pneumonia

A case is described of fulminant community-acquired pneumococcal pneumonia in a 16 year old girl with no previous history of respiratory disease or any predisposing factors. She required extracorporeal membrane oxygenation (ECMO) until the diagnosis could be made and appropriate antibiotic therapy established.     

Pediatric penetrating thoracic trauma: a five-year experience

Penetrating thoracic trauma is managed nonoperatively in 85% of adult patients. We hypothesized that similar trauma in children would lead to proportionately more vital tissue damage and a higher rate of operative intervention. The pediatric penetrating thoracic trauma experience of a level one trauma center was analyzed over a five-year period. Data reviewed included circumstances of injury, Pediatric Trauma Score (PTS), interventions performed, and outcome. Of 61 children with thoracic trauma, 13 had penetrating injuries. Of these 13, seven were unintentional (five from firearms); the rest were caused by assaults. Seven patients (54%) underwent thoracotomy or laparotomy. All five patients with a PTS < 8 underwent surgical intervention, whereas only two of the eight patients with a PTS > or = 8 needed surgery (P < 0.05). There was one death. We reached the following conclusions: 1) Children with penetrating thoracic trauma are more likely to require surgical intervention than adults. 2) Penetrating thoracic trauma in children should elicit a thorough search for operative lesions. 3) About half these injuries are unintentional, and thus potentially preventable.     

Invasive pulmonary aspergillosis due to Aspergillus terreus: 12-year experience and review of the literature

A 12-year retrospective analysis was done to identify and evaluate in detail cases of invasive pulmonary aspergillosis (IPA) caused by Aspergillus terreus. We identified 13 A. terreus infections among 133 total cases of confirmed invasive aspergillosis; 11 were IPA and 2 were primary peritoneal infections. Of the 11 patients with IPA, 7 developed neutropenia during hospitalization, and the remaining four were receiving immunosuppressive agents. Ten patients with IPA died; one liver transplantation patient without neutropenia survived after treatment with amphotericin B, itraconazole, and a pulmonary lobectomy. Six patients developed disseminated disease, with the heart the most common extrapulmonary site identified (four patients). These cases demonstrate that IPA caused by A. terreus rapidly progresses in immunocompromised patients receiving amphotericin B and illustrate the need for sensitive diagnostic tests and more effective antifungal agents.     

Pediatric trauma registry: analysis of 1200 cases

OBJECTIVES: We developed the first Spanish Pediatric Trauma Registry in order to collect and evaluate information concerning aspects of injuries in our pediatric population. METHODS: From January'95 to September'97, 28,713 children younger than 16 years were treated in our Hospital for acute injury: 1,200 were admitted and included in our database. Our file-registry consists of 108 data points including: patient identification, type, place and mechanism of injury, pre-hospital care, transport, assessment on admission, severity scores, diagnostic studies, injuries, treatments and morbidity-mortality. RESULTS: Accidents were more frequent in males (69%) than in females. The age-group predominantly was 12-15 years old (34%). Accidents were more frequent in the street (35.3%) than at home (18.7%) or school (14%). Falls and traffic-related accidents were the leading cause of injury (38 and 21.1%, respectively). The 16.7% of cases had Pediatric Trauma Score < or = 8 (n = 201). The 3.6% of this sustained multiple trauma (43 cases with Injury Severity Score > or = 15). Musculoskeletal and head trauma was the most frequent (62 and 42.3%, respectively). Surgical or orthopedic procedures were performed in 678 patients (56.5%). Average length of stay were 4.8 days (range 1-93 days), and functional impairments at discharge were found in 33.9% of patients older than 3 years (n = 338). Mortality rate in our series was 0.5% (n = 6), and 13.9% in children with ISS > or = 15. CONCLUSIONS: The utility of this Registry is to know the epidemiology of our injured pediatric population, to review patient care, to develop prevention programs and to compare results with other centers so potential deficiencies can be identified and corrected.     

Survival of patients with congenital diaphragmatic hernia during the ECMO era: an 11-year experience

The pure TdI-1 polypeptide that blocks miniature endplate potentials (MEPPs) and abolishes or reduces endplate potentials (EPPs) below the action potential threshold was identified from the crude fraction of Tityus discrepans venom. The toxin is a potent reversible non-depolarizing muscle relaxant that blocks more than 95% of the EPP at a 2 microM (0.1 mg/ml) concentration. On a molar basis, TdI-1 is as potent as or more potent than many muscle relaxants since, at the concentration used, the toxin suppressed more than 95% of the EPP. Using matrix-assisted laser desorption time of flight (MALD-TOF) ionization mass spectrometry, TdI-1 was found to have an unusally large mol. wt for a scorpion toxin, close to 48,000. The N-terminal sequence of the first 23 residues of TdI-1 was also determined. The fragment differs from the N-terminal sequences of all 140 peptidic scorpion toxins found in the SWISSPROT and PIR databases using the search engine of the felix.EMBL-Heidelberg.de computer (European Molecular Biology Laboratory, Heidelberg, Germany.     

Pediatric all-terrain vehicle trauma: a 5-year statewide experience

BACKGROUND/PURPOSE: This is a retrospective review of the pediatric all-terrain vehicle trauma victims who presented to the five major trauma centers serving the state of West Virginia during the 5-year period from January 1991 to December 1995. The purpose of this research is to characterize the nature of the injuries and the individuals injured to better appreciate the magnitude of the problem of ATV-related injuries in the pediatric population. METHODS: This study is a retrospective review of these 218 consecutive pediatric patients from trauma registry data and their medical records. RESULTS: Two hundred eighteen patients between the ages of 2 years and 16 years presented during the study period. Boys outnumbered girls three to one. The average Injury Severity Score (ISS) was 8.76, the average Glasgow Coma Score (GCS) was 14.4, and the average Trauma Score (TS) was 15.2. The most common injuries were orthopedic followed by head and facial injuries. The majority of the children did not wear helmets, and their injuries resulted in an average hospital length of stay of 4.3 days. Thirty-eight percent of the children required surgery. There were a total of four deaths for a mortality rate of 1.8%. The estimated total hospitalization cost for the 218 patients was $1,918,400.00. CONCLUSIONS: All-terrain vehicle-related trauma remains an ongoing safety concern facing society today. Every physician who cares for children should address this important issue when talking to children and parents about safety issues and injury prevention.     

Clinical cardiotoxicity following anthracycline treatment for childhood cancer: the Pediatric Oncology Group experience

PURPOSE: To determine the incidence of clinical cardiotoxicity from anthracycline chemotherapy in children with cancer and to identify associated risk factors. PATIENTS AND METHODS: The study population consisted of 6,493 children with cancer who had received anthracycline chemotherapy on Pediatric Oncology Group (POG) protocols from 1974 to 1990. Cardiotoxicity, defined as congestive heart failure not due to other causes, abnormal measurements of cardiac function that prompted discontinuation of therapy, or sudden death from presumed cardiac causes, was determined by a review of protocol records. RESULTS: Cardiotoxicity was confirmed in 106 patients (1.6%): 58 had congestive heart failure, 43 had changes in measures of cardiac function that prompted the discontinuation of therapy, and five died suddenly from presumed cardiac causes. In a multivariate analysis, factors that contributed to the relative risk (RR) of toxicity were a cumulative anthracycline dose > or = 550 mg/m2 of body-surface area (RR = 5.2), maximal dose > or = 50 mg/m2 (RR = 2.8), female sex (RR = 1.9), black race (RR = 1.7), presence of trisomy 21 (RR = 3.4), and exposure to amsacrine (RR = 2.6). Cardiotoxicity within 1 year after the completion of anthracycline treatment (early cardiotoxicity) represented 89.5% of all cases. CONCLUSION: Early clinical cardiotoxicity in children treated with anthracycline is rare. A high maximal dose, or cumulative dose of anthracycline, female sex, black race, presence of trisomy 21, and treatment with amsacrine increase the risk for anthracycline-associated cardiotoxicity.     

Traumatic rupture of hydatid cysts: a 12-year experience from an endemic region

Adrenomedullin (AM), a potent hypotensive peptide, is produced in numerous tissues including adrenal gland, kidney, brain and pituitary gland, where it acts to modify sodium homeostasis. Central AM administration dose-dependently inhibits sodium appetite. AM antisense oligonucleotide treatment significantly lowered peptide content in the hypothalamic paraventricular (PVN) nucleus and exaggerated the consumption of sodium. These results support a physiologic role for adrenomedullin gene products in the central regulation of sodium homeostasis.     

Internet-based support for bioscience research: a collaborative genome center for human chromosome 12

This paper describes an approach that provides Internet-based support for a genome center to map human chromosome 12, as a collaboration between laboratories at the Albert Einstein College of Medicine in Bronx, New York, and the Yale University School of Medicine in New Haven, Connecticut. Informatics is well established as an important enabling technology within the genome mapping community. The goal of this paper is to use the chromosome 12 project as a case study to introduce a medical informatics audience to certain issues involved in genome informatics and in the Internet-based support of collaborative bioscience research. Central to the approach described is a shared database (DB/12) with Macintosh clients in the participating laboratories running the 4th Dimension database program as a user-friendly front end, and a Sun SPARCstation-2 server running Sybase. The central component of the database stores information about yeast artificial chromosomes (YACs), each containing a segment of human DNA from chromosome 12 to which genome markers have been mapped, such that an overlapping set of YACs (called a "contig") can be identified, along with an ordering of the markers. The approach also includes 1) a map assembly tool developed to help biologists interpret their data, proposing a ranked set of candidate maps, 2) the integration of DB/12 with external databases and tools, and 3) the dissemination of the results. This paper discusses several of the lessons learned that apply to many other areas of bioscience, and the potential role for the field of medical informatics in helping to provide such support.     

Congenital diaphragmatic hernia: experience with preoperative stabilization and delayed surgery without ECMO and inhaled nitric oxide

Despite many advances in the management of congenital diaphragmatic hernia (CDH), the condition carries a mortality rate of 40-50% usually consequent to pulmonary hypoplasia and/or persistent pulmonary hypertension. Several centers have reported improved survival with preoperative stabilization and delayed surgery, which is now an accepted method of management. This is a retrospective analysis of all neonates with respiratory distress at birth due to CDH who were treated at our institution with neither extracorporeal membrane oxygenation nor nitric oxide being used. The medical records of all neonates with CDH and respiratory distress at birth who were treated at this institution from August 1, 1992 through March 1, 1997 were reviewed. There were 21 patients, 11 male and 10 female. There were 17 full-term and 4 premature infants; two premature infants at 30 and 34 weeks' gestation were not resuscitated because of severe associated congenital anomalies. Surgery was performed from 5 to 144 hr (mean 45 hr) in 18 infants. One infant died during preoperative stabilization from severe pulmonary hypoplasia and pulmonary hypertension and one infant died postoperatively from the same conditions. Seventeen of 19 infants (89.5%) survived and were discharged home. Three infants (17.6%) who failed to thrive due to severe gastroesophageal reflux (GER) required fundoplication. Eleven infants (64.7%) who had sepsis proven by blood culture responded satisfactorily to appropriate antibiotics. Preoperative stabilization and delayed surgery has been a satisfactory form of management in our series. The significant complication was sepsis, which must be addressed.     

