No abstract available.
Esophagus* ; Transplants*

Sclerema neonatorum (SN) is a rare disease characterized by firm, indurated, waxy skin lesions extending throughout the body, sparing the fat-free soles, palms, and genitalia. The prognosis of SN is generally very poor, with a high fatality rate. We report the case of a full-term infant with delayed onset of SN showing good prognosis. A 4-month-old Korean male infant presented with diffuse waxy, sclerotic skin lesions on the whole body, sparing the face, nipples, and genital area, which began developing at 2 months of age. Histopathologic findings of the sclerotic skin lesions showed wide, fibrous intersecting bands in the subcutaneous fat tissue. Only sparse infiltration of lymphocytes and histiocytes was observed in the fat lobules and septa. Based on clinical presentation and histopathologic findings, he was diagnosed with SN. The patient survived with conservative care and had mild improvement of the skin lesions on his follow-up visit at 12 months of age.
Follow-Up Studies ; Genitalia ; Histiocytes ; Humans ; Infant* ; Lymphocytes ; Male ; Nipples ; Prognosis* ; Rare Diseases ; Sclerema Neonatorum* ; Skin ; Subcutaneous Fat

Amipaque is a water soluble, non-ionic myelographic contrast media, and owing to its high diagnostic accuracy and safety, its use is gradually increasing. The authors studied the complications after Amipaque lumbermyelography in 61 patients with low back pain during the period from Jan. 1981 to Nov. 1981 in Chung Ang University Hospital. The results were as follows; 1. Total complication rate was 52%(32 of 61) and there was nosexual difference in its occurrence. 2. In total, no difference in complication rate was found between head-up positioned group with a degree of 30degrees (group I) after procedure and head-up positioned group with a degree of 70degrees (group II) but female patients had more complication rate in group I than in group II (75% vs 50%).Headache was more common in group I and nausea was more common in group II. 3. Headache was most common complicaiton (44%) and there was no sexual difference in its occurrence. 4. No significant difference in complication rate was found between patients proved to have HBP and patients to have not. 5. Complications were less common in patients with punctured level of L4-5 than in patients with L2-3 or L3-4 level puncture.
Contrast Media ; Female ; Headache ; Humans ; Low Back Pain ; Metrizamide ; Myelography ; Nausea ; Punctures ; Water

Epstein-Barr virus rarely causes encephalitis which has a benign outcome. About 90% of children have a benign clinical course without neurologic sequelae. However, 10% have residual persistent deficits and a mortality rate of up to 10% has also been reported. An 11-month-old boy was admitted after general weakness and poor oral intake. On day 7 of hospitalization, three vomiting episodes occurred and followed by a seizure. Brain T1-weighted magnetic resonance imaging (MRI) showed a hyperintensity with mild diffusion restriction in the cortex and subcortical white matter of the bilateral frontal, parietal, and occipital lobes. Analysis of a cerebrospinal fluid (CSF) sample revealed WBC count of 10 /microL (neutrophils 21%, lymphocytes 78%), red blood cell count of 19,000 /uL. CSF EBV polymerase chain reaction (PCR) was positive. Positive results were also obtained for serum EBV viral capsid antigen (VCA) IgM (>4 U/mL), IgG (>8 U/mL), EBV Ebstein Barr nuclear antigen (EBNA) IgG (>8 U/mL). Despite therapy with acyclovir, phenobarbital and steroids, a brain MRI conducted on day 34 showed extensive parenchymal volume atrophy and secondary ventricular dilatation, diffuse progressive signal change in the entire cerebrum and diffuse gyral enhancement in the entire cerebrum. The patient was discharged on day 129 and was transferred to other hospital. After 3month of discharge, the patient's mental status was still drowsy, both arms and legs showed rigidity, and deep tendon reflex were hyperactive. We report an 11-month-old child with rapidly progressive severe encephalitis associated with Epstein-Barr virus.
Acyclovir ; Arm ; Atrophy ; Brain ; Capsid ; Cerebrospinal Fluid ; Cerebrum ; Child ; Diffusion ; Dilatation ; Encephalitis* ; Erythrocyte Count ; Herpesvirus 4, Human* ; Hospitalization ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Infant ; Leg ; Lymphocytes ; Magnetic Resonance Imaging ; Male ; Mortality ; Occipital Lobe ; Phenobarbital ; Polymerase Chain Reaction ; Rabeprazole ; Reflex, Stretch ; Seizures ; Steroids ; Vomiting

