Eosinophilic gastroenteritis

Eosinophilic gastroenteritis is a rare clinicopathogenic entity of an unknown aetiology in which a variety of digestive symptoms are associated to the eosinophilic infiltration in the intestinal wall. The clinical features depend on the site of eosinophilic infiltration, and the association with peripheral eosinophilia is very frequent. The radiologic findings were not specific and the diagnostic is based on the endoscopic and/or peroral gastrointestinal biopsy. The response to steroid therapy is spectacular. The etiology is unknown but we review the recent literature.     

Primary biliary cirrhosis associated with idiopathic thrombocytopenic purpura

A case of primary biliary cirrhosis (PBC) associated with idiopathic thrombocytopenic purpura (ITP) is reported. The patient is a 59-year-old man. When he was 49 years old, he was diagnosed with ITP and received steroid therapy that successfully increased platelet numbers. However, the steroid therapy failed to normalize the elevated gamma-glutamyl transpeptidase. Ten years after this episode, he suffered from general itching and malaise and exhibited a gradual increase of serum biliary enzyme levels. Immunologically, IgM was increased and anti-mitochondrial antibody was positive. Histological findings of liver needle biopsy showed chronic non-suppurative destructive cholangitis, confirming the diagnosis of PBC. To date, very few PBC cases associated with ITP have been reported. Our case is the second one in Japan. PBC and ITP in our patient seemed to develop simultaneously, but the effect of steroid therapy on the two conditions was different. This result suggests that the autoimmune process may have been different in PBC and ITP in the present patient.     

Axotomy increases CNTF receptor mRNA in rat spinal cord

Chronic eosinophilic pneumonia (CEP) is a rare disorder of unknown etiology characterized by striking systemic and pulmonary manifestations such as fever, weight loss, blood eosinophilia, characteristic fluffy peripheral opacities on chest radiograph, and a prompt response to corticosteroid therapy. While the initial phase has been well documented, there is very limited information concerning the long-term natural history and treated course of this condition. We report the clinical and laboratory findings together with the long-term follow-up data on 12 patients with classic CEP who were followed up for a mean of 10.2 years (range, 4 to 13 years). The most striking feature of the long-term follow-up was the occurrence of relapses of CEP (often on multiple occasions) when corticosteroid therapy was discontinued or the dose was tapered. In those nine patients in whom steroid withdrawal was commenced, there was a clinical, hematologic, and radiologic relapse in seven (58 percent). However, prompt reinstitution of therapy led to a rapid resolution of symptoms. By contrast, two patients (17 percent) showed no evidence of relapse when steroid therapy was discontinued. A further three patients (25 percent) are maintained on a regimen of low-dose steroid therapy with no episodes of relapse. Reassuringly, all 12 patients are well at the end of a long period of follow-up. These data suggest that the long-term prognosis for patients with CEP is excellent but the majority will require long-term low-dose oral corticosteroid therapy in order to prevent relapse.     

Adrenocortical carcinoma: surgery and mitotane for treatment and steroid profiles for follow-up

Adrenocortical carcinoma (ACC) is a rare disease with a poor prognosis. It has been difficult to establish a strict treatment program for ACC, and better treatment alternatives and diagnostic tools must be sought. Even though surgery is the treatment of choice, the role of surgery in advanced disease has been questioned. Eighteen consecutive patients were treated at our unit over a 22-year period (1975-1997). All patients underwent surgery and were followed by our protocol, which includes urinary steroid profiles, clinical examinations, analysis of steroid hormones, and radiologic investigations. Twelve patients received mitotane with drug concentration measurements to deliver an effective, nontoxic dose. The median duration of mitotane treatment was 12 months. Few side effects were observed. Four patients with low-stage tumors underwent second-look operations with no pathologic findings. Five patients were subjected to repeat operations, and the mean duration of the disease-free interval before repeat surgery for these patients was 59 months. There was a significant positive correlation between the disease-free interval and the observed survival after repeat surgery. Eleven patients with intentionally curative surgery had their urinary steroid profiles tested several times postoperatively. For five patients preoperative urine samples were also available. Steroid profiles indicated recurrent disease despite normal radiologic findings in two of these five patients. The follow-up ranged from 6 weeks to 24 years. The predicted 5-year survival was 58% according to the Kaplan-Meier method. We conclude that monitoring serum concentrations of mitotane makes long-term treatment possible with few side effects; steroid profile analysis can be used for early detection of tumor recurrence; and repeat surgery for recurrence is of value for patients with long disease-free intervals.     

