Diagnostic and treatment difficulties in systemic lupus erythematosus coexisting with sickle cell disease.

BACKGROUND: Patients with Sickle cell disease present with a wide range of symptoms and signs which overlap with other chronic illnesses. This often leads to a delay in diagnoses of the associated disorder. OBJECTIVE: In view of the high prevalence of SCD in our environment, it is important to know that this association can occur and should be considered when patients present with pyrexia, arthritis, seizures and a recurrent rash. CASE REPORT: We present an 8-year-old boy with sickle cell disease who presented with recurrent fever, back pains, and 'cutaneous eruptions' to multiple drugs. He had several admissions within two years due to above symptoms. Examination at the onset of the illness revealed a young boy with fever, no jaundice, and periorbital oedema and generalized lymphadenopathy. He had scanty fluffy hair and post inflammatory hyper pigmentation on the trunk and extremities. He also had a hepatomegaly. He was started on Ibuprofen and ceftraixone to which he reacted. He had corticosteroids and antimalarials and improved. During the 2-year-follow up period he was admitted for recurrent seizures, arthritis and a leg ulcer. On last visit to hospital, he developed a malar rash. Discoid rash with mouth ulcers. A diagnosis of SLE was made in the patient. CONCLUSION: There are only 23 reported cases of SLE occurring in patients with sickle cell disease in literature, suggesting that the association is rare, it should be considered in patients with sickle cell disease presenting with pyrexia, rash and seizures.

Postoperative changes of erythrocyte sedimentation rate, plasma viscosity and C-reactive protein levels after hip surgery.

We measured the behavior of the erythrocyte sedimentation rate (ESR), plasma viscosity (PV), and C-reactive protein (CRP) in 66 patients who underwent hip implant operations, from day 0 to day 21 postoperation. We analyzed the behavior of these parameters after the trauma of the operation. We identified a subset of patients with evidence of superficial clinical infection/inflammation, where the magnitude and duration of the response to surgery was different from the main group. PV and CRP show less variability between patients and consequently are better indicators of the acute phase response than the ESR which requires a series of values in order to demonstrate a trend. PV can be obtained simply and rapidly, which would make it a powerful tool in determining persistent inflammation.

Mallet deformity of the finger. Five-year follow-up of conservative treatment.

We reviewed 31 patients at a mean of five years after mallet deformity of the finger had been treated with a thermoplastic splint. Intra-articular fractures were present in 35% of patients. Osteoarthritic changes had developed in 48%, most in association with fracture, and 29% had a swan-neck deformity. There was a loss of extension greater than 10 degrees in 35%; the average deficit at the interphalangeal joint was 8.3 degrees and the average flexion arc was 48.5 degrees. Despite these findings, patient satisfaction was generally high, with little evidence of functional impairment.

Treatment of Morton's neuroma by neurolysis.

Thirty-five patients who had undergone neurolysis for Morton's neuroma were reviewed at a mean of 21.4 months. Those patients who had received diagnostic lidocaine (local anesthetic) injections as an evaluation tool before the operation did extremely well after this operation. Overall patient satisfaction was found to be extremely high, with 17 of 35 patients enjoying complete relief of their pain and 12 of 35 reporting minimal discomfort with activity. The likelihood of persistent symptoms seemed to be related to the presence of associated foot disorders.

An unusual manifestation of bleeding diathesis in a patient with osteogenesis imperfecta.

We present the anaesthetic and surgical management, and post-operative course of a patient with osteogenesis imperfecta which exemplifies the problems associated with this condition. The observed petechial haemorrhagic rash is not characteristic of the bleeding tendency in osteogenesis imperfecta nor characteristic of acute disseminated intravascular coagulation. Despite potential life-threatening complications the patient made a good recovery and was discharged 3 weeks after surgery.

Survey of spinal therapeutic procedures in the United Kingdom.

The type and frequency of spinal therapeutic work being undertaken in the United Kingdom (UK) by clinicians with an interest in the surgical treatment of disorders of the spine (primary and secondary subspecialty interest) were evaluated by means of a postal questionnaire. The willingness of respondents to take part in postgraduate spinal training was determined along with issues regarding accessibility of spinal services to non-specialist physicians in the health service in the UK. The results of 450 respondents provided insight into the types of procedures taking place, for example: primary spinal decompression was regularly carried out by 76% of surgeons, while at least 20% of respondents regularly carried out 66% of the procedures surveyed. We found that 10% of surgeons indicated that they were prepared to participate actively in postgraduate spinal surgical training.

Is fine needle aspiration of the tonsil superior to a surface swab for isolating its core flora in recurrent tonsillitis?

