Pediatric Crohn’s disease with severe morbidity manifested by gastric outlet obstruction: two cases report and review of the literature

Crohn’s disease (CD) presenting as gastric outlet obstruction is rare but serious clinical presentation of CD causing severe morbidity. However, there have been few case reports concerning this disorder in East Asian children and adolescents. The current case report describes 2 pediatric patients with CD who had had gastric outlet obstruction as an initial symptom of CD. Two pediatric patients developed postprandial vomiting, bloating, and unintentional weight loss. The upper endoscopy result indicated that there was pyloric obstruction with mucosal edema, inflammation and ulcers. The serologic test and colonoscopy results suggested CD. These patients were treated with infliximab, and endoscopic balloon dilation without surgery and showed remarkable improvement in obstructing symptoms with maintaining clinical and biochemical remission. This case report elucidates the benefits of early intervention using infliximab and endoscopic balloon dilation to improve gastric outlet obstruction and achieve baseline recovery in patients with upper gastrointestinal B2 phenotype of CD.

Reversible Cerebral Vasoconstriction Syndrome after Heart Transplantation

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe headaches with or without other acute neurological symptoms, and diffuse segmental constriction of cerebral arteries that resolves spontaneously within 3 months. A 44-year-old woman underwent heart transplantation due to primary amyloidosis with heart involvement. She started to have a seizure after three hours after the heart transplantation, and her consciousness was not recovered. Computed tomography and transcranial doppler sonography were used to diagnose RCVS, and contracted vessels were recovered after oral nimodipine administration.

Evaluation of Osteoporotic Vertebral Bone and Disc Degeneration Induced by Malnutrition during Growth Period: Biomechanical and Histomorphometrical Study / 체질인류학회지

The aim of the present study is to analyze the generation of osteoporotic vertebral bone induced by malnutrition during growth period and analyze its effects for disc degeneration, based on biomechanical and histomorphometrical study. Mechanical and histomorphological characteristics of lumbar vertebral bones and discs of rats with calcium free diet (CFD) were detected and tracked by using high resolution in-vivo micro-computed tomography (in-vivo micro-CT), finite element (FE) and histological analysis. Twenty female Sprague-Dawley rats (6 weeks old, approximate weight 170g) were randomly divided into two groups (CFD group: 10, NOR group: 10). The CFD group was maintained on a refined calcium-controlled semisynthetic diet without added calcium, to induce osteoporosis. All lumbars (L1~L6) were scanned by using in vivo micro-CT with 35 micrometer resolution at 0, 4, 8 weeks to track the effects of CFD on the generation of osteoporosis. The results of morphological characteristics showed that BV/TV, Tb.Th, Tb.N in CFD group were significantly decreased over time (p<0.05), while those in NOR group were statistically increased over time (p<0.05) in the most lumbars (L1~L6). We also investigated the contrary tendency in Tb.Sp and SMI, compared to the above results in each group. In the simulated compression test using FE models, the structural effective modulus of CFD group significantly decreased (p<0.05), whereas that of NOR group was statistically increased, depending on the measuring time (p<0.05). The present study observed remarkable histological changes of nucleus pulposus and annulus fibrous by water loss in CFD group, compared with NOR group. These findings indicated that calcium insufficiency was the main factor in the generation of osteoporosis and it induced lumbar vertebral disc degeneration. This study is a valuable experiment to firstly evaluate osteoporotic vertebral bone and disc degeneration induced by malnutrition during growth period from a biomechanical and histomorphometrical point of view.

Increased Pulsatility Index in Acute Lacunar Infarction with Type II Diabetes

BACKGROUND: The pulsatility index (PI) measured by a transcranial Doppler (TCD) has been postulated to reflect the vascular resistance that is distal to the artery being examined. Therefore, pathologies of small perforating arteries may affect the PI of the proximal artery. Microangiopathy is a common vascular complication of diabetes mellitus (DM), which may contribute to the development of small infarctions involving the perforating artery, and may be reflected on the PI. METHODS: We enrolled patients with acute cerebral infarctions who were examined by TCD, MRI, and MRA and fulfilled the following criteria: 1)an infarction of less than 2 cm size involving a single perforating arterial territory; 2)no significant arterial stenosis on MRA; and 3)no cardioembolic sources. Patients were divided into either a group with DM, or without and TCD findings were compared. RESULTS: The DM group showed higher PI than non-DM (0.99 v.s. 0.85 for the right middle cerebral artery; 1.02 v.s. 0.85 for the left middle cerebral artery; and 0.94 v.s. 0.78 for the basilar artery). The mean flow velocity was comparable between the groups. Multivariate linear regression analysis revealed that the duration of DM was a significant predictor of elevated PI of the bilateral MCA and basilar artery and that age was another significant predictor in the case of basilar artery. CONCLUSIONS: The elevated PIs in DM patients suggest the possible role of diabetic microvascular complications in the development of the lacunar infarction. The PI measurement using TCD may be a useful marker of the lacunar infarction, especially in DM patients.

