Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare, life-threatening mucocutaneous diseases, usually attributable to drugs and infections. Corticosteroids have been used in the management of TEN for the last 30 years. This remains controversial and is still much debated. TEN can occur despite administration of high doses of systemic corticosteroids. The psychiatric side effects of corticosteroids can include headache, insomnia, depression, and mood disorders with or without psychotic episodes. Steroid-induced psychosis is dealt with by tapering or discontinuing the steroid; antipsychotics are also sometimes used. We report a case of an 11-year-old boy who was admitted with TEN. He had also been diagnosed as having nephrotic syndrome in the past. Remission was achieved through induction therapy and by maintaining the use of steroids. After a full-dose intravenous dexamethasone for TEN, he showed psychotic symptoms. We diagnosed him as having steroid-induced psychosis. We tapered the steroid use and initiated an atypical antipsychotic medication, olazapine and intravenous immunoglobulin (IV-IG). His symptoms dramatically improved and he was discharged.
Adrenal Cortex Hormones ; Antipsychotic Agents ; Child ; Depression ; Dexamethasone ; Epidermal Necrolysis, Toxic ; Headache ; Humans ; Immunoglobulins ; Mood Disorders ; Nephrotic Syndrome ; Psychotic Disorders ; Sleep Initiation and Maintenance Disorders ; Steroids ; Stevens-Johnson Syndrome

PURPOSE: Soluble transferrin receptor (sTfR) is a truncated extracellular form of the membrane transferrin receptor produced by proteolysis. Concentrations of serum sTfR are related to iron status and erythropoiesis in the body. We investigated whether serum sTfR levels can aid in diagnosis and treatment of iron deficiency anemia (IDA) in children. METHODS: Ninety-eight patients with IDA were enrolled and were classified according to age at diagnosis. Group 1 comprised 78 children, aged 6-59 months, and group 2 comprised 20 adolescents, aged 12-16 years. RESULTS: In group 1, patients' serum sTfR levels correlated negatively with mean corpuscular volume; hemoglobin (Hb), ferritin, and serum iron levels; and transferrin saturation and positively with total iron binding capacity (TIBC) and red cell distribution width. In group 2, patients' serum sTfR levels did not correlate with ferritin levels and TIBC, but had a significant relationship with other iron indices. Hb and serum sTfR levels had a significant inverse relationship in both groups; however, in group 1, there was no correlation between Hb and serum ferritin levels. In 30 patients of group 1, serum sTfR levels were significantly decreased with an increase in Hb levels after iron supplementation for 1 month. CONCLUSION: Serum sTfR levels significantly correlated with other diagnostic iron parameters of IDA and inversely correlated with an increase in Hb levels following iron supplementation. Therefore, serum sTfR levels can be a useful marker for the diagnosis and treatment of IDA in children.
Adolescent ; Anemia, Iron-Deficiency* ; Child* ; Diagnosis* ; Erythrocyte Indices ; Erythropoiesis ; Ferritins ; Humans ; Iron ; Membranes ; Proteolysis ; Receptors, Transferrin* ; Transferrin

PURPOSE: Blood glucose control in diabetic mothers during pregnancy is very important because it can affect fetal and neonatal outcomes. We therefore investigated the clinical outcomes of infants of diabetic mothers in relation to the maternal HbA1c level. METHODS: The subjects were diabetic mothers and their newborns who were admitted in Wonkwang University Hospital from July 2007 to June 2012. We retrospectively reviewed the medical records of 73 neonates, out of the 128 born to diabetic mothers and investigated neonatal and maternal characteristics based on the differences in maternal HbA1c levels. 55 neonates was excluded because maternal HbA1c was not measured. RESULTS: The mean time for testing the maternal HbA1c was 30+/-5.0 weeks, and the mean level was 6.7+/-1.4%. The mean birth weight in neonates was 3,094+/-831.6 g and was higher in the group with > or =7% HbA1c than in the group with < or =6.4% HbA1c (3,370+/-950.8 g vs. 2,855+/-661.97 g, P=0.0113). When assessing neonatal morbidity, the rates of hypoglycemia (56.0% vs. 28.6%, P=0.0381), large baby (36.0% vs. 4.8%, P=0.0015) and congenital heart disease (CHD) (28% vs. 0%, P=0.0055) were higher in the group with > or =7% HbA1c than in those with < or =6.4% HbA1c. Serum blood sugar level was significantly lower in the group with > or =7% HbA1c than those with < or =6.4% HbA1c (26.5+/-10.5 vs. 35.9+/-8.3 mg/dL, P=0.0196). When considering the rate of large babies (> or =4,000g), there were no differences in birth weights between both groups. CONCLUSION: If the maternal HbA1c level during pregnancy is above 7% with glucose control failure, infants born to diabetic mothers are susceptible to hypoglycemia, large baby syndrome and CHD.
Birth Weight ; Blood Glucose ; Glucose ; Heart Defects, Congenital ; Humans ; Hypoglycemia ; Infant* ; Infant, Newborn ; Medical Records ; Mothers* ; Pregnancy ; Retrospective Studies

