The term histiocytic medullary reticulosis first was introduced by Scott and Robb-Smith. It is a clinicopathologic syndrome characterized by wasting, fever, lymphadenopathy, hepatosplenomegaly, pancytopenia, and is often accompanied by jaundice, purpura. Cardinal pathologic feature are systemized proliferation of atypical, neoplastic, erythrophagocytic D. We are here reporting one case which considered compatible for HMR, with a few elementary reviewed literatures.
Fever ; Jaundice ; Lymphatic Diseases ; Pancytopenia ; Purpura

BACKGROUND: Propofol is used as an induction and maintenance agent for general anesthesia but it can cause adverse reactions like hyperlipidemia, growth of microorganisms, and pulmonary embolisms. Microemulsion propofol was developed to avoid these side effects but incidence and severity of pain on injection is higher than with lipid emulsion propofol. We aimed to compare the effects of sufentanil in analgesic doses for reducing the injection pain of microemulsion propofol. METHODS: The candidates included eighty patients, 19-60 years old and ASA I-II. They were randomly classified into four groups and pretreated with normal saline, sufentanil 0.1 microg/kg, 0.2 microg/kg or 0.3 microg/kg before injection of microemulsion propofol. Five minutes after receiving pretreatment drug, 2 mg/kg of microemulsion propofol was injected and VAS was recorded. RESULTS: There were no significant differences in the incidence of injection pain among the groups. Severity of injection pain was significantly lower in the sufentanil 0.3 microg/kg group than normal saline and sufentanil 0.1 microg/kg group. Significant differences in blood pressure and heart rate were observed in sufentanil groups only after endotracheal intubation. One patient each in sufentanil 0.1 microg/kg and 0.3 microg/kg group experienced mild cough, one from sufentanil 0.3 microg/kg group experienced dizziness and another showed signs of hypoxia. One patient each in normal saline and sufentanil 0.1 microg/kg group showed clinical symptoms of phlebitis in the injection area. CONCLUSIONS: Pretreatment with sufentanil 0.3 microg/kg reduced the severity of microemulsion propofol injection pain without increasing arterial blood pressure and heart rate after endotracheal intubation.
Anesthesia, General ; Anoxia ; Arterial Pressure ; Blood Pressure ; Cough ; Dizziness ; Heart Rate ; Humans ; Hyperlipidemias ; Incidence ; Intubation, Intratracheal ; Phlebitis ; Propofol ; Pulmonary Embolism ; Sufentanil

Sirenomelia is rare congenital malformation characterized by fusion of the lower extrimities associated anomalies included absence of urogenital system (bilateral renal agenesis, absent ureter, urinary bladder, absent internal and external genitalia), induced severe oligohydramnios, anorectal atresia, single umbilical artery, pulmonary hypoplasia. The incidence is 1:60000 births. Survival is extremely rare, and only possible, in the absence of bilateral renal agenesis. In view of the dismal prognosis, early diagnosis allows for earlier and less traumatic therapeutic abortion. We report a case of sirenomelia without oligohydramnios diagnosed in early second trimester.
Abortion, Therapeutic ; Early Diagnosis ; Ectromelia* ; Female ; Humans ; Incidence ; Oligohydramnios* ; Parturition ; Pregnancy ; Pregnancy Trimester, Second* ; Prognosis ; Single Umbilical Artery ; Ureter ; Urinary Bladder ; Urogenital System

OBJECTIVE: We investigated the expression of chemokine receptors in human ovarian cancer to understand the role of chemokines in ovarian cancer development and metastasis. METHODS: Twenty-two cases of epithelial ovarian cancer were studied for expression of 13 chemokine receptors such as CXCR1-CXCR5 and CCR1-CCR8 by using semi- quantitative RT-PCR and immunohistochemistry. Moreover, we studied the relationship between the chemokine receptors expression and lymph nodes metastasis of ovarian cancers. RESULTS: As compared with normal ovarian tissues, ovarian cancer tissues showed higher mean expression levels of CCR1,3,4,5,7,8 and CXCR1,3,4. Of chemokine receptors, CCR7 revealed the significantly higher levels of expression in ovarian cancer tissues relative to normal tissues. In the cases of retroperitoneal lymph nodes metastasis, increased expression of CCR2,4 and CXCR 1,3,4 was observed although there was no statistical significance. CONCLUSION: These results suggest that there is a complex chemokine/chemokine receptor network in pathogenesis and the way of lymph node metastasis of ovarian cancer rather than a specific chemokine or chemokine receptor.
Chemokines ; Humans ; Immunohistochemistry ; Lymph Nodes ; Neoplasm Metastasis ; Ovarian Neoplasms* ; Receptors, Chemokine*

