Allogenic Peripheral Blood Stem Cell Transplant-Induced Membranous Glomerulonephropathy as Chronic Graft-Versus-Host Disease / 대한내과학회지

Heavy proteinuria in the nephrotic range is an uncommon, often unrecognized manifestation of graft-versus-host disease (GVHD) following hematopoietic stem cell transplantation. A few isolated case reports have been published in the Korean literature involving a small number of patients who developed membranous nephropathy as GVHD after peripheral blood stem cell transplantation (PBSCT). A 17-year-old female was diagnosed with non-Hodgkin's lymphoma. Following remission, she underwent allogeneic PBSCT. Shortly thereafter, she developed acute GVHD, which was managed by medical therapy with prednisolone and cyclosporine. Approximately 13 months following PBSCT, the patient developed proteinuria without peripheral edema. Pulsed steroid therapy was initiated three times, but her condition did not improve. Twenty months after PBSCT, she developed nephrotic range proteinuria. A renal biopsy was performed, and the diagnosis was histologically consistent with membranous nephropathy. Because the response to steroids was not satisfactory, the dose of cyclosporine was increased. Approximately 3 months after renal biopsy, the proteinuria disappeared. Given the recent increase in the incidence of GVHD-mediated renal disease, in particular, renal biopsy is indispensable to the diagnosis of nephropathy and to the prevention of disease progression.

Pulmonary Arterial Hypertension is Normalized Following Six Years of Inhaled Iloprost Treatment in a Patient with Systemic Sclerosis

Pulmonary arterial hypertension is a critical manifestation of systemic sclerosis (SSc) and is a main cause of death. Several treatment modalities for SSc have been identified, with effects that improve quality of life and mortality rates. However, whether these drugs can also normalize pulmonary arterial pressure, remains unclear. Here, we report the case of a woman with diffuse SSc with pulmonary arterial hypertension, who had a functional status equivalent to the New York Heart Association class III. The patient was treated with inhaled iloprost. After six years of inhaled iloprost therapy, echocardiography showed that pulmonary arterial pressure normalized, accompanied by improvement in functional capacity. Inhaled iloprost might not only normalize pulmonary arterial pressure, but also improve the functional status of patients with SSc with pulmonary arterial hypertension.

Recurrent Upper Gastrointestinal Hemorrhage due to Hemosuccus Pancreaticus from True Splenic Artery Aneurysm / 대한내과학회지

Hemosuccus pancreaticus, defined as bleeding from the papilla of Vater via the pancreatic duct, is a rare cause of recurrent upper gastrointestinal bleeding. We report the case of a 67-year-old man with recurrent gastrointestinal bleeding, who was subsequently diagnosed with hemosuccus pancreaticus caused by rupture of a true splenic artery aneurysm. The patient had chronic pancreatitis after considerable delay and unnecessary surgical small bowel exploration. The patient was cured with distal pancreatectomy because concomitant arcuate ligament syndrome precluded the angiographic approach via the celiac trunk, and tortuous dilatation of the distal pancreatic duct could not exclude the main duct type of intraductal papillary mucinous neoplasm (IPMN). In the surgical specimen, the pancreatic duct contained a hematoma and was lined by normal epithelium, indicating rupture of the splenic artery aneurysm that bled into the pancreatic duct.

Effect of Cabergoline Treatment on Cushing's Disease Caused by Pituitary Macroadenoma after Incomplete Transsphenoidal Surgery

The present case involves a 56-year-old woman with Cushing's disease due to pituitary macroadenoma. The patient had suffered from central obesity, general weakness for 1 year. Her serum cortisol levels were elevated throughout the observation period and the dexamethasone test failed to suppress the cortisol secretion. Plasma adrenocorticotropic hormone (ACTH) levels were significantly elevated (386 pg/mL). Sellar magnetic resonance imaging revealed a 3.1-cm pituitary tumor occupying the sellar region with extension to parasellar area. The pituitary mass was removed by transsphenoidal surgery incompletely and was pathologically identified as compatible to ACTH-producing pituitary adenoma by immunohistochemistry. Thereafter, cabergoline (1 mg/wk) was administered for the remnant adenoma, which gradually reduced ACTH levels in 7 days before starting radiation therapy. This case demonstrates the efficacy of cabergoline to treat Cushing's disease caused by pituitary macroadenoma.

