SYSTEMIC SCLEROSIS OCCURRING IN A PATIENT WITH ADAMANTIADES-BEHcET'S DISEASE

We report a patient who developed SS while being treated withazathioprine for ABD. This is a hitherto unreported associationbetween the two conditions. KEY WORDS: Scleroderma, Behçet's disease, Systemic sclerosis     

LIFE-THREATENING HEMORRHAGE IN A PATIENT WITH GASTRIC CANCER AND ACQUIRED HEMOPHILIA

We report a case of a patient with life-threatening hemorrhage caused by the presence of acquired factor VIII inhibitors after gastrectomy for signet-ring cell carcinoma of the stomach. Acquired factor VIII inhibitors should be taken into consideration as a cause of acquired bleeding tendency among patients with gastrointestinal malignancies especially when the coagulation tests are unusual.     

AEROMONAS SOBRIA INFECTION WITH SEVERE MUSCLE DEGENERATION IN A PATIENT WITH ALCOHOLIC LIVER CIRRHOSIS

A 56-yr-old male who had been followed for alcoholic liver disease was admitted for abdominal pain and a high fever. Gastrointestinal endoscopy revealed bleeding esophageal varices that were treated by endoscopic sclerotherapy. Blood culture on admission was positive for Aeromonas sobria . Then skin bullas and ulcers and severe muscle degeneration developed. The patient died despite extensive treatment with antibiotics. A. sobria infection in patients with liver cirrhosis is rare.     

BENEFICIAL EFFECT OF ORAL MESALAZINE POWDER ADMINISTRATION ON GASTRIC INVOLVEMENT OF BEHÇET's DISEASE

Behçet's disease (BD) is a multisystem immune‐mediated inflammatory disorder that involves the gastrointestinal tract. Although intestinal BD chiefly affects the ileocecum and colon, it may involve the entire alimentary tract. Upper gastrointestinal involvements of BD are not common, and its treatment has been rarely reported. Mesalazine is known to be an effective anti‐inflammatory agent for treatment of distal intestinal lesions of inflammatory bowel disease by its delayed drug release system. Here we show a case of chronic active, steroid‐dependent BD involving the upper gastrointestinal tract who was treated with ground mesalazine powder. Endoscopy performed 8 weeks after the treatment showed marked improvement of the gastric ulcers. This is the first report of intestinal BD treated with oral administration of mesalazine powder, and our results suggest that it may be a beneficial and effective new therapy for the upper gastrointestinal lesion of BD.     

ORBITAL PSEUDOTUMOUR AND LACRIMAL INVOLVEMENT DEVELOPING IN A PATIENT WITH RIEDEL'S STRUMA RECEIVING STEROIDS

We present a patient with hypothyroidism secondary to Riedel's struma, treated with thyroxine and prednisolone, who developed clinical and radiological features of a unilateral orbital pseudotumour with lacrimal gland involvement; the histology of which was indistinguishable from that of the thyroid gland.     

ADDISON'S DISEASE PRESENTING AS A HYPERCALCAEMIC CRISIS IN A PATIENT WITH IDIOPATHIC HYPOPARATHYROIDISM

A patient with idiopathic hypoparathyroidism complicated by Addison's disease is described. The development of adrenal insufficiency was accompanied by a hypercalcaemic crisis. The hypercalcaemia appeared to result from a reduction in the filtered load and enhanced renal tubular reabsorption of calcium. Cortisone therapy was followed by the characteristic renal tubular leak of calcium associated with hypoparathyroidism and a return of the serum calcium to normal. The possible mechanisms for these changes in calcium homeostatis are discussed.     

SMALL EARLY GASTRIC CANCER OCCURRING IN A YOUNG WOMAN WITH NODULAR GASTRITIS

We found a small gastric cancer in a 25‐year‐old woman with nodular gastritis. Endoscopically, the cancer was identified as a whitish area in the gastric antrum. There was also a miliary pattern in the gastric antrum and corpus. In addition, serology and histology revealed the patient to have been infected by Helicobacter pylori. Histological examination of the resected stomach showed that the cancer was poorly differentiated adenocarcinoma with signet‐ring cell restricted to the mucosal layer. In the surrounding mucosa, there were chronic inflammatory cell infiltrates and enlarged lymphoid follicles with germinal centers. Our case suggests that nodular gastritis may be at a high risk for the development of gastric cancer of poorly differentiated type.     

