Rapid-onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation in Saudi Arabia.

Rapid-onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) is a rare disease, but could be fatal if not diagnosed early. It mimics many other diseases and it may take few years after the onset of rapid obesity to have the other clinical features. Therefore, any patient with rapid-onset obesity after the age of 2 years should have high index of suspicion and long term follow up. We report a case of ROHHAD in Saudi Arabia and we highlight the clinical features and the importance of early diagnosis and management.

Defining common outcome metrics used in obstructive sleep apnea.

Sleep-disordered breathing a spectrum that ranges from snoring through disorder of increased airway resistance, to overt sleep apnea affects many clinical disease outcomes. Traditionally, disease outcomes have been measured by polysomnography, with the most common metric being the apnea hypopnea index (AHI). Multiple other clinical metrics are commonly used to assess the severity and impact of disease on important outcomes of obstructive sleep apnea (OSA). These allow assessment of sleepiness, quality of life, performance, and medical, especially cardiovascular outcomes. Currently the available metrics only partially explain the associated disease outcomes in different patients. This review highlights the available clinical, physiological and biomarker metrics in measuring OSA and associated co-morbidities and defines treatment goals.

Asthma and insulin resistance in morbidly obese children and adolescents.

BACKGROUND: Obesity is well recognized as a state of increased insulin resistance and has been implicated as a significant risk factor for both asthma prevalence and asthma severity in children and adolescents. However, little is known about the specific factors that relate asthma and obesity. Recently, the pro-inflammatory state in obesity and its association with insulin resistance have been recognized. We hypothesize that the effect of morbid obesity on asthma is related to insulin resistance. METHODS: The patient cohort in the obesity management program at the Children's Hospital of Wisconsin was retrospectively reviewed. Variables were collected from the program data base and chart review was done for missing variables. Patients were considered to have asthma if the evaluating physician confirmed the diagnosis through history and/or the patient had been on inhaled corticosteroids. Insulin resistance (IR) was calculated using a homeostasis model assessment (HOMA). Multivariate logistic regression was performed to identify variables that were significantly related to the odds of having asthma. RESULTS: Of the 415 patients included in the study, 146 (35%) were asthmatic and 269 (65%) were non-asthmatic. The asthma (AG) and non-asthma (NAG) groups were similar with respect to mean age (11.3 vs. 11.5 years), gender (45% vs. 43% males), mean body mass index (BMI) (36.4 vs. 34.9), and exposure to smoking (43% vs. 42%). Fhx of asthma was significantly higher in AG (71%) compared to NAG (40%). IR level+/-SD was 8.5+/-9.7 in AG compared to 5.3+/-6.7 in NAG (p<0.0001). Multivariate regression analysis found the following variables to be associated with having asthma: younger age (p<0.05), smoking exposure (p<0.05), positive Fhx of asthma (p<0.0001, odds ratio of 3.1), and IR (p<0.0001, odds ratio of 4.1). CONCLUSION: Morbidly obese asthma patients have a higher degree of insulin resistance compared to morbidly obese non-asthma patients. We speculate that the pro-inflammatory state of insulin resistance may contribute to the pathogenesis of asthma in obese patients. Future prospective studies should address insulin resistance as a possible risk factor for asthma in obese children and adolescents.

Cystic fibrosis and renal disease: a case report.

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disease that is predominantly seen in the Caucasian population and involves multiple organs. Traditionally it has been thought that the kidney is the only organ which does not seem to be generally affected by the disease although the cystic fibrosis transmembrane conductance regulator (CFTR) gene is expressed in the kidney. CASE PRESENTATION: We report the case of an 11 year old boy with cystic fibrosis and nephrotic syndrome and review the literature that describes nephrotic syndrome and renal involvement in cystic fibrosis. CONCLUSION: With continued advances in the management of cystic fibrosis and improvement in life expectancy, several unrecognized co-morbidities are expected to emerge. It is important to screen patients for possible co-morbidities. Urine analysis may be helpful in this group of patients and any proteinuria should raise the suspicion of cystic fibrosis-related renal disease.

Trimethoprim-sulfamethoxazole plus topical antibiotics as therapy for acute otitis media with otorrhea caused by community-acquired methicillin-resistant Staphylococcus aureus in children.

OBJECTIVE: To report our experience in identification and treatment of acute otitis media (AOM) with otorrhea secondary to community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA), which is seen in children at increasing rates. DESIGN: Clinical and laboratory records were retrospectively reviewed between January 2003 and December 2003. SETTING: Primary pediatric clinic. PATIENTS: Six pediatric patients who had AOM with otorrhea caused by CA-MRSA. MAIN OUTCOME MEASURES: Clinical resolution of AOM with otorrhea. RESULTS: All patients had acute-onset otorrhea associated with their AOM. Five patients had tympanostomy tubes and 1 had perforation of the tympanic membrane. None of the patients were responding to treatment with oral antibiotics (amoxicillin sodium-clavulanate potassium, cefpodoxime proxetil, and cefprozil) or fluoroquinolone ear drops (ofloxacin, ciprofloxacin). Specimens were obtained from the ears for cultures, and MRSA was present in the cultures. The organisms were resistant to levofloxacin and erythromycin in all patients and resistant to clindamycin hydrochloride in 2 patients. The cultures were sensitive to trimethoprim-sulfamethoxazole, gentamicin sulfate, rifampin, and vancomycin hydrochloride. All patients were treated successfully with oral trimethoprim-sulfamethoxazole and ear drops (gentamicin sulfate or polymyxin B sulfate-neomycin sulfate-hydrocortisone [Cortisporin]). CONCLUSIONS: The rising rate of CA-MRSA as a cause for many pediatric infections is a major concern. It is very important to obtain cultures from patients with nonresponsive or persistent otorrhea with AOM to look for MRSA and determine the sensitivity of the pathogen to antibacterial therapy. Trimethoprim-sulfamethoxazole is a good choice for initial, empirical therapy when combined with a topical agent for AOM with otorrhea if CA-MRSA is suspected. Further studies are needed to determine whether there is a link between the overuse of topical fluoroquinolones in pediatric patients and the recent rising rate of CA-MRSA.