Pseudarthrosis and ¶delayed consolidation of the distal phalanges.

10.5580/4c0 ◽  
2010 ◽  
Vol 3 (1) ◽  
Author(s):  
Juan Carlos Caruso ◽  
Juan Martín Patiño

Las osteopetrosis (enfermedad de Albers-Schönberg) es un síndrome con cuatro tipos clásicos e instituye una displasia ósea secundaria a la falta de resorción de hueso por anormalidad de los osteoclastos, lo cual provoca un tejido óseo duro y quebradizo,propenso a fracturas difíciles de tratar quirúrgicamente. Se han publicado escasos artículos sobre el tema; por este motivo, decidimos presentar a dos pacientes con fracturas diafisarias de húmero con osteopetrosis, ambas tratadas en forma incruenta.Los objetivos son comunicar nuestra experiencia y el método de tratamiento de dicha afección y realizar una revisión bibliográfica acerca del tema. Creemos que el tratamiento de elección para las fracturas diafisarias de húmero en pacientes con osteopetrosises el incruento, ya que su tipo de tejido óseo dificulta la implementación de cualquier osteosíntesis. Además, la colocación de implantes puede provocar algunas complicaciones, como infecciones, retraso de la consolidación y seudoartrosis. La cirugía se reserva para ciertos casos, como en pacientes con riesgo de desarrollar deformidades incapacitantes, aquellos que han sufrido fracturas repetidas, con retraso de la consolidación, seudoartrosis, quienes no responden al tratamiento incruento o con unadeformidad previa.AbstractOsteopetrosis (also known as Albers-Schönberg disease) is a syndrome that includes four classic types and is characterized by bone dysplasia and lack of bone resorption due to abnormal osteoclastic activity and consequent development of brittle and hard bone that is prone to fractures that are difficult to treat surgically. Herein we present two cases of osteopetrosis with diaphyseal fractures of the humerus, both managed with non-surgical treatment. The objectives of our manuscript are to document our experience in the management of these cases and review the literature. The non-invasive treatment provides the best outcome for dyaphyseal fractures on the humerus in patients with osteopetrosis, given that the quality of the bone in these patients impairs the implementation of osteosynthesis. In addition, the placement of implants can lead to complications such as infections, delayed consolidation and pseudoarthrosis, among others. Surgical treatment should reserved for certain patients such as those with delayed consolidation, pseudoarthrosis, a history of repeated fractures, pre-existing deformity and those who are at risk for the development of disabling deformities or do not respond to non-surgical treatment.


Author(s):  
L.A. Beauchamp ◽  
M.J. Scoble ◽  
H.S. Mitri

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1872-1872
Author(s):  
Josep Ribera ◽  
Albert Oriol ◽  
Mireia Morgades ◽  
Josep Sarrá ◽  
Salut Brunet ◽  
...  

Abstract Current therapeutic protocols for adult ALL consider MRD together with the classical risk factors (age, WBC count, immunophenotype, cytogenetics and speed in response to therapy) for treatment decisions. The aim of the prospective study ALL-AR-03 from the Spanish PETHEMA Group was to evaluate the feasibility of sequential MRD detection and the response to a differentiated therapy according to MRD levels (assessed by cytofluorometry at the end of induction and consolidation therapy) in HR Ph- adult ALL pts. HR ALL included one or more of the following: age 30–60 yr, WBC count >25×109/L and 11q23 or MLL rearrangements. Induction therapy included VCR, DNR and PDN for 4 wk. In pts with slow cytologic response (≥ 10% blasts in BM assessed on d14) intensified induction with HD-ARA-C and mitoxantrone was administered. Early consolidation therapy included 3 cycles with rotating cytotoxic drugs including HD-MTX, HD-ARA-C and HD-ASP. Pts. with slow cytologic response on d14 or MRD level >0.05% after consolidation were assigned to allogeneic SCT (related or unrelated) and those with standard cytologic response on d14 and MRD level <0.05% after consolidation received 3 additional cycles of delayed consolidation (identical to those of early consolidation) followed by maintenance therapy up to 2yr in CR. On May 2006,119 patients were evaluable (mean (SD) age 37(14) yr, 64 males, 75 precursor B-ALL, 44 T-ALL, WBC 67(87) ×109/L). Induction death 7(%), resistance 20 (%), CR 93 (78%) and MRD<0.1% in 64% were observed. Early consolidation was completed in 63 patients. MRD<0.05% was observed in 77% at the end of consolidation. Allo-SCT was performed in 20 pts (TRM 6, relapse 1, CCR 13) and delayed consolidation and maintenance in 38 (toxic death 2, relapse 7, CCR 29). Two-yr DFS for the whole series was 35±13%. No significant differences were observed between pts receiving chemotherapy or SCT. Slow cytologic response was associated with a lower CR. No initial variable was associated with a higher probability of MRD negativity after induction or consolidation. Neither the negativity of MRD nor the pattern of clearance of MRD were associated with a better DFS. These preliminary results suggest that in HR adult ALL pts with adequate response to induction and adequate clearance of MDR the results of late consolidation and maintenance are not hampered by avoiding allo-SCT.


