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Biomedical subjects

J D Lloyd-Still

Publications and source records attributed to J D Lloyd-Still.

At least 19 recordsLinked to original sources
PubMed1991

Essential fatty acid status and fluidity of plasma phospholipids in cystic fibrosis infants.

The fatty acid (FA) patterns of cord serum phospholipids (PLs) were examined in 4 cystic fibrosis (CF) newborns, 8 non-CF siblings, and 22 normal control subjects. Plasma from 27 newly diagnosed CF infants and 38 normal infants aged less than 2 y were studied for comparison. CF cord-blood PLs had patterns similar to those of CF siblings and to normal newborns, but the pattern for CF did not shift toward adult patterns during infancy as did patterns for normal infants. CF infants at diagnosis exhibited a deficiency pattern in which 18: 2 omega 6, 20:4 omega 6, 22:4 omega 6, an omega 3 polyunsaturated fatty acids (PUFAs) were significantly subnormal and 18:3 omega 6, total saturated fatty acids, and total monounsaturated fatty acids were significantly elevated compared with normal infants. In PLs in CF infants, although mean chain length of FAs was low, mean melting point was elevated 2.4 degrees C and double-bond index was low (both P less than 0.001), implying a significantly lessened fluidity. Nutritional supplements of both omega 6 and omega 3 PUFAs are cated.

Aging↗
PubMed1991

Impact of orthotopic liver transplantation on mortality from pediatric liver disease.

In 1983 we assessed the implications for hepatic transplantation programs by studying mortality from liver disease in a tertiary care children's hospital. The current study reviews the impact of orthotopic liver transplantation (OLT) on survival for the period 1984-1989. Findings showed that deaths from liver disease decreased from 9.2 to 3.8 per year. Twenty-eight infants and children were referred and underwent OLT, with a 64% survival rate. Deaths from biliary atresia, which used to account for 24% of the total, have been reduced to 4.3%. Deaths from liver failure in infancy (which decreased from 49% to 39%) still present formidable challenges for transplantation. The implications of these findings are discussed.

Adolescent↗
PubMed1990

Acylcarnitine is low in cord blood in cystic fibrosis.

Carnitine metabolites (total, free, short and long chain) were analyzed in cord blood of cystic fibrosis (n = 5), non-CF siblings (n = 7), and controls (n = 8). Total acylcarnitine (short and long chain combined) was significantly lower (less than 0.001) in CF compared to both control groups. Total and free carnitine showed no significant differences between the three groups. These findings are compatible with disturbed fatty acid metabolism in utero and may be related to the increased energy expenditure characteristic of CF infants.

Acylation↗
PubMed1990

Carnitine metabolites in infants with cystic fibrosis.

Decreased acylcarnitine levels have been found in cord blood of CF infants compared to siblings and controls. We therefore measured carnitine metabolites in blood and urine in 15 newly diagnosed (average age 4 mos) CF infants and followed the levels for one year. No consistent abnormality in carnitine status was detected in newly diagnosed infants; levels normalized at one year after therapy with predigested formula containing carnitine supplements. This data does not provide support for a primary abnormality of carnitine metabolism in CF.

Age Factors↗
PubMed1989

Neurovascular and thromboembolic complications of inflammatory bowel disease in childhood.

Vascular complications occurred in 3.3% (6 of 180) of children with chronic inflammatory bowel disease. Clinical disease activity was severe in four of six patients who presented with these symptoms. In contrast to adults, in whom deep-vein thrombotic complications predominate, the majority of children had involvement of the CNS including encephalopathy, arteritis, arterial occlusion, and thromboembolism. One patient died 3 1/2 years later from progressive intestinal and neurological deficits: morbidity included blindness, epilepsy, and developmental delay. Hematological parameters varied widely. Prevention is not always possible, but risk factors include: (a) family history of collagenosis (83% incidence), (b) severe inanition and immobility, (c) delayed hospitalization, and (d) clinical presentation with cutaneous vasculitis. Therapy must be individualized.

Adolescent↗
PubMed1989

Total fatty acids of hair lipids in cystic fibrosis.

In a preliminary study, total fatty acids of lipids removed from hair of subjects of either sex with cystic fibrosis (n = 17; average age 8.3 years) and controls (n = 24; average age 9.1 years) were analyzed by gas chromatography. In contrast to the blood lipids in cystic fibrosis which display various fatty acid changes as a depression in 18:2 and increases in 16:0, 16:1, and 18:1, such profiles did not occur with the hair lipids. With the latter, total fatty acids in cystic fibrosis showed decrements in 18:1 and in the lesser concentrations of 20:1 and members below C14 as compared to the respective control series.

Adolescent↗
PubMed1988

A prospective evaluation of iron chelation therapy in children with severe beta-thalassemia. A six-year study.

Sixteen patients (age range, 3 to 17 years) with transfusion-dependent beta-thalassemia major were studied prospectively, beginning at the onset of chelation therapy with deferoxamine (desferrioxamine). A liver biopsy specimen was obtained from each patient at the start of the study, and periodically thereafter. Liver histologic features, iron content, and iron excretion were assessed during the course of the study. Hepatic iron levels from liver biopsy specimens appeared to correlate well with serum ferritin levels in the younger less heavily iron-loaded patients; however, in patients with higher serum ferritin levels, hepatic iron appeared to reach a saturation level. Fourteen of the 16 patients showed a pattern of marbled fibrosis of the liver in their initial biopsy specimens. Follow-up biopsy specimens from nearly all of the patients showed a substantial reduction in iron concentration, but only two of seven patients showed improvement in the degree of hepatic fibrosis three to five years later. Patients less than 8 years old exhibited a normal pattern of linear growth until approximately the age of 10 years, followed by a progressive decrease to the 30th to 40th percentile. Two patients, aged 18 and 22 years, died of cardiac disease during the study. These findings suggest that chelation therapy in patients with transfusion-dependent thalassemia needs to be initiated at an early age, possibly before 3 years, if significant liver fibrosis and growth impairment are to be effectively prevented.

