Nancy N. Huang

Growth patterns in children with cystic fibrosis

The growth patterns of 50 children with cystic fibrosis have been studied. Serialmeasurements of height and weight were obtained monthly or bimonthly. Bone age was determined at the end of the observation period in 40 children. Significant retardation of physical growth was observed in all age periods; it was most pronounced in the preadolescent and adolescent age groups. The medians of the height and weight measurements were between the third and tenth percentiles of children with normal health. Skeletal maturation was delayed in 38 per cent of the children studied. A significant correlation was demonstrated between growth retardation and the severity of pulmonary involvement; no correlation was found between retardation of growth and the degree of pancreatic insufficiency. Upon institution of therapy, most children had a normal or above-normal rate of gain in weight. Such gains were greatest when therapy was initiated during infancy.

The fatty acid composition of the serum chylomicrons and adipose tissue of children with cystic fibrosis of the pancreas

Summary 1. The fatty acid compositions of theserum chylomicrons and of adipose tissues of 15 children with cystic fibrosis of the pancreas and of 12 controls of similar ages but without clinical evidence of impaired intestinal digestion and absorption were determined. 2. The serum chylomicrons of childrenwith cystic fibrosis were found to contain increased amounts of palmitoleic and oleic acids and decreased amounts of palmitic, stearic, and linoleic acids. 3. Two types of fatty acid patterns of depot fat are described in children with cystic fibrosis: (a) In the case of little or no absorption of fat the composition of the depot fat reflects active endogenous lipogenesis from carbohydrate; this pattern is also observed in very young infants without cystic fibrosis. (b) In most of the older children with cystic fibrosis the impairment in fat absorption is rarely extreme. In them, the increased tendency to selective absorption of oleic acid is superimposed upon an increased endogenous lipogenesis to produce a pattern characterized by relatively high content of palmitoleic and oleic acids and relatively low content of palmitic, stearic, and linoleic acids. This pattern of depot fat is also observed in adults with the malabsorption syndrome. 4. Improvement in the absorption of fatin children with cystic fibrosis following the administration of Cotazym failed to produce a significant change in the abnormal pattern of the depot fat in the majority of the children studied for periods of 3 to 9 months. In fact, the content of oleic acid may continue to increase with Cotazym therapy. 5. In view of the demonstration that oleic acid and perhaps other monounsaturated fatty acids are more readily absorbed than most of the other fatty acids commonly found in foods, a diet with relatively high monounsaturated fatty acid content and low in saturated acids may be helpful in improving the nutritional status of these patients. 6. This study may also serve as a basisfor studying the relationship of diet, and dietary fat in particular, to coronary artery disease.

Clinical features, survival rate, and prognostic factors in young adults with cystic fibrosis☆

The medical records of 142 patients with cystic fibrosis were reviewed. The patient group included 78 males and 64 females; three patients were black. Periods of observation ranged from two to 25 years (mean, 14.5 years). The analysis focused on clinical evaluation at age 18 years and included information gained at an earlier age. Evaluation at age 18 years was based on Shwachman and Kulczyckis (S-K) scoring system, Brasfield chest roentgenographic scoring system, pulmonary function measurements, height-adjusted weight percentile, sputum bacteriologic results, number of hospitalizations for treatment of pulmonary infections prior to the age of 18 years, time of onset of clubbing, and frequency of complications. There were no significant differences between the sexes in clinical features. Median survival from the time of diagnosis to the conclusion of the study period (1955 to 1984) was 22 years for females and 25 years for males (NS). Median length of survival beyond the age of 18 years was eight years for females and 12 years for males (NS). Stepwise logistic regression and Cox regression analysis applied to 11 variables identified the S-K clinical score at 18 years of age as the best predictor of survival to the age of 23 years. The median durations of survival after the age of 18 years for patients with clinical scores of 30 to 49, 50 to 64, and 65 to 75 at age 18 were five, seven and a half, and 12 years, respectively (p less than 0.0001). Low clinical score, low weight percentile, and Pseudomonas cepacia colonization of the lower respiratory tract at the age of 18 years indicated a poor prognosis. On the other hand, high clinical score, good weight percentile, and colonization with Staphylococcus aureus alone were likely to be found in patients with mild disease and an increased likelihood of long-term survival with preserved pancreatic function.

Comparison of serum levels following the administration of oral and parenteral preparations of penicillin to infants and children of various age groups

Summary 1. Penicillin levels in the serum of infants and children of various age groups were obtained following the administration of two oral and two parenteral preparations of penicillin. The various preparations were given in single doses of 10,000 units per pound of body weight. 2. Distinctly higher and more sustained blood levels were observed in the full-term newborn and premature infants than in older infants and children after the administration of comparable doses of all four preparations. The possible explanations for these differences have been discussed. 3. Minor differences have been observed in the levels following the administration of orally administered potassium penicillin-G and orally administered procaine penicillin. 4. Orally administered penicillin in doses of 10,000 units per pound of body weight produced levels of at least 0.03 unit per millititer for a minimum of six hours in full-term newborn and premature infants, for a minimum of four hours in infants up to 2 years of age, and for at least two hours in children over 2 years. 5. In all age groups intramuscularly administered potassium penicillin-G in doses of 10,000 units per pound of body weight resulted in very high initial levels. The levels declined rapidly, however, so that the 6-hour levels were comparable to those observed after the oral administration of penicillin in all age groups except the youngest newborn infants. 6. Intramuscularly administered procaine penicillin in doses of 10,000 units per pound of body weight produced high, sustained levels for six hours; detectable levels were often demonstrable at the end of twentyfour hours. 7. Suggestions have been made for dosage regimens for orally administered penicillin.

