scholarly journals Physical and Neurological Development of a Girl Born to a Mother with Methylmalonic Acidemia and Kidney Transplantation and Review of the Literature

Children ◽  
2021 ◽  
Vol 8 (11) ◽  
pp. 1013
Author(s):  
Alessia Marcellino ◽  
Cristiana Alessia Guido ◽  
Silvia Bloise ◽  
Saverio Mallardo ◽  
Sara Isoldi ◽  
...  
Keyword(s):  
Transplant Recipient ◽  
Methylmalonic Acid ◽  
Kidney Transplant Recipient ◽  
Methylmalonic Acidemia ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Neurological Effects ◽  
First Time ◽  
Significant Weakness

Background: actual literature suggests that children of methylmalonic acidemia patients are mostly healthy, but data are only partial, especially regarding long-term outcome. Therefore, our aim was to evaluate the possible long-term neurological effects of fetal exposure to high levels of methylmalonic acid in a child of a renal transplant recipient. Methods: we retrospectively evaluated the clinical and neurological records of a girl whose mother is a kidney transplant recipient affected by methylmalonic acidemia. Subsequently, we compared our results with the ones already published. Results: the girl’s weight and stature were within the normal range in the first years of life but, starting from 4 years of age, she became progressively overweight. Regarding the neurodevelopment aspects, for the first time we performed a complete and seriated neuropsychological evaluation, highlighting a mild but significant weakness in the verbal domain, with a worsening trend at three-year revaluation. Conclusions: since children of MMA patients are exposed to methylmalonic acid, the efforts of the physicians caring for these children should be directed on careful evaluation of growth, prevention of obesity and regular neurological examination together with structured neuropsychological tests to achieve a better insight in possible complications of pregnancy in patients suffering from this condition.

scholarly journals Depression in young adults

2004 ◽  
Vol 10 (1) ◽  
pp. 4-12 ◽  
Author(s):  
Daniel J. Smith ◽  
Douglas H. R. Blackwood
Keyword(s):  
Young Adults ◽  
Age At Onset ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Bipolar Spectrum Disorders ◽  
Spectrum Disorders ◽  
Atypical Presentations ◽  
Genetic Loading ◽  
First Time

Depression, as a heterogeneous collection of disorders, is likely to include subgroups that are more genetic in origin. In common with other neuropsychiatric disorders such as schizophrenia, Alzheimer's disease and Huntington's disease, earlier age at onset in depression is associated with higher genetic loading and poorer long-term outcome. Adolescents and young adults with depression are also at high risk of developing a bipolar illness. This article reviews depressive illnesses that occur for the first time in adolescence and young adulthood. Case studies are used to discuss atypical presentations and the evolving concept of bipolar-spectrum disorders.

scholarly journals A Revised Surgical Strategy for the Distal Tibiofibular Interosseous Osteochondroma

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Huarui Yang ◽  
Kangquan Shou ◽  
Shijun Wei ◽  
Zhi Fang ◽  
Qiwen Hu ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Ankle Joint ◽  
Tibial Osteotomy ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Potential Damage ◽  
The Stability ◽  
And Function ◽  
First Time

Osteochondroma is one of the most common benign bone tumor; however, the surgical treatment still remains a challenge for those that occur at the distal tibiofibular interosseous location. Previously, the transfibular approach has been successfully described, but the potential damage of the syndesmosis would give rise to the instability of the ankle joint and thus may result in the unfavorable long-term outcome. Here, a revised strategy which can protect the syndesmotic complex is introduced. From 2010 to 2017, eleven patients with the distal tibiofibular interosseous osteochondroma who underwent the revised surgery were collected. The distal fibular osteotomy and posterior tibial osteotomy were performed to keep the inferior syndesmosis intact for better stability of the ankle joint. Both the anterior tibiofibular ligaments (AITFL) and posterior tibiofibular ligaments (PITFL) have been preserved successfully, and thus, the stability of the ankle joint has been maintained due to our strategy. The VAS and AOFAS scores were utilized to assess the clinical outcome and function. Postoperatively, all the patients were pain-free and were able to wear the appropriate shoes at the last follow-up. Preoperative and postoperative AOFAS scores were 93.63±6.91 and 47.27±5.27 (P<0.05), respectively. Moreover, the average VAS score was 1.73±0.27 (compared with preoperative as 7.45±2.15, P<0.05), demonstrating obvious improvement after the operation. To our best knowledge, this is the first time to perform the resection of the distal tibial interosseous osteochondroma involving the fibula without interrupting the inferior syndesmotic complex especially the AITFL and PITFL. We believe that this strategy may pave a new way for optimized clinical outcome for these patients with distal tibiofibular interosseous osteochondroma. This clinical trial study is registered with number ChiCTR1900024690.

