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Protein C deficiency.

Severe homozygous protein C deficiency is a rare but serious problem in the newborn, with a clinical presentation of purpura fulminans. We have presented such a case in an 1,870 gm female neonate. Salient clinical findings in this case include DIC associated with extensive ecchymosis and subsequent gangrene of the skin, thrombotic complications that began on the third day of life. There was no precipitating infection. The progressive gangrenous necrosis of heel and toes was refractory to heparin therapy, but there was clinical improvement after treatment with fresh frozen plasma. Our patient's level of protein C antigen was less than 3% (normal 70% to 130%). Levels of other vitamin-K-dependent factors, as well as factor V, factor VIIIC, and antithrombin III were normal. A heterozygous protein C deficiency was documented in the mother and father. Presently the child is receiving warfarin sodium (Coumadin) therapy and is clinically stable.

Adolescent↗

Key role of the alternate complement pathway in hyperacute rejection of rat hearts transplanted into fetal sheep.

Hyperacute rejection (HAR) mediated by xenoreactive natural antibodies (XNA), which are thought to develop in early infancy, is a major impediment to transplantation between widely disparate species. The ability to diagnose certain forms of congenital heart defects early in prenatal life suggests the potential for these defects to be corrected by cardiac transplantation, prior to the development of XNA and host immunocompetence. This study investigated whether discordant cardiac xenotransplantation into fetal and neonatal recipients might obviate HAR due to the relative lack of XNA. Six neonatal lambs at 3 days (n=3) or 7 days (n=3) of life, and two fetal lambs at 125 and 142 days of gestation (term = 145 days) received cardiac grafts from adult Wistar-Furth (275-350 g) rats. All eight cardiac xenografts showed clinical evidence of HAR, with rapid swelling, loss of contractility, and ecchymosis and a mean survival time of 12.5 +/- 6.4 min. Sections of explanted grafts showed classical histologic features of HAR, including interstitial hemorrhages, platelet microthrombi, and edema, without leukocyte infiltration. Immunopathology of grafts harvested from fetal recipients showed a lack of significant intragraft deposition of sheep IgM, IgG, or C1q, but widespread endothelial labeling for C3, factor B, and properdin. In contrast, grafts in neonatal recipients showed IgM, IgG, and C1q deposition, as well as C3, factor B, properdin, and terminal complement (C) components. Fibrin deposition and platelet thrombi were seen in both groups of recipients. Injection of cobra venom factor resulted in prolongation of cardiac xenograft survival in neonatal lambs (n=3) to 12 hr. Analysis by immunohistology showed that normal sera from neonatal and adult, but not fetal, sheep contained IgM and IgG XNA reactive with rat cells. In conclusion, rodent grafts transplanted into fetal sheep undergo HAR, likely through direct activation of the alternate pathway of C, whereas neonatal lambs acquire XNA in the very early postnatal period and reject rat hearts through activation of both the classical and alternate pathways of C. Thus, at least in some species combinations, cardiac transplantation during the early postnatal period, or even in utero, may still be subject to development of HAR.

Acute Disease↗

Advances in facial rejuvenation surgery.

There have been many important contributions to the literature on the subject of facial rejuvenation surgery. There has been a trend toward less invasive techniques that offer patients shorter recovery periods with levels of aesthetic satisfaction comparable to those of more invasive and complex procedures. Techniques reviewed in this article include midface suture meloplication, and purse-string suture suspension of the superficial musculoaponeurotic system to effect changes in the lower one third of the face. Another exciting area is the use of fibrin sealants and autologous platelet gels to enhance the adhesion of the tissue flaps at closure and reduce the amount of ecchymosis and edema in the recovery period. Their ability to decrease bruising, swelling, and lessen the frequency of complications has the potential of increasing patient satisfaction through a quicker return to normal activities.

Blood Platelets↗

Enfuvirtide plasma levels and injection site reactions using a needle-free gas-powered injection system (Biojector).