No abstract available.

No abstract available.

PURPOSE: IgA nephropathy (IgAN) is one of the major causes of end-stage renal disease. Mass school urine screening (SUS) has been performed to enable early detection of chronic renal diseases, including IgAN. We wanted to evaluate the patients with IgAN, including those diagnosed through SUS. METHODS: Between 1998 and 2010, 64 children were diagnosed with IgAN based on renal biopsy results obtained at the Pediatric Nephrology Department, OO University Hospital. We divided these patients into the SUS group (37 cases), diagnosed through SUS, and the symptomatic (Sx) group (27 cases), diagnosed clinically. The medical records of both groups were analyzed retrospectively. RESULTS: The mean age of the SUS and Sx groups was 10.8+/-2.7 and 9.5+/-3.4 years (P>0.05), respectively. Both groups had a higher proportion of male patients. The time from the notification of an abnormal urinary finding to a hospital visit or renal biopsy was shorter in the Sx group than in the SUS group. Regarding clinical manifestations, there were fewer cases with gross hematuria (P<0.001) and edema (P=0.008) in the SUS group, but there were no differences in terms of the therapeutic regimen and treatment duration. Regarding laboratory parameters, the Sx group had a higher white blood cell count (P=0.007) and lower hemoglobin (P=0.007) and albumin (P=0.000) levels. There were no differences in the renal biopsy findings in both groups, based on the history of gross hematuria or the severity of proteinuria. However, in all 64 patients with IgAN, the light microscopy findings (Hass classification) were related to a history of gross hematuria or the severity of proteinuria. CONCLUSION: There were no significant clinical and histological differences between the groups, as both had early stage IgAN. Although SUS facilitates the early detection of IgAN, long-term, large-scale prospective controlled studies are needed to assess the benefits of early diagnosis and treatment in chronic renal disease progression.
Biopsy ; Child ; Early Diagnosis ; Edema ; Glomerulonephritis, IGA* ; Hematuria ; Humans ; Immunoglobulin A* ; Kidney Failure, Chronic ; Leukocyte Count ; Male ; Mass Screening* ; Medical Records ; Microscopy ; Nephrology ; Proteinuria ; Renal Insufficiency, Chronic

Toxic epidermal necrolysis (TEN) is a rare, acute, serious, and potentially fatal skin disease, in which cell death causes the epidermis to separate from the dermis. It is thought to be a hypersensitivity complex that affects the skin and mucous membranes, and is caused by certain medications, infections, genetic factors, underlying immunologic disease, or more rarely, cancers. We report two cases of TEN associated with deflazacort (DFZ), a derivative of prednisolone, used in the first episode of nephrotic syndrome (NS). The skin eruption appeared on the 4(th) and 5(th) weeks after DFZ administration, while NS was in remission. The widespread lesions were managed by intensive supportive treatment, discontinuation of DFZ, and oral administration of cyclosporine. Both patients showed a rapid improvement in symptoms of TEN without any complications or relapse of NS.
Administration, Oral ; Cell Death ; Cyclosporine* ; Dermis ; Epidermis ; Humans ; Hypersensitivity ; Immune System Diseases ; Mucous Membrane ; Nephrotic Syndrome* ; Prednisolone ; Recurrence ; Skin ; Skin Diseases ; Stevens-Johnson Syndrome*