The flora of the respiratory tract of patients with cystic fibrosis of the pancreas

Summary 1. Bacterial flora of the upper and lowerrespiratory tract of patients with cystic fibrosis of the pancreas and those with a chronic respiratory tract of patients with cystic fibrocauses have been studied. 2. The results showed a markedly increased incidence of Staph. aureaus in the throat and bronchial secretions, and of Ps. aeruginosa in cultures obtained from all the sites of the respiratory tract of CF patients. 3. In non-CF patients there was a significantlylower incidence of cultures yielding pathogens. Pneumococci and H. influenzae were isolated more frequently in non-CF patients than in CF patients. 4. A total of 251 strains of Staph. aureus which were isolated from nasopharyngeal and bronchial secretions of CF and non-CF patients were tested for their bacteriophage patterns. The results showed no difference in t the distribution of phage type of Staph. aureus isolated from the 2 groups of patients. 5. Antibiograms of a total of 101 strainsof Staph. aureus from the bronchial secretions and 109 strains from the nasopharyngeal secretions of CF patients were determined against 12 antibiotics. No significant difference was found in the antibiotic susceptibility pattern of the different phage types tested except that the type 80/81 strains were highly resistant to penicillin, streptomycin, and tetracycline. 6. All the strains of Staph. aureus tested were highly susceptible to bacitracin, ristocetin, vancomycin, and novobiocin, and fairly susceptible to neomycin, oleandomycin, erythromycin, chloramphenicol, and kanamycin, in decreasing order. The antibiotic susceptibility patterns of a total of 70 strains of Ps. aeruginosa against 7 antibiotics showed that these organisms were highly resistant to the antibiotics tested. Only polymyxin, neomycin, and oxytetracycline were fairly effective against some strains.

Pregnancy in patients with cystic fibrosis.

Abstract As more patients with cystic fibrosis reach adulthood, questions arise about the potential hazards of pregnancy. We reviewed the medical records of eight women with cystic fibrosis who had...

Carbenicillin in patients with cystic fibrosis: Clinical pharmacology and therapeutic evaluation

Carbenicillin, a new semisynthetic penicillin (alpha-carboxybenzylpenicillin) with activity against Pseudomonas aeruginosa , has been studied. Forty per cent of 110 strains of P. aeruginosa and most strains of Proteus mirabilis and Proteus morganii were susceptible to 62.5 μg per milliliter or less of carbenicillin. Of strains of Staphylococcus aureus tested, the majority were inhibited by 6.25 μg per milliliter of less of carbenicillin. Concurrent administration of probenecid is necessary to maintain effective serum levels for 2 to 4 hours after an intravenous dose. About 80 per cent of an intravenous dose is excreted in urine within 4 hours. Only 1 per cent of the antibiotic inhaled as an aerosol is recovered in urine. Fifty-four patients with cystic fibrosis, during 85 episodes of acute exacerbations of their pulmonary infections, had favorable clinical and radiologic responses to carbenicillin therapy. Transient elimination of P. aeruginosa was achieved in a small number of patients, but there was a striking increase in isolation of Klebsiella pneumoniae in end-treatment cultures. A rise of serum glutamic oxaloacetic transaminase activity was found in 40 per cent of the patients treated.

Visual disturbances in cystic fibrosis following chloramphenicol administration

Optic neuritis and retrobulbar neuritis with marked visual impairment occurred in 9of 33 patients with cystic fibrosis, who received long-term therapy with chloramphenicol. Two patients had severe visual impairment and permanent residual effects with partial optic atrophy. Vitamin B complex has been demonstrated to be therapeutic in some instances. It is thought that the visual impairment probably represents a neurotoxic effect of chloramphenicol rather than a complication of cystic fibrosis. All patients with cystic fibrosis who receive long-term therapy with chloramphenicol must have frequent tests for visual acuity and periodic ophthalmologic examinations.

Protein metabolism in cystic fibrosisof the pancreas

Metabolic balance studies were performed on 5 infants with cystic fibrosis of thepancreas. One of the subjects had hypoproteinemia and intermittent edema. Large quantities of nitrogen were excreted in the stools when the infants received a soybean formula. This was most marked in the hypoproteinemic patient and was considered to be the pathogenetic explanation of his hypoproteinemia. Nitrogen retention was significantly higher when the infants received an evaporated cows milk formula. A paracreatic enzyme preparation led to significant increase in nitrogen retention when added to soybean feedings but not when added to evaporated cows milk feedings. It is recommended that infants with cystic fibrosis of the pancreas should not be fed soybean milk preparations.

KANAMYCIN IN THE TREATMENT OF INFECTIONS IN INFANTS AND CHILDREN

Kanamycin sulfate, originally isolated and studied by Umezawa and his associates at the Japanese National Institute of Health and at Tokyo University, has wide antimicrobial Preliminary studies in the United States have confirmed this finding as reported in February 1958.6 The present study was undertaken to obtain preliminary information on the use of this drug in infants and children. Certain bacteriological studies were made on freshly isolated bacterial species. Serum concentrations of the drug were analyzed following a variety of intramuscular doses of kanamycin sulfate. Finally, a group of infants and children was treated with this new antimicrobial drug The results of these studies are reported below.