Predictive factors for early response to methimazole in children and adolescents with Graves disease: a single-institute study between 1993 and 2013

PURPOSE: We aimed to investigate the predictive factors for early response to methimazole (MMI) in pediatric patients with Graves disease (GD). METHODS: Our study included 44 pediatric patients who were diagnosed with GD between January 1, 1993, and December 31, 2013, and were available for follow-up, achieving a normalization of thyroid functions (TFs) at the Chonbuk National University Hospital Pediatric Department. We retrospectively analyzed TFs such as tri-iodothyronine (T3), free thyroxine (fT4), thyroid-stimulating hormone (TSH), and thyroid antibody levels at diagnosis. We also examined their family history of thyroid disease, symptoms at presentation, and normalization time for TF after treatment. We divided our clinical series of patients into the following 4 age groups: <7 years old, 7-12 years old, 13-15 years old, and 16-18 years old. RESULTS: At diagnosis, the time of normalization of T3 was significantly shorter in the higher antimicrosomal antibody (AMA) group compared with the lower AMA group (2.53 months vs. 6.18 months) (P<0.05). However, the time of normalization of T3/fT4/TSH had no significant correlations with other variables such as age, sex, a family history of thyroid diseases, thyroglobulin, thyroid-stimulating immunoglobulin, or antithyroglobulin antibody (ATA). CONCLUSION: Higher serological titers of AMA at diagnosis may have prognostic value in the response to initial MMI treatment in pediatric hyperthyroid GD patients.

Predictive factors for early response to methimazole in children and adolescents with Graves disease: a single-institute study between 1993 and 2013

PURPOSE: We aimed to investigate the predictive factors for early response to methimazole (MMI) in pediatric patients with Graves disease (GD). METHODS: Our study included 44 pediatric patients who were diagnosed with GD between January 1, 1993, and December 31, 2013, and were available for follow-up, achieving a normalization of thyroid functions (TFs) at the Chonbuk National University Hospital Pediatric Department. We retrospectively analyzed TFs such as tri-iodothyronine (T3), free thyroxine (fT4), thyroid-stimulating hormone (TSH), and thyroid antibody levels at diagnosis. We also examined their family history of thyroid disease, symptoms at presentation, and normalization time for TF after treatment. We divided our clinical series of patients into the following 4 age groups: <7 years old, 7-12 years old, 13-15 years old, and 16-18 years old. RESULTS: At diagnosis, the time of normalization of T3 was significantly shorter in the higher antimicrosomal antibody (AMA) group compared with the lower AMA group (2.53 months vs. 6.18 months) (P<0.05). However, the time of normalization of T3/fT4/TSH had no significant correlations with other variables such as age, sex, a family history of thyroid diseases, thyroglobulin, thyroid-stimulating immunoglobulin, or antithyroglobulin antibody (ATA). CONCLUSION: Higher serological titers of AMA at diagnosis may have prognostic value in the response to initial MMI treatment in pediatric hyperthyroid GD patients.

Prevalence of orofacial clefts in Korean live births

OBJECTIVE: The aim of this study was to investigate the prevalence of orofacial clefts and identify the characteristics of other birth defects associated with orofacial clefts in Korea. METHODS: This study used data from the Congenital Anomaly Survey conducted by the Korea Institute for Health and Social Affairs. The survey was conducted on birth defects documented during 2005 to 2006 in 2,348 medical institutes in Korea. This study was performed using data from medical insurance claims of the National Health Insurance Corporation. The prevalence of orofacial clefts was defined as the number of cases per 10,000 live births. RESULTS: Among the 883,184 live births, 25,335 infants had birth defects, which included 980 infants with orofacial clefts. The prevalence of total orofacial clefts in the total live births was 11.09 per 10,000, accounting for 3.9% of all birth defects. The most common orofacial cleft was cleft palate only (n=492), followed by cleft lip only (n=245) and cleft lip with cleft palate (n=243), with prevalence rates of 5.57, 2.77, 2.75 per 10,000 live births, respectively. While malformations of the circulatory system; digestive system; eyes, ears, face, and neck; and musculoskeletal system were most frequently encountered among infants with a cleft lip with or without a cleft palate, anomalies of most organ systems were notably observed among infants with cleft palate only. CONCLUSION: The prevalence of orofacial clefts in Korea was similar or slightly lower than that of other countries. This study informs present status of orofacial clefts and gives baseline data to lay the foundation stone for Korea's registry system of orofacial clefts.

