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Background

Multiple myeloma (MM) is a hematologic malignancy of plasma cell origin. MM primarily affects bone marrow, but extramedullary sites can also be involved. Myelomatous pleural effusion (MPE) is an atypical and rare complication of MM. We aimed to systematically study the incidence and clinicopathologic profile of patients with MPE in a real-world setting.

Patients and Methods

In this retrospective study, 415 consecutive patients with MM managed at a tertiary care center in North India during a study period of January 1, 2010 to December 31, 2015 were evaluated for MPE. The patients with MPE were analyzed for their clinical profile, diagnosis, treatment, and outcomes.

Results

Of these 415 patients, 11 (2.65%) patients had MPE. The median age of the study population was 50 years with male preponderance. The majority of these patients had immunoglobin (Ig)G Kappa disease. All patients had higher than International Staging System stage I disease. MPE was a presenting feature at MM diagnosis in 45.45% (n = 5) of the patients, whereas the rest developed MPE during follow-up. MPE presented predominantly (81.8%) as a unilateral effusion. Concurrent extramedullary involvement at other site was seen in 45.45% (n = 5), with 3 (27%) patients having concurrent myelomatous ascites. Six of these were managed aggressively, whereas 5 patients opted for palliation. The outcomes were dismal (90.9% mortality), with a median survival of 2.47 months.

Conclusion

MPE is a rare entity, and positive outcomes of therapy remain low with dismal prognosis.  相似文献   
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Background

Despite significant pain relief following total hip arthroplasty (THA) in patients with ankylosing spondylitis, a small subset of patients presenting with extra-articular extension contracture of hips remains unsatisfied.

Methods

We retrospectively evaluated the patients with ankylosing spondylitis who underwent simultaneous bilateral THA and had extensor tightness of both hips preoperatively. They were managed with modified Z-plasty of iliotibial band. Patients with windswept deformity, commonly seen in bilateral hip arthritis caused by ankylosing spondylitis, were excluded.

Results

Between July 2011 and June 2015, out of 148 patients with bilateral hip involvement, 10 patients (20 hips) had extension contracture of both hips that was addressed during surgery. All patients were followed up for a minimum of 2 years. They could sit comfortably on a chair of height 18 inches with hips and knees flexed to at least 90°. The mean postoperative sum range of motion was 144.6° with an average hip flexion of 95° (range, 90°-105°). None of them had recurrence of extension contracture. There was significant improvement in range of motion and hence ambulation and function. No radiolucent lines exceeding 2 mm were seen in any of the zones around either of the components as evaluated in latest X-rays.

Conclusion

Extension contracture of hip although rare is a noticeable problem and needs to be addressed during THA. Modified Z-plasty technique of iliotibial band is a reliable method in managing these patients.  相似文献   
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Purpose

This study aimed to investigate the association between target volume margins and clinical outcomes for patients with inoperable non-small cell lung cancer (NSCLC) treated with concurrent chemoradiation therapy.

Methods and materials

We reviewed the records of 82 patients with inoperable NSCLC treated between 2009 and 2016 with concurrent chemoradiation. All patients received positron emission tomography–based treatment planning, 4-dimensional computed tomography simulation to define an internal target volume, and daily cone beam computed tomography. We quantified variations in target volume margins with a margin deviation index (MDI), calculated as the percentage change in equivalent uniform dose between the original planning target volume (PTV) and a standard reference PTV 10 mm beyond the original gross tumor volume, consistent with the minimum margins mandated by recent NSCLC trials. Greater MDIs equated to smaller effective target volume margins. We dichotomized patients by the upper tercile MDI value (5.8%). Endpoints included time to locoregional progression and time to grade ≥ 3 radiation esophagitis (RE3) or radiation pneumonitis (RP3), modelled with the Fine-Gray method.

Results

Median follow-up was 37.8 months (range, 5.9-58.1 months). Larger MDIs correlated with smaller clinical target volume (CTV) + PTV margins, larger gross tumor volumes, later treatment year, and intensity modulated radiation therapy use. The risk of locoregional progression did not differ for MDI ≥5.8% versus <5.8% (adjusted hazard ratio: 0.88; P = .76), but the risk of RE3 or RP3 was decreased for MDI ≥5.8% (adjusted hazard ratio: 0.27; P = .027). Patients with MDI ≥5.8% were treated with smaller CTV + PTV margins (median, 5.6 vs 8 mm; P < .0001) and a marginally lower volume of esophagus receiving ≥50 Gy (median, 31.1% vs 35.3%; P = .069).

Conclusions

Smaller margins were used for larger tumors but were not associated with an increase in locoregional failures. Additional studies could clarify whether smaller margins, when used alongside modern radiation therapy techniques, decrease treatment-related toxicity for inoperable NSCLC.  相似文献   
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Methylmalonic acidemia (MMA) is most common inherited type of organic acidemia. It has diverse presentation in older infants without any initial apparent symptoms. MMA sometimes present with sudden metabolic decompensation, which may mimics common emergencies like septic shock and diabetic ketoacidosis (DKA) without early recognition can be fatal. In born error of metabolism especially organic acidemia should be suspected in any infant presented with severe high anion gap metabolic acidosis. We report two cases of MMA in infants presented acutely mimicking DKA and septic shock.  相似文献   
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