Carolyn R. Denning

Pulmonary hypertension and cor pulmonale in cystic fibrosis of the pancreas

The present study was concerned with the pathogenesis of pulmonary arterialhypertension in 21 patients with the pulmonary complications of cystic fibrosis. Respiratory function tests failed to distinguish between patients with normal pulmonary arterial pressure and those with pulmonary hypertension. On the other hand, the pulmonary arterial pressure was directly related to the degree of hypoxia. Relief of hypoxia, either acutely by breathing high-oxygen mixtures, or chronically by the treatment of respiratory infection and bronchial drainage, returned pulmonary arterial pressure toward, or to, normal. These physiologic observations imply a predominant role for hypoxia in the pathogenesis of pulmonary hypertension in cystic fibrosis, and indicate its potential reversibility in this disease.

Blood and Liver Concentrations of Vitamins A and E in Children with Cystic Fibrosis of the Pancreas

Extract: The median vitamin A level in plasma of patients with cystic fibrosis of the pancreas (CFP) (18 μg/100 ml) was less than half that of a control group (39 μg/100 ml), whereas the median vitamin A level in liver (57 μg/g) was nearly 3.5 times higher (Table II.). Concentrations of vitamin E in both the plasma and the liver of the patients were less than one-third those of the control group. The data suggest that vitamin A-supplemented children with CFP are unable to maintain normal circulating levels of vitamin A because of a defect in mobilization or transport from storage tissue, rather than because of tissue depletion. Low blood levels of vitamin E, however, reflect tissue depletion and not defective transport.Speculation: The cause of the apparent defect in ability to mobilize or transport vitamin A from the liver in children with CFP may be due to a limited ability to synthesize transport proteins or to a reduced activity of hepatic retinyl ester hydrolase enzymes These alternatives are under investigation.

Treatment of pulmonary infections in patients with cystic fibrosis: A comparative study of ticarcillin and gentamicin

The effectiveness of ticarcillin against Pseudomonas aeruginosa in acute exacerbations of pulmonary infection in patients with cystic fibrosis was evaluated. Seventy-one percent of patients treated with ticarcillin alone responded favorably. The response rate was similar in patients treated with a combination of ticarcillin plus gentamicin or with gentamicin alone. Severity of the underlying disease was the most important determinant of response to treatment. Ticarcillin-resistant organisms were recovered during treatment in 50% of patients who received this drug; recovery of them was not prevented by the inclusion of gentamicin in the therapeutic regimen nor did they interfere with clinical improvement. The ticarcillin-resistant strains persisted at follow-up, two to six months after completion of therapy, in only one of ten patients. No serious toxicity to ticarcillin was noted during the study period.

Parotid and submaxillary calcium in human cystic fibrosis

Abstract Examination of salivary calcium concentration by atomic absorbtion spectrophotometry revealed that: (1) parotid as well as submaxillary total calcium was elevated in C F patients; (2) most of the elevation in submaxillary calcium was in the dialysable fraction; (3) submaxillary calcium concentration in C F could not be correlated with disease severity; and (4) heterozygotes did not exhibit elevations in submaxillary calcium.

Discoloration of the teeth in patients with cystic fibrosis of the pancreas: Histologic studies☆

Abstract Detailed histologic studies of teeth obtained from thirty-two patients with cystic fibrosis of the pancreas were undertaken with regard to the presence, distribution, and nature of the peculiar discoloration of the teeth observed in such patients. A yellow discoloration in dentine was usually observed in ground sections of these teeth when viewed by tungsten incandescent light. Fluorescence microscopy of these sections revealed fluorescent patterns which appeared to parallel the yellowish patterns observed under tungsten light. No such abnormalities were discernible in decalcified sections studied under tungsten light.

Discoloration of the teeth in a 24-year-old patient with cystic fibrosis of the pancreas not primarily associated with tetracycline therapy. Report of a case.