Autoimmune pancreatitis: CT and MR characteristics

OBJECTIVE: Our goal was to elucidate the CT and MR imaging characteristics in patients with autoimmune pancreatitis, which is a reversible chronic pancreatitis with an autoimmune cause. CONCLUSION: On CT and MR imaging, a capsulelike rim, which is thought to correspond to an inflammatory process involving peripancreatic tissues, appears to be a characteristic finding of autoimmune pancreatitis. Also, diffuse pancreatic enlargement along with hypointensity on T1-weighted MR images and delayed enhancement on dynamic CT and MR studies are other features of this disorder.     

Acute generalized exanthematous pustulosis, induced by nystatin

BACKGROUND: Although ERCP is commonly performed in children, the effect of findings at ERCP on the subsequent management of pediatric pancreatitis is unknown. METHODS: We retrospectively reviewed charts to determine the impact of ERCP on the management of recurrent acute or chronic pancreatitis in 17 consecutive children (3 boys and 14 girls, 3 to 16 years, mean 11.2 years) with recurrent acute (n = 13) or chronic pancreatitis (n = 4) who underwent ERCP. Radiographs were reviewed in a blinded manner, and the effect of ERCP findings on subsequent management was determined. RESULTS: In 16 of 17 patients (94%), the pancreatic duct was successfully visualized. Of the 16 studies, 9 (56%) had abnormal findings. A change in therapy occurred in all 9 patients as a result of the findings at ERCP. Of the 7 patients with a prior abnormal CT or ultrasound, 5 (71%) had an abnormal ERCP, all resulting in a change in therapy. Three of the 9 patients (33%) without radiographic abnormalities had an abnormal ERCP that, in each case, resulted in a change in therapy. Overall, findings at ERCP altered therapy in 52% of pediatric patients studied with recurrent acute or chronic pancreatitis. A prior abnormal CT had a high predictive value with respect to ERCP resulting in a change in management (83%). CONCLUSIONS: ERCP is useful in the management of pediatric recurrent acute or chronic pancreatitis; abnormalities are found at a rate similar to those found in adults.     

HMG-CoA reductase inhibitor and risk of stroke

A 75-year-old-man who had been occupationally exposed to asbestos over several decades was admitted complaining of dry cough and mild exertional dyspnea. Chest X-ray films revealed the presence of bilateral infiltrates. An open biopsy showed the lung pathology to be desquamative interstitial pneumonitis (DIP), and detected a calcified plaque of the left parietal pleura at cost-phrenic sulcus, which on microscopic inspection demonstrated thin needle-like structures suggestive of asbestos particles. A hormonal examination showed decreased levels of tri-iodothyronine and tetraiodothyronine and an increased level of thyroid stimulating hormone. Additionally, a positive microsome test indicated autoimmune hypothyroidism. A serological study was positive for anti-nuclear antibody and direct Coombs, and showed heightened levels of IgG. The patient was placed on oral prednisolone therapy; one month later, his symptoms had subsided, and his lung function and chest x-ray findings had improved significantly. In addition, the abnormal values for autoimmune and thyroid function eventually returned to normal ranges. These findings suggest that DIP and humoral immune dysfunction were caused by asbestos exposure, and responded well to steroid treatment.     

Possible transmission of sarcoidosis via allogeneic bone marrow transplantation

Allogeneic bone marrow transplantation (BMT) was performed in a 34-year-old man for non-Hodgkin's lymphoma. Two years before bone marrow harvest, pulmonary sarcoidosis was diagnosed in the donor. After steroid therapy, disease of the donor was in clinical remission with only minor radiological signs at the time of BMT. On day 90 after BMT, active sarcoidosis was diagnosed in the recipient. Besides radiologic signs and increased angiotensin converting enzyme levels, diagnosis was proved by characteristic histologic changes in lung and liver biopsies. Immunosuppressive therapy was changed from high dose cyclosporine to high dose methylprednisolone and symptoms promptly resolved within 10 weeks. This case indicates the possibility of transmission of sarcoidosis by marrow transplantation.     

