Nontyphoidal Salmonella Meningitis in an Immunocompetent Child

Salmonella meningitis is rare yet poses causes significant neurological morbidity in children. Infants, especially those under 3 months of age, and those with immunocompromised states, such as malignancy, malaria, and human immunodeficiency virus infection, are at increased risk for developing Salmonella meningitis. Herein, we describe a case of Salmonella meningitis in a previous healthy 8-year-old girl who presented with high fever, vomiting, and altered mental status. Group D Salmonella species were isolated in cerebrospinal fluid culture, and no abnormal findings were noted in brain magnetic resonance imaging. Immunoglobulin levels and lymphocyte subset counts were within the normal ranges, and no genetic mutation responsible for primary immunodeficiency disease was detected by next-generation sequencing. The patient’s condition improved rapidly with third-generation cephalosporin, and no complications or sequalae developed. Nontyphoidal Salmonella can cause meningitis in immunocompetent children and can be successfully treated with early administration of antibiotics.

Niemann-Pick Disease Type C Diagnosed Using Neonatal Cholestasis Gene Panel / 대한소화기학회지

Niemann-Pick disease type C (NPC) is a neurovisceral lysosomal storage disorder caused by mutations in the NPC1 and NPC2 genes. These mutations cause the accumulation of unesterified cholesterol and other lipids in the lysosomes. NPC has a broad spectrum of clinical manifestations, depending on the age of onset. A 15-day-old infant presented at the Seoul National University Children's Hospital with neonatal cholestasis and hepatosplenomegaly, with the onset of jaundice at 5 days of age. Despite supportive treatment, the patient was considered for a liver transplant because of progressive liver failure. Unfortunately, the patient died from gastrointestinal bleeding before undergoing the transplant. The neonatal cholestasis gene panel revealed two novel likely pathogenic variants in the NPC1 gene (c.1145C>G [p.Ser382*] and c.2231_2233del [p.Val744del]). The patient was diagnosed with NPC, and both parents were found to be carriers of each variant. In infants presenting with neonatal cholestasis, a gene panel can help diagnose NPC.

Quantitative Analysis of Bulbocavernosus Reflex Activity in Patients with Spinal Cord Injury

OBJECTIVE: To quantify activities of bulbocavernosus reflex (BCR) using root mean square (RMS) value of motor unit action potentials in power spectrum analysis. METHOD: Twenty-six male patients with spinal cord injury were studied. The BCRs were evaluated by one examiner. The patients were assigned into absent, hypoactive and normal reflex groups according to the degree of BCR activity. The electrophysiological activities of the BCRs were evaluated by the RMS value of the motor unit action potentials in power spectrum analysis recorded from the bulbocavernosus muscle using concentric needle electrode. RESULTS: The BCR by clinical examination was absent in 6 patients, hypoactive in 10 patients and normal in 10 patients. RMS values of absent, hypoactive and normal BCR groups were 15.1+/-3.6 uV, 26.7+/-13.2 uV and 36.3+/-4.8 uV, respectively. RMS values of three groups were significant different (p=0.028) and showed significant correlation (r=0.714, p= 0.005). CONCLUSION: There was strong correlation between RMS value of motor unit action potentials of bulbocavernosus muscle and degree of BCR activities estimated by clinical examination in patients with spinal cord injury.