PITUITARY PRO-OPIOMELANOCORTIN-CELL CARCINOMA OCCURRING IN CONJUNCTION WITH A GLIOBLASTOMA IN A PATIENT WITH CUSHING''S DISEASE AND SUBSEQUENT NELSON''S SYNDROME

Pituitary carcinoma is defined as a malignant pituitary tumour associated with blood- or lymph-borne metastases. Cushing's disease is frequently present in patients with this condition. After adrenalectomy for Cushing's disease, a 37-year-old man developed Nelson's syndrome resulting from a pituitary carcinoma with metastases to the spinal cord, cauda equina, heart, liver, and pancreas. The primary tumour and its metastases showed immunocytochemical staining for ACTH, beta-lipotrophin, and variably for beta-endorphin and alpha-melanocyte stimulating hormone (alpha-MSH). A coincidental glioblastoma was also present. Nine cases of Cushing's disease associated with pituitary carcinoma, including the present patient, are documented in the literature. The case reported is only the second in which immunohistochemical staining of the primary pituitary tumour and its metastases was performed, and the first in which ACTH-related peptides, in addition to ACTH itself, were demonstrated in the carcinoma cells.     

ARTICULAR INVOLVEMENT IN EUROPEAN PATIENTS WITH LYME DISEASE. A REPORT OF 32 ITALIAN PATIENTS

Lyme disease (LD) is a well recognized multisystem disorder,caused by the spirochaete Borrelia burgdorferi. It involvesthe skin, nervous system and heart. Arthritis is described in50—60% of American patients but seems to be less commonin Europe. We studied the pattern of articular involvement ina group of 67 Italian patients with LD. Thirty-two (48%) of67 had rheumatological manifestations. The prevalence of arthritisin our patients was 16/67 (24%), the remaining 16 experiencingonly arthralgia. The pattern of articular involvement did notdiffer from the literature and was most often mono- or oligoarthritiswith polyarticular arthralgia. We noted an early onset of articularsymptoms (range 1—3 months). Incidence of early and latemanifestations of LD were in keeping with previous reports.In addition, interpreting serological tests for antibodies againstB. burgdorferi and the real prevalence of arthritis in LD iscomplicated by the possible existence of sero-negative LD andby the effect of early antibiotic treatment KEY WORDS: Lyme arthritis, Infectious arthritis, Borrelia burgdorferi, North America, Incidence, Prevalence, Comparison, Antibiotics     

A BROMOCRIPTINE-RESPONSIVE CORTICOTROPH ADENOMA SECRETING α-MSH IN A PATIENT WITH CUSHING''S DISEASE

Clinical and in-vitro investigations have been performed on a corticotroph adenoma removed from a patient suffering from Cushing's disease. Prior to surgery, the patient's Cushing's disease had been successfully controlled, clinically and biochemically, by long term administration of bromocriptine. After selective adenomectomy, tumour tissue was investigated by a perfused isolated cell column technique. It was shown that the tumour cells secreted immunoreactive- (IR)- ACTH and IR-alpha-MSH and that the release of both peptides was promptly suppressed by dopamine. Chromatographic analysis of the secreted IR-alpha-MSH revealed a high proportion of acetylated alpha-MSH; smaller amounts of desacetyl alpha-MSH and diacetyl alpha-MSH were present. The relevance of these findings to the proposal that certain corticotroph adenomas are derived from the intermediate lobe of the pituitary is discussed. It is concluded that there is little direct evidence for involvement of the residual zona intermedia of the adult human pituitary in the development of Cushing's disease.     

INTRAVENOUS PULSE METHYL PREDNISOLONE IN THE SUCCESSFUL TREATMENT OF SEVERE SARCOID POLYNEUROPATHY WITH PULMONARY INVOLVEMENT

Abstract: :Severe widespread sarcoid polyneuropathy with associated pulmonary involvement was treated successfully in a 26 year old man using 'pulse' methyl prednisolone. After he had failed to respond clinically to a two months course of high-dose oral prednisolone, a regimen of intravenous methyl prednisolone 1 g once a week for eight weeks was instituted, along with 10 mg prednisolone orally daily. Neurological improvement began after the third dose with complete remission achieved after the eighth week. More than 12 months after cessation of treatment he remains in good health with no clinical or laboratory evidence of sarcoidosis.