Pancreatic-biliary ascariasis: experience of 300 cases

BACKGROUND: Infestation with Ascaris lumbricoides is seen worldwide. Recently, there has been much interest in the pancreatic-biliary complications of Ascaris infection. In this study, we present our experience of 300 patients seen in a tertiary referral center. MATERIALS AND METHODS: Case charts of patients seen in the Department of Gastroenterology, University of Damascus, Syria, were analyzed, retrospectively, over a 5-yr period (September of 1988 to August of 1993). During this period, 1666 endoscopic retrograde cholangiopancreatographic studies were performed and pancreatic-biliary ascariasis was diagnosed in 300 patients (18%). RESULTS: The most common presenting symptom was abdominal pain, seen in 98% of patients (294 patients). Complications observed were ascending cholangitis (48 patients; 16%), acute pancreatitis (13 patients; 4.3%), and obstructive jaundice (4 patients; 1.3%). History of worm emesis was present in 25% of patients (76 patients). Most patients (240 patients; 80%) had previously undergone a cholecystectomy or an endoscopic sphincterotomy (232 patients; 77%). Worms were successfully extracted endoscopically in all except two patients, and there were no procedure-related complications. CONCLUSIONS: In endemic countries, ascariasis should be suspected in patients with pancreatic-biliary disease, especially if a cholecystectomy or sphincterotomy has been performed in the past. Endoscopic management results in rapid resolution of symptoms and prevents development of complications.     

Sturge-Weber syndrome: experience with 14 cases

We report a first documented case in Senegal with simple transposition of the great arteries diagnosed in a 2 months old girl treated by Rashkind atrioseptostomy. Our patient benefited from clinical examination, ECG (15 derivations), chest X ray and standard laboratory tests. Pulsed-Doppler, two dimensional and TM echocardiography have been performed with an ATL MK 600 echocardiograph. Cardiac catheterism, angiocardiography and Rashkind procedure have been realized in our Department. These data are discussed and compared to the literature. At admission this patient presents with major cyanosis and polypnea. At examination, there is a 3/6 murmur at the left sternal border and a subclavicular continuous murmur. Laboratory tests showed metabolic acidosis and severe hypoxemia. Chest x-ray showed a cardio-thoracic ratio at 0.64 with increased pulmonary vascular markings. ECG showed right ventricular hypertrophy. Echocardiography-Doppler revealed ventriculo-arterial discordance with restrictive atrial septal defect and persistent ductus arteriosus. Rashkind procedure was followed by an increased aortic saturation. After 6 weeks there was an improvement of cyanosis and cardiac failure. Diagnosis of transposition of the great arteries is actually easier with development of ultrasonography which is useful when performed by experienced cardiologist. Spontaneous prognosis of this malformation is very poor. Rashkind atrioseptostomy is an important step for the initial treatment of transposition of the great arteries in terms of survival before open heart surgery.     

Collagenous colitis: presentation of 12 cases

Collagenous colitis (CC) is a cause of chronic aqueous diarrhea with normal radiologic study and endoscopic appearance of the colonic mucosa. Histologically, it is defined by the presence of a thickened subepithelial collagenous band and inflammatory changes of the mucosa. The cause of CC is currently unknown, although several mechanisms have been proposed, such as an inflammatory, autoimmune, origin, disregulation in collagen synthesis, plasma vasculosis and a possible role of bacterial or drug toxins. The clinicopathological data of 12 patients (9 females and 3 males) with a mean age of 52.4 years diagnosed by histologic criteria are presented. Aqueous diarrhea was observed in all the patients with a mean number of 5.4 stools/day during a time period between 3 weeks and 10 years (mean, 14.7 months). In a 6 patients allergies and/or associated diseases, mainly rheumatologic diseases were found. Laboratory and endoscopic data were normal or unspecific, with colon biopsy being carried out in all the patients. Several treatments were tested with good response with sulphasalazine derivatives, corticoids, antibiotics, and mebeverine, with no solution to the diarrhea in 2 patients. A review of the literature is also provided.     