Neonatal cerebral sinovenous thrombosis (CSVT) is a rare disease with severe neurological sequelae. Methylenetetrahydrofolate reductase (MTHFR) is a key enzyme in the folate cycle, and mutations in MTHFR are associated with vascular diseases. Here, we report the case of a newborn with MTHFR mutation-associated CSVT. Analysis of MTHFR in the patient detected heterozygous C677T (677CT) and A1298C (1298AC) mutations. Analysis of MTHFR in the patient's mother did not detect a C677T (677CC) mutation but detected a homozygous A1298C (1298CC) mutation. Our results suggest that the presence of heterozygous MTHFR C677T and A1298C mutations affect thrombophilic activity in the neonate, resulting in the development of refractory seizure and CSVT. Moreover, presence of the homozygous MTHFR A1298C mutation in the patient's mother, who did not show any symptoms associated with thrombophilic activity, and conditions during gestation may have affected the patient's condition.
Cerebral Infarction ; Folic Acid ; Humans ; Infant, Newborn ; Methylenetetrahydrofolate Reductase (NADPH2) ; Mothers ; Pregnancy ; Rare Diseases ; Seizures ; Stroke ; Thrombosis* ; Vascular Diseases

Segmental neurofibromatosis, a subtype of neurofibromatosis type 1, is characterized by neurofibromas and/or café-au-lait spots limited to an area or segment of the body. Checkerboard pattern is a rare type of cutaneous mosaic manifestation, characterized by squares or broad ribbons of affected skin with sharp demarcation at the midline. Herein, we report the case of a patient with bilateral segmental neurofibromatosis with lentiginosis showing a checkerboard pattern. Our patient had multiple hyperpigmented macules on her entire body in a checkerboard pattern since birth. Several café-au-lait patches were observed on the left buttock and right axilla. A neurofibroma was incidentally found beneath the café-au-lait patch by histological examination, which showed ill-defined spindle cells with elongated nuclei at the deep dermis that stained positive for S-100. Based on the clinical presentation and histopathologic results, the patient was diagnosed with bilateral segmental neurofibromatosis with lentiginosis showing a checkerboard pattern.
Axilla ; Body Patterning ; Buttocks ; Dermis ; Humans ; Lentigo ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1 ; Parturition ; Skin

BACKGROUND: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease characterized by tissue-bound and circulating autoantibodies directed against BP180 and/or BP230 antigens. Various inflammatory cells are involved in the development of blister in BP. OBJECTIVE: The aim of this study was to evaluate the correlation between peripheral leukocyte counts and BP severity. METHODS: We retrospectively included 60 patients with BP, who had not been treated with systemic steroid at the time of blood sampling. The patients were classified into two groups, those with admission history (admission group) and those without admission history (non-admission group). Disease severity was evaluated using three parameters: admission history, initial steroid dosage, and modified version of a pemphigus scoring system. We evaluated the correlation between peripheral leukocyte counts and disease severity measured by the three parameters. RESULTS: The admission group showed a significant increase in disease severity measured by initial steroid dosage and severity score compared with the non-admission group. Additionally, the admission group had increased total leukocyte, eosinophil, and neutrophil counts. In the correlation study, the peripheral eosinophil and neutrophil counts showed positive correlation with BP severity evaluated by both initial steroid dosage and the pemphigus scoring system. CONCLUSION: Peripheral eosinophil and neutrophil counts can be used as a marker in predicting disease severity in patients with BP.
Autoantibodies ; Blister ; Eosinophils* ; Humans ; Leukocyte Count ; Leukocytes ; Neutrophils* ; Pemphigoid, Bullous* ; Pemphigus ; Retrospective Studies ; Statistics as Topic

Purpose: To identify factors associated with the clinical response to low-dose dexamethasone therapy (LDDT) in preterm infants for bronchopulmonary dysplasia (BPD). Methods: We used a retrospective medical record review to evaluate preterm infants who were born before 32 weeks of gestation or with a birth weight less than 1,500 g. All infants were admitted to the neonatal intensive care unit at a tertiary academic hospital between January 2010 and June 2019, and received LDDT for BPD. The preterm infants’ respiratory severity scores (RSS) were calculated from the first day of LDDT to the day of extubation, or the last day of LDDT. A good response was defined as a decreasing RSS with a slope greater than 0.181. A poor response was defined as a non-decreasing RSS, or a decreasing RSS with a slope less than 0.181 during LDDT. A total dose of 1.1 mg/kg was administered for 10 days for each single course of LDDT. Results: A total of 51 preterm infants were included in the final analysis. Thirty preterm infants (58.8 %) were in the good response group, and 21 preterm infants (41.2%) were in the poor response group. There were no significant differences in gestational age, birth weight, and sex between the good response group and poor response group. Preterm premature rupture of membrane and histologic chorioamnionitis were significantly associated with a poor response to LDDT. Higher RSS on the first day of the LDDT was associated with a good response to LDDT. Conclusion Antenatal infection and/or inflammation may be associated with an unfavorable response to postnatal LDDT for BPD. Preterm infants with more severe respiratory failure seem to benefit more from LDDT for BPD.

