Diagnostic Stewardship on the Extra-Analytical Phase to Secure Quality Assurance of Diagnostic Tests for Clostridioides difficile Infection

Background@#Clostridioides difficile infection (CDI) is the most essential cause of antimicrobial-related diarrhea in hospitalized patients. It is also considered as an important hospital-acquired infection. Adequately performing the laboratory diagnostic tests for CDI is critical for the control and treatment of CDI in healthcare facilities. This study focused on the management of the extra-analytical phase to secure quality assurance of the diagnostic tests for CDI. @*Methods@#We analyzed the volume and pattern of requested C. difficile - related tests from patients in 2018. The culture-positive rate was compared with the stool quality. Finally, the clinical characteristics of antibiotic-related diarrhea were compared based on whether toxigenic C. difficile was isolated. @*Results@#Culture plus toxin enzyme immunoassay was the most common pattern; however, it showed low positive rates for toxigenic C. difficile . The culture-positive rates were not different according to the Bristol stool form scale. The significant risk factors for the detection of toxigenic C. difficile in patients with antibiotic-associated diarrhea were inhibitor-combined penicillin or cephalosporin (odds ratio [OR], 5.060; 95% confidence interval [CI], 1.317–19.447; P =0.0183) and extended-spectrum cephalosporin (OR, 16.224; 95% CI, 3.166–83.134; P =0.0008). @*Conclusions@#The pre-pre-analytical errors are easy to overlook in routine laboratory procedures. Our findings could provide a good example of the management of the extra-analytical phase to secure quality assurance of the diagnostic tests for CDI.

Low-dose, Once-a-day Treatment of Topiramate in Benign Childhood Epilepsy with Centrotemporal Spikes / 대한소아신경학회지

PURPOSE: Benign childhood epilepsy with centrotemporal spikes (BECTS) is known to have a good prognosis, and is easily controlled with antiepileptic drugs. In this study, we evaluate the efficacy of low-dose topiramate (TPM), once at night time in children with BECTS, and compare with that of oxcarbazepine (OXC). METHODS: OXC was used as a first drug in 58 patients with BECTS at the department of pediatrics, Chonnam National University hospital from Jan. 2003 to Nov. 2010. Their medical records were reviewed, focusing on the onset age of seizure, the dosage of drugs, response to treatment and other clinical features. Since Aug. 2008 through Apr. 2011, some newly diagnosed patients with BECTS were given a low-dose (25mg) of TPM, once at nighttime. The efficacy was reviewed in 14 children, who had been followed up in the outpatient clinic for longer than twelve months. RESULTS: Forty five out of 58 (77.6%) patients were well controlled with OXC. Thirteen out of 58 (22.4%), seizures recurred, on whom TPM (n=9) or lamotrigine (n=4) was added. Nine (64.3%) of the 14 patients who were started with low dose TPM became seizure-free for at least 12 months. However, Two out of 14, the dose of TPM had to be increased up to 100 mg/day and 75 mg/day, respectively. In three, seizure was controlled with OXC. CONCLUSION: A single low-dose TPM would be as effective as the conventional OXC without any adverse effects in children with BECTS.

The Poor Seizure Outcome of Acute Febrile Encephalopathy in Children / 대한소아신경학회지

PURPOSE: The aim of this study was to describe the clinical course and outcome of the patient with epilepsy after acute febrile encephalopathy. METHODS: Medical records of the twenty patients with acute febrile encephalopathy from Mar. 2003 to Dec. 2011, were reviewed. The outcome of epilepsy over 12 months after discharge from encephalopathy was investigated and compared with non-epilepsy group on several clinical and laboratory aspects. RESULTS: All revealed negative on viral study. Eight of 20 patients evolved to epilepsy eventually and 3 out of them had a status epilepticus as initial manifestation. Five of them remained medically intractable. Two showed ongoing violent behavior during follow-up period and 1 had motor weakness of lower limbs for a year. All had non-specific brain MRI findings, except the one who showed suspected cerebritis of right basal ganglia on follow-up study. Five of 8 epilepsy patients showed epileptic discharges on the first electroencephalogram. Two of them showed sustained frontal spikes and one who had abnormal brain MRI findings showed left temporal spike on follow-up EEG. Longer period of altered mentality and more frequent epileptiform discharges on initial EEG appeared to be related with progression to epilepsy during follow-up period. CONCLUSION: Acute febrile encephalopathy in children may be related to poor seizure outcome and resultant psychomotor problems. Further studies including laboratory exams to define its pathophysiology would be needed.

