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At least 217 records · Page 12Linked to original sources
PubMed2001

Intravesical Jackson-Pratt drain for urinary diversion after augmentation cystoplasty.

PURPOSE: Augmentation cystoplasty has become a primary form of bladder management in children with a noncompliant bladder. Excellent urinary drainage is required for anastomotic healing and the removal of mucous buildup. Suprapubic drainage traditionally involves a Malecot catheter, although poor irrigation and dislodgment of this type of catheter are well-known complications. We report the placement of an intravesical Jackson-Pratt drain for urinary diversion in augmented bladders. MATERIALS AND METHODS: We reviewed our use of an intravesical Jackson-Pratt drain for urinary diversion between 1995 and 1999 in 17 patients. Postoperative catheter drainage and irrigation characteristics were assessed as well as catheter related complications. RESULTS: Average patient age was 13 years (range 3 to 27). The majority of patients underwent ileal (11) or sigmoid (4) cystoplasty and 1 each underwent composite and ureteral cystoplasty. Drains remained in place an average of 27 days (range 6 to 57). All patients had excellent drainage during the postoperative period. Irrigation was subjectively easier than with a Malecot catheter. Average cost of a latex-free Malecot catheter was 2.7-fold that of a Jackson-Pratt drain. No catheters became nonfunctional before removal, although 1 was inadvertently pulled during patient transfer. CONCLUSIONS: A Jackson-Pratt drain provides excellent urinary drainage in patients undergoing augmentation cystoplasty. Multiple openings along the tube seem to improve irrigation in contrast to the single opening in a Malecot catheter, which often aspirates a region of the augmented bladder. The ready availability, decreased cost, ease of irrigation, increased pliability with decreased chance of dislodgment and lack of latex make an intravesical Jackson-Pratt drain a superior choice for augmented neurogenic bladder.

Adolescent↗
PubMedDate not supplied

Bronchial rupture after direct chest trauma in a child.

Tracheobronchial injuries are rare in trauma patients, and most often occur after motor vehicle accidents. Occasionally, other mechanisms cause airway disruption. The pliability of the chest wall in children greatly adds to the differences in injuries when compared with adult trauma patients. We present the case of a six-year old girl with an isolated right-sided bronchial rupture after direct trauma to the chest.

Anastomosis, Surgical↗
PubMed2001

A comparison of dermal and cadaveric pericardial grafts in the modified Horton-Devine procedure for Peyronie's disease.

PURPOSE: Penile curvature due to Peyronie's disease is often treated with plaque incision or excision and grafting. The advantages of various graft materials have long been debated. We studied the outcomes of dermal and cadaveric pericardial grafts after plaque incision or excision for Peyronie's disease. MATERIALS AND METHODS: We retrospectively evaluated all modified Horton-Devine procedures performed between February 1998 and April 2000. Dermal graft harvested from the lower abdomen or commercially available cadaveric pericardium was used to cover the corporeal defect. A followup telephone questionnaire was administered to determine patient satisfaction and function. RESULTS: We reviewed the records of 27 men, including 18 and 9 who received a dermal and pericardial graft, respectively. We successfully contacted 24 patients, including 15 with a dermal and 9 with a pericardial graft, to evaluate satisfaction. Average postoperative followup was 10 months. Of the 24 patients, 22 reported successful coitus and 15 (63%) needed assistance to achieve erection rigid enough for satisfactory coitus. Eight patients reported recurrent curvature, which was minimal and did not hamper sexual function. Overall 11 of the 15 patients with a dermal and 8 of the 9 with a pericardial graft would undergo the surgery again or recommend it to others. CONCLUSIONS: The results of the dermal and cadaveric pericardial grafts were comparable. The minimal preoperative preparation, decreased patient morbidity and pliability of cadaveric pericardium make it an attractive suitable graft substitute in the modified Horton-Devine procedure. Longer followup and larger patient series are needed to confirm the advantages of cadaveric pericardium.

Adult↗
PubMed1998

[Direct vision mitral valve reconstruction: a long-term follow-up of 46 cases].

