Oculomotor paralyses in the course of diabetes--concerning 12 cases

PURPOSE: Our purpose is to study the clinical features of the oculomotor paralyses in the course of the diabetes, to assess the risk factors and to show the importance of the diagnosis and the cerebral imaging. MATERIAL AND METHODS: We report a series of 12 diabetic patients with OMP collected at the department of ophthalmology in UHC Ibn Rochd in Casablanca between 1995 and 1998. RESULT: Our study was about 9 men and 3 women, the mean age was 58 years 6 months. The diabetes was type I in 2 cases and type II in 10 cases. All the patients complained of diplopia with headache. The paralysis of VI has been noticed in 8 cases, it was bilateral in 5 cases. Unilateral Ill palsy occurred in 4 cases. Brain computed tomography was normal in 10 cases and pathologic in 2 cases. Treatment consisted in alternating occlusion in all patients. Concomitance was achieved, after 4 to 6 months of treatment. CONCLUSION: The metabolic abnormalities due to hyperglycemia and the ischemic phenomena due to the diabetic macroangiopathy explain these palsies.     

A vital role of tubulin-tyrosine-ligase for neuronal organization

Tubulin is subject to a special cycle of detyrosination/tyrosination in which the C-terminal tyrosine of alpha-tubulin is cyclically removed by a carboxypeptidase and readded by a tubulin-tyrosine-ligase (TTL). This tyrosination cycle is conserved in evolution, yet its physiological importance is unknown. Here, we find that TTL suppression in mice causes perinatal death. A minor pool of tyrosinated (Tyr-)tubulin persists in TTL null tissues, being present mainly in dividing TTL null cells where it originates from tubulin synthesis, but it is lacking in postmitotic TTL null cells such as neurons, which is apparently deleterious because early death in TTL null mice is, at least in part, accounted for by a disorganization of neuronal networks, including a disruption of the cortico-thalamic loop. Correlatively, cultured TTL null neurons display morphogenetic anomalies including an accelerated and erratic time course of neurite outgrowth and a premature axonal differentiation. These anomalies may involve a mislocalization of CLIP170, which we find lacking in neurite extensions and growth cones of TTL null neurons. Our results demonstrate a vital role of TTL for neuronal organization and suggest a requirement of Tyr-tubulin for proper control of neurite extensions.     

TGF-β is responsible for NK cell immaturity during ontogeny and increased susceptibility to infection during mouse infancy

A large gap in our understanding of infant immunity is why natural killer (NK) cell responses are deficient, which makes infants more prone to viral infection. Here we demonstrate that transforming growth factor-β (TGF-β) was responsible for NK cell immaturity during infancy. We found more fully mature NK cells in CD11c(dnR) mice, whose NK cells lack TGF-β receptor (TGF-βR) signaling. Ontogenic maturation of NK cells progressed faster in the absence of TGF-β signaling, which results in the formation of a mature NK cell pool early in life. As a consequence, infant CD11c(dnR) mice efficiently controlled viral infections. These data thus demonstrate an unprecedented role for TGF-β in ontogeny that can explain why NK cell responses are deficient early in life.     

Important neuronal toxicity of microtubule-bound Tau in vivo in Drosophila

The microtubule-associated protein Tau is found in large amount in axons of neurons and is involved in human neurodegenerative diseases called tauopathies, which include Alzheimer's disease. In these diseases, the Tau protein is abnormally hyperphosphorylated and one therapeutic strategy currently under consideration consists in inhibiting Tau phosphorylation. However, the consequences of an excess of hypophosphorylated Tau onto neuronal physiology have not been investigated in vivo. Here we studied how important is Tau phosphorylation for axonal transport and neurohormone release in vivo, using the Drosophila model. Surprisingly, our results demonstrate a stronger toxicity of hypophosphorylated Tau for neuronal function, when compared with normal or pseudophosphorylated Tau. This reveals a potential limit of the current therapeutic strategy aimed at inhibiting Tau phosphorylation.     

Rhabdomyolyse et syndrome des loges des deux avant-bras lors d’une chirurgie robotique de longue durée

Robotic assisted laparoscopic surgery allows for a more precise dissection than classical laparoscopic surgery. However, it sometimes imposes specific exaggerated postures and extralong procedure duration. Combining these two factors may increase the risk for postural complications in at-risk patients. We report the case of an obese 30-year-old female patient who underwent a 12-hour duration robotic laparoscopic surgery for severe endometriosis, in Trendelenburg position. This was complicated by a two forearms rhabdomyolysis, with subsequent compartment syndrome with multiple neuropathy. Physicians must be aware of the cumulative risk for postural complications when extreme positions are associated to long duration procedures in predisposed patients.     

Long-term outcome of liver transplantation for autoimmune hepatitis: A French nationwide study over 30 years

Background & Aims

Autoimmune hepatitis (AIH) is a rare indication for liver transplantation (LT). The aims of this study were to evaluate long-term survival after LT for AIH and prognostic factors, especially the impact of recurrent AIH (rAIH).

Methods

A multicentre retrospective nationwide study including all patients aged ≥16 transplanted for AIH in France was conducted. Early deaths and retransplantations (≤6 months) were excluded.

Results

The study population consisted of 301 patients transplanted from 1987 to 2018. Median age at LT was 43 years (IQR, 29.4–53.8). Median follow-up was 87.0 months (IQR, 43.5–168.0). Seventy-four patients (24.6%) developed rAIH. Graft survival was 91%, 79%, 65% at 1, 10 and 20 years respectively. Patient survival was 94%, 84% and 74% at 1, 10 and 20 years respectively. From multivariate Cox regression, factors significantly associated with poorer patient survival were patient age ≥58 years (HR = 2.9; 95% CI, 1.4–6.2; p = 0.005) and occurrence of an infectious episode within the first year after LT (HR = 2.5; 95% CI, 1.2–5.1; p = 0.018). Risk factors for impaired graft survival were: occurrence of rAIH (HR = 2.7; 95% CI, 1.5–5.0; p = 0.001), chronic rejection (HR = 2.9; 95% CI, 1.4–6.1; p = 0.005), biliary (HR = 2.0; 95% CI, 1.2–3.4; p = 0.009), vascular (HR = 1.8; 95% CI, 1.0–3.1; p = 0.044) and early septic (HR = 2.1; 95% CI, 1.2–3.5; p = 0.006) complications.

Conclusion

Our results confirm that survival after LT for AIH is excellent. Disease recurrence and chronic rejection reduce graft survival. The occurrence of an infectious complication during the first year post-LT identifies at-risk patients for graft loss and death.