The intestinal immune system maintains oral tolerance to harmless antigens or nutrients. One mechanism of oral tolerance is mediated by regulatory T cell (Treg)s, of which differentiation is regulated by a subset of dendritic cell (DC)s, primarily CD103+ DCs. The aryl hydrocarbon receptor (AhR), a ligand-activated transcription factor, plays an important role in regulating immunity. The intestines are exposed to various AhR ligands, including endogenous metabolites and phytochemicals. It was previously reported that AhR activation induced tolerogenic DCs in mice or in cultures of bone marrow-derived DCs. However, given the variety of tolerogenic DCs, which type of tolerogenic DCs is regulated by AhR remains unknown. In this study, we found that AhR ligand 3,3'-diindolylmethane (DIM) inhibited the development of CD103+ DCs from mouse bone marrow cells stimulated with Flt3L and GM-CSF. DIM interfered with phosphorylation of STAT3 and STAT5 inhibiting the expression of genes, including Id2, E2-2, IDO-1, and Aldh1a2, which are associated with DC differentiation and functions. Finally, DIM suppressed the ability of CD103+ DCs to induce Foxp3+ Tregs.
Animals ; Bone Marrow Cells ; Dendritic Cells* ; Granulocyte-Macrophage Colony-Stimulating Factor ; Immune System ; Intestines ; Ligands ; Mice ; Phosphorylation* ; Phytochemicals ; Receptors, Aryl Hydrocarbon ; Transcription Factors

Isolated ACTH deficiency is a rare cause of secondary adrenocortical insufficiency. The clinical presentation can be similar to that of primary adrenal insufficiency, but most of them may be nonspecific. A female patient of 25 months of age, complainig short stature, showed hypocortisolemia without ACTH & cortisol stimulation by insulin-induced hypoglycemia test. All the other hormone state was normal. Left hand AP view revealed delayed bone age(3 month) compared with chronological age. No radiologic abnormality was found in sella MRI and adrenal CT. Here we report a case of isolated ACTH deficiency presented by short stature.
Addison Disease ; Adrenocorticotropic Hormone ; Female ; Hand ; Humans ; Hydrocortisone ; Hypoglycemia ; Magnetic Resonance Imaging

PURPOSE: Single umbilical artery (SUA), the most common malformation of the umbilical cord, has long been of interest because of its association with congenital malformations. This retrospective study was aimed to evaluate the incidence of SUA and its associated malformations. METHOD: The study population was inborn neonates of Kang-Nam Sacred Heart Hospital of Hallym University. We reviewed retrospectively the medical records of 15 infants with SUA, including 1 infant identified in prenatal ultrasonography. RESULTS: The incidence of SUA was 0.2% (15/9,351 cases). The mean birth weight was 2,658 gram (ranged from 1,060 to 3,700gram), and the mean gestational age was 38 weeks (ranged from 32 to 40 + 3weeks). Six cases (40%) of 15 cases with SUA were associated with congenital malformations : urogenital malformations in 4 cases, cardiovascular malformations in 3 cases, and musculoskeletal malformations in 3 cases. Only 1 case had abmormal karyotype, trisomy 18. CONCLUSION: Because SUA is highly associated with congenital malformations newborns with SUA should have a thorough and complete physical examination at birth and physicians should also be alert to the possibility of congenital malformations not evident at initial examination. Furthermore, prenatal detection of SUA has potential importance, since it may indicate that further work is necessary to rule out associated malformations and to help determine whether management should beltered.
Birth Weight ; Gestational Age ; Heart ; Humans ; Incidence ; Infant ; Infant, Newborn ; Karyotype ; Medical Records ; Parturition ; Physical Examination ; Retrospective Studies ; Single Umbilical Artery* ; Trisomy ; Ultrasonography, Prenatal ; Umbilical Cord

We studied the clinical characteristics, location of epileptogenic regions, and the surgical outcomes in 18 patients with intractable epilepsy associated with previous CNS infections. All patients underwent an extensive presurgical evaluation and 11 patients had intracranial EEG monitoring. On the basis of presurgical evaluation, epileptic regions were localized to the mesial temporal (n = 12) and the neocortical (n = 6) regions. The age of the time of CNS infection was significantly younger and the latent period of non-febrile seizures after CNS infection was longer in patients with mesial temporal lobe epilepsy (MTLE). MRI showed hippocampal atrophy and hippocampal signal changes in 11 of 12 patients with MTLE. Among 6 patients with neocortical epilepsy (NE) 5 patients had normal MRI and one showed cerebral hemi-atrophy. Surgery was successful (class I & II) in all patients with MTLE, however, in the patients with neocortical epilepsy, seizure-free results were not achieved in any patients after resective surgery (6 patients) and only 2 patients achieved Class II outcomes after a second epilepsy surgery consisting of neocortical resection. Patients with MTLE after CNS infection were differentiated from the group of neocortical epilepsy by an earlier onset of CNS infection, a prolonged latent period and a higher frequency of meningitis. The characteristic pathology in this group was hippocampal sclerosis and the surgical result was excellent. Neocortical epilepsy following CNS infection usually had no focal lesion on MRI and was associated with a relatively poor surgical result. This study suggested that the surgical outcome was influenced by the type of epileptic syndromes rather than the etiology of seizures. The association of MTLE with the younger age of CNS infections and with meningitis more frequently suggested that the neocortical neurons during infancy or early childhood may be more resistant to the epileptogenesis, or that the CNS infections in patients with MTLE might be milder in severity to cause selective injuries to the hippocampal neurons during their vulnerable stage.
Adolescence ; Adult ; Encephalitis/surgery* ; Epilepsy/surgery* ; Female ; Human ; Male ; Meningitis/surgery*

