Familial hemangioblastoma and von Hippel-Lindau's disease: case report.

Thirty-five members of a family affected with von Hippel-Lindau's disease (VHL) were examined and lesions were found in nine of them. Four of the lesions were located in the central nervous system; two cerebellar, one spinal, one cerebral. Eye lesions were present in eight of the nine patients and complications developed in seven; six unilateral and one bilateral blindness. Both mortality and morbidity were found to be higher than in other series. This is attributed to insufficient screening of the patients with familial VHL, the late diagnosis was established only after the onset of complications. The importance of screening tests in VHL risk patients is emphasized.     

Comparison of the Exposure Obtained by Endoscope and Microscope in the Extended Trans-Sphenoidal Approach

Objective: Trans-sphenoidal surgery is often combined with other approaches for the treatment of middle cranial base tumors. By combining a maxillotomy with trans-sphenoidal approach, significantly wider exposure to these regions is gained. However, endoscope-assisted techniques have also been used for sellar and parasellar and upper clival regions. Methods: An extended trans-sphenoidal approach was performed on 10 cadaver heads using the operating microscope and was repeated with a 0-degree endoscope. The mean horizontal and vertical distances were measured and pictured for each technique, and both distances were compared using a parametric paired Student's t-test. Results: The mean horizontal distances in the 10 specimens were 19.5 ± 1.8 mm by microscope and 27.5 ± 2.2 mm by endoscope, and the mean vertical distances were 25.8 ± 1.9 mm by the microscope and 34.5 ± 3.5 mm by the endoscope. Conclusion: The aim of this study was to quantify the amount of exposure obtained with an extended trans-sphenoidal approach and to compare both endoscopic and microscopic techniques. Using the endoscope in conjunction with the operating microscope may provide additional exposure and better access in skull base surgery.     

Role of interleukin 6 in the growth of myeloma-derived cell lines.

The role of interleukin 6 (IL-6) in the growth of five multiple myeloma-derived cell lines was characterized. The U266 and RPMI 8226 cell lines demonstrated increased DNA synthesis when cultured with exogenous IL-6, expressed IL-6 cell surface receptors (IL-6Rs) and expressed mRNA for IL-6R. However, these cells did not secrete detectable IL-6 protein, and a neutralizing antibody to IL-6 did not inhibit their growth. Three other myeloma-derived cell lines ARH-77, IM-9 and HS-Sultan did not respond to exogenous IL-6, secrete IL-6 or express cell surface IL-6Rs. The IL-6 responsive cell lines bore late B-cell surface antigens (Ags), CD38 and PCA-1, whereas those lines which were non-IL-6 responsive strongly expressed B1 (CD20) and B4 (CD19) Ags, representing earlier stages in B-cell differentiation. Finally, the two IL-6 responsive cell lines did not express Epstein-Barr virus (EBV) proteins; in contrast, EBV encoded proteins typically expressed during latency could be detected in the three non-IL-6 responsive lines, confirming infection with virus. These studies clarify the heterogeneity observed in the myeloma cell line phenotype and biology and suggest that the U266 and RPMI 8226 cell lines, which express IL-6 cell surface receptors and are IL-6 responsive, may be useful for further study of IL-6 signal transduction in and related IL-6 mediated growth of myeloma in vivo. In contrast, those cell lines which are IL-6-independent provide a model for further study of EBV transformation and IL-6-dependent growth mechanisms in malignancy.     

Use of the free prefabricated temporoparietal fascia-chondro-cutaneous flap nourished by only arterial inflow: an arterial flap

Summary Pedicled temporoparietal fascial flaps have been used extensively in head and neck reconstruction for many years. Also, the fascia has been grafted, and the skin graft transformed into a vascularized pedicled flap (prefabricated temporoparietal fasciocutaneous flap). On the other hand, some authors have used free temporoparietal fascial flaps for defects that require to be filled in lower and upper extremity wounds. In this case, a free prefabricated temporoparietal fascio-chondro-cutaneous flap having only an arterial inflow was used in eyelid reconstruction.Presented at the 14th Congress of Turkish Plastic and Reconstructive Surgery, Ankara, Turkey, October 1992     

A new method for the correction of secondary unilateral cleft lip nose

Summary In 1982, Dibbell described a procedure to correct this distortion by rotating and advancing the nostril medially and superiorly. We used this method in our department for several years, but as Dibbell himself experienced, we didn't always obtain satisfactory results. Like many authors, I agree that the best approach for proper correction of the secondary unilateral cleft lip nose deformity is the external approach using transcolumellar incisions, because the lower lateral cartilage exposure is inadequate with Dibbell's method. I added the transcolumellar incision similar to Bardach's to solve this problem. In this paper, this combination technique will be presented and contrasted with Dibbell's and Bardach's techniques.     