scholarly journals TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report

2021 ◽  
Vol 8 ◽  
Author(s):  
Marie Nagai ◽  
Takahiro Uchida ◽  
Muneharu Yamada ◽  
Shuuhei Komatsu ◽  
Kohei Ota ◽  
...  
Keyword(s):  
Transplant Recipient ◽  
Kidney Transplant ◽  
Kidney Transplant Recipient ◽  
Underlying Disease ◽  
High Dose ◽  
Histopathological Analysis ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Tafro Syndrome ◽  
First Case

A 57-year-old man who received a kidney transplant 4 years previously owing to unknown underlying disease presented with thrombocytopenia and fever. Hepatosplenomegaly and lymphadenopathy were observed, and development of prominent anasarca and worsening of renal function yielded the diagnosis of TAFRO syndrome. He was treated with high-dose steroids and plasmapheresis, and a thrombopoietin receptor agonist was administered for refractory thrombocytopenia. However, his general condition worsened, and he died on day 92. Histopathological analysis of a kidney autopsy specimen showed thrombotic microangiopathy characterized by glomerular endothelial swelling, mesangiolysis, and double contours of the glomerular capillary walls. His bone marrow showed megakaryocytic hyperplasia with mild reticulin fibrosis. Interestingly, these clinical and pathological features were remarkably similar to those the patient demonstrated before the kidney transplant, suggesting the recurrence of TAFRO syndrome. TAFRO syndrome is a rare systemic disorder whose concept has recently been established, but information on its long-term outcome is scarce. To our knowledge, this is the first case of TAFRO syndrome developing in a kidney transplant recipient, which suggests that disease recurrence occurs many years after the kidney transplant.

Long-term consequences of atrial fibrillation after aortic valve replacement

2017 ◽  
Vol 25 (3) ◽  
pp. 179-191 ◽  
Author(s):  
Hiroki Kohno ◽  
Hideki Ueda ◽  
Kaoru Matsuura ◽  
Yusaku Tamura ◽  
Michiko Watanabe ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Β Blocker ◽  
First Time ◽  
New Onset

Background Atrial fibrillation is a common complication after cardiac surgery, but the risk factors and long-term outcome after primary isolated aortic valve replacement remains to be clarified. Methods A single-center retrospective study was conducted on 157 patients who underwent first-time isolated aortic valve replacement between April 1999 and February 2015. Fifty-eight patients developed new-onset atrial fibrillation within 6 months postoperatively, and they were compared with patients who remained in sinus rhythm. Multivariate analyses, which incorporated the propensity score patient matching technique, were conducted to evaluate the long-term outcome of new-onset postoperative atrial fibrillation and identify patients at risk of developing this arrhythmia. Results At a mean follow-up of 52.4 months (range 8.4–200.7 months), mortality was significantly higher in patients who developed atrial fibrillation compared to those who remained in sinus rhythm (2.8%/patient-year vs. 0.2%/patient-year, respectively; p < 0.05). Patients developing atrial fibrillation were also at an independently increased risk of stroke and readmission during follow-up. Risk analysis revealed that advanced age (>70 years) and absence of a postoperative β-blocker were predictors of atrial fibrillation. Conclusions New-onset atrial fibrillation after first-time isolated aortic valve replacement correlated significantly with late morbidity and mortality. Advanced age and absence of a postoperative β-blocker may increase the incidence of atrial fibrillation.

scholarly journals Earliest pulmonary vein potential guided cryoballoon ablation is associated with better clinical outcomes than conventional cryoballoon ablation: a result from two randomized clinical studies

