No abstract available.
Facial Asymmetry* ; Prognathism*

BACKGROUND: Hemophagocytic lymphohistiocytosis(HLH) is a rare and fatal disorder in children. Recently its clinical characteristics and etiology of uncontrolled activation of cellular immune system in genetically predirected patients have keen elucidated. The authors analyzed the clinical characteristics and response to immunosuppressive agents of HLH patients in single institute. METHODS: The authors retrospectively analyzed various clinical data including CSF and bone marrow examination at diagnosis and follow up period in the 6 patients who were diagnosed as HLH at Asan Medical Center during last 2 years. Antithymocyte globulin(ATG : 10 mg/kg/day) and methylprednisolone(methyl-PD: 5 mg/kg/day) for 5 consecutive days as induction treatment and cyclosporin A(CsA) as maintenance treatment after induction with weekly intrathecal methotrexate for 5-6 weeks were given to the recently diagnosed 3 patients. RESULTS: All the patients except one were infants. Persistent fever, hepatosplenomegaly and pancytopenia were observed in all the patients. Family history of suspicious HLH was observed in 4 patients(67%). The characteristic elevated serum triglyceride(TG) level and/or decreased fibrinogen level were noted in all. Mild to moderate CSF pleocytosls with increased lymphocytes and monocytes was also observed in all during the disease course. Variable degree of nonqr-Langerhans cell histiocytic infiltration and hemophagocytosis were observed in all the cases. Of the 3 patients treated with ATG and methyl-PD, one achieved complete remission and the others achieved partial remission within 2 weeks of treatment, but all expired after 5 months, remission with CsA. Regardless of treatment regimen, all the 6 patients expired due to CNS sequelae of HLH. CONCLUSIONS: HLH mainly developed in infants. Persistant fever, hepatosplenomegaly and pancytopenia were observed in most cases with the characteristic change of serum TG and/or fibrinogen level. HLH should be included in the differential diagnosis in patients with these features especially when the family history of suspicious HLH is present, and histologic comfirmation of HLH could be easily accomplished with bone marrow study in most cases. Remission induction of HLH could be achieved with immunosuppressive treatment but it was difficult to maintain long term remission.
Bone Marrow ; Bone Marrow Examination ; Child ; Chungcheongnam-do ; Cyclosporine ; Diagnosis ; Diagnosis, Differential ; Fever ; Fibrinogen ; Follow-Up Studies ; Humans ; Immune System ; Immunosuppressive Agents ; Infant ; Lymphocytes ; Lymphohistiocytosis, Hemophagocytic* ; Methotrexate ; Monocytes ; Pancytopenia ; Remission Induction ; Retrospective Studies

BACKGROUND: Despite the development of empiric antibiotics in treatment of neutropenic fever(NF) in pediatric cancer patients, bacterial infection is still the most important cause of death in these patients. In this study the authors analyzed clinical characteristics and tried to find out the possible risk factors of the pediatric cancer patients who succumbed to the documented bacterial infection after episodes of neutropenic fever. METHODS: 17 pediatric cancer patients expired after episodes of neutropenic fever(NF) in the pediatric department of Asan Medical Center from Mar. 1990 to Feb. 1996 were grouped by the results of bacterial culture. 7 cases (Group A) had documented bacterial culture results and 10 cases (Group B) had negative culture results. The clinical characteristics of these two groups were analyzed retrospectively. RESULTS: There were no differences in the types of cancer between the two groups. All the documented bacteria were gram negative organisms and all cultured from the blood. There were no differences between the two groups in the treatment duration, known risk factors of infection before and at the onset of fever, antibiotics administered, and interval from the onset of NF to the administration of antibiotics. The response rate of initial empiric antibiotics was lower in group A(14%) compared to Group B(40%), even though all the cultured organisms except 2 cases in Group A were sensitive to initial empirical antibiotics. Septic shock was the cause of death in 86% of Group A patients, but only in 10% of Group B patients. The other causes of death were progression of cancer, bleeding, hepatic failure, adult respiratory distress syndrome and multiple organ failure, which showed no difference between the 2 groups. The interval from the onset of fever to death was significantly shorter in Group A(6.2+/-2.3 days) compared to Group B(24.9+/-18.6 days). CONCLUSIONS: Bacterial infection is still the most frequent cause of death after NF. Most patients succumbed to the documented bacterial infection showed rapid progression of bacteremia to septic shock despite administration of sensitive antibiotics. The known risk factors of infection before the onset of NF and other treatment factors shows no differences between the two groups in this study. These results suggest that the bacteremia is obvious risk factor of poor outcome after NF episode, and antibiotics alone may be insufficient to prevent the rapid progression of septic shock in these patients.
Anti-Bacterial Agents ; Bacteremia ; Bacteria ; Bacterial Infections ; Cause of Death* ; Chungcheongnam-do ; Fever* ; Hemorrhage ; Humans ; Liver Failure ; Multiple Organ Failure ; Respiratory Distress Syndrome, Adult ; Retrospective Studies ; Risk Factors ; Shock, Septic

