A small percentage of individuals have the neurological anomaly of central precocious puberty (CPP). Common neurologic causes of CPP include a tumor or congenital lesions. Although Arnold-Chiari malformation can be caused by congenital or acquired causes, it is unusual in patients with CPP. We present the case of a girl aged 4.5 years who complained of breast budding. Her neurological examination and growth pattern were normal. She had no endocrinological abnormality, except for true precocious puberty. We performed brain magnetic resonance imaging, which showed an Arnold-Chiari type 1 malformation. Currently, this case represents the youngest girl who exhibited both Arnold-Chiari type 1 malformation and precocious puberty. Furthermore, it is likely that there is a meaningful association between the brain lesion and precocious puberty in this case.
Arnold-Chiari Malformation ; Brain ; Breast ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Neurologic Examination ; Puberty, Precocious

BACKGROUND: The aggravating factors still remained unclear in inflammatory bowel disease (IBD). Despite many different therapeutic approaches, many patients do not respond to the therapy. The anti-inflammatory effect of insulin-like growth factor-binding protein-3 (IGFBP-3) was suggested because of its capability of nuclear factor-κB (NF-κB) signaling inhibition. Therefore, we hypothesized that the up-regulation of IGFBP-3 would inhibit an inflammatory process. METHODS: Lipopolysaccharides (LPS) treated intestinal epithelial cell 6 (IEC-6) and dextran sodium sulfate (DSS) induced colitis mice were used as colitis models. Exogenous IGFBP-3 expression was accomplished using the adenoviral vector system expressing IGFBP-3 (Ad/IGFBP-3). The inflammatory responses and relevant cellular responses in IEC-6 cells influenced by IGFBP-3 expression were evaluated by western blotting, 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assay, and reactive oxygen species (ROS) measurement. The severity of colitis was evaluated with the colon tissues of DSS-induced mouse model. RESULTS: We found that the IGFBP-3 expression reduced the production of inflammatory cytokines (cyclooxygenase-2, interleukin-1β, tumor necrosis factor-α) and ROS formation. IGFBP-3 expression also induced cell viability and inhibited NF-κB activation. In line with this data, the severity of DSS-induced mouse colitis was greatly ameliorated by the treatment of IGFBP-3 expressing adenoviral particles characterized with less weight loss and preserved colon length compared with the mice treated with DSS alone. The histopathology of the colon showed the reducing signs of colitis in Ad/IGFBP-3 treated DSS-mice group. CONCLUSION: Therefore, our data suggest that Ad/IGFBP-3 up-regulation reduces colonic inflammatory response as a novel therapeutic protocol for IBD.
Animals ; Blotting, Western ; Cell Survival ; Colitis ; Colon* ; Cytokines ; Dextrans ; Epithelial Cells ; Humans ; Inflammatory Bowel Diseases ; Insulin-Like Growth Factor Binding Protein 3 ; Lipopolysaccharides ; Mice ; Necrosis ; Reactive Oxygen Species ; Sodium ; Up-Regulation* ; Weight Loss

The authors regret that two co-authors were missing in the article.

Cap polyposis is extremely rare in children. We report a case of an 11-month-old male infant who visited our hospital because of rectal prolapse and small amount of hematochezia lasting several days. He also had an epidermal nevus in the sacral area. Colonoscopy showed erythematous, multilobulated, circumferential, polypoid lesions with mucoid discharge from the rectum. He was diagnosed with cap polyposis by endoscopy and histologic examination. He was treated with surgical resection, and was closely followed up. In the relevant literature, there is no report of cap polyposis in an infant. We report the first case of cap polyposis in the youngest infant.
Child ; Colonoscopy ; Endoscopy ; Gastrointestinal Hemorrhage ; Humans ; Infant* ; Male ; Nevus* ; Rectal Prolapse ; Rectum

