Tocopherol levels in infants < or = 1000 grams receiving MVI pediatric.
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Biomedical subjects
Publications and source records attributed to W J Byrne.
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The presence of gastrointestinal (GI) tract hemangiomatosis was demonstrated by computed tomography in two children who presented with lower GI tract bleeding. Marked vascular enhancement of the bowel during rapid intravenous administration of contrast material and dynamic scanning is characteristic, similar to that seen with hemangiomas elsewhere in the body. Barium examinations demonstrate submucosal lesions within the bowel and are useful to approximate the length of bowel involvement.
Isolated congenital short small intestine is a rare anomaly. Of six (one male, five females) previously reported cases, four died in infancy from intractable diarrhea. We report the case of 7-year-old boy with this syndrome in whom a 2-year period of parenteral feeding at home allowed normal weight gain, growth, and development while intestinal adaptation occurred. Parenteral feeding was discontinued at age 2 1/3 years, and for the past 5 years his weight has remained between the tenth and 25th percentiles and his stature between the 25th and 50th percentiles. His development has been normal and he functions at or above grade level at school. Coefficient of fat absorption has increased from 54% to 81%. Vitamin B12 absorption has improved but has not normalized. He remains lactose intolerant. We believe his survival, growth, and development would have been compromised if he had not received a prolonged period of parenteral feeding.
Although rare in children, achalasia can be the cause of debilitating symptoms and growth retardation. During a 4-year period, six patients (mean age 9.9 years) underwent a modified Heller operation (anterior esophageal myotomy) without complication. A concomitant modified Belsey fundoplication was performed in three patients who were judged at the time of operation to be at high risk for postoperative gastroesophageal reflux. Preoperative symptoms of dysphagia, postprandial vomiting, retrosternal pain, and pulmonary complications were eliminated in all patients. Follow-up interviews seven to 48 months (mean 23 months) following operation revealed normal diet and normal growth in all six children, with no recurrence of preoperative symptoms or evidence of gastroesophageal reflux. Technical details which we believe contribute to success in the operative management of pediatric achalasia include the transthoracic approach and the selective performance of complementary anti-reflux procedures.
A patient with hematochezia and a false negative Meckel's scan is presented. A Meckel's diverticulum was subsequently diagnosed on barium small bowel follow-through. Meckel's diverticulum is discussed with emphasis on the relationship of barium and radionuclide studies.
Three cases of ureteropelvic junction obstruction are presented in which the only symptom was recurrent abdominal pain. Results of physical examination and urinalysis were normal. Ultrasound established the correct diagnosis in the two patients in whom it was performed. Because it is safe, involves no radiation exposure, and is useful in evaluating the gallbladder, pancrease, and liver, as well as both kidneys, abdominal ultrasound should be performed prior to contrast radiography in the evaluation of children with recurrent abdominal pain.
Pancreatic ultrasonography and the determination of serum amylase and amylase creatinine clearance ration were performed on 17 normal children and on 35 children with abdominal pain. In all 17 normal subjects the echodensity of the pancreas was equal to or greater than that of the liver. Nineteen patients with abdominal pain had reduced echodensity of the pancreas when compared to that in normal children, and two of the 19 had pseudocysts. Serum amylase values and/or amylase creatinine clearance ratio correlated poorly with ultrasonic and clinical evidence of pancreatitis.
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Basal and pentagastrin-stimulated gastric acid secretion was measured in 26 healthy newborn infants on the 1st and 2nd days of life. Basal acid outputs (BAO) on day 1 (0.378 mEq/hr) and day 2 (0.388 mEq/hr) were similar and not significantly different from the maximal acid outputs (MAO) on day 1 (0.413 mEq/hr) and day 2 (0.452 mEq/hr). When calculated on a mEq/kg/hr basis, BAO on day 1 was 0.110 with an MAO of 0.122. The BAO on day 2 was 0.114 with an MAO of 0.133. Fasting serum gastrin levels obtained before the acid studies on each day were elevated (mean, day 1 = 100 pg/ml; mean, day 2 = 108 pg/ml). These findings suggest that either gastric acid secretion in the newborn is maximal under basal condition or that newborn parietal cells are unresponsive to pentagastrin on day 1 and 2 of life.
