Short Cilia, Immunodeficiency, and Cystic Fibrosis in a Mother-Daughter Pair.

Patients with chronic rhinosinusitis (CRS) that is refractory to maximal medical and surgical therapy should be evaluated for other primary conditions. Cystic fibrosis (CF), primary immunodeficiency (PID), and primary ciliary dyskinesia (PCD) are potential risk factors for refractory CRS. These conditions present with variable disease severity and diagnosis may be delayed into adulthood. We report a case of a mother-daughter pair with CRS refractory to maximal medical management. The patients were further evaluated and found to have features consistent with CF, PID, and PCD. All 3 are rare disorders and thought to cause CRS in isolation. Patients with refractory CRS should be further evaluated to identify alternative diagnoses and ensure proper management. Refractory CRS may be multifactorial, with different risk factors simultaneously contributing to its persistence.

Exploration of the Hungarian Version of Test Your Memory in General Practice: A Cross-Sectional Correlational Study of a Convenience Sample of Middle-Aged and Older Adults.

BACKGROUND: Dementia is a significant health issue worldwide. Mild cognitive impairment (MCI) can transform into dementia over time. General practitioners (GPs) may be the first to notice the cognitive deficit; therefore, it is crucial for them to have access to a screening test that can be administered quickly and efficiently. We explored the Hungarian version of the Test Your Memory self-administered dementia screening test in general practice for the early detection of dementia and cognitive impairment. METHODS: In the four Hungarian cities with medical universities, 368 patients over the age of 50 attending GPs filled out the questionnaire within the framework of our cross-sectional study. RESULTS: The total scores of the test showed a significant correlation with education and type of occupation and a significant negative correlation with age. The results of this research showed that the clock drawing and recall subtest scores deteriorate at the earliest age. CONCLUSION: The test can be filled out in a GP's office easily and two of its subtests can raise the possibility that patients may need further assessment, especially if they have symptoms, at an earlier age than the other subtests.

Impact of Hemoglobin Level in Ex Vivo Heart Perfusion on Donation After Circulatory Death Hearts: A Juvenile Porcine Experimental Model.

BACKGROUND: Ex vivo heart perfusion (EVHP) of donation after circulatory death (DCD) hearts has become an effective strategy in adults; however, the small circulating volume in pediatrics poses the challenge of a low-hemoglobin (Hb) perfusate. We aimed to determine the impact of perfusate Hb levels during EVHP on DCD hearts using a juvenile porcine model. METHODS: Sixteen DCD piglet hearts (11-14 kg) were reperfused for 4 h in unloaded mode followed by working mode. Metabolism, cardiac function, and cell damage were compared between the low-Hb (Hb, 5.0-5.9 g/dL; n = 8) and control (Hb, 7.5-8.4 g/dL; n = 8) groups. Between-group differences were evaluated using 2-sample t-tests or Fisher's Exact tests. RESULTS: During unloaded mode, the low-Hb group showed lower myocardial oxygen consumption (P < 0.001), a higher arterial lactate level (P = 0.001), and worse systolic ventricular function (P < 0.001). During working mode, the low-Hb group had a lower cardiac output (mean, 71% versus 106% of normal cardiac output, P = 0.010) and a higher arterial lactate level (P = 0.031). Adjusted cardiac troponin-I (P = 0.112) did not differ between the groups. Morphological myocyte injury in the left ventricle was more severe in the low-Hb group (P = 0.028). CONCLUSIONS: Low-Hb perfusate with inadequate oxygen delivery induced anaerobic metabolism, resulting in suboptimal DCD heart recovery and declined cardiac function. Arranging an optimal perfusate is crucial to organ protection, and further endeavors to refine the priming volume of EVHP or the transfusion strategy are required.

A Pediatric Primary Cardiac Spindle Cell Neoplasm With a Rare PDGFRA::USP8 Gene Fusion: A Case Report.

We report a case of a primary cardiac spindle cell neoplasm with concerning histological features and a rare PDGFRA::USP8 gene fusion in a 3 year old boy. The patient presented with a large cardiac mass predominantly in the right ventricle, originating from the ventricular septum. The mass was resected with grossly negative margins. Pathology revealed an unclassified spindle cell neoplasm with a PDGFRA::USP8 gene fusion. This gene fusion has only been previously reported twice in the medical literature, one in a pediatric cardiac sarcoma and the other in an abdominal soft tissue tumor in an adult woman. The patient is alive and well with no evidence of recurrence 11 months after excision.

