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When comparing firearm fatalities for children under 16 years of age before and after handgun legislation enactment in Maryland, firearm fatalities increased overall. A decrease in accidental deaths in the home perhaps reflects a response to public education and awareness. More aggressive handgun legislation is imperative to reverse this public health trend.

Nephroblastomatosis (NB), a persistence of abnormal embryonal renal tissue beyond 36 weeks' gestation, is often associated with Wilms' tumor. The exact relationship of NB to the development of Wilms' tumor is unclear. Four cases are presented that elucidate the entire morphological spectrum of this disease. Analyses of these cases suggest these conclusions: (1) the NB complex is a spectrum of lesions from benign multifocal nodular renal blastema, resembling residual nephrogenic zones of immature fetal kidney, to Wilms' tumor; (2) infantile NB is a premalignant variant of Wilms' tumor with a favorable outcome usually, when treated early; (3) neonatal nephromegaly requires a complete evaluation and follow-up imaging; persistence mandates biopsy; (4) "second-look" laparotomy is unnecessary with state-of-the-art imaging; and (5) standardization of terminology is essential.

An increase in the awareness of the problem of gunshot fatalities in children has occurred nationwide over the last year. Unintentional firearm deaths are more common among children and young adults. These deaths may be addressed by preventive measures beyond those available for pediatric firearm suicide and homicide. This study focuses on the incidence of fatal gunshot injuries in children under 16 years of age during the years 1979 through 1987 in our state. Over the past 9 years 132 children (0 to 16 years) were shot and killed as a result of firearm injuries (M:F ratio, 3:1). No difference in absolute number in racial distribution existed. Deaths were classified as homicide, 61 (46%); accidental, 33 (25%); suicide, 29 (22%); undetermined, 7 (5%); and other, 2 (1.5%). Over 70% of these fatal injuries occurred in the home environment. Types of weapons involved included handguns (48%), shotguns (22%), rifles (17%), unspecified weapon (12%), and air rifle (1%). In a large number of cases, guns were found in the home unsecured. The perpetrator was known by the victim in 64 instances, while unknown in 27. Thirty-nine self-inflicted wounds and two shotgun blasts in the line of fire accounted for the remaining deaths. The most common anatomical injury and ultimate cause of death was cranial central nervous system (CNS) (62%), followed by chest/mediastinum (20%), abdomen (10%), and other (8%). A child who has sustained a firearm injury is more likely to know the perpetrator, be killed in the home by a readily available unsecured firearm, and die from severe head injury.

Twenty-one children with human immunodeficiency virus (HIV) infection required surgical intervention during the course of their disease. There were 11 females and 10 males (age range, 3 months to 6 years). The children underwent 54 operative procedures after diagnosis of their disease. These included placement of central venous catheter (23 patients), open lung biopsy (11), incision and drainage of perirectal abscess (4), incision and drainage of soft tissue abscess (5), myringotomy (2), diverting colostomy (3), Nissen fundoplication (1), and other (5). All 21 patients had clinical AIDS by the Centers for Disease Control CDC classification. To date, there have been 12 deaths in the 21 patients (57%) due to progressive deterioration with the patient's disease. Most procedures were adjuncts for diagnostic and therapeutic intervention in a population of children with a uniformly fatal disease. The knowledge of various high risk groups for AIDS must heighten the surgeon's awareness to the growing and significant pediatric segment of the HIV population, the complications of their disease, and the surgeon's limited role in treating these problems.

This study was designed to investigate the motility of the small bowel of the lamb under the conditions of experimental intestinal atresia. Of 26 fetal lambs operated upon (50 to 90 days gestation), 13 came to term. All term lambs exhibited a type 3a atresia. Six were successfully repaired and had intestinal myoelectric activity monitored for periods from 2 to 27 hours. A slow wave pattern (12 to 13/min) was present in the bowel of control lambs and in the proximal dilated bowel and the microbowel of atretic lambs, confirming the presence of functional smooth muscle. Spike potentials, which indicated circular muscular contractions, occurred 10% of the time in the control intestine, 5% in the proximal dilated gut (P less than .2), and 0% in the microbowel (P less than .001). A previously undescribed myoelectric transient of variable amplitude and frequency (6 to 24/min) was identified in all lambs studied. Histologic evaluation demonstrated villous hyperplasia in the microbowel and mucosal flattening in the distended bowel. The data suggest that the quiescent microbowel distal to the atresia contributes significantly to the functional obstruction.

Acute suppurative thyroiditis is rare in childhood. It presented in two clinically euthyroid children over a 2-year period. Repeated drainage for recurrent abscesses was necessary in one child. Thyroiditis without abscess was present in the second. After treatment and resolution of the infection, contrast studies with barium demonstrated an internal fistula extending from the left pyriform sinus to the ipsilateral thyroid lobe. Thyroid scans showed decreased uptake in the left lobe. Thyroid-function tests were normal. A third infant developed acute respiratory distress at 2 weeks of age from an enlarging left neck mass without evidence of infection. Exploration revealed an intrathyroid cyst with fistulous communication to the left pyriform sinus. Fistulas arise from the third pharyngeal pouch as a branchial remnant which is exclusively left-sided. Administration of antibiotics, which are effective against oral flora, combined with initial drainage of suppuration control infection. Only then can the diagnosis be made by contrast study of the hypopharynx and upper esophagus. Operative excision of the entire epithelial tract and adjacent thyroid tissue is essential to prevent recurrent thyroiditis and abscess.

