The evaluation and treatment of gastrointestinal disease in children with cystinosis receiving cysteamine

OBJECTIVES: Cysteamine prevents organ damage in children with cystinosis, but may cause gastrointestinal (GI) symptoms. In this study we evaluated the nature of GI disease, and the value of omeprazole in controlling GI symptoms in these children. STUDY DESIGN: Upper GI disease was evaluated with endoscopy, gastrin levels, and acid secretion studies after oral administration of cysteamine, before and after 16 weeks of therapy with omeprazole. A symptom score was devised. RESULTS: Eleven children (mean age, 5.7 years) were studied. After cysteamine ingestion, before and after omeprazole therapy, the mean maximum acid output was significantly higher than the mean basal acid output. The maximum acid output was measured within 60 minutes of cysteamine ingestion and was reduced by omeprazole therapy (P<.01). The mean peak gastrin level was 30 minutes postcysteamine and was higher than baseline (P<.01). The initial mean symptom score (maximum score, 14) was 6.9 and fell to 0.7 (P<.0001) after 16 weeks of omeprazole therapy. At endoscopy, two children had diffuse gastric nodularity, and nearly all had cystine crystal deposits. CONCLUSIONS: GI symptoms in children with cystinosis receiving cysteamine are often acid-mediated and improve with omeprazole. Cystine crystals were detected in the GI tract and may signify inadequate treatment with cysteamine.     

Transformations in processing interaural time differences between the superior olivary complex and inferior colliculus: beyond the Jeffress model

Interaural time differences (ITDs) are used to localize sounds and improve signal detection in noise. Encoding ITDs in neurons depends on specialized mechanisms for comparing inputs from the two ears. Most studies have emphasized how the responses of ITD-sensitive neurons are consistent with the tenets of the Jeffress model. The Jeffress model uses neuronal coincidence detectors that compare inputs from both sides and delay lines so that different neurons achieve coincidence at different ITDs. Although Jeffress-type models are successful at predicting sensitivity to ITDs in humans, in many respects they are a limited representation of the responses seen in neurons. In the superior olivary complex (SOC), ITD-sensitive neurons are distributed across both the medial (MSO) and lateral (LSO) superior olives. Similar response types are found in neurons sensitive to ITDs in two signal types: low-frequency sounds and envelopes of high-frequency sounds. Excitatory-excitatory interactions in the MSO are associated with peak-type responses, and excitatory-inhibitory interactions in the LSO are associated with trough-type responses. There are also neurons with responses intermediate between peak- and trough-type. In the inferior colliculus (IC), the same basic types remain, presumably due to inputs arising from the MSO and LSO. Using recordings from the SOC and IC, we describe how the response types can be described within a continuum that extends to very large values of ITD, and compare the functional organization at the two levels.     

Monaural and binaural processing in the ventral nucleus of the lateral lemniscus: a major source of inhibition to the inferior colliculus

The ventral nucleus of the lateral lemniscus (VNLL) is a major source of input to the inferior colliculus. This paper reviews recent studies of neural responses in the VNLL of the unanesthetized rabbit. The VNLL has generally been viewed as a monaural nucleus, with its neurons responding primarily to stimulation of the contralateral ear. In the rabbit, the VNLL is divided into a medial division (VNLLm) comprising neurons intercalated in the medial limb of the lemniscus, a compact lateral division (VNLLl), and a dorsal division. The VNLLm contains an abundance of neurons sensitive to interaural temporal disparities (ITDs), one of the major binaural cues for sound localization. These neurons respond only at the onset of tones, and therefore appear to encode the ITDs of transients. Even in the VNLLl, many neurons are sensitive to binaural stimulation. The VNLLl contains a variety of neurons with different discharge patterns, the two most common of which are sustained and onset. The discharge patterns, frequency-tuning and dynamic ranges of VNLLl neurons indicate that this division is able to supply the inferior colliculus with a variety of inputs, each serving a different function in the analysis of sound.     