Re-irradiation of unresectable recurrent head and neck cancer: using Helical Tomotherapy as image-guided intensity-modulated radiotherapy

PURPOSE: Re-irradiation (re-RT) is considered a treatment option for inoperable locoregionally recurrent head and neck cancer (HNC) after prior radiotherapy. We evaluated the efficacy and safety of re-RT using Helical Tomotherapy as image-guided intensity-modulated radiotherapy in recurrent HNC. MATERIALS AND METHODS: Patients diagnosed with recurrent HNC and received re-RT were retrospectively reviewed. Primary endpoint was overall survival (OS) and secondary endpoints were locoregional control and toxicities. RESULTS: The median follow-up period of total 9 patients was 18.7 months (range, 4.1 to 76 months) and that of 3 alive patients was 49 months (range, 47 to 76 months). Median dose of first radiotherapy and re-RT was 64.8 and 47.5 Gy10. Median cumulative dose of the two courses of radiotherapy was 116.3 Gy10 (range, 91.8 to 128.9 Gy10) while the median interval between the two courses of radiation was 25 months (range, 4 to 137 months). The response rate after re-RT of the evaluated 8 patients was 75% (complete response, 4; partial response, 2). Median locoregional relapse-free survival after re-RT was 11.9 months (range, 3.4 to 75.1 months) and 5 patients eventually presented with treatment failure (in-field failure, 2; in- and out-field failure, 2; out-field failure, 1). Median OS of the 8 patients was 20.3 months (range, 4.1 to 75.1 months). One- and two-year OS rates were 62.5% and 50%, respectively. Grade 3 leucopenia developed in one patient as acute toxicity, and grade 2 osteonecrosis and trismus as chronic toxicity in another patient. CONCLUSION: Re-RT using Helical Tomotherapy for previously irradiated patients with unresectable locoregionally recurrent HNC may be a feasible treatment option with long-term survival and acceptable toxicities.

Palliative Irradiation Using Helical Tomotherapy in Recurrent Pelvic Tumors with Prior Radiotherapy / 대한방사선종양학회지

PURPOSE: We retrospectively investigated the effect of irradiation using helical tomotherapy in recurrent pelvic tumors that underwent prior irradiation. MATERIALS AND METHODS: Fourteen patients with recurrent pelvic tumors consisting of rectal cancer (57.1%), cervical cancer (35.7%) and cancer with an unknown origin (7.1%) were treated with tomotherapy. At the time of irradiation, median tumor size was 3.5 cm and 7 patients complained of pain originating from a recurrent tumor. The median radiation dose delivered to the gross tumor volume, clinical target volume, and planning target volume was 50 Gy, 47.8 Gy and 45 Gy, respectively and delivered at 5 fractions per week over the course of 4 to 5 weeks. Treatment response and duration of local disease control were evaluated using the Response Evaluation Criteria in Solid Tumors (ver. 1.0) and the Kaplan-Meyer method. Treatment-related toxicities were assessed through Common Terminology Criteria for Adverse Events (ver. 3.0). RESULTS: The median follow-up time was 17.3 months, while the response rate was 64.3%. Symptomatic improvement appeared in 6 patients (85.7%). The median duration time of local disease control was 25.8 months. The rates of local failure, distant failure, and synchronous local and distant failure were 57.1%, 21.4%, and 7.1%, respectively. Acute toxicities were limited in grade I or II toxicities, except for one patient. No treatment related death or late toxicity was observed. CONCLUSION: Helical tomotherapy could be suggested as a feasible palliative option in recurrent pelvic tumors with prior radiotherapy. However, to increase treatment effect and overcome the limitation of this outcome, a large clinical study should be performed.

