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Michał Krekora, Mariusz Grzesiak, Maciej Słodki, Ewa Gulczyńska, Iwona Maroszyńska, Maria Respondek-Liberska, Frank A. Chervenak and Laurence B. McCullough

Abstract

INTRODUCTION: The aim of this study was to present our current practice of counseling patients and families with the most severe congenital malformations in the 3rd trimester of pregnancy and to develop practical guidelines for our team and involved healthcare/ socialcare professionals. MATERIAL & METHODS: It was a retrospective evaluation of a series of fetal cases in 2017 from single tertiary center. Maternal obstetrical medical history, time of prenatal detection of the anomaly (1st, 2nd or 3rd trimester), time between last fetal echocardiography and delivery, type of delivery, neonatal birth weight and time of neonatal demise. The total study group was subdived into early demise (during the 1st day after delivery) or late demise > 1st day after delivery. RESULTS: Mean maternal age was 30,4 +/- 5,6 years, and varied between 26 and 38 years. No chronic maternal diseases were found in medical history and no congenital malformations were present in previous children. All women had 1st trimester ultrasound, in 9 cases, it was reported as normal (with NT measurement < 2 mm), in 2 cases extracardiac abnormalities were detected: diaphragmatic hernia and omphalocele ( in both fetal karyotype 46,XY). In nine cases, the abnormalities were detected in midgestation and with maternal wish to continue the pregnancies. There were 8 neonatal deaths within 60 minutes after delivery, including one intrapartum death and 3 “late” neonatal deaths in the intensive care unit (on 12th, 21st and 22nd day). We stress upon the prenatal team approach and counseling of future parents, in order to prepare them for poor neonatal outcome. CONCLUSIONS: 1. In the most severe cases when fetal or neonatal demise was suspected, the two different opinions of specialists might not be enough and a third opinion should be recommended before final decision. 2. A Fetal Team of specialists is necessary in cases of expected fetal/neonatal demise in order to prepare a written report of recommended perinatal management for all sides involved in this difficult problem.

Open access

Anna Iacoi, Alexander Brobeil, Malena Götte, Christian Enzensberger, Vera Müller, Stefan Gattenlöhner and Roland Axt-Fliedner

Abstract

Pulmonary capillary haemangiomatosis (PCH) is a rare disorder of the lung, well described in adult literature. PCH is characterized by capillary proliferation, infiltrating the interstitium and alveolar walls. This leads to development of respiratory distress and to end-stage pulmonary hypertension. Mostly young adults are affected. The affection of newborn is described in less than ten cases in literature in the past forty years. PCH is a mostly deadly ending disease. We present a preterm born infant with antepartal diagnosed hydrops fetalis, who died 30 minutes after birth. Autopsy revealed PCH as lethal reason and not cardial disease as presumed before.

Open access

Ersin Kasim Ulusoy

Abstract

Purpose

Mean platelet volume (MPV) is an indicator of platelet (Plt) function and activation. The purpose of this study is to demonstrate whether MPV and MPV/Plt ratio, indicators of Plt activation, are increased in migraine patients, compared to tension-type headache (TTH) and healthy control groups, in our large-scale study, and whether these two primary headache types with unknown pathophysiology may be differentiated by using MPV and MPV/Plt ratio.

Materials and methods

Eighty patients diagnosed with migraine as per the 2004 diagnosis criteria of the International Headache Society (IHS), 80 patients with TTH who have applied to the Neurology Clinic of our hospital, and 80 healthy subjects who have applied to the Family Practice Clinic and Blood Bank were enrolled in this study. MPV and MPV/Plt ratio in both patients of similar age and gender who were diagnosed with migraine as well as in the TTH group and healthy control group were compared.

Findings

The MPV/Plt ratio was 0.046±0.008 in migraine patients, 0.037±0.009 in patients with TTH, and 0.036±0.009 in the healthy control group. This difference was statistically significant (p<0.05). The cutoff value of MPV/Plt ratio for differentiating migraine and TTH was 0.037. The sensitivity of this value was 92.5%, while specificity was 55%.

Conclusion

We have demonstrated that the most commonly confused primary headache types may be differentiated by using MPV and MPV/Plt ratio, and therefore, the disability caused by migraine and unsuitable, unnecessary, and long-term drug therapies may be avoided in patients.

Open access

Yelda Dere, Volkan Karakuş, Özcan Dere and Nazan Özsan

Abstract

Synchronous malignancies are rare conditions in oncology practices, generally seen as solid tumors with hematological neoplasms. However, occurrence of two different hematological malignancies in the same patient is extremely rare. Two primary malignancies should be considered especially in patients with extraordinary presentations and treatment resistance.

Open access

Amina H Hassab, Dalia A Nafea, Rania S Swelem and Basma M Ghazal

Abstract

Background

Acute myeloid leukemia (AML) is a heterogeneous hematologic malignancy characterized by a clonal expansion of myeloid blasts. Treatment strategies of patients with AML are based on various prognostic factors, including age and performance status of the patient, as well as cytogenetic and molecular characteristics of the leukemic clone.

Our aim was to study the expression of cluster of differentiation (CD)25 in adult Egyptian patients with newly diagnosed AML and to assess its prognostic relevance.

Methods

This study was conducted on 50 newly diagnosed AML patients at the Hematology Unit, Internal Medicine Department, Alexandria Main University Hospital. All patients were subjected to full history taking, thorough clinical examination, and laboratory investigations, including detection of CD25 expression on blast cells by flow cytometry. Conventional karyotyping was done on 11 patients at the time of diagnosis.

Results

In our study group, 12 patients were positive for CD25 expression, and this positivity was associated with worse overall survival and shorter leukemia-free survival. On evaluating the response to treatment among CD25-positive AML patients with normal karyotype, they had lower complete remission rates and higher relapse and death rates.

