Clinico-epidemiological features of congenital nonbullous ichthyosiform erythroderma in the eastern province of Saudi Arabia

BACKGROUND: A total of 10 455 new dermatology patients were seen in the dermatology clinics of King Fahd Hospital of the University (KFHU), Al-Khobar, Eastern Saudi Arabia, between January 1990 and December 1995. We identified 21 patients with a histopathologically confirmed diagnosis of congenital nonbullous ichthyosiform erythroderma (CNBIE). We have reviewed the epidemiological and clinical features of these patients. OBJECTIVE: To document the epidemiological and clinical features of patients with CNBIE in eastern Saudi Arabia. METHODS: We used the dermatology outpatient department (OPD) logbooks to identify diagnosed cases of CNBIE from new patients presenting with different dermatological problems over a 6-year period. We used specifically designed data-collection protocol forms to extract epidemiological and clinical data from the patients' medical records. These were entered into a computer database and analysed using standard statistical software. RESULTS: A total of 21 patients (five males, 16 females) with a male : female ratio of 0.31 : 1 were identified from a total of 10 455 new patients seen in our dermatology clinics over the study period. The occurrence rate of CNBIE in our clinics was 0.2%, or two per 1000 new dermatology cases. Nineteen (90%) of 21 CNBIE patients were born with collodion membranes. Eighty-one per cent of our patients had a positive family history of CNBIE. Consanguinity among the parents of our CNBIE patients was significantly high at 95%. CONCLUSIONS: To the best of our knowledge, this preliminary study is the first report of its kind from Saudi Arabia (documenting the clinico-epidemiological features of CNBIE patients in the Eastern Province). The high rate of parental consanguinity among the parents of our Saudi CNBIE patients may account for the high incidence rate of this genodermatosis in eastern Saudi Arabia. In comparison with results of other studies that reported a low occurrence rate of CNBIE among dermatology patients, our results were of a significantly higher rate.     

Computed tomography in chronic seizure disorder caused by glioma

Since the introduction of computed tomography (CT) in 1974, 137 patients underwent cortical resection for intractable epilepsy. The CT scans of 32 of these patients demonstrated an isolated calcified or hypodense lesion of unknown origin. The mean interval from onset of the seizure disorder to surgery was 11 years. The referring physicians frequently considered these lesions to be of nonneoplastic origin, which usually delayed surgical management. Twenty-six of the 32 patients, however, proved to have a cerebral glioma, seven showing anaplastic changes. Because the majority of these patients were found to have a neoplasm and especially because the CT scan could not distinguish between a well-differentiated glioma and those with anaplastic changes, we recommend early surgical treatment in patients with chronic seizure disorders who are found to have an isolated lesion of unknown origin on CT scan.     

Synthesis of 3-acetoxyazetidin-2-ones and 3-hydroxyazetidin-2-ones with antifungal and antibacterial activity

The synthesis of a series of 3-acetoxyazetidin-2-ones 3a–n and 3-hydroxyazetidin-2-ones 6a–j is reported together with the antibacterial and antifungal evaluation of these compounds. An additional series of 3-acetoxyazetidin-2-ones 11a–h which possess a free carboxylic acid group on the N-1 aryl ring were obtained by treatment of suitably substituted Schiff bases 10a–h with acetoxyacetyl chloride. The novel bicyclic structures 7-acetoxy-6-phenyl-5-thia-1-azabicyclo[4.2.0]octan-8-one 13 and 7-hydroxy-6-phenyl-5-thia-1-azabicyclo[4.2.0]octan-8-one 14 were also obtained. Many of the compounds displayed antifungal activity in vitro when evaluated against the pathogenic fungi Cryptococcus neoformans, Candida albicans, Candida tropicalis, Candida parapsilosis, Candida glabrata, and Trichosporon cutaneum, while 3-acetoxyazetidin-2-ones 11a–h containing a free carboxylic acid group on the N-1 aryl ring displayed antibacterial activity against Staphylococcus aureus, Proteus vulgaris, Pseudomonas aeruginosa, Bacillus subtilis, Klebsiella aerogenes and Escherischia coli.     

Simultaneous MR acquisition of arterial and brain signal-time curves.

Regional cerebral blood flow (rCBF) provides important information about local neuronal functional and cerebrovascular status. Determination of rCBF requires sequential measurements of tracer concentration in arterial blood and brain tissue unless the tracer is trapped in the brain in proportion to rCBF. Since gadopentate dimeglumine is not trapped within brain tissue, we have developed the simultaneous dual FLASH pulse sequence (SDFLASH) which sequentially measures the MR signal change in both the internal carotid artery and brain parenchyma simultaneously during the passage of a bolus of paramagnetic contrast material.     

