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Biomedical subjects

G D Ferry

Publications and source records attributed to G D Ferry.

At least 37 records · Page 2Linked to original sources
PubMed1986

Splanchnic bed vascular malformations and the development of portal hypertension.

The vascular lesions, angiomas, and arteriovenous malformations are known to be associated with a number of complications. These lesions, when present in the liver, may precipitate heart failure in infants. Recent reports suggest that although the prognosis is good for the majority of infants who survive the neonatal period, the lesions are capable of causing portal hypertension later in life. We have encountered three patients whose vascular lesions were in continuity with the portal circulation who subsequently went on to develop portal hypertension. One patient had angiomas of the liver, one splenic and colonic angiomas, and the third had diffuse arteriovenous malformations of the intestine. Data are presented and literature is reviewed, which suggest that any vascular lesion that is capable of substantially increasing portal blood flow may lead to the development of portal hypertension. The treatment of such lesions should be aggressive. If surgical management is not possible, alternative treatments such as embolization should be attempted early in order to increase the chance for successful therapy.

Arteriovenous Malformations↗
PubMed1985

Guide to early diagnosis of biliary obstruction in infancy. Review of 143 cases.

Presenting characteristics, long-term outcome, and techniques used in the diagnosis of 143 infants with suspected biliary obstruction are reviewed. Sixty-nine patients had surgically confirmed extrahepatic disease and 74 had intrahepatic disorders. A disproportionate number of infants with intrahepatic disease were boys (p = 0.013), low birthweight (p = 0.001), or had siblings with liver disease (p = 0.017). An initial total bilirubin of 20 mg/dl or greater was rare except in the intrahepatic disease category of neonatal hepatitis of known cause (p = 0.006). The initial percutaneous liver biopsy correctly predicted the ultimate diagnosis in 94 percent of all 143 patients. A methodological outline to diagnosis is presented, emphasizing early recognition of symptoms and careful follow-up with hepatobiliary imaging, liver biopsy, and surgical exploration, if required, until definitive diagnosis is made. This approach has aided us in reducing the age of diagnosis of biliary atresia from 12.8 +/- SD 7.3 weeks during the period 1971 through 1979 to 6.8 +/- SD 2.6 weeks from 1980 to 1982 (p = 0.0015). Eighteen-month survival has improved from 25 to 60 percent.

Bile Ducts↗
PubMed1983

Clinical response to short-term nasogastric feeding in infants with gastroesophageal reflux and growth failure.

When conventional treatment with upright positioning and thickened feedings fails to control vomiting associated with gastroesophageal reflux (GER) of infancy, malnutrition and growth failure may result. If this occurs, fundoplication is usually recommended. In this study, 12 infants with growth failure associated with GER were given a trial of short-term (11.1 +/- SE 1.6 days) continuous-drip nasogastric (NG) feedings prior to surgical referral. Five of 10 infants for whom long-term follow-up (3-12 months) was obtained showed immediate weight gain, cessation of vomiting, and long-term resolution of growth failure without the need for surgery. All infants who had a favorable long-term response showed evidence of catch-up growth during the first 7 days of NG feeding. Infants who did not begin catch-up growth during the first 7 days did not benefit from longer periods (up to 21 days) of NG feeding. Poor response to NG feedings was associated with the presence of other medical problems (p = 0.024), including chronic pulmonary disease, malabsorption, cerebral palsy, and laryngomalacia. Four infants who did require fundoplication still showed no improvement in growth 2-6 months after surgery. In infants with GER and growth failure without other complicating disorders, a 7-10-day course of NG feeding may improve nutrition and bring about a permanent resolution of vomiting.

Enteral Nutrition↗
PubMed1982

Complications of gastroesophageal reflux in patients with cystic fibrosis.

Seven patients with cystic fibrosis who had complications of gastroesophageal reflux including abdominal pain, peptic esophagitis, upper gastrointestinal hemorrhage, and esophageal stricture are described. We believe that these are gastrointestinal complications of CF and that they may be responsible for significant morbidity. The mechanical influence of a depressed diaphragm caused by hyperinflation, along with increased abdominal pressure with chronic coughing, may contribute to GER in CF. Early detection and treatment are important not only to prevent esophageal complications but also to increase the quality of life by relief of pain and by avoiding the resultant decrease in appetite, which can contribute to malnutrition.

