Cutaneous polyarteritis nodosa and ulcerative colitis.

Vasculitis in many forms has been reported rarely in association with ulcerative colitis. We report the occurrence of necrotizing vasculitis in a 48-year-old man 19 years after the onset of ulcerative colitis and 5 years after total colectomy with rectal preservation. The disease was limited to small arteries of skin and muscle consistent with the syndrome of cutaneous polyarteritis nodosa that has been reported with regional enteritis but not previously with ulcerative colitis.     

Intensive chemotherapy for peripheral T-cell lymphomas.

Forty-two patients with previously untreated peripheral T-cell lymphomas (PTCL) were treated with an intensive chemotherapy protocol. Either the BACOP or the m-BACOD regimen was used for induction. Patients achieving complete clinical remission after three courses were given intensive consolidation and maintenance chemotherapy similar to the L10/L17M protocol designed by the Memorial Sloan-Kettering Group for acute lymphoblastic leukemia and lymphoblastic lymphoma. There were 27 (64 per cent) males and 15 (36 per cent) females. The median age was 54 years (mean 53, range 15 to 68). Seven of them (17 per cent) had stage I disease, four (10 per cent) stage II, seven (17 per cent) stage III and 24 (57 per cent) stage IV. Eighteen patients (43 per cent) had B symptoms and four (10 per cent) had bulky disease. According to the Working Formulation, the histology was diffuse mixed in 16 patients (38 per cent), diffuse large cell in 18 (43 per cent), diffuse immunoblastic in four (10 per cent) and unclassifiable in four (10 per cent). According to a modified Japanese Lymphoma Study Group's classification, the histology in 24 patients (57 per cent) was the pleomorphic type, in 13 (31 per cent) immunoblastic-lymphadenopathy-like (IBL-like), and in five (12 per cent) unclassifiable. The overall complete remission rate was 67 per cent. Twenty-five per cent of the complete responders relapsed and the DFS of the CR patients was 62 per cent at three years. The overall survival of all patients at three years was 52 per cent. Patients with stage I, II and III disease had significantly better CR rate (100 per cent versus 42 per cent, p = 0.001) and overall survival (82 per cent versus 35 per cent at three years, p = 0.01) than those with stage IV disease but the relapse rate and DFS of CR patients were similar. This study shows that the prognosis of patients with PTCL can be improved by intensive therapy.     

Reversal of diastolic cerebral blood flow in infants without brain death

The reversal of diastolic cerebral blood flow has been regarded as a characteristic waveform of brain death and a useful confirming sign. We report 2 patients who had diastolic flow reversal but survived. One, a 1-month-old boy with status epilepticus, had reversal of diastolic cerebral blood flow detected by Doppler ultrasound soon after admission. Reversal disappeared after medical management for increased intracranial pressure and seizure control. He recovered without sequelae. The other, a 6-month-old girl with choroid plexus papilloma, had reversal of diastolic flow during abrupt clinical deterioration. Emergent surgical removal of the tumor was performed and she survived with hemiparesis and psychomotor retardation. Our patients demonstrated that even in the presence of diastolic reversal of cerebral blood flow, prompt and effective treatment can avoid a fatal outcome.     

Laparoscopic nephrectomy in a porcine model.

Laparoscopic nephrectomy was performed in 15 male pigs, the procedure was successful in 14. Extraction of the intact kidney through a 5-cm lower abdomen incision was done in 7 animals; complete destruction and evacuation of the kidney was accomplished by a round-knife suction device through a 1-cm port in another 7 pigs. Grossly, the specimen consisted of sausage-like tubular renal tissue and a small amount of tissue debris. Pathology revealed that the glomerular and tubular structures were well preserved, no interstitial hematoma could be found. Four ports were usually used, one 1-cm umbilical camera port, one 0.5-cm port for ureter traction, and two 1-cm working ports along the midclavicular line. All the pigs recovered uneventfully. The average operation time was 3 h 20 min. The application of endo-GIA (United States Surgical Corporation) for renal hilum reduced the operative time to 2 h 20 min. Complications included renal vein tear during endoclip application and cutting in the first case, mild subcutaneous emphysema in 2 cases. This first pig received exploratory laparotomy for the repair and ligation of the renal vein. No more major complications occurred with increasing experience. From this porcine experiment, we conclude that the combination of laparoscopy, a tissue destroyer and an endobag for the entrapment of kidney seem to be a promising technique for clinical nephrectomy.     

Metastatic septic endophthalmitis in pyogenic liver abscess

In a consecutive series of 180 patients with pyogenic liver abscess, three patients (two men and one woman, between 46 and 75 years of age) had metastatic Klebsiella endophthalmitis. The incidence of metastatic endophthalmitis was 1.7% in patients with pyogenic liver abscess, 5.2% in patients with Klebsiella liver abscess, and 7.8% in patients with Klebsiella liver abscess having Klebsiella bacteremia. Despite aggressive therapeutic measures, the men permanently lost their vision and the woman eventually required an evisceration of her right eye. Delayed recognition and/or treatment as well as the nature of bacteria probably contributed to the tragic outcome. The findings suggest that a high index of suspicion is critical and a combined effort of the internist and ophthalmologist is mandatory.     

Calciphylaxis – a topical overview

'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis.