Bullous pemphigoid in children. Report of three cases

Bullous pemphigo?d (BP) is an acquired immunobullous disease that usually affects adults and rarely children. About 60 cases of infant PB have been reported. Diagnosis is based on immunofluorescence investigations. Clinical, histological and immunopathological findings in childhood PB appear to be not different from the adult. Nevertheless, oral mucosal and palm and sole lesions seem to occur more frequently. We report 3 cases of children BP in one girl and 2 boys, aged respectively of 7.2 and 3 years. Palm and sole were affected in 1 case, and no oral mucosal lesion was noted. Recovery was reached in all cases under dapsone. Characteristics of BP in children are discussed with comparison to literature data.     

Genotype/phenotype correlations in Arab patients with familial Mediterranean fever

OBJECTIVES: To study the phenotype/genotype correlations in Arab patients with familial Mediterranean fever (FMF). PATIENTS AND METHODS: The study was performed in a 3-year period (February 1998-February 2001). Patients were seen in the pediatric FMF clinic of Jordan University Hospital, and the diagnosis of FMF was made according to published criteria. Screening for mutations was carried out by direct sequencing of the entire coding sequence of exon 10 and its donor splice site and by restriction endonuclease testing for mutations in exon 2. A total of 278 patients with clinically positive FMF were screened. RESULTS: Of the 278 patients, 50 (18%) had 2 mutations identified, and 76 (27%) other patients had only 1 mutation identified. The 50 patients with 2 mutations are the subject of this report. The M694V/M694V and the M694V/V726A and M694I/M694I genotypes were the most common (30%, 16%, and 14%, respectively). Three homozygous genotypes (M694V/M694V, V726A/V726A, and M694I/M694I) and 2 compound heterozygous genotypes (M694V/V726A and V726A/M680I) accounted for 78% of mutations. The difference in the mean severity score (14 +/- 2) of the M694V/M694V group and the V726A/V726A (mean severity score, 10 +/- 3) and M694I/M6941 (mean severity score, 6 +/- 1) groups was statistically significant (P =.003 and.0, respectively). The difference between the M649V/M694V group and the M694V/V726A (mean severity score, 15 +/- 2) was not statistically significant (P = 0.31). CONCLUSIONS: The genotypes M694V/M694V and M694V/V726A have a severe clinical course in Arab patients with FMF, whereas the M694I/M694I is associated with mild disease.     

Embolization of head and neck hypervascular lesions. A retrospective study of 5 cases

Embolisation of head and neck hypervascular tumours and arterioveinous malformations (AVMs) is now a well-established therapeutic procedure. The purpose of this study was to analyse the technique and to evaluate the safety and value of preoperative embolization of hypervascular lesions. We retrospectively reviewed the records of 5 patients, agged between 14 and 47 years, with hypervascular tumours and AVMs in the head and neck. Lesions were distributed as follow: nasal angiofibroma (n = I), nasal angioleiomyoma (n = I), nasopharyngeal angiofibroma (n = I), aneurysmal bone cyst in the posterior element of the second cervical vertebra (n = I) and AVM of the inferior lip (n = I). Angiography and embolization were carried out at the same time. Complete exclusion of lesions was obtained without any complications. Complementary surgical resection was performed with not notable blood loss.     

Liver regeneration after major hepatectomy. Evaluation of dogs hepatectomy

OBJECTIVES: The aim of our study is to evaluate anatomic regeneration and metabolic derangement of the liver after major resection in dogs. METHODS: This is an experimental study on 9 dogs; we divided the dogs in two groups: the first group (5 dogs) underwent at one go major hepatectomy (90% of the liver).The second group (4 dogs) underwent successively a resection of 75% of the liver and a second resection of 90% of the restored liver six months later. All dogs underwent a metabolic and morphologic studies of the liver and of their kidney function. RESULTS: In the first group; all dogs which underwent 90% hepatic resection died 48 hours after the surgical resection of hepatic insufficiency. The ultra microscopic study showed the role of portal hypertension in hepatic degeneration on the first group. In the second group, the dogs survived the first resection, and our study shows a regeneration of the liver after resection and sub normal hepatic function. CONCLUSION: The liver is able to regenerate after minimally resection but major resection must be done by successively resection to avoid hepatic dysfunction, but the time between resection must be evaluate later.     

Painful traumatic neuroma after a finger stick

In this case report we present a patient with a painful traumatic neuroma after a finger stick by lancet. The patient benefited from surgical excision of the lesion, and her symptoms were completely abolished with additional sympathetic nerve blocks.     