This prospective study was designed to explore the possible role of fine needle aspiration of recurrently inflamed tonsils in determining their core flora. Seventy-seven patients undergoing tosillectomy for recurrent tonsillitis were included. Colonies grown from fine needle aspirate and surface swab were compared with tonsillar core culture obtained after tosillectomy. The pathogens isolated by fine needle aspiration culture were not greatly different from surface swab results in comparison to core isolates. The results of fine needle aspirate do not appear to confer any advantage over surface swab in representing core flora. These findings would seem to indicate no clinical use for fine needle aspiration in the management of recurrent tonsillitis.

Post intubation laryngeal sequelae in an intensive care unit.

The incidence, severity and pattern of post-intubation laryngotracheal sequelae in a 12 bed multidisciplinary intensive care unit (ICU) were assessed in this prospective study. One-hundred and fifty consecutive patients requiring intubation for more than 24 hours for various indications were studied. Evaluation of the larynx and trachea was done using a fibreoptic bronchoscope introduced through the endotracheal tube prior to elective extubation. Rigid bronchoscopy and direct laryngoscopy were performed in very small children and adults requiring tracheostomy. One-hundred and thirty-one (87.6 per cent) patients had visible laryngeal pathology in the immediate post-extubation period. Thirteen (8.6 per cent) had long term sequelae. A high incidence of long term sequelae was noted in patients with seizures (25 per cent) followed by patients with head injury (19 per cent). Steroid therapy failed to offer any significant protection but resulted in doubling of pulmonary sepsis. A grading system was adopted to classify acute laryngotracheal injury and a significant correlation was found between the presence of slough in the immediate post-extubation period and subsequent development of long term sequelae. There was also a significant correlation between a deeper insertion of the endotracheal tube and development subsequently of long term sequelae. The significance of these findings is discussed.

Sensorineural hearing loss in neurobrucellosis.

A case of neurobrucellosis presenting to the otologist with sensorineural hearing loss (SNHL) as the predominant clinical feature is reported. The diagnostic features and treatment options are discussed and the need for prolonged combination treatment to prevent relapse and further deterioration of hearing stressed. SNHL in neurobrucellosis has hitherto been reported principally in neurology literature as something of an incidental finding and so escapes the attention of otologists. It is hoped that this report will alert otologists in areas where brucellosis is endemic to the need to include tests for brucellosis in the routine diagnostic screening for SNHL. Practitioners in other locations should also consider this possibility when dealing with patients who have visited or lived in endemic regions.

Rhinocerebral aspergillosis.

Aspergillosis is increasingly being recognised as a common fungal infection of the paranasal sinuses. Although the disease is almost endemic in neighbouring Sudan, there are few reported cases from the Kingdom of Saudi Arabia. We report four cases of sinus aspergillosis with involvement of the skull bases and/or intracranial spread; a condition we have termed rhinocerebral aspergillosis. Invasive aspergillosis in our subgroup of patients occurs in otherwise healthy patients with normal immune status, quite unlike most reported cases in the western literature. The causative agent in all our patients was identified as aspergillus flavus, similar to patients reported from Sudan. This is again at variance with the case reports from other geographical locations, where aspergillus fumigatus is the commonest causative agent. Saudi Arabia would appear to represent a distinct geographical enclave, together with Sudan, where rhinocerebral aspergillosis of the sinuses and skull base may be more common than has previously been realised.

Otological manifestations of thalassaemia intermedia: evidence of temporal bone involvement and report of a unique cholesteatoma-like lesion.

Thalassaemia intermedia should be considered in any chronically anaemic patient presenting from the Middle East with hearing impairment. We report here three Saudi siblings with thalassaemia intermedia and features of severe bone marrow expansion, particularly invading the temporal bone. They were seen first for their otological problems before they had access to proper haematological evaluation. One member was admitted for surgical exploration of a cholesteatoma, which was then found to be marrow expansion of the temporal bone. Screening of the family revealed two more anaemic siblings with thalassaemia intermedia. Audiological examination of all the family members showed that only the two affected members had a high frequency sensori-neural hearing loss. Bone marrow expansion into the temporal bone is a rare feature of thalassaemia intermedia. Cholesteatoma-like lesion has not been previously described. It has to be considered in all cases of symptomatic thalassaemia intermedia manifesting with cavitation and lytic lesions in the mastoid system. The likelihood that sensorineural hearing loss may complicate the thalassaemias is raised and the possible mechanism for such involvement discussed. The proper management for different otological manifestations of the thalassaemias is suggested. These cases would suggest a more extensive involvement of the temporal bone in the thalassaemias than has been previously recognized. Further large scale studies are required to illuminate the subject.

Immediate skin sensitivity test reactivity in Nigerians with allergic rhinitis.

A report of skin tests on 62 patients with allergic rhinitis is presented. Detailed allergy history was obtained using Bencard case history chart. Skin test using Bencard skin testing kit was done on each patient. Results showed house dust was most common agent with 74.19% and house dust mite next with 70.38%. Most of the patients fall within the age group 21-30 years. The need to obtain extracts for the skin test locally is stressed as that will make the result more meaningful.