The Zavanelli maneuver: 12 years of recorded experience

OBJECTIVE: To evaluate the applicability and safety of the Zavanelli maneuver (manual return to the vagina of the partially born but undeliverable fetus with subsequent extraction by cesarean). DATA SOURCES: MEDLINE computer database, Science Citation Index, and Index Medicus, 1985 through 1997 using the search terms Zavanelli maneuver, cephalic replacement, abdominal rescue, head entrapment, and shoulder dystocia. METHOD OF STUDY SELECTION: Efforts to return 103 partially born but undeliverable fetuses to the uterus have been reported in 30 publications since the first case was reported in 1985. All cases were accepted for review, successful or not. TABULATION, INTEGRATION, AND RESULTS: Clinically relevant features were extracted from each case report. In 92 cases, fetuses presented by the vertex. Cephalic replacement was successful in 84 of those. In 11 cases, the fetus presented by the breech. Podalic replacement was successful in all of those. Seven women suffered tissue-disruptive trauma. There were no maternal deaths or fetal injuries ascribed to the maneuver. CONCLUSION: The 92% overall success rate is remarkable for a new procedure; even more noteworthy, in most cases it was applied by operators with no experience with it, and for cephalic replacement, it was used only after conventional maneuvers had failed. Using it early in the treatment of obstructed partial vaginal delivery is recommended.     

Pediatric hepatic trauma: does clinical course support intensive care unit stay?

PURPOSE: The objective of this study is to determine if grade of liver injury predicts outcome after blunt hepatic trauma in children and to initiate analysis of current management practices to optimize resource utilization without compromising patient care. METHODS: A retrospective review of 36 children who had blunt hepatic trauma treated at a pediatric trauma center from 1989 to present was performed. Hepatic injuries graded (AAST Organ Injury Scaling) ranged from grade I to IV. Injury Severity Score (ISS), Glasgow Coma Score (GCS), transfusion requirements, liver transaminase levels, associated injuries, intensive care unit (ICU) length of stay, and survival were analyzed. RESULTS: Mean (+/-SEM) age was 6.6+/-0.8 years, mean grade of hepatic injury was 2.4+/-0.2, mean ISS was 17+/-2.6, mean GCS was 13+/-1, and mean transfusion was 15.4 mL/kg of packed red blood cells (PRBC). There were three deaths with a mean ISS of 59+/-9 and a mean GCS of 3+/-0. Death was not associated with a high-grade liver injury, survivors versus nonsurvivors, 2.3+/-0.2 versus 2.7+/-0.3, but was associated with ISS, 13+/-1.4 versus 59+/-9 (P = .005) and GCS, 14+/-1 versus 3+/-0 (P = .005). Only one patient (grade III, ISS = 43) underwent surgery. There were no differences in mean ISS or GCS between grades I to IV patients. The hepatic injury grades of patients requiring transfusion versus no transfusion were significantly different, 3.4+/-0.2 versus 2.2+/-0.2 (P = 0.04). Abused patients had high-grade hepatic injuries and significant laboratory and clinical findings. Alanine aminotransferase (ALT) and aspartate aminotransferase (AST) were significantly higher in grade III and IV injuries than in grades I and II, 1,157+/-320 versus 333+/-61 (P= .02) and 1,176+/-299 versus 516+/-86 (P= .04), respectively. No children with grade I or II injury had a transfusion requirement or surgical intervention. There were no liver-related complications. CONCLUSIONS: Mortality and morbidity rates in pediatric liver injuries, grades I to IV, correlate with associated injuries not the degree of hepatic damage. ALT, AST, and transfusion requirements are significantly related to degree of liver injury. Low-grade and isolated high-grade liver injuries seldom require transfusion. Blunt liver trauma rarely requires surgical intervention. In retrospect, the need for expensive ICU observation for low-grade and isolated high-grade hepatic injuries is questionably warranted.     

Endodermal sinus tumor: report of 12 cases

Twelve cases of endodermal sinus tumor were reviewed. There were 10 females and 2 males with a median age at presentation of 3 years. The primary site was sacrococcygeal in 4 patients, vaginal in 3, retroperitoneal in 2, and testicular, ovarian and left chest wall in one each. The diagnosis rested on histopathological examination and elevation of serum alfa feto protein levels (median 46,200 ng/ml). Two patients had Stage I disease, 9 had Stage III and one had Stage IV disease. Patients were managed by surgery and chemotherapy (BVP regime). All patients on BVP (even those lost at later stages), had achieved clinical remission with the first cycle of treatment.