Effect of Otitis Media and Cerumen Occlusion on Body Temperature Measured by Thermometers / 소아감염

PURPOSE: To examine the effect of unilateral otitis media and unilateral cerumen occlusion of the ear canal on thermometers. METHODS: One hundred eighty six children with unilateral otitis media, fifty children with unilateral cerumen occlusion, and fifty children with neither otitis media nor cerumen were enrolled. Temperature was measured in both ear canals using thermometers. After 15 minutes, second temperature was measured again in both ears. Unilateral otitis media was graded by video otoscope for 7 grades. Differences in temperatures between affected ears and unaffected ears were analyzed. RESULTS: No temperature difference between the normal and cerumen groups was observed. The mean temperature of the otitis media ear canal was 0.13+/-0.20degrees C higher than that of the intact ear canal (36.99+/-0.54degrees C vs 36.86+/-0.52degrees C; P<0.001). There was no statistically significant temperature difference between grades. CONCLUSION: Unilateral otits media can affect estimation of body temperature measured by thermometers.

Clinical Spectrum of Norovirus Gastroenteritis Compared to Rotavirus Gastroenteritis at a Single Center in Gwanju, Korea during 2005-2006; Compared to Rotaviral Gastroenteritis / 소아감염

PURPOSE:We evaluated the clinical features of Norovirus gastroenteritis compared with Rotavirus gastroenteritis in hospitalized children. METHOD:We detected causative agents in 3,261 samples of children hospitalized with gastroenteritis symptoms at a single center of pediatrics between 2005 and 2006. Among 266 and 303 samples which tested positive for Norovirus and Rotavirus, we selected 73 and 182 samples of children with relatively pure gastroenteritis symptoms and retrospectively analyzed the corresponding medical records. RESULTS:The male-to-female ratio of the Norovirus (+) and Rotavirus (+) groupswas 1.43:1 and 1.56:1 both groups were predominantly in males. The mean age of the Norovirus (+) and Rotavirus (+) groups was 36.7 and 24.4 months, respectively the children in the former group were older than the children in the latter group. The incidence in the Norovirus (+) group was more concentrated in the winter. The symptoms in the Norovirus (+), in decreasing order, included vomiting, diarrhea, and fever. The duration of vomiting, diarrhea, and fever was 2.1, 1.2, and 1.2 days. The maximum number of episodes of vomiting and diarrhea per day was 3.5 and 4.5, respectively. The severity score was 10.16. The symptoms inthe Rotavirus (+) group, in decreasing order, included diarrhea, vomiting, and fever. The duration of diarrhea, vomiting, and fever was 2.2, 4.3, and 2.2 days, respectively. The maximum number of episodes of vomiting and diarrhea per day was 3.3 and 6.5, respectively. The severity score was 11.9. The severity in the Norovirus (+) group was somewhat lower than the Rotavirus (+) group. The younger the child, the more severe the symptoms in the Norovirus (+) group. There was no difference between mono-and co-infection in severity and between the two groups regarding the hematologic findings. CONCLUSION:Based on the findings reported herein, additional studies about prophylaxis, as well as the epidemiology and clinical features of pediatric Norovirus gastroenteritis, are required.

A Case of Miller-Dieker Syndrome with Infantile Spasm and Lennox-Gastaut Syndrome / 대한소아신경학회지

Miller-Dieker syndrome is a contiguous gene deletion syndrome involving chromosome 17p13.3, which is characterized by type 1(classical) lissencephaly and typical craniofacial abnormalities. Children with Miller-Dieker syndrome have profound psychomotor retardation, seizures that often are intractable, chronic feeding problems that lead to recurrent pneumonia, and shortened lifespan. We have experienced a Miller-Dieker syndrome female who has lived to 8years, showing severe mental and motor retardation and intractable epilepsy. She was diagnosed as Miller-Dieker syndrome in the neonatal period, showing typical facial features, type 1 lissencephaly, and chromosome 17p13.3 microdeletion in fluorescence in situ hybridization. Infantile spasm occurred at 4 months of age and progressed to Lennox-Gastaut syndrome at 3 years and 6 months, both of which were not controlled by antiepileptic drugs.

