Cystic Fibrosis Goals of this session:
To review the pathophysiology of Cystic Fibrosis To review the relevant physical findings of CF To place the pathophysiology of CF into a clinical context through a case presentation
Pathophysiology review:
Mutation in CFTR transmembrane receptor (on chromosome 7)
ATP Binding Cassette protein that acts as regulated chloride channel
Phenotypic expression of disease varies widely, dependent on type of mutation
Physical and chemical abnormalities in bodily secretions, especially mucus
Respiratory secretions cause chronic infections by P. aeruginosa (now also B. cepacia)
persistent, productive cough, hyperinflation of the lungs on CXR, and PFTs consistent
As the disease progresses, patients develop chronic bronchitis +/- bronchiectasis
Pancreatic insufficiency +/- Failure to Thrive
panopacification of the paranasal sinuses +/- Nasal Polyps
Meconium Ileus +/- Small Bowel Obstruction
Focal Biliary Cirrhosis +/- Cholelithiasis
Sperm transport defects OR Secondary amenorrhea
CC: cough, dyspnea, and poor weight gain HPI: 15 ½ year old girl with CF, dx’d in infancy, p/w pulmonary exacerbation. Briefly, she was born with meconium ileus, which led to the diagnosis of CF. She had a colostomy as an infant with closure during the first year of life. She was then relatively stable for a number of years with minimal symptoms. Over the past year, she has had increasing respiratory sxs, with increased frequency of hospitalizations and poor weight gain. In 4/04, she was admitted for a gastrostomy tube and IV antibiotics. She was again admitted in 10/04. In general, she improves on IV antibiotics but quickly deteriorates subsequently to ceasing the medications. During her most recent admission in 11/04 for a pulmonary exacerbation she was treated with IV Tobramycin and Ceftazidime for Pseudomonas positive sputum cxs and she was placed on an increased calorie diet with minimal subsequent weight gain. Since discharge, she has continued to have a daily cough and dyspnea. She has missed over half of her school year. Her sputum is green, though she has transient hemoptysis. She has intermittent sinusitis. PMH: as above Medications: Pancrease, Xopenex, Hypertonic Saline, Pulmozyme, Zyrtec, MVI, Peptamin through Gtube, Calcium, Prednisone, Zithromax, Prevacid, Flonase, Flovent, Singulair, Mephytoin, Albuterol ROS: hx sinusitis; poor PFTs in past; malnutrition related to CF; occasional elev. Gluc.; nonmenstruating; PE: Ht 154 cm; Wt 33kg; Tafebrile; BP not recorded; P 125; RR20; O2sat 92% RA Gen: very thin girl, alert, NAD HEENT: nasal turbinates boggy CV: regular rate and rhythm, S1, S2, no murmurs noted Lungs: nl AP diameter, no incr’d resp effort, fair air entry b/l; decreased air entry at LLL; scattered crackles b/l Abd: soft, nontender, nondistended, no hepatosplenomegaly Ext:+clubbing bilaterally Sputum Cx: Pseudomonas sensitive to Zosyn, Gentamycin, Amikacin, & Imipinem
HUMAN RIGHTS COMMITTEE Hesse v. Australia Communication No. 1087/2002 15 July 2002 CCPR/C/75/1087/2002 ADMISSIBILITY Date of registered communication: 26 February, 6 August 2001, and 10 May 2002 The Human Rights Committee, established under article 28 of the International Covenant on Civiland Political Rights, Decision on inadmissibility 1. The author of the communication
SAN FELIPE, 28 de OCTUBRE DEL 2010 .- DICTA ORDENANZA COMUNAL SOBRE DERECHOS MUNICIPALES, PERMISOS, CONCESIONES Y SERVICIOS VISTOS: 1. El Decreto Ley Nº 3.063 de 1979, sobre Rentas Municipales; y las modificaciones introducidas por la Ley 20033. 2. La Ordenanza Nº 5 de fecha 10 de Junio de 1980 y sus modificaciones posteriores sobre derechos municipales, por conces