Zinc in CSF of patients with febrile convulsion

OBJECTIVE: This prospective study was carried out from July-December 1999 to see the status of zinc in CSF of children with febrile convulsion and to compare this to that of control. METHODS: Forty-two cases of febrile convulsion and 30 controls (fever without convulsion) were enrolled into the study. CSF zinc was estimated by atomic absorption spectrophotometry (AAS) in Atomic Energy Center, Dhaka and compared between the two groups. RESULTS: The mean zinc level in CSF in the study sample was 40.19mgm/L and that in control was 74.98mgm/L. This difference was statistically significant (p<0.001). CONCLUSION: The study concludes that a significantly lower of zinc exists in CSF of children with febrile. However no relationship was found between CSF zinc status with age, sex, degree & duration of fever and time of lumbar puncture after convulsion.     

Optimization of Schwann cell adhesion in response to shear stress in an in vitro model for peripheral nerve tissue engineering

The design of nerve guidance channels (NGCs) is evolving to produce a favorable environment for neural regeneration. We created an in vitro model to evaluate the interactions between three centrally important components of this altered host environment: (1). Schwann cells, (2). substrate, and (3). sustained mechanical stimulus in the form of shear stress with laminar fluid flow. Preconfluent Schwann cells were plated on slides coated either with laminin, poly-D-lysine, type IV collagen, or fibronectin. These slides were placed into custom-designed, parallel-plate, flow chambers and were administered laminar fluid flow at a rate of 15 mL/min for 2 h. Schwann cell adhesion assays demonstrated that laminin (mean, 86.1%; SEM, 4.47%) and fibronectin (mean, 81.7%; SEM, 3.24%) were statistically superior to collagen type IV (mean, 57.7%; SEM, 3.96%) and poly-D-lysine (mean, 58.0%; SEM, 4.97%) (p < 0.001). Fibronectin (mean, 12.20%; SEM, 0.374%) induced statistically greater Schwann cell proliferation than did laminin (mean, 8.14%; SEM, 0.682%) (p < 0.001). Therefore, we recommend that fibronectin should be used as an important component of NGCs with further in vivo studies. As mechanical stress is an integral part of the host environment, our study is the first to incorporate this factor into an in vitro model for peripheral nerve tissue engineering.     

Lamin expression in human adipose cells in relation to anatomical site and differentiation state

Familial partial lipodystrophy-Dunnigan variety (FPLD) is an autosomal dominant form of lipodystrophy resulting in a loss of sc fat from the trunk and limbs with retention of fat in the visceral depots, face, and neck. Specific point mutations in the gene encoding the nuclear lamina proteins, lamins A and C, have been established to cause this syndrome. We undertook studies to determine which members of the lamin family were expressed in human fat cells, to examine the effect of differentiation state on lamin A and C expression in human preadipocytes, and to test the hypothesis that regional variation in lamin A/C expression might underlie the stereotyped anatomical pattern of FPLD. Lamins A, C, and B1, but not B2, were expressed in sc mature human adipocytes. Subcutaneous preadipocytes expressed all four lamins, with lamin A and C expression increasing with ex vivo differentiation. Consistent with previously reported resistance to ex vivo differentiation, omental preadipocytes did not show an increase in lamin A or C mRNA under these conditions. Lamin A/C mRNA levels were similar in isolated mature adipocytes and preadipocytes from omental, sc, and neck sites. However, lamin C was consistently lower, and the ratio of lamin A/C mRNA was higher in sc mature adipocytes compared with omental mature adipocytes. We conclude that the depot-specific pattern of lamin A/C expression does not provide clues to the mechanism of FPLD. Nonetheless, these studies provide new information regarding the expression of lamin isoforms in normal human adipose cells, which will inform future studies of the molecular pathogenesis of FPLD.     

Late spontaneous extrusion of a wooden intraorbital foreign body

Wooden intraorbital foreign body is characteristic for delayed manifestation, silent progression and unpredictable outcome. A silent wooden intraorbital foreign body is difficult to diagnose clinically. Spontaneous expulsion of entire foreign body is rare.