OBJECTIVE: To improve the long-term results of direct vision mitral valve reconstruction. METHOD: From July 1983 through July 1995, 46 patients with mitral valve disease were treated by a direct vision reconstructive techniques. Rheumatic valve stenosis and incompetence were noted in 39 (patients), nonrheumatic insufficiencies in 5, and congenital disease in 2. According to leaflet pliability, type I normal leaflet motion was notedin 17 patients, type II leaflet prolapse in 3 and type III restricted leaflet motion in 29. Cardiac function (NYHA classification) showed class II in 2, class in 25 III, and class IV in 19. Valvuloplasty and annuloplasty were performed with various techniques according to the pathanatomical changes of the valve. Prosthetic obturators self made were employed in 29 patients. RESULT: The operative mortality rate was 6% (3/46). In the mitral valve replacement group it was 10% (27/265). Two to 10 years (mean 65 months) follow-up showed grade I cardiac function in 6 patients, grade II in 17, grade III in 8, and grade IV in 3. CONCLUSION: Good long-term results can be achieved by valve reconstruction with lower mortality and incidence of thromboembolism than.

Adolescent↗
PubMed2002

[Tracheostomy in pediatrics patients].

This is a case study of 79 children who under-went a tracheostomy with three different techniques, used in succession on three different groups of patients: surgical, percutaneous with progressive probes and translaryngeal technique. The pediatric patient, only under the age of 10, starts showing clear-cut anatomical differences compared to an adult, which gradually become more marked the younger the patient is. The causes of increased difficulties that can normally be found in these patients are obviously due to the reduced diameter of the airway and, above all, the high degree of pliability of the cartilaginous frame. After the discovery that even minimal external pressure can cause the collapse of the tracheal walls, it was decided to adopt the systematic use of a rigid bronchoscope, in order to be able to offer internal support to the anterior wall. The following advantages were obtained in the various techniques: In surgery it permitted the reduction, through the protrusion of the trachea, of the size of the operatory field, of the tracheal opening and consequently of the local trauma. It also caused less bleeding and reduced the risk of lesion to the pleural dome which are very frequent when a bronchoscope is not used. In the percutaneous method the bronchoscope allowed the application of the technique without complications, even in infants of only a few months' old, because it eliminated tracheal squashing, caused by the introduction of the needle, dilators and especially cannula, and the relative danger of lesion to the posterior wall of the trachea. This complication which was always impending in the original technique, which does not involve the use of a rigid bronchoscope, is the main reason which lead to the ban on PDT for patients younger than 16-18. In the translaryngeal method the advantages are of minor importance because they are limited to the initial stage of the procedure, the introduction of the needle and guide wire which are quicker and more precise. As regard the dilation, the TLT mode has a high level of intrinsic safety and is perfectly suitable to the anatomy of the child. In fact it is carried out from the inside towards the outside, causing a tendency to evertion of the tracheal wall which the physician must even limit with external pressure. It is remarkable that this contrast in pressure and the resulting compression of the peristomal tissues creates advantages, which are essential in younger children, that cannot be found in other tracheal techniques. These advantages are the complete absence of loss of blood, minimal local trauma and a perfect adherence of the stoma to the cannula, particularly effective at level of the tracheal wall.

Adolescent↗
PubMed2002

Speech and swallowing function after reconstruction with a radial forearm free flap or a pectoralis major flap for tongue cancer.

BACKGROUND AND PURPOSE: The tongue plays a more significant role in English than in Mandarin, both in apical-palatal consonant production and tense-lax distinction. Theoretically, the same surgical intervention may produce a less significant impact on postoperative Mandarin production. The impact of tongue reconstruction on Mandarin articulation has not been reported. This study compared the tongue function outcome obtained using two methods of tongue reconstruction, radial forearm free flap transfer and pectoralis major flap transfer. METHODS: Twenty-five patients with carcinoma of the tongue underwent tumor resection. The surgical defects were reconstructed using a pectoralis major flap in six patients and a radial forearm flap in 19 patients. Swallowing and speech function were evaluated 6 months to 5 years after the reconstruction. Speech intelligibility and a Mandarin articulation test were used to evaluate the articulation proficiency before and after surgery. Clinical evaluation of deglutition included a questionnaire on dietary habits and a swallowing rating of 1 to 7. RESULTS: Clinical evaluation showed that patients with free flap reconstruction had more intelligible speech (p = 0.014) even after total glossectomy. Assessment of data obtained by clinical questionnaire showed no significant difference between the two groups in swallowing function. Motility due to flap pliability increased speech intelligibility but had little effect on swallowing function. CONCLUSION: Our results suggest that radial forearm flap transfer is better than pectoralis major flap transfer in preserving speech function and that there is no significant difference between the two methods of reconstruction in their impact on swallowing function.

Deglutition↗
PubMed2002

[Development of better tolerated prosthetic materials: applications in gynecological surgery].