Etiological agents of otomycosis in Nigeria.

A total of 159 suspected cases of otomycosis comprising 101 adults and 58 children, 61 males and 98 females were investigated. Of these, 36 cases were confirmed specifically of mycotic etiology on the basis of microscopic demonstration of fungal structures in epithelial debris/plugs and positive culture. Another 31 cases positive for fungi by culture but negative for direct microscopy were considered of doubtful fungal etiology. The predominant etiological agents in the confirmed cases were Aspergillus niger (13 cases), followed by A. flavus (6), Candida albicans (6) and C. parapsilosis (4). Other species represented were Pseudoallescheria boydii (2), C. guilliermondii (1), Aspergillus sp. (unidentified) (2), and Candida sp. (unidentified) (1). One case was of mixed infection due to A. niger and C. albicans. Fungal cultures from normal healthy ear canals of 46 persons were positive in 17 cases, the predominant fungus being Aspergillus (mainly A. niger).

Epidemic yellow fever in eastern Nigeria, 1986.

An epidemic of yellow fever occurred in the eastern part of Nigeria during the second half of 1986. Oju, in Benue State, was the most heavily affected region, but yellow fever also occurred in surrounding areas, particularly Ogoja, in Cross River State. In Oju, the mean attack and mortality rates were 4.9% and 2.8%, respectively. Sex and age specific rates were highest in males and in the 20-29 yr age group. The overall case fatality rate was approximately 50%. Diagnosis was confirmed by IgM capture enzyme-linked immunosorbent assay (ELISA) and complement fixation (CF) tests. Entomological investigations implicated Aedes africanus as the epidemic vector. Oju alone probably had about 9800 cases of yellow fever with jaundice, and some 5600 deaths. Outbreaks of this nature could be prevented by inclusion of yellow fever in the Expanded Programme on Immunisation, in areas subject to recurrent epidemics.

Microdistribution of immature African blackflies resulting from water velocity and turbulence preferences.

1. The velocity preferences of larval and pupal blackflies were studied experimentally by comparing the colonization of plastic strings placed in different velocity ranges; and also by investigating the simuliid microdistribution under natural conditions in the river. This required a novel method described for taking spot measurements of water velocity. 2. Simulium mcmahoni de Meillon, S. hirsutum Pomeroy and S. cervicornutum Pomeroy were most abundant in slow velocities (0.3-1.0 m s-1), S. colasbelcouri Grenier & Ovazza and S. hargreavesi Gibbins preferred 1.1-1.8 m s-1, S. squamosum Enderlein and S. vorax Pomeroy 1.9-2.2 m s-1. 3. The larvae of S. hargreavesi and S. cervicornutum preferred a higher velocity range compared with their pupae. 4. In moderate velocities (1.1-1.4 m s-1), the abundance of S. hargreavesi was greater under turbulent conditions than in smooth-flowing water, but this was reversed at very high velocities (2.3-2.6 m s-1). 5. Velocity had no apparent effect on substrate preference when substrates of different flexibilities were compared for three blackfly species. All avoided the most flexible substrates and preferred ones consisting of rigid articulating plates. Anisopteran predators were found to have a similar substrate choice.

Incidence of Menière's disease.

A study of the incidence of Menière's disease in the otolaryngological population of a black community is presented. 34 patients out of a total attendance of 8,550 had proven Menière's disease, according to defined criteria, giving an incidence of 0.4 per cent. This compares favourably with the incidence in other racial groups but a peculiarly high proportion of female patients is noted. Some of the reasons for the apparent rarity of the disease in negroes are discussed and it is suggested that improved facilities may in future show that Menière's disease is no more rare in negroes than in other racial groups.

Bilateral otorrhagia and orbital hematoma complicating subgaleal hematoma.

A patient with bilateral otorrhagia and orbital hematoma following massive subgaleal hematoma is reported for the first time. The pathogenesis of the extensive subgaleal hematoma as well as the production of these unusual complications is discussed. This case would suggest that the weakest points in the attachment of the epicranial aponeurosis to the base of the calvarium are its attachments to the squamous part of the temporal bone and to the frontal bone under the eyebrow.

The chronic discharging ear in Nigeria.

A study of 386 Nigerian patients with chronic suppurative otitis media is presented. The disease usually starts in early life, even though presentation in hospital may be delayed until adult life. The important aetiological factors were found to be essentially socio-economic. Tubo-tympanic disease dominates the clinical picture and the rarity of the attic retraction type of chronic ear disease would suggest exceptionally good eustachian function in the population studied, but further study is required in this respect. It is suggested that complicated tympano-plastic procedures are in general inappropriate in the chronic ear population of this study.