Diminished concentrations of the gut neuropeptide, vasoactive intestinal peptide (VIP), have been measured by radioimmunoassay in man and mouse models of Hirschsprung's disease. This in vitro study was designed to ascertain the functional response to VIP in aganglionic colon. Seven piebald lethal (PLM) mice with histologically verified aganglionosis and seven normal littermates (NLM) were sacrificed. Distal colonic segments were placed in standard oxygenated tissue baths and responses to electrical field stimulation (EFS), acetylcholine (ACh), and VIP recorded and analyzed by a motility index (MI). Aganglionic colonic tissues from PLM exhibited marked basal contractile activity in contrast to NLM (MI = 19.5 +/- 2.0 SEM v 6.5 +/- 3.6 SEM, P less than .01). In NLM tissues, VIP reduced the MI to ACh challenge by 49% (P less than .01), while in PLM tissues, a nonsignificant 22% reduction was observed. VIP blocked the response to EFS in NLM tissues, while no response was elicited to EFS in PLM tissues. An in vitro deficit in the VIP inhibitory response to ACh challenge is apparent in PLM with distal colonic aganglionosis. The increased basal activity and reduction in responsiveness to VIP, observed in the PLM tissues, support a generalized reduction in the function of the inhibitory innervation of the aganglionic colon.

Ectopic calcitonin has never before been described in association with pediatric malignancy. This is the first report of ectopic calcitonin in a child with adrenocortical carcinoma. It may prove to be a useful tumor marker in this, as well as other childhood malignancies.

The Reyes technique for the management of severe, acute corrosive injuries of the esophagus has been modified and successfully applied to two children and one adult whose cases are reported. None developed stricture and two were uncomplicated. Esophagomegaly, motility dysfunction, upper gastrointestinal bleeding, and pneumonitis complicated the hospitalization of one child whose subsequent course has been asymptomatic. Until more experience can be recorded, widespread use is not yet recommended.

Testicular torsion results in irreversible histologic changes in the ipsilateral testis, which may induce alterations in contralateral testicular histology and in fertility. It was hypothesized that these ipsilateral changes could be minimized by splitting the tunica albuginea at the time of detorsion, thus decompressing the testicular "closed compartment syndrome." Unilateral spermatic cord torsion was induced in prepubertal, male Sprague Dawley rats for a period of 0, 4, 8, or 12 hours. At the time of detorsion, capsulotomy was performed on half the animals. The mature rats were killed 35 days after detorsion and the testes examined histologically. Testicular capsulotomy did not alter the significant histologic changes observed in the affected testis following spermatic cord torsion.

This report is of a 2-month-old child with spontaneous perforation of the common bile duct diagnosed by 99m-Tc disofenin nuclear hepatobiliary imaging. Of the agents available, the radiopharmaceutical properties of this compound allow the most accurate, safe, high-resolution imaging of the hepatobiliary tree in the diagnostic evaluation of jaundiced infants.

Total Intestinal Aganglionosis is a rare, uniformly fatal condition with absence of ganglia from the duodenum to the rectum. A neonate with this extreme form of Hirschsprung's disease is presented with a review of 12 previously reported cases. Smooth muscle strips from this infant's gastrointestinal tract demonstrated viable cholinergic receptors, absence of intrinsic neuronal innervation, and colonic contractile activity to the purported peptidergic neurotransmitter cholecystokinin. Four aspects of total intestinal aganglionosis are noteworthy: (1) one-third of patients presented between four and eight days of age after passing meconium on the first day of life; (2) at laparotomy, no intestinal distention, obstruction, or transition zone was evident; (3) hypertrophic nerve fibers seen in classic Hirschsprung's disease were absent in one-quarter of patients; and (4) a high incidence of affected siblings occurred in the previously reported cases. This form of Hirschsprung's disease may represent a distinct entity with autosomal recessive inheritance and significant risk for recurrence.

Clinical and experimental investigations of intestinal atresia have reduced mortality and clarified etiology. However, the morbidity of this lesion remains excessive and its pathophysiology uncertain. The purpose of this project was to produce the first long-term model of intestinal atresia and thereby to study the motility of the adapting bowel. Intestinal atresia was induced in nine fetal lambs at midgestation by mesenteric avulsion. At term, this experimental group and eight control lambs underwent resection with anastomosis, decompression gastrostomy, and implantation of electrodes proximal and distal to the anastomosis. Vigorous resuscitation and constant intensive care were critical for survival. Slow wave activity (SWA) and spike potential activity (SPA) were recorded in the first 24 hours and every 48 hours thereafter for a 6-hour interval. Three of nine lambs with atresia and five of the eight control animals survived over 48 hours (chronic). Analysis of 220 hours of recording showed, in the first 24 hours of life, a statistically significantly different SWA of 16.4 cycles/min (+/- 1.15 SD) in the chronic atretic survivors compared with 13.1 cycles/min (+/- 2.6 SD) in the acute (died less than 48 hours) atretic group (P less than .05). The SWA of the chronic atretic survivors was similar to the SWA of the chronic nonatretic control animals, 15.1 cycles/min (+/- 1.4 SD). SPA was present on the first day of life in the microintestine of the chronic atresia group in contrast to the acute animals of this study and of Doolin and Hill's previous study. Delayed function has been attributed to the absence of SPA in the microintestine of acute models.(ABSTRACT TRUNCATED AT 250 WORDS)

The technique and scoring system of 24-hr pH esophageal monitoring has been modified to evaluate gastroesophageal reflux in infants and children. The data from two pediatric controls and five clinical cases are presented and compared to normal adult values. This test has better objectivity, precision, sensitivity, and reliability than contrast studies, endoscopy, esophageal biopsy, acid perfusion, or acid reflux tests. The 24-hr pH monitoring assists the evaluation of sphincter maturation, pulmonary disease, and the significance of body position. With more experience, this technique could identify children at risk fo developing severe complications of reflux esophagitis and aid in the selection of candidates for surgical intervention.