BACKGROUND: Diabetic ketoacidosis (DKA) is an acute complication of pediatric type 1 diabetes mellitus (T1DM). We aimed to determine the risk factors and clinical characteristics of children and adolescents with DKA. METHODS: We retrospectively evaluated 59 episodes of DKA in 43 patients who were hospitalized for DKA between January 2006 and December 2015. DKA was classified as mild, moderate, or severe, according to patient history of DKA. The clinical and laboratory characteristics of these subgroups were compared. RESULTS: The average patient age was 11.98 ± 4.40 years (range, 1.3~17.9 years). Moderate episodes were the most common, with 21 episodes (35.6%), followed by 19 severe (32.2%) and 19 mild episodes (32.2%). Significant differences were observed between the subgroups regarding their characteristics of aggravated. Severe DKA episodes were more often observed during the winter season. Recurrent DKA cases were determined in 31 episodes (52.5%) who were significantly older, had higher body mass index, and lower serum C-peptide levels than the newly diagnosed DKA group. Female patients experienced severe and moderate cases more frequently (P = 0.041), which included two deaths. Only blood glucose levels were significantly higher in these fatal cases (P = 0.022). CONCLUSION: Even among the patients who had previously experienced DKA and older children with low serum C-peptide levels, there was no reduction in the severity of DKA. We recommend that patients with DKA be carefully treated and continuously informed regarding the importance of maintaining proper blood glucose levels, regardless of their previous history of DKA, age, or regularity of insulin therapy.
Adolescent ; Blood Glucose ; Body Mass Index ; C-Peptide ; Child ; Diabetes Mellitus ; Diabetes Mellitus, Type 1 ; Diabetic Ketoacidosis* ; Female ; Humans ; Insulin ; Retrospective Studies ; Risk Factors ; Seasons

Erdheim-Chester disease (ECD) is a rare form of proliferative non-Langerhans cell histiocytosis that involves multiple organs and is associated with a high mortality. The prognosis of ECD is variable, and it mainly depends on the involved anatomic sites. The treatment modalities have not been standardized, but interferon-α (IFN-α) has been reported to be effective in the management of ECD. ECD usually affects middle aged individuals with a slight male predominance but is extremely rare in children. We present an uncommon case of a 4-year-boy diagnosed with ECD who was treated with IFN-α and corticosteroid. He remained disease-free for 3 years after the completion of treatment.
Child ; Child, Preschool ; Erdheim-Chester Disease ; Histiocytosis ; Humans ; Male ; Middle Aged ; Mortality ; Prognosis

Primary gastric tumors are very rare in children. Burkitt lymphoma is a common type of non-Hodgkin's lymphoma, and gastric Burkitt lymphoma usually occurs in the aged. When involving the gastrointestinal tract, primary gastric Burkitt lymphoma is very rare in younger childhood. Many gastric lymphomas including mucosa-associated lymphoid tissue lymphoma are associated with Helicobacter pylori infection or acute bleeding symptom. We report a seven-year-old boy who presented with only some vomiting and postprandial pain. His upper gastrointestinal endoscopy and biopsy revealed a large primary Burkitt lymphoma with no acute bleeding and no evidence of H. pylori infection. After chemotherapy, he remains in remission.
Biopsy ; Burkitt Lymphoma* ; Child* ; Drug Therapy ; Endoscopy, Gastrointestinal ; Gastrointestinal Tract ; Helicobacter pylori ; Hemorrhage ; Humans ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone ; Lymphoma, Non-Hodgkin ; Male ; Stomach Neoplasms ; Vomiting

Prompt malaria diagnosis is crucial so antimalarial drugs and supportive care can then be rapidly initiated. A 15-year-old boy who had traveled to Africa (South Africa, Kenya, and Nigeria between January 3 and 25, 2011) presented with fever persisting over 5 days, headache, diarrhea, and dysuria, approximately 17 days after his return from the journey. Urinalysis showed pyuria and hematuria. Blood examination showed hemolytic anemia, thrombocytopenia, disseminated intravascular coagulation, and hyperbilirubinemia. Plasmapheresis and hemodialysis were performed for 19 hospital days. Falciparum malaria was then confirmed by peripheral blood smear, and antimalarial medications were initiated. The patient's condition and laboratory results were quickly normalized. We report a case of severe acute renal failure associated with delayed diagnosis of falciparum malaria, and primary use of supportive treatment rather than antimalarial medicine. The present case suggests that early diagnosis and treatment is important because untreated tropical malaria can be associated with severe acute renal failure and fatality. Physicians must be alert for correct diagnosis and proper management of imported tropical malaria when patients have travel history of endemic areas.
Acute Kidney Injury* ; Adolescent ; Africa ; Anemia, Hemolytic ; Antimalarials ; Delayed Diagnosis* ; Diagnosis ; Diarrhea ; Disseminated Intravascular Coagulation ; Dysuria ; Early Diagnosis ; Fever ; Headache ; Hematuria ; Humans ; Hyperbilirubinemia ; Kenya ; Malaria* ; Male ; Nigeria ; Plasmapheresis ; Plasmodium falciparum ; Pyuria ; Renal Dialysis ; Renal Insufficiency ; Thrombocytopenia ; Urinalysis

