Body mass index as an indicator of obstructive sleep apnea in pediatric Down syndrome.

OBJECTIVE: Our objective was to determine if higher body mass index (BMI) increases the likelihood of, obstructive sleep apnea (OSA) in pediatric Down syndrome (DS) patients. METHODS: We performed a, retrospective chart review of 63 DS patients evaluated by overnight polysomnography from December 1995 to February 2005. Patients aged less than 2 years were excluded. Remaining patients were grouped, according to presence (n=19) or absence (n=33) of OSA based on apnea hypopnea index (AHI). OSA, and non-OSA DS groups were age matched while blinded to patient attributes other than age and OSA, status. Patients without appropriate age matches were excluded. We recorded various patient information, including age, sex, height, weight, number of apneas, number of hypopneas, respiratory distress index (RDI), apnea-hypopnea index (AHI), lowest oxygen saturation during sleep, mean oxygen saturation, number of arousals per hour, and mean time spent in REM sleep. We calculated BMI using the, standard kg/m(2) formula and converted this into a Z-score. RESULTS: Fifty-two DS patients were analyzed with average age of 9.3+/-4.5 years (10.2+/-4.2 in 33 OSA patients, 7.8+/-4.3 in 19 non-OSA patients). There were 28 males and 24 females. The OSA group mean BMI Z-score was 2.09+/-0.94, and the non-OSA group Z-score was 1.4+/-1.40. The Z-scores for BMI were statistically significant between OSA and non-OSA patients with p=0.03 by t-test. CONCLUSIONS: When age and sex adjusted, BMI has a statistically significant association with the presence of OSA in Down syndrome patients. The incidence of OSA also increases with increasing age in this population.

Cleft palate lateral synechia syndrome: an opportunity for unique surgical closure.

OBJECTIVES: To report two cases of cleft palate lateral synechia (CPLS) syndrome in a single family and describe surgical closure using the synechia. STUDY DESIGN: Case report and literature review. METHODS: A case report is presented with a review of the literature of cleft palate in conjunction with lateral synechia. Clinical presentation with photographic images of surgical repair is presented as well as a genetic workup with pedigree. RESULTS: A 6-week-old male presented for evaluation of a cleft palate. Mucosa-lined, fibromuscular tissue bands were noted connecting the floor of mouth and the free edges of a bilateral complete secondary cleft palate. No other craniofacial, digital, genital or limb defects were noted. The patient's 13-month-old sister had similar synechial bands and cleft palate at birth. Another older sibling had cleft palate without synechia. The patient's mother and maternal great grandmother had cleft palates at birth without synechia. The three children share a common mother but have three different fathers. Genetic analysis failed to reveal chromosomal defects or a mutation in the interferon regulatory factor 6 (IRF6) gene, a locus linked to Van der Woude syndrome. At 2 years of age, the index patient was growing and feeding well. His intra-oral bands remained intact and were incorporated in the surgical repair using a novel approach. CONCLUSIONS: Since more otolaryngologists are performing cleft surgeries, the awareness of the differential diagnoses associated with a cleft palate is important. CPLS is an extremely rare condition. The report of this family supports the suspected pattern of autosomal dominant inheritance with variable expressivity. The unusual surgical approach will be discussed.

Airway evaluation and management in 7 children with malignant infantile osteopetrosis before hematopoietic stem cell transplantation.

Malignant infantile osteopetrosis (MIOP) is a rare disorder caused by dysfunctional osteoclasts. The classic MIOP features, such as frontal bossing, micrognathia, and small thorax, may place these children at risk for developing obstructive sleep apnea (OSA) and chronic hypoxemia. To objectively document OSA, airway evaluations were performed; results impacted management. We reviewed the records of 7 MIOP patients treated at St Jude. Six underwent polysomnograms during prehematopoietic stem cell transplantation (HSCT) evaluation. To determine the existence of a relationship between OSA and radiologic imaging, initial chest radiographs and bone mineral density studies were reviewed. Pre-HSCT patients had a median apnea-hypopnea index of 17.51 (normal, 0 to 2), with <25% being central events, thus indicating OSA. The median minimal oxygen saturation was 79%, indicating intermittent hypoxemia. Neither chest radiographs nor bone mineral density correlated with severity of OSA. Four patients received tracheostomies before or during HSCT. Three surviving children underwent polysomnograms 1 year after HSCT, and median apnea-hypopnea index was 1.3, indicating near to complete resolution of OSA. Resolution of OSA may have been multifactorial. Using a quantitative approach, we demonstrate that MIOP children have OSA and hypoxemia; thus, these children should have airway evaluations and treatments to potentially reduce the risk of life-threatening pulmonary complications.

Aural involvement in loxoscelism: case report and literature review.

An 11-year-old male presented with fever, rash, and a necrotic lesion on the lobule of the left ear. The lesion became tender and formed an eschar over 4 days. The patient developed leukocytosis, hemolytic anemia, and proteinuria, and was diagnosed with systemic loxoscelism from a brown recluse spider bite. He was managed with supportive therapy and improved in 4 days. Loxoscelism is a clinical diagnosis which should be suspected in an otherwise healthy patient with a necrotic wound, particulary in the endemic Southern and Midwestern United States. Physicians should be aware of this disease entity and its complications.

Methicillin-resistant Staphylococcus aureus as a pathogen in deep neck abscesses: a pediatric case series.

OBJECTIVES: Pediatric deep neck space abscesses are frequently treated by the otolaryngologist. We report four children with deep neck abscesses caused by methicillin-resistant Staphylococcus aureus (MRSA), including the first described case of descending mediastinitis caused by MRSA in a child. METHODS: Records from March 2001 to April 2002 were reviewed. RESULTS: Four patients presented with neck swelling, and three of these also had leukocytosis. All abscess cultures were positive for MRSA. Abscess drainage with antibiotic therapy successfully treated three cases without complication. The fourth case developed descending mediastinitis but survived after additional surgical treatment and prolonged antibiotic therapy. CONCLUSIONS: MRSA should be considered as a potential pathogen in deep neck space abscesses. A high index of suspicion is needed as well as aggressive treatment including incision and drainage along with culture-directed medical therapy. Surgical drainage may be the most important aspect of therapy.

Genistein induces G2 arrest in malignant B cells by decreasing IL-10 secretion.

Chronic B cell malignancies are often chemoresistant and the development of new therapeutic modalities is a high priority. Many B cell malignancies have autocrine production of IL-10, which regulates B cell growth and differentiation. Here we demonstrate that the soy isoflavone genistein, a tyrosine kinase inhibitor, rapidly decreased IL-10 secretion followed by upregulation of IFNgamma and inhibition of cell proliferation with pre-dominantly G2 arrest. The antiproliferative effects of genistein were reversed by the addition of exogenous IL-10. Genistein downregulated cdc25C and cdk1 as well as anti-apoptotic proteins survivin and Ian-5. After genistein withdrawal, the G2M arrested cells reentered the cell cycle and underwent apoptosis, which was significantly augmented by fludarabine. We conclude that genistein can sensitize malignant B cells to the action of other chemotherapeutic agents by modulating the cytokine profile and controlling cell cycle progression.