Stem Cell Dynamics in an Experimental Model of Stroke / 전남의대학술지

We investigated the migration of endogenous neural stem cells (NSCs) toward an infarct lesion in a photo-thrombotic stroke model. The lesions produced by using rose bengal dye (20 mg/kg) with cold light in the motor cortex of Sprague-Dawley rats were also evaluated with sequential magnetic resonance imaging (MRI) from 30 minutes through 8 weeks. Migration of NSCs was identified by immunohistochemistry for nestin monoclonal antibody in the lesion cortex, subventricular zone (SVZ), and corpus callosum (CC). The contrast to noncontrast ratio (CNR) on MRI was greatest at 12 hours in DWI and decreased over time. By contrast, T1-weighted and T2-weighted images showed a constant CNR from the beginning through 8 weeks. MRI of the lesional cortex correlated with histopathologic findings, which could be divided into three stages: acute (edema and necrosis) within 24 hours, subacute (acute and chronic inflammatory cell infiltration) at 2 to 7 days, and chronic (gliofibrosis) at 2 to 4 weeks. The volume of the infarct was significantly reduced by reparative gliofibrosis. The number of nestin+ NSCs in the contralateral SVZ was similar to that of the ipsilateral SVZ in each group. However, the number of nestin+ NSCs in the ipsilateral cortex and CC increased at 12 hours to 3 days compared with the contralateral side (p<0.01) and was reduced significantly by 7 days (p<0.01). Active emigration of internal NSCs from the SVZ toward the infarct lesion may also contribute to decreased volume of the infarct lesion, but the self-repair mechanism by endogenous NSCs is insufficient to treat stroke causing extensive neuronal death. Further studies should be focused on amplification technologies of NSCs to enhance the collection of endogenous or transplanted NSCs for the treatment of stroke.

Stem Cell Dynamics in an Experimental Model of Stroke / 전남의대학술지

We investigated the migration of endogenous neural stem cells (NSCs) toward an infarct lesion in a photo-thrombotic stroke model. The lesions produced by using rose bengal dye (20 mg/kg) with cold light in the motor cortex of Sprague-Dawley rats were also evaluated with sequential magnetic resonance imaging (MRI) from 30 minutes through 8 weeks. Migration of NSCs was identified by immunohistochemistry for nestin monoclonal antibody in the lesion cortex, subventricular zone (SVZ), and corpus callosum (CC). The contrast to noncontrast ratio (CNR) on MRI was greatest at 12 hours in DWI and decreased over time. By contrast, T1-weighted and T2-weighted images showed a constant CNR from the beginning through 8 weeks. MRI of the lesional cortex correlated with histopathologic findings, which could be divided into three stages: acute (edema and necrosis) within 24 hours, subacute (acute and chronic inflammatory cell infiltration) at 2 to 7 days, and chronic (gliofibrosis) at 2 to 4 weeks. The volume of the infarct was significantly reduced by reparative gliofibrosis. The number of nestin+ NSCs in the contralateral SVZ was similar to that of the ipsilateral SVZ in each group. However, the number of nestin+ NSCs in the ipsilateral cortex and CC increased at 12 hours to 3 days compared with the contralateral side (p<0.01) and was reduced significantly by 7 days (p<0.01). Active emigration of internal NSCs from the SVZ toward the infarct lesion may also contribute to decreased volume of the infarct lesion, but the self-repair mechanism by endogenous NSCs is insufficient to treat stroke causing extensive neuronal death. Further studies should be focused on amplification technologies of NSCs to enhance the collection of endogenous or transplanted NSCs for the treatment of stroke.