Abstract A case is presented in which there was marked discoloration of the permanent dentition in a 24-year-old woman with cystic fibrosis of the pancreas who could not possibly have received a “tetracycline” until she was 8 years of age and who is not known to have received tetracycline until the age of 12. This case is presented to support the suggestion that the discoloration of the teeth seen in patients with cystic fibrosis of the pancreas should not necessarily be assumed to be entirely the result of “normal” tetracycline deposition but that there may be some other factor (or factors) within the spectrum of cystic fibrosis of the pancreas itself which also plays either a predisposing or a definitive role.

Biological Activities of Cystic Fibrosis Serum. IV. Stimulation of the Calcium Mediated K + Efflux from Rat Submandibular Gland Fragments

Summary: Cystic fibrosis (CF) and heterozygote sera stimulate a significant K+ efflux from rat submandibular gland fragments in the presence of 1 mM ouabain. This sensitive parameter can be maximally stimulated by as little as 0.5% CF serum and is inhibited by the calcium channel blocker D600 and EGTA. Specific receptor blockers propranolol, phenoxybenzamine or atropine do not inhibit the CF serum-stimulated K+ efflux and agonists do not supramaximally stimulate K+ efflux when added with serum. CF serum-induced K+ efflux did not result in the leakage of lactic dehydrogenase into the bathing media nor did it mimic the action of the calcium ionophore A23187 when added in the presence of D600. In addition, ultrafiltrates of CF serum (less than 10,000 daltons) also stimulated K+ efflux from rat submandibular gland tissue fragments.Speculation: Cystic fibrosis serum factor(s) may be altering membrane permeability to calcium which results in the release of K+ from rat submandibular gland fragments.

Hypercatabolic hypoproteinemia with lymphangiectasia of the small bowel: A case report†

A 6-month-old girl with protein losing enteropathy due to intestinal lymphangiectasiais presented. The findings of an I 131 albumin turnover study as well as observations made by light and electron microscopy of a jejunal biopsy are reported. From these findings, it appears that protein is lost into the intestinal lumen by way of increased activity of the goblet cells.

Tetracycline discoloration of teeth: Diagnosis by long-wave and short-wave ultraviolet light: A comparative study

T here is ample literature documenting the discoloration-inducing effects of various members of the tetracycline family when administered to patients during the period of tooth formation. However, since other tooth-discoloring factors are also known to exist, suitable techniques for differentiating tetracyclineinduced discoloration from discoloration due to other factors have been sought. One of the more widely employed methods has been examination of the dentition for evidence of a characteristic fluorescence which is observable when tetracycline-discolored teeth are viewed under ultraviolet light and, conversely, for the absence of fluorescence when the discoloration is the result of factors other than tetracycline deposition. It has been established that tetracycline fluorescence can be excited by ultraviolet light of varying wavelengths. As a matter of fact, some investigators have employed a short-wave light source of approximately 2,537 A, and others have used a long-wave light source of approximately 3,660 A. This study was undertaken to determine, if possible, the comparative attributes of ultraviolet sources of these varying wavelengths in inducing fluorescence of tetracycline-discolored teeth. The comparative efficacy of using the 2,537 A (short-wave*) and the 3,660 A (long-wave*) ultraviolet light sources was determined by studying the observ-

CONTINUOUS USE OF ORAL ANTIBIOTICS IN THE MANAGEMENT OF PATIENTS WITH CYSTIC FIBROSIS

This study was designed to determine the effectiveness of continuous oral antibiotics versus antibiotics used only during episodes of illness in patients with cystic fibrosis. Twenty-two patients with cystic fibrosis were divided into two groups according to age, sex, and pulmonary status, and in a double blind fashion one group (A) was given cloxicillin (50 mg/Kg/day) and the other group (B) a placebo for 12 months. All other parts of the basic C.F. treatment program remained unchanged. The patients were evaluated quarterly using the following parameters: sputum volume and color, cough, physical examination, CBC, ESR, chest radiograph, pulmonary function tests and blood gas analysis. Of the 11 patients in group A, 10 improved or remained stable and 1 showed deterioration, whereas, in group B, 6 Improved or remained stable and 5 showed worsening. The data indicates that there is less progression of the pulmonary component of cystic fibrosis in patients who received continuous cloxicillin.