No abstract available.
Pyelonephritis, Xanthogranulomatous* ; Wilms Tumor*

The authors experienced 2 cases of bacterial intracranial aneurysm, the mortality of which has been reported to by very high and the management of which is controversial. Some authors have recommended observation with serial angiography during antimicrobial therapy and surgery for aneurysms that enlarge or remain patent. Others have argued for promt surgical removal. Bothof our patients had multiple aneurysms and were treated with appropriate, high-dose antibiotics, but intracerebral homorrhage(ICH) occurred during antibiotiecs treatment in both cases and they underwnet emergent operation, but both died.
Aneurysm ; Angiography ; Anti-Bacterial Agents ; Humans ; Intracranial Aneurysm* ; Mortality

Cryoglobulinernia occurs in about 5% of the cases of multiple myeloma. The most common finding in patient with cryoglobulinemia is ulceraticn that oceurs about ankle, hands, and occasionally the ears, upon prolonged exposue to cold. A 59-year old male had had 5 years of pain in his ears. There were black or dark brown colored ischemic ulcerations on his both helix. He also had mottled purpuric patches on his both ankles. A test for cryoglobulinema was positive. X-ray examination of the skull showed multiple punched but lesions. The bone marrow study revealed myeloma cell infiltration.
Ankle ; Bone Marrow ; Cryoglobulinemia* ; Ear ; Hand ; Humans ; Male ; Middle Aged ; Multiple Myeloma* ; Skull ; Ulcer

OBJECTIVE:Ultrasound estimation of amniotic fluid volume (AFI) is a critical component of antenatal surveillance. Alterations in AFI have classically been considered an indication of fetal compromise. Thus, this study was undertaken to determine whether there is any association between idiopathic polyhydramnios or oligohydramnios and adverse perinatal outcome. METHODS:Women delivered between December 1999 and November 2005, and who underwent ultrasonography from 26 to 41 weeks gestation were analyzed. Twenty-seven consecutive women with singleton pregnancies complicated by idiopathic polyhydramnios (amniotic fluid index>25) and 31 women complicated by oligohydraminos (amniotic fluid index<5) were included in this study. We reviewed the perinatal outcomes including preterm delivery, birth weight, primary cesarean section rate and indication, Apgar score (1-min and 5-min), and admission to NICU (neonatal intensive care unit). These findings were compared with those of 50 matched control patients with normal amniotic fluid volume. RESULTS:We observed that idiopathic oligohydramnios was significantly associated with primary perinatal outcome (Cesarean delivery for fetal distress) as well as secondary outcome variables such as birthweight, SGA (small for gestational age), Apgar score<7 at 1-min, 5-min, and NICU admission rates. On the other hand, idiopathic polyhydramnios did not correlate with any other perinatal outcome, except for rates of LGA (large for gestational age). CONCLUSION:The present study suggests that AFI is a weak predictor of perinatal outcome than has been classically suggested. But we demonstrated that the AFI identification of polyhydramnios was helpful in identifying LGA, and idiopathic oligohydramnios was a significant predictor of poor perinatal outcome.
Amniotic Fluid ; Apgar Score ; Birth Weight ; Cesarean Section ; Female ; Hand ; Humans ; Critical Care ; Oligohydramnios* ; Polyhydramnios* ; Pregnancy ; Ultrasonography