BACKGROUND: This study was designed to determine the optimal anesthetic depth for the maintenance and recovery in interventional neuroradiology. METHODS: Eighty-eight patients undergoing interventional neuroradiology were randomly allocated to light anesthesia (n = 44) or deep anesthesia (n = 44) groups based on the value of the bispectral index (BIS). Anesthesia was induced with propofol, alfentanil, and rocuronium and maintained with 1-3% sevoflurane. The concentration of sevoflurane was titrated to maintain BIS at 40-49 (deep anesthesia group) or 50-59 (light anesthesia group). Phenylephrine was used to maintain the mean arterial pressure within 20% of preinduction values. Recovery times were recorded. RESULTS: The light anesthesia group had a more rapid recovery to spontaneous ventilation, eye opening, extubation, and orientation (4.1 +/- 2.3 vs. 5.3 +/- 1.8 min, 6.9 +/- 3.2 min vs. 9.1 +/- 3.2 min, 8.2 +/- 3.1 min vs. 10.7 +/- 3.3 min, 10.0 +/- 3.9 min vs. 12.9 +/- 5.5 min, all P < 0.01) compared to the deep anesthesia group. The use of phenylephrine was significantly increased in the deep anesthesia group (768 +/- 184 vs. 320 +/- 82 microg, P < 0.01). More patients moved during the procedure in the light anesthesia group (6/44 [14%] vs. 0/44 [0%], P = 0.026). CONCLUSIONS: BIS values between 50 and 59 for interventional neuroradiology were associated with a more rapid recovery and favorable hemodynamic response, but also with more patient movement. We suggest that maintaining BIS values between 40 and 49 is preferable for the prevention of patient movement during anesthesia for interventional neuroradiology.
Alfentanil ; Anesthesia* ; Anesthesia, General ; Arterial Pressure ; Consciousness Monitors ; Hemodynamics ; Humans ; Phenylephrine ; Propofol ; Radiology, Interventional ; Ventilation

We investigated the effect of propofol and fentanyl on microelectrode recording (MER) and its clinical applicability during subthalamic nucleus (STN) deep brain stimulation (DBS) surgery. We analyzed 8 patients with Parkinson's disease, underwent bilateral STN DBS with MER. Their left sides were done under awake and then their right sides were done with a continuous infusion of propofol and fentanyl under local anesthesia. The electrode position was evaluated by preoperative MRI and postoperative CT. The clinical outcomes were assessed at six months after surgery. We isolated single unit activities from the left and the right side MERs. There was no significant difference in the mean firing rate between the left side MERs (38.7+/-16.8 spikes/sec, n=78) and the right side MERs (35.5+/-17.2 spikes/sec, n=66). The bursting pattern of spikes was more frequently observed in the right STN than in the left STN. All the electrode positions were within the STNs on both sides and the off-time Unified Parkinson's Disease Rating Scale part III scores at six months after surgery decreased by 67% of the preoperative level. In this study, a continuous infusion of propofol and fentanyl did not significantly interfere with the MER signals from the STN. The results of this study suggest that propofol and fentanyl can be used for STN DBS in patients with advanced Parkinson's disease improving the overall experience of the patients.
Aged ; Anesthetics, Intravenous/*pharmacology ; *Deep Brain Stimulation ; Electrodes, Implanted ; Female ; Fentanyl/*pharmacology ; Humans ; Magnetic Resonance Imaging ; Male ; Microelectrodes ; Middle Aged ; Parkinson Disease/*prevention & control ; Propofol/*pharmacology ; Severity of Illness Index ; Subthalamic Nucleus/*drug effects/physiology ; Tomography, X-Ray Computed