MR imaging of muscular sarcoidosis after steroid therapy

The nodular type of muscular sarcoidosis has been known to show characteristic MR findings: however, MR imaging features after steroid therapy have not been reported. A 48-year-old man with nodular type of muscular sarcoidosis is reported. Prior to steroid therapy, axial MR images showed peripheral increased signal intensity and central star-shaped decreased signal intensity. Coronal images showed an inner stripe of decreased signal intensity and outer stripes of increased signal intensity. After steroid therapy, axial images showed only the central star-shaped area of decreased signal intensity. Coronal images showed only the inner stripe of decreased signal intensity. It is important to know that the central area will continue to exist after steroid therapy.     

Inferior vena caval thrombosis associated with acute pancreatitis: an unusual vascular complication--its presentation and management

Vascular thrombosis and systemic hypercoagulable states are known complications of acute pancreatitis. They are thought to be secondary to the release of proteolytic enzymes of the pancreas. Inferior vena caval thrombosis is an extremely rare complication of chronic pancreatitis and has, to the authors' knowledge, never been reported in acute pancreatitis. The clinical presentation and radiographic findings are reviewed to illustrate the disease spectrum. Early treatment with intravenous heparin appears to be an effective therapy. Familiarity with this complication will aid physicians in its early diagnosis. However, a high degree of suspicion for this complication is necessary to make a diagnosis.     

Peculiarities of treating endocrine ophthalmopathy in patients with toxic goiter combined with diabetes mellitus

Thyroid pathology is among autoimmune diseases the incidence of which is now on the increase. Endocrine ophthalmology (EO) often accompanying thyroid disorders may cause deterioration or loss of vision. EO can be corrected by several methods. The most pronounced effect is achieved by x-ray therapy on the orbit and intermittent glucocorticosteroid (GCS) large-dose therapy. GCS, however, affect carbohydrate metabolism. In physiological levels, GCS effects on regulation of carbohydrate metabolism are not significant, but high GCS concentrations may provoke hyperglycemia and even steroid diabetes.     

Tracheobronchiomegaly (Mounier-Kuhn syndrome)

Tracheobronchiomegaly is a rare congenital disorder due to defective development of elastic and muscle tissues in the upper airways. The characteristic radiologic features shown by bronchography include marked dilatation of the tracheobronchial system and diffuse saccular bronchiectasis. These findings and recurrent episodes of pneumonia lead to a progressive loss of pulmonary function. Even with vigorous medical therapy the prognosis is poor and severe respiratory failure eventually develops.     

Macroamylasemia in HIV infection

A case of macroamylasemia was seen in a 40-year-old HIV-positive bisexual male treated at the Fort Worth-Tarrant County Health Department (Ryan White Clinic). Macroamylasemia is a rare condition encountered sometimes in persons with HIV infection. Apart from the setting of HIV infection and acquired immunodeficiency syndrome, macroamylasemia is seen also in various conditions including liver disease, diabetes, cancer, malabsorption, and autoimmune disorders. Although this biochemical phenomenon requires no therapy, it should be considered in the differential diagnosis of patients who have persistently high levels of serum amylase and yet do not exhibit any clinical symptoms of pancreatitis or salivary gland inflammation.     

Septicemia occurring after colonoscopic polypectomy in a splenectomized patient taking corticosteroids

Although a transient bacteremia may occur in approximately 4% of patients after colonoscopic procedures, clinically significant bacteremia or endocarditis is exceedingly rare. To our knowledge, the case we describe herein is the first reported case of septicemia due to Flavobacterium meningosepticum and Escherichia coli after colonoscopic polypectomy. Our patient was probably immunocompromised and, therefore, predisposed to bacteremia after the procedure, because she was asplenic, diabetic, and receiving long-term steroid therapy for her chronic autoimmune disorders. Several recent reports have suggested that immunocompromised patients are more likely to develop septicemia after endoscopic procedures.     