A case of Bickerstaff's brainstem encephalitis in childhood / 소아과

Bickerstaff's brainstem encephalitis (BBE) is a rare disease diagnosed by specific clinical features such as 'progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks' and 'disturbance of consciousness or hyperreflexia' after the exclusion of other diseases involving the brain stem. Anti-ganglioside antibodies (GM, GD and GQ) in the serum or cerebrospinal fluid (CSF) are sometimes informative for the diagnosis of BBE because of the rarity of positive findings in other diagnositic METHODS: brain magnetic resonance imaging (MRI), routine CSF examination, motor nerve conduction study, and needle electromyography. We report a rare case of childhood BBE with elevated anti-GM1 antibodies in the serum, who had specific clinical symptoms such as a cranial polyneuropathy presenting as ophthalmoplegia, dysarthria, dysphagia, and facial weakness; progressive motor weakness; altered mental status; and ataxia. However, the brain MRI, routine CSF examination, nerve conduction studies, electromyography, somatosensory evoked potentials, and brainstem auditory evoked potentials were normal. BBE was suspected and the patient was successfully treated with intravenous immunoglobulins.

Three Cases of Acute Ophthalmoplegia without Ataxia / 대한소아신경학회지

Acute ophthalmoplegia is caused by various etiologies; cerebrovascular diseases, tumors, infections, diabetes mellitus, multiple sclerosis, and myasthenia gravis. Acute ophthalmoplegia without ataxia(AO), regarded atypical Miller-Fisher syndrome, can be defined as a progressive, relatively symmetric ophthalmoplegia by 4 weeks without ataxia or limb weakness, on circumstance of ruling-out other diseases. The additional features that are strongly supportive of the diagnosis of AO are as follows: 1) a history of infectious symptoms within 4 weeks before the onset of neurological symptoms; 2) cerebrospinal fluid albuminocytologic dissociation; and 3) presence of anti-GQ1b IgG antibody. AO has been sporadically reported, but there is still short of information for its clinical and laboratory characteristics in children. We report three children with AO, who were presented with acute ophthalmoplegia without other abnormal neurologic symptoms. All of the patients met the diagnostic criteria of AO, but only one of them had a positive serum anti-GQ1b antibody. So, we need to suspect the diagnosis of AO, even in the cases with negative result of serum anti-GQ1b antibody.

A Case of Basal Cell Nevus Syndrome / 대한소아신경학회지

Basal cell nevus syndrome(BCNS) is a rare autosomal dominant disorder characterized by variable developmental anomalies and predisposition to cancers. The main manifestations include multiple basal cell carcinomas of skins, odontogenic keratocysts, facial dysmorphism, skeletal abnormalities including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We experienced a case of 12-year-old girl with the clinical features of basal cell nevus syndrome.

A Case of Radiologically Suspected Mesenteric Plexiform Neurofibromas in a Patient with Type I Neurofibromatosis / 대한소아신경학회지

Neurofibromatosis type I(NF-1) is an autosomal dominant neurocutaneous syndrome characterized by cafe-au-lait spots, optic glioma, skeletal dysplasia, and iris hamartoma. Mesenteric plexiform neurofibromas(PNF) have been rarely reported in NF-1, especially in children. We report a case of radiologically suspected mesenteric PNF along the celiac axis in an 8-year-old boy who had cafe-au-lait spots and a family history of maternal NF-1.

