Patients with complicated Pott's disease: Management in a rehabilitation department and functional prognosis

AbstractObjectiveThe objective is to study the rehabilitation management and to assess autonomy in daily life activities as well as walking recovery in patients with complicated Pott's disease.Patients and methodsRetrospective study in nine patients over a period of 8 years extending from 2000 to 2008, collated in the Department of Physical Medicine and Functional Rehabilitation, CHU Sahloul, Sousse, Tunisia.ResultsThe mean age of our patients was 43.8 years; sex ratio was 5/4. The spine involvement of tuberculosis was dorsal in seven cases, dorso-lumbar in one patient, and multiple (cervical, dorsal and lumbar) in one case. All patients were paraplegic with a neurological involvement of the bladder. They had prior antituberculosis chemotherapy for at least 8 months. Decompression surgery was performed in six cases. Two female patients presented disorders of spinal posture during treatment requiring surgical revision with osteosynthesis. All patients received additional rehabilitation care. Following a mean duration of hospitalisation in the Rehabilitation department of 47 days with twice-daily sessions of tailored physiotherapy, three patients remained in complete paraplegia, autonomous in wheel-chair and with vesical and sphincter incontinence. The measure of functional independence (MFI) was at admission/discharge 71/92.ConclusionRehabilitation takes an important place in the medico-surgical management in Pott's disease, to limite or compensate the disabilities and handicap related to this pathology

A recessive form of hyper-IgE syndrome by disruption of ZNF341-dependent STAT3 transcription and activity.

Heterozygosity for human () dominant-negative (DN) mutations underlies an autosomal dominant form of hyper-immunoglobulin E syndrome (HIES). We describe patients with an autosomal recessive form of HIES due to loss-of-function mutations of a previously uncharacterized gene, ZNF341 is a transcription factor that resides in the nucleus, where it binds a specific DNA motif present in various genes, including the promoter. The patients\u27 cells have low basal levels of STAT3 mRNA and protein. The autoinduction of STAT3 production, activation, and function by STAT3-activating cytokines is strongly impaired. Like patients with DN mutations, ZNF341-deficient patients lack T helper 17 (T17) cells, have an excess of T2 cells, and have low memory B cells due to the tight dependence of STAT3 activity on ZNF341 in lymphocytes. Their milder extra-hematopoietic manifestations and stronger inflammatory responses reflect the lower ZNF341 dependence of STAT3 activity in other cell types. Human ZNF341 is essential for the transcription-dependent autoinduction and sustained activity of STAT3