OBJECTIVES: To assess the use of the Biojector B2000 needle-free gas-powered injection system for subcutaneous administration of enfuvirtide in HIV-infected patients and to compare this system with standard needles and syringes with respect to ease of use, severity of injection site reactions (ISR), and enfuvirtide plasma levels. DESIGN: An observational study among 32 treatment-experienced HIV clinic patients receiving enfuvirtide. METHODS: Adult patients were assessed before and after switching from standard needles to the Biojector for enfuvirtide administration. Patients used the Biojector for up to 24 weeks and rated ease of use from 0 (easy) to 3 (difficult). ISR were graded from 0 to 31 for signs and symptoms (erythema, induration, pruritus, nodules/cysts, ecchymosis), duration of individual lesions, and number of lesions. Plasma was collected pre-dose and 1 h post-dose for enfuvirtide measurement. The high-pressure liquid chromatography with tandem mass spectrometry method used was specific for enfuvirtide over its known plasma metabolite. Wilcoxon rank sum tests were used to compare needle-based and Biojector outcomes. RESULTS: The Biojector was rated as being significantly easier to use (P < 0.001) and reduced the occurrence of ISR compared with standard needles (P < 0.001). Enfuvirtide plasma levels were not statistically different between the two administration methods at either pre-dose trough (P = 0.41) or 1 h post-dose (P = 0.74). CONCLUSIONS: The Biojector needle-free injection system was easy to use for enfuvirtide administration and was associated with a decreased severity of ISR. Plasma enfuvirtide levels pre-dose and 1 h post-dose were comparable when injecting with standard needles or the Biojector.

Adult↗

Spontaneous orbital and periocular hemorrhage in a patient with epithelioid hemangioma.

Epithelioid hemangioma, or angiolymphoid hyperplasia with eosinophilia, is a benign vascular lesion that infrequently involves the orbit. We report a case of spontaneous orbital and periocular hemorrhage in a patient with epithelioid hemangioma of the orbit. Ophthalmic examination was accompanied by CT and MRI, followed by histopathologic diagnosis. The patient presented with headache, eyelid bruising, and double vision. Ophthalmic examination showed ecchymosis, proptosis, and limited extraocular movements of the affected eye. Imaging studies showed a cystic right orbital mass. Histologic examination showed plump endothelial cells accompanied by lymphocytes, plasma cells, and eosinophils. A diagnosis of epithelioid hemangioma was rendered. To our knowledge, this is the first reported case of spontaneous orbital and periocular hemorrhage associated with epithelioid hemangioma. Epithelioid hemangioma should be considered in the differential diagnosis for patients presenting with spontaneous orbital or periocular hemorrhage.

Adult↗

Initial experience with fibrin sealant in pendulous urethral reconstruction. Is early catheter removal possible?

PURPOSE: We assessed whether fibrin sealant promotes early catheter removal after pendulous urethral reconstruction. MATERIALS AND METHODS: Between 1997 and 2003, 43 men with complex stricture disease underwent pendulous urethral reconstruction by a single surgeon (AFM). In 18 consecutive cases with a mean stricture length of 7.24 cm from 2000 to 2003 fibrin sealant was applied over a ventral onlay anastomosis performed with running 5-zero polydiaxanone suture. Results were compared with those in 25 men who underwent the procedure without fibrin sealant from 1997 to 2000, who served as a control group (mean stricture length 8.30 cm, p = 0.418). Catheter duration was recorded after anastomosis integrity was radiographically confirmed. RESULTS: All 18 patients with sealant were completely healed within 14 days, which was significantly shorter than in controls (p <0.001). In the sealant group increased stricture length was associated with longer catheter time (p = 0.677). Our impressions were that patient satisfaction was enhanced by early catheter removal, and postoperative penile edema and ecchymosis were decreased in the fibrin sealant group. CONCLUSIONS: Fibrin sealant appears to promote early catheter removal and enhanced wound healing after pendulous urethral reconstruction.

Fibrin Tissue Adhesive↗

Control of bleeding in children with Dengue hemorrhagic fever using recombinant activated factor VII: a randomized, double-blind, placebo-controlled study.