Seventeen pediatric patients, ages 9.25--20.5 yr, were placed on a program of home parenteral nutrition (HPN) for severe, symptomatic Crohn's disease. Prior therapy with sulfasalazine in 14, adrenocorticosteroids in 12, inpatient total parenteral nutrition in 7, and/or surgical resections in 6 failed to suppress disease activity. Remission was attained in 12 of the 17 after one course of HPN alone. Four patients had surgical procedures and 1 required steroids in addition to HPN. Remissions have been maintained in 4 of those 12 for a mean duration of 315 days after discontinuation of HPN. Of the 8 who relapsed after a mean duration of 68 days, second courses of HPN were undertaken in 7 and third courses in 2. All 17 had a marked improvement in disease symptoms while receiving HPN in addition to gaining weight sufficient to place them at a higher percentile on standard growth charts. Ten patients demonstrated "catch-up" growth and 4 others increased their height appropriately. A chromium 51-labeled albumin stool collection of greater than 1% in 5 of 7 patients at the completion of a HPN course correlated with relapse within 4 mo. Serial radiographic contrast studies and erythrocyte sedimentation rates were not predictive of prolonged remissions. Home parenteral nutrition complications were minimal, with only one episode of sepsis per 5.8 catheter experience years. It is concluded that HPN is a safe and effective means of inducing remissions and providing optimal nutritional support in pediatric patients with severe Crohn's disease. Patients with less than adequate response to standard medical management should be considered candidates for this therapeutic modality.
One hundred and six patients were placed on a home parenteral nutrition program because of severe gastrointestinal tract lesions. In 41, sufficient improvement allowed the resumption of oral alimentation. Forty-eight remain on the program. Seventeen, including ten with malignant disease, died from causes not related to home parenteral nutrition. All patients achieved and maintained the appropriate weight for age and body build. In the pediatric patients, normal or accelerated linear growth occurred. Complications included sepsis, 18 episodes in 12 patients; local infection of the catheter, 14 in six; catheter thrombosis, six in five; ketoacidosis, one in one; contaminated solutions, one in one, and essential fatty acid deficiency, one in one. Home parenteral nutrition is a relatively safe and effective alternative for long term nutritional support.
To evaluate the role of gastroesophageal reflux (GER) as a possible cause of recurrent pulmonary disease, 30 children, aged 1 to 18 years, were studied prospectively with esophageal function tests. These included esophagram (30 patients), esophageal manometry (29 patients), pH probe (Tuttle) test (29 patients), and esophagoscopy with esophageal biopsy (23 patients). The patients studied had either chronic asthma or two or more documented pneumonias within a one-year period. Nineteen (63%) had GER based on two or more positive tests. Eighteen had positive Tuttle tests; 13 had abnormal manometry studies; nine had esophagitis on biopsy; six had esophagitis on esophagoscopy; and five had reflux on esophagram. Of those with GER, 17 had a history of nocturnal cough and eight vomited during infancy. Children with recurrent pulmonary disease should have esophageal function testing to exclude GER as the cause.
During the past 3 yr, 17 patients with chronic ulcerative colitis and 6 with Crohn's disease who had severe rectal and colonic involvement underwent excision of the rectal mucosa without removal of the rectal muscle in combination with total colectomy and cutaneous ileostomy as a 1- or 2-stage procedure. This operative technique has cured each of the patients of their primary colonic and rectal disease and has obviated many of the unpleasant complications that often occur after total proctectomy, such as impotence, prolonged perineal drainage, and bladder dysfunction. The operation has the further advantages of lower operative blood loss, shorter operative time, and earlier safe ambulation. On the basis of the favorable experience with mucosal proctectomy, sphincterotomy, and perineal drainage in 23 patients, none of whom experienced major complications, we believe that this operation warrants further clinical trial in patients with inflammatory bowel disease involving the rectum, which is refractory to medical therapy. Total proctectomy might eventually find scant application in patients with inflammatory bowel disease.
Although home parenteral nutrition (HPN) has been used successfully for adult patients, no extensive experience with children has been reported. During the past three years, we have managed 34 patients, ages 1 1/2 months to 20 1/2 years, on a HPN program for periods ranging from 23 to 786 days. Silastic Broviac catheters were inserted into the superior vena cava through the jugular or cephalic veins or into the inferior vena cava through the saphenous vein. The catheters were brought out onto the chest or lower abdominal wall through a subcutaneous tunnel. Solutions were infused over a 10--14-hour period each day, using a volumetric pump system. All patients improved their nutritional status. Twenty-three of 29 on the program for more than two months showed an increase in height. All patients evidenced a significant decrease in symptomatology. All resumed per group activities while on HPN and were able to continue their education or work. At present 24 patients including 15 with Crohn's disease no longer receive HPN. Administration of HPN through a Broviac catheter is a safe, successful technique for maintaining an optimal nutritional status in children with severe digestive disorders, and permits resumption of a more normal daily lifestyle. Following HPN, bowel adaptation and initiation of full oral alimentation become possible in many patients.
Three patients on home total parenteral nutrition utilizing a synthetic amino acid solutions developed a dermatitis consistent with acrodermatitis enteropathica after 30, 43 and 62 days of therapy. The dermatitis resolved after changing to casein hydrolysate in 2 patients and after full oral alimentation in the third. Although measured serum zinc levels were repeatedly normal to elevated in all patients, sufficient retrospective data analysis combined with literature review on this subject, clearly implicates zinc deficiency as the etiology for their dermatitis. Adequate zinc supplementation of synthetic amino acid solutions is essential in order to avoid this complication.