Biomedical subjects
Publications and source records attributed to J P Keating.
Newborns with neonatal cirrhosis may have confusing clinical pictures. The demonstration of a small liver on abdominal radiographs may be helpful in suggesting the diagnosis of intrauterine hepatic damage.
A newborn infant with aganglionosis of the entire colon and terminal ileum also had congenital lip pits and a submucous cleft palate. These anomalies may be added to the growing list of conditions associated with Hirschsprung's disease.
Fifteen children followed as outpatients with chronic inflammatory disease of the colon were given sulfasalazine in doses from 1 to 4 gm/day (22 to 68 mg/kg, or 0.69 to 2.33 gm/m2). No correlation was found between the dose/m2 administered and the total serum sulfapyridine levels. However, 11 of 15 patients achieved SP levels greater than or equal to 17 micrograms/ml, a level approximating that reputedly associated with therapeutic efficacy. Patients who were either slow acetylators or slow hydroxylators of sulfapyridine had total SP levels significantly higher than patients who were both rapid acetylators and hydroxylators (20.0 +/- 1.2 vs 14.6 +/- 1.6). Total SP serum levels were not correlated with the activity of the disease. No toxic levels (greater than 50 micrograms/ml of SP) were encountered. We conclude that a dose of SASP in the range of 1.5 to 2.0 gm/m2 can be safely administered to children and is usually associated with serum SP levels considered in the therapeutic range. Although one-third of children are both rapid acetylators and hydroxylators and will have somewhat lower SP levels, the routine monitoring of SASP therapy with SP levels is not necessary for management of disease.
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Researchers conducted a study to analyze hospital fire safety procedures and training, with a particular emphasis on verbal alarm systems. Their findings are presented, along with recommendations for fire safety training and a suggestion from a verbal fire alarm message.
A patient with a hyperalimentation catheter in the spinal canal is presented. This resulted in an extradural fluid mass and partial paraplegia that eventually cleared.
An 11-year-old boy was seen with gradual onset of jaundice. Dilated bile ducts were demonstrated by ultrasound and computerized axial tomography. The obstruction was caused by an unusual benign tumor, a granular cell myoblastoma. Cure was effected by resection and choledochojejunostomy.
Blood substrate and hormone concentration were determined in 16 children with Reye syndrome prior to and following administration of hypertonic glucose. Baseline concentrations of lactate, pyruvate, alanine, glutamine, glutamate, proline, hydroxyproline, lysine, and aspartate were elevated (p less than 0.01), whereas citrulline and arginine were low. All substrate concentrations were below or within the normal range following 36 hours of therapy except those of lactate, pyruvate, and aspartate. Urea nitrogen excretion was reduced (p less than 0.05) on the second day of therapy. Plasma concentrations of insulin and growth hormone increased and glucagon decreased during the first day. Cortisol remained elevated throughout the study period. We conclude that the high circulating concentrations of substrates are the result of both increased mobilization and decreased clearance and that hypertonic glucose infusion suppresses substrate mobilization. A primary abnormality of the mitochondria could explain the metabolic perturbations that occurred. A possible relationship between the encephalopathy in this disorder and an insult to both brain and brain capillary mitochondria is discussed.
Antral webs and diaphragms are considered a rare cause of gastric outlet obstruction in infants and children. A radiographic diagnosis of antral web was made in 28 children during a 26-mo period. Nonbilious, often projectile vomiting was the predominant presenting symptom in 24 infants who were less than 6 mo of age. The older children complained of pain, vomiting, fullness after eating, and eructation. The characteristic radiologic finging was a wirelike transverse septum 1--2 cm proximal to the pylorus. Poor antral filling was an important early clue in the radiographic search for the webs. Pyloroplasty was performed in 20 patients, frequently after failure of medical management of symptoms. Coexistent congenital abnormalities were present in 28% of patients. A search for environmental and familial factors has failed to elucidate any teratogenic influences in this population.
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A method of clinical staging for infants with necrotizing enterocolitis (NEC) is proposed. On the basis of assigned stage at the time of diagnosis, 48 infants were treated with graded intervention. For Stage I infants, vigorous diagnostic and supportive measures are appropriate. Stage II infants are treated medically, including parenteral and gavage aminoglycoside antibiotic, and Stage III patients require operation. All Stage I patients survived, and 32 of 38 Stage II and III patients (85%) survived the acute episode of NEC. Bacteriologic evaluation of the gastrointestinal microflora in these neonates has revealed a wide range of enteric organisms including anaerobes. Enteric organisms were cultured from the blood of four infants dying of NEC. Sequential cultures of enteric organisms reveal an alteration of flora during gavage antibiotic therapy. These studies support the use of combination antimicrobial therapy in the treatment of infants with NEC.
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Gastric outlet obstruction in infants and children may be due to a partial, prepyloric antral diaphragm. Twelve new patients are added to the 32 described previously. Onset of symptoms varied from shortly after birth to five years. Nonbilious vomiting was the most common presenting symptom. Radiographic evaluation requires specific technique for demonstration of the web and to differentiate this from pylorospasm and pyloric stenosis. Gastroscopy was employed in three patients. Repair usually consisted of incision of the web and construction of a patulous gastric outlet. All patients remained asymptomatic after operation. The etiology of the webs remains unknown, but they may result from an excessive local endodermal proliferation early in gastric development.
Serum bile acid patterns were determined in 31 infants ranging in age from 4 days to 24 weeks. Fifteen infants with extrahepatic biliary atresia had a mean concentration of serum bile acids of 90 microng/ml +/- 47 SD;; 16 infants with neonatal hepatitis had a mean concentration of 60 +/- 35.5 SD. The chenodeoxycholate/cholate ratio was greater than one in 13 infants (87%) with atresia and in 10 infants (66%) with neonatal hepatitis. Except for the tendency of a higher total concentration of serum bile acids in infants with atresia, a single serum bile acid value does not differentiate neonatal hepatitis from extrahepatic biliary atresia. The high proportion of chenodeoxycholate in extrahepatic atresia is different from the pattern in other types of cholestatic disease and may reflect an underlying hepatitis.
Total parenteral nutrition has been used in the treatment of more than 100 children with gastrointestinal illnesses, including 90 under the age of 6 months. The incidence of complications has been reduced markedly as experience was acquired. Total parenteral nutrition has been especially valuable in patients with intractable diarrhea syndrome and it has been used successfully in the management of infants with overwhelming systemic infections.
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