OBJECTIVES: We evaluated the efficacy and safety of recombinant activated factor VII (rFVIIa) in children aged < 18 years old with grade II or grade III Dengue hemorrhagic fever (DHF) who required blood component therapy for controlling bleeding episodes. STUDY DESIGN: Patients were randomized to the rFVIIa group or placebo group in a ratio of 2:1. rFVIIa or placebo (100 microg/kg body weight) was given by intravenous bolus injection. When bleeding was not effectively controlled, a second dose of rFVIIa or placebo (100 microg/kg) was given 30 min after the first dose. RESULTS: Nine and 16 patients received placebo and rFVIIa, respectively. The demographics, bleeding manifestations and grade of DHF were similar for the rFVIIa and placebo groups. Apart from petechiae and ecchymosis, one to four additional bleeding sites were found in each patient, including hematemesis (n = 15), epistaxis (n = 14), gum bleeding (n = 12), melena (n = 7), hypermenorrhea (n = 4), hematochezia (n = 2) and hematuria (n = 2). The mean total dose of rFVIIa (138.4 +/- 50.9 microg/kg) and placebo (145.4 +/- 53.7 microg/kg) were comparable. The efficacy of bleeding control at 2 h after the first dose was completely ceased (rFVIIa 75.0% versus placebo 44.4%), decreased (rFVIIa 18.7% versus placebo 11.2%), and unchanged or worsened (rFVIIa 6.3% versus placebo 44.4%). Some patients with active bleeding received platelet concentrates 3-12 h after the first dose of rFVIIa or placebo. The subsequent efficacy of bleeding control at 6, 12 and 24 h was comparable between the two groups. The cumulative use of red blood cells (rFVIIa 31.3% versus placebo 33.3%) and plasma (rFVIIa 25% versus placebo 22%) during the 24-h period was not significantly different between the two groups. In contrast, platelet concentrate requirement in the rFVIIa group (6.3%) was lower than the placebo (33.3%). No clinical evidence of thromboembolic complications or mortality as a result of bleeding was observed. CONCLUSION: rFVIIa appears to be a useful adjunctive treatment to blood component transfusion for controlling active bleeding in children with DHF especially when platelet concentrate is not readily available.

Adolescent↗

A prospective, randomized, double-blind, controlled clinical trial comparing laser-assisted lipoplasty with suction-assisted lipoplasty.

BACKGROUND: The authors randomized and prospectively analyzed their clinical experience with the use of internal neodymium:yttrium-aluminum-garnet low-level laser-assisted lipoplasty compared with suction-assisted lipoplasty. METHODS: Suction-assisted lipoplasty was generated through a SmartLipo machine and delivered into the subcutaneous tissues through 2-mm solid optical probes. Ipsilateral suction-assisted lipoplasty and contralateral laser-assisted lipoplasty were performed on one or more comparable topographic areas of the body in the same patient. Laser-assisted lipoplasty and suction-assisted lipoplasty sides of 25 patients were compared with preoperative and postoperative photographs at 3 to 5 days, 12 to 15 days, and 6 to 11 months. Statistical analysis considered surgeon and patient satisfaction, time used in the procedures, learning curves, lipocrits, operative technique, postoperative pain, edema, ecchymosis, time of recovery, body mass index, DNA proteins, free fatty acids, and cytologic patterns of post-laser-assisted lipoplasty and suction-assisted lipoplasty adipocyte architecture. Photographs were sent to the patients (blinded to the operated sides) and two plastic surgeons unfamiliar with the cases for evaluation of results. RESULTS: All patients completed the preestablished follow-ups. No complications were observed. Less pain, lower lipocrits, higher triglycerides, and DNA cellular membrane traces were detected in the laser-assisted lipoplasty sides. All other considerations studied showed no differences with either technique in the three periods of the follow-up controls. Cytologic studies showed more damage of the adipocytes in the laser-assisted lipoplasty sides. CONCLUSIONS: No major clinical differences for suction-assisted lipoplasty versus laser-assisted lipoplasty were found. Higher concentrations of free-fatty acids after laser-assisted lipoplasty must alert us to possible hepatic and renal toxicity.

Adult↗

Long-lasting results with hydroxylapatite (Radiesse) facial filler.

