1/44. A morbid course in a girl with mixed connective tissue disease.We describe an 18-year-old girl with a 13-year history of mixed connective tissue disease whose clinical course was unique: she ultimately developed end-stage kidney and bowel disease that led to a protracted morbid clinical course. We report this case to alert pediatric nephrologists to the importance of early recognition of possible intestinal disease in these patients. Unfortunately, no therapy is currently known to reverse the pathological process in the bowel, but multiorgan transplantation might be an option if the numerous medical complications of end-stage bowel disease can be successfully controlled.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/44. Unusual presentation of graft-versus-host disease in pediatric liver transplant recipients: evidence of late and recurrent disease.Graft-versus-host disease (GvHD) is a multi-organ disease caused by mature donor T cells that are activated by alloantigens expressed by the host antigen-presenting cells. GvHD has been reported after solid organ transplantation with two principal presentations: humoral and cellular. In the cellular type of GvHD after liver transplantation the symptoms are identical to the GvHD after bone marrow transplant, except that the liver is spared because it lacks host antigens. We have described three cases of intestinal GvHD after pediatric liver transplant with an unusual recurrent late presentation in two patients. Two patients were female, and their age at the time of transplant was 8 and 9 months, respectively, and one was an 8-month-old male. They all received reduced liver allografts of identical blood type from three different donors. One patient received two doses of donor bone marrow cell infusion. Two patients received double immunosuppressive therapy constituted by tacrolimus at a dose of 0.05 mg/kg p.o. b.i.d. and steroids 10 mg p.o. daily. One patient received a triple drug immunosuppression with tacrolimus (0.05 mg/kg p.o. b.i.d.), steroids (10 mg p.o. daily) and mycophenolate mofetil (125 mg p.o. b.i.d.). diagnosis of intestinal GvHD was confirmed histologically on intestinal biopsies performed at the time of presentation of the clinical symptoms or at autopsy.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
3/44. Laparoscopic surgery after orthotopic liver transplantation.Laparoscopic surgery is currently a widely accepted approach to several surgical fields because of its advantages in terms of postoperative pain reduction and easy patient recovery. This approach may be useful even in solid-organ transplantation surgery as a diagnostic or treatment procedure in some surgical complications. From July 1991 to December 1998, we performed 142 liver transplantations on 129 patients. During the postoperative period, many complications occurred. Here we report two cases of intestinal occlusion caused by adhesions and three cases of lymphocele, all approached with laparoscopic surgery. In all cases but one, we were able to complete the surgery by laparoscopic means; in one of the two occlusions, the procedure was switched to laparotomy because of a choledochojejunal anastomosis lesion. The three cases of lymphocele must be considered in a particular manner because such cases, to our knowledge, have never been described in the literature. They always presented with a late-onset right pleural effusion and were located in the retrohepatic, retrogastric, and left paracaval areas, close to the esophageal hiatus. In conclusion, we believe a laparoscopic approach is a useful strategy to solve some surgical complications in patients who underwent orthotopic liver transplantation; however, the use of laparoscopic surgery in this field is strictly connected to the surgeon's experience and versatility.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/44. Persistent annular erythema of infancy associated with intestinal Candida colonization.We report a case of persistent annular erythema of infancy in a 4-month-old boy. Physical and laboratory parameters showed no sign of internal disease or specific infection except a massive candida albicans colonization (> 103 organisms/mm3) of the lower gastrointestinal tract. Oral treatment with amphotericin b for 2 weeks resulted in a complete remission of the skin lesions indicating Candida colonization as a trigger.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
5/44. Ectopic prostate: case report of a presacral mass presenting with obstructive symptoms.We report the unusual case of a 78-year-old man who presented with obstructive bowel symptoms and a 2.5-cm presacral mass. The mass was excised and found on pathologic examination to be ectopic prostate tissue complete with a muscular stroma. review of the literature revealed a number of case reports describing variably sized fragments of ectopic prostate tissue involving a variety of organs, including spleen, uterine cervix, bowel wall, pericolic fat, anal submucosa, seminal vesicle, testis, and urinary bladder. However, to our knowledge, this case is unique in that it presented as a relatively large, isolated presacral mass causing functional bowel impairment. The ectopic location can be related to the normal embryonic development of the prostate, rectum, and bladder.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
