Selective ACTH insensitivity, achalasia, and alacrima: a multisystem disorder presenting in childhood.
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Adrenal Cortex Diseases↗
Biomedical subjects
W E Berquist
Publications and source records attributed to W E Berquist.
Increased risk of gallstones in children receiving total parenteral nutrition.
Twenty-one children receiving long-term total parenteral nutrition were prospectively evaluated for the presence of gallstones. Using ultrasonography, nine children (43%) were found to have cholelithiasis, and five have since undergone cholecystectomy. Only children with ileal disorders or previous resection developed stones. In the select group of patients with ileal disorders or previous resection, the prevalence of stones was 64%, nearly twice that which has been observed in similarly defined adults not receiving total parenteral nutrition. Data from this study suggest that the prolonged administration of parenteral nutrition significantly enhances the risk of gallstone formation already imposed by a previous ileal resection or disorder. Periodic ultrasonograms provide a safe and accurate means of monitoring high-risk patients during and after prolonged total parenteral nutrition therapy.
Adolescent↗
Achalasia: diagnosis, management, and clinical course in 16 children.
Clinical features, radiographic and esophageal manometry findings, and treatment results in 16 patients less than 15 years old with achalasia are described. Esophageal manometry performed in 15 patients showed results similar to those found in adults: (1) increased resting lower esophageal sphincter pressure, (2) incomplete or failure of relaxation of the lower esophageal sphincter on swallowing, and (3) ineffective or absence of peristalsis in all. The most common symptoms in the 16 patients were: dysphagia in 15, postprandial vomiting in 13, and retrosternal pain in five. The average duration from onset of symptoms to diagnosis was 28 months. The esophagram was diagnostic in all patients. Pneumatic dilation was the initial treatment in eight and was successful for more than 1 year in five. Two patients required two dilations and were then symptom-free for more than 1 year, but required a Heller myotomy. The remaining patients underwent Heller myotomy following failure of the second dilation. Three patients underwent myotomy and two patients had myotomy with fundoplication as initial treatment; only one remained symptomatic. Esophageal dilation using a pneumatic dilator should be the initial treatment of choice in school-aged children. However, if more than two dilations are required within 1 year, surgical management is recommended.
Adolescent↗
Gastroesophageal reflux in children: a clinical review.
The diagnosis of gastroesophageal reflux requires careful consideration of the patient's clinical history and initial evaluation of presenting symptoms. In cases where overt vomiting in noted, the initial evaluation should include a barium esophagram and upper gastrointestinal series. The diagnosis of gastroesophageal reflux may not be established by one test alone, but may require many tests to confirm the presence of significant reflux and to assess its sequellae. It is imperative to demonstrate that the extent and timing of GER is not merely physiologic. Continuous intraesophageal pH monitoring has proven to be the most sensitive test for gastroesophageal reflux and better identifies its frequency, duration, and relationship to other symptoms. Manometry assesses the competence of the lower esophageal sphincter and integrity of esophageal peristalsis. The standard acid reflux test is a provocative test of gastroesophageal reflux. Gastric outlet obstruction, both organic and functional, may be primary causes of gastroesophageal reflux, and may be evaluated with barium contrast studies and scintigraphic stomach-emptying studies. Poor esophageal transit and clearance are contributing factors which promote esophagitis. Treatment of gastroesophageal reflux requires identification of the primary cause, and selection of the therapeutic modality appropriate to the severity of reflux and its associated sequellae. In those patients with severe clinical sequellae, the most effective treatment for gastroesophageal reflux is surgical fundoplication.
Child↗
Occlusion of the vena cava in infants receiving central venous hyperalimentation.
During a five year period at UCLA Hospital, 118 Silastic, Broviac, central venous hyperalimentation catheters were inserted into 94 infants who were less than one year of age. The indications for total parenteral nutrition were malabsorption syndrome in 26, short bowel syndrome in 24, diarrhea in 15, nutritional support following operation in 11 and miscellaneous in 18. One hundren and two catheters were placed into the vena cava by way of the saphenous vein at the groin, and 16 were inserted through the external or internal jugular veins. The duration of catheter use varied from six to 925 days, a mean of 104 days. In six infants, obstruction of the vena cava developed, five in the inferior vena cava and one in the superior vena cava. Swelling of the extremity was minimal, and the thrombosis was insidious in each patient. None of the patients had pulmonary emboli. Although two of the six patients eventually died, in neither was thrombosis of the cava believed to be related. Thrombosis of the vena cava is a frequent sequela of long term central venous hyperalimentation in infants, but it is rarely fatal and produces remarkably few serious complications.
Catheters, Indwelling↗
Aluminum as a factor in the bone disease of long-term parenteral nutrition.
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Adult↗
Effect of theophylline on gastroesophageal reflux in normal adults.
