Thomas F. Dolan

Clinical features as predictors of functional status in children with cystic fibrosis

Previous clinical studies in patients with cystic fibrosis have demonstrated substantial variability in the symptoms present at diagnosis and in subsequent survival rates. In this study we assessed the association between features present at diagnosis and the clinical course of cystic fibrosis in 89 patients. The 5- and 10-year outcomes for children with cystic fibrosis were better than has been generally appreciated. Overall, two thirds of the patients had either improved or remained at the same level of morbidity 5 years after diagnosis. Children who presented with isolated gastrointestinal symptoms had a good clinical course; some actually improved clinically during the first 5 to 10 years after diagnosis. In contrast, children who presented with respiratory disease frequently had clinical deterioration during the follow-up period. Neither age at presentation nor the initial level of morbidity was significantly related to subsequent outcome. We conclude that clinical features apparent at diagnosis are valuable prognostic indicators in children with cystic fibrosis.

Complications of iodide therapy in patients with cystic fibrosis

Forty-seven of 55 patients receiving long-term daily iodile therapy as part of the therapeutic regimen for cystic fibrosis of the pancreas developed goiters. Goiters usually appeared 2 to 3 years after onset of therapy, with a range of 3 months to 12 years. Fourteen patients receiving iodides had laboratory or clinical evidence of hypothyroidism; 2 of these 14 did not have a goiter. No goiters were observed among 55 patients with cystic fibrosis not receiving iodides. Fourteen of 55 children receiving iodide therapy developed nasal polyposis whereas no polyps were seen in 40 patients not receiving iodide. This may be due to the age distribution of patients studied, since most children in the 5 to 15 year age range received iodide therapy.

Evaluation of Arthritis and Arthralgia in the Pediatric Patient

The records of 250 consecutive children presenting to a university pediatric service with joint complaints of unknown cause were reviewed to determine the frequency of diagnoses and the utility of laboratory data and physical examination findings. Eighteen per cent of children had orthopedic disorders (Group I), 17.6 per cent had autoimmune disorders (Group II), 19.6 per cent had joint complaints related to a bacterial infection (Group III), and 44 per cent had miscellaneous problems (Group IV). Autoimmune or infectious dis orders were eight times as likely if temperature was ≥38 C. and/or erythrocyte sedimentation rate was ≥30 mm/h was present than if absent (65% vs. 8% re spectively) ; the sensitivity of fever and/or elevated erythrocyte sedimentation rate was 93 per cent for Group II and III patients. The presence of rash was predictive of an autoimmune disorder in 67 per cent of the instances; a positive joint examination was seen disproportionately in Group I patients. A negative joint examination all but ruled out an infectious etiology. Other test results, such as diagnostic radiograms, WBC ≥ 15,000 per cu mm; or a positive ANA or rheumatoid factor were predictive but not sensitive indicators of selected groups. If the etiology of a childs joint complaints is unknown, the likelihood of an orthopedic, autoimmune or infectious disorder may be suggested by reviewing temperature and ESR data and skin and joint findings.

Hemolytic Anemia and Edema as the Initial Signs in Infants with Cystic Fibrosis Consider This Diagnosis Even in Absence of Pulmonary Symptoms

This is a report of five infants eventually proven to have cystic fibrosis of the pancreas, who presented with hemolytic anemia and edema. Since the sweat test is often unreliable in edermatous states, the possibility of cystic fibrosis should be considered. Use of either a protein hydrolysate or addition of pancreatic enzymes will improve nutritional status, and when edema disappears, a definite sweat test can be performed. Evidence of vitamin E deficiency in some of the cases is presented, and the possibility of this as a cause of anemia is raised.

Predicting Fever Response of Children with Pneumonia Treated with Antibiotics

In order to study predictors of fever response in children with radiologic pulmonary infiltrates treated with antibiotics, 156 children with pneumonia were evaluated with slide test C-reactive protein (CRP), white blood cell count (WBC), erythrocyte sedimentation rate (ESR), blood cultures, acute and con valescent viral and mycoplasma titers, and then followed clinically. Both CRP (+) at a serum dilution of 1:50 and WBC ≥ 15,000 were better predictors of rapid resolution of fever while the patient was receiving antibiotics than were ESR ≥ 30 or temperature ≥ 40 C. WBC ≥ 15,000 was nearly as specific but more sensitive than CRP (+) 1:50 for resolution of fever in either 8, 12 or 24 hours. Positive blood or lung bacterial cultures, but not four-fold or greater viral or mycoplasma titer increases, were also associated with rapid resolution of fever. WBC ≥ 15,000 is useful in predicting rapid fever response in children with pneumonia treated with antibiotics.

Mild Cystic Fibrosis Presenting as an Asymptomatic Distended Appendiceal Mass: A Case Report

A case of cystic fibrosis presenting as a right lower quadrant mass in a five-year-old white female is presented. At laparotomy, a mucoid, impacted appendix was found which microscopically suggested the diagnosis of cystic fibrosis. Cystic fibrosis must be added to conditions presenting with abdominal masses.

Bronchial asthma and allergic rhinitis associated with inhalation of pancreatic extracts.

Five parents of children with cystic fibrosis developed allergic bronchospasm after inhaling pancreatic extracts sprinkled on their children’s food. There was a positive history of allergy, but no past history of asthma in any of the 5 parents. Asthma responded to bronchodilators, and use of a face mask during preparation of meals prevented recurrences. To date, asthma precipitated by inhalation of pancreatic extracts has not been seen in any of the patients with cystic fibrosis.

Abnormal Sweat Chloride in Auto-immune Hypothyroidism

A case of an 11-year-old girl with autoimmune hypothyroidism and a positive sweat test is presented. When the hypothyroidism was corrected the sweat test reverted to normal.