A Canadian children's hospital's experience with cystic echinococcosis over 30 years: A case series.

Background: Cystic echinococcosis (CE) or hydatid disease caused by the cestode Echinococcus granulosus sensu lato is an uncommon infection in Canada especially among children. There are limited reports describing the clinical presentation and management in Canadian children. Methods: The medical records of all children diagnosed with CE at a quaternary paediatric centre in Ontario between January 1988 and August 2021 were retrospectively reviewed. The clinical course, management, and outcomes of each case were summarized. Results: We report two paediatric cases of cystic echinococcosis (CE) in detail and review four additional cases seen at our institution over 33.5 years. The first case was a previously healthy 12-year-old boy with pulmonary CE resulting in unilateral lung collapse and mediastinal shift, who was presumedly infected while living in the Middle East. The second case was a previously healthy 3-year-old girl with pulmonary CE acquired locally in southern Ontario. Four other cases of CE with hepatic involvement (median age 12.5 years) were identified during the study period. Five out of six patients received both surgical and medical therapy. Conclusion: CE is a rare but serious disease seen in southern Canada that has historically been associated with travel or migration. Due to changes in urban wildlife landscapes and increased global migration, CE may become more prevalent in Canadian children. We describe the first locally acquired case in rural southern Ontario diagnosed at our centre. Prompt recognition of this infection in children by health care providers is important to prevent morbidity and mortality.

Historique: L'échinococcose kystique (ÉK), ou hydatidose, causée par le cestode Echinococcus granulosus sensu lato, est une infection peu courante au Canada, particulièrement chez les enfants. Peu de rapports en décrivent la présentation clinique et la prise en charge chez les enfants canadiens. Méthodologie: Les auteurs ont procédé à l'analyse rétrospective des dossiers médicaux de tous les enfants ayant reçu un diagnostic d'ÉK dans un centre pédiatrique de soins quaternaires ontarien entre janvier 1988 et août 2021. Ils ont résumé l'évolution clinique, la prise en charge et le résultat clinique de chaque cas. Résultats: Les auteurs font un compte rendu détaillé de deux cas pédiatriques d'ÉK et analysent quatre autres cas observés à leur établissement sur une période de 33,5 ans. Le premier cas d'ÉK pulmonaire a touché un garçon de 12 ans auparavant en santé, probablement infecté alors qu'il habitait au Moyen-Orient, et a entraîné un collapsus pulmonaire unilatéral et une déviation médiastinale. Le deuxième cas d'ÉK pulmonaire a été observé chez une fillette de trois ans auparavant en santé qui a été infectée dans le sud de l'Ontario. Les auteurs ont relevé quatre autres cas d'ÉK comportant une atteinte hépatique (âge médian de 12,5 ans) pendant la période de l'étude. Cinq des six patients ont reçu à la fois un traitement chirurgical et médical. Conclusion: L'ÉK est une maladie rare, mais grave dans le sud du Canada. Elle était auparavant associée à un voyage ou une migration. En raison des changements aux paysages fauniques urbains et de la migration mondiale accrue, elle pourrait devenir plus prévalente chez les enfants canadiens. Les auteurs décrivent les premiers cas d'acquisition dans les régions rurales du sud de l'Ontario, diagnostiqués à leur centre. Il est important que les dispensateurs de soins dépistent cette infection rapidement chez les enfants pour éviter la morbidité et la mortalité.

L-citrulline attenuates lipopolysaccharide-induced inflammatory lung injury in neonatal rats.

