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Biomedical subjects

C L Kien

Publications and source records attributed to C L Kien.

At least 55 records · Page 3Linked to original sources
PubMed1987

Dietary carbohydrate assimilation in the premature infant: evidence for a nutritionally significant bacterial ecosystem in the colon.

Carbohydrate energy absorption and breath hydrogen concentration were measured in 12 premature infants 28-32 wk gestational age and 2-4 wk postnatal age. Each of two groups of six infants were randomly assigned to receive one of two formulas that differed only in carbohydrate source: 100% lactose (LAC) or 50% lactose: 50% glucose polymer (LAC + GP). In 11 infants the peak breath hydrogen concentration suggested extensive colonic fermentation (range 44-239 ppm/5% CO2 or 44-239 microL/L per 50 mL/L CO2). An approximate 100% increase in lactose intake in the LAC group was associated with a similar increase in breath hydrogen concentration at 30, 60, and 120 min. None of the infants exhibited diarrhea or vomiting or developed delayed gastric emptying. Carbohydrate energy absorption (mean +/- SD) was, respectively, 86 +/- 5% and 91 +/- 3% in the LAC and the LAC + GP groups (p greater than 0.05). Thus, colonic bacterial fermentation may be critical to energy balance and to the prevention of osmotic diarrhea in premature infants fed lactose.

Bacteria↗
PubMed1987

A synthesis of research on nutrition education at the elementary school level.

This literature review emphasizes the importance of teacher training, teaching strategies, parental involvement, curricular concerns, administrative support, and social/cultural factors on the impact of nutrition education programs at the elementary school level. The characteristics, implementation, and effectiveness of selected elementary nutrition education programs are reviewed, and instructional methods and materials shown effective through research are identified and discussed.

Culture↗
PubMed1986

Vitamin E-deficient spinocerebellar syndrome due to intestinal lymphangiectasia.

A 23-year-old woman with congenital intestinal lymphangiectasia developed a progressive spinocerebellar syndrome and neuropathy. The clinical findings were typical of vitamin E deficiency, which was documented in the patient. Intestinal lymphangiectasia is an additional vitamin E-deficient disease that may cause this neurologic syndrome.

Adult↗
PubMed1985

Tissue nitrogen-sparing effect of high protein diet in mice with or without ascites tumor treated with Acinetobacter glutaminase-asparaginase.

Forty-eight tumor-free mice and 32 mice bearing Ehrlich ascites tumor were randomized into 2 treatments, Acinetobacter glutaminase-asparaginase (AGA) (600 IU/kg/day for 7 days) and 0.9% NaCl controls, and into 2 or 3 isocaloric diets, normal protein (NP) (20 g protein/100 g diet), high protein (HP) (58 g protein/100 g diet), and zero protein (ZP) (tumor-free mice only). In tumor-free, NP-fed mice, AGA caused percentage reductions (P less than 0.01) in the nitrogen content of liver (50%), intestine (42%), thymus (89%), spleen (75%), and carcass (20%), but HP prevented this effect on intestine and carcass and caused percentage increases in the nitrogen content of liver (53%), intestine (36%), thymus (122%), and carcass (25%). In Ehrlich ascites tumor mice (NP or HP fed) AGA caused markedly lower (P less than 0.01) tumor burdens and increased nitrogen content of intestine (HP), kidney (NP and HP), and spleen (NP and HP). Ehrlich ascites tumor, AGA-treated, HP-fed mice ate 31% less food (P less than 0.01) (compared to NP) but HP resulted in percentage increases in the nitrogen content of liver (18%; P = 0.05), intestine (25%; P less than 0.05), and thymus (164%; P less than 0.01). In the Ehrlich ascites tumor, AGA group the HP diet caused higher hematocrit and serum total protein (both, P less than 0.05). Adverse nutritional effects of AGA seen in normal mice were markedly diminished in tumor-bearing animals. The observed nitrogen-sparing effects of the high protein: energy ratio may be relevant to humans and to other forms of neoplasia and chemotherapy.

Acinetobacter↗
PubMed1983

Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency.

