• Title/Summary/Keyword: recurrent disease

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Efficacy of conservative treatment of perianal abscesses in children and predictors for therapeutic failure

  • Boenicke, Lars;Doerner, Johannes;Wirth, Stefan;Zirngibl, Hubert;Langenbach, Mike Ralf
    • Clinical and Experimental Pediatrics
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    • v.63 no.7
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    • pp.272-277
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    • 2020
  • Background: The optimal management of perianal abscess in children is controversial. Purpose: To evaluate the efficiency of conservative treatment of perianal abscess in children and identify parameters that predict therapy failure. Methods: All cases of children younger than 14 years of age with perianal abscesses between 2001-2016 were evaluated. Results: Of the 113 enrolled patients, 64 underwent subsequent surgery for advanced disease (primary surgery group). Conservative treatment was initiated in 49 patients (primary conservative group) but was stopped because of inefficiency in 25 patients, who were referred for surgery after a median 7.03 days (range, 2 to 16 days). The other 24 patients (48%) initially achieved complete remission after conservative treatment, but 10 were readmitted after a median 34 months (range, 3 to 145 months) with recurrent disease. There were no significant differences in permanent success after conservative treatment between infants (10 of 29, 34%) and older children (4 of 20 [20%], P=0.122). Overall, conservative treatment alone was effective in only 14 of 113 patients. Recurrence after surgery occurred in 16 patients (25%) in the primary surgery group and 11 patients (22%) in the primary conservative group (P=0.75). Univariate analysis of predictors for conservative treatment failure revealed inflammatory values (C-reactive protein and white blood count, P=0.017) and abscess size (P=0.001) as significant parameters, whereas multivariate analysis demonstrated that only abscess size (odds ratio, 3.37; P=0.023) was significant. Conclusion: Conservative treatment of perianal abscess is permanently efficient in only a minority of children but is not associated with a higher recurrence rate after subsequent surgery. Abscess size is a predictor for therapy failure.

Congenital central hypoventilation syndrome combined with Hirschsprung disease diagnosed in the neonatal period (신생아에서 진단된 Hirschsprung 병을 동반한 congenital central hypoventilation syndrome 1례)

  • Choi, Jin Hyun;Oh, Jin Hee;Kim, Jong-Hyun;Koh, Dae Kyun;Hong, Seung-Chul
    • Clinical and Experimental Pediatrics
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    • v.49 no.4
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    • pp.446-450
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    • 2006
  • Congenital central hypoventilation syndrome (CCHS) or Ondine's curse is a very rare sleep disorder that is the result of a congenital failure of the autonomic control of ventilation caused by insensitivity of the chemoreceptor to hypercapnea during sleep. Gastrointestinal motility disorders, particularly a congenital megacolon (Hirschsprung disease) is often combined with CCHS. This combination can be explained by a defect in the migration of neuronal cells from the neural crest (neurocristopathy) during the intrauterine period. A diagnosis of CCHS is made by confirming the failure of adequate ventilation in response to hypercapnea and hypoxia during sleep and the exclusion of other diseases. Young infants frequently show atypical clinical courses, and their conditions are frequently complicated with the long-term sequela of hypoxemic episodes. Therefore, a high index of suspicion and active treatment with mechanical ventilation are important for reducing recurrent hypoxemic episodes in the neonatal period. This paper reports the follow up of a case of CCHS in a neonate who showed frequent intractable apnea and cyanosis and was given artificial mechanical ventilation during sleep.

A Case of Recurrent Herpes Simplex Virus Disease of a Preterm Infant, Who Needed Continuous Oral Acyclovir Suppressive Therapy (지속적인 경구용 Acyclovir 억제요법이 요구된 미숙아의 재발성 단순포진 바이러스 감염 1례)

  • Kim, Sung Seok;Hong, Ki Woong;Kim, Eun Ryoung;Kim, Young-Don;Lee, Kyoo Man
    • Clinical and Experimental Pediatrics
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    • v.46 no.9
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    • pp.939-943
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    • 2003
  • Neonatal herpes simplex virus(HSV) infections result in significant morbidity and mortality. Although acyclovir treatment has improved survival, severe neurological sequelae can occur in the majority of survivors. HSV infections limited to the skin, eyes and mouth(SEM) can cause neurologic impairment. A direct correlation exists between the development of neurologic deficits and the frequency of cutaneous HSV recurrences. National Institutes of Allergy and Infectious Diseases(NIAID) Collaborative Antiviral Study Group conducted a phase I/II trial of continuous oral acyclovir therapy for the suppression of cutaneous recurrences. We describe a preterm infant who had two recurrences after neonatal SEM disease had been treated with intravenous acyclovir, and there were no more recurrences after continuous oral acyclovir suppressive therapy for six months. We report this case with a review of related literature.