Spontaneous intracranial internal carotid artery dissection in a child with psoriasis / 소아과

A 13-year-old girl with psoriasis of the elbow, trunk, and face suddenly developed a severe headache followed by left hemiparesis and facial palsy. Brain magnetic resonance imaging showed an acute infarction of the right temporofrontal lobe and basal ganglia on the T2- and diffusion-weighted images. Cerebral angiography showed pre-occlusive irregular scalloped stenosis (99%) in the proximal M1 segment of the right middle cerebral artery and a web-like stenosis at the supraclinoid portion of the right internal carotid artery (ICA) suggestive of a spontaneous intracranial ICA dissection. The patient was administered a low dose of dipyridamole, and a rehabilitation program was initiated. Headache, left motor weakness, and facial droop improved within a week. However, mild left facial palsy and reduced fine motor function of the left hand were still present after 3 weeks. We report a rare case of spontaneous intracranial ICA dissection in a child with psoriasis.

Spontaneous intracranial internal carotid artery dissection in a child with psoriasis / 소아과

A 13-year-old girl with psoriasis of the elbow, trunk, and face suddenly developed a severe headache followed by left hemiparesis and facial palsy. Brain magnetic resonance imaging showed an acute infarction of the right temporofrontal lobe and basal ganglia on the T2- and diffusion-weighted images. Cerebral angiography showed pre-occlusive irregular scalloped stenosis (99%) in the proximal M1 segment of the right middle cerebral artery and a web-like stenosis at the supraclinoid portion of the right internal carotid artery (ICA) suggestive of a spontaneous intracranial ICA dissection. The patient was administered a low dose of dipyridamole, and a rehabilitation program was initiated. Headache, left motor weakness, and facial droop improved within a week. However, mild left facial palsy and reduced fine motor function of the left hand were still present after 3 weeks. We report a rare case of spontaneous intracranial ICA dissection in a child with psoriasis.

Diamond-Blackfan anemia: long-term follow-up of six cases / 소아과

PURPOSE: Diamond-Blackfan anemia (DBA) is a rare heterogeneous genetic disorder of infancy and early childhood. It is characterized by red cell aplasia, congenital anomalies, and a predisposition to cancer. Corticosteroids and red cell transfusions are the mainstays of therapy. We describe our experience of 6 cases of DBA that were encountered over a period of 16 years. METHODS: Medical records of 6 patients diagnosed to have DBA and admitted to the Chonnam National University Hospital between 1992 and 2008 were retrospectively reviewed. RESULTS: Three patients were males. The age at diagnosis ranged from 3 to 18 months (median, 5.5 months). Heart defects were observed in 4, polydactyly in 2, and strabismus in 1 patient. The median number of transfusions was 3 (range, 2 -8). All patients responded to initial treatment with steroids and had a hemoglobin level > or =9 g/dL with a median of 12.5 days (range, 7-22 days). Three patients are currently not receiving steroid therapy. A minimum dose of oral prednisolone ( or =9 mg/dL in 3 cases. Red cell transfusion was infrequently required in 1 patient. In the median follow-up of 14 years, there was no development of malignancy. No significant side effects of steroids were found, except for short stature in 2. CONCLUSION: The majority of DBA patients achieved complete response and under maintenance therapy with low dose of steroids. Close observation is needed to monitor steroid side effects, cardiac function, and development of malignancy. A nation-wide survey is necessary to further characterize this rare disease in Korean children.

Congenital hernia of the lung through the azygoesophageal recess / 소아과

A lung hernia, defined as the protrusion of pulmonary tissue and pleural membranes through a defect in the thoracic wall, is a rare event. It can be congenital or acquired, and cervical, thoracic, or diaphragmatic in location. We report the rare occurrence of a congenital atraumatic lung herniation through the azygoesophageal recess. An 8 -month-old male infant, who was born at 3 5 weeks gestation, had a chronic cough. Chest radiography showed haziness at the right lower lobe of the lung (RLL). Chest computed tomography (CT) revealed herniation of the RLL through the azygoesophageal recess. If persistent unilateral haziness is observed on chest radiography, the possibility of lung herniation should be considered.