Characteristics of children with Kawasaki disease requiring intensive care: 10 years' experience at a tertiary pediatric hospital

Background/Purpose

Kawasaki disease (KD) is a febrile systemic vasculitis, and some patients may develop serious complications requiring intensive care. We aim to ascertain the clinical presentations and outcomes of these patients.

Mucopolysaccharidosis III in Taiwan: Natural history,clinical and molecular characteristics of 28 patients diagnosed during a 21‐year period

Mucopolysaccharidosis type III (MPS III, Sanfilippo syndrome) has a variable age of onset and variable rate of progression. However, information regarding the natural history of this disorder in Asian populations is limited. A retrospective analysis was carried out for 28 patients with MPS III (types IIIA [n = 3], IIIB [n = 23], and IIIC [n = 2]; 15 males and 13 females; median age, 8.2 years; age range, 2.7–26.5 years) seen in six medical centers in Taiwan from January 1996 through October 2017. The median age at confirmed diagnosis was 4.6 years. The most common initial symptom was speech delay (75%), followed by hirsutism (64%) and hyperactivity (54%). Both z scores for height and weight were negatively correlated with age (r = –.693 and ?0.718, respectively; p < .01). The most prevalent clinical manifestations were speech delay (100%) and intellectual disability (100%), followed by hirsutism (93%), hyperactivity (79%), coarse facial features (68%), sleep disorders (61%), and hepatosplenomegaly (61%). Ten patients (36%) had epilepsy, and the median age at the first seizure was 11 years. Thirteen patients (46%) experienced at least one surgical procedure. At the time of the present study, 7 of the 28 patients had passed away at the median age of 13.0 years. Molecular studies showed an allelic heterogeneity without clear genotype and phenotype correlations. MPS IIIB is the most frequent subtype among MPS III in the Taiwanese population. An understanding of the natural history of MPS III may allow early diagnosis and timely management of the disease facilitating better treatment outcomes.     

Association Between Periodontitis Needing Surgical Treatment and Subsequent Diabetes Risk: A Population‐Based Cohort Study

Background: It is well known that patients with diabetes have higher extent and severity of periodontitis, but the backward relationship is little investigated. The relationship between periodontitis needing dental surgery and subsequent type 2 diabetes mellitus (DMT2) in those individuals without diabetes was assessed. Methods: This is a retrospective cohort study using data from the national health insurance system of Taiwan. The periodontitis cohort involved 22,299 patients, excluding those with diabetes already or those diagnosed with diabetes within 1 year from baseline. Each study participant was randomly frequency matched by age, sex, and index year with one individual from the general population without periodontitis. Cox proportional hazards regression analysis was used to estimate the influence of periodontitis on the risk of diabetes. Results: The mean follow‐up period is 5.47 ± 3.54 years. Overall, the subsequent incidence of DMT2 was 1.24‐fold higher in the periodontitis cohort than in the control cohort, with an adjusted hazard ratio of 1.19 (95% confidence interval = 1.10 to 1.29) after controlling for sex, age, and comorbidities. Conclusions: This is the largest nation‐based study examining the risk of diabetes in Asian patients with periodontitis. Those patients with periodontitis needing dental surgery have increased risk of future diabetes within 2 years compared with those participants with periodontitis not requiring dental surgery.     

Presenting Symptoms of Nonerosive and Erosive Esophagitis in Pediatric Patients

Children and adolescents with symptomatic gastroesophageal reflux disease (GERD) and erosive esophagitis (EE) of grade ≥2 (n=45) or nonerosive esophagitis (NEE) (n=45) were assessed to determine the relationship between presenting symptoms, esophagitis severity, and patient age. Overall, regurgitation/vomiting, abdominal pain, and cough were the most frequent symptoms. The prevalence and severity of anorexia/feed refusal was significantly greater in EE versus NEE children; this symptom was also significantly more prevalent in younger (1–5 years) children (both NEE and EE groups) compared to older children. Cough was significantly less severe in NEE adolescents than in younger children. Cough, anorexia/feed refusal, and regurgitation/vomiting were more severe and heartburn was less severe in EE children aged 1–5 years compared with older patients. In conclusion, GERD in children manifests differently than that in adults and symptoms vary with patient age. Symptoms were not predictive of presence or lack of mucosal damage.     

