Multiple congenital bilateral trigger digits in a 2-year-old child: case report

Trigger finger is a rare condition in children. In this paper, we report on a 2-year-old boy with multiple congenital bilateral trigger digits. The patient had no history of perinatal trauma, viral or bacterial infections, or metabolic disorders. The patient was treated with physiotherapy for one year. At the one-year follow-up, the boy presented with six trigger fingers (3 on the right hand, 3 on the left hand). Neither thumb was involved. The six trigger fingers were treated surgically: first, the right-hand trigger fingers and, six months later, those of the left hand. After each operation, a 4-week brace in extension was applied to the operated hand. The symptoms were completely resolved after surgical treatment. Many authors have recommended surgical release for the treatment of trigger finger in children; empirical treatment with physiotherapy may be an option when symptoms present or appear at an older age

Role of Notch2 pathway in mature B cell malignancies

In recent decades, the Notch pathway has been characterized as a key regulatory signaling of cell-fate decisions evolutionarily conserved in many organisms and different tissues during lifespan. At the same time, many studies suggest a link between alterations of this signaling and tumor genesis or progression. In lymphopoiesis, the Notch pathway plays a fundamental role in the correct differentiation of T and B cells, but its deregulated activity leads to leukemic onset and evolution. Notch and its ligands Delta/Jagged exhibit a pivotal role in the crosstalk between leukemic cells and their environment. This review is focused in particular on Notch2 receptor activity. Members of Notch2 pathway have been reported to be mutated in Chronic Lymphocytic Leukemia (CLL), Splenic Marginal Zone Lymphoma (SMZL) and Nodal Marginal Zone Lymphoma (NMZL). CLL is a B cell malignancy in which leukemic clones establish supportive crosstalk with non-malignant cells of the tumor microenvironment to grow, survive, and resist even the new generation of drugs. SMZL and NMZL are indolent B cell neoplasms distinguished by a distinct pattern of dissemination. In SMZL leukemic cells affect mainly the spleen, bone marrow, and peripheral blood, while NMZL has a leading nodal distribution. Since Notch2 is involved in the commitment of leukemic cells to the marginal zone as a major regulator of B cell physiological differentiation, it is predominantly affected by the molecular lesions found in both SMZL and NMZL. In light of these findings, a better understanding of the Notch receptor family pathogenic role, in particular Notch2, is desirable because it is still incomplete, not only in the physiological development of B lymphocytes but also in leukemia progression and resistance. Several therapeutic strategies capable of interfering with Notch signaling, such as monoclonal antibodies, enzyme or complex inhibitors, are being analyzed. To avoid the unwanted multiple “on target” toxicity encountered during the systemic inhibition of Notch signaling, the study of an appropriate pharmaceutical formulation is a pressing need. This is why, to date, there are still no Notch-targeted therapies approved. An accurate analysis of the Notch pathway could be useful to drive the discovery of new therapeutic targets and the development of more effective therapies

Post-traumatic malunion of the proximal phalanx of the finger. Medium-term results in 24 cases treated by "in situ" osteotomy

We report the clinical and radiographic medium-term results obtained for 20 patients (24 fingers) treated surgically for post-traumatic malunion of the proximal phalanx of the finger. In all cases we performed a corrective osteoclasia or osteotomy at the site of malunion, followed by miniplate and screw fixation or by screw fixation only. The corrective osteoclasia was performed when malalignment was addressed within six weeks after injury. Two patients who had two fractures underwent additional surgery (tenolysis and/or capsulolysis) to improve function and ROM. At the final follow-up, at a mean of 24 months after corrective surgery, good or excellent clinical and radiographic results were obtained in all the patients. The pseudoclaw deformity disappeared in all cases in which a volar angulation deformity was present. An average improvement of about 30% in the range of motion of the MP and PIP joints was observed; only 4 patients complained of mild pain at the maximum degrees of articular excursion of the MP and PIP joints. All the patients presented an improvement in grip strength. The mean DASH score in our series was 5 points. In two of the four cases treated by an intra-articular corrective osteotomy, mild radiographic signs of osteoarthritis at the MP joint were present. The data for this study confirm that "in situ" osteotomy stabilized by miniplates and/or screws is an effective procedure to correct post-traumatic malunions of the proximal phalanges of the fingers

Immunomodulatory effect of ibrutinib: Reducing the barrier against fungal infections

