Otorhinolaryngological manifestations of sickle cell disease.

OBJECTIVES: Although sickle cell disease is the most common of all hereditary disorders, there are a few publications about the effects of the disease on the functions of the ears, nose and throat. In this review, we present an overview of the clinical manifestations of sickle cell disease in general and highlight the problems specifically presenting in the field of otorhinolaryngology. METHODS: We review the pathophysiology of sickle cell disease and its clinical features in general. Then, we review its manifestations in ear, nose and throat diseases. CONCLUSION: Sickle cell disease is one of the most common hemoglobinopathies. It can cause severe pain crises and dysfunction of virtually every organ system in the body, ultimately causing premature death. There is high prevalence (55%) of obstructive adenotonsillar hypertrophy in children and adolescents with sickle cell disease. A very significant reduction has been observed in the rate of pain crises following tonsillectomy in patients with sickle cell anaemia. Prevalence rate for sensorineural hearing loss in older children and adult patients is reporting a range of 11-41%. Priapism of the turbinates is a cause of nasal obstruction in sickle cell anaemia which needs partial turbinectomy. Extramedullary haematopoiesis should be considered in the differential diagnosis of any paranasal sinus mass presenting in a patient with known chronic anaemia.

Two cases of Stevens-Johnson syndrome following intake of klavox with review of literature.

We present two case reports of Stevens-Johnson syndrome (triad of fever, stomatitis and conjunctivitis) due to klavox (amoxicillin + clavulanic acid) which has been reported in few publications as etiology but it is not commonly seen. We conclude that klavox can precipitate development of SJS.

The role of chromosomal aberrations in premalignant and malignant lesions in head and neck squamous cell carcinoma.

The objectives of this study are to uncover the molecular mechanisms involved in head and neck squamous cell carcinoma (HNSCC) pathogenesis by studying the chromosomal aberrations in both premalignant and malignant patients and to highlight the genotype of HNSCC in Upper Egypt. From March 2001 to December 2003, prospective study was conducted in 41 patients with precancerous, 79 patients with cancerous laryngeal, oesophageal, nasopharyngeal, nasal, and oral lesions and 50 controls in ENT department, Sohag Faculty of Medicine, Sohag, Egypt. Samples taken by punch biopsy were frozen and stored at -80 degrees C and were subjected to histopathological examination. Metaphase cells were digitally imaged and karyotyped. Karyotypes have been analysed via anatomical image capture and compared with standard human chromosome ideograms. In precancerous lesions, there were 41% 3p loss, 51% 3q gain, 29% 8q gain, and 22% 11q13 gain. In malignant lesions, there were 63% 3p13-p24 loss, 59.5% 5q12-23 loss, 49.5% 8p22-p23 loss, 45.5% 9p21-p24 loss, 40.5% 18q22-q23 loss, 66% 3q gain, 39% 8q gain, and 16% 11q13 gain. In conclusion, early diagnosis of HNSCC can be achieved by DNA extraction from suspicious lesions in high-risk groups (smokers and alcoholics) and examination of chromosomal aberrations of 3p, 3q, 8q, and 11q13. If there are high percent of chromosomal aberrations in these chromosomes, active intervention should be done (chemoprevention and regular follow-up of head and neck examination for very early detection and management).

The flow cytometric analysis of premalignant and malignant lesions in head and neck squamous cell carcinoma.

We attempted to identify the molecular mechanisms involved in Head and Neck Squamous Cell Carcinoma (HNSCC) pathogenesis by measuring the nuclear DNA content (ploidy) in premalignant (potentially malignant) and malignant patients as compared to normal controls, and to determine whether DNA ploidy could be used to predict the clinical outcome. From March 2001 to December 2003, the analysis was carried out in a set of 41 patients with premalignant lesions and 79 suffering from squamous cell carcinoma of laryngeal, oesophageal, nasopharyngeal, nasal and oral lesions and 50 controls. Representative samples were taken by punch biopsy and processed using standard formol-paraffin technique for histopathological examination. Fifty micrometer thick sections of paraffin-embedded tissues were analyzed to detect the DNA content by image cytometry. Of the potentially malignant patients, 46% had diploid lesions, 37% had tetraploid lesions and 17% had aneuploid lesions. While of the patients with cancer, 90% had aneuploid lesions, 10% had diploid lesions and none had tetraploid lesions. DNA diploidy tended to occur earlier in the progression from premalignant to malignant lesions and this helps us early detection of HNSCC by DNA from lesions in high risk groups and examination of its ploidy. Knowledge of tumor cell ploidy by DNA image cytometry may facilitate the evaluation of malignant and premalignant lesions in HNSCC. The present findings are promising to supplement clinical and histopathological parameters in evaluating prognosis and to demonstrate methods that are readily applicable for routine diagnostic work.