Urodynamic Findings and Voiding Symptoms according to LesionSites in Stroke

OBJECTIVE: To investigate urodynamic findings and voiding symptoms according to the location of brain lesion after stroke. METHOD: Twenty-six patients with stroke (19 infarction, 7 hemorrhage) who had complained of voiding dysfunction were studied. Brain MRI was performed to identify the suprapontine lesion or pontine lesion. Intravesical pressure and voiding control function of the external urethral sphincters were evaluated by urodynamic study with electromyographic study of the external urethral sphincter. Also voiding symptoms were evaluated. We classified voiding dysfunction into three subgroups by urodynamic findings as follows: detrusor hyperactivity, detrusor hypoactivity, and normal. Functions of the external urethral sphincters were divided into normal, impairment of external urethral sphincter volitional control (IEUS), and detrusor-sphincter dyssynergia (DSD). The symptoms of voiding dysfunction were categorized into three types as a irritative, obstructive or mixed type. RESULTS: In patients with suprapontine lesion (n=22), 11 (50%) showed hyperactive detrusor and 6 (27.3%) showed hypoactive detrusor. However, in the pontine lesion (n=4), one patient (25%) was normal and the others were hypoactive detrusor. Fourteen cases (64%) of the suprapontine lesion and 1 case (25%) of pontine lesion demonstrated normal external urethral volitional control. Seven of 11 patients with irritative symptoms showed detrusor overactivity. Five of 9 patients with obstructive symptoms showed hypoactive detrusor. CONCLUSION: We concluded that hyperactive detrusor in suprapontine lesion and hypoactive detrusor in pontine lesion were dominant. However, voiding symptoms in stroke patients were various according to the external urethral sphincter function as well as the detrusor activity.

Determination of Neurological Impairment Level in Thoracic SpinalCord Injuries using Dermatomal Somatosensory Evoked Potentials

OBJECTIVE: To determine sensory levels of injury using dermatomal somatosensory evoked potentials (SEPs) and compare with the neurological level of injury determined by ASIA standard in patients with thoracic spinal cord injury. METHOD: By stimulating segmental thoracic dermatomes, cortical SEPs were studied in nine spinal cord injured patients from T2 to T12 (9 men, mean age 41.8) and 20 normal adult men (mean age, 28.3). The SEP studies were performed bilaterally. RESULTS: In eight cases (44%) of the paraplegics tested, the neurological level of injury by dermatomal SEPs was same compared to the level of injury assessed by ASIA standard. In 15 cases (83%), there were no or one level difference of the level of injury between the levels by SEPs and ASIA standard. CONCLUSION: This study suggests that dermatomal SEP can be a useful tool in determination of the neurological level of injury in patients with thoracic spinal cord injury.

Evaluation of Vascular Response using Impedance Plethysmography in Patients with Spinal Cord Injury

OBJECTIVE: To determine abnormal vascular response to cuff ischemia in patients with spinal cord injury (SCI). METHOD: Ankle blood pressure (ABP) and ankle-brachial index (ABI) in 20 SCI patients (14 men, 6 women, mean age 39.8 years) and control group (14 men, 6 women, mean age 40.2 years) were measured using impedance plethysmography at rest and after distal thigh cuff compression for 5 and 10 minutes. The patients were divided into tetraplegia (10), paraplegia (10), complete injury (8) and incomplete injury (12). RESULTS: There was no significant difference in ABP and ABI at rest and 5 minutes of ischemic compression between the patients and control groups. However, ABP and ABI decreased more at 10 minutes of ischemic compression in SCI compared to control groups (p<0.001). Changes of ABP and ABI between tetraplegia and paraplegia were not different. Complete injury of SCI decreased more than incomplete injury at 10 minutes of ischemia in the APB and ABI (p<0.001). CONCLUSION: Vascular control was significantly impaired in patients with spinal cord injury. Impedance plethysmography can be a useful and objective tool in evaluation of vascular response for the patients with spinal cord injury.

A Case of Type IV Sacral Agenesis: A case report

Sacral agenesis is an uncommon condition characterized by absence of different segments of lumbar spine along with total or partial absence of sacrum. It does not have an established etiology but may be associated with insulin dependent diabetes mellitus in the mother. Motor deficits are present and correspond to the level of vertebral agenesis. Sensation is better preserved than motor function. Orthopedic deformities such as hip dislocation, flexion contractures, genu recurvatum, posterior compartment atrophy, scoliosis and so on are observed. Urinary and bladder dysfunction are constant and it can lead to fatal kidney damage, finally. We report one case of type IV sacral agenesis with review of literature.

