Caffeine and bowel recovery after elective colorectal resection: author’s reply

Techniques in Coloproctology -     

Typical Jaccoud’s arthropathy in a patient with sarcoidosis

Jaccoud’s arthropathy (JA) is a deforming, non-erosive form of arthritis initially described in rheumatic fever but recently observed more frequently in patients with systemic lupus erythematosus. However, cases of JA have been described in association with other diffuse connective tissue diseases, neoplasias, and infection. We describe a rare case of sarcoidosis in a female subject who developed JA in her hands later in the course of the disease.     

Monckeberg’s sclerosis in a patient with systemic sclerosis

Monckeberg’s sclerosis (MS) is one of the non-inflammatory vascular diseases characterized by calcification of the media of small and medium-sized muscular arteries, but is distinct from atherosclerosis. We present a case of MS that was incidentally detected by plain X-ray in a patient with systemic sclerosis. We took CT angiographs of the patient’s lower extremities for the differential diagnosis of vascular calcification, which was confirmed. To determine if systemic sclerosis is a risk factor for MS, we reviewed plain X-rays from 43 well-documented systemic sclerosis patients, but we did not detect any cases of MS. We therefore conclude that systemic sclerosis may not be a risk factor for MS.     

Hypertrophic cranial pachymeningitis in a patient with Cogan’s syndrome

A 63-year-old man presented with chronic headache and bilateral hearing loss. A physical examination showed bilateral conjunctivitis. Circulating anti-Cogan peptide antibodies were detected by dot blot analysis. He was diagnosed as having Cogan’s syndrome (CS). Steroid therapy led to dramatic improvement of his symptoms and abnormal laboratory findings. During a tapering course of steroid therapy, he suffered from headache. An ophthalmoscopic examination revealed papillary edema. Magnetic resonance imaging of the brain showed hypertrophic cranial pachymeningitis (HCP). After steroid pulse therapy, HCP was improved. To our knowledge, this is the first case of CS complicated with HCP.     

Diffuse disseminated Candidiasis in a patient with Felty’s syndrome: a case report

Severe granulocytopenia predispose patients with Felty’s syndrome to severe infectious diseases. The following report deals with an occurrence of chronic disseminated candidiasis in a patient with Felty’s syndrome who presented with prolonged and severe granulocytopenia. To the best of our knowledge this coexistence has never been described before. Affiliated to the Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel.     

Nephrocalcinosis and hypokalemia in a patient with primary Sjögren’s syndrome

Clinically significant renal involvement is uncommon in primary Sjögren’s syndrome, amid which tubulointerstitial disorders, distal renal tubular acidosis (dRTA) particularly, account for the majority. Conversely, Sjögren’s syndrome comprises at least half the patients presenting with renal tubular acidosis. While underlying dRTA itself is an important cause of nephrocalcinosis and urolithiasis, nephrocalcinosis is rarely a presenting feature of primary Sjögren’s syndrome. I report a 41-year-old female contracting nephrocalcinosis and hypokalemia as complications of primary Sjögren’s syndrome with dRTA, hereby to emphasize the importance of alkali therapy.     

Cutaneous polyarteritis nodosa in a patient with Crohn’s disease

Abstract

A 19-year-old Japanese woman with a 4-year history of Crohn’s disease (CD) developed high fever, polyarthralgia, and painful subcutaneous nodules of the legs. A skin biopsy showed panarteritis with fibrinoid necrosis in the deep dermis. Endoscopic examination showed aphthous lesions in the entire colon. She was diagnosed with cutaneous polyarteritis nodosa (PAN) associated with CD. Steroid therapy improved her symptoms. To our knowledge, this is the first Japanese case of cutaneous PAN associated with CD.     

Kaposi’s sarcoma in a patient with severe relapsing polychondritis

This report describes the case of a young man who developed Kaposis sarcoma (KS) after corticosteroid treatment for severe tracheal involvement of relapsing polychondritis (RP). The etiopathogenetic mechanisms that may have led to the evolution of this unusual neoplasm are discussed. To our knowledge, this is the first case reported of concomitant RP and KS.     

Adalimumab-induced interstitial pneumonia in a patient with Crohn’s disease

There are several reports of anti-tumor necrosis factor(TNF)-induced lung disease,especially in patients with rheumatologic diseases.Adalimumab is an antiTNF drug used to induce and maintain remission in patients with immune-mediated diseases,such as Crohn’s disease.Although pulmonary disorders could be an extra-intestinal manifestation of inflammatory bowel disease,biologic therapy could also be a cause of lung injury.Only few cases of adalimumab-induced lung toxicity have been reported,and the majority of them were in patients with rheumatologic diseases.Lung injury secondary to anti-TNF therapy should,after ruling out other etiologies,be considered in patients who have a temporal association between the onset of respiratory symptoms and the exposure to these drugs.A compatible pattern in the biopsy and the clinical improvement after discontinuation of the anti-TNF drug would strongly support the diagnosis.     

Cutaneous polyarteritis nodosa in a patient with Crohn’s disease

A 19-year-old Japanese woman with a 4-year history of Crohn’s disease (CD) developed high fever, polyarthralgia, and painful subcutaneous nodules of the legs. A skin biopsy showed panarteritis with fibrinoid necrosis in the deep dermis. Endoscopic examination showed aphthous lesions in the entire colon. She was diagnosed with cutaneous polyarteritis nodosa (PAN) associated with CD. Steroid therapy improved her symptoms. To our knowledge, this is the first Japanese case of cutaneous PAN associated with CD.     

