Functional and psychosocial impact of COVID-19 pandemic on rheumatic patients’ quality of life in Saudi Arabia

Quality of Life Research - The COVID-19 pandemic might add to the stressors experienced by people living with rheumatic diseases. This study aimed to examine rheumatic patients’ functional...     

Immunoglobulin replacement therapy contribution in agammaglobulinemia: 10 case reports]

Intravenous immunoglobulin (Ig IV) has been used for many years in the treatment of primary antibody deficiencies. We performed a retrospective study of the clinical features and outcome of agammaglobulinemia children who received prolonged Ig IV infusions. PATIENTS AND METHODS: Ten children, 9 male et 1 female, with agammaglobulinemia diagnosis were studied for the clinical manifestations before and during the Ig IV replacement therapy. Serum Ig levels were quantified by nephelometry. Circulating B ant T cells were counted by immunofluorescence labeling by monoclonal antibodies. T-cell functions were assessed by using mitogen and antigen -induced T-cell proliferation assays in vitro. Patients clinical status was evaluated respectively, before initiation and at every moment (when patients had an infection) of the replacement therapy. RESULTS: Ig IV therapy was performed for 866 cumulated months, median 108 months. The median Ig IV doses administered to the 10 patients was 500 mg/kg/month. Residual serum IgG mean level was 3,9 g/L. All patients had 99 bacterial infections/year before Ig IV, mainly respiratory tract infections (48,5%), and 4 patients had bronchiectasis before Ig replacement therapy. The number of infection/year fall to 25 during IgIV replacement, and the infection/patient/year rate decreases significantly. One patient developed an Echovirus 27 meningoencephalitis during this treatment. CONCLUSION: Ig IV therapy with residual IgG mean level of 3,9 g/l reduced significantly the rate of bacterial infections. The use of specific antibiotherapy and respiratory kinesitherapy led to a lower rate of respiratory tract infections, and the stabilisation of the bronchiectasis. However this intravenous replacement therapy does not protect against viral meningoencephalitis.     

Anatomy of the arterial vascularization of the lips

Summary The findings from 12 dissections of previously injected facial masks, 8 dissections of the face following intrarterial injection of a red solution of Latex Neoprene, and a corrosion cast specimen allowed us to study the arterial supply of the lips. The arterial supply of the upper lip arises mainly from the superior labial arteries, but also from the subseptal arteries and from the subalar arteries. There is a figure of 8 shaped anastomotic system between these arteries lying on the upper lip. The arterial supply of the lower lip arises from the inferior labial arteries and from branches of the mental artery. A constant inferior labial arterial network was shown at the level of the lower lip arising in a fifth of cases from a T-shaped inferior labial artery. All these recent anatomic findings help us to improve our understanding of plastic surgery of the lips.

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Vascularisation artérielle des lèvres

Résumé Douze dissections de masques faciaux préalablement injectés à la gélatine minium, huit dissections de face après injection artérielle par une solution de latex néoprène rouge, et une injectioncorrosion a permis l'étude de la vascularisation artérielle labiale. La lèvre supérieure reçoit sa vascularisation artérielle des artères labiales supérieures principalement, mais aussi des artères sous-narinaires branches des artères faciales, et des artères de la sous-cloison. Il existe un système d'anastomose fréquent entre les trois artères décrivant un aspect de 8 de chiffre couché au niveau de la lèvre supérieure. La lèvre inférieure est vascularisée par les artères labiales inférieures et par des branches de l'artère mentonnière ; il existe de manière constante au niveau de la lèvre inférieure une arche artérielle labiale inférieure provenant une fois sur cinq d'une artère labiales inférieure en T unique. Toute ces données anatomiques récentes permettent d'envisager dans de meilleures conditions la chirurgie réparatrice des lèvres.

     

Contribution to carrier detection and genetic counselling in X linked retinoschisis.

X linked retinoschisis (RS) is a vitreoretinal disease resulting from microcystic degeneration of the macula associated with peripheral lesions. The disease gene has already been assigned to the distal short arm of the X chromosome (Xp22.2) by linkage studies. In order to contribute both to a better localisation of the RS locus and to genetic counselling in RS families, we have carried out a clinical and genetic analysis in seven pedigrees. We show, first, that in contrast with previous reports, heterozygote carriers frequently express the disease, and display peripheral retinal alterations similar to those found in affected males. Second, while distal markers DXS16, DXS207, and DXS43 are closely linked to the disease locus, a high level of recombination events was found with centromeric markers, namely DXS274, DXS41, and DXS164. These findings must be taken into account for both carrier detection and prenatal diagnosis in X linked RS.     

Detection and molecular typing of human papillomaviruses: prevalence of cervical infection in the Tunisian central region

There are compelling molecular and epidemiological data which indicate that infection with certain genital human papillomaviruses (HPVs), such as HPV 16 and HPV 18, has a critical role in initial changes that lead to cervical and probably other anogenital cancers. These observations prompted us to investigate the prevalence of cervical infection with genital human papillomaviruses in Tunisia. We used the polymerase chain reaction (PCR) to detect and type HPV DNA. The prevalence of HPV infection in a population of 106 Tunisian women recruited at the Offices Nationaux de la Famille et de Population (ONFP) was 13.6%. Molecular HPV typing indicated a high prevalence of HPV at high oncogenic risk; Our results indicate that the infection with genital human papillomaviruses is frequent in the Tunisian population.     