The Bruton tyrosine kinase (BTK) inhibitor ibrutinib is increasingly used in the treatment of chronic lymphocytic leukemia (CLL). Moreover, very promising results have been reported in other B-cell malignancies, including primary central nervous system lymphoma (PCNSL). Although well-tolerated in the majority of patients, ibrutinib demonstrates in some cases troublesome toxicities, including invasive fungal infections (IFIs). In the present review, we summarize clinical manifestations of IFIs in patients treated with ibrutinib, generally characterized by an early onset, mild clinical manifestations, asymptomatic/low symptomatic pulmonary localization and high incidence of central nervous system (CNS) involvement. IFI risk appears particularly increased in patients receiving ibrutinib associated with other immune modulator agents, especially with steroids or immune-chemotherapy. Moreover, the immunomodulatory effect of ibrutinib is described, pointing the attention on the involvement of specific molecules targeted by ibrutinib in innate and adaptive response to fungal infection. Overall, the findings indicate the ibrutinib may rapidly impair innate immune cell functions, while concomitantly restoring an effective protective potential of adaptive immune compartment. A correct awareness, especially when other predisposing factors are present, is warranted about the potential risk of IFIs in ibrutinib-treated patients

Fracture energy of sustainable geopolymer composites with and without the addition of slaughterhouse by-products as fibre-reinforcement: An experimental investigation

This work focuses on the development and on the mechanical characterization of geopolymer composites to be used as sustainable plasters or mortars. The starting point of the work is the synthesis of a geopolymer binder, which is subsequently used for the production of two different mortars: the first one is obtained by simply adding fine aggregates (control mortar), while in the second case, also slaughterhouse wastes (SHW) are included in the admixture. Nowadays, slaughtering industry produces a large amount of biological wastes, that are usually discarded via incineration or landfills. Among SHW, horns and hooves, which are rich in keratin, can be potentially used as additives in the manufacturing process of mortars, both in the form of fibre-reinforcement, as well as fine aggregates and fillers. In the second mortar considered in this work, 2% of SHW fibres and 2% of SHW filler are added in the admixture. The mechanical properties of the three products are experimentally investigated and compared to each other. Apart for compressive strength, close attention is paid to the flexural behaviour and to the determination of fracture energy. To evaluate the effect of SHW addition on crack propagation and width, digital image correlation technique is also adopted

Obesity as a social phenomenon: A narrative review

BACKGROUND: obesity is one of the most prevalent diseases all over the world. Because of its high social impact, the broadest possible approach on several levels - and not limited only to clinical aspect - is needed to better understand and face the challenges obesity poses to public health. OBJECTIVES: to analyse, through the main evidence, the so- cial impact of weight excess in the general population and the actions aimed at mitigating its negative effects. DESIGN: narrative review. SETTING: data obtained from the sources included in the study were gathered and analyzed in five macroareas: Health Inequality, Society, Work, Impact on Social Medicine (focused on the Italian model), and Social Costs. RESULTS: each category showed a bilateral relationship with obesity having a significant impact for the community. CONCLUSIONS: for each field, various actions should be taken at institutional level. Many recommendations and actions have already been taken worldwide, but they alone seem to be not enough. This work points out that, in order to combat obesity and bring about a slowdown of this pandemic, the en- tire scientific community and institutions must work together to identify and design programmes that are truly effective

An 81-Year-Old Man with a 6-Year History of Chronic Lymphocytic Leukemia Presenting with Disease Flare Following Ibrutinib Discontinuation

Patient: Male, 81-year-old Final Diagnosis: Chronic lymphocytic leukemia Symptoms: Fever Medication: — Clinical Procedure: — Specialty: Hematology Objective: Background: Case Report: Conclusions: Unusual clinical course Chronic lymphocytic leukemia (CLL) is a mature B-cell neoplasm and the most common leukemia in adults in Western countries. Novel agents, including BTK inhibitors and the BCL2 inhibitor venetoclax, have dramati-cally changed the treatment landscape. Moreover, a disease flare, characterized by sudden worsening of clinical symptoms, radiographic findings of rapidly worsening splenomegaly or lymphadenopathy, and laboratory changes (increased absolute lymphocyte count or lactate dehydrogenase), is a phenomenon described in up to 25% of patients with CLL after ibrutinib discontinuation. We describe a patient with CLL with disease flare after ibrutinib discontinuation due to disease progression and describe the subsequent management of vene-toclax initial treatment in the course of the disease flare. We describe the case of an 81-year-old man with a 6-year history of CLL who was treated with multiple lines of therapy and developed worsening of disease-related signs and symptoms with fever, marked increase of lym-phocyte count, acute worsening of renal function, and increase in lymph nodes and spleen size following ces-sation of targeted therapy with ibrutinib at the time of disease progression. There was subsequent overlap-ping of ibrutinib during the venetoclax dose escalation period to prevent disease flare recurrence. Our report highlights the problem of disease flare after ibrutinib discontinuation in order to avoid associated patient morbidity, underscoring the importance of awareness of this phenomenon and focusing on the addition of venetoclax at time of progression in ibrutinib-treated patients, as a temporary overlap strategy, to prevent disease flare