Clinical features of thoracic endometriosis: A single center analysis

OBJECTIVE: To analyze the diagnostic profiles and treatment outcomes of patients with thoracic endometriosis at a university hospital. METHODS: A retrospective review of medical records was performed for patients diagnosed with thoracic endometriosis at Gangnam Severance Hospital, Yonsei University College of Medicine, between January 2007 and January 2014. RESULTS: Fifteen patients (median age, 35 years; range, 23-48 years) were evaluated. Patients presented with catamenial hemoptysis (n=8), or catamenial pneumothorax (n=7). Patients with catamenial pneumothorax were significantly older than those presenting with hemoptysis (P=0.0002). Only 3 patients (20%) had coexisting pelvic endometriosis. All patients underwent chest computed tomography; lesions were shown to predominantly affect the right lung (right lung, n=13, 86.7%; left lung, n=2, 13.3%), and were mainly distributed on the right upper lobe (n=9, 60%). Ten patients underwent video-assisted thoracoscopic surgery, and 1 patient underwent a thoracotomy. Intraoperatively, endometriosis-specific findings were observed in 8/11 patients (72.7%); a further 5/11 patients (45.4%) had histologically detectable endometriosis. Over the follow-up period (mean, 18.4 months; range, 2-65 months) 5/15 patients (33%) had clinical signs of recurrence. Recurrence was not detected in any of the 5 catamenial pneumothorax patients that received adjuvant hormonal therapy after surgery. CONCLUSION: The diagnosis and management of thoracic endometriosis requires a multidisciplinary approach, based upon skillful differential diagnosis, and involving careful gynecologic evaluation and assessment of the cyclicity of pulmonary symptoms. Imaging findings are non-specific, though there may be laterality towards the right lung. Since symptom recurrence is more common in those with presenting with pneumothorax, post-operative adjuvant medical therapy is recommended.

Single-port access versus conventional multi-port access total laparoscopic hysterectomy for very large uterus

OBJECTIVE: The aim of this study was to compare the surgical outcomes of single-port access (SPA) and conventional multi-port access total laparoscopic hysterectomies (TLH) among patients with very large uteri (500 g or more). METHODS: Fifty consecutive patients who received TLH for large uterine myomas and/or adenomyoses weighing 500 g or more between February 2009 and December 2012 were retrospectively reviewed. SPA and conventional TLH were each performed in 25 patients. Surgical outcomes, including operation time, estimated blood loss, postoperative hemoglobin change, postoperative hospital stay, postoperative pain, and perioperative complications, were compared between the two groups. RESULTS: There were no significant demographic differences between the two groups. All operations were completed laparoscopically with no conversion to laparotomy. Total operation time, uterus weight, estimated blood loss, and postoperative hemoglobin change did not significantly differ between the two groups. Postoperative hospital stay was significantly shorter for the SPA-TLH group compared to that of the conventional TLH group (median [range], 3 [2.0-6.0] vs. 4 [3-7] days; P=0.004]. There were no inter-group differences in postoperative pain at 6, 24, and 72 hours after surgery. There was only one complicated case in each group. CONCLUSION: SPA-TLH in patients with large uteri weighing 500 g or more is as feasible as conventional TLH. SPA-TLH is associated with shorter hospital stays compared to that of conventional TLH.

Osteopetrosis Associated with Hydrocephalus and Rickets: A Case Report / 대한소아신경학회지

Osteopetrosis or albers-Schonberg disease is extremely rare disease. It is characterized by osteoporosis, stunted growth, deformity, increased likelihood of fractures, also patients suffers anemia, recurrent infections and hepatosplenomegaly. However, we recently came upon a 14-month-old female as the 1st child of osteopetrosis with hydrocephalus and rickets. She has the typical symptoms such as nystagmus, osteosclerosis -especially in skull. Brain Magnetic Resonance Imaging (MRI), MRI shows hydrocephalus and x-ray finding are consistent with rickets. This is the first report of osteopetrosis with hydrocephalus and rickets in Korea by pediatrician.