A case of neonatal major burn by electrical heating pad / 대한주산의학회잡지

Burn in neonates have been reported following the use of pulse oximeters, phototherapy blanket, infrared heating lamp, laryngoscope, and warming bottle. This case reports a newborn who had major burn injuries of 45% total body surface area (TBSA) including 3rd degree burns of 20% TBSA on her back, buttocks, both thighs and heels by exposure to an electrical heating pad for 3 hours. She developed significant systemic response, showing disseminated intravascular coagulation, electrical imbalance, jaundice, hypoalbuminemia, acute renal failure, and persistent pulmonary hypertension of the newborn. The potential hazard of the electric heating pad is reported in order to alert clinicians to this specific risk, to stimulate concern about other similar problems with materials in contact with skin, and to prevent burn of newborns in the neonate unit.

Radiation Therapy Combined with (or without) Cisplatin-based Chemotherapy for Patients with Nasopharyngeal Cancer: 15-years Experience of a Single Institution in Korea / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: This retrospective study was carried out to evaluate the efficacy and toxicity of radiation therapy (RT) with/without cisplatin-based chemotherapy in nasopharyngeal cancer (NPC). MATERIALS AND METHODS: One hundred forty six patients with NPC received curative RT and/or cisplatin-based chemotherapy. Thirty-nine patients were treated with induction chemotherapy (IC), including cisplatin and 5-fluorouracil followed by RT. Another 63 patients were treated with concurrent chemoradiotherapy (CCRT) using cisplatin, and 22 patients were treated with IC followed by CCRT. The remaining 22 patients were treated with RT alone. RESULTS: One hundred four (80.0%) patients achieved complete response (CR), and 23 (17.7%) patients achieved partial response (PR). The patterns of failure were: locoregional recurrences in 21.2% and distant metastases in 17.1%. Five-year overall survival (OS) and progression free survival (PFS) were 50.7% and 45.0%, respectively. Multivariate Cox stepwise regression analysis revealed CR to chemoradiotherapy to be a powerful prognostic factor for OS. CR to chemoradiotherapy and completion of radiation according to the time schedule were favorable prognostic factors for PFS. A comparison of each treatment group (IC --> RT vs. CCRT vs. IC --> CCRT vs. RT alone) revealed no significant differences in the OS or PFS. However, subgroup analysis showed significant differences in both OS and DFS in favor of the combined chemoradiotherapy group compared with RT alone, for stage IV and T3-4 tumors. Grade 3-4 toxicities were more common in the combined chemoradiotherapy arm, particularly in the CCRT group. CONCLUSIONS: This study was limited in that it was a retrospective study, much time was required to collect patients, and there were imbalances in the number of patients in each treatment group. Combined chemoradiotherapy remarkably prolonged the OS and PFS in subgroup patients with stage IV or T3-4 NPC.

A Case of Prader-Willi Syndrome with an Unusually Large 15q Deletion Due to an Unbalanced Translocation to Chromosome 2 45,XX,-15, der(2) t(2;15)(q37:q13)

Prader-Willi syndrome is a disease of chromosome 15, which is characterized by severe hypotonia and feeding difficulty in neonates, followed by development of obesity, mental retardation, and hypogonadism. Approximately 70% of the patients have a paternal deletion on chromosome 15q11-13, which is mainly a microdeletion, and a large deletion due to an unbalanced structural translocation of the proximal long arm of chromosome 15 to several other chromosomes is rarely found. We encountered a neonatal case with Prader-Willi syndrome who had sustained hypotonia and feeding difficulty. On high-resolution chromosome analysis, deletion of the short arm and the proximal part of the long arm of chromosome 15, with unbalanced translocation of the remaining part of chromosome 15(q13-qter) to the terminal part q37 of chromosome 2, was shown to be . Through FISH (Fluorescence in situ hybridization) and methylation-specific DNA PCR, we confirmed the deleted q11-13 was derived from the father.

