A case of purpura fulminans secondary to transient protein C deficiency as a complication of chickenpox infection

Purpura fulminans is a rare but dramatic disease which occurs most commonly during or after an infection. It is characterized by extensive involvement of the skin and extremities and involvement of visceral organs. Purpura fulminans, when occurring after a viral infection such as varicella, is usually characterized by purpuric lesions involving the trunk, usually with sparing of the visceral organs. In this report we describe a child with purpura fulminans due to a transient protein C deficiency as a complication of chickenpox infection. A seven-year-old girl developed bruise-like lesions on her extremities on the fifth day after eruption of varicella exanthem. She had no previous history of bleeding tendency or thrombosis. Family history was also negative. On the seventh day of her illness she was admitted to Marmara University Hospital with widespread echymotic an partially crusted chickenpox lesions. CBC, urinalysis and blood chemistries were within normal limits. She had a prolonged aPT and apt with low serum fibrinogen and high D-dimers suggestive disseminated intravascular coagulation (DIC). Protein C activity was low. Punch skin biopsy was consistent with purpura fulminans. She was treated with heparin and fresh frozen plasma which helped her to recover clinically as well as hematologically. She was discharged with still low protein C activity that returned to normal by the next follow-up visit.     

Hemoperitoneum secondary to the rupture of superficial veins of a uterine leiomyoma

The most frequent cause of gynaecological hemoperitoneum is ruptured ectopic pregnancy. An uncommon cause of hemoperitoneum is rupture of uterine leiomyoma. We report one case of massive intraperitoneal hemorrhage and hypovolemic shock due to ruptured uterine leiomyoma vessels. The patient was treated with myomectomy and the postoperative course was uneventful. These case suggest that massive intra peritoneal hemorrhage associated with uterine leiomyoma should be considered in women with hypovolemic shock and pelvic mass.     

Hyperaluminemia in renal failure: the influence of age and citrate intake

Following the occurrence of aluminum encephalopathy in four patients with chronic renal failure, we studied 34 azotemic patients seen during the same year and five volunteers who took varying combinations of aluminum hydroxide and an alkalinizing citrate (Shohl's) solution. We found that the four encephalopathic cases were older than the 34 azotemic patients (68 years +/- 14 SD, vs 50 +/- 13, p less than 0.05), had a higher mean serum aluminum value (727 micrograms/l +/- 320 vs 92 +/- 73, p less than 0.005), had taken more aluminum hydroxide (5 g/day +/- 0.9 vs 1.6 +/- 1.8, p less than 0.01), and more Shohl's solution (64 ml/day +/- 19 vs 20 +/- 29, p less than 0.01). In all 38 patients the serum aluminum values correlated directly with age (p = 0.01), aluminum hydroxide (p = 0.001) and concomitant citrate intake (p = 0.004). In the five healthy volunteers the 24-hour urinary aluminum excretion increased from a baseline of 22 micrograms +/- 19 SD to 167 +/- 109 (p = 0.05) during aluminum hydroxide intake, rising to 580 +/- 267 (p = 0.01) during the simultaneous intake of citrate and aluminum hydroxide. Corresponding serum aluminum values were 11 micrograms/l +/- 2 SD, 44 +/- 34 (p = 0.1), and 98 +/- 58 (p less than 0.05). Thus citrate seems to enhance aluminum absorption and may cause encephalopathy in patients with chronic renal failure, especially the elderly.     

Gastric cancer and other organ cancer history in the parents of patients with gastric cancer.

A case-control study in a population from the Eastern Black Sea region of Turkey was performed to evaluate the risk of gastric cancer development in patients with gastric cancer reporting gastric cancer or other organ cancer history in their parents. Gastric cancer and/or other organ cancer history in the parents were found in 215 of 1240 patients with gastric cancer versus 73 of 1240 controls (odds ratio (OR) 3.35, P<0.001). The frequency of gastric cancer history among the parents was significantly different between the patients and the controls (148 versus 25, respectively; OR 6.59, P<0.001). The frequency of other organ cancer history was not significantly different between the patients and the controls (63 versus 48, respectively; OR 1.33, P>0.05). The frequency of gastric cancer history was significantly higher than the frequency of other organ cancer history in the parents of the patients (OR 4.51, P<0.001). Gastric cancer history was significantly higher in first- to third-degree relatives of the patients reporting gastric cancer and/or other organ cancer history in their parents than in the controls (OR 14.72, P<0.001). Familial clustering of gastric cancer defined by the presence of at least four cancer cases in family members, including parents, was reported by 12% of the patients. Overall, the result of this study of gastric cancer and other organ cancer history in the parents of patients with gastric cancer may suggest a genetic susceptibility for gastric carcinoma. A high risk of gastric cancer occurred in subjects reporting a gastric cancer history in their parents, and there was a higher predisposition to gastric cancer compared with other organ cancer in relatives and a familial clustering of the disease.     