Conclusions

Expression of CD25 in AML patients at presentation can be considered a poor independent prognostic factor.

Open access

Henu Kumar Verma, Saikrishna Lakkakula and Bhaskar V.K.S. Lakkakula

Abstract

Sickle cell anemia (SCA) is one of the inherited hemoglobin disorders with substantial morbidity and early mortality. Hydroxyurea is the US Food and Drug Administration (FDA)-approved medication that has emerged as the primary disease-modifying therapy for SCA. Our purpose is to summarize the available evidence regarding the pharmacology, clinical efficacy, and safety of hydroxyurea therapy for the treatment of SCA. The electronic databases PubMed and Embase were searched from their starting dates to May 31, 2016. Databases were searched using the following terms: sickle cell, hydroxyurea, nitric oxide, dosing, therapeutic, and safety monitoring. Hydroxyurea therapy may cause severe myelosuppression when used in patients with SCA. SCA patients are initially treated with hydroxyurea at 10 or 20 mg/kg, and then the dose- is escalated to mild myelosuppression using a standardized regimen. Routine blood monitoring should be performed while the patient receives hydroxyurea treatment. Hydroxyurea can increase fetal hemoglobin (HbF) level and ameliorate some of the vascular symptoms in patients with SCA. Hydroxyurea therapy may help to avoid frequent hospitalizations, especially in patients with vaso-occlusive crisis. Taken together, available evidence suggests that hydroxyurea represents an inexpensive and effective treatment option that should be offered to patients with SCA.

Open access

Rie Tabata, Chiharu Tabata and Ryoji Yasumizu

Abstract

Here, we report a rare case of massive bone marrow necrosis, which – from the clinical findings and images – mimics disseminated bone metastasis. The patient was suffering from severe bone pain with elevated levels of serum alkaline phosphatase (ALP) and lactate dehydrogenase (LDH); moreover, strong incorporation of 18F-fluorodeoxyglucose in multiple bones was observed by positron emission tomography/computed tomography.

The underlying disease was Waldenström’s macroglobulinemia, which was thought to transform to cluster of differentiation 5 (CD5)-positive diffuse large B-cell lymphoma (DLBCL). The case showed a highly aggressive course, although the original Waldenström’s macroglobulinemia was in the stable state.

Clinicians should be aware of the co-occurrence of non-immunoglobulin-producing immature lymphoma, even with good course of Waldenström’s macroglobulinemia, and should pay attention to accompanying massive bone marrow necrosis, which mimics multiple cancer metastases to the bone. To the best of our knowledge, the present case is the first report of CD5-positive DLBCL transformed from CD5-negative Waldenström’s macroglobulinemia.

Open access

Aneta Neskoromna-Jędrzejczak, Katarzyna Bogusiak, Krzysztof Chojnowski, Marta Robak and Jacek Treliński

Abstract

The preparation of patients with hemophilia before surgical operations and dental procedures constitutes a significant clinical challenge. This article presents the implantoprosthetic rehabilitation of a patient with severe hemophilia B (factor IX activity <1%). The patient was prepared for the surgical procedure with recombinant factor IX concentrate (Rixubis) during the clinical surgery study. Tooth extraction and the implantation of four dental implants in the mandible were planned: one dental implant of 3.7 mm diameter and 10 mm length in the place of tooth 35, and another of 3.2 mm diameter and 10 mm length in the place of tooth 37. The next two implants were implemented 1 month later: one implant 3.7 mm in diameter and 10 mm in length in the place of tooth 46, and another implant 3.2 mm in diameter and 10 mm in length in the area of tooth 44. Appropriate substitution of the missing coagulation factor, together with the use of local hemostatic therapy, allowed dental implantation to be performed without excessive blood loss in this patient with severe hemophilia B.

Open access

Takeshi Sugimoto, Kazuhide Morimoto, Hiromi Hashimoto, Yukie Kaneda, Shinya Ohata and Yoshiro Yasutomo

Abstract

An 88-year-old female was admitted with autoimmune hemolytic anemia (AIHA). Coagulation test revealed severe prolongation of activated partial thromboplastin time (APTT). APTT cross-mixing test with patient plasma and normal plasma demonstrated an inhibitory pattern. Several intrinsic coagulation factor activities, particularly factor IX, showed remarkable decreases, and the inhibitor titers for coagulation factors VIII and IX were elevated. Although AIHA with existing antiphospholipid (aPL) antibodies was diagnosed initially, purpura developed on extremities intermittently during the clinical course. Considering the possibility of coexisting acquired hemophilia, APTT cross-mixing test with patient’s plasma and equal amount of the recombinant factor VIII product instead of normal plasma was performed. The APTT value on equal mixing samples with patient plasma and recombinant factor VIII product was decreased to within the normal range, and coagulation factor IX activity was restored. These results indicate that the recombinant factor VIII product had a neutralizing effect on aPL antibodies. We concluded that recombinant factor VIII product may lead to the repair of incorrect results from the APTT-dependent diagnostic system in the presence of aPL antibodies.

Open access

Monika Adamska, Anna Komosa, Tatiana Mularek, Joanna Rupa-Matysek and Lidia Gil

Abstract

Cardiac amyloidosis is a rare and often-misdiagnosed disorder. Among other forms of deposits affecting the heart, immunoglobulin-derived light-chain amyloidosis (AL amyloidosis) is the most serious form of the disease. Delay in diagnosis and treatment may have a major impact on the prognosis and outcomes of patients. This review focuses on the presentation of the disorder and current novel approaches to the diagnosis of cardiac involvement in AL amyloidosis.