BACKGROUND: This study examined the patient satisfaction and longevity of Radiesse, a calcium hydroxylapatite compound, as a facial soft-tissue filler for the glabellar lines, the lips, the nose, the face, and the infraorbital region. METHODS: Radiesse, 0.5 to 2.0 ml, was injected into the designated area in each of the 40 Argentinian male and female patients in the study, using a fine needle. The age range of the patients was 25 to 60 years. RESULTS: Patients were observed over a period of 18 months. No systemic effects, no immunologic responses, and minimal adverse events (ecchymosis and hematoma in 5 percent of the subjects) occurred with the treatment. In the patient satisfaction survey, 87 percent of the patients rated their level of satisfaction as acceptable or better. CONCLUSIONS: Radiesse was shown to be a highly effective, long-lasting material for facial soft-tissue augmentation. The safe and well-tolerated compound drew high marks in patient satisfaction. A precise injection technique helped minimize potential effects such as nodule formation and helped avoid persistent overcorrection, livid coloring, and asymmetry. Radiesse can be considered a very good option for an aesthetic soft-tissue facial filler when real volumetric augmentation is needed.

Adult↗

Use of the scrotal remnant as a tissue-expanding musculocutaneous flap for scrotal reconstruction in Paget's disease.

Wide excision of extramammary Paget's disease of the penoscrotal region may leave large defects that cannot be closed easily. The authors describe their experience with a series of 6 patients in whom reconstruction of the scrotal defect was undertaken using the scrotal remnant raised as a stretchable musculocutaneous flap. It was observed that as little as a third of the residual scrotum could be expanded to resurface the entire scrotum. All flaps survived completely. Severe scrotal edema and ecchymosis were observed in 1 patient but the symptoms resolved completely with Trendelenburg positioning. The penile defects were resurfaced individually with thick skin grafts. Good-quality take with no chordee was observed in all patients after initial reconstruction. One patient developed penile contracture after reexcision of recurrent disease. Mean follow-up was 22 months (range, 3-60 months). Large defects of as much as two thirds of the scrotum may be reconstructed successfully using the tissue-expanding scrotal musculocutaneous flap.

Aged↗

Small incision transcutaneous levator aponeurotic repair for blepharoptosis.

PURPOSE: Patients presenting with blepharoptosis due to disinsertion or thinning of the levator aponeurosis require surgical repair. A minimally invasive approach directed specifically at the levator aponeurotic anatomic defect may provide benefits to the patient. Proposed advantages of a small eyelid incision (8-13 mm) include less local anesthetic and tissue distortion, less ecchymosis and edema, decreased operative times, a shortened recovery period, and improved surgical results. We describe our results with the use of a small incision ptosis repair in select patients. SUBJECT AND METHODS: A retrospective chart review between January 2000 and September 2001 included 91 patients and 118 eyelids with blepharoptosis due to aponeurotic disinsertion, corrected by a minimally invasive approach. The small incision technique comprised levator aponeurotic resection and advancement. Pre- and postoperative upper eyelid marginal reflex distances (MRD1), eyelid contour, need for reoperation, and complications (overcorrection, undercorrection, hematoma, and infection) were recorded. RESULTS: The average preoperative MRD1 measured 0.5 +/- 1.1 mm, with a range of -2.0 to 2.5 mm. The average postoperative MRD1 measured 2.6 +/- 0.8 mm, with a range of 1 to 5.5 mm (P < 0.0001). Of the 118 eyelids corrected by a small incision technique, there were 4 overcorrections, 3 undercorrections, 1 failure, 1 postoperative ptosis procedure of the contralateral upper eyelid secondary to Hering's law ptosis, and 4 patients requesting surgical treatment of dermatochalasis. No symptomatic dry eye, exposure keratopathy, or other complication resulted in association with the overcorrections. No contour abnormalities, infections, hematomas, or other complications were noted. CONCLUSION: Our experience suggests that utilization of a small skin incision is safe, precise, and efficient and allows for more rapid recovery from surgery. The authors note a decreased incidence of reoperation and postoperative complaints compared with historical larger-incision cases.

Adult↗

Brachial plexus birth palsy: a 10-year report on the incidence and prognosis.

Sixty-one cases of brachial plexus birth palsies were documented in 30,451 live births at Kaiser Foundation Hospital, San Francisco, between January 1972 and December 1982, for an incidence of 2.0/1,000 births. Thirty-eight patients were evaluated in follow-up ranging from 1 year to 11 years 6 months. Associated birth traumas include facial palsy, clavicle fracture, arm ecchymosis, and cephalohematoma. The prognosis was excellent, with full recovery in 95.7% of cases. The presence of a palsy did not preclude the development of dominant use of the extremity. Right-handedness was noted in 73% of right-sided palsies. This study showed that the incidence of palsies has not declined in the past 10 years. Risk factors and associated birth injuries were similar to those in other reports. The severity of palsies has lessened, and early recovery is usual.

