The predicted 4-year survival rate of a child with a Wilms tumor that is confined to one kidney and is grossly excised is:
Following nephroureterectomy for Wilms tumor, the need for chemotherapy and/or radiation therapy is determined by the histology of the tumor and the clinical stage of the patient (Table below). Essentially, patients who have disease confined to one kidney that is completely excised surgically receive a short course of chemotherapy and can expect a 97% 4-year survival, with tumor relapse rare after that time. Patients with more advanced disease or with unfavorable histology receive more intensive chemotherapy and radiation. Even in stage IV, cure rates of 80% are achieved. The survival rates are worse in the small percentage of patients considered to have unfavorable histology.
Staging of Wilms tumor:
A premature infant boy has been started on enteral feeds shortly after birth, but develops feeding intolerance 2 weeks postnatally. He displays abdominal tenderness, distention, and bloody stools. An abdominal radiograph is obtained and is shown in Fig. below. What should be the next step in management?
The radiograph demonstrates pneumatosis intestinalis, in conjunction with the clinical scenario described, describes Bell stage II necrotizing enterocolitis (NEC). In all infants suspected of having NEC, feedings are discontinued, a nasagastric tube is placed, total parenteral nutrition (TPN) is started, and broad-spectrum parenteral antibiotics are given. The infant is resuscitated, and inotropes are administered to maintain perfusion as needed. Intubation and mechanical ventilation may be required to maintain oxygenation. Subsequent treatment may be influenced by the particular stage of NEC that is present. Patients with Bell stage II disease merit close observation. Serial physical examinations are performed looking for the development of diffuse peritonitis, a fixed mass, progressive abdominal wall cellulitis, or systemic sepsis. If infants fail to improve after several days of treatment or if abdominal radiographs show a fixed intestinal loop, consideration should be given to exploratory laparotomy.
Abdominal radiograph of infant with necrotizing enterocolitis. Arrows point to area of pneumatosis intestinalis.
An infant girl is found to have persistent jaundice after birth. A metabolic screen is normal, ultrasound demonstrates an absent gallbladder, and a technetium-99m iminodiacetic acid scan shows radionuclide that is concentrated in the liver but not excreted into the intestine. Which of the following is true?
This patient has biliary atresia, a rare disease characterized by fibroproliferative obliteration of the biliary tree, which progresses toward hepatic fibrosis, cirrhosis, and end-stage liver failure. Surgical treatment is the first-line therapy, consisting of creation of a hepatoportoenterostomy (Kasai procedure). Numerous studies suggest that the likelihood of surgical success is inversely related to the age at the time of portoenterostomy. Infants treated prior to 60 days of life are more likely to achieve successful and long-term biliary drainage than older infants. Although the outlook is less favorable for patients after the 12th week, it is reasonable to proceed with surgery even beyond this time point, as the alternative is certain liver failure. Approximately one-third of patients remain symptomfree after portoenterostomy; the remainder requires liver transplantation due to progressive liver failure. Independent risk factors that predict failure of the procedure include bridging liver fibrosis at the time of surgery and postoperative cholangitic episodes.
The leading cause of death for children older than 1 year of age is:
Injury is the leading cause of death among children older than 1 year. In fact, trauma accounts for almost half of all pediatric deaths, more than cancer, congenital anomalies, pneumonia, heart disease, homicide, and meningitis combined. Motor vehicle collisions are the leading cause of death in people age 1 to 19 years, followed by homicide or suicide (predominantly with firearms) and drowning. Unintentional injuries account for 65% of all injury-related deaths in children younger than 19 years. Each year, approximately 20,000 children and teenagers die as a result of injury in the United States. For every child who dies from an injury, it is calculated that 40 others are hospitalized and 1120 are treated in emergency departments. An estimated 50,000 children acquire permanent disabilities each year, most of which are the result of head injuries. Thus, the problem of pediatric trauma continues to be one of the major threats to the health and well-being of children.
A "double bubble" on an abdominal radiograph in an infant is characteristic of:
Whenever the diagnosis of duodenal obstruction is entertained, malrotation and midgut volvulus must be excluded. Other causes of duodenal obstruction include duodenal atresia, duodenal web, stenosis, annular pancreas, or duodenal duplication cyst. The classic finding on abdominal radiography is the "double bubble" sign, which represents the dilated stomach and duodenum (Fig. below). In association with the appropriate clinical picture, this finding is sufficient to confirm the diagnosis of duodenal obstruction.
Abdominal X-ray showi ng "double bubble" sign in a newborn infant with duodenal atresia. The two "bubbles" are numbered.
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