Peritonitis in peritoneal dialysis patients after renal transplantation

Background. The occurrence of peritonitis in peritoneal dialysis patients after renal transplantation during immunosuppression might increase morbidity and mortality. Hence the timing of catheter removal is still controversial. The associated risk factors of this complication have not been analyzed. Methods. We analyzed, retrospectively, the incidence of peritonitis within 90 days after transplantation, its associated morbidity and mortality, as well as risk factors. From 1980 until March 1995, 238 consecutive kidney transplants in peritoneal dialysis patients were performed. Univariate and multivariate logistic regression analysis were used to identify risk factors for the development of peritonitis. Results. 232 cases (141 men, 91 women) were available for analysis. In 191 patients, the catheter was removed with a mean interval after transplantation of 122 days (range 0-573). Thirty peritonitis episodes with predominantly Staphylococcus aureus (10/30) or Gram-negative bacteria (12/30) were observed. Independent risk factors before transplantation were the total number of peritonitis episodes (P<10^-5), previous peritonitis with S. aureus bacteria (P<10^-5), and male sex (P<0.004). Risk factors after transplantation were technical surgical problems (P<10^-5), more than two rejection episodes (P<0.02), permanent graft non-function (P<0.026), and urinary leakage (P<0.035). Conclusions. Transplantation without simultaneous peritoneal catheter removal is feasible. However, this increases the risk of peritonitis after transplantation. Early catheter removal should be considered seriously in those patients at risk. When peritonitis develops, antibiotic treatment should be directed against Gram-positive as well as Gram-negative bacteria until culture results are available. Keywords: catheter; peritoneal dialysis; peritonitis; risk factors; transplantation      

Solitary plasmacytoma. Apropos of a case of craniofacial involvement]

The authors report the case of a 24 years-old man who had a first tumor in the right maxilla diagnosed as Ewing's sarcoma and treated by chemotherapy and radiotherapy. A second tumor appeared in the skull vault twenty seven months later. The histological diagnosis after removal of the lesion was IgG Lambda plasmacytoma. The revision of the histological cut of the maxillary tumor retained the same diagnosis. The patient is without evidence of local recurrence and systemic diffusion of the disease two years after treatment. The authors insist on the necessity of immunochemistry to establish the diagnosis.     

Proton MR spectroscopy in three children with Tay-Sachs disease

Background: Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM₂ gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. Objective: To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Materials and methods: Three children aged 10, 20 and 21 months were examined. Results: On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. Conclusions: The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease.     

Diagnosis, management, and results of treatment for aortopulmonary window

The aortopulmonary window is a communication between the ascending aorta and the pulmonary trunk in the presence of two separate arterial valves. This uncommon congenital anomaly is reported rarely in the literature. We present here our experience with 16 patients, emphasizing the importance of early closure of the defect by a transaortic approach. We performed surgery on 16 patients over a period of 13 years using a transaortic approach under cardiopulmonary bypass. The median age of the patients at the time of operation was 6.5 months, with a range from 1 month to 11 years. Preoperative pulmonary arterial systolic pressure ranged from 30 to 100 mmHg. Associated cardiac anomalies were present in 7 of the patients, and were repaired at the same stage. The defect was between the ascending aorta and the proximal pulmonary trunk in 13 patients, and between the ascending aorta and the distal pulmonary trunk, with overriding of the orifice of the right pulmonary artery, in 3 patients. For closure, we used a patch of 0.4 mm Gore-Tex in 11, and gluteraldehyde-treated autologous pericardium in 5 of the patients. One patient died during surgery. The mean follow-up period for the surviving 15 patients was 52.2 months, with a range from 12 to 130 months. All the patients were in good condition during the follow-up, and no residual defects have been detected. Aortopulmonary window is a rare congenital cardiac anomaly, which can be repaired with very good operative results if surgery is performed before the development of irreversible pulmonary hypertension. We advise early correction of the defect with a transaortic patch, repairing all associated cardiac anomalies at the time of diagnosis.     

Bone anomalies in von Recklinghausen disease

28 cases of neurofibromatosis are reported. Principals bone anomalies are reviewed. The skeletal manifestations are numerous and varied. These anomalies interest specially cranial vault, spheno-orbital area and vertebral spine. It is imperative to keep in mind skeletal manifestations because neurologic and cutaneous signs can be absent.