Birth Injuries↗

Higher activity by opaque endometriotic lesions than nonopaque lesions.

BACKGROUND: Higher activity by early endometriosis than advanced endometriosis has been reported. However, the pattern of activity in individual colored endometriotic lesions in pelvic cavity is unknown. We investigated the variation in activity of the different colored morphologic lesions as proposed by the current revised American Society of Reproductive Medicine (ASRM) classification in women with endometriosis. METHODS: Peritoneal fluid (PF) and biopsies were collected during laparoscopy from 45 infertile women with pelvic endometriosis and 15 women without endometriosis. PF concentrations of hepatocyte growth factor (HGF), vascular endothelial growth factor (VEGF), interleukin-6 (IL-6) and monocyte chemotactic protein-1 (MCP-1) were measured by enzyme-linked immunosorbent assay (ELISA). The immunohistochemical staining of HGF and proliferating cell nuclear antigen (PCNA) in opaque and nonopaque endometriotic lesions was examined using respective antibodies, and a computer-analyzed modified quantitative-histogram score (Q-H score) was used to quantify the immunoreaction. RESULTS: Opaque red lesions were defined as nontransparent lesions, including polypoid excrescence, blood bleb or ecchymosis, and nonopaque lesions were defined as translucent or transparent lesions, including vesicular bleb or clear papule. The women with endometriosis containing dominant opaque red lesions showed significantly higher PF concentrations of HGF, VEGF, IL-6 or MCP-1 than those with either nonopaque lesions or black lesions or control women. There was no difference in any of these cytokine or chemokine levels between women harboring nonopaque lesions and black lesions. Interestingly, PF concentrations of all these markers were significantly reduced in women containing true black lesions and true white lesions after excluding the presence of coexisting red spot from dominant black or white lesions. In a parallel fashion, the immunoexpressions of HGF and PCNA in intact tissue as shown by the Q-H score were significantly stronger in the opaque red lesions than in the nonopaque lesions or black lesions. CONCLUSIONS: Opaque red lesions display a higher activity than with nonopaque red lesions in both PF and intact tissue. This indicates different histogenesis in their generation in the pelvic cavity.

Adult↗

Purpura fulminans secondary to Xanthomonas maltophilia sepsis in an adult with aplastic anemia.

Purpura fulminans is a rare disease characterized by purpura ecchymosis, hypotension, and fever associated with disseminated intravascular coagulation. It often begins as a benign infectious process and subsequently progresses to a severe, catastrophic outcome. It is recognized to originate from congenital or acquired protein C deficiency. We present an unusual case of an adult with Xanthomonas maltophilia sepsis that subsequently developed into purpura fulminans with involvement of the four extremities. We discuss the importance of the protein C system in coagulation homeostasis and its relationship to purpura fulminans.

Anemia, Aplastic↗

Disseminated cellulitic cryptococcosis in the setting of prednisone monotherapy for pemphigus vulgaris.

Cryptococcal infections are seen mainly in immunocompromised hosts. The cutaneous manifestations of infection can include papules, plaques, nodules, vesicles, ulcers, ecchymosis, and, rarely, cellulitis. Cryptococcal meningitis is a life-threatening complication of this disease. Pemphigus vulgaris is a rare auto-immune blistering disease that can also be life-threatening. Treatment of pemphigus commonly entails both corticosteroids and steroid-sparing agents. We present a case of disseminated cryptococcal infection in a patient with pemphigus vulgaris treated with high dose corticosteroids as monotherapy. This case provides an opportunity to discuss the difficulty of managing two potentially mortal conditions in which the treatment of pemphigus vulgaris may exacerbate the disseminated cryptococcal infection.

Antifungal Agents↗

Successful steroid pulse treatment in childhood acquired haemophilia with nephrotic syndrome.