The Factors Associated with Becoming Obese Children: in 6th Grade Children of Elementary Schools in Busan

BACKGROUND: The purpose of this study was to find the factors associated with becoming obese children during the past 2 years in 6th grade children of elementary school and then to evaluate the high risk groups for childhood obesity. METHODS: The 738 children from May to June, 2001 in 6th grade of 5 elementary schools in Busan were the subjects of this study. The children's height and weight in 4th grade and 6th grade were obtained from school health records. We calculated the body mass index in 4th and 6th grades and classified into 3 weight groups (normal, at risk for obesity, and obese) according to the age and sex-specific BMI established by the Korean Academy of Pediatrics. If children's weight group in 4th grade was changed to heavier weight group in 6th grade, we defined it as increased group. Otherwise, we defined it as non-increased group. The association between the increase in weight group and parental and children's characteristics were analyzed by x2-test, x2-test for the trend, t-test, ANOVA and multiple logistic regression. RESULTS: Among the total, 3.3% of children with normal weight and 16.9% of children at the risk for obesity in 4th grade were transformed to increased group in 6th grade, respectively (P or =2 hours/day for the past two years. These findings suggest promotion of educational plan to prevent childhood obesity.

Comparative analysis of the relationship between basal bone and teeth in normal occlusion and Angle's Class I malocclusion / 대한치과교정학회지

In order to analyze the relationship between teeth and basal bone for the maintainance of the good occlusion, the mesiodistal width of teeth, the basal arch width and the basal arch length were measured on the study model of the normal occlusion group and Angle's class I malocclusion group(non-extraction group, extraction group). The Maximum tooth material, the percentage of basal arch width to maximum tooth material, the percentage of basal arch length to maximum tooth material and the percentage of basal arch width plus basal arch length to maximum tooth material were calculated, and then statistical analysis was done. From this study, the obtained results were as follows; 1. In maxilla, the percentage of basal arch width to maximum tooth material was 46.9+/-2.6% in normal occlusion group, 49.4+/-3.9% in non-extraction group, and 42.5+/-3.3% in extraction group. In mandible, that was 46.6+/-2.4 % in normal occlusion group, 47.5+/-4.0% in non-extraction group, and 42.6+/-2.6% in extraction group. 2. In maxilla, the percentage of basal arch length to maximum tooth material was 33.4+/-1.9% in normal occlusion group, 33.9+/-1.8% in non-extraction group, and 28.7+/-2.5% in extraction group. In mandible, that was 34.4+/-4.3% in normal occlusion group, 36.5+/-1.9% in non-extraction group, and 31.5+/-2.5% in extraction group. 3. In maxilla, the percentage of basal arch width plus basal arch length to maximum tooth material was 80.3+/-3.4% in normal occlusion group, 83.3+/-4.8% in non-extraction group, and 71.2+/-4.3% in extraction group. In mandible, that was 81.0+/-5.2% in normal occlusion group, 84.0+/-5.4% in non-extraction group, and 74.1+/-4.1 % in extraction group. 4. In Maxilla, the 95% confidence interval of the percentage of basal arch width to maximum tooth material was 46.3-47.5% in normal occlusion group, 48.1-50.7% in non-extraction group, and 41.7-47.2% in extraction group. In mandible, that was 46.1-47.2% in normal occlusion group, 46.1-48.8% in non-extraction group, and 42.0-43.3% in extraction group. 5. In maxilla, the 95% confidence interval of the percentage of basal arch length to maximum tooth material was 32.9-33.9% in normal occlusion group, 33.3-34.5% in non-extraction group, and 28.1-29.2% in extraction group. In mandible, that was 33.4-3.4 % in normal occlusion group, 35.8-37.2% in non-extraction group, and 30.9-33.1% in extraction group. 6. In maxilla, the 95% confidence interval of the percentage of basel arch width plus basal arch length to maximum tooth material was 79.5-81.0% in normal occlusion group, 81.6-84.9% in non extraction group, and 70.1-72.2% in extraction group. In mandible, that was 79.8-82.2% in normal occlusion group, 82.1-85.5% in non-extraction group, and 73.1-75.1% in extraction group. 7. There was correlation between maxilla and mandible in the maximum tooth material, the basal arch width, the basal arch length, the percentage of basal arch width to maximum tooth material, the percentage of basal arch length to maximum tooth material and the percentage of basal arch width plus basal arch length to maximum tooth material, but not in the basal arch length of male of the extraction group. *A thesis submitted to the Council of the Graduate School of Kyungpook national University in partial fulfillment of the requirements for the degree of Master of Dental Science in December, 1991.