6/44. One-month-old infant with multiple ulcers of stomach, small bowel, large bowel, and protein-losing enteropathy: case report.Multiple inflammatory ulcers of the gastrointestinal tract are rare in young infants. Most cases are caused by infectious organisms, vasculitis, or an autoimmune process. We report a 1-month-old infant who was healthy until he presented with an inflammatory mesenteric cyst, and multiple ulcers of the stomach, duodenum, jejunum, ileum, and colon. Histologically, the ulcerations were sharply demarcated, full thickness, and filled with macrophages. He had a low serum albumin and IgG due to protein-losing enteropathy. He was treated with supportive care and immunomodulating drugs. The gastrointestinal inflammation resolved by 3 and 1/2 years of age. The medications were withdrawn at 5 and 1/2 years of age he had no relapse of clinical symptoms. He continues to have asymptomatic mild hypoalbuminemia and low serum IgG. We could not find a report of a similar clinical presentation and outcome.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
7/44. septo-optic dysplasia and unexpected adult death--an autopsy approach.A 20-year-old woman who suffered from septo-optic dysplasia died unexpectedly following a presumed viral gastroenteritis. autopsy and neuropathological examination confirmed optic nerve hypoplasia with absence of the septum pellucidum. Marked adrenal gland hypoplasia reflected hypothalamic hypopituitarism. The small and large intestines were dilated and filled with liquid fecal material. This case demonstrates that individuals with septo-optic dysplasia may be at risk of unexpected death at all ages. The complexity of mechanisms of death in rare dysmorphic conditions may be overlooked if relevant clinical information is not available at the time of autopsy and unless specific steps are taken to clearly delineate the underlying features of the condition.- - - - - - - - - - ranking = 0.062026815581742keywords = nerve (Clic here for more details about this article) |
8/44. Polyglandular endocrine failure in a patient with amyloidosis secondary to familial mediterranean fever.familial mediterranean fever (FMF) is 1 of the major causes of secondary amyloidosis. Renal involvement is the main clinical complication and it mostly presents with nephrotic syndrome and chronic renal failure. Although deposition of amyloid has been reported in several endocrine glands such as the adrenal, thyroid, and testes, clinically significant functional impairment is uncommon. Herein, we describe a patient in whom the diagnosis of FMF was based on molecular screening and who presented with recurrent hypoglycemic attacks and extensive amyloid deposition affecting various organ function including adrenal, thyroid, parathyroid, testes, intestinal system, and the heart.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
9/44. Disseminated aspergillosis inciting intestinal ischaemia and obstruction.Invasive aspergillosis is an opportunistic infection that characteristically affects the immunocompromised host, resulting in a high degree of morbidity and mortality. Although the portal of entry is usually pulmonary, there are rare reports of invasive aspergillosis localized to the gastrointestinal tract. In addition, haematological spread may develop, with life threatening disseminated infection involving the vital organs and the gastrointestinal tract. Although disseminated infection is well recognized, the CT findings of gastrointestinal disease have not been reported to our knowledge. We describe the CT findings in a patient with invasive aspergillosis involving the gastrointestinal tract, which resulted in intestinal ischaemia complicated by small bowel obstruction.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
10/44. Combination therapy including pentoxifylline for entero-Behcet's disease.Behcet's disease is a chronic relapsing vasculitis characterized by recurrent aphthous oral and genital ulcerations with uveitis. Multiple organs can be involved. Entero-Behcet's disease is often uncontrollable, relapsing, and can cause acute intestinal bleeding or perforation. We utilized a combination therapy including 600 mg of pentoxifylline per day, in two doses, to treat three female patients and observed the subsequent changes in clinical symptoms, serum C reactive protein levels, and endoscopic findings. In all three patients, clinical symptoms as well as serum C reactive protein levels improved immediately. Endoscopically, lower intestinal lesions were significantly reduced or healed in all of them. Combination therapy including pentoxifylline appears to be clinically effective in the patients with entero-Behcet's disease.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
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