Laryngotracheoesophageal Cleft Type 3 and Severe Laryngotracheomalacia; Delayed Surgical Repair, a Treatment Challenge with an Excellent Outcome.

Laryngotracheoesophageal clefts (LTEC) are rare malformations which involve the upper respiratory and digestive tract. Surgical repair should be undertaken promptly to maintain a secure airway and prevent serious pulmonary aspiration. This paper reports the first case of LTEC type 3 with severe laryngotracheomalacia that was brought to Mofid children's hospital in late infancy with a poor health status. Delayed defect correction was our team strategy for the patient when she had achieved good weight gain. At the age of 22 months in collaboration with the pediatric surgical and otolaryngologist team, the repair of the laryngeal cleft was done with lateral open approach method. She was discharged with tracheostomy and gastrostomy. In the next six months follow up after the surgery tracheostomy decannulation and gastrostomy tube removal were done and the infant is now in regular follow-up.

Prevalence of Extended-Spectrum and Metallo ß-Lactamase Production in AmpC ß-Lactamase Producing Pseudomonas aeruginosa Isolates From Burns.

BACKGROUND: Pseudomonas aeruginosa is one of the most common causes of nosocomial infections. Resistance of P. aeruginosa to ß-lactam antibiotics may be the result of acquired resistance through mutation and over production of various antibiotic inactivating enzymes. This research aimed to determine the prevalence of extended-spectrum ß-lactamases (ESBL) and metallo ß-lactamase (MBL) production as well as the presence of their related genes among AmpC ß-lactamase producing P. aeruginosa isolated from burns. OBJECTIVES: The current study aimed to determine the prevalence of class A ESBL and MBL production in relation to the presence of their related genes among AmpC ß-lactamase producing P. aeruginosa isolated from burns. MATERIALS AND METHODS: The antimicrobial susceptibility of 51 P. aeruginosa isolates from patients with burns was examined against 13 antibiotics by the disc diffusion method. Minimum inhibitory concentrations (MIC) for imipenem and ceftazidime were measured by the microdilution method. AmpC production was detected by AmpC disc and the modified three-dimensional extract tests. ESBL phenotype was confirmed by the double disc synergy test (DDST). Presence of ß-lactamase genes was detected by specific primers and polymerase chain reaction (PCR). RESULTS: All isolates were multidrug resistant. AmpC, ESBL and MBL production were observed in 35 (68.6%), 20 (39.2%) and 19 (37.3%) isolates, respectively. Overall, 43 isolates (84.3%) carried ß-lactamase genes, out of which 31 (60.8%) harbored bla AmpC , 20 (39.2%) had bla TEM and 11 (21.6%) carried bla PER -1 genes. Among the AmpC producers, two isolates (6.5%) carried bla AmpC + bla ESBL , 13 (41.9%) had bla AmpC + bla MBL and six (19.4%) produced the three enzymes. CONCLUSIONS: A high prevalence of multiple ß-lactamase production was observed among the AmpC producers (60%), of which the majority co-produced AmpC and MBL. The current study results showed correlation between ß-lactamase production and the presence of antibiotic resistance genes in the isolates.

Pulmonary alveolar proteinosis in children: a case series.

Pulmonary alveolar proteinosis, (PAP) is a rare disease of unknown etiology, characterized by accumulation of intraalveolar proteinaceous material which is rich in lipid and positive on periodic acid-Schiff stain. Two clinically different pediatric types have been defined as congenital PAP which is fulminant and fatal, and a late-onset PAP which is similar to the adult form and less severe. Eight children with late-onset PAP were hospitalized from 1998 to 2005 in Mofid Children Hospital. Characteristics of these patients and the methods of diagnosis and treatment are presented in this case series.