PURPOSE: To compare the size, circularity and repeatability between the conventional manual technique and the open-ring guided continuous curvilinear capsulorrhexis (CCC) technique in cataract surgery. METHODS: The present study comprised 120 eyes divided into two equal groups. In the first group (group A), CCC was performed using a 5.3-mm open ring-shaped caliper and in the second group (group B), CCC was performed using the conventional manual technique. Photographs were taken after each surgical step (CCC, phacoemulsification, Intraocular lens insertion and corneal stromal hydration). The diameter, area and circularity of the anterior capsule were compared between the two groups. RESULTS: The anterior capsule diameter was 5.32 +/- 0.26 mm for group A (coefficient of variation 0.049) and 5.14 +/- 0.64 mm for group B (coefficient of variation 0.125); the area was 22.9 +/- 0.71 mm2 for group A (coefficient of variation 0.031) and 21.40 +/- 1.37 mm2 for group B (coefficient of variation 0.064). Circularity was 0.93 +/- 0.24 for group A (coefficient of variation 0.258) and 0.86 +/- 0.78 for group B (coefficient of variation 0.907). CONCLUSIONS: The open ring-guided CCC technique resulted in an increase of diameter and capsulorrhexis area. This method showed superior circularity and repeatability compared to conventional manual technique and can be useful in cataract surgery.
Capsulorhexis* ; Cataract ; Chlormequat ; Eye ; Lenses, Intraocular ; Phacoemulsification

PURPOSE: To investigate the efficacy of botulinum toxin type A chemodenervation in various types of esotropia(ET). METHODS: Enrolled eleven esotropic patients treated with botulinum toxin type A and examined the amount of esotropic correction and success rate of less than 10 PD (prism diopter) of postinjection deviation at 5 months after injection. RESULTS: Among the eleven esotropic patients, there were 7 cases of infantile ET, 2 cases of partially accommodative ET and 2 cases of basic ET. The mean preinjection deviation was 30.5 +/- 7.3 PD. The amount of correction was 17.7 +/- 4.3 PD and correction rate of deviation was 57.5%. In 6 of 11 cases, the postinjection deviation was within 10 PD and therefore success rate was 54.5%. In 5 patients (45.5%), their ET was undercorrected. In patients with under 20 PD of preinjection deviation, success rate was 75% (3/4) and those with over 20PD, the rate was 42.9% (3/7). In partially accommodative ET, 2 (100%) of 2 cases were aligned within 10PD. CONCLUSIONS: In comitant ET, botulinum toxin chemodenervation shows high undercorrection rate. The effects of botulinum toxin type A chemodenervation were better for relatively small angle and in partially accommodative ET than nonaccommodative one. But further study with more cases is needed.
Botulinum Toxins* ; Botulinum Toxins, Type A* ; Esotropia* ; Humans ; Nerve Block

Thyroid ophthalmopathy is an autoimmune disease that affect the orbital and periorbital soft tissue, characterized by bulging eye (exophthalmos) and compressed orbital structures, such as the optic nerves. The indications for surgical treatment for thyroid ophthalmopathy include decreased visual acuity caused by optic neuropathy, conjunctivitis and progressive facial deformity caused by exophthalmos. Orbital wall decompression by nasal endoscopy resulte in good cosmetic effects and visual recovery. Balanced orbital decompression is considered to be a safe and effective surgery that can help avoid postoperative diplopia. We introduce three successful cases of orbital wall decompression for the treatment of thyroid ophthalmopathy.
Autoimmune Diseases ; Congenital Abnormalities ; Conjunctivitis ; Decompression ; Diplopia ; Endoscopy ; Exophthalmos ; Optic Nerve ; Optic Nerve Diseases ; Orbit ; Thyroid Gland ; Visual Acuity

Thyroid ophthalmopathy is an autoimmune disease that affect the orbital and periorbital soft tissue, characterized by bulging eye (exophthalmos) and compressed orbital structures, such as the optic nerves. The indications for surgical treatment for thyroid ophthalmopathy include decreased visual acuity caused by optic neuropathy, conjunctivitis and progressive facial deformity caused by exophthalmos. Orbital wall decompression by nasal endoscopy resulte in good cosmetic effects and visual recovery. Balanced orbital decompression is considered to be a safe and effective surgery that can help avoid postoperative diplopia. We introduce three successful cases of orbital wall decompression for the treatment of thyroid ophthalmopathy.

Hashimotos thyroiditis has been associated with a various autoimmune disorders. The immunologic mechanisms involved in the pathogenesis of these disorders have not always been thought to be the same. Although it was demonstrated that there were high prevalence of abnormal thyroid function and autoantibody in autoimmune hemolytic anemia(AIHA) and Fisher-Evans syndrome(FES), AIHA combined with Hashimotos thyroiditis is rare in Korean literature. It was suggested that a common immunologic mechanism may be involved in the pathogenesis of both disease and the possibility of multiple autoimmune syndrome might present in autoimmune hematologic disorders. We experienced a 74-year old woman with a 12-year history of a hypothyroidism due to Hashimotos thyroiditis was hospitalized with sudden development of warm AIHA with positive Direct & Indirect Coombs test and pericardial effusion. Her thyroid function test showed subclinical hypothyroidism with the maintenance dosage of levothyroxine(100pg/day). With glucocorticoid and plasmapheresis, AIHA and pericardial effusion were corrected successfully. It is suggested that the prudent immunologic study is needed for the anemia developed in patients with Hashimotos thyroiditis with or without hypothyroidism.
Aged ; Anemia ; Anemia, Hemolytic, Autoimmune* ; Coombs Test ; Female ; Humans ; Hypothyroidism ; Pericardial Effusion* ; Plasmapheresis ; Prevalence ; Thyroid Function Tests ; Thyroid Gland* ; Thyroiditis*