BACKGROUND: Prenatal or postnatal lung inflammation and oxidative stress disrupt alveolo-vascular development leading to bronchopulmonary dysplasia (BPD) with and without pulmonary hypertension. L-citrulline (L-CIT), a nonessential amino acid, alleviates inflammatory and hyperoxic lung injury in preclinical models of BPD. L-CIT modulates signaling pathways mediating inflammation, oxidative stress, and mitochondrial biogenesis-processes operative in the development of BPD. We hypothesize that L-CIT will attenuate lipopolysaccharide (LPS)-induced inflammation and oxidative stress in our rat model of neonatal lung injury. METHODS: Newborn rats during the saccular stage of lung development were used to investigate the effect of L-CIT on LPS-induced lung histopathology and pathways involved in inflammatory, antioxidative processes, and mitochondrial biogenesis in lungs in vivo, and in primary culture of pulmonary artery smooth muscle cells, in vitro. RESULTS: L-CIT protected the newborn rat lung from LPS-induced: lung histopathology, ROS production, NFκB nuclear translocation, and upregulation of gene and protein expression of inflammatory cytokines (IL-1ß, IL-8, MCP-1α, and TNF-α). L-CIT maintained mitochondrial morphology, increased protein levels of PGC-1α, NRF1, and TFAM (transcription factors involved in mitochondrial biogenesis), and induced SIRT1, SIRT3, and superoxide dismutases protein expression. CONCLUSION: L-CIT may be efficacious in decreasing early lung inflammation and oxidative stress mitigating progression to BPD. IMPACT: The nonessential amino acid L-citrulline (L-CIT) mitigated lipopolysaccharide (LPS)-induced lung injury in the early stage of lung development in the newborn rat. This is the first study describing the effect of L-CIT on the signaling pathways operative in bronchopulmonary dysplasia (BPD) in a preclinical inflammatory model of newborn lung injury. If our findings translate to premature infants, L-CIT could decrease inflammation, oxidative stress and preserve mitochondrial health in the lung of premature infants at risk for BPD.

Effects of Health Anxiety, Social Support, and Coping on Dissociation with Mediating Role of Perceived Stress during the COVID-19 Pandemic.

BACKGROUND: Our study aimed to examine whether health anxiety, social support, and ways of coping relate to dissociation directly or only through the mediation of perceived stress, moderated by the time of measurement (lockdown). We investigated the effect of perceived stress on different forms (sub-scales) of dissociation. METHODS: A cross-sectional survey was conducted by an online form at two points in time: the beginning and the later stage of the COVID-19 pandemic. RESULTS: We received a total of 1711 responses. Perceived stress moderately correlated with dissociation in both international and Hungarian samples. Health anxiety showed a strong direct and indirect correlation with dissociation. Regarding social support, the support of family significantly decreased the dissociative experiences in the Hungarian sample mediated by perceived and direct stress. In the international sample, goal-oriented coping strategies strongly decreased all dissociation scales in the first measurement, through the mediation of perceived stress. As for the Hungarian sample, positive thinking was found to decrease dissociation by decreasing perceived stress. CONCLUSION: health anxiety, coping, and social support appeared to influence dissociation directly and through the mediation of perceived stress. Social support, mainly support of the family and problem-focused coping strategies may decrease the level of stress, this way decreasing dissociative behavior.

[The effect of surgical psychoeducation on the outcome of spinal surgery]. / A mutéti felkészítés hatásának vizsgálata gerincmutétet követoen.

Background and purpose – Interdiscipli­ nary researches demonstrate that patients’ fears and anxieties about surgery play a key role in the success of postoperative recovery. Psychoeducation is a professional information transfer method that aims to increase patients’ knowledge about their dis­ ease, and how to cope with it, and to emo­ tionally process the problems associated with the disease. If patients feel competent in their own healing process after surgery, they will experience less pain and become self­sufficient sooner, thereby the number of nursing days spent in the clinic reduces.
Methods – In this study the effect of psycho-education before spinal surgery on the use of postoperative analgetics was investigated. Results – The drug consumption of the study group who had been previously administered patient education is significantly reduced in comparison the control group.
Conclusion – Cooperation of a psychologist in surgical therapy promotes early recovery of patients in physical and mental well­being and reduces the costs of rehabilitation as well.

Cardioprotective Actions of a Glucagon-like Peptide-1 Receptor Agonist on Hearts Donated After Circulatory Death.