Late-onset multiple carboxylase deficiency is characterized clinically by skin rash, alopecia, seizures and ataxia and occasionally by candidiasis and developmental delay. Biochemically, these individuals exhibit findings consistent with a combined deficiency of the biotin-dependent carboxylases. We have found that the activity of the enzyme biotinidase is also deficient in the sera of five affected children (0 to 3% of mean control activity, 5.80 +/- 0.89 nmol X min-1 X ml-1 serum), and believe that it represents the primary biochemical defect in this disease. Biotinidase catalyzes the removal of biotin from the epsilon-amino group of lysine, through which biotin is covalently bound to the four known human carboxylases, thereby regenerating biotin for reutilization. The deficient activity in our patients was not due to an inhibitor, particularly biotin. It is also not a consequence of feedback control in affected individuals under treatment with pharmacologic doses of biotin. The biotinidase activities of the parents of those children who were available for study were intermediate between deficient and normal values (46% to 65% of mean normal activity). Children lacking biotinidase activity are unable to recycle biotin, and are thus entirely dependent upon exogenous biotin to prevent deficiency. Our findings indicate that the primary biochemical defect in late-onset multiple carboxylase deficiency is in biotinidase activity which is inherited as an autosomal recessive trait.

Amidohydrolases↗
PubMed1983

Phenotypic variation in biotinidase deficiency.

Biotinidase deficiency is the usual biochemical defect in biotin-responsive late-onset multiple carboxylase deficiency. We reviewed the clinical features of six patients with the enzyme deficiency and compared them with features described in the literature in children with late-onset MCD. In all of the reported probands, MCD was diagnosed because they had metabolic ketoacidosis and organic aciduria in addition to various neurologic and cutaneous symptoms, such as seizures, ataxia, skin rash, and alopecia. Although in several of our patients biotinidase deficiency was also diagnosed because they manifested a similar spectrum of findings, others never had ketoacidosis or organic aciduria. Thus the initial features of biotinidase deficiency usually include neurologic or cutaneous symptoms, whereas organic aciduria and MCD are delayed, secondary manifestations of the disease. These findings suggest that biotinidase deficiency should be considered in any infant or child with any of these neurologic or cutaneous findings, with or without ketoacidosis or organic aciduria. If the diagnosis cannot be excluded, such individuals should be given a therapeutic trial of pharmacologic doses of biotin.

Amidohydrolases↗
PubMed1983

Manifestations of chronic selenium deficiency in a child receiving total parenteral nutrition.

A child receiving total parenteral nutrition for about 1 1/2 yr developed intermittent leg muscle pain and tenderness and elevation in serum activities of glutamic oxaloacetic transaminase, glutamic pyruvic transaminase, and creatine kinase. Approximately 6 months later he developed white fingernail beds. Detailed cardiological evaluation revealed no evidence of cardiac muscle cell damage despite markedly elevated serum activities of the MB isoenzyme of creatine kinase. Retrospective analyses of serum and 24 hr urine samples collected during this period demonstrated very low serum Se concentration (0.003 to 0.007 micrograms/g) and urine excretion (0.0 to 8.9 micrograms/day) Se. Intravenous Se supplementation (42 micrograms/day elemental Se as H2SeO3) and a prospective study of biochemical Se status were begun 33.5 months after initiation of total parenteral nutrition. Serum Se concentration and whole blood glutathione peroxidase activity and Se concentration were respectively 0.020 mu/g, 3.5 EU/g Hb, and 0.018 microgram/g, 1 month after intravenous Se therapy was started. These very low values increased after further Se therapy. Serum enzyme activities markedly improved and the fingernail bed abnormalities resolved after therapy.

Alanine Transaminase↗
PubMed1983

Increased whole-body protein turnover in sick children with newly diagnosed leukemia or lymphoma.

Using a single dose, [15N]glycine turnover technique, whole body rates of proteins synthesis and breakdown were assessed in six healthy children and in eight children with newly diagnosed leukemia (DeWys, W. D. Cancer Res., 42: 721s-726s, 1982) or lymphoma (Baracos, V., Rodemann, H. P., Dinarello, C. A., and Goldberg, A. L. N. Engl. J. Med., 308: 553-558, 1983). Based on excretion of 15N as urinary ammonia, synthesis (g protein per kg body weight per day) was significantly (p less than 0.025) higher in the cancer patients [5.4 +/- 1.5 (S.D.)] compared to the controls (3.6 +/- 0.9); breakdown was also higher (p less than 0.02) in the patients (5.5 +/- 1.8) compared to the controls (3.1 +/- 1.1). When only the seven patients with leukemia were considered, there also were significant increases in synthesis (5.4 +/- 1.6, p less than 0.05) and breakdown (5.4 +/- 1.9, p less than 0.025) compared to controls. Increases in both synthesis and breakdown were also observed in the patients when the protein turnover data were expressed as a function of the rate of creatinine excretion or the calculated lean body mass. We conclude that whole body protein turnover is increased in sick children at the time of diagnosis with some forms of newly diagnosed cancer.

Ammonia↗
PubMed1982

A method for assessing carbohydrate energy absorption and its application to premature infants.

