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Purpose@#Despite enzyme replacement therapy (ERT) and/or allogeneic hematopoietic stem cell transplantation, individuals with mucopolysaccharidosis (MPS) I or II often experience significant growth deficiencies. This study aimed to assess the safety and efficacy of recombinant human growth hormone (hGH) treatment in children diagnosed with MPS I or II. @*Materials and Methods@#A total of nine pediatric patients—four with MPS I and five with MPS II—underwent treatment with ERT and hGH at Samsung Medical Center. @*Results@#The mean hGH dose administered was 0.26±0.03 mg/kg/week. In the MPS I group, three patients showed an increase in height Z-score from –4.09±0.83 to –3.68±0.43 after 1 year of hGH treatment, and to –3.10±0.72 by the end of the hGH regimen. In the MPS II group, while the height Z-score of four patients decreased according to standard growth charts, it improved from 1.61±1.79 to 2.71±1.68 based on the disease-specific growth chart through hGH treatment. Two patients discontinued hGH treatment due to lack of efficacy after 22 and 6 months each of treatment, respectively. No new-onset neurological symptoms or necessity for prosthetic or orthopedic surgery were reported during hGH treatment. @*Conclusion@#This study provides insights into the impact of hGH on MPS patients, demonstrating its potential to reverse growth deceleration in some cases. Further research is needed to explore the long-term effects of hGH on changes in body composition, muscle strength, and bone health in this population.
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Food allergy is an immune-mediated adverse reaction that occurs mainly by food ingestion. Some children with food allergies manifest fatal symptoms like anaphylaxis. Oral immunotherapy (OIT) may offer an effective therapeutic modality for persistent and severe forms of food allergies. We report our experience with OIT in 3 patients with IgE-mediated hen’s egg allergy. Our treatment strategy consists of 1–3 days of initial escalation, 47 to 65 weeks of build-up phase, and 1 year of maintenance phase. Lactobacillus plantarum CJLP133, 1×1010 colony-forming unit/day was taken during OIT. As a result, 1 patient achieved successful desensitization, and 1 patient reached maintenance therapy, but did not obtain desensitization. In addition, 1 patient withdrew from treatment due to anxiety symptoms. Despite the limited number of patients, we experienced and herein presented 3 cases of OIT in egg allergy. More trials of OIT need to be performed as a treatment option in Korean children with food allergies.
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PURPOSE: We aimed to describe the difference in the epidemiologic and clinical characteristics by age groups of the children with hand injuries requiring surgery who visited the emergency department (ED) of a community hospital that runs a hand center. METHODS: We reviewed 388 consecutive children with hand injuries requiring surgery, aged < 16 years, who visited the ED from January 2011 through September 2016. Information was obtained regarding age and gender of the children, seasonal and daily distribution of the visits, location, cause, site, and level of the injury, the diagnosis, and presence of serious injury. The children were classified into 3 age groups; toddlers (0 to 3 years), preschoolers (4 to 6 years), and schoolers (7 to 15 years). Severe injury was defined as amputation or crush injury. RESULTS: Mean age of the children was 7.4 ± 5.0 years and boys accounted for 65.7%. The most frequent visits occurred during the weekend (53.1%) and in spring (30.7%), and most children visited the ED with injuries that occurred at home and indoors (55.2% and 79.9%, respectively). The most common cause, site, and level of the injury were sharp object (34.8%), fingers other than the thumb and index finger (64.7%), and the distal phalanx (46.7%), respectively. In the toddler group, domestic, indoor, door-related, and distal phalanx injuries were more common than in the schooler group (P < 0.001). Physical contact or sharp object-related injuries increased with increasing age (P < 0.001). Severe injuries were more common among the toddlers than the schoolers (P < 0.001). CONCLUSION: In the toddler group, domestic, indoor, door-related, distal phalanx, and severe injuries were more common than in the schooler group. These characteristics by age groups would aid in preventing hand injury in children, especially toddlers.
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Criança , Humanos , Amputação Cirúrgica , Diagnóstico , Emergências , Serviço Hospitalar de Emergência , Dedos , Traumatismos da Mão , Mãos , Hospitais Comunitários , Pediatria , Estações do Ano , PolegarRESUMO
BACKGROUND: Somatostatin has been shown to offer a distinct advantage over antisecretory drugs in the management of peptic ulcer bleeding (PUB). However, rebleeding rates are still high in spite of endoscopic and medical treatment. In this study, we intended to determine whether combined therapy of a proton pump inhibitor (PPI) plus somatostatin is more beneficial than a PPI alone in patients with PUB. METHODS: We enrolled 90 consecutive patients who presented with PUB between January 2006 and October 2007. All the patients were managed with endoscopic hemostasis and divided into two treatment groups: 1) PPI alone (group A) and 2) PPI plus somatostatin (group B). The primary outcome was rebleeding within 72 hours. The secondary outcomes were rebleeding in 30 days, packed red blood cells (pRBC) transfused, length of hospital stay, need for surgery, and in-hospital mortality. RESULTS: Forty-five patients in the PPI group (A) and 45 patients in the PPI plus somatostatin group (B) were studied. There was no difference between the two groups with respect to clinical and endoscopic features at admission. After medical treatment, there was no difference between groups A and B in rebleeding at 72 hours (11% vs. 13%, p = NS), rebleeding in 30 days (13% vs. 16%, p = NS), pRBC transfused (mean, 3.2 vs. 4.5 units, p = NS), length of hospital stay (mean, 7.4 vs. 8.4 days, p = NS), and in-hospital mortality (2% vs. 7%, p = NS). CONCLUSIONS: Combined therapy with PPI and somatostatin did not result in better outcomes than PPI alone.