Flow injection amperometric determination of L-dopa, epinephrine or dopamine in pharmaceutical preparations

OBJECTIVE: The association of systemic lupus erythematosus (SLE) and multiple myeloma (MM) is an uncommon event. We report the relapse of SLE in a patient with a previous history of MM, treated with chemotherapy and, subsequently, with alpha-2b interferon (alpha-2b IFN) as a maintenance therapy. The case is discussed in light of past relevant literature. METHODS: The history and clinical, laboratory and radiographic findings of the patient, as well as the subsequent therapeutic approach are discussed. In our review of the literature, journal articles are identified by Medline search. RESULTS: We describe the case of a woman who developed a multiple myeloma 14 years after a diagnosis of SLE. A careful literature review confirms that the association of these two diseases has been reported only in a few cases. When the plasma cell neoplasia occurred, SLE had been quiescent for several years; the patient was treated with prednisone-melphalan and, subsequently, with alpha-2b IFN as a maintenance therapy. On admission to our department, SLE was in a relapse phase, probably because of IFN treatment. The disease was poorly responsive to steroid therapy and required the use of cytotoxic drugs. CONCLUSIONS: The coexistence of SLE and MM is very rare and the possible pathogenetic mechanisms underlying this association remain unclear. The use of interferon in a patient with an autoimmune disease always invites caution.     

First report of association of chronic pancreatitis, primary biliary cirrhosis, and systemic sclerosis

We report an original observation of chronic pancreatitis associated with primary biliary cirrhosis and systemic sclerosis. The diagnosis of each of these conditions was unequivocally confirmed. Pancreatic involvement in this case was asymptomatic. The association of chronic pancreatitis with primary biliary cirrhosis has been previously reported and pancreatitis has been associated with other autoimmune disorders. We hypothesize about the underlying pathogenic mechanisms of chronic pancreatitis in our case.     

Practical approaches to pediatric gastrointestinal radiology

Radiologic evaluation of the pediatric gastrointestinal tract requires knowledge of the specific problems acutely afflicting the gastrointestinal tract in children and the specialized approaches required for accurate radiologic diagnosis. Efficacious radiologic evaluation and accurate diagnosis are possible only when the radiologist is aware of the presence and significance of clinical findings. The radiologist must take an active role in formulating a presumptive diagnosis and determining the best course of radiologic examination. Careful correlation of clinical and radiologic findings is necessary to assure accurate diagnosis and appropriate treatment.     

Ormond's disease

Three cases of Ormond's disease (syndrome) are described, and the aetiopathogenesis, clinical picture and diagnostics of the disease are dealt with in this report. Besides, the traditional therapy and the new therapeutic possibilities are discussed. For a disease of low incidence and assumed autoimmune origin malignancy cannot be excluded. Therefore, surgical exploration and histological verification are indispensable interventions in every case of Ormond's disease. In two of the present cases, steroid was administered in defence of percutaneous nephrostomy. In two cases, the recently recommended anti-oestrogen therapy was successful. Stagnation, or even regression, of the process followed. The authors are sure that they were the first in Hungary to administer anti-oestrogen to patients suffering from IFR.     

Eosinophilic gastroenteritis presenting with acute pancreatitis

We present the findings on a 27-year-old male with eosinophilic gastro-enterocolitis accompanied with acute pancreatitis. Acute pancreatitis may be induced by pancreatic duct obstruction caused by marked swelling of the papillary region of the duodenum due to eosinophil infiltration. After prednisolone treatment, clinical manifestations rapidly improved; the serum amylase decreased and the peripheral eosinophilia was recovered. The serum interleukin-5 (IL-5) level was high at diagnosis and decreased by prednisolone therapy; however, IL-5 was detected by enzyme immunoassay even during clinical remission. These results indicate that eosinophilia is mediated by IL-5, and detectable levels of IL-5 indicate the possibility of relapse.