Judy Palmer

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.

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...

Pulmonary resection for complications of cystic fibrosis

Pulmonary sequelae account for a large proportion of the morbidity and mortality of cystic fibrosis. Bronchiectasis, hemoptysis, and abscess formation are often not responsive to conservative medical therapy. Pulmonary resection in selected cystic fibrosis patients is safe and therapeutically rewarding. Eleven pulmonary resections in ten patients with cystic fibrosis were performed. Patients ranged from 2.5 to 19 years of age. Indications for resection were: (1) abscess and bronchiectasis (nine patients), (2) atelectasis and mass (one patient), and (3) life-threatening hemorrhage (one patient). Surgical resection was employed only for medically refractory lesions which were life-threatening or contaminated otherwise functional lungs. Resection was limited to the most severely diseased areas, sparing functional lung parenchyma when possible. In this series, 9 lobectomies, 1 pneumonectomy, and 1 segmentectomy were performed. Preoperative management included aggressive chest physiotherapy and postural drainage, rigid bronchoscopic lavage, and broad-spectrum parenteral antibiotics. All patients were extubated in the operating room immediately postoperatively. Tracheostomy was not employed. There were no perioperative complications. All patients experienced subjective improvement. Objectively, improvement following surgical resection included: decreased cough and sputum production, and decreased incidence of exacerbations of pulmonary infections. Limited pulmonary resection when combined with intensive preoperative pulmonary toilet is a safe adjunct in the treatment of bronchiectasis and hemoptysis secondary to cystic fibrosis. Pulmonary resection should be limited to only severely destroyed lung parenchyma which is refractory to medical management. In contradistinction to other authors we have not found tracheostomy a necessary adjunct in surgical management.

Evaluation of a Paper-patch Test for Sweat Chloride Determination

This article evaluates a recently developed qualitative sweat patch test (SPT). A battery- powered stimulator with polymer pad electrodes is used to stimulate sweating, followed by the application of a paper patch which collects the sweat and shows color change on sweat chloride levels above 40 to 50 mEq/l. Our data consist of 66 patients with cystic fibrosis, 75 control subjects, and 37 relatives of patients with cystic fibrosis, excluding 22 subjects (11%) with insuffacient sweat. There were no false-negative results among 66 patients with cystic fibrosis, and in the control group, 71 of 75 subjects gave negative results and the other four gave positive results. Of the 37 relatives, eight gave positive results; all of the eight subjects are parents of patients with cystic fibrosis. Our results indicate that SPT is useful and reliable when there is sufficient sweat. This portable and simple patch test may serve as an excellent screening test for cystic fibrosis.

1219 RESPIRATORY EVALUATION OF CHILDREN IN A CHLORINE GAS DISASTER: IDENTIFICATION OF RISK FACTORS

On 9/30/77 a chlorine gas leak at a water treatment facility in Philadelphia forced the evacuation of a housing project. In the next 12 hours, 56 children were brought to the emergency room and evaluated by physical exam (PE), chest x-ray (CXR), and pulmonary function tests (PFT). 29/56 were well and had normal PE. All 29 remained well on followup at 24 hrs. and again at 3 mos. A positive past respiratory history (PPRH) was present in only 5.27/56 were symptomatic and had one or more abnormal findings on initial evaluation, (25 PE, 2 CXR, 10 PFT). Arterial blood gas (ABG) studies showed hypoxia in 7/26 patients (pO2 55-89 torr) Therapy consisted of IPPB and physical therapy and postural drain age; 10/27 required bronchodilators for severe or persistant pronchospasm which occurred in 8/10 with a PPRH but in only 4/17 without a PPRH. Abnormal PFT were found in 7/10 with a PPRH but in only 3/17 without a PPRH. On followup, 10 were symptomatic at 1 week, 6(5 with PPRH) were symptomatic at 3 months.In summary, acute exposure to chlorine gas may cause respiratory symptoms and transient abnormality of PFT and ABG, especially in children with asthma. PE and PFT were useful in identifying those patients requiring hospitalization. A PPRH identified the majority of those who developed persistant respiratory difficulty.

