Improved screening for cystic fibrosis-related diabetes by an integrated care team using an algorithm.

OBJECTIVE: To determine whether implementation of a standardized, clinic-based algorithm improves compliance with cystic fibrosis-related diabetes (CFRD) screening guidelines. STUDY DESIGN: A CFRD screening algorithm was developed as part of a quality improvement initiative through collaboration between the pediatric pulmonary and endocrine divisions and implemented prospectively to children aged 8-17 years in our CF center for a 6-month period. The primary outcome measure was the percentage rate of CF patients who were appropriately screened with an oral glucose tolerance test (OGTT) during the quality improvement period as compared to the year prior. RESULTS: Ninety-seven percent (37/38) of OGTTs were appropriately ordered by providers, and 89% (34/38) of patients obtained the OGTT at the completion of the quality improvement period. Compared with the percentage of eligible patients completing the OGTT the year prior, the use of the algorithm significantly improved screening (P = 0.03). Data collected 1-year post-algorithm implementation revealed 97% (33/34) of OGTTs were ordered and 79% (27/34) of OGTTs were completed. The use of the algorithm 1-year post-implementation did not reveal a significant improvement in screening when compared to the reference year and implementation period (P = 0.08). CONCLUSIONS: Implementation of a clinical algorithm resulted in a statistically significant improvement in screening during the quality improvement period, but this improvement was not sustained the following year despite continued physician compliance with ordering the OGTT. Barriers to patient compliance need to be explored.

XLA-associated neutropenia treatment: a case report and review of the literature.

X-linked agammaglobulinemia (XLA) is a primary B-cell deficiency syndrome with an incidence of 5 to 10 cases per million. The current treatment approach includes intravenous immunoglobulin and aggressive antibiotic regimens for infections. Besides recurrent infections, XLA patients may present with other manifestations, such as alopecia, enteropathy, amyloidosis, and neutropenia. Neutropenia, which has been shown in up to 25% of affected patients, might also contribute to the degree of severity of bacterial infections that have been reported in these cases. Here we present our experience with the granulocyte colony-stimulant factor, filgrastim (Neupogen), in the treatment of neutropenia in a 14-month-old child with XLA.

Neuralgic amyotrophy associated with antibiotic therapy.

OBJECTIVE: To report a case of neuralgic amyotrophy associated with antibiotic therapy. CASE SUMMARY: A 22-year-old male with cystic fibrosis had been nonadherent to treatment for 4 years; when he returned to the clinic with symptoms, his forced expiratory volume in 1 second dropped from 84% predicted to 43% predicted. He was admitted to the hospital for treatment after failing to improve on oral ciprofloxacin and inhaled tobramycin. Treatment was initiated with intravenous tobramycin 560 mg daily and piperacillin/tazobactam 4.5 g infused every 6 hours. He continued inhaled tobramycin 300 mg twice daily, his home doses of pancreatic replacement enzymes and vitamins, albuterol 2.5 mg by high flow nebulizer (HFN) 4 times daily, and dornase alpha 2.5 by HFN daily. Sputum cultures were positive for methicillin-resistant Staphylococcus aureus, and intravenous vancomycin 1 g every 8 hours was added to the treatment regimen on hospital day 7. The patient developed bilateral shoulder pain followed by decreased function of his upper extremities 2 days later. He was treated with oral ibuprofen 600 mg every 6 h and oral cyclobenzaprine 5 mg daily, which improved his pain, but the shoulder stiffness remained throughout his hospital stay and persisted for 2 months following discharge. These symptoms resolved but recurred rapidly (within 24 h) and were more debilitating following a second exposure to the same antibiotics at the same doses 8 months later when the patient was readmitted for treatment of another cystic fibrosis-related pulmonary exacerbation. DISCUSSION: To our knowledge, this is the first case report illustrating neuralgic amyotrophy triggered by exposure to the antibiotics vancomycin, tobramycin, and piperacillin/tazobactam. After analysis of the case, ruling out other possibilities and using the Naranjo probability scale, we found that there is a highly probable likelihood that the symptoms presented by our patient were secondary to his drug therapy. Neuralgic amyotrophy is a rare condition of unknown etiology that has never before been associated with administration of these antibiotics, individually or in combination. Because of the specifics of the clinical history, we were unable to ascertain whether this complication was due to a single antibiotic or to the combination. It is quite possible that vancomycin was the only culprit, but impossible to ensure with the available evidence. CONCLUSIONS: Clinicians should be aware of this adverse reaction when facing similar complex neurologic symptoms in patients who are receiving the antibiotic treatment described here, especially vancomycin.