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
J Yonekawa ◽  
Y Mizutani ◽  
D Yamashita ◽  
Y Makino ◽  
T Hiramatsu ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Pulmonary Vein ◽  
Cryoballoon Ablation ◽  
Short Term ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Funding Sources ◽  
Baseline Characteristics ◽  
First Time

Abstract Background With regards to short-term outcome in atrial fibrillation (AF), the benefit of cryoballoon ablation (CBA) by pressing a balloon against the earliest pulmonary vein (PV) potential site during pulmonary vein isolation, (earliest potential [EP]-guided CBA) has been previously demonstrated. Objective The present study aimed to evaluate the long-term outcome of the EP-guided CBA. Methods This study included 136 patients from two randomized studies, who underwent CBA for paroxysmal AF for the first time. Patients were randomly assigned to the EP-guided and conventional CBA groups in each study. In the EP-guided CBA group, we pressed a balloon against the EP site when the time to isolation (TTI) after cryoapplication exceeded 60 s and 45 s in the first and second studies, respectively. The patients were followed up for 1 year after procedure. We compared the clinical outcomes between the EP-guided CBA group (68 patients) and the conventional CBA group (68 patients). Results No significant differences in baseline characteristics were observed between the two groups. Compared with the conventional CBA group, the EP-guided CBA group had a significantly higher success rate at TTI ≤90 s (98.5% vs. 90.0%, P&lt;0.001); lower touch-up rate and total cryoapplication; and shorter procedure time, and fluoroscopy time. The recurrence at 1-year after ablation was significantly lower in the EP-guided CBA group than in the conventional CBA group (6.0% vs. 19.4%; P=0.019). Conclusions The EP-guided CBA approach can facilitate the ablation procedure and achieve low recurrence at 1-year after ablation. FUNDunding Acknowledgement Type of funding sources: None. Earliest potential [EP]-guided CBA The recurrence at 1-year after ablation

scholarly journals Predicting Phrenic Nerve Palsy in Patients Undergoing Atrial Fibrillation Ablation With the Cryoballoon—Does Sex Matter?

2021 ◽  
Vol 8 ◽  
Author(s):  
Alexander Pott ◽  
Hagen Wirth ◽  
Yannick Teumer ◽  
Karolina Weinmann ◽  
Michael Baumhardt ◽  
...  
Keyword(s):  
Recovery Time ◽  
Nerve Palsy ◽  
Female Gender ◽  
Current Evidence ◽  
Patient Specific ◽  
Phrenic Nerve Palsy ◽  
Term Outcome ◽  
Long Term Outcome ◽  
First Time

Background: Phrenicus nerve palsy (PNP) is a typical complication during pulmonary vein isolation (PVI) using the cryoballoon with the ominous potential to counteract the clinical benefit of restored sinus rhythm. According to current evidence incidence of PNP is about 5–10% of patients undergoing Cryo-PVI and is more frequent during ablation of the RSPV compared to the RIPV. However, information on patient specific characteristics predicting PNP and long-term outcome of patients suffering from this adverse event is sparse.Aim of the Study: To evaluate procedural and clinical characteristics of AF patients with PNP during cryoballoon PVI compared to patients without PNP.Methods and Results: Between 2013 and 2019 we included 632 consecutive AF patients undergoing PVI with the cryoballoon in our study. 84/632 (13.3%) patients experienced a total number of 89 PNP during the ablation procedure. 75/89 (84%) cryothermal induced PNP recovered until the end of the procedure (transient PNP, tPNP), whereas 14/89 (16%) PNP hold beyond the end of the procedure (non-transient PNP, ntPNP). Using multivariate logistic regression, we found that sex and BMI are strong and independent predictors of cryothermal induced non-transient PNP during cryoballoon PVI with an odds ratio of 3.9 (CI: 95%, 1.1–14.8, p = 0.04) for female gender. Interestingly, all patients (14/14, 100%) with a non-transient PNP experienced complete PNP resolution after a mean recovery time of 68 ± 79 days.Conclusion: Our data indicate for the first time, that female sex and lower BMI are independent predictors for non-transient PNP caused by cryoballoon PVI. Fortunately, during follow up all PNP patients resolved completely with a median recovery time of 35 days.

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