Adolescent↗
PubMed1981

Childhood acute leukemia: a search for occult extramedullary disease prior to discontinuation of chemotherapy.

Between January 1978 and September 1979, 29 children with acute leukemia in complete continuous remission for three or more years were examined for evidence of occult extramedullary disease immediately prior to discontinuation of chemotherapy. Bilateral open wedge testicular biopsy demonstrated the presence of bilateral leukemic infiltrates in 2/13 boys. Gallium scans prior to biopsy had identified unilateral infiltration in an enlarged testis in one patient but failed to identify microscopic disease in the opposite testis or in the testes of the second patient. Percutaneous kidney and liver biopsies, pelvic ultrasonography, intravenous pyelogram, skeletal survey, cranial computed axial tomography scan, electroencephalography, and ophthalmologic examinations failed to demonstrate evidence of occult disease. Except for testicular biopsy, this study does not support extensive clinical or invasive procedures to identify extramedullary disease prior to discontinuation of chemotherapy.

Adolescent↗
PubMed1978

Value of fiberoptic gastrointestinal endoscopy in infants and children.

Fiberoptic gastrointestinal endoscopy was performed on 52 patients between the ages of 2 months and 16 years. The procedures were safely and easily done with premedications consisting of meperidine, promethazine, and chlorpromazine, and atropine, with diazepam given at the time of the procedure. Fiberoptic endoscopy was particularly helpful in localizing the site of upper gastrointestinal hemorrhage, in retrieving foreign objects, and in removing colonic polyps. In contrast, endoscopy added little to the clinical history and roentgenographic studies in children with recurrent abdominal pain or vomiting, in whom we believe fiberoptic endoscopy is not indicated unless the symptoms or history appear inconsistent with the diagnosis of functional bowel disease.

Abdomen↗
PubMed1977

Cholesterol ester storage disease: clinical, biochemical, and pathological studies.

Of three siblings affected with cholesterol ester storage disease, two died at ages 7 and 9 years, respectively, with hepatic scarring and portal hypertension. Lipid storage was documented in both patients, as were esophageal varices and aortic plaques in the older child. The third affected sibling, followed to 13 years of age, has hepatomegaly, hyperlipidemia, short stature, adrenal calcification, and acid lipase deficiency. Leukocyte extracts demonstrated deficiency of acid lipase in this patient. This autosomal recessive condition may be allelic with Wolman disease with a more malignant course in this family than in most reported cases.

Child↗
PubMed1976

Treatment of skin irritation around enterostomies with cholestyramine ointment.

Eight patients with inflammation of the skin about the cutaneous stoma responded to local application of a 20% cholestyramine ointment in a short period of time. Two children treated with the hydrophilic base (Aquaphor) alone did not respond to treatment. The ileal bile acid patterns of two children with ileostomies were found to be different from that of the control group. Ileostomy patients had a higher concentration for total bile acids and a higher percentage of free bile acids.

Bile Acids and Salts↗
PubMed1976

Modular Formula: an approach to management of infants with specific or complex food intolerances.

Modular Formula is a dietary preparation for use in treating infants with complex malabsorptive disorders and for weaning infants from total parenteral nutrition. With this formula the physician can alter the quality of the various nutrients normally fed to infants as well as concentrations of those nutrients. Infants who cannot tolerate existing proprietary formulas will often thrive with the judicious use of this flexible formula. Modular Formula provides the clinician with a useful aid in the diagnosis and treatment of chronic diarrhea of infancy.

Diarrhea, Infantile↗
PubMed1975

Variation in the phenotypic expression of beta-glucuronidase deficiency.

Defiency of beta-glucuronidase was demonstrated in serum, leukocytes, and cultured skin fibroblasts of two unrelated patients. One patient died at 2 9/12 years with a phenotype consistent with severe mucopolysaccharidosis; the other is 14 years of age and well, except for hypertension and obstructive lesions of large blood vessels. Analysis of urinary mucopolysaccharides revealed impaired degradation of dermatan sulfate and, to a lesser extent, of heparan sulfate. Cultured skin fibroblasts accumulated excess glycosaminoglycans (the term glycosaminoglycans is synonymous with acid mucopolysaccharides) as indicated by 35-SO-4 uptake.

Adolescent↗