BATH TUB NEAR-DROWNING AS A MANIFESTATION OF THE CHILD NEGLECT SYNDROME: CLINICAL, BIOCHEMICAL AND SOCIAL ASPECTS

Among 17 children with near-drowning seen in a 6 year period at an urban Pediatric center, 10 involved bath tub accidents (average age 1.2 yrs.). The most important determinant of outcome was their condition on admission. All of 6 children who were alert and breathing did well on minimal therapy (O2 and NaHCO3) with an average of 2.8 hosoital days. Four apneic comatose patients required in addition, steroids, blood transfusion, tracheal intubation and mechanical ventilation, and were hospitalized an average of 14 daysi 3/4 died. Among those in whom studies were obtained less than 2 hours after the accident, findings were as follows: acidosis (mean pH 7.0) in S cases; hypercapnia (mean pCO2 62) in 4 cases; a-A gradient (mean pO2 91) in 3 cases (all receiving O2); and mild hyponatremia (mean Na 132) in 6 cases.In 9/10 cases the child was unattended in the bath tub. Social Service was consulted in 7/10 cases, and formal child neglect forms were filed in 6 cases. Two cases had been previously reported for neglect. One mother was under psychiatric care. In 3 cases severe social disorganization was evident. In 5 cases the parents were unaware of the hazard of leaving infants unattended in the bath tub. Prevention of bath tub near-drowning will require a more active effort by health professionals to educate such high risk families about this danger.

Pregnancy in Patients with Cystic Fibrosis

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 a total of 11 completed pregnancies and had been evaluated within 1 year before conception. In five women (Group 1), the overall maternal condition was little affected by the pregnancy, and in three women (Group 2), the mothers condition deteriorated during and after pregnancy and did not return to the pregravid state. With regard to pregravid status, significant differences between patients in Group 1 and Group 2 were found in Shwachman-Kulczycki clinical scores, weight for height values, Brasfield chest radiograph scores, and pulmonary function. A quantitative assessment of pregravid nutritional and pulmonary status is useful in counseling women with cystic fibrosis about the risk of pregnancy.

1710 CYSTIC FIBROSIS AND PREGNANCY |[mdash]| PREDICTIVE FACTORS

With improved survival in cystic fibrosis (CF), women with CF are now seeking advice about the risk of pregnancy. To identify predictive factors, 8 pregnancies in 7 women, evaluated within 1 year prior to conception, were studied. Five pregnancies occurred in 4 women who did well (Group I) and 3 pregnancies occurred in 3 women who deteriorated and did not regain pregravid nutritional or pulmonary status post partum (Group II).The groups were similar in age and height. Group I had better weight gain during pregnancy, fewer hospitalizations, normal length gestation and normal birth weight infants. There were no deaths during pregnancy, however, 2 women in Group II died post partum. Although 2 infants of Group II mothers were premature, all infants survived and none had CF. These data suggest that comprehensive assessment of pulmonary and nutritional status is useful to predict the maternal outcome of pregnancy in CF.

PULMONARY COMPLICATIONS OF HYPOGAMMAGLOBULTNEMIA

Ten boys with congenital hypogammaglobulinemia from 4 families have been followed for 7-18 years. Five were diagnosed in infancy. Serum IgG was below 1 mg/ml in all but one (2.1 mg/ml after therapy), IgA below 0.05 mg/ml in all and IgM below 0.1 mg/ml in all but one (0.2 mg/ml). All received gammaglobulin from diagnosis and 5 plasma for ½ to 4 years. All had multiple episodes of acute bronchitis and pneumonia beginning early. H.influenzae (non-typeable) and Staph.aureus were the major pathogens, viruses were not found. One child age 10, died of bronchopneumonia and sepsis complicating adrenocorticosteroid treated chronic myositis. He and 3 of the 7 surviving children aged 7, 9 and 13 years, have no definite chronic pulmonary disease, though acute infections were often followed by a persistent cough in spite of intensive antibiotic therapy. This phenomenon led to bronchiectasis in the other 6. Two died of pulmonary insufficiency at ages 15 and 20. Among those surviving with bronchiectasis, 2 have severe restrictive and obstructive patterns in pulmonary function and 3 aged 16-21 had lobectomy. Pathological examination revealed severe bronchiectasis and sclerosis of bronchial arteries. The severity of pulmonary disease could not be correlated with initial serum immunoglobulin levels, proportion of B lymphocytes in peripheral blood, age at onset of gammaglobulin therapy, serum immunoglobulin levels during therapy or antibiotic therapy.