Attempted treatment of factor H deficiency by liver transplantation

Complement factor H (FH) deficiency is one of the causes of atypical hemolytic uremic syndrome (HUS). Most patients with FH deficiency associated HUS progress to end-stage renal disease despite plasma therapy. Moreover, the disease invariably recurs in the graft kidney and causes graft failure. We confirmed FH deficiency in a 30-month-old boy with recurrent HUS of 2 years duration, and attempted an auxiliary partial orthotopic liver transplantation (APOLT) to overcome the sustained intractable dependency on plasma therapy. APOLT restored the plasma FH level, without HUS recurrence, for 7 months. However, thereafter he suffered from serious infectious complications associated with immunosuppression and finally died 11 months after APOLT. In conclusion, although APOLT showed clinical and laboratory improvement for some period in this patient, the final fatal outcome suggests that liver transplantation should be cautiously applied to patients with HUS associated with FH deficiency.     

Neuronal tumors of the central nervous system: radiologic findings and pathologic correlation.

Pure neuronal and mixed neuronal-glial tumors of the central nervous system are uncommon but fascinating because they are less aggressive than the more common glial tumors and their prognosis is excellent. Neurologic manifestations are varied and include seizures, symptoms of increased intracranial pressure, and neurologic deficits according to tumor location. Many neuronal tumors of the central nervous system demonstrate characteristic radiologic findings. At magnetic resonance (MR) imaging, gangliocytomas demonstrate low signal intensity on T1-weighted images, high signal intensity on T2-weighted images, and frequent enhancement on gadolinium-enhanced T1-weighted images. Characteristic MR imaging findings of Lhermitte-Duclos disease are a nonenhancing mass in a cerebellar hemisphere with a striated pattern. Central neurocytomas are typically located in the lateral ventricles near the foramen of Monro with a characteristic attachment to the septum pellucidum. Ganglioneurocytoma is a rare variant of central neurocytoma that is characterized by differentiation toward ganglion cells. In ganglioglioma, a well-defined cystic mass with a solid mural nodule is typically seen. Extension of enhancement to the leptomeninges is characteristic of desmoplastic infantile ganglioglioma and correlates with the firm dural attachment of the solid component. Dysembryoplastic neuroepithelial tumor has a well-demarcated, multilobulated or gyriform appearance.     

Metastatic Disease to the Clivus Mimicking Clival Chordomas

Objectives/Hypothesis A comprehensive review of the literature of clival metastases and presentation of two additional cases.Study Design Literature review and report of two cases.Methods A literature review of the MEDLINE database (1950 to January 19, 2013) was performed to identify all cases of patients with metastatic disease to the clivus. Additionally, two novel cases are presented.Results In total, 47 cases were identified in the literature, including the two cases presented in this study. Metastatic disease to the clivus is the initial presenting symptom of the primary malignancy in 36% (13/36) of the cases. When there was a history of malignancy, the median interval of time to clival metastases was 24 months (range 1 to 172 months). Clinical symptoms manifested often as cranial neuropathies, with at least abducens palsies as the initial presenting symptom in 61.9% (26/42) of patients. Tumor pathology was diverse, but several pathologies were seen more commonly: prostate carcinoma (18.1%, 9/47), hepatocellular carcinoma (10.6%, 5/47), and thyroid follicular carcinoma (8.5%, 4/47).Conclusion Although clival metastases are extremely rare, they are an important part of the differential of clival masses as they can be the presenting symptom of distant malignancy.Level of Evidence 4.     