In a randomized, double-blind manner, 15 normal adults were given a single oral loading dose of anhydrous theophylline (6.5 +/- 0.9 mg/kg) and nine normal adults were given placebo. All subjects were tested for serum theophylline levels, acid reflux (using intraesophageal pH probe), and lower esophageal sphincter pressure (LESP) at 1.5, 4, and 8 hr after treatment. Of subjects with negative baseline acid reflux tests, eight of 13 (61.5%) given theophylline developed positive acid reflux tests compared with none of eight subjects given placebo. One of nine (11%) placebo subjects reported heartburn compared with 11 of 15 (73%) subjects given theophylline. The maximum mean percent change in LESP from baseline was +5.62 +/- 28.8% in the placebo group and -25.01 +/- 23.7% in the theophylline group (p = 0.01) at 4 hr after treatment. While only two of nine (22%) adults given placebo had at least a 14% reduction in LESP following treatment, all of the 15 subjects sustained a minimum of 14% relaxation in LESP. This study confirms that oral theophylline at therapeutic serum levels inhibits LESP and induces gastroesophageal reflux (GER) measured by acid reflux tests in most normal adults.
Adult↗
Congenital vascular band between the ileum and gallbladder producing intermittent partial small bowel obstruction.
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Child, Preschool↗
Effectiveness of Nissen fundoplication for gastroesophageal reflux in children as measured by 24-hour intraesophageal pH monitoring.
Intraesophageal 24-hr pH monitoring was performed on each of six children with severe symptomatic GER both before and after (mean, 10.1 mo.) fundoplication. The frequency and duration of reflux as measured by esophageal pH below 4.0 was less following fundoplication than in control patients (p less than 0.01). The 24-hr esophageal pH correlates more closely with GER than does measurement of the LESP. The duration to be the best available test which correlates with detection of reflux and correction following fundoplication. The Nissen fundoplication appears to effectively prevent GER.
Adolescent↗
Gastroesophageal reflux-associated recurrent pneumonia and chronic asthma in children.
Forty of 82 patients with recurrent pneumonias and/or clinical asthma were found to have gastroesophageal reflux (GER) by the criteria of two or more of five tests positive for GER. Of 36 patients with GER followed for response to therapy, 32 patients attempted medical therapy and four had fundoplications. Ten of 32 (31%) patients on medical therapy had improvement in symptoms but none became asymptomatic. Twenty patients who failed a trial of medical therapy also had fundoplications for a total of 24 patients surgically treated. Of these, 22 (92%) had improvement or became asymptomatic. All seven patients with diagnosed GER and recurrent pneumonias responded to medical antireflux management or fundoplication. GER is an important treatable cause of recurrent pneumonias and/or chronic asthma in children.
Adolescent↗
Esophageal muscular ring and the VACTERL association: a case report.
A 2-year-old girl with persistent vomiting of solid foods was evaluated; the diagnosis was esophageal muscular ring. This rare congenital anomaly and its differentiation from esophageal stricture are discussed. This case represents a variation of the esophageal malformation associated with the VACTERL association.
Abnormalities, Multiple↗
Long-term home parenteral nutrition in pediatrics: ten years of experience in 102 patients.
One hundred two pediatric patients received all or part of their nutritional needs parenterally at home during the past decade. All received total parenteral nutrition (TPN) at night during an 8- to 12-h infusion. Patients with short bowel syndrome (33%), inflammatory bowel disease (23%), chronic intractable diarrhea (15%), chronic idiopathic intestinal pseudo-obstruction syndrome (10%), and malignancy (10%) made up the largest groups. The mean duration of parenteral support was 735 days (range, 90-3650 days); the mean number of catheters per patient was 2.1 (range, 1-8). Twenty-one patients continue to receive full or partial home TPN: four for more than 10 years and seven for more than 5 years. Fifty-one no longer require it and have had healing of mucosa or bowel adaptation. Complications related to administration of fluid and electrolytes were quite rare. Biotin deficiency was recognized once. Thirty-one have died, but only 13 deaths were related to TPN. Sepsis in nine and liver failure in two were the most common causes of death in the TPN-related group. Three of 21 still on home TPN have graduated either from high school or college. All but one of the school age children attend regular school; one attends a school for the medically disabled, another attends a school for the mentally gifted.
Adolescent↗
Severe cytomegalovirus infection and immunosuppressive therapy in pediatric liver transplantation.
Twenty-two post-orthotropic liver transplant (OLT) recipients were studied to investigate the clinical, laboratory and histopathological differences between rejection and CMV infection. The mean age at the time of transplantation was five years. Nine of 22 (41%) patients developed positive CMV, CF-IgG and IgM antibody titers and cultures for CMV following surgery, and three (group 1a) developed interstitial pneumonitis. CMV specific inclusion bodies were found in lung and liver biopsies. Two patients in group 1a were treated successfully with DHPG and decreasing immunosuppressive treatment, while the third died. Clinical presentation of rejection episodes were similar in all groups. CMV infected patients (group 1) received more transfusions of blood and blood products than the non-infected patients (group 2). Rejection episodes occurred sooner and more frequently in group 1a than in group 1b (CMV infected-asymptomatic) and group 2 (non-infected). Group 2 received fewer steroid boluses as well as azathioprine and OKT 3. A percutaneous liver biopsy with routine stains helped detect CMV when inclusion bodies were seen. We conclude that culture proven CMV infection is common post-OLT. Severe CMV infection occurred more frequently in those who had received greater doses of immunosuppressive therapy for possible graft rejection. Monitoring CMV infection following OLT is absolutely necessary. After OLT, decreasing the immunosuppressives and using antiviral agents are important in the management of CMV infection.
Adolescent↗