We present a case of tacrolimus-induced encephalopathy after successful kidney transplantation. An 11-year-old girl presented with sudden onset of neurologic symptoms, hypertension, and psychiatric symptoms, with normal kidney function, after kidney transplantation. The symptoms improved after cessation of tacrolimus. Magnetic resonance imaging (MRI) showed acute infarction of the middle cerebral artery (MCA) territory in the right frontal lobe. Three days later, she had normal mental function and maintained normal blood pressure with left hemiparesis. Follow-up MRI was performed on D19, showing new infarct lesions at both cerebral hemispheres. Ten days later, MRI showed further improvement, but brain single photon emission computed tomography (SPECT) showed mild reduction of uptake in both the anterior cingulate gyrus and the left thalamus. One month after onset of symptoms, angiography showed complete resolution of stenosis. However, presenting as a mild fine motor disability of both hands and mild dysarthria, what had been atrophy at both centrum semiovale at 4 months now showed progression to encephalomalacia. There are two points of interest in this case. First, encephalopathy occurred after administration of tacrolimus and improved after discontinuation of the drug. Second, the development of right-side hemiplegia could not be explained by conventional MRI; but through diffusion tensor imaging (DTI) and diffusion tensor tractography (DTT) of white matter tract, visualization was possible.
Angiography ; Atrophy ; Blood Pressure ; Brain ; Cerebrum ; Child ; Constriction, Pathologic ; Diffusion ; Diffusion Tensor Imaging ; Dysarthria ; Encephalomalacia ; Follow-Up Studies ; Frontal Lobe ; Gyrus Cinguli ; Hand ; Hemiplegia ; Humans ; Hypertension ; Infarction ; Kidney ; Kidney Transplantation ; Magnetic Resonance Imaging ; Middle Cerebral Artery ; Neurologic Manifestations ; Paresis ; Tacrolimus ; Thalamus ; Tomography, Emission-Computed, Single-Photon

Nephrotic syndrome is a clinical syndrome characterized by heavy proteinuria, hypoalbuminemia, edema and hyperlipidemia. Causes of idiopathic nephrotic syndrome include minimal change nephrotic syndrome (MCNS), focal segmental glomerulosclerosis (FSGS) and mesangial proliferation. Other causes of nephrotic syndrome are rare genetic disorders and secondary diseases associated with drugs, infections, or neoplasia. Since February 2009, a swine-origin H1N1 influenza virus (S-OIV) from Mexico has been spread among humans in unexpected rapidity. S-OIV is markedly different from seasonal influenza, in that many of those affected are previously healthy young people. While pulmonary complications of S-OIV infection have been frequently documented, renal complications have not been as widely recognized. We report a case of 4 year-old boy who had developed nephrotic syndrome after S-OIV infection with good response after steroid treatment.
Edema ; Glomerulosclerosis, Focal Segmental ; Humans ; Hyperlipidemias ; Hypoalbuminemia ; Influenza, Human ; Mexico ; Nephrosis, Lipoid ; Nephrotic Syndrome ; Orthomyxoviridae ; Oseltamivir ; Proteinuria ; Seasons

PURPOSE: Juvenile xanthogranuloma usually affects the anterior uvea before the age of 1 year, and it may less frequently involve the corneoscleral limbus. We report a case of juvenile xanthogranuloma of the corneoscleral limbus in a 7-year-old boy. METHODS: A 7-year-old boy was referred for the evaluation of a painless limbal mass in the left eye, noticed 1 month previously. On examination, the visual acuity was 16/20 (OD) and 10/20 (OS) without correction, and the corrected visual acuity was 20/20 in both eyes. The other ocular findings were normal, except for an elevated, well circumscribed, smooth surfaced mass measured 9x6 mm at the upper temporal corneoscleral limbus of the left eye. After the excision of the mass, the frozen biopsy and the immunohistochemical staining for CD68 and S100 protein were performed. RESULTS: The lesion consisted of a red-brown, vascular, and raised mass. Histopathologically, there was a chronic granulomatous inflammation with stromal fibrosis, which included many giant cells immunoreactive for CD68 and S100 protein. There was no evidence of recurrence during follow-up period of 5 months and 20 months. CONCLUSIONS: The case was diagnosed as juvenile xanthogranuloma by the clinical and histopathologic findings. The juvenile xanthogranuloma of the corneoscleral limbal involvement is very rare and it can be treated with the surgical excision for the purpose of improvement of cosmetic problems without any recurrence. It seems to have very good prognosis when completely excised.
Biopsy ; Child ; Fibrosis ; Follow-Up Studies ; Giant Cells ; Humans ; Inflammation ; Male ; Prognosis ; Recurrence ; Uvea ; Visual Acuity ; Xanthogranuloma, Juvenile*