Fatty marrow conversion of the proximal femoral metaphysis in transient bone marrow edema syndrome

Introduction In the proximal femoral metaphysis, hematopoietic marrow is predominant during the adult stage of life. The conversion of hematopoietic marrow to fatty marrow in the proximal femoral metaphysis has been suggested as an etiologic factor of ischemia in the pathogenesis of femoral head osteonecrosis. To determine whether the chronology of fatty marrow conversion of the proximal femoral metaphysis is related to transient bone marrow edema syndrome of the hip, a case control study was conducted on 10 patients with the disease.Materials and methods There were 8 men and 2 women with a mean age of 33 years (range 19–45 years). The 10 patients were matched with 20 controls for gender and age (5-year range). T1-weighted MRI scans of their hips were reviewed. Marrow of the greater trochanter becomes fatty before puberty, and thus, the greater trochanter can be used as a built-in control. The signal intensity of the proximal femoral metaphysis was compared to that of the greater trochanter.Results In all patients, the signal intensity of the proximal femoral metaphysis was isointense (fatty marrow) relative to that of the greater trochanter. In control subjects, the signal intensity was isointense in 8 (40%) and hypointense (hematopoietic marrow) in 12 (60%) (p<0.05).Conclusion The current study shows that the proximal femoral metaphysis is predominantly fatty in transient bone marrow edema syndrome. The conversion of hematopoietic to fatty marrow is known to correlate with decreases in intramedullary blood flow. Thus, the current study suggests that an ischemia of the proximal femur secondary to fatty marrow conversion of the proximal femoral metaphysis might be a cause of transient bone marrow edema syndrome of the hip.     

Actual long-term outcome of extrahepatic bile duct cancer after surgical resection

OBJECTIVES: The objectives of this study were to analyze the actual long-term outcome after the surgical resection of extrahepatic bile duct cancer and to identify the characteristics shared by long-term survivors (5 years or longer). SUMMARY BACKGROUND DATA: Although reported 5-year survival rates of extrahepatic bile duct cancer lie between 20% and 30%, these data are not reflecting the actual cure rate. Some patients survive longer than 5 years with recurrent disease. In some patients, recurrence is detected after 5 years. Accordingly, true cure rate is probably substantially lower than the 5-year survival rate. METHODS: One hundred fifty-one patients from a total of 282 patients with extrahepatic bile duct cancer (excluding ampulla of Vater cancer) underwent surgical resection between 1986 and 1997. We analyzed the actual survival outcome and postresection prognostic factors after resection, which included hepatobiliary resection (HBR; extended either right or left hepatectomy, caudate lobectomy, and hilar bile duct resection, n = 23), bile duct resection (BDR; n = 25), and pancreatoduodenectomy (PD; n = 103). We also compared the clinicopathologic characteristics of actual long-term survivors (n = 49) with those who survived longer than 5 years and with short-term (<5 years) survivors. RESULTS: Forty-nine of the 151 resection cases (32.5%) survived 5 years or longer; there was no 5-year survivor in the nonresected cases. The actual 5-year survival rate was 47.8% after HBR (11 of 23), 28.0% after BDR (7 of 25), and 30.1% after PD (31 of 103) (P = 0.083). Tumor histology and lymph node metastasis were identified as independent prognostic factors by multivariate analysis. Some long-term survivors had poor postoperative prognostic factors such as T3, lymph node metastasis, or microscopic margin involvement, but none with a poorly differentiated tumor. Seven long-term survivors had recurrent disease at 5 years, and recurrence was detected after 5 years in 8 more patients. Therefore, the actual cure rate (<19.2%) was substantially less than the 5-year survival rate. CONCLUSIONS: In cases of extrahepatic bile duct cancer, resection should be considered and efforts should be made to obtain a tumor-free margin. An aggressive surgical approach will give some survival benefit to the patients with even advanced disease. Long-term follow up is needed before declaring "a cure," because late recurrence after 5 years is detected not infrequently. Adjuvant therapy, local and systemic, needs to be further developed.     