Background Heart transplantation with a donation after circulatory death (DCD) heart is complicated by substantial organ ischemia and ischemia-reperfusion injury. Exenatide, a glucagon-like peptide-1 receptor agonist, manifests protection against cardiac ischemia-reperfusion injury in other settings. Here we evaluate the effects of exenatide on DCD hearts in juvenile pigs. Methods and Results DCD hearts with 15-minutes of global warm ischemia after circulatory arrest were reperfused ex vivo and switched to working mode. Treatment with concentration 5-nmol exenatide was given during reperfusion. DCD hearts treated with exenatide showed higher myocardial oxygen consumption (exenatide [n=7] versus controls [n=7], over 60-120 minutes of reperfusion, P<0.001) and lower cardiac troponin-I release (27.94±11.17 versus 42.25±11.80 mmol/L, P=0.04) during reperfusion compared with controls. In working mode, exenatide-treated hearts showed better diastolic function (dp/dt min: -3644±620 versus -2193±610 mm Hg/s, P<0.001; Tau: 15.62±1.78 versus 24.59±7.35 milliseconds, P=0.02; lateral e' velocity: 11.27 ± 1.46 versus 7.19±2.96, P=0.01), as well as lower venous lactate levels (3.17±0.75 versus 5.17±1.44 mmol/L, P=0.01) compared with controls. Higher levels of activated endothelial nitric oxide synthase (phosphorylated to total endothelial nitric oxide synthase levels: 2.71±1.16 versus 1.37±0.35, P=0.02) with less histological evidence of endothelial damage (von Willebrand factor expression: 0.024±0.007 versus 0.331±0.302, pixel/µm, P=0.04) was also observed with exenatide treatment versus controls. Conclusions Acute treatment of DCD hearts with exenatide limits myocardial and endothelial injury and improves donor cardiac function.

The Effects and Differences of Social Support, Depression, and Vital Exhaustion during the COVID-19 Pandemic among International and Domestic University Students.

BACKGROUND: Our study aimed to assess the differences between domestic and international students in terms of social support, vital exhaustion, and depression during the period of COVID-19 and to examine the relationships and potential effects of these factors on each other. METHODS: The online cross-sectional survey was conducted via Google Forms® at three time intervals during the pandemic. RESULTS: Here, 1320, 246, and 139 students completed our questionnaires in the different time intervals. The international students reported significantly lower values in terms of perceived social support. Concerning depression, the international female students reported higher values than the domestic female students. Significant correlations were found in both samples between vital exhaustion and depression, as well as between perceived social support and depression. CONCLUSION: In this study, the international students reported lower levels of perceived social support and higher levels of depression, particularly among females. The correlations between depression, social support, and vital exhaustion might highlight protective and risk factors. These findings emphasize the importance of addressing social support and mental health among university students, especially among international students who have a difficult time finding social support during times of stress, such as during the COVID-19 pandemic.

[Connection between satisfaction with relationship-, life, well-being and the development of the family structure].

INTRODUCTION: The study is focusing on the relationships between several different, widely researched topics - such as satisfaction with relationship and life, well-being and the development of family structure - and examining them from different perspectives. In general, satisfaction with life, just as the subjective well-being displays high correlation with relationship satisfaction, which has effect on the development of the family structure as well. According to our assumption, those people who live in a happy and balanced relationship/marriage are happier and more satisfied with their lives. All of this is substantially influenced by one of the most important issues in people's lives/relationships, that is childbearing. This is also considerable for the development of life satisfaction and well-being. Moreover, a more balanced relationship is supposed to have a positive relationship with family cohesion, flexibility and communication within the family. METHOD: In the present study, we examined on our entire database the relationship between life satisfaction, well-being, relationship satisfaction and the possible changes in all of these relationships depending on the changes in the family structure. In our analysis, we also examined the effect of children's presence in the family in relation with relationship satisfaction. 192 people participated in the present study. RESULTS: According to the results, life satisfaction and the optimal communication within the family have a positive effect on relationship satisfaction. Satisfaction with family communication proved to be the strongest predictor. Despite our hypothesis, the number and age of children - more children and younger age - did not show a negative correlation with relatinship satisfaction. CONCLUSION: Not just during a crisis, under optimal circumstances, several factors affect relationship satisfaction, but the main influencing aspect is an optimal family structure. In a well-functioning family, the optimal level of communication has a positive effect on the development of the relationship, whereas disintegration and inadequate communication can lead to severe dysfunction both in terms of the relationship and the family unit. The results from the analysis show important conclusions and reveal new research directions, which should be examined in a larger sample in the future.

Fatal Pediatric Streptococcal Infection: A Clinico-Pathological Study.