Nosocomial outbreak of acute gastroenteritis in a neonatal intensive care unit in tunisia caused by multiply drug resistantSalmonella wien producing SHV-2 beta-lactamase

In a Tunisian hospital 27 babies, including 12 who were premature, in a single intensive care unit suffered acute gastroenteritis in the period from January to May 1988. The mean age at the onset of gastroenteritis was 8.4 days; nine babies died.Salmonella wien was isolated from stools (all babies) and blood (4 babies). It was also isolated from the stools of one nurse and from a mattress. Twelve of the babies had received cefotaxime, which was successfully replaced by oral colimycin. The outbreak was stopped by the implementation of infection control measures. All isolates ofSalmonella wien were of the same biotype, and had the same antibiotic resistance pattern (third generation cephalosporins, monobactams, aminoglycosides, chloramphenicol, trimethoprim and sulphonamides) and plasmid DNA restriction pattern. The isolates were all susceptible to a combination of cefotaxime and clavulanic acid (a -lactamase inhibitor), which displayed synergy, suggesting the presence of a -lactamase (geometric mean MICs 11.24 µg/ml for cefotaxime alone and 0.24 µg/ml in combination with 0.1 µg/ml potassium clavulanate). All isolates produced TEM-1 and SHV-2 -lactamase which was not transferable toEscherichia coli by conjugation. The presence of the SHV-2 enzyme inSalmonella wien may allow it to adapt to newer -lactams which is a cause for concern in this hospital.     

Intrafamilial phenotypic variability of Hallervorden-Spätz syndrome in a Tunisian family

We report clinical features, CT-scan and MRI findings of three siblings with Hallervorden-Sp?tz disease, one of them followed for more than 20years. Patient 1 presented at age 10 with progressive generalized dystonia. Five years later she had violent dystonic spasms with opisthotonos and marked oro-mandibular involvement. Later, dystonia stabilized and examination showed dysarthria, multiple deformities related to dystonic posturing, retinal degeneration and no cognitive impairment. Cerebral CT-scan showed bilateral pallidal lucencies and T2 cerebral MRI showed the "eye of the tiger" sign.Patient 2 developed delusions and hallucinations at age 18 with acute phases of hetero-aggressivity diagnosed as "schizophrenic" in a psychiatric hospital. At age 20, he developed oromandibular dystonia, severe dysarthria and epilepsy. Cerebral MRI showed the same "eye of the tiger" sign.Patient 3 presented at age 37 with isolated psychiatric features similar to those of patient 2, also diagnosed "schizophrenic" in a psychiatric hospital. Neurological examination showed mild postural tremor of the hands and intermittent cervical dystonia. He was stabilized with neuroleptic treatment.This family shows marked intrafamilial variability of age at onset, symptom at onset (with "schizophrenic" features unusually described and leading to misdiagnosis), clinical presentation (almost entirely dystonic in one patient and almost entirely psychiatric in two others) and course of the disease (slowly progressive in one sibling, rapidly deteriorating in the second and stabilization in the third). Classification and nosology of subtypes of HSD are discussed.     

Correlation of optic nerve sheath diameter measurements by computed tomography and magnetic resonance imaging

BackgroundTraditionally, intracranial pressure is measured by direct ventriculostomy, which is invasive. Noninvasive measures such as bedside ultrasound and magnetic resonance imaging have been advocated and utilized recently to assess the intracranial pressure. The role of this study is to determine the degree of agreement between measurements of the optic nerve sheath diameter by computed tomography (CT) and magnetic resonance imaging (MRI).Materials and MethodsRetrospective chart review of 100 consecutive patients who had both MRI and CT scan of the head from January 1, 2011, until March 31, 2013, at our center was performed. A discrepancy of 0.2 mm between the 2 measurements was set as acceptable difference. The measurements of optic nerve sheath diameter (ONSD) were compared for agreement between the 2 modalities using the method by Bland and Altman.ResultsA total of 100 patients with both MRI and CT scan of the head were selected. Of these 100 patients, 24 were male and 76 were female. The average age was 63 years. No ONSD abnormality was detected in any of the patients. The discrepancy in measurements of the ONSD between CT and MRI in transverse plane was less than the predetermined cut-off value of 0.2 mm. Within-subject variance was estimated at 0.0058 for both CT and MRI.ConclusionComparable results without significant discrepancy as predetermined by the study groups were obtained from CT scan. Measurement of ONSD by CT scan can be used to indirectly asses the intracranial pressure in addition to clinical assessment and other signs of increased intracranial pressure on CT scan.     

Intraspecific 16S rRNA gene diversity among clinical isolates of Neisseria species

In the present work, nearly the entire 16S rRNA gene sequences of 46 clinical samples of Neisseria spp. were determined, and the aligned sequences were analyzed to investigate the diversity of 16S rRNA genes in each commensal Neisseria species. Two 16S rRNA types were identified in two Neisseria sicca strains, three 16S rRNA types in five Neisseria macacae strains, fourteen 16S rRNA types in twenty Neisseria flavescens isolates, and fourteen 16S rRNA types in nineteen Neisseria mucosa isolates. The number of nucleotides that were different between 16S rRNA sequences within specie ranged from 1 to 15. We found high intraspecific sequence variation in 16S rRNA genes of Neisseria spp. strains.