Pseudoprogression and Pseudoresponse in the Management of High-Grade Glioma : Optimal Decision Timing According to the Response Assessment of the Neuro-Oncology Working Group

  • Chang, Ji Hyun;Kim, Chae-Yong;Choi, Byung Se;Kim, Yu Jung;Kim, Jae Sung;Kim, In Ah
    • Journal of Korean Neurosurgical Society
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    • v.55 no.1
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    • pp.5-11
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    • 2014
  • Objective : We evaluated pseudoprogression (PsPD) following radiation therapy combined with concurrent temozolomide (TMZ), and we assessed pseudoresponse following anti-angiogenic therapy for patients with recurrent disease using the Response Assessment of the Neuro-Oncology Working Group. Methods : Patients who were pathologically confirmed as having high-grade glioma received radiotherapy with concurrent TMZ followed by adjuvant TMZ. Bevacizumab (Avastin) with CPT-11 were used as a salvage option for cases of radiologic progression. Magnetic resonance imaging (MRI) was routinely performed 1 month after concurrent radiochemotherapy (CRT) and every 3 months thereafter. For cases treated with the bevacizumab-containing regimen for progressive disease, MRI was performed every 2 months. Results : Of 55 patients, 21 (38%) showed radiologic progression within 4 weeks after CRT. Of these patients, 16 (29%) showed progression at second post-CRT MRI (etPD) and five (9%) showed improvement (PsPD). Seven of thirty-four initially non-progressed patients showed progression at the second post-CRT MRI (ltPD). No difference in survival was observed between the etPD and ltPD groups (p=0.595). Five (50%) of ten patients showed a radiological response after salvage bevacizumab therapy. Four of those patients exhibited rapid progression immediately after discontinuation of the drug (drug holiday). Conclusion : Twelve weeks following treatment could be the optimal timing to determine PsPD or true progression. MRI with gadolinium enhancement alone is not sufficient to characterize tumor response or growth. Clinical correlation with adequate follow-up duration and histopathologic validation may be helpful in discriminating PsPD from true progression.

Clinical Study of Stroke Type (뇌졸중(腦卒中) 환자(患者) 형태(形態)에 관(關)한 임상연구(臨床硏究))

  • Youn, Hyoun-min;Ahn, Chang-beohm;Song, Choon-ho;Son, In-seok;Jang, Kyung-jeon
    • Journal of Acupuncture Research
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    • v.20 no.2
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    • pp.29-41
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    • 2003
  • Clinical observation was made on 52 cases of Stroke that were confined through brain CT, MRI scan. The Stroke cases wee classified into the following kinds cerebral infarction, cerebral hemorrhage, cerebellar or brain stem infarction, cerebellar or brain stem hemorrhage. And among the 52 cases of Stroke cerebral infarction was noticed in 75.00%, cerebral hemorrhage in 11.54%, cerebellar or brain stem infarction in 9.52%, cerebellar or brain stem hemorrhage in 3.85%. The ratio between males and females was 1.74:1 in the whole groups of Stroke and most cases were over 60 of age. As the time of hospitalization, most patients hospitalized from 1 day after stroke to 7 days after stroke. And as the course of hospitalization, most patients hospitalized first. Among the preceding disease at the onset of Stroke hypertention was noted in 32.69%, and deabetes mellitus or heart problem was noted frequently(15.39%). Electrocardiography findings were as follows: The normal was noted in 53.85%, the abnormal in 46.15%. And as the abnormal, left ventricular hypertrophy was noted in 17.54%. The predisposing factors or conditions at the onset of brain infarction were usually initiated during the time of sleeping and those of brain hemorrhage chiefly during the time of exercising like overwork or walking etc. It was noted that smoking a pack of cigarette showed highest disease rate(33.33%) among the average of smoking amount of one day in case of man. Prior to attack, the most chiefly complain was dyspnea or discomfort on chest region. And 30.70% of patients had no previous sign. There were a large number of recurrent cases. The first attack was noted in 71.15%, the 2nd attack in 23.08%, the 3rd attack in 5.77%.