Immediate hypersensitivity to Parthenium hysterophorus. II. Clinical studies on the prevalence of parthenium rhinitis

The airborne pollen of the South American weed, Parthenium hysterophorus (American feverfew), accidentally introduced into India was found to be responsible for severe allergic rhinitis. A random clinical survey conducted on 2035 residents of Bangalore city with the aid of questionnaires and skin tests revealed that 7.1% of the study population was suffering from allergic rhinitis due to exposure to Parthenium pollen. Skin-prick tests performed on 1294 clinic patients suffering from nasobronchial allergy during the past 4 years have also shown that 42.5% were sensitive to Parthenium pollen. IgE and IgG antibodies specific for Parthenium pollen allergens were demonstrable in the sera of Parthenium-sensitive rhinitis patients. The specificity of these antibodies to Parthenium allergens was established by ELISA. A 7- to 11-fold higher stimulation was observed when lymphocytes from two Parthenium-sensitive rhinitis patients were treated in vitro with Parthenium pollen extract. To our knowledge, nowhere in the world has such a high incidence of allergic rhinitis due to a single pollen ever been reported.     

Optic nerve dysfunction in thyroid eye disease: CT

Optic nerve dysfunction in thyroid eye disease is thought to be due to compression of the optic nerve by enlarged extraocular muscles near the orbital apex. High-resolution computed tomography (CT) scans of 78 orbits of 31 patients with thyroid eye disease were reviewed. Axial scans alone were inadequate for demonstrating compression of the optic nerve. With a coronal reformatted scan from the axial scans, a muscular index was devised and measured to reflect extraocular muscle impingement on the optic nerve. Orbits with optic nerve dysfunction had significantly higher muscular indices than those without optic nerve dysfunction, supporting the hypothesis that optic nerve dysfunction is usually secondary to compression by enlarged extraocular muscles. Muscular indices of 67% or greater in patients with optic nerve dysfunction were diagnostic of compressive optic neuropathy, while muscular indices of less than 50% appeared to exclude optic nerve compression. A single case of optic nerve dysfunction without muscular compression is also discussed.     

t(1;3)(p36;q21) in acute nonlymphocytic leukemia: a new cytogenetic- clinicopathologic association

A number of specific chromosomal abnormalities have been associated with distinctive clinical and/or morphological subtypes of acute nonlymphocytic leukemia (ANLL) in recent years. We have studied three patients with ANLL and t(1;3)(p36;q21). Each had weakness as their major complaint, a moderately severe anemia and, for ANLL, a relatively high platelet count. All three demonstrated abnormalities of the megakaryocytic, erythroid and granulocytic lineages. Most striking was the dysmegakaryocytopoiesis. The blasts in all three patients showed relatively few azurophilic granules, one to four prominent nucleoli, and rare peroxidase positivity. No patient had Auer rods. No patient responded to standard chemotherapy regimens. The data suggest that t(1;3)(p36;q21) identifies a new cytogenetic-clinicopathologic subtype of ANLL.     

Focal signal-intensity variations in the posterior internal capsule: normal MR findings and distinction from pathologic findings

Cranial magnetic resonance (MR) imaging studies in 117 control patients were reviewed to evaluate for focal signal-intensity variations in the posterior internal capsule. Rounded foci of increased signal intensity were found near the junction of the posterior limb and retrolenticular portion of the internal capsule on axial T2-weighted images in 56% of patients imaged at 1.5 T and in 50% imaged at 0.5 T. Corresponding hypointense foci were found on T1-weighted images in 64% of control patients imaged at 1.5 T and in 69% imaged at 0.5 T. With all sequences, the foci were homogeneous and well defined, without mass effect, and bilaterally symmetric. Comparison was made with MR imaging studies in 32 patients with pathologic involvement of the posterior internal capsule region. Symmetric morphology and signal intensity, sharply defined margins, confinement to a characteristic location, signal intensity approximating that of cortical gray matter on T2-weighted images, and isointensity or hypointensity on spin-density-weighted images appear to characterize normal posterior capsular foci and distinguish them from foci of pathologic lesions. A region that stained less intensely than the surrounding internal capsule was noted in tissue blocks from two normal brain specimens, corresponding in morphology and position to the signal-intensity changes seen on MR images. The properties of this focus suggest that it may represent fibers of the parietopontine tract.     

Interactive segmentation of cerebral gray matter, white matter, and CSF: Photographic and MR images

Digital photography of postmortem brain slices was compared with magnetic resonance imaging (MRI) for morphological analysis of human brain atrophy. In this study, we used two human brains obtained at autopsy: a cognitively defined nondemented control (70-yr-old male) and a demented Alzheimer's disease (AD) subject (82yr-old female). For each of two brains, interactive manual image segmentation was performed by two observers on two image sets: (a) four coronal T1-weighted MR images (5 mm slices); and (b) four digitized photographic images from comparable rostrocaudal levels. Microcomputer image analysis software was used to measure the areas of three segmented cerebral compartments—gray matter (GM), white matter (WM) and CSF—for both image types. Resegmentation error was defined as the absolute difference between the areas derived from two segmentation trials divided by the value from trial 1 and multiplied by 100. This yielded the percent difference between the area measurements from the two trials. We found intea-observer agreement was better (error rates 1–18%) than inter-observer agreement (3–70%) with best agreement for WM and least for CSF, the smallest object class. MRI overestimated GM area relative to digitized photographs in the control but not the AD brain. The results define limitations of manual image segmentations and comparison of MRI with pathologic section photographic images.