Oral manifestations of sickle cell disease.

Sickle cell disease is one of the most common autosomal recessive genetic diseases. It gives rise to abnormally shaped red blood cells with altered function, the primary clinical features being haemolytic anaemia and vascular occlusion. Acute complications are frequent and variable and include chest syndrome, stroke, infection mainly due to asplenia, bone pain and priapism. Other chronic complications which can occur are bone necrosis, nephropathy and heart, lung and skin disorders. Oral lesions are also very common and include aseptic pulp necrosis, mucosal damage due to anaemia, fungal infections due to numerous antibiotic therapies, dental eruption delays, bone pain and osteomyelitis of the maxilla, and oral neuropathies, including of the mental nerve of the chin. The oral care of sickle cell patients requires specific precautions such as good management of local anaesthetics, rigorous anti-infective prophylaxis as well as controlled prescription of analgesics. Regular oral follow-up of sickle cell patients is necessary.

Management of Amelogenesis Imperfecta: A 15-Year Case History of Two Siblings.

OBJECTIVE: Amelogenesis imperfecta (AI) is a heterogenous genetic disorder that interferes with normal enamel formation in the absence of systemic disorders. The patients' main concerns are caries susceptibility, poor esthetics, and generalized sensitivity. There is a broad clinical spectrum, from discolorations to consequent enamel alterations. This case report describes the 15-year case study and the full-mouth rehabilitation of two siblings affected by a hypocalcified AI. Clinical Considerations: In these two patients, conservative care with stainless steel crowns and direct composite restorations was undertaken to restore function and esthetics and to reduce sensitivities in primary and mixed dentitions. The difficulties in monitoring resulted in severe infectious complications (dental abscess with cutaneous fistula), important dental defects, and loss of spaces with subsequent malocclusion. In the young adult dentition, they were treated by extractions, root canal therapies, and new restorations: stainless steel crowns for permanent molars, direct composite restorations (with strip crowns) for incisors and maxillary canines (to improve the crown morphology as well as to mask the discolorations and the malpositions), and adjusted composite crown molds using a thermoforming procedure for premolars and the mandibular canines. The main difficulties were rapid tooth surface loss, bonding to atypical enamel, developing dentition, long-term follow-up. CONCLUSION: Restoring function and esthetics in AI-affected patients is a challenge from primary to adult dentition. Early corrections are essential to avoid dental damage and for psychological benefits. This clinical report highlights the adhesive rehabilitation for anterior and premolar areas and the difficulty of patient follow-up.

Acute Methiopropamine Intoxication After "Synthacaine" Consumption.

Use of methiopropamine (MPA), a synthetic metamfetamine analog, has been detected since 2011 in Europe, but there is limited information on its acute toxicity. A 30-year-old man was admitted to the emergency department in a confused state, with paranoid delusion, auditory and visual hallucinatory experiences, and incoherent speech following the use of "synthacaine" (a slang term derived from "synthetic" and "cocaine"). Toxicological screening for pharmaceuticals and drugs of abuse by liquid chromatography-diode-array detector, gas chromatography-mass spectrometry and liquid chromatography-tandem mass spectrometry (LC-MS-MS) detected MPA, which was subsequently quantified by a specific LC-MS-MS method. Of note, 13 h after presentation to the emergency department, the plasma concentration of MPA was 14 ng/mL. This case report confirms the toxicity of MPA and the need for toxicological analysis to confirm the substance actually ingested by users of new psychoactive substances.

[Palliative tendon transfer for reanimation of the wrist and finger extension lag. Report of 14 transfers for radial nerve palsies and ten transfers for brachial plexus lesions]. / Les transferts palliatifs de réanimation de l'extension du poignet et des doigts. A propos de 14 transferts pour paralysie radiale et dix transferts pour lésion plexique.

This retrospective study is based on 23 males and one female, of an average age of 36.2 years that presented to us between 1982 and 2000 with an average follow up of 61 months, with fully established paralysis of wrist and fingers extension. Fourteen patients had isolated radial nerve palsy, while ten patients had brachial plexus lesions. 1) The tendon transfer for radial nerve palsy was: PT to ECRB, FCU to ED + EPL and PL to APL + EPB; 2) for brachial plexus injury, the tendon transfer was: PT (n = 4) or FDS III or IV (n = 5) to ECRB, FCU (n = 8) or FDS IV (n = 1) to ED + EPL, PL to APL + EPB and wrist arthrodesis with transfer of FDS IV to ED + EPL and PL to APL + EPB. The results were evaluated according to the degree of wrist movement, MP extension of long fingers, opening of first commissure, thumb opposition, grip power and the subjective evaluation of results. Concerning the radial nerve palsy: results are excellent in nine cases and good in one case. An active extension of the wrist of 38 degrees was obtained as well as MP extension of 0 degree with the wrist straightened. Thumb oppositioned was conserved (Kapandji = 8.2), opening of the first commissure 40 degrees and grip power was 20 kg. Concerning the brachial plexus lesions: results are excellent in five cases and good in the other five. An active wrist extension of 32 degrees was obtained, as well as MP extension deficit of 16 degrees with wrist straightened. Opposition was concerned (Kapandji = 7.2), opening of first commissure of 38 degrees and grip power of 13 kg. The functional results are satisfactory, but the analytic study shows some effect of tenodesis of MP extension.

[Inflammatory arteriopathy of the arms in the course of Horton disease. Report of six cases]. / Artériopathie inflammatoire des membres supérieurs au cours de la maladie de Horton. A propos de six cas.

We report 6 cases of upper limb involvement in giant cell arteritis; upper limb involvement revealed the disease in 4 cases and clinical symptoms were present in 5 (upper limb pain, Raynaud's phenomenon). Upper limb pulses were not palpable and blood pressure unmeasurable in all. Duplex ultrasonography found signs of inflammatory arteriopathy in 4 cases (hypoechogenous halo of the arterial wall and acceleration of flow velocity). Arteriography was performed in 5 cases and showed long and regular stenoses. In the last case, arteriography was not done because the duplex exploration gave an easier diagnosis. With this technique, the diagnosis of upper limb involvement, frequent in autopsy series of giant cell arteritis, might be made more often. Corticosteroid therapy is indicated and surgery should be discussed only in emergency situations.