Infant hearing screening with an automated auditory brainstem response screener and the auditory brainstem response

From 1653 babies hospitalized in the Veterans General Hospital—Taipei from 1993 to 1995, 260 infants at risk of hearing impairment were selected. The risk criteria of hearing impairment for neonates were based on the recommendation of the US Joint Committee on Infant Hearing, 1990 Position Statement. All these infants were screened with the Algo-1 Plus, an automated auditory brainstem response (ABR) screener at a mean postconceptional age of 40.7 ± 4.5 weeks. Thirty-nine cases (39/260, 15%) involving 57 ears (57/520, 11%), failed the screening. Except for one infant who died, the babies had an ABR test for both air- and bone-conducted stimuli and an otological examination. The case-specific incidence of conductive hearing deficit at the initial ABR test was 5.4%. The prevalence of sensorineural hearing deficits was between 2.3% confirmed and 3.1% including infants who did not have follow-up tests. The n-value that indicated agreement between the Algo-1 and ABR results was 0.64, and the overall efficiency of using Algo-1 to correctly identify pass or failure of the ABR was 83%.     

Spinal drop metastasis of choroid plexus papilloma.

A rare case of choroid plexus papilloma (CPP) with spinal drop metastasis is presented. A 51-year-old woman was operated on for a tumor of the fourth ventricle with histopathological diagnosis of CPP. Seven years later, she was re-admitted with low back pain. MRI showed multilobulated tumor at the lumbosacral subarachnoidal space with histopathological diagnosis of CPP. Thus, CPP can spread via the cerebrospinal fluid pathways and cause drop metastasis.     

The evaluation of serum leptin level and other hormonal parameters in children with severe malnutrition

OBJECTIVES: Protein-energy malnutrition (PEM) is a clinical problem caused by inadequate intake of one or more nutritional elements, and remains as one of the most important health problems in developing countries. The aim of this study is to determine the relationship among leptin concentrations, body weight and concentrations of some serum hormones, e.g., basal GH, IGF-1, basal cortisol and IGF-BP3, in severe malnourished children, and to determine the effects of leptin in malnourished children. DESIGN AND METHODS: The study group consisted of 36 children diagnosed with PEM. Thirty healthy children were enrolled as the control group. After an overnight fast and before initiation of feedings, fasting venous blood samples were obtained from a forearm vein with needle technique for routine tests, and leptin, IGF-1, IGF-BP3, basal GH and cortisol levels were measured. Tests were carried out in the laboratories of the Department of Biochemistry by commercial kits. RESULTS: Serum leptin levels of infants with marasmus and kwashiorkor were significantly lower than that of the controls (2.09 +/- 0.93 and 2.27 +/- 1.01, 6.82 +/- 2.28 ng/ml, respectively, P < 0.001). However, there was no significant difference between serum leptin levels in children with marasmus and those with kwashiorkor (P > 0.05). Serum IGF-1 and IGF-BP3 levels were significantly lower in malnourished children (P < 0.001, both). Also, basal GH and cortisol levels were significantly higher in malnourished children (P < 0.001, both). There was a positive correlation among serum leptin levels and IGF-1 and IGF-BP3 levels and also a negative correlation among serum leptin levels and basal GH and cortisol levels in children diagnosed with marasmus or kwashiorkor and the control group. CONCLUSIONS: The decrease of energy intake and adipose tissue and serum IGF-1 levels in children with PEM may result in decrease of leptin secretion. Decrease in serum leptin levels may initiate food intake by increasing appetite and stimulating the secretion of cortisol and GH that might increase energy expenditure through an autocrine mechanism. Moreover, serum leptin level may be an important signal to reflect the metabolism of children with PEM.     

Thyroglossal Duct Cyst Associated with Xanthogranulomatous Inflammation

Xanthogranulomatous inflammation (XGI) is an uncommon destructive chronic inflammatory process mainly occurring in the kidney and gallbladder, characterized by the accumulation of foamy histiocytes, multinucleated giant cells (Touton type), cholesterol clefts and chronic inflammatory cells. The head and neck region is an uncommon site for XGI. This type of inflammatory reaction has been defined in branchial cleft cyst, salivary gland tumors following fine-needle aspiration biopsies, Rathke’s cleft cyst in the pituitary gland, and colloid cyst in the 3rd ventricle. We present herein a unique case of ruptured thyroglossal duct cyst leading to XGI, characterized by an infiltrative subcutaneous central neck lesion, clinically mimicking a thyroid carcinoma. In addition, we also summarize current insights into the pathogenesis of XGI in the head and neck region.     

Rickets with Dorfman-Chanarin syndrome

BACKGROUND: Dorfman-Chanarin syndrome is a rare, autosomal recessive, inherited, lipid storage disease. It is characterized by nonbullous congenital ichthyosiform erythroderma, leukocyte vacuoles and variable involvement of the liver, muscles and central nervous system, due to errors of triacylglycerol metabolism. To date only 32 cases of this syndrome have been described worldwide. AIMS: To report the case of a boy with Dorfman-Chanarin syndrome with rickets. CASE REPORT: A boy of Turkish origin was born of a nonconsanguineous marriage after an uncomplicated perinatal period. On examination the patient had failure to thrive, diffuse erythroderma and ichthyosis and clinical features suggesting rickets. A light-microscopic examination of peripheral smear revealed vacuolated leukocytes typical of Jordans' anomaly. Lipid analysis showed an increase in triacylglycerol and very low density lipoprotein. A radiographic study of the wrist revealed rickets. CONCLUSION: The clinical progression in these patients ranges from mild to fatal. We believe that patients with ichthyosis should be evaluated bearing in mind the possibility of Dorfman-Chanarin syndrome. This may increase the number of reported Dorfman-Chanarin syndrome cases, and the pathogenesis and progression of the disease will become clearer.     

Neuromuscular effects of cisatracurium in morbidly obese patients

Obesity is associated with significant changes in body composition and function that may alter the pharmacodynamics and pharmacokinetics of various drugs. In this study, we investigated the neuromuscular effects of cisatracurium in morbidly obese as compared to control group of normal body weight patients. In the morbidly obese group (n = 20), corrected weight was used to calculate the drug doses. In the control group (n = 20), the dose was calculated on ideal body weight (IBW). 0.15 mg/kg(-1) cisatracurium was administered as the neuromuscular blocker. Neuromuscular effects were recorded at T0 (onset time), T1 (appearance of first stimulus of TOF), T25 (25% recovery of T1) and T25-75 (time of T25 to T75, recovery time). T0 was determined as 177 +/- 23 s and 168 +/- 19 s in the morbidly obese, and control group, respectively. T25 was determined as 46 +/- 7 min and 56 +/- 8 min, in the morbidly obese and control group, respectively (p < 0.05). T25-75 was determined as 11 +/- 5 min and 14 +/- 6 min in the morbidly obese and control group, respectively (p < 0.05). Intubation conditions were determined as good in 13, excellent in 7 patients in the morbidly obese group, and as good in 4 and excellent in 16 patients in the control group (p < 0.05). As different neuromuscular effects of cisatracurium were detected, we conclude that ne uromuscular agents must be monitored in the morbidly obese patients.