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Open access

Kamila Skwierawska, Anna Waszczuk-Gajda, Agnieszka Perkowska-Ptasińska, Grzegorz Basak and Wiesław W. Jędrzejczak

Abstract

Termin gammapatia monoklonalna o znaczeniu nerkowym (MGRS) w akronimie różni się jedynie jedną literą od gammapatii monoklonalnej o nieokreślonym znaczeniu (MGUS), jednakże w znaczeniu klinicznym jest to zupełnie inna jednostka. W przebiegu MGRS białko produkowane przez klon komórek uszkadza nerki, przez co może prowadzić do ich niewydolności. W niniejszym artykule dokonano przeglądu piśmiennictwa dotyczącego jednostek chorobowych zaliczanych do grupy MGRS, ich podziału ze względu na typ uszkodzenia nerek i charakterystykę deponowanych w nich złogów. W pracy omówiono także współczesne możliwości leczenia w poszczególnych jednostkach chorobowych zaliczanych do MGRS.

Open access

Jiayu Huang, Yun Lian and Sixuan Qian

Abstract

Older patients with acute myeloid leukemia (AML) are encumbered with poor long-term outcomes due to patient and disease characteristics. Hypomethylating agents (HMAs), acting as DNA methyltransferase (DNMT) inhibitors, have been established as a new treatment option, but they have been associated with relatively low response rates (15%–20% complete remission) when administered separately for treating elderly with AML. However, appropriate combination therapies with decitabine or azacitidine have flourished. The results of randomized trials of various combinations of HMAs with chemotherapy, histone deacetylase inhibitors, monoclonal antibodies, immunomodulatory agents, kinase inhibitors, or bexarotene are summarized.

Open access

Cristiane da Silva Rodrigues de Araújo, Mirna Maira Bezerra Calazan do Carmo, Claudia de Alvarenga Maximo and Sílvia Maia Farias de Carvalho

Abstract

Adult T-cell leukemia/lymphoma (ATL) related to the human T-cell lymphotropic virus type I (HTLV-I) is a malignant lymphoproliferative disease. ATL is classified in four subtypes: lymphoma, acute, smoldering and chronic. We analyzed, retrospectively, 46 consecutive patients with T-cell disease with ATL diagnosed from 1995 to 2007. ATL diagnose was confirmed in 37 of these patients. There were 26 females and 11 males (70% vs 30%, respectively, p=0.014). The median age was 42 years old. Twenty-five were nonwhite and twelve were white (67.6% vs 32.4%, respectively, p=0.033).Twenty two patients had the acute form, eight had chronic form and seven had lymphomatous form. Two of them had osteolytic lesions. There were two cases with pulmonary infiltrates; one patient had ATL associated to Hansen’s disease. All cases had antibodies to HTLV-I confirmed by Western Blot, polymerase chain reaction (PCR) was performed in 22 cases. Flow cytometry revealed positivity for CD4 and CD25 in most cases. The mean and median overall survival time was 11.3 months and 2 months, respectively. The family´s study showed that 60% of the mothers were HTLV-I seropositive. These data emphasize the importance of a serologic screening for HTLV and immunophenotyping to differentiate ATL from others T-lymphoproliferative disorders.

Open access

Bembnista Ewa, Kubiak Agnieszka, Matuszak Paula and Kozłowska-Skrzypczak Maria

Streszczenie

W procesie przygotowania komórek krwiotwórczych (k.k.) do transplantacji istotne jest wdrożenie oraz utrzymanie procedur pozwalających na zachowanie mikrobiologicznego bezpieczeństwa przeszczepu. Do głównych źródeł zanieczyszczenia przeszczepu należy: powietrze, personel, woda oraz materiał. Kontaminacja przeszczepu może mieć miejsce na etapie pozyskania, przetwarzania oraz dystrybucji k.k. Jakość materiału transplantacyjnego powinna być kontrolowana na wszystkich kluczowych etapach jego przetwarzania. Bankowanie k.k. może odbywać się w systemie otwartym lub zamkniętym. System przetwarzania k.k., otwarty lub zamknięty, znacząco wpływa m. in. na kryteria kwalifikacji pomieszczeń oraz przygotowania pracowników do pracy. Jednakże niezależnie od systemu przetwarzania k.k., kontrola mikrobiologiczna powinna obejmować zarówno materiał transplantacyjny, jak i środowisko jego przewarzania. Formalne wymogi dla banków komórek zawarte są w Rozporządzeniach Unii Europejskiej, Ustawie Transplantacyjnej oraz Rozporządzeniach Ministra Zdrowia. Wdrożenie oraz utrzymanie procedur odpowiedniego przygotowania pracowników, sprzętów oraz pomieszczeń do pracy w znaczący sposób wpływają na zmniejszenie ryzyka kontaminacji przeszczepu, a tym samym na kliniczne parametry leczenia transplantacyjnego.