A Familial Case of Idiopathic Hypogonadotropic Hypogonadism Occurred in a Boy and His Sister / 대한소아내분비학회지

Isolated gonadotropin deficiency can be idiopathic or a part of X-linked Kallmann syndrome associated with anosmia. There have been several trials to reveal the genetic mutations that affect gonadotropin secretion, and approximately 10% of sporadic patients have mutations in either gonadotropin releasing hormone receptor (GnRHR) or KAL1 gene. Here we report one familial cases of idiopathic hypogonadotropic hypogonadism occurred in a boy and his elder sister. They presented with delayed puberty and hypoplastic gonads, but normal sense of smell. We performed GnRHR and KAL1 mutation analysis, but could not find any mutation.

Comparison of Clinical Efficacy of Newfactan(R) versus Surfacten(R) for the Treatment of Respiratory Distress Syndrome in the Newborn Infants

Newfactan(R) is a domestically developed, bovine lung-derived, semi-synthetic surfactant. The aim of this study was to compare the clinical efficacy of Newfactan(R) with that of Surfacten(R) in the treatment of respiratory distress syndrome (RDS). Newfactan(R) or Surfacten(R) was randomly allocated to 492 newborn infants who were diagnosed as RDS and required surfactant instillation in four participating hospitals. The comparisons were made individually in two subsets of infants by birth weight (or=1,500 g group [n=239]). Short-term responses to surfactant and acute complications, such as the total doses of surfactant instilled, response type, extubation rate, ventilator settings, changes in respiratory parameters, air leak, patent ductus arteriosus, pulmonary hemorrhage, and intraventricular hemorrhage, and mortality during the 96 hr after surfactant instillation were measured. Long-term outcome and complications, such as total duration of intubation, bronchopulmonary dysplasia and periventricular leukomalacia, and ultimate mortality were measured. There were no significant differences in demographic and perinatal variables, shortterm responses to surfactant and acute complications, and long-term outcome and complications between Newfactan(R) and Surfacten(R) in both birth weight groups. We concluded that Newfactan(R) was comparable to Surfacten(R) in the clinical efficacy in the treatment of RDS in both birth weight groups.

Extubation Time by Birth Weight and the Predictors for Success/Failure at the First Extubation in Extremely Low Birth Weight Infants / 소아과

PURPOSE: To outline the aspects of extubation by birth weight and find the predictors for success/ failure at the first extubation in extremely low birth weight infants. METHODS: One hundred thirteen extremely low birth weight infants(< 1,000 g) who were admitted to NICU at Samsung Seoul Hospital between Jan. 2000 and Jun. 2004 were enrolled. Clinical characteristics that are thought to be related with extubation success or failure were compared with the success and the failure of the first extubation. RESULTS: As the birth weight decreased, extubation success day was significantly delayed:16+/-3 day(d) in 900-999 g; 20+/-3 d in 800-899 g; 35+/-4 d in 700-799 g; 37+/-9 d in 600-699 g; 49+/-12 d in < or =599 g. 25 out of 113 infants(22%) failed the first extubation. Preterm premature rupture of membrane was associated with extubation success, and air leak was associated with extubation failure, with a borderline significance. Postnatal and corrected age and body weight at the first extubation, nutritional status, and ventilator settings were not associated with extubation success or failure. Extubation success day was significantly delayed, and the incidence of late-onset sepsis and mortality was significant higher in the failure of the first extubation. CONCLUSION: We could not find significant predictors for success/failure at the first extubation. The failure of the first extubation had an increased risk of late-onset sepsis and death. Further studies are needed to find the predictors for extubation success/failure.