Pathophysiology of Neuropathic Pain in Type 2 Diabetes: Skin denervation and contact heat�Cevoked potentials

OBJECTIVE

Neuropathic pain due to small-fiber sensory neuropathy in type 2 diabetes can be diagnosed by skin biopsy with quantification of intra-epidermal nerve fiber (IENF) density. There is, however, a lack of noninvasive physiological assessment. Contact heat–evoked potential (CHEP) is a newly developed approach to record cerebral responses of Aδ fiber–mediated thermonociceptive stimuli. We investigated the diagnostic role of CHEP.

RESEARCH DESIGN AND METHODS

From 2006 to 2009, there were 32 type 2 diabetic patients (20 males and 12 females, aged 51.63 ± 10.93 years) with skin denervation and neuropathic pain. CHEPs were recorded with heat stimulations at the distal leg, where skin biopsy was performed.

RESULTS

CHEP amplitude was reduced in patients compared with age- and sex-matched control subjects (14.8 ± 15.6 vs. 33.7 ± 10.1 μV, P < 0.001). Abnormal CHEP patterns (reduced amplitude or prolonged latency) were noted in 81.3% of these patients. The CHEP amplitude was the most significant parameter correlated with IENF density (P = 0.003) and pain perception to contact heat stimuli (P = 0.019) on multiple linear regression models. An excitability index was derived by calculating the ratio of the CHEP amplitude over the IENF density. This excitability index was higher in diabetic patients than in control subjects (P = 0.023), indicating enhanced brain activities in neuropathic pain. Among different neuropathic pain symptoms, the subgroup with evoked pain had higher CHEP amplitudes than the subgroup without evoked pain (P = 0.011).

CONCLUSIONS

CHEP offers a noninvasive approach to evaluate the degeneration of thermonociceptive nerves in diabetic neuropathy by providing physiological correlates of skin denervation and neuropathic pain.Type 2 diabetic neuropathy is frequently complicated with neuropathic pain, suggesting the involvement of small-diameter thermonociceptive nerves (1). Two approaches have been developed for the diagnosis of small-fiber sensory neuropathy: psychophysical assessments by measuring thermal thresholds on quantitative sensory testing (2,3) and pathological evaluations by quantifying intra-epidermal nerve fiber (IENF) density in punch skin biopsies (4–6). These examinations provide psychophysical and neuropathological evidence of small-diameter thermonociceptive nerve degeneration and serve as the definition of small-fiber sensory neuropathy (7,8). These two tests are sensitive in detecting the negative symptoms of diabetic neuropathy, for example, the elevation of thermal thresholds (9). However, the neurophysiological correlates of these tests with positive symptoms such as neuropathic pain are limited, and the physiological consequences of thermonociceptive nerve involvements in diabetic neuropathy have rarely been explored.In recent years, several groups, including our own, have established contact heat–evoked potential (CHEP) as a clinically feasible approach to examine the physiology of thermonociceptive nerves (10,11). CHEP, by activating Aδ fibers, can be recorded at the vertex for clinical use (12). Thus, CHEPs provide a noninvasive technique to objectively evaluate the physiology of thermonociceptive nerve dysfunctions. This approach raises the possibility of whether CHEP can be a noninvasive diagnostic tool complementary to IENF density and thermal thresholds and whether CHEP parameters can reflect the neurophysiological correlates of reduced IENF density in diabetic neuropathy. Additionally, since the CHEP amplitude parallels the perceived intensity of heat pain in normal subjects (11,13), a further critical issue is whether CHEP could demonstrate positive symptoms or signs of neuropathic pain in diabetic neuropathy. These include the spontaneous forms, such as a burning sensation, and the evoked forms, such as hyperalgesia to thermal stimuli.To address the above issues, our study investigated the following: 1) the changes in CHEP and its diagnostic role in diabetic neuropathy, 2) the correlations of CHEP with the skin innervation and thermal thresholds, and 3) the relationship of CHEP and neuropathic pain in diabetic neuropathy.