UNLABELLED: Meshes have come to be widely used for surgical repair of the dysfunctional pelvic floor. The problem to date has been mesh intolerance. History. The first meshes were made with silver filigrees or stainless steel. Non-metallic and non-absorbable synthetic prostheses include nylon, silastic, polytetrafluoroethylene as well as expansive polyester and polypropylene forms. Most of the absorbable prostheses are made of polyglycolic acid and polyglactine 910. Classification. Four groups of biomaterials can be described according to pore size. Mechanical and biological properties. The mechanical properties of meshes have been tested industrially for resistance, pliability, elasticity and ductile qualities. These properties depend on type of tissue structure (woven or knitted) and the type of fiber used (mono and multi-filaments). The goal is to obtain a "silent" material, i.e. a material which does not trigger a host tissue reaction. Introducing the foreign body induces a "scarring" response. This fibroblastic reaction replaces the inflammatory reaction, leading to progressive colonization of the prosthesis. The major risk is infection caused by a disturbance of the inflammatory phase and bacterial development. Bacteria can be trapped in fibrotic tissue, with the risk of delayed infection. Immunological reactions may have an additive effect. These problems are not encountered with absorbable meshes. An ideal implant material must: not undergo physical modification by tissue fluids, be chemically inert, not trigger inflammatory or foreign body cell response in body tissues, be noncarcinogenic and nonallergenic, be capable of resisting mechanical stress and sterilization, and be able to be manufactured in the necessary shape. Polyester, polypropylene and expansive polytetrafluoroethylene fulfill these criteria. The ideal mesh. Eleven criteria are proposed. Complications for hernia repair. Infection and seroma are the most frequent complications with micro-porous meshes. Macro-porous meshes can cause erosive phenomena and adhesions. Retraction of synthetic tissues is observed in 20 to 30% of cases. Meshes in gynecology. In gynecology surgery, meshes made their first appearance in trans-abdominal sacrocolpopexy and slings. A detailed review of complications found in 32 articles studying slings and 22 studying sacrocolpopexy with approximately 10 types of meshes shows that intolerance of slings has oscillated between 1% with Prolene and 31% with Gore-Tex; for abdominal sacrocolpopexy the rate was between 1.7% with Prolene and 20% with Teflon. Rejection phenomena appear during the first year and are proportional to the surface area of the synthetic tissue and the proximity of the vaginal scar. New materials have been proposed over the last ten years for prolapse surgery, notably for cystocele, which accounts for 70% of all repair procedures. Nearly fifteen studies have reported a level of intolerance reaching 6%, the large majority of the meshes used being Prolene meshes. Our personal experience with 87 repair procedures has led us to the conclusion that Prolene is the most adapted mesh, allowing free tension between the bladder and the anterior vaginal wall. CONCLUSION: Continuous evaluation is needed to study these replacement materials which should in theory, improve the rate of recurrence, which is at present 20% with classic procedures not using a mesh.

Absorbable Implants↗
PubMed2001

[Study on vibration mode of different phonatory source and compensation after partial laryngectomy].

OBJECTIVE: To investigate the nature of pathological voice production and compensatory mechanism after partial laryngectomy. METHODS: Vocal function of 93 cases following partial laryngectomy (28 cases of horizontal partial laryngectomy, 35 vertical partial laryngectomy, 30 horizontal-vertical partial laryngectomy) were examined by acoustic analysis, aerodynamic analysis and videostroboscopic examination. RESULTS: Horizontal supraglottic laryngectomy(HL) resulted in slight dysphonia after operation. The cover of vocal fold was hypertrophic and edematous. Mucosa wave was increased. Acoustic analysis showed significant difference between normal and horizontal laryngectomy groups(P < 0.05). Vertical laryngectomy(VL) resulted in moderate-severe dysphonia. Acoustic analysis was significantly worse in VL than in normal group(P < 0.01). The nonglottic phonatory source was the contralateral ventricular fold or contralateral vestibular mucosa (arytenoid mucosa, root mucosa of glottis) to approximate the reconstructed flap. It was noted that contralateral vocal fold did not participate in vibration. Horizontal-vertical partial laryngectomy (HVL) resulted in moderate-severe dysphonia. Acoustic analysis of HVL was significantly worse than that of normal phonation(P < 0.01). The site of compensatory mucosa vibration upon phonation was the contralateral vocal fold or contralateral hypertrophic arytenoid mucosa. The thinner the flap, the better the vocal quality was. Vocal quality of VL was worse than that of HVL in regard to shimmer, jitter, normalized noise energy, maximal phonation time and harmonic-noise ratio, but there was no significant difference between them. CONCLUSIONS: Partial laryngectomy has different vibrating compensatory modes: Voice function of horizontal partial laryngectomy was the best as it preserved the normal vibration mode; vertical partial laryngectomy was the worst with the nonglottic vibrating source of reconstructed flap-ventricular fold. The reconstructed flap, ventricular fold, mucosa of epiglottis and arytenoid take predominantly part in neoglottal vibration. The situation, volume and pliability characteristic of reconstructed flap was also important to vocal quality.