PURPOSE AND CONTEXT: Streptococcal Infection (SI) is an important cause of pediatric death in children, yet limited reports exist on autopsy findings in fatal SI cases. METHOD: Case records (1997-2019) of SI with no pre-existing risk factors were reviewed and selected. Their clinical and pathological findings in the autopsy reports were analyzed. RESULTS: In our cohort of 38 cases based on bacterial culture results, SI was most commonly caused by Streptococcus pneumoniae (SPn; 45%) and Streptococcus pyogenes (SPy; 37%). 92% of decedents had some prodromal symptoms prior to terminal presentation. The clinical course was often rapid, with 89% found unresponsive, suddenly collapsing, or dying within 24 hours of hospital admission. 64% of deaths were attributed to sepsis, more frequently diagnosed in the SPy group than in the SPn group (71% vs 48%). Pneumonia was found in both SPn and SPy groups, whereas meningitis was exclusively associated with SPn. CONCLUSION: Our study shows fatal SI is most commonly caused by either SPn or SPy, both of which are frequently associated with prodromal symptoms, rapid terminal clinical course, and evidence of sepsis. Postmortem diagnosis of sepsis is challenging and should be correlated with clinical features, bacterial culture results, and autopsy findings.

How I approach: The workup and management of patients with progressive transformation of the germinal center.

Progressive transformation of the germinal center (PTGC) is a common and underrecognized cause of pediatric lymphadenopathy. PTGC may be associated with numerous systemic medical conditions that require further workup and management, including malignancy, autoimmune conditions, lymphoproliferative conditions, immunodeficiency, and infection. Given the breadth and rarity of the associated conditions, workup should be tailored to the individual patient and occur in a tiered approach. Patients with PTGC require ongoing follow-up, given their long-term risk of malignancy and recurrent PTGC.

Health anxiety, perceived stress, and coping styles in the shadow of the COVID-19.

BACKGROUND: In the case of people who carry an increased number of anxiety traits and maladaptive coping strategies, psychosocial stressors may further increase the level of perceived stress they experience. In our research study, we aimed to examine the levels of perceived stress and health anxiety as well as coping styles among university students amid the COVID-19 pandemic. METHODS: A cross-sectional study was conducted using an online-based survey at the University of Debrecen during the official lockdown in Hungary when dormitories were closed, and teaching was conducted remotely. Our questionnaire solicited data using three assessment tools, namely, the Perceived Stress Scale (PSS), the Ways of Coping Questionnaire (WCQ), and the Short Health Anxiety Inventory (SHAI). RESULTS: A total of 1320 students have participated in our study and 31 non-eligible responses were excluded. Among the remaining 1289 participants, 948 (73.5%) and 341 (26.5%) were Hungarian and international students, respectively. Female students predominated the overall sample with 920 participants (71.4%). In general, there was a statistically significant positive relationship between perceived stress and health anxiety. Health anxiety and perceived stress levels were significantly higher among international students compared to domestic ones. Regarding coping, wishful thinking was associated with higher levels of stress and anxiety among international students, while being a goal-oriented person acted the opposite way. Among the domestic students, cognitive restructuring as a coping strategy was associated with lower levels of stress and anxiety. Concerning health anxiety, female students (domestic and international) had significantly higher levels of health anxiety compared to males. Moreover, female students had significantly higher levels of perceived stress compared to males in the international group, however, there was no significant difference in perceived stress between males and females in the domestic group. CONCLUSION: The elevated perceived stress levels during major life events can be further deepened by disengagement from home (being away/abroad from country or family) and by using inadequate coping strategies. By following and adhering to the international recommendations, adopting proper coping methods, and equipping oneself with the required coping and stress management skills, the associated high levels of perceived stress and anxiety could be mitigated.

[Examination of emotion regulation difficulties, coping, impulsivity, external-internal control and sensation seeking among occasional and regular marijuana users].

INTRODUCTION: Several studies have investigated the relationship between personality and psychoactive substance use. Researches of personality and marijuana use suggests that certain personality traits differentiate between occasional and regular marijuana consumers. Understanding the relationship between individual persona - lity traits and marijuana use is a key step in the development of prevention and treatment methods. In the current study, we present the development of emotion regulation difficulties, coping with stress, impulsivity, external-internal control and sensation seeking in occasional and regular marijuana