IL-1beta and IL-1 Receptor Antagonist Gene Polymorphisms in Myasthenia Gravis

BACKGROUND: Myasthenia gravis (MG) is an autoimmune disorder characterized by an immune response against the nicotinic acetylcholine receptor at the neuromuscular junction. Genetic factors as well as abnormalities of immune regulation can increase the likelihood of MG. Proinflammatory cytokines interleukin (IL)-1alpha, IL-1beta, and their receptor antagonist (IL-1Ra) play major roles in initiating and modulating immune responses. The aim of the present study was to analyze IL-1beta and IL-1 Ra gene polymorphisms between MG patients and healthy controls. METHODS: TaqI restriction fragment polymorphism (RFLP) in exon 5 of IL-1beta and variable numbers of an 86-bp tandem repeat (VNTR) in intron 2 of IL-1Ra were analyzed in 80 patients with MG and 94 matched healthy control individuals. RESULTS: In IL-1beta TaqI RFLP, the genotype of A1/A1 and A1/A2 were 92.5% and 7.5% in patients with MG. In healthy controls, the frequencies of each genotype were 93.6% and 6.4% respectively. IL-1Ra polymorphism, the genotypes of A1/A1, A1/A2 and A1/A3 were 81.3%, 16.3%, and 2.5% in patients with MG. In healthy controls, the frequencies of each genotype were 87.2%, 7.4% and 3.2% respectively. There was no significant difference in the genotype frequencies of IL-1beta TaqI RFLP and IL-1Ra polymorphism between patients and the control group. CONCLUSIONS: These data suggested that the IL-1beta and IL-1Ra gene polymorphisms may not be associated with MG. However, further study is needed to clarify the possible role of IL-1beta and IL-1Ra gene polymorphisms in the susceptibility to myasthenia gravis.

Measurements of Brain Stem and Cerebellum on Brain MRI: for the Differential Diagnosis Between Multiple System Atrophy and Idiopathic Parkinson's Disease

BACKGROUND: Multiple system atrophy (MSA) and idiopathic Parkinson's disease (IPD) are two common neurodegenerative disorders presenting with parkinsonism. Since a brain MRI study is an available method for differentiating MSA from IPD, we tried to find further values of brain MRI studies in differentiating MSA from IPD. METHODS: We measured anteroposterior and transverse diameters (AD and TD, respectively) of the brain stem of T2-weighted axial images. We graded the severity of atrophy (grade 0: none; grade 1: mild; grade 2: moderate; and grade 3: severe) of cerebellar vermis and hemispheres on the midsagittal and parasagittal planes. RESULTS: There were 36 patients with probable MSA and 40 patients with IPD. We calculated a parameter multiplying AD of the midbrain by TD of the midbrain. The mean of the AD x TD of the midbrain was 1007.5 +/- 161.8 mm2 in patients with MSA, and it was significantly smaller than that of those with IPD (1113.3 +/- 118.7 mm2). When the cut off value was decided as 1050 mm2, the sensitivity of the parameter for the diagnosis of MSA was 83.3% and specificity was 80%. The frequency of cerebellar atrophy was 72.2% in patients with MSA, and it was significantly higher than that of those with IPD (37.5%). CONCLUSIONS: Measurements of the brain stem, particularly the midbrain, and cerebellum areas on brain MRI are helpful methods for the differential diagnosis of patients with MSA from those with IPD.

CTLA-4 Polymorphism in Myasthenia Gravis

BACKGROUND: Cytotoxic T lymphocyte associated antigen 4 (CTLA-4) plays a role in down-regulating both the cellular and the humoral responses by suppressing the ongoing responses of activated T-cells. There are evidences to suggest the genetic contribution of the CTLA-4 locus to a number of autoimmune diseases, such as insulin dependent diabetes mellitus, multiple sclerosis and systemic lupus erythematosus. The aim of the present study is to analyze CTLA-4 gene polymorphism in patients with myasthenia gravis (MG) compared to healthy controls. METHODS: Thirty healthy controls and 31 patients with MG were genotyped into G/G, A/G and A/A of CTLA-4 gene polymorphism at position 49 and the relationship with the clinical feature was analysed. RESULTS: In the patients with MG, the genotype frequencies of G/G, A/G and A/A were 61.3%, 35.5% and 3.2%, respectively. In healthy controls, the frequencies of each genotype were 50%, 43% and 7%, respectively. There was no significant difference in the genotype frequencies of CTLA-4 gene between patients with MG and the control group. There were also no significant differences in the genotype frequencies of CTLA-4 gene between ocular and generalized MG. CONCLUSIONS: These data suggest that the CTLA-4 polymorphism at position 49 do not affect the development of MG. However, further study is needed to clarify the possible role of the CTLA-4 polymorphism in the susceptibility to MG.

The Effect of Initial Serum Neuron-Specific Enolase Level on Clinical Outcome in Acute Carotid Artery Territory Infarction

The prediction of functional outcome in patients with acute cerebral infarction depends on many factors. Various techniques have been applied to predict severity and outcome after cerebral infarction. Neuron-specific enolase (NSE) is a component of a specific brain enzyme and a useful marker of brain injury. We evaluated the relation between initial serum NSE level and short- and long-term clinical outcome in 59 patients with acute cerebral infarction and in 38 age-matched healthy controls. Serum NSE levels were determined in patients with carotid artery (CA) territory cerebral infarction within 24 hours of onset. Brain MRI was performed four to seven days after stroke. Patients were divided into two groups: large CA territory infarction with a lesion extending cortex (cortex group), and small subcortical CA territory infarction (subcortical group) with a lesion confined to the subcortical white matter. We compared the initial serum NSE levels of the two groups. National Institute of Health Stroke Scale (NIHSS) was determined at admission and seven days after onset and the modified Rankin's scale was used at the 3 months follow-up after onset. Serum NSE levels were significantly elevated in patients with acute cerebral infarction compared with the normal controls (13.88 +/- 5.47 ng/dl vs. 8.15 +/- 1.53 ng/dl, p < 0.05). The initial ( < 24 h) serum NSE level was higher in the cortical group than in the subcortical group (16.68 +/- 5.70 ng/dl vs. 10.98 +/- 3.34 ng/dl, p < 0.05). NIHSS on admission and on the 7th day correlated with the initial serum NSE level (p < 0.05), as were more severe functional outcomes, as determined 3 months after onset (p < 0.05). This study shows that initial serum NSE level may be a useful marker for severity in acute ischemic stroke, and that it may be well correlated with short-term and long-term functional outcomes.