Light and electron microscopic study of primary atrophic rhinitis mucosa.

BACKGROUND: This study examined the light and the electron microscopic changes in the mucosa of primary atrophic rhinitis and compared them with the normal nasal mucosa to establish the changes in the surfactant system. A prospective original study was performed. METHODS: Twenty cases of primary atrophic rhinitis were randomly selected in the outpatient clinic of Sohag University Ear, Nose, and Throat Department. Ten volunteers with no history of chronic nasal disease and with normal rhinopharyngeal picture were selected as control cases. A small punch biopsy was performed with a small forceps under local anesthesia from the medial edge of the inferior turbinate. After histological laboratory preparations, semithin sections (0.5-1 microm) were prepared by using an LKB ultramicrotome. The sections were stained by toluidine blue, examined by light microscope, and photographed. Ultrathin sections (500-800) from selected areas of the trimmed blocks were made and collected on copper grids. The ultrathin sections were then contrasted in uranyl acetate for 10 minutes and examined by electron microscope Jeol JEM-1010. RESULTS: Light microscopy of primary atrophic epithelium revealed typical nonkeratinized stratified squamous epithelium to keratinized squamous epithelium with a thin layer of keratin on the surface. Well-developed desmosomes appeared between the epithelial cells. In the lamina propria, no submucosal glands could be seen. Electron microscopy of it revealed that the stratification of the epithelium increased. Sloughing of the superficial cells was observed in some regions of the proliferating epithelium. Epithelial penetration with neutrophils (mainly) and lymphocytes (some) could be seen. No numerous multilamellar bodies (MLBs) could be detected in these cases. An increased amount of collagenous fibers was observed in the basement membrane. CONCLUSION: In primary atrophic rhinitis, the epithelial cells revealed that desmosomes are characteristic of the metaplastic squamous epithelium and had no MLB in their cytoplasm, and cells in which occasional MLBs had been established showed interdigitations between them and the surrounding cells, suggesting an association between the surfactant deficiency and the development of desmosomes.

Prevalence of middle ear pathologies in children with bilateral sensorineural hearing loss.

OBJECTIVES: In poor countries, hearing aids are too expensive for sensorineural hearing loss (SNHL) children's parents to offer for their children. These children may have middle ear problem, this will aggravate the level of hearing loss which may lead to delay in their ability to speak. This study is to highlight the prevalence of middle ear pathology in SNHL children. METHODS: Two hundred children with bilateral sensorineural hearing loss (SNHL) were selected in our study from the outpatient clinic of ENT department of Sohag University Hospital, Egypt. Children were classified into three categories according to their middle ear status. They were normal middle ear, middle ear with unhealthy tympanic membrane or otitis media with intact drum and chronic suppurative otitis media with perforation. RESULTS: Seventy percent of cases were normal, 25% had middle ear problem with intact tympanic membrane and 5% had chronic suppurative otitis media with perforation. CONCLUSION: Thirty percent of SNHL children have middle ear pathology which aggravate the degree of hearing loss. Regular evaluation of SNHL children to treat those having middle ear pathology medically and/or surgically and this may help those having no ability to have hearing aids to learn language early.

Proboscis lateralis: clinical and radiological features.

Proboscis lateralis is one of the rare craniofacial congenital anomalies which presents as an obvious deformity of the nose. It tends to occur in male offspring of consanguineous marriages. This is a report of computerized tomography of this rare anomaly. In proboscis lateralis, the nasal cavity on one side is completely normal, while on the affected side it is replaced by a trunk-like process attached to the medial portion of the orbital roof. This anomaly occurs sporadically as an isolated defect or in association with other anomalies. It is usually associated with developmental failure of the paranasal sinuses and the nasolacrimal duct.

Proboscis lateralis: a report of two cases.

Proboscis Lateralis (PL) is one of the congenital anomalies of the nose which presents as obvious deformity. This is a report of two cases of the rare anomaly PL. One presented with right PL and the second presented with left PL. In this rare anomaly, the nasal cavity on one side is completely normal while on the affected side, the nasal cavity is replaced by a tube of skin and soft tissue attached to the inner canthus of the eye. This anomaly occurs sporadic as an isolated defect or in association with other anomalies. It is usually associated with failure of the paranasal sinuses and the nasolacrimal duct development. Embryologically, this anomaly is a result of imperfect fusion of the lateral nasal wall and maxillary processes (Int. J. Pediatr. Otorhinolaryngol. 23 (1992) 275).