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A Case of Pulmonary Aspergilloma in Bronchogenic Cyst Associated with An Actinomycosis (흉부 방선균증이 동반된 기관지 기원 낭내 폐 국균종)

  • Kim, Gun Hyun;Kim, Kwang Hyun;Kim, Min Seon;Park, Jae Eun;Kim, Dae Jin;Son, Hyuk Su;Kim, Yeon Jae;Lee, Byung Ki;Huh, Dong Myung;Gu, Mi Jin
    • Tuberculosis and Respiratory Diseases
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    • v.57 no.6
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    • pp.584-588
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    • 2004
  • Pulmonary aspergilloma usually arises in preexisting lung cavities characterized by recurrent hemoptysis. Although surgical resection of the aspergilloma is the best treatment, most patients are poor candidates for surgery because of far-advanced underlying pulmonary disease. On the other hand, pulmonary actinomycosis is a chronic, indolent bacterial infection and follows aspiration of oropharyngeal material. Bronchiectasis and obstructive lung disease are often associated underlying conditions. We report a case of pulmonary aspergilloma in bronchogenic cyst associated with an actinomycosis in 21-year-old woman treated by thoracoscopic surgery with a review of literature.

Surgical treatment of recurrent pseudochylothorax occurring after therapy of tuberculous pleurisy (결핵성 흉막염 치료 후 반복되는 가성유미흉의 수술적 치료)

  • Yi, Jae Ryung;Kim, Woo Sik;Jeong, Eun Jung;Jung, Yu Na;Lee, Hee Sook;Jo, Gi Ho;Lee, Ji Yeon
    • Journal of Yeungnam Medical Science
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    • v.31 no.1
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    • pp.65-68
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    • 2014
  • Pseudochylothorax is an uncommon pleural effusion disease characterized by the presence of cholesterol crystals or high lipid content not resulting from a disrupted thoracic duct. Most of the cases reported so far had been found in patients with long-standing pleural effusion due to a chronic inflammatory disease such as old tuberculous pleurisy or chronic rheumatoid pleurisy. Authors encountered a case of pseudochylothorax in a 45-year-old man who had been treated for tuberculous pleurisy 6 years before his visit to authors' hospital. After that, he had visited the emergency department many times for removal of pleural effusion. The patient's chest X-ray revealed dyspnea and large left-sided pleural effusion. Although a large amount of pleural fluid was removed with a drainage catheter, massive pleural effusion was likely to recur, and the underlying lung was able to fully re-expand. Accordingly, decortication was done, and the patient's symptom was improved without postoperative complications.

Deep Tissue Invasion of Dermatofibrosarcoma Protuberance (융기성 피부섬유육종의 심부 침습정도에 대한 고찰)

  • Kim, Kyoung-Hoon;Bae, Yong-Chan;Nam, Su-Bong;Choi, Soo-Jong;Kang, Cheol-Uk
    • Archives of Plastic Surgery
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    • v.36 no.4
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    • pp.417-421
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    • 2009
  • Purpose: Dermatofibrosarcoma protuberans(DFSP) is a moderate - degree malignant tumor of soft tissue from dermis to fat layer with high recurrences(11% to 73%) due to its local infiltrative characteristic. Many debates and controversies in deciding accurate surgical margin were presented before, but references about depth of invasion and appropriate surgical excision level were not properly made out. Therefore, we tried to identify the degree of tissue invasion of DFSP. Methods: Twenty patients, including 8 patients with recurrent lesions, over last 10 years were reviewed retrospectively. Different surgical margins were applied according to the location and based on histopathologic result, we have defined as a 'deep tissue invasion' if there were infiltration of tumor cell into fascia or underlying muscle layer was present. All invaded tissue including dermis, fat, fascia and muscle were excised until no tumor cell was found during intraoperative frozen section biopsy. And comparative analysis of deep tissue invasion according to age, primary site, duration of disease and recurrence was done. Results: Thirteen patients(65%) showed deep tissue invasion and incidence was found to be increasing with age(over 30 years old). All patients with DFSP on head and neck region revealed deep tissue invasion followed by trunk(54%) and lower extremities(50%). There was no relationship between duration of disease and deep tissue invasion. Conclusions: It is clear that many cases of DFSP had a deep tissue invasion. And high prevalence of deep tissue invasion with age, primary site was intimately associated. So, underlying deep tissue must be completely examined and excised sufficiently throughout the operation for clear resection of DFSP with no recurrences, especially when age is over 30s and on head and neck region.