Analysis and management of pathogens isolated from patients with complicated facial lacerations and abrasions

Plastic surgeons commonly encounter patients with facial lacerations and/or abrasions in the emergency room. If they are properly treated, facial wounds generally heal well without complications. However, infection can sometimes cause delayed wound healing. We performed wound culture for the early detection of infection and to promote the healing of infected facial wounds. We included 5033 patients with facial wounds who visited the emergency room of Kangnam Sacred Heart Hospital between January 2018 and February 2021. Among them, 104 patients underwent wound culture. We analysed the pathogens isolated and the patients' age, sex, wound site, mechanism of injury, wound healing time, time from injury to culture, time to culture results, and dressing methods used. Pathogens were isolated in slightly less than half of the patients (38.46%); among them, Staphylococcus epidermidis was the most common (47.5%). Methicillin-resistant coagulase-negative staphylococci were isolated in six (15%) patients. Patients with complicated wounds had a longer mean wound healing time (10.83 ± 5.91 days) than those with non-complicated wounds (6.06 ± 1.68 days). Wound culture of complicated facial wounds resulted in the isolation of various types of pathogens, including antibiotic-resistant bacteria and fungi. We recommend the use of wound culture for early detection of infection to prevent delayed wound healing.     

Traffic related pollution and heart rate variability in a panel of elderly subjects

BACKGROUND: Particulate air pollution has been associated with increased cardiovascular deaths and hospital admissions. To help understand the mechanisms, the types of particles most involved, and the types of persons most susceptible, the association between exposure to summertime air pollution and heart rate variability (HRV) was examined in a panel study of 28 elderly subjects. METHODS: Subjects were seen once a week for up to 12 weeks and HRV (SDNN, r-MSSD, PNN50, low frequency/high frequency ratio (LFHFR)) was measured for approximately 30 minutes at each session using a defined protocol. Temperature, day of the week, and hour of the day were controlled, and dummy variables for each subject were controlled for subject specific risk factors. RESULTS: PM2.5 was associated with r-MSSD (-10.1% change for an interquartile range (IQR) increase in exposure (95% CI -2.8 to -16.9)) and PNN50, but stronger associations were seen with black carbon, an indicator of traffic particles, which was also associated with SDNN (-4.6% per IQR (95% CI -2.0 to -7.2)) and LFHFR. Secondary particles were more weakly associated with r-MSSD, as was ozone. No associations were seen with SO2 or NO2. CO had similar patterns of association to black carbon, which disappeared after controlling for black carbon. Black carbon had a substantially higher effect on SDNN in subjects who had had a previous myocardial infarction (-12.7%, 95% CI -5.7 to -19.25). CONCLUSIONS: Particles, especially from traffic, are associated with disturbances of autonomic control of the heart.     

IL-10 promoter gene polymorphism associated with the occurrence of chronic GVHD and its clinical course during systemic immunosuppressive treatment for chronic GVHD after allogeneic peripheral blood stem cell transplantation

BACKGROUND: The current study attempted to evaluate the association between IL-10 promoter gene polymorphism and transplant outcomes including the occurrence of chronic graft-versus-host disease (GVHD) and its clinical course during systemic immunosuppressive treatment (IST) among 60 recipients of cytokine-mobilized peripheral blood stem cell (PBSC) from HLA-matched sibling donors. METHODS: We analyzed 3 single-nucleotide polymorphisms in proximal region of IL-10 promoter gene (-1082/-819/-592). RESULTS: In the current study, only two haplotypes (1082*A/819*T/592*A [ATA] and 1082*A/819*C/592*C [ACC]) were found. An increased occurrence of chronic GVHD was noted dependent on the IL-10 haplotypes (43% vs. 68% vs. 96% in ACC/ACC vs. ATA/ACC vs. ATA/ATA haplotype, P=0.003). In a logistic regression based on multinomial model, ATA/ATA homozygote had 7-fold increasing risk of the development of chronic GVHD compared with ACC/ACC homozygote. The incidence of chronic GVHD at 1 year was 46%+/-20%, 64%+/-10%, and 82%+/-5% in ACC/ACC, ATA/ACC and ATA/ATA group, respectively (P=0.0266). Plus, the duration of systemic IST was significantly shorter in recipients without ATA-haplotype comparing with those with ATA haplotype (339 days vs. 1,146 days, P=0.0091). CONCLUSION: IL-10 promoter gene polymorphism was found to be apparently associated with chronic GVHD after allogeneic peripheral blood stem cell transplantation from HLA-matched sibling donors.