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

Pontine hematomas are very rare lesions that in the past found at postmortem examination rather than diagnosed from the clinical presentations. Computed tomography has particularly stimulated further neurosurgical interest in this relatively rare condition, making surgical evacuation possible in some cases. Four patients suffering from pontine hemorrhages, revealed by computed tomography, are discussed. The classification regard to the site, clinical features and outcomes of this lesion are discussed in an attempt to establish general criteria for adequate management. With regard to the site, three types of hemorrhage are to be considered the tegmentotectal(type I), the tegmentolateral(type II) and the tegmentobasilar(type III), 2 patients(type I II, treated conservatively) had a fair outcome, and 2 patients(one of type III, treated conservatively, and one of type I, by surgical evacuation) had a death. Surgical management for the pontine hemorrhages should be reserved for the type I(tegmontotectal) and type II(tegmentolateral), whenever the neurological conditions deteriorate progressively.
Autopsy ; Classification ; Hematoma ; Hemorrhage* ; Humans ; Prognosis*

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*

PURPOSE: Recently, the conception and cognition that enuresis was resolved spontaneously, have changed. We reviewed the attitudes of the primary care physicians who make diagnose and treat nocturnal enuresis. METHODS: From January 2006 to February 2007, a total of 293 primary care physicians in Daegu city participated in this survey. Questionnaires included questions about physicians' opinions on the appropriated age for diagnosis of enuresis, the likely causes of enuresis, etc. Physicians are grouped in two according to whether enuresis is major field of their subspecialty; the pediatrician & urologist group and the other physician group. RESULTS: 59.2% of pediatricians and urologists thought that enuresis is defined as the nightly involuntary release of urine by children of the age of 5 to 6, while 49.6% of other physicians did. For the causes of enuresis, most of clinicians checked "yes" to the question that "Under- developed bladder and nerve" and "Emotional problems". In the patient's behavioral reactions related to enuresis, "Lack of concentration in home and school" and "Frequent urination" were most responded. Attendance to the education program of enuresis in last five years and willing to participate in education program was statistically different among pediatricians-urologists and other physicians. Regarding the treatment of enuresis, most physicians used imipramin widely, but pediatricians and urologists preferred desmopressin. Alarm was the last one in treatment modality. CONCLUSIONS: This study revealed that pediatricians and urologists are attending more to the educational places and knowing much about the recent information on enuresis when compared to other primary care physicians, regarding the diagnostic age and treatment modality of enuresis. The education of enuresis for primary physicians is more needed.
Child ; Cognition ; Deamino Arginine Vasopressin ; Enuresis ; Fertilization ; Humans ; Nocturnal Enuresis ; Physicians, Primary Care ; Primary Health Care ; Surveys and Questionnaires ; Urinary Bladder

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

BACKGROUND: Arthroscopic rotator cuff repair generally has a good clinical outcome but shoulder stiffness after surgery due to subacromial adhesion is one of the most common and clinically important complications. Sodium hyaluronate (HA) has been reported to be an anti-adhesive agent in a range of surgical procedures. However, there are few reports of the outcomes of arthroscopic rotator cuff repair of the shoulder. This study examined whether a subacromial injection of HA/carboxymethylated cellulose (CMC) affected the postoperative shoulder stiffness and healing of rotator cuff repair, as well as the safety of an injection. METHODS: Between January 2008 and May 2008, 80 consecutive patients with arthroscopic rotator cuff repair were enrolled. The patients were assigned randomly to the HA/CMC injection group (n = 40) or control group (n = 40). All patients were evaluated using the visual analog scale (VAS) for pain, passive range of motion at 2, 6 weeks, 3, 6, 12 months after surgery, and the functional scores at 6, 12 months postoperatively. Cuff healing was also evaluated using CT arthrography or ultrasonography at 6 or 12 months after surgery. RESULTS: The HA/CMC injection group showed faster recovery of forward flexion at 2 weeks postoperatively than the control group but the difference was not statistically significant (p = 0.09). There were no significant difference in pain VAS, internal rotation, external rotation and functional scores between two groups at each follow-up period. The functional scores improved 6 months after surgery in both groups but there were no differences between the two groups. The incidence of unhealed rotator cuff was similar in the two groups. There were no complications related to an injection of anti-adhesive agents including wound problems or infections. CONCLUSIONS: A subacromial injection of an anti-adhesive agent after arthroscopic rotator cuff repair tended to produce faster recovery in forward flexion with no adverse effects on cuff healing. However, its anti-adhesive effects after rotator cuff repair should be considered carefully with further studies.
Adult ; Aged ; Arthroscopy/*adverse effects/*methods ; Carboxymethylcellulose Sodium ; Drug Carriers ; Female ; Humans ; Hyaluronic Acid/adverse effects/*therapeutic use ; Male ; Middle Aged ; Pain ; Prospective Studies ; Range of Motion, Articular ; Recovery of Function ; Rotator Cuff/injuries/*surgery ; Shoulder Joint/physiology ; Tissue Adhesions/*prevention & control ; Treatment Outcome ; Viscosupplements/adverse effects/*therapeutic use

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

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*

PURPOSE: Stem cell transplantation (SCT) has gained worldwide acceptance as a treatment for hematologic disorders. This study was performed to evaluate the clinical characteristics and outcomes of acute kidney injury after SCT in children. METHODS: The records of 53 patients who were treated with SCT at the pediatric department of Yeungnam University Hospital between January, 1996 and April, 2009 were used as subjects. Their were divided into two groups ; 'Early renal insufficiency' (ERI, n=18) and 'Non-early renal insufficiency' (NERI, n=35). ERI had greater than 25% of drop in GFR after SCT. RESULTS: Total 53 patients were analyzed. In cord blood SCT (n=11), ERI was 4 (36.4%) and NERI was 7 (63.6%). In bone marrow SCT (n=16), ERI was 8 (50.0%) and NERI was 8 (50.0%). In autologous peripheral blood SCT (n=26), ERI was 6 (23.1%) and NERI was 20 (76.9%). There is no difference in both groups according to kinds of SCT. GVHD was developed in 22 patients, and there is no difference in each group. Twenty two of 53 pateints died. ERI was 12 (66.7%) and NERI was 10 (28.6%). Acute renal failure is most important cause of the deaths. CONCLUSION: Out of 53 pediatric patients who were treated with SCT, 18 patients had greater than 25% of drop in GFR. There is no difference in both groups according to kinds of SCT. GVHD was found in 22 patients and there is no relation between GVHD development and acute kidney injury.
Acute Kidney Injury ; Bone Marrow ; Fetal Blood ; Humans ; Renal Insufficiency ; Stem Cell Transplantation ; Stem Cells