Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses / 소아과

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-Barre syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.

A Case of Eosinophilic Fasciitis Presenting as Pitting Edema of the Lower Extremities

Eosinophilic fasciitis is a rare disease characterized by diffuse fasciitis with peripheral eosinophilia and progressive induration and thickening of the skin and soft tissues. We report a 19-year-old female who presented with pitting edema in both lower extremities. She had a history of excessive physical activity before her symptoms developed. Physical examination revealed 2+ pitting edema in both lower legs. She complained of mild pain in both knee joints and feet, with no tenderness or heating sensations. Laboratory results were unremarkable except for severe eosinophilia. Parasite infection, venous thrombosis, and cardiac and renal problems were excluded. A magnetic resonance imaging study of both lower extremities revealed increased signal intensity in the subcutaneous lesions, consistent with superficial inflammation of the fascia. Mixed perivenular lymphoplasmacytic and eosinophilic infiltration in the subcutaneous lesion were observed on biopsy. The patient was treated with corticosteroids, resulting in remarkable improvement in both edema and eosinophilia.

Use of Continuous Positive Airway Pressure in Obstructive Sleep Apnea Syndrome / 대한소아신경학회지

Obstructive sleep apnea syndrome (OSAS) is a disorder of breathing during sleep characterized by prolonged partial upper airway obstruction or intermittent complete obstruction that disrupts normal ventilation during sleep and normal sleep patterns. Untreated OSAS can cause various problems such as cognitive deficit, attention deficit hyperactivity disorder, hypertension, cardiovascular disease, failure to thrive and mood disorder. Therefore, early diagnosis and proper management is very important. The gold standard of diagnosis of OSAS is overnight polysomnography. Treatment includes the use of continuous positive airway pressure (CPAP) and weight loss in obese children. Here, we present an obese 15-year-old boy presented with obstructive sleep apnea and attention deficit. He was diagnosed with OSAS by polysomnography and was successfully treated with CPAP.

The safety of influenza vaccination in children with egg allergy

PURPOSE: Although influenza vaccine contains some residual egg protein (ovalbumin), recent studies have been reported that the influenza vaccine is even safe for patients with egg allergy. The object of this study was to assess the safety of influenza vaccination and estimate the risk factors of allergic reactions to influenza vaccination in children with egg allergy. METHODS: The medical records of 108 children were reviewed retrospectively, those were diagnosed as egg allergy at Department of Pediatrics in Severance Children's Hospital between January 2006 and December 2011. All of them were vaccinated with very low ovalbumin concentration (< or =0.12 microg/mL). Patients were vaccinated in graded doses by the international guideline. Subjects without allergic reactions to influenza vaccine among egg allergy patients were recruited as control subjects. RESULTS: Only 12 subjects of patients had adverse reactions to influenza vaccination. There were no significant relationships between adverse reactions to influenza vaccine and their own history of other allergy, history of breast feeding or graded vaccination. The egg allergy symptoms or egg-specific IgE levels were not associated with adverse reactions to influenza vaccination. CONCLUSION: These results show that patients with egg allergy may have adverse reactions with influenza vaccine, but severe adverse reactions are rare as general population. Therefore, the patients with egg allergy can be safe vaccinated with influenza vaccine, regardless of severity of allergic reaction after egg ingestion or methods of vaccination.

Effects of mixture of fibrin-fibronectin sealant system and calcuim carbonate in periodontal intrabony defects / 대한치주과학회지

Calcium carbonate(CC) is biocompatible and gradually absorb to be replaced by bone when implanted into bone tissue. Fibrin-fibronectin sealant system (FFSS) is a product of human-derived plasma. The effect is hemostasis, tissue fixation and adhesion, We expect synergic effects of this two materials in periodontal regeneration. When FFSS was grafted with bone graft in intrabony defects, could be eliminated exofolication of bone graft materials. This study evaluated above materials for periodontal regeneration of 6mm intrabony defects in 36 patients. Flap surgery was carried in 14 defects of control group. experimental group 1 was 11 defects grafted with calcium carbonate, experimental group 2 was 11 defects which were grafted with calcium carbonate with FFSS. The clinical parameters evaluated included changes in attachment level, probing depth, gingival recession at 6 months. Postsurgery probing depth reduction was 3.1 +/- 0.9mm in control, 3.8 +/- 1.6mm in experimental group 1, 4.1 +/- 1.1mm in experimental group 2. The result clinically and statistically improved compared to baseline(P<0.01), but the difference found among the groups were not statistically significant. Postsurgery clinical attachment level was 1.6 +/- 1.2mm in control, 3.5 +/- 2.0mm in experimental group 1, 3.3 +/- 1.2mm in experimental group 2. All of the control and experimental groups resulted in a statistically significant reduction from baseline(P<0.01). The reduction of the experimental groups were statistically significant from control(P<0.05). But the change between experimental group 1 and experimental group 2 was not statistically significant. We conclude that mixture of CC and FFSS is effective to periodontal regeneration in intrabony defect.