Open access

Joanna Płużańska and Maria Respondek-Liberska

Abstract

The prenatal detection of congenital anomalies of heart walls is very rare. We present a unique series of 8 cases with prenatal echocardiographic monitoring, treatment and postnatal follow-up, providing new insight into this “mysterious” heart problem

Open access

Tetsuya Goto, Chika Ndubuisi, Tatsuya Kobayashi, Takafumi Kiuchi, Kunihiko Kodama, Keiichi Sakai and Kazuhiro Hongo

Abstract

The extent of resection has been shown to influence the outcome of brain tumours. The concept of brain plasticity is to prevent damage to the eloquent areas while maximizing the extent of tumor resection. The present case report describes the usefulness of the near-infrared spectroscopy (NIRS) in evaluation of the possibility of functional preservation for surgeries in eloquent areas. We present a 46-year-old woman, who had previously undergone four sessions of internal decompression surgery for left frontal anaplastic ependymoma. She later underwent radical tumor resection surgery involving the superior and middle frontal gyri, anterior parietal gyrus, corpus callosum, coronal radiation and basal ganglia. Postoperatively, her right hemiparesis did not deteriorate and she could ambulate without much aid. Multichannel NIRS system revealed that hot spot location was in the right superior frontal gyrus and parietal lobe by the same task of right knee joint movement. We judged that her right brain motor function shifted to the contralateral hemisphere by the long course of her illness. It might be possible that if the NIRS was used earlier around the third or fourth perioperative period, with a reliable confirmation of migration of the right motor function to opposite side, the option of a more aggressive tumor resection may have been attempted. NIRS can be a useful and sensitive tool for predicting the location of eloquent areas and monitoring the extent of brain plasticity between surgeries.

Open access

F. Salle, A. Jaume, G. Castelluccio and E. Spagnuolo

Abstract

The ideal treatment for intracranial aneurysms has been highly controversial in the last few decades. It is particularly difficult to decide between clipping vs. coiling when it comes to an aneurysm that has already been treated. The authors performed a review of the literature published in the last ten years amongst the main neurosurgical publications and make recommendations based on this evidence and the surgical experience of the eldest author of this paper (ES). A series of cases of recurrent, incompletely coiled aneurysms treated with surgery is presented. Conclusions: aneurysms with a convenient configuration and location for either clipping or coiling might be better managed by surgical clipping in young patients considering that this treatment achieves higher rates of occlusion with a lower incidence of rebleeding. In elderly patients, each case must be discussed.

Open access

Marcus André Acioly, Marcílio Diogo de Oliveira Barbosa, Rosemary Tavares Pontes, Marcos Müller and Guilherme Brasileiro de Aguiar

Abstract

Background and object

Foix-Chavany-Marie syndrome (FCMS) is a rare type of pseudobulbar palsy, which is characterized by anarthria or severe dysarthria and bilateral central facio-linguo-velo-pharyngo-mastigatory paralysis with “automatic voluntary dissociation”. We report on a patient who suffered a reversible FCMS following a spear gun trauma through the cranial base leading to right operculo-insular contusion.

Case Report

This 28-year-old lady attempted suicide by shooting a spear gun into the head through her right submandibular region. Major vessel injury was ruled out and the patient was taken to the operating room for shaft removal. Postoperatively, we observed the mouth half open, drooling saliva, inability to move her tongue, anarthria, bilateral facial weakness, and loss of the gag reflex. Yawning was otherwise preserved resulting in a clinical diagnosis of FCMS. Postoperative imaging demonstrated a right operculoinsular contusion. Symptoms were fully recovered after two years of follow-up.

Conclusion

FCMS is a rare and severe form of pseudobulbar palsy. Unilateral lesions are exceptional but should be recognized, as we presented. Generally, the outcome is moderate to poor but the occurrence in brain trauma can be associated with complete functional recovery.

Open access

Pablo Ajler, Alvaro Campero, Federico Landriel, Ezequiel Goldschmidt, Santiago Hem and Antonio Carrizo

Abstract

Purpose

Acromegaly is an unusual disorder caused by abnormal oversecretion of growth hormone by pituitary adenomas. Transsphenoidal surgery is frequently the first management option. The objective of this article is to establish the effectiveness of a transnasal transsphenoidal approach in the treatment of GH-producing adenomas, and to identify risk factors for disease persistence.

Methods

We conducted a retrospective review of 81 patients treated for acromegaly with transsphenoidal microsurgery between 2006 and 2010.

Results

Macroadenomas accounted for 66.7% of the cases, contrast-enhanced MRI revealing cavernous sinus invasion in 28.4% of the patients (23 subjects). Cure was achieved in 72.8% (59 of 82). All microadenomas (27 cases) were managed effectively with surgery whereas cure rates stood at 66.7% for macroadenomas. Monovariate analysis showed that disease persistence was statistically associated with three variables. Odds ratio for remission stood at 1.68 for microadenomas and 0.033 for cavernous sinus invasion (p<0.001). Preoperative GH values were statistically associated with cure during follow up (p<0.05). Multivariate logistic regression analysis showed that only cavernous sinus invasion continued to be significantly associated with disease persistence (OR 3.52, p<0.05).

Conclusion

The transnasal approach proves effective in the treatment and cure of acromegaly. Cavernous sinus invasion is a major predictor of disease persistence.