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) has been described in association with systemic disorders such as Gardner's syndrome (intestinal polyposis, osteomas, and benign soft-tissue tumors), Turgot's syndrome (glioma-polyposis syndrome) and familial adenomatous polyposis (FAP) of the colon. FamiIial adenomatous polyposis is an autosomal dominant disorder characterized by hundreds of polyps throughout the entire colon, and in all patients carcinoma of the color, develops at the age of 40 to 50 years. We describe a family with familial adenomatous polylpcsis coli and congenital hypertrophy of the retinal pigment epithelium. All of them except mother showed flat, weII-demarcated, round to oval pigmented patches of CHRPE. We emphasize the importance of CHRPE as a clinical marker in identifying patients With FAP since they are at risk for cancer.
Adenomatous Polyposis Coli* ; Biomarkers ; Colon ; Gardner Syndrome ; Humans ; Hypertrophy ; Mothers ; Osteoma ; Polyps ; Retinal Pigment Epithelium* ; Retinaldehyde*

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) has been described in association with systemic disorders such as Gardner's syndrome (intestinal polyposis, osteomas, and benign soft-tissue tumors), Turgot's syndrome (glioma-polyposis syndrome) and familial adenomatous polyposis (FAP) of the colon. FamiIial adenomatous polyposis is an autosomal dominant disorder characterized by hundreds of polyps throughout the entire colon, and in all patients carcinoma of the color, develops at the age of 40 to 50 years. We describe a family with familial adenomatous polylpcsis coli and congenital hypertrophy of the retinal pigment epithelium. All of them except mother showed flat, weII-demarcated, round to oval pigmented patches of CHRPE. We emphasize the importance of CHRPE as a clinical marker in identifying patients With FAP since they are at risk for cancer.
Adenomatous Polyposis Coli* ; Biomarkers ; Colon ; Gardner Syndrome ; Humans ; Hypertrophy ; Mothers ; Osteoma ; Polyps ; Retinal Pigment Epithelium* ; Retinaldehyde*

PURPOSE: To investigate the efficacy and the risk factors for failure and complications of mitomycin C (MMC)-augmented needle revision (NR) of failed filtering blebs. METHODS: Thirty eyes whose intraocular pressure (IOP) did not decrease after the cutting of scleral flap sutures were recruited. NR was performed with a 26-gauge needle under a slit-lamp. Immediately following NR, all eyes received a subconjunctival injection of 0.1 ml of 0.02 mg/ml (group A) or 0.1 mg/ml (group B) MMC at the adjacent conjunctiva. The mean follow-up period after the last needle revision was 13.2+/-4.2 months. RESULTS: The mean IOP decreased from 25.7+/-10.6 mmHg before surgery to 13.7+/-2.5 mmHg at the last follow-up in group A and from 24.1+/-6.8 mmHg to 17.7+/-8.6 mmHg in group B (group A: p=0.001, group B: p=0.065). Subconjunctival hemorrhage, hyphema, and a shallow anterior chamber appeared in most cases. However, choridal detachment and avascular changes of the conjunctiva were observed in group B. CONCLUSIONS: IOP decreased in both groups A and B, but severe complications appeared only in group B. NR followed by a subconjunctival injection of 0.02 rather than 0.1 mg/ml is a safe and effective method.
Anterior Chamber ; Blister ; Conjunctiva ; Eye ; Follow-Up Studies ; Hemorrhage ; Hyphema ; Intraocular Pressure ; Mitomycin ; Needles ; Risk Factors ; Sutures