Evaluation of the harmonized alert sensing technology device for hemodynamic monitoring in chronic hemodialysis patients

The Harmonized Alert Sensing Technology (HASTE) device was developed to overcome the primary shortcomings of interval based noninvasive blood pressure (BP) monitoring. This study was conducted to assess the reliability of the HASTE system compared with standard cuff BP values in patients on hemodialysis. A total of 1,370 HASTE measurements were compared with oscillometric standard cuff systolic BP values in 42 sessions of 15 patients on hemodialysis. The average discrepancy between the HASTE and cuff systolic BP was 1.41 +/- 16.90 mm Hg. Compared with cuff measurements, 31% of systolic BP fell within a range of 5 mm Hg difference, 57% of systolic BP fell within 10 mm Hg, and 73% of systolic BP fell within a 15 mm Hg band. According to British Hypertension Society standards or Association for the Advancement of Medical Instrumentation criteria, the current HASTE method did not perform well. Technology to provide noninvasive hemodynamic monitoring is, however, in its developmental stage. The effort at continuous systolic pressure monitoring using existing, readily available, and frequently used techniques is exciting. Although the HASTE system as currently configured and calibrated did not adequately perform, variations in site analysis and conversion factors may increase pressure sensitivity and tracking over the course of a standard dialysis treatment.     

Very fast analysis of impurities in immunoglobulin concentrates using conjoint liquid chromatography on short monolithic disks

Transferrin and albumin are often present in immunoglobulin G (IgG) concentrates and are considered as impurities. Therefore, it is important to determine their concentration in order to obtain a well-characterized biological product. Here, we describe their determination based on conjoint liquid chromatography (CLC). The established method combines two different chromatographic modes in one step: affinity and ion-exchange chromatography (IEC) combined in one column. Therefore, two CIM Protein G and one CIM quaternary amine (QA) monolithic disks were placed in series in one housing forming a CLC monolithic column. Binding conditions were optimized in a way that immunoglobulins were captured on the CIM Protein G disks, while transferrin and albumin were bound on the CIM QA disks. Subsequently, transferrin and albumin were eluted separately by a stepwise gradient with sodium chloride, whereas immunoglobulins were released from the Protein G ligands by applying low pH. A complete separation of all three proteins was achieved in less than 5 min. The method permits the quantification of albumin and transferrin in IgG concentrates and has been successfully validated.     

Delayed prefabricated arterial composite venous flaps: an experimental study in rabbits

Prefabrication of composite arteriovenous flaps with implantation of an autologous graft (cartilage) or an alloplastic material (porous polyethylene) was studied in 40 rabbits. Abdominal flaps based on bilateral epigastric pedicles were elevated. An ear cartilage graft or a porous polyethylene implant was inserted under the flap. Two weeks after the operation, 10 flaps with cartilage graft and 10 flaps with porous polyethylene were raised, converted to arteriovenous flaps, and resutured in place in the experimental groups. In the other 20 rabbits of the control groups, the flaps (10 with cartilage graft and 10 with porous polyethylene) were raised and resutured in place as conventional axial flaps. At the end of the second and fourth week postoperatively, samples were obtained from the flap tissues (including a part of the graft or implantation material) and were prepared for histologic examination in all rabbits. The viable areas of all flaps were assessed at the end of fourth week after the second operation. The mean survival rates were 99.4%, 99.7%, 99.5%, and 99.8% in the arteriovenous and control flaps prefabricated with cartilage graft and the arteriovenous and control flaps prefabricated with porous polyethylene respectively. The features of wound healing in the experimental and control groups were similar. The study showed that arteriovenous perfusion can nourish a prefabricated flap containing an implanted material (autologous or alloplastic) and these 2-week delayed composite flaps have a similar survival rate to delayed prefabricated conventional axial flaps.     

Two cases of macrocephaly and immune deficiency

Macrocephaly describes a head circumference greater than two standard deviations above the mean and is a feature of a number of genetic syndromes. Here we report on two patients with microcephaly, immune deficiency and anemia. In addition, one case had periventricular leukomalacia and the other case had myelinisation delay in periventricular white matter development. These cases may represent a distinct new syndrome.     

A Rare Cause of Renovascular Hypertension: Takayasu Arteritis with Only Renal Artery Involvement

Takayasu arteritis is a chronic inflammatory disease that affects mainly the aorta, main branches of aorta, and pulmonary arteries with unknown etiology. Disease affecting solely the renal arteries is rare. We will present a case that had hypertension, hypokalemia, and metabolic alkalosis where the etiology was type 2 Takayasu arteritis, affecting renal arteries.