A method was developed for assessing indirectly the fecal excretion of carbohydrate-derived energy. Then, eight healthy premature infants (28 to 32 wk gestation, postnatal age 12 to 30 days) were randomly assigned to receive one of two formulas that differed only in the carbohydrate source: 100% lactose or 50% lactose: 50% glucose polymer (lactose + glucose polymer). Excreta collections were analyzed for total nitrogen, urea nitrogen, ammonia, fat, and total energy. Carbohydrate energy absorption was calculated. The formulas were well tolerated and stool frequency, energy intake, weight gain, and nitrogen balance were not different in the two formula groups. Also, there were no significant intergroup (lactose versus lactose + glucose polymer) differences in the coefficients (%) (x +/- SD) of fat absorption (90 +/- 6 versus 93 +/- 5) or carbohydrate energy absorption (96 +/- 1 versus 95 +/- 3). Thus, net carbohydrate-energy absorption appeared normal in these premature infants who showed no clinical formula intolerance.

Absorption↗
PubMed1981

Comparative analysis of fecal content of medium and long chain triglycerides using two methods.

Accurate assessment of apparent total fat absorption in individuals receiving medium chain triglycerides (MCT) as the predominant source of dietary fat depends upon the method used for fecal fat analysis. Erroneous conclusions may be drawn if the method does not give quantitative recovery of MCT. A simple, previously described, gravimetric technique is shown in this paper to give comparable MCT recoveries to another somewhat more arduous method.

Child↗
PubMed1981

Congenital cholinergic nervous system dysfunction in identical twins.

We report identical male twins with secretory diarrhea, craniofacial abnormalities often found in G syndrome, hypospadias, and abnormalities of the cholinergic nervous system including achalasia, decreased tearing, corneal hypesthesia, positive Mecholyl test (eye and esophagus), and decreased sweating. The children also lacked fungiform papillae but were clinically distinct from patients with either the Riley-Day syndrome or acquired dysfunction of the autonomic nervous system. Assessment of autonomic nervous system function should be considered in infants with recurrent diarrhea or swallowing disorders or in children with morphologically characteristics suggesting G syndrome.

Abnormalities, Multiple↗
PubMed1981

Biotin-responsive in vivo carboxylase deficiency in two siblings with secretory diarrhea receiving total parenteral nutrition.

Two siblings with a congenital syndrome of secretory diarrhea and seizures developed progressive skin rash, alopecia, and mucocutaneous candidiasis while receiving biotin-free total parenteral nutrition. Abnormally low urinary biotin excretion was associated with these clinical findings, but the serum concentration of biotin was within the normal range. There was also increased urinary excretion of lactic acid, 3-hydroxyisovaleric acid, 3-hydroxypropionic acid, and 3-methylcrotonylglycine. The younger of the two children subsequently died with severe metabolic acidosis. In the oder sibling, intravenous treatment with biotin (200 micrograms/day) resulted in resolution of the organic aciduria. A larger dose (10 mg/day) appeared to be required for rapid improvement in the skin lesions. These cases suggest that clinically significant biotin deficiency can occur in patients with chronic diarrhea receiving biotin-free total parenteral nutrition.

Biotin↗
PubMed1981

Protein-energy nutritional status of pediatric cancer patients.

Surveys of protein-energy nutritional status were conducted in pediatric cancer outpatients (28) and inpatients (71). In the outpatients, the approximate prevalences of severe (greater than 40%) deviations of weight, triceps skinfold, and arm muscle area below the medians for height-age were respectively 0, 14, and 0%. In the inpatients, these prevalences were respectively 1, 20, and 6%. The percentages of inpatients with abnormally low (less than 5th percentile) weight-for-height, triceps skinfold, and arm muscle area were 14, 14, and 20%, respectively. All inpatients with triceps skinfold or arm muscle area less than 60% of the median for height-age had corresponding percentile values of less than 15.

Body Height↗
PubMed1981

Fecal characteristics in healthy young adults consuming defined liquid diets or a free-choice diet.

Fat and nitrogen absorption and fecal characteristics were studied in 10 healthy, young adults who ingested a free-choice diet for 12 days and then isocaloric amounts of one of two nutritionally complete liquid defined formula diets during a succeeding 12-day period. Apparent fat absorption was 98 to 99% of intake with both defined formula diets and 95% of intake on the free-choice diet. Nitrogen digestibility was determined during the liquid diet period, and averaged 95 to 98% of intake. There were no statistically significant differences between the liquid diets and the free-choice diets with respect to fecal wet or dry weight, transit time, or stool frequency. Individual stool weights averaged 83 to 96 g on the free-choice or liquid formula diets and were comparable to other values reported in the literature. These results support the theory that a critical volume of feces must accumulate in the colon before defecation occurs, regardless of diet composition.

Absorption↗