Neonatal screening for cystic fibrosis in Wales and the West Midlands: clinical assessment after five years of screening

Article Abstract:

Screening programs for diseases are based on the premise that the disease results in early symptoms and that if diagnosed early, it will respond to treatment. Screening for cystic fibrosis has been performed for a decade in Great Britain, and while the disease does indeed have early symptoms, there is no agreement on the ideal treatment program. Justification for cystic fibrosis screening has been based on the improved medical condition of affected children who are diagnosed earlier by screening, but studies of this issue have not been well designed. This article evaluated the benefit of early diagnosis performed in a screening program conducted since 1985. In the five years since the program's start, 227,183 newborns were screened and 246,959 were not. Screening using initial laboratory tests identified 58 infants without risk factors for cystic fibrosis who had the disease. Another 13 infants whose initial tests were negative (false negatives) also had the disease. Among unscreened infants, 66 with cystic fibrosis were eventually identified on the basis of clinical symptoms, 35 of whom were lacking risk factors for the disease. Screened infants were diagnosed at a significantly earlier age, an average of 9 weeks of age, while unscreened infants were identified at an average of 51 weeks (one year). Thirty percent of the screened group had both respiratory and gastrointestinal symptoms at diagnosis, compared with 75 percent of the unscreened group. Growth, overall health, and laboratory test results did not differ significantly between the groups. However, the unscreened group babies were hospitalized significantly more for problems associated with cystic fibrosis. Four deaths occurred, all in unscreened infants. The ongoing follow-up should confirm whether early diagnosis and treatment are beneficial in children with cystic fibrosis. (Consumer Summary produced by Reliance Medical Information, Inc.)

Diagnosis, Testing, Medical screening, Health screening

---

Neonatal diaphragmatic paralysis caused by chest drains

Article Abstract:

The inability to breathe after ventilation commonly occurs in premature infants or in cases where damage to the central nervous system or neuromuscular injury has impaired breathing. A case is described of a male infant delivered by cesarean section at 32 weeks gestation to a mother with preeclamptic toxemia, a toxic condition of pregnancy characterized by increasing high blood pressure, headaches, albumin (a blood protein) in the urine, and accumulation of fluid in the lower extremities. The infant developed rapid breathing and required breathing assistance at four hours of age. A chest X-ray confirmed respiratory distress syndrome, and the infant was given the drug atracurium to paralyse the diaphragm to facilitate use of the ventilator. The paralysis lasted 48 hours and the infant then developed pneumothoraces, or collections of air in the lungs. Tubes were inserted into the right and left lung cavities to drain the air. He required only minimal ventilatory support at seven days of age. However, when attempts were made to discontinue artificial ventilation, he was unable to breathe properly. Further testing showed that the infant had a damaged phrenic nerve, which controls the movement of the diaphragm. The damage to this nerve may have been caused by the insertion of the chest drains. The infant was on the ventilator for 52 days, gradually improved, and was thriving without respiratory problems at three month follow-up. This case illustrates the possibility of phrenic nerve injury as a result of inserting chest drains, and emphasizes the diagnostic importance of nerve conduction studies in such cases. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Causes of, Medical examination, Newborn infants, Respiratory therapy, Respiratory insufficiency, Diaphragm, Diaphragm (Anatomy), Paralysis, Pediatric respiratory diseases, Respiratory therapy for newborn infants, Respiratory insufficiency in children, Cervical plexus

---

Two enteric coated microspheres in cystic fibrosis

Article Abstract:

Cystic fibrosis is an eventually fatal, genetic disease that affects the respiratory system and exocrine glands. It usually begins in infancy and is characterized by repeated lung infections, a high amount of sodium in the sweat, and impaired function of the pancreas. The affected pancreas does not secrete the enzymes needed for digestion and absorption. Children with cystic fibrosis are typically underweight and appear malnourished. Preparations of pancreatic enzymes are often given to cystic fibrosis patients to aid food digestion and absorption, but the body breaks these substances down; only a small amount may actually reach the intestines. However, the breakdown of these preparations can be prevented if the enzyme capsules are coated with a special substance. A recent study of 39 patients examined the effectiveness of Pancrease and Creon, enteric coated capsules of pancreatic enzymes, and compared them by examining the amount of fat absorbed from the diet, the amount of nitrogen excreted, and the patient's weight change during the study. No differences were found between the two preparations and it was concluded that Pancrease and Creon are equally effective. It should be mentioned that the doses required to improve fat absorption to normal or near normal levels were often higher than those recommended by the manufacturers. Future studies including a larger population of cystic fibrosis patients may show an advantage of Creon, but this awaits further study. (Consumer Summary produced by Reliance Medical Information, Inc.)

Product/Service Evaluation, Drug therapy, Digestive enzymes, Pancreatin, Pancreatic insufficiency, Pancrease, Creon

Similar abstracts:

• Abstracts: Instruments for the functional assessment of older patients. "Silent" strokes and dementia
• Abstracts: Food fights: deciding about diet and disease. Basic and clinical aspects of regional fat distribution. Changing dietary patterns
• Abstracts: External cephalic version at term: how high are the stakes? Abdominal decompression: new data from a previous study
• Abstracts: Elevated circulating levels of tumor necrosis factor in severe chronic heart failure. The search for the ideal positive inotropic agent
• Abstracts: Transplacental passage of insulin in pregnant women with insulin-dependent diabetes mellitus: its role in fetal macrosomia

This website is not affiliated with document authors or copyright owners. This page is provided for informational purposes only. Unintentional errors are possible.