Biliary-enteric fistula is a rare disease, and the common causes of biliary-enteric fistula are gallstone, peptic ulcer, malignancy and trauma. It is known that the most common type of biliary-enteric fistula is the cholecysto-duodenal fistula, yet the combination of choledocho-duodeno-colonic fistula is a rare finding. A 78-year-old woman was admitted because she had suffered with right upper quadrant pain, a febrile sense and chills for 2 days. We confirmed the choledocho-duodeno-colonic fistula by performing gastroduodenoscopy, abdominal CT and an upper GI series. So, we report here on an usual case of choledocho-duodeno-colonic fistula, along with a review of the relevant literatures.
Aged ; Biliary Fistula ; Chills ; Female ; Fistula* ; Gallstones ; Humans ; Peptic Ulcer ; Rare Diseases ; Tomography, X-Ray Computed

BACKGROUND: A decreased level of serum arginine vasopressin(AVP) and an increased sensitivity to an exogenous AVP is expected in patients with septic shock who often require a high infusion rate of catecholamines. The goal of the study was to determine whether an exogenous AVP infusion to the patients with septic shock would achieve a significant decrement in infusion rate of catecholamine vasopressors while maintaining hemodynamic stability and adequate urine output. METHODS: Eight patients with septic shock who require a high infusion rate of norepinephrine had received a trial of 4-hour AVP infusion with simultaneous titration of norepinephrine. Hemodynamic parameters and urine output were monitored during the AVP infusion and the monitoring continued up to 4 hours after the AVP infusion had stopped. RESULTS: Mean arterial pressure showed no significant changes during the study period(p=0.197). Norepinephrine infusion rate significantly decreased with concurrent AVP administration(p=0.001). However, beneficial effects had disappeared after the AVP infusion was stopped. In addition, hourly urine output showed no significant changes throughout the trials(p=0.093). CONCLUSION: Concurrent AVP infusion achieved the catecholamine vasopressor sparing effect in the septic shock patients, but there was no evidence of the improvement of renal function. Further study may be indicated to determine whether AVP infusion would provide an organ-protective effect to the septic shock patients.
Arginine* ; Arterial Pressure ; Catecholamines ; Hemodynamics ; Humans ; Norepinephrine ; Shock, Septic*

PURPOSE: p16 gene, mapped to the 9p21 chromosomal region, has emerged as a candidate tumor suppressor gene in human neoplasm. It is an inhibitor of cyclin-dependent kinase and inhibits Rb phosphorylation. In a variety of tumors including childhood acute lymphoblastic leukemia (ALL), deletion and/or mutation of the p16 gene has been found. Despite their high frequency, the prognostic importance of p16 alterations is still controversial in ALL and has been reported to be either unfavorable or similar to that of other patients. We studied the correlation between loss of p16 protein confirmed by immunohistochemical staining and clinical outcomes of patients diagnosed as ALL. METHODS: We performed an immunohistochemical staining for p16 protein in 74 cases of bone marrow biopsy slide initially diagnosed as ALL between January 1998 and December 2006. We reviewed the clinical manifestations, laboratory findings, treatment outcomes retrospectively. RESULTS: Of 74 slides, 12 were negative for p16 protein. Seven were males and 5 were females with a median age at diagnosis was 5.8 (1.3-18.8) years. Initial WBC were 17,225 (500-403,300)/microL. By immunologic surface marker analysis, 7 patients were early pre-B CALLA (+) and 5 patients were T-cell ALL. Two patients of intermediate risk group had relapsed and died. Three patients had family history of breast cancer. Four patients died and overall survival rates were 53.5+/-18.7%. CONCLUSION: Loss of p16 protein is supposed to be an independent risk factor of childhood ALL associated with poor outcomes. In clinical setting, the clinician must take into account p16 status, not only at the genomic but also at the protein level. Further clinical experience on thoroughly investigated cases will help a better understanding between p16 status and clinical outcomes.
Biopsy ; Bone Marrow ; Breast Neoplasms ; Female ; Genes, p16 ; Genes, Tumor Suppressor ; Humans ; Leukemia ; Male ; Phosphorylation ; Phosphotransferases ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Risk Factors ; Survival Rate ; T-Lymphocytes