Adolescent↗
PubMed1988

Complex carbohydrate intolerance: diagnostic pitfalls and approach to management.

Complex carbohydrate intolerance occurred in three of 105 patients with protracted diarrhea of infancy. Nosocomial gastroenteritis complicated a primary disorder of carbohydrate absorption (primary glucose galactose malabsorption, two; primary sucrase isomaltase deficiency, one) in all patients. Their course was characterized by protracted diarrhea, variable degrees of villus atrophy on intestinal biopsy tissue, and negative caloric balance requiring intravenous alimentation for periods varying from 6 to 16 weeks. Dietary management required rigid exclusion of all offending carbohydrates from the diet. Delay in the diagnosis of primary carbohydrate intolerance varied from 2 weeks to 6 months. Complex carbohydrate intolerance may be more common than has been reported, and should be considered in all infants with protracted diarrhea of infancy when there is persistent carbohydrate intolerance.

Carbohydrate Metabolism, Inborn Errors↗
PubMed1986

The spectrum of Epstein-Barr virus hepatitis in children.

The clinical and pathologic features of Epstein-Barr virus (EBV) hepatitis in 3 children are described. Manifestations included fever, hepatomegaly, disseminated intravascular coagulation, and failure of uptake of technetium by the reticuloendothelial system of the liver. Histologic features may mimic chronic active hepatitis and lymphoid malignancy. Two patients underwent exploratory laparotomy because of suspected tumor. Recognition of the wide spectrum of hepatic involvement in infectious mononucleosis is important in the differential diagnosis of hepatomegaly. Diagnosis should be made by measurement of IgM-specific EBV antibodies.

Child↗
PubMed1979

A clinical scoring system for chronic inflammatory bowel disease in children.

A clinical scoring system for the assessment of children with chronic inflammatory bowel disease has been devised. A close correlation is demonstrated between severity of disease and the level of serum albumin. The clinical score is simple to perform, sensitive to changes in clinical status, reproducible by different observers, and specifically designed to evaluate inflammatory bowel disease in children and adolescents. The clinical score is a useful adjunct in the management of children with chronic inflammatory bowel disease and can be used in prospective studies of various therapeutic modalities.

Adolescent↗
PubMed1979

Chronic diarrhea of childhood and the misuse of elimination diets.

A prospective study was undertaken of 108 children referred for outpatient evaluation of chronic recurrent diarrhea. The majority of the children did not have a serious underlying disorder. Elimination diets (milk free, egg free, wheat free) were widely prescribed for the treatment of chronic diarrhea and were given for longer intervals than originally recommended. Elimination diets sometimes resulted in inadequate caloric intake and failure to thrive. Wheat (gluten)-free diets were prescribed for over one month in 59% of children without a specific diagnosis being made. These findings indicate that elimination diets are frequently misused, and prolonged adherence to elimination diets may result in nutritional damage. The indiscriminate use of wheat-free diets for the treatment of chronic diarrhea may be masking the diagnosis of celiac disease and may account for the low incidence of this disorder in the United States.

Chicago↗
PubMed1979

Serum glycine-conjugated bile acids in pediatric hepatobiliary disorders.

Measurements of serum bile acids (glycine conjugates of cholic, chenodeoxycholic, deoxycholic, and lithocholic acids) by radioimmunoassay in a variety of pediatric hepatobiliary disorders showed elevations in neonatal hepatitis syndromes, cholestasis, and hepatitis of extrahepatic or intrahepatic origin. Measurements of individual serum bile acids failed to differentiate between the various neonatal hepatitis syndromes. In one patient with cholestasis, the increased levels of bile acids observed returned to normal following therapy with cholestyramine and phenobarbital. In chronic active hepatitis the serum bile acid values correlated well with the bilirubin and SGOT in response to therapy with corticosteroids. These data confirm suggestions that serum cholylglycine and chenodeoxycholylglycine levels are a sensitive indicator of disturbed hepatic function and can be used in monitoring the course, activity, and therapeutic response in various hepatitis syndromes. In Reye's syndrome and protracted diarrhea of infancy, elevations in serum bile acids were detected without associated hyperbilirubinemia and provided additional evidence of disturbed hepatic function.

Adolescent↗
PubMed1979

Negative effects of oral fatty acid supplementation on sweat chloride in cystic fibrosis.

Essential fatty acid supplementation with oral safflower oil (1 gm/kg/day) to 11 cystic fibrosis patients (aged 6 months to 14 years) for one year produced no significant change in sweat chloride concentration (mEq/liter) or sweat rate (gm/min/m2), Addition of vitamin E (10 mg/kg/day) to the safflower oil had no effect on sweat chloride concentration or rate compared to placebo. No clinical improvement could be detected compared to a control group. These results do not support previous reports of the effects of fatty acid supplementation on sweat electrolyte concentrations in cystic fibrosis.

Adolescent↗