BACKGROUND/AIMS: Chronic liver disease leads to liver fibrosis, and although the liver does have a certain regenerative capacity, this disease is associated with dysfunction of the liver vessels. C-reactive protein (CRP) is produced in the liver and circulated from there for metabolism. CRP was recently shown to inhibit angiogenesis by inducing endothelial cell dysfunction. The objective of this study was to determine the effect of CRP levels on angiogenesis in a rat model of liver dysfunction induced by bile duct ligation (BDL). METHODS: The diameter of the hepatic vein was analyzed in rat liver tissues using hematoxylin and eosin (H&E) staining. The expression levels of angiogenic factors, albumin, and CRP were analyzed by real-time PCR and Western blotting. A tube formation assay was performed to confirm the effect of CRP on angiogenesis in human umbilical vein endothelial cells (HUVECs) treated with lithocholic acid (LCA) and siRNA-CRP. RESULTS: The diameter of the hepatic portal vein increased significantly with the progression of cirrhosis. The expression levels of angiogenic factors were increased in the cirrhotic liver. In contrast, the expression levels of albumin and CRP were significantly lower in the liver tissue obtained from the BDL rat model than in the normal liver. The CRP level was correlated with the expression of albumin in hepatocytes treated with LCA and siRNA-CRP. Tube formation was significantly decreased in HUVECs when they were treated with LCA or a combination of LCA and siRNA-CRP. CONCLUSION: CRP seems to be involved in the abnormal formation of vessels in hepatic disease, and so it could be a useful diagnostic marker for hepatic disease.
Angiogenic Proteins/genetics/metabolism ; Animals ; Bile Ducts/surgery ; C-Reactive Protein/*analysis/genetics/metabolism ; Cells, Cultured ; Disease Models, Animal ; Hepatic Veins/abnormalities ; Hepatocytes/cytology/metabolism ; Human Umbilical Vein Endothelial Cells ; Humans ; Lithocholic Acid/pharmacology ; Liver/metabolism/pathology ; Liver Cirrhosis/etiology ; Liver Diseases/metabolism/*pathology ; Male ; Microscopy, Fluorescence ; Mitochondria/drug effects/metabolism ; RNA Interference ; RNA, Small Interfering/metabolism ; Rats ; Rats, Sprague-Dawley ; Real-Time Polymerase Chain Reaction ; Serum Albumin/genetics/metabolism

PURPOSE: The aim of this study was to investigate the potential effects of mild hypoxia in the mature and immature brain. METHODS: We prepared organotypic slice cultures of the hippocampus and used hippocampal tissue cultures at 7 and 14 days in vitro (DIV) to represent the immature and mature brain, respectively. Tissue cultures were exposed to 10% oxygen for 60 minutes. Twenty-four hours after this hypoxic insult, propidium iodide fluorescence images were obtained, and the damaged areas in the cornu ammonis 1 (CA1), CA3, and dentate gyrus (DG) were measured using image analysis. RESULTS: In the 7-DIV group compared to control tissue, hypoxia-exposed tissue showed decreased damage in two regions (CA1: 5.59%+/-2.99% vs. 4.80%+/-1.37%, P=0.900; DG: 33.88%+/-12.53% vs. 15.98%+/-2.37%, P=0.166), but this decrease was not statistically significant. In the 14-DIV group, hypoxia-exposed tissue showed decreased damage compared to control tissues; this decrease was not significant in the CA3 (24.51%+/-6.05% vs. 18.31%+/-3.28%, P=0.373) or DG (15.72%+/-3.47% vs. 9.91%+/-2.11%, P=0.134), but was significant in the CA1 (50.91%+/-5.90% vs. 32.30%+/-3.34%, P=0.004). CONCLUSION: Although only CA1 tissues cultured for 14 DIV showed significantly less damage after exposure to hypoxia, the other tissues examined in this study showed a tendency towards less damage after hypoxic exposure. Therefore, mild hypoxia might play a protective role in the brain.
Anoxia* ; Brain ; Dentate Gyrus ; Fluorescence ; Hippocampus ; Neuroprotective Agents* ; Oxygen ; Propidium