PURPOSE: Hypertension is defined as average systolic blood pressure and/or diastolic blood pressure that is > or =95th percentile for gender, age, and height on > or =three occasions. Knowing that blood pressure values increase in children as they grow older, the purposes of this study were to measure blood pressure by an oscillometric device and to determine normal values and percentile curves for children. METHODS: Systolic and diastolic blood pressures were measured twice with an oscillometric device in 3,545 boys and 3,145 girls under six years of age, in Seoul. Using this data, we determined average blood pressure values and percentile curves based on gender and age; we subdivided these values into blood pressures of 50th, 90th, 95th, and 99th percentiles, by percentile of height. The regression coefficients and standard deviations of the systolic and diastolic blood pressure values were obtained from linear regression models. RESULTS: Older boys and girls had higher systolic and diastolic blood pressure values. Older boys and girls in the same percentile of height for age had higher systolic and diastolic blood pressure values. Taller boys and girls within the same age group had higher systolic and diastolic blood pressure values. CONCLUSION: Blood pressure standards based on gender, age, and height were obtained via an oscillometric method. Llimitation of this study is that the study population was not from the whole country, but exclusively from Seoul. Nonetheless, the data from this study will be helpful in diagnosing and managing hypertension in Korean children.
Blood Pressure ; Child ; Humans ; Hypertension ; Linear Models ; Reference Values

PURPOSE: Kidney transplantation (KT) is an ideal treatment for pediatric patients with end-stage renal disease (ESRD). We report the clinical outcomes of pediatric ESRD patients who underwent KT in a single regional center. METHODS: We retrospectively investigated the medical records of 60 pediatric patients who were diagnosed with ESRD and underwent KT in our hospital between January 1985 and June 2016. RESULTS: A total of 60 children and adolescents (40 male, 20 female; mean age, 13.86±4.26 years) were included in this study. Six patients (10.0%) underwent KT immediately after receiving the diagnosis of ESRD, while the others underwent KT after dialysis treatment (mean period of dialysis, 368.7±4,41.8 days). The mean donor age (50 living-related [83.3%], 10 deceased [16.7%]) was 40.0±12.85 years and the male:female ratio was 1.07:1. The most common cause of ESRD was chronic glomerulonephritis. The overall survival rates at 1, 3, and 5 years after KT were 98%, 98%, and 96%, respectively, while the graft survival rates at 1, 3, and 5 years were 93%, 86%, and 68%, respectively. Children who underwent KT before 10 years of age had better monthly growth rates than those who underwent KT later than 10 years of age. CONCLUSIONS: KT is performed less frequently in children than in adults, but causes of ESRD vary and clinical outcomes after KT greatly affect the growth and development of pediatric patients. Therefore, further analysis and monitoring of clinical progression after KT in pediatric ESRD patients are necessary.
Adolescent ; Adult ; Child ; Diagnosis ; Dialysis ; Female ; Glomerulonephritis ; Graft Survival ; Growth and Development ; Humans ; Kidney Failure, Chronic ; Kidney Transplantation* ; Kidney* ; Male ; Medical Records ; Retrospective Studies ; Survival Rate ; Tissue Donors

PURPOSE: Kidney transplantation (KT) is an ideal treatment for pediatric patients with end-stage renal disease (ESRD). We report the clinical outcomes of pediatric ESRD patients who underwent KT in a single regional center. METHODS: We retrospectively investigated the medical records of 60 pediatric patients who were diagnosed with ESRD and underwent KT in our hospital between January 1985 and June 2016. RESULTS: A total of 60 children and adolescents (40 male, 20 female; mean age, 13.86±4.26 years) were included in this study. Six patients (10.0%) underwent KT immediately after receiving the diagnosis of ESRD, while the others underwent KT after dialysis treatment (mean period of dialysis, 368.7±4,41.8 days). The mean donor age (50 living-related [83.3%], 10 deceased [16.7%]) was 40.0±12.85 years and the male:female ratio was 1.07:1. The most common cause of ESRD was chronic glomerulonephritis. The overall survival rates at 1, 3, and 5 years after KT were 98%, 98%, and 96%, respectively, while the graft survival rates at 1, 3, and 5 years were 93%, 86%, and 68%, respectively. Children who underwent KT before 10 years of age had better monthly growth rates than those who underwent KT later than 10 years of age. CONCLUSIONS: KT is performed less frequently in children than in adults, but causes of ESRD vary and clinical outcomes after KT greatly affect the growth and development of pediatric patients. Therefore, further analysis and monitoring of clinical progression after KT in pediatric ESRD patients are necessary.
Adolescent ; Adult ; Child ; Diagnosis ; Dialysis ; Female ; Glomerulonephritis ; Graft Survival ; Growth and Development ; Humans ; Kidney Failure, Chronic ; Kidney Transplantation* ; Kidney* ; Male ; Medical Records ; Retrospective Studies ; Survival Rate ; Tissue Donors