A Study of Predictive Factors of Treatment Response to Sertraline in Patients with Obsessive-Compulsive Disorder / 신경정신의학

OBJECTIVES: The pharmacologic treatment of obsessive-compulsive disorder(OCD) with serotonergic antidepressant agents is well established in clinical psychiatry on the basis of serotonin hypothesis. Studies on predictive factors of treatment response to OCD are relatively scarce. The author compared the demographic factors, age of onset, length of illness, family history of mental illness, previous drug history for OCD, severity and type of symptoms between the drug responders and non-responders to find out the predicitive factors that may be related to treatment response. METHODS: The subjects were 31, both inpatients and outpatients who met the diagnostic criteria for OCD by DSM-IV and scored 20 or higher on the Yale-Brown Obsessive-Compulsive Scale(Y-BOCS) completed 12-week trial of sertraline. Y-BOCS, National Institute of Mental Health Obsessive-Compulsive Scale(NIMHOCS), Clinical Global Impression Scale(CGI) were scored to measure the changes of obsessive-compulsive symptoms, and Hamilton Anxiety Scale(HAM-A) was measured at each visit. RESULTS: 1) The responders, defined as achieving at least a 40% decrease in the Y-BOCS total score and more than a 2 point decrease in CGI score, were 17(54.8%), and non-responders were 14(45.2%). Responders tended to show later onset(p<0.1), and shorter duration of illness(p<0.05), and less previous drug therapy for OCD(p<0.05) than non-responders. There were no significant differences in family history of mental illness between the two groups. 2) The baseline Y-BOCS compulsive score was significantly lower in responders(p<0.05), although there was no significant difference in the baseline severity of other measures. 3) The obsession of contamination and washing behaviors were more frequent, but compulsive counting was not shown, in responders(p<0.05). The pathological doubt tended to be more common in nonresponders(p<0.1). CONCLUSIONS: In summary, the patients with shorter duration of illness, less prior drug therapy for OCD, and less severe compulsive behaviors, and the obsession of contamination and washing behaviors are expected to be better outcome with sertraline, whereas compulsive counting are the predictor of poor response.

Study on the Body Temperature Measuring Time and Accuracy and Reliability of Tympanic Thermometer / 기본간호학회지

This study was to investigate the method for shortening the body temperature(BT) because it takes a long time and is impractical to measure axillary or oral BT with mercury thermometer. The first approach was to identify BT change according to the measuring time and determine the clinically not statistically available and optimal BT measuring time. The second was to test the accuracy of tympanic thermometer. It can measure BT within a few seconds, so if it is approved accurate, we can save BT measuring time by substitute tympanic thermometer for mercury thermometer. This study was conducted from 1, to 30 June, 1996. The subjects were 12 men students of medical college and 29 women students of nursing school. The results were as follows ; 1) The 3, 5, 7, 9, 11, 13 minute-measured axillary BT and 3, 5, 7, minute-measured BT showed somewhat linear relationship with time. It was difficult to find the optimum measuring time which were clinically significant. 2) For axillary temperature, the measuring time which were not statistically different was 11 and 13 minute. But the real BT difference between 3 and 13 minute, or between 5 and 13 minute were very small and was within the range of daily variation. 3) For oral temperature, there was no intervals which showed the statistically insignificant. But like as axillary temperature, the difference between 3 and 7, or 5 and 7 minute were trivial by 0.3degrees C and by 0.1degrees C respectively. 4) tympanic temperatures were lower than oral BTs which were measured with mercury thermometer by 0.26degrees C(with ear tug) and 0.15degrees C(without ear tug). 5) The reliability of repeated measure tympanic temperature was better than without ear tug. With above results, we can't determine the optimal and clinically significant oral and axillary measuring time using mercury thermometer. However, because the real differences between measuring times were very small, so we recommend further study for the aged, the infants and the febrile patients. And we can't sure the accuracy of tympanic temperature but the reliability was better with ear tug than without ear tug.