1996 ◽  
Vol 21 (2) ◽  
pp. 151-163 ◽  
Author(s):  
M. ABE ◽  
H. SHIRAI ◽  
M. OKAMOTO ◽  
T. ONOMURA

Ten patients aged 3 to 13 years (mean, 9 years and 7 months) underwent forearm lengthening by callotasis. The indications for lengthening were shortening and/or deformity of the forearm due to exostosis of the distal ulna in five cases, enchondroma of the distal ulna in one, growth disturbance after fracture of the distal radius in one, radial club hand in one, congenital amputation of the forearm in one and congenital dislocation of the radial head in one. Four had lengthening of the ulna, one of the radius and five of both the radius and the ulna. The average lengthening achieved was 30 mm. Complications encountered were pin track discharge in three cases, callus fracture in five, delayed consolidation of the callus in one and no callus formation in one. Review after 1 to 7 years follow-up (with a mean of 4 years and 9 months) showed satisfactory improvement in appearance and function especially in patients who had tumorous conditions or traumatic epiphyseal arrest.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 1333-1333 ◽  
Author(s):  
Josep-Maria Ribera ◽  
Mireia Morgades ◽  
Juana Ciudad ◽  
Pere Barba ◽  
Rodrigo Martino ◽  
...  

Abstract Introduction: Recent studies have shown that young to middle-aged adults who receive a pediatric-inspired chemotherapy regimen for treatment of Ph-neg ALL do not appear to require an alloHSCT if they achieve good response on MRD testing after induction therapy. Patients (pts) who are not good MRD responders achieve better outcomes with alloHSCT than their counterparts who do not receive alloHSCT. However, it is not clear if this approach can be translated to adult ALL pts with HR features at baseline. The aim of the prospective ALL-HR-11 trial from the Spanish PETHEMA Group was to evaluate the response to a differentiated post-induction therapy (chemotherapy or alloHSCT) according to MRD levels (assessed by 8-color, centrally-performed flow cytometry at the end of induction-week 5- and consolidation therapy-week 17-) in HR Ph-neg adult ALL patients. Patients and methods: HR ALL included one or more of the following parameters at baseline: age 30-60 yr, WBC count >30x109/L for B-cell precursor ALL or >100x109/L for thymic T-ALL, pro-B, early or mature T-ALL, 11q23 or MLL rearrangements or complex karyotype. Induction therapy included vincristine, prednisone, daunorubicin and asparaginase (E coli native or PEG according to center availability) for 4 weeks (Induction-1). FLAG-Ida was administered as intensified induction (Induction-2) in pts not achieving CR or those in CR with MRD≥0.1% at the end of induction. For pts in CR and MRD<0.1% early consolidation therapy included 3 cycles with rotating cytotoxic drugs with high-dose methotrexate, high-dose ARA-C and high-dose asparaginase (E coli native or PEG). These pts continued with delayed consolidation (identical to that of early consolidation) followed by maintenance therapy up to 2 yr. in CR if MRD levels after consolidation were <0.01%, otherwise they were assigned to alloHSCT. Pts in CR after Induction-2 received one consolidation cycle and were assigned to alloHSCT. Results: On June 2015, 115 HR ALL pts were evaluable [mean (SD) age 38(13) yr, 67 males, 80/114 precursor B-ALL, 34/114 T-ALL, WBC count 56(96) x109/L]. Results of Induction-1: therapy-related death: 4(4%), resistance: 11 (10%), CR: 95(86%). MRD<0.1% at the end of induction was observed in 75% of CR patients. Induction-2 was administered to 33 patients (no CR: 11, CR and MRD≥0.1%: 22). No differences in the CR rate or in the rate of MRD clearance after induction were observed according to the type of asparaginase administered, although significantly increased hepatic toxicity in consolidation was observed in patients treated with PEG-asparaginase. The 2-yr DFS and OS probabilities for whole series were 51%±18% and 62%±13%. By intention-to treat after Induction-1 36 pts were assigned to alloHSCT and 68 to delayed consolidation and maintenance. The 2-yr DFS and OS probabilities were 54%±25% and 49%±20%, respectively, for pts assigned to alloHSCT, and 50%±22% and 73%±17%, respectively, for those assigned to chemotherapy (P=0.002 for OS comparison). Patients with MRD<0.1% at the end of induction and <0.01% at the end of consolidation (n=51) showed a 2-yr DFS and OS of 55%±25% and 81%±18%, respectively. Conclusions: The preliminary results of this trial, in which the post-induction therapy decision is only based on MRD results, suggest that in HR, Ph-neg adult ALL pts with adequate MRD response after induction and after consolidation the results of therapy are not hampered by avoiding alloHSCT. Supported by grants RD12/0036/0029 (RTICC, FEDER), PI14/01971 FIS, Instituto Carlos III, and SGR225 (GRE), Spain Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3291-3291
Author(s):  
Josep-Maria Ribera ◽  
Albert Oriol ◽  
Mireia Morgades ◽  
Josep Sarra ◽  
Pau Montesinos ◽  
...  