Aged↗
PubMed1992

[Influence of systolic left ventricular blood flow direction on genesis of senile calcification of the aortic valve].

To assess the factors which may initiate and accelerate degenerative senile calcification of the aortic valve, two-dimensional echocardiograms and the clinical characteristics of 259 consecutive cases with senile calcification of the aortic valve were studied. The results were compared with those of similar studies among 186 consecutive cases with the normal aortic valves. An aortic cusp with an area of increased echo greater than 3 mm in width and with decreased pliability was regarded as calcified. Among patients with calcification of one aortic cusp, 114 exhibited calcification of a noncoronary cusp, 17 calcification of the left coronary cusp and 3 calcification of the right coronary cusp (p < 0.001). Among patients with calcification of 2 aortic cusps, 39 had calcification of a noncoronary and left coronary cusps, 3 calcification of the left and right coronary cusps and 16 calcification of the right and noncoronary cusps (p < 0.001). In patients with calcification of their aortic valves, the end-diastolic angle between the interventricular septum and the ascending aorta was 102 +/- 10 degrees; whereas, it was 89 +/- 10 degrees in the control group (p < 0.001). There were no differences in frequency of aortic root calcification, mitral annular calcification, hypertension, ischemic heart disease, hyperlipidemia, hyperuricemia, or hyperglycemia, between patients with and without calcification of their aortic valves. Of the female patients ranging in age from 65 to 74 years, 88% in those with calcification of 3 cusps and 30% in those with calcification of one cusp (p < 0.05) had mitral annular calcification.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1964

PNEUMATOSIS CYSTOIDES INTESTINALIS INVOLVING THE LEFT SIDE OF THE COLON.

Pneumatosis cystoides intestinalis involving the left side of the colon has been infrequently described. The condition was recognized 234 years ago when it was discovered in a cadaver. It was not until 1946, however, that a description of this lesion appeared in the radiological literature of North America. Pathologically, it is characterized by the presence of subserosal or submucosal cysts, varying from one millimetre to several centimetres in diameter. The complications of this process are pneumoperitoneum and intestinal obstruction. Three patients are described, the first of whom had partial bowel obstruction. Radiolucent gas-filled cysts along the bowel wall were observed on the radiographs. An additional radiological sign noted by the authors was fixation and elongation of the sigmoid colon with loss of pliability of the bowel wall which persisted on the evacuation film.

Barium Sulfate↗
PubMed1992

Direct imaging of the tricuspid valve annular motions by fiberoptic cardioscopy in dogs with tricuspid regurgitation. II. Does flexible ring annuloplasty preserve the annular motions?

Applying the technology of direct imaging by fiberoptic cardioscopy in a working-heart condition, we studied the tricuspid valve annular motions with flexible rings in nine dogs with chronic tricuspid regurgitation. Three annuloplasty studies with a totally flexible polytetrafluoroethylene ring, an elastic silicone ring, and a rigid metal ring were compared with a nonannuloplasty study. The annuloplasty was performed by reducing tricuspid valve annular area to 65% of that of nonannuloplasty condition. The anuli were observed and recorded on a videotape in real time. The three rings effectively reduced and remodeled the dilated anuli and improved the valve coaptation. The patterns of annular motions with the polytetrafluoroethylene and silicone ring were similar to that of normal anulus; the free wall anulus contracted centripetally, and the septal anulus moved toward the free wall side during systole. Pliability of both the anteroseptal and posteroseptal commissures with the flexible rings made these motions possible. The polytetrafluoroethylene ring followed a natural undulation of the anulus, whereas the silicone and rigid metal rings forced the annular planes to horizontal ones. Percent reductions of tricuspid valve annular area were 25%, 21%, and 23% in the nonannuloplasty, polytetrafluoroethylene, and silicone ring studies, respectively, without significant differences in annular contraction. In contrast, rigid metal rings completely disturbed the annular motions. These findings indicated that the two flexible rings did preserve the physiologic annular motions to the degree that the anuli had in the chronic tricuspid regurgitation condition. Especially, the totally flexible polytetrafluoroethylene ring preserved not only the annular motions but also the natural undulation, which resulted in reinforcing the valve coaptation. We believe that the flexible ring, especially a totally flexible one, is superior to the rigid ring in the aspect of reinforcing the valve coaptation to prevent further regurgitation.