Hepatocellular carcinoma in a non-cirrhotic patient with Wilson’s disease

We report the exceptional case of hepatocellular carcinoma in a non-cirrhotic patient, whose Wilson’s disease was diagnosed at the unusual age of 58 years. The liver histology revealed macrovesicular steatosis with fibrosis, but no cirrhosis. The disease was treated with D-penicillamine for 3 years until acute discomfort in the right upper quadrant led to detection of multifocal hepatocellular carcinoma, which was successfully resected. The histological examination confirmed the malignant nature of the 4 lesions, which were classified according to Edmondson and Steiner as poorly differentiated hepatocellular carcinoma grade 3. The non-tumoral parenchyma showed 80% steatosis with ballooned cells, lobular inflammation, septal fibrosis but no cirrhosis. Hepatocellular carcinoma is rare in Wilson’s disease, especially in the absence of cirrhosis. The literature’s 28 published cases are reviewed and the contributory role of copper in the hepatocarcinogenic process is discussed.     

Pituicytoma in a patient with Cushing’s disease: case report and review of the literature

There are scant prospective studies defining improvements in critical outcome measures with hormone replacement in hypopituitarism secondary to brain injury. We review the tests of cognition and physical function and summarize their use for subjects that are deficient in anterior hormone production during anterior pituitary hormone replacement in brain injury and propose these as the minimal tests that are feasible for a physician to perform in a clinical setting. We summarize the studies conducted to assess outcome measures after brain injury and also report preliminary findings for improvements in cognition and physical function in subjects with brain injury and GH deficiency.     

Crohn’s disease: a patient’s perspective

Aim As healthcare providers for Crohn’s disease, we assume that we have a good understanding of the disease progression and its symptoms. The aim of this study was to gather information about what patients with Crohn’s disease think are relevant to their symptoms and what helps them cope with this lifelong benign disease. Materials and methods A questionnaire was sent to all patients with a diagnosis of Crohn’s disease seen in the Digestive Disease Center in the last 5 years. The returned forms were downloaded into a database and sent for analysis. Results Sixty-two percent of respondents were female. One third were between the ages of 35 and 50 years. Seventy percent were married. Thirty-eight percent had a graduate degree, 19% were unemployed. Fifty percent still smoked, half of them less than one pack a day. Sixty-eight percent said that their symptoms affected work, and one fourth changed jobs due to this. Foods worsened symptoms in 60%, with a decrease in symptoms while on low fiber foods and white meats. Lifestyle change worsened symptoms in 66%. A change in the caregiver was not a significant stressor. More than half used Remicade, with one third stating that it was helpful. Eight percent had never used steroids. Alcohol increased symptoms in 40%. Factors that did not cause a significant change were children at any age, pregnancy, menopause, and hormone replacement therapy. Surgery caused half the patients to improve for many years, although one third felt a lowered self-esteem postoperatively. Conclusion Patients with Crohn’s disease should be managed in a more comprehensive manner to provide optimal care. Thus, a team approach that includes a dietician and counselor should be considered as an integral part of this team. This will allow patients to have enhanced skills to cope with changes in their symptoms, whether they are due to the disease itself or the changes in their routine.     

Uhl’s anomaly: A one and a half ventricular repair in a patient presenting with cardiac arrest

Uhl’s anomaly, first reported in 1952, is an extremely rare congenital cardiac defect characterized by partial or complete loss of the right ventricular myocardium and unknown etiology. Fewer than 100 cases have been described. The response to medical management is poor and there is no known ideal surgical approach or timing for treatment. We report the case of a previously active adolescent male presenting with cardiac arrest, who underwent successful bidirectional cavopulmonary anastomosis (“Glenn” anastomosis) with right atrial reduction and right ventricular free wall plication.     

Dysphagia aortica in a young patient with Behcet’s Disease: Case report

Dysphagia aortica is a rare aetiology of dysphagia resulting from an abnormality in thoracic aorta that causes extrinsic compression on the oesophagus. Dysphagia aortica includes aortic aneurysm, aortic dissection or even tortuous aorta and is seldom considered in the differential diagnosis of dysphagia.Herein, we report a 30-year-old man with Behcet’s disease who presented with rapid progressive dysphagia and diagnosed as dysphagia aortica caused by saccular aortic aneurysm complicated by large para-aortic haematoma compressing the oesophagus. The case reveals the importance of early and proper identification of the rare causes of dysphagia in young adults with complaint of dysphagia and history of recurrent oral and genital ulcers in absence of obvious cardiovascular diseases.     

Rapidly-progressive glomerulonephritis in a patient with Behcet’s disease: successful treatment with intravenous cyclophosphamide

We report a case of rapidly-progressive glomerulonephritis complicating Behcets disease (BD). A 44-year-old male has suffered from recurrent oral ulcers and retinal vasculitis developed 2 years ago. He complained of abdominal pain and papulopustular skin lesions. Multiple ulcers were seen on the colon on colonoscopy. Routine renal work-up revealed heavy proteinuria and hematuria. Renal biopsy demonstrated crescentic glomerulonephritis. Most symptoms improved after steroid therapy, except for urinary abnormalities. At this point, intravenous monthly cyclophosphamide pulse therapy was undergone. After the sixth pulse therapy, proteinuria and hematuria were dramatically improved and renal function was well preserved.