Coronal thick CT reconstruction: an alternative for initial chest radiography in trauma patients

It has been proposed that the imaging workup of trauma patients be accelerated by omitting the initial chest radiography (CR) and directly performing a computed tomography (CT); however, the baseline CR is then lacking. The purpose of this study was to assess if coronal thick reconstructions generated from chest CT could present an adequate alternative for CR. Sixty trauma patients underwent bedside CR and multidetector row chest CT in the emergency room. The image quality of thoracic anatomical structures, the diagnostic accuracy for chest pathology, and the depiction of indwelling devices were assessed on both modalities. Main pulmonary arteries and perihilar bronchi were equally visualized with both modalities. Central bronchi, retrocardial lung parenchyma, diaphragm, descending aorta, and vertebral pedicles were better visualized on thick CT reconstructions, whereas peripheral lung vessels were better depicted on CR (p<0.05). The accuracy to delineate various pathological findings did not differ between both modalities, except for a higher sensitivity to diagnose bronchial cuffing on CR (p<0.05). The location of indwelling devices was similarly and correctly depicted with both modalities. Coronal thick CT reconstructions provide a similar image quality and diagnostic accuracy compared with CR. These reconstructions may serve as an equivalent baseline image in trauma patients in whom emergency radiological evaluation has to be accelerated.     

Amyand’s hernia: a case report of prospective ct diagnosis in the emergency department

The diagnosis of Amyands hernia, the development of acute appendicits within an inguinal hernia, is rarely made preoperatively and is often confused clinically with an incarcerated right inguinal hernia. The use of CT to prospectively diagnose Amyands hernia and corresponding imaging findings are not well described in the literature. We report a case of Amyands hernia, which was correctly diagnosed by CT in a female patient presented to the emergency department with right lower quadrant pain and clinical suspicion of a strangulated omentocele.     

A prognostic index (bladder prognostic index) for bilharzial-related invasive bladder cancer

PURPOSE: Bladder cancer is still the most common solid tumor among adult males in Egypt because of the prevalence of bilharzial infestation, especially in the countryside. In this prospective study, we have recorded the prognostic factors for 180 patients with invasive bladder cancer for whom standard radical cystectomy had been performed to develop a prognostic index (bladder prognostic index) that defines high risk patients who are more vulnerable to disease relapse after surgery and who may benefit from additional therapy. PATIENTS AND METHODS: The study was performed between January 1997 and December 1999, in which 180 patients with histopathologically proved invasive bladder cancer associated with bilharziasis underwent radical cystectomy or anterior pelvic exenteration. After surgery, patients were regularly followed for a minimum of 2 years. RESULTS: Our patients included 141 males and 39 females. Squamous cell carcinoma was the most common type (53.3%), and most of the tumors were grade II (61.1%). A total of 173 patients had their tumors operable, while 7 were inoperable. We had 5 (2.8%) operative related mortalities. At 5 years postoperatively, free and overall survival rates for the whole group of patients were 31.44%+/-5.9% and 32.5%+/-6.8%, respectively. Tumor pathologic stage, grade, and nodal affection were the only significant factors with impact on survival (P=0.008, 0.051, and 0.004, respectively). These 3 prognostic indexes were used to design a model to predict an individual patient's risk factor for recurrence. Patients were then assigned to one of the 4 risk groups according to the score achieved in this prognostic index (0=low risk, 1=intermediate risk, and 2 or 3=higher risk). These 4 risk groups had distinctly different rates of disease-free survival, i.e., 91.7%, 53%, 13%, and 7% for low, intermediate, and higher risk groups, respectively. CONCLUSION: Although this prognostic index appears to be of a significant clinical relevance, it needs to be more validated on a larger number of patients, and it could be a surrogate variable for biologic factors responsible for the heterogeneity of bladder cancer.     

Treating pain related to inoperable pancreatic cancer in tropical areas: the advantage of CT-guided celiac plexus block and splanchnic nerves neurolysis

Many sub-Saharan African countries have recently acquired computed tomography scanners that make interventional radiology possible, especially for the treatment of cancer pain. We report the case of a patient with severe abdominal pain related to advanced pancreas cancer. After unsuccessful morphine treatment, he underwent CT-guided alcohol injection for neurolysis of the celiac plexus and splanchnic nerves. This report describes the technique and discusses its potential applications in tropical countries.