We encountered a 2-year-old boy with acquired haemophilia, which rarely occurs in children, who was complicated with nephrotic syndrome. In mid-August 2001, he was diagnosed to have nephrotic syndrome based on the presence of massive proteinuria and hypoalbuminaemia. Activated partial thromboplastin time (APTT) was normal at 42.4 s at that time. After starting prednisone administration of 2 mg kg(-1) day(-1), the proteinuria disappeared immediately. However, in early October the same year, subcutaneous ecchymosis and intramuscular bleeding occurred for no apparent reason, and from the examination results his APTT was 106.4 s, factor VIII (FVIII) activity was <1%, and the anti-FVIII inhibitor titre was 6.9 BU ml(-1). As a result, he was diagnosed to have acquired haemophilia. The anti-nuclear antibody and anti-phospholipid antibody were negative. With recombinant activated FVII, haemostasis was obtained, and after administering three courses of steroid pulse therapy (methyl prednisolone: 20 mg kg(-1) day(-1) x 3 days), the anti-FVIII inhibitory activity disappeared. An analysis of the immunological and coagulation properties of his FVIII autoantibodies showed the anti-FVIII inhibitory activity to be mediated by IgG(1) antibody. In other words, his FVIII inhibitor was a Th1 dominant and it provided a good response to treatment. These findings correlate with those of previous reports. The patient thereafter frequently demonstrated a recurrence of nephrotic syndrome. As a result, he is presently being managed with cyclosporine. However, no recurrence of the anti-FVIII titre has been observed.

Autoantibodies↗

Main clinical manifestations of a bleeding diathesis: an often disregarded aspect of medical and surgical history taking.

A suitable clinical evaluation of a bleeding diathesis is often forgone. The young doctor is often unprepared to describe in an accurate way the different types of bleeding. An adequate classification and adequate clinical information about a bleeding diathesis are instead of paramount importance. Bleeding may be cutaneous, mucous, articular, muscular, parenchymal, intracavitary, orificial. Each of these sites and forms may have diagnostic implications. An accurate description of the several forms of cutaneous bleeding (petechiae, purpuric spots, ecchymosis, haematomas, etc.) is needed for referrals and for controls. The correct evaluation of cutaneous bleeding manifestations of children (battered child syndrome) is absolutely important for clinical and medico-legal purposes. The same is true for the battering syndrome seen in women abused by their spouses. The grading of haemarthrosis in haemophilia patients is important for the follow-up. A proper description of haematuria is essential in suggesting the probable site of bleeding (kidney or bladder or urethra). A proper evaluation of bleeding may give also useful information on the general health status of the patients (presence of anaemia, poor nutrition, renal insufficiency, etc.). The combination of bleeding and thrombosis in the same patient is also a clinical challenge. The relationship between haemorrhage and thrombosis may be sequential or concomitant. Sequential thrombosis may occur in a patient confined in bed for a brain haemorrhage. Concomitant thrombosis and bleeding occur in DIC and in patients with thrombosis being treated with anticoagulants. Finally, it should be kept in mind that a proper evaluation of the bleeding diathesis of a given patient may help the caring doctor in ordering appropriate laboratory tests (e.g. a platelet count for petechiae, a PTT for a patient with haemarthrosis, etc.).

Abdomen↗

Outpatient penile implantation with the patient under a novel method of crural block.

Although local anaesthesia for penile implants has been substantially reported, its methodology, simplicity and reliability left room for improvement. We would like to report on an innovative penile crural block using local anaesthesia in patients who underwent penile implantation as outpatient surgery. From March 1987 to March 1991, a total of 21 organically impotent men, aged from 27 to 77 years, received penile prosthesis implantation. All these were performed under pudendal nerve block as an outpatient procedure. From August 1992 to January 2003 a proximal dorsal nerve block with peripenile infiltration and penile crural block was developed to replace the anaesthesia method of pudendal nerve blocks in 137 consecutive patients (aged from 35 to 83 years) undergoing penile implants. The anaesthetic effects and postoperative results with the crural block were very satisfactory. Common immediate side-effects included puncture of the vessels, subcutaneous ecchymosis, transient palpitations and dilation pain, but there were no significant late complications. In the group of pudendal nerve blockage, 42.9% patients (nine of 21) experienced severe aching pain over the perineum for 1-2 weeks postoperatively, whereas the newly developed method of crural block markedly reduced these adverse effects. This new anaesthetic method proved to be reliable, simple, and safe with fewer complications. It offers the advantages of less morbidity, preservation of patient's privacy, reduced adverse effects of anaesthesia, and a more-rapid return to activity with minimal complications.

Adult↗