Abstract Background and aim: Current therapeutic protocols for adult ALL consider MRD together with the baseline risk factors (age, WBC count, immunophenotype, cytogenetics) and speed in response to therapy for treatment decisions. On the other hand, the systematic use of allogeneic SCT for all adult patients (pts) with Ph- HR-ALL is still a matter of debate. The aim of the prospective study ALL-AR-03 from the Spanish PETHEMA Group was to evaluate the response to a differentiated therapy (chemotherapy or allogeneic SCT) according to early bone marrow blast clearance and MRD levels (assessed by cytofluorometry at the end of induction and consolidation therapy) in HR Ph- adult ALL patients. Patients and methods: HR ALL included one or more of the following baseline parameters: age 30–60 yr, WBC count >25x109/L and 11q23 or MLL rearrangements. Induction therapy included vincristine, prednisone and daunorubicin for 4 weeks. In pts with slow cytologic response to therapy (≥10% blasts in bone marrow assessed on d14) intensified induction with high dose ARA-C and mitoxantrone was administered. Early consolidation therapy included 3 cycles with rotating cytotoxic drugs including high-dose methotrexate, high-dose ARA-C and high-dose asparaginase. Pts. with slow cytologic response on d14 or MRD level >0.05% after consolidation were assigned to allogeneic SCT (related or unrelated) and those with standard cytologic response on d14 and MRD level <0.05% after consolidation received 3 additional cycles of delayed consolidation (identical to those of early consolidation) followed by maintenance therapy up to 2yr in CR. Results: On June 2008,192 patients were evaluable (mean (SD) age 37(10) yr, 105 males, 119 precursor B-ALL, 73 T-ALL, WBC count 65(99) x109/L). Induction death: 17(9%), resistance: 12 (6%), CR: 163 (85%). MRD<0.1% was observed in 64% of CR patients. Early consolidation was completed in 126 patients. MRD<0.05% was observed in 65% at the end of consolidation. On June 2008, allogeneic SCT was performed to 30 pts (15 from HLA-identical siblings and 15 MUD), TRM 11 pts, relapse 4, CCR 15. Delayed consolidation and maintenance was administered to 79 pts (toxic death 4 pts, relapse 21, CCR 54). Four-yr DFS for the whole series was 36±7% (37±19% for pts assigned to SCT and 56±12% for those assigned to chemotherapy). Slow cytologic response was associated with a lower CR probability and higher induction death. No baseline variable was associated with a higher probability of MRD negativity after induction or consolidation. Conclusions: These results suggest that in HR Ph- adult ALL pts with adequate response to induction and adequate clearance of MDR the results of therapy are not hampered by avoiding allogeneic SCT. Supported by grants P-EF/07 from FIJC and RD 06/0020/1014 from Instituto Carlos III


2009 ◽  
Vol 0 (1) ◽  
pp. 57
Author(s):  
G. Zimmermann ◽  
P. Henle ◽  
M. Kusswette ◽  
A. Moghaddam ◽  
A. Wentzensen ◽  
...  

2017 ◽  
Vol 3 (3) ◽  
pp. 1-3
Author(s):  
Dembélé B ◽  
Diouf AB ◽  
Daffé B ◽  
Gueye AB ◽  
Sarr L ◽  
...  

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