An Infant Case of Citrin Deficiency with Corresponding Biochemical Features and a Heterozygous SLC25A13 Mutation (SLC25A13 이형접합 유전자 변이와 부합하는 생화학적 소견을 가진 영아 시트린 결핍증 1례)

  • Kang, Su Min;Chi, Yang Hyun;Lee, Jun Hwa
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.3
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    • pp.155-159
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    • 2015
  • Citrin deficiency (OMIN #605814) is an autosomal recessive disorder caused by the SLC25A13 gene mutation with abnormal biochemical findings, including increased serum ammonia, citrulline, arginine, galactose, serum threonine-to-serine ratio, serum pancreatic secretory trypsin inhibitor, and alpha-fetoprotein. Citrin deficiency can manifest in three ways: in newborns as neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), in older children as failure to thrive and dyslipidemia caused by citrin deficiency (FTTDCD), and in adults as citrullinemia type 2 (CTLN2) with recurrent hyperammonemia and neuropsychiatric symptoms. We report a 35-day-old asymptomatic patient with citrin deficiency who had abnormal biochemical findings.

10 year follow up of a boy with Lesch Nyhan Syndrome

  • Kim, Sook Za;Song, Wung Ju
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.11 no.1
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    • pp.88-98
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    • 2011
  • Lesch-Nyhan syndrome is a X-linked recessive disorder caused by a deficiency of the enzyme hypoxanthine-guanidine phosphoribosyltransferase (HPRT), enzyme to recycle purines. Case history: born induced vaginal delivery at 40 weeks complicated by premature membrane ruputure, body weight 2.820 gm. He showed failure to thrive showing severe protein aversion like milk products and pink daper. Developmental delay revealing rolling over at 10.5 month, followed by regression. Seizure at 2 months, His poor oral feeding was lifelong problem. Weak crying, spastic, choreoathetoid movement. Self mutilating behavior noted and diagnosed at age 3 years. No family history of consanguinity and neurological disorders. Method: Laboratory test, physical exam, imaging study and molecular. Clinical follow up Treat ment with allopurinol. Result: uric acid 10.5 mg/dL (N 3.5-7.9), APRT 151.1uM/ min/ml pro(25.7-101), HPRT 7.6 (N 233.5-701) and c.151C>T hemizygote (p,Arg51X). Abdominal sonogram showed staghorn calculi in both kidneys, brain MRI brain atrophy. Clinical follow up showed, seizure at 2 mo, developmental delay (head control and, rolling over at at 11mo, pointing body part at 2 yr 7 mo, eye hand coordination at 2 y 11mo,creeping at 3 y 7 mo, speaking words at 6 y 6 mo ),and developmental regression at 3 yr of age. Sleeping problem including insomnia and severe constipation. Self mutilating behavior (lip bite) started at 2.5 yr, neurologic sx including intermittent upward gaze accompanied by swallowing difficulty at 3 y 7 mo grand mal seizure at 4.5 yr and spastic extremity and trunchal hypotonia and choleoathetoid movement and ataxia at 6.5 yr. Scoliosis with severe spasticity at 9 yr 9 mo. Acute life threatening episode with irregular breathing at 9 yr and 9 mo, Emaciation and nephrolithiasis and recurrent pneumonia. Died suddenly at 10 yr 3 mo. Conclusion: life long feeding problem, chronic gut motility dysfunction, sleeping difficulty and progressing neurologic deterioration and nephrolithiasis despite normal serum uric acid maintence by allopurinol treatment.

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