Adult-Onset Still’s Disease in Saudi Arabia

We present a series of 14 Saudi patients diagnosed to have adult-onset Still’s disease (AOSD). The clinical and laboratory pattern of AOSD in our series is more or less similar to that in other reported series, apart from having lower cardiac and pulmonary involvement than western series. The disease course was relatively benign, with only half the cases showing recurrences, which were controlled by alterations in the dosage of corticosteroid and NSAIDs. A comparison with other series is given. Received: 11 May 2000 / Accepted: 15 December 2000     

Is Crohn's Disease an Immunodeficiency?

The current hypothesis for the etiology of Crohn's disease proposes an excessive immune response, largely T-cell driven, possibly against endogenous bacteria. Standard therapy is therefore directed towards suppression of this immune response. An alternative theory of pathogenesis accounts for epidemiologic and pathophysiologic observations that have been hitherto underemphasized, namely, (1) genetic disorders with deficiencies in neutrophil function can give rise to a clinical and pathologic syndrome indistinguishable from Crohn's; (2) abnormal neutrophil function is well described in Crohn's disease; (3) a group of bacteria implicated in other chronic inflammatory disorders causes impairment of neutrophil function; and (4) 20th century environmental risk factors for Crohn's disease may directly suppress neutrophil function and may have led to a shift in the dominant gut flora with similar effects. We propose that some cases of Crohn's disease result from the interaction of environmental and genetic influences leading to impaired mucosal neutrophil function, resulting in failure to effectively clear intramucosal microbes effectively. While encompassing existing data, this hypothesis proposes a proximate defect in the mucosal immune response. If this paradigm were correct, new therapeutic approaches might involve strategies to alter intestinal flora and stimulate neutrophil function.     

Outpatient Blind Percutaneous Liver Biopsy in Infants and Children: Is it Safe?

Background/Aim:

We aim to investigate the safety of outpatient blind percutaneous liver biopsy (BPLB) in infants and children with chronic liver disease (CLD).

Patients and Methods:

BPLB was performed as an outpatient procedure using the aspiration Menghini technique in 80 infants and children, aged 2 months to 14 yrs, for diagnosis of their CLD. Patients were divided into three groups: Group 1 (<1 year), group 2 (1–6 yrs), and group 3 (6–14 yrs). The vital signs were closely monitored 1 hr before biopsy, and then 1, 2, 6, and 24 hrs after biopsy. Twenty-four hours pre- and post-biopsy complete blood counts, liver enzymes, prothrombin time (PT), and abdominal ultrasonography, searching for a biopsy-induced hematoma, were done for all patients.

Results:

No mortality or major morbidities were encountered after BPLB. The rate of minor complications was 17.5% including irritability or “pain” requiring analgesia in 10%, mild fever in 5%, and drowsiness for >6 hrs due to oversedation in 2.5%. There was a statistically significant rise in the 1-hr post-biopsy mean heart and respiratory rates, but the rise was non-significant at 6 and 24 hrs except for group 2 where heart rate and respiratory rates significantly dropped at 24 hrs. No statistically significant difference was noted between the mean pre-biopsy and the 1, 6, and 24-hrs post-biopsy values of blood pressure in all groups. The 24-hrs post-biopsy mean hemoglobin and hematocrit showed a significant decrease, while the 24-hrs post-biopsy mean total leucocyte and platelet counts showed non-significant changes. The 24-hrs post-biopsy mean liver enzymes were non-significantly changed except the 24-hrs post-biopsy mean PT which was found to be significantly prolonged, for a yet unknown reason(s).

Conclusions:

Outpatient BPLB performed by the Menghini technique is safe and well tolerated even in infants and young children. Frequent, close monitoring of patients is strongly recommended to achieve optimal patient safety and avoid potential complications.     

Assessment of Endothelial Function in Alzheimer's Disease: Is Alzheimer's Disease a Vascular Disease?

OBJECTIVES: To compare endothelial function of people with Alzheimer's disease (AD) with that of people without. DESIGN: Case-control study. SETTING: Geriatric medicine outpatient clinic of a university hospital. PARTICIPANTS: Twenty-five patients with AD who were free of vascular risk factors and 24 healthy elderly controls were enrolled. Exclusion criteria were diabetes mellitus, hypertension, dyslipidemia, evident stroke, smoking, documented coronary artery disease, history of myocardial infarction, heart failure, acute or chronic infection, malignancy, peripheral artery disease, renal disease, rheumatologic diseases, alcohol abuse, and certain drugs that may affect endothelial function. Both groups underwent comprehensive geriatric assessment and neuropsychiatric assessment. MEASUREMENTS: Endothelial function was evaluated according to flow-mediated dilation (FMD) from the brachial artery. RESULTS: Mean age +/- standard deviation was 78 +/- 5.9 in the group with AD (11 female and 14 male) and 72.1 +/- 5.8 in the control group (9 female and 11 male). Multiple linear regression analysis revealed that FMD was significantly lower in patients with AD (median 3.45, range 0-7) than controls (median 8.41, range 1-14) (P < .001), independent of age. It was also found that FMD values were inversely correlated with the stage of the disease as determined according to the Clinical Dementia Rating scale (r=-0.603, P < .001). CONCLUSION: Endothelial function is impaired in patients with AD. Endothelial function was worse in patients with severe AD. These findings provide evidence that vascular factors have a role in the pathogenesis of AD.     

Oral Crohn's disease: Is it a separable disease from orofacial granulomatosis? A review

Symptomatic oral Crohn's disease is comparatively rare. The relationship between orofacial granulomatosis, (where there is granulomatous inflammation and ulceration of the mouth in the absence of gastrointestinal disease) and true oral Crohn's disease is discussed along with the plethora of clinical oral disease presentations associated with both disorders and the differential diagnosis of oral ulceration in patients presenting to a gastroenterological clinic. Specific oral syndromes are outlined including the association between oral manifestations in Crohn's disease and the pattern of intestinal disease and their relationship to other recorded extraintestinal manifestations. The histological and immunological features of oral biopsies are considered as well as the principles of management of symptomatic oral disease. At present, it is suggested that both orofacial granulomatosis and oral Crohn's disease appear to be distinct clinical disorders.     

Aspirin in Patients with Coronary Artery Disease: Is it Simply Irresistible?

Journal of Thrombosis and Thrombolysis -     

Is Crohn's disease rare in India?

BACKGROUND: Crohn's disease (CD) is believed to be rare in India. OBJECTIVE: To analyze the data pertaining to patients with CD seen in a tertiary referral center. METHODS: Data on patients with chronic inflammatory bowel disease attending our Unit over a 5-year period were analyzed. The diagnosis of CD was established by the presence of characteristic segmental bowel involvement, consistent histological picture, exclusion of infectious causes, relapsing nature of the disease, response to appropriate drug therapy, and lack of evidence for another etiological factor. RESULTS: Of the 25 patients (age range 12-52, mean 31.7, years) 13 were men. Abdominal pain was present in 21 (84%) patients, diarrhea in 20 (80%), blood per rectum in 11 (44%) and fever in 4 (16%). The ileocecal region was involved in 6 (24%) patients and anal canal in 4 (16%); 19 (76%) had patchy involvement of different segments of the large bowel. Colonoscopic biopsies revealed granulomas in 12 (48%). During a mean follow up of 36.4 (range 6-54) months, 15 (60%) patients had 21 relapses. Despite initial response to 5-aminosalicylic acid in five patients, 23 needed glucocorticoid therapy at least once. CONCLUSIONS: CD may not be rare in India. Because of the high prevalence of intestinal tuberculosis there is a possibility that CD may be under-recognized in India.