Animals↗
PubMed1992

Gunshot wounds to the face. Current concepts.

The primary goal in reconstructing a gunshot wound to the face is identical to that for a major injury of the hand or lower extremity: primary healing of the soft tissue. This prevents the cascade of soft-tissue infection, further delay in healing, bone graft infection, and soft-tissue contracture. Current craniofacial techniques and methods of fixation allow for concomitant bony reconstruction without compromising primary healing of the soft tissues. The result is not only that the soft tissue is healed but also that the original contour, stretch, and pliability are maintained. Once the soft tissue collapses around inadequate bony reconstruction, it is difficult to restore the original situation. Despite the techniques outlined, there is no substitute for sound surgical judgment. Inevitably, cases will arise when the ideal reconstruction cannot be performed because of other injuries, inappropriate initial management at referring institutions, or medical complications. Compromises are then required in the interest of the patient's overall care, with the knowledge that the best chance to restore the anatomy of the hard and soft tissue is in the first few days after the injury.

Bone Transplantation↗
PubMed2005

Axial pattern composite prefabrication of high-density porous polyethylene: experimental and clinical research.

Currently, various alloplastic materials are being used for reconstruction of three-dimensional structures, and high-density porous polyethylene is so far the best and the most commonly used material. Various indications for high-density porous polyethylene have been defined for closure of craniofacial defects, correction of congenital anomalies, and aesthetic augmentations. A common property of various studies published so far is that after being fixed to the bone or underlying structures, high-density porous polyethylene has been covered primarily or by skin flaps. For reconstruction of complex three-dimensional structures such as the ear and nose, the success of current methods is limited by the thinness and pliability of the skin flap. In this study, the authors' aim was to investigate the graftability of high-density porous polyethylene after prefabrication with an axial pedicle and to explore possible clinical applications in light of the new data obtained. In the experimental study, three-dimensional implants (rectangular prism) carved from high-density porous polyethylene were prefabricated using bilateral superficial epigastric arteries and veins of 25 New Zealand rabbits. After a waiting period of 2 to 6 weeks in five groups, control samples were obtained and the prefabricated implants that had been left in place were directly grafted. The results showed that high-density porous polyethylene was vascularized 75 percent after 4 weeks and 90 percent after 5 weeks, and 95 percent of the grafts had survived after 8 weeks. In the clinical study, three nose defects, three ear defects, and one hard palate defect in seven patients ranging in age from 21 to 72 years were reconstructed using the same method. High-density porous polyethylene has been prefabricated and directly grafted for the very first time on a clinical basis. No serious complications have been observed, except for minimal graft loss in two patients. It is obvious that full-thickness skin grafts that are thinner than flaps will adapt better to the fine details of high-density porous polyethylene and will highly increase the detail obtained in the reconstruction of three-dimensional defects.

Abdomen↗
PubMed1992

The trapezius myocutaneous flap.

The three trapezius myocutaneous flaps remain valuable adjuncts in head and neck reconstructive surgery. Overall, the lower vertical trapezius flap has the most versatility and reliability, and the least morbidity. There are several advantages of this flap compared to the pectoralis myocutaneous (PMC) flap. It has a thin vascular pedicle, and thus does not add excessive bulk to the neck, as the PMC can. Also, the skin island has less subcutaneous tissue and therefore more pliability than the PMC, which is a definite advantage for oral cavity and oropharyngeal defects. The trapezius flap generally has less hair than the PMC flap, and the donor scar is better hidden.

Humans↗
PubMed2005

The identification of Isopar H in vinyl flooring.

Vinyl flooring manufacturers use plasticizers to decrease the viscosity and increase the pliability of vinyl. Several ignitable liquid plasticizers used in the manufacture of vinyl flooring were identified and investigated in this study. Twenty-nine collections from five major vinyl manufacturers, a total of 72 samples, were analyzed using passive headspace concentration in accordance with the American Society for Testing and Materials (ASTM E 1412-00) and gas chromatographic-mass spectrometric (GC-MS) analysis as described in ASTM E 1618-01 (1,2). Norpar products and TXIB (2,2,4-trimethyl-1,3-pentanediol diisobutyrate) are ignitable liquids common to the manufacture of vinyl flooring and were identified in all recently obtained samples. Isopar H is an ignitable liquid found in various products such as charcoal starters, copier toners, and some solvents (2). Of the 29 collections analyzed, Isopar H was only identified in Armstrong's Interflex-Traditions pattern.

Journal Article↗
PubMed2005

Use of a galeopericranial flap for the reconstruction of anterior cranial base defects.

BACKGROUND: To evaluate the efficacy of using a galeopericranial flap for reconstruction of anterior cranial base defects. METHODS: In Linkou Chang Gung Memorial Hospital from February 1994 to November 2003, 25 patients who had tumors of the skull base underwent craniofacial resection, and a galeopericranial flap was used to reconstruct the anterior cranial base defect. The galeopericranial flap was developed and based on at least 1 side of the supraorbital or supratrochlear arteries and veins; it was only raised from the scalp after the tumor had been removed. It was then turned inwardly and intracranially and was transposed to lie between the skull base bone and the dura. No free skin or bone grafts were used. RESULTS: Two patients experienced flap failure, and salvage was subsequently performed using a free vastus lateralis flap for skull base repair. Thus, the flap failure rate was 8% (2/25). CONCLUSIONS: The delicate nature and great pliability of a galeopericranial flap make it unique and competent for reconstructions of anterior skull base defects. Its ready availability, valid strength, and sufficient axial blood supply provide a satisfactory barrier for isolating the cranial cavity from the underlying respiratory tract, with minimal morbidity and mortality.

Adolescent↗
PubMed2005

[Experimental study of bFGF modulating rabbit articular chondrocytes cultured in vitro and seeded onto polylactic acid scaffold coated with different materials].

OBJECTIVE: Culturing rabbit articular chondrocytes in vitro and seeding on polylactic acid (PLA) coated with lecithin and poly-l-lysine modulated by bFGF to find a suitable method for cartilaginous tissue engineering. METHODS: The articular chondrocytes were isolated enzymatically from the articular cartilage of young rabbits, and cultured in vitro. Collecting the chondrocytes of the third passage and seeding on three-dimensional scaffold of polylactic acid coated with lecithin and poly-l-lysine. At the same time, basic fibroblast growth factor (bFGF) was added. Proceeding series of detections when the cell-scaffold complexes were cultured more than two weeks, such as macroscopic, invert microscope, light microscope, scanning electron microscope and immunohistochemistry of collagen II. RESULTS: The cell-scaffold complexes modulate by bFGF could not only keep their original shapes, but also maintain the stable homogeneous three-dimensional distribution of chondrocytes without cell falling during the cultivation. At the same time, the complexes were gradually decreasing the consistency, however, increasing the Pliability with elasticity and lubrication surface. After the second week, the complexes were gradually reorganized into the mature engineered cartilage with typical cartilaginous histological structure with rich collagen II. CONCLUSION: bFGF can facilitate the regeneration and maturation of tissue-engineered articular cartilage.

Animals↗
PubMed1991

A 15-year prospective study of treatment of rapidly progressive systemic sclerosis with D-penicillamine [see comment].

A 15-year (1973-1988) prospective study was conducted to determine the effectiveness of D-penicillamine (D-pen) in the treatment of rapidly progressive systemic sclerosis (SSc) of recent onset. Sixty-nine consecutive patients fulfilling strict criteria for rapidly progressive diffuse SSc of less than 18 months of duration were enrolled. Sixty received at least 750 mg/day of D-pen for at least 6 months, whereas 9 did not complete 6 months of treatment because of toxicity, noncompliance or death. In 58 of the 60 patients treated for longer than 6 months, there was an arrest in the progression of skin sclerosis followed by regression with softening, increased pliability and reappearance of sweating and hair. In these cases, the extent of sclerotic skin decreased from a maximum of 64.6 +/- 23.1% total body surface to 15.7 +/- 13.2%. In addition, in the group of patients that received D-pen for longer than 6 months, SSc renal disease was uncommon and pulmonary involvement was not progressive. The overall survival in this group was 88.3%. In conclusion, our prospective study showed that the administration of D-pen resulted in significant improvement of skin sclerosis and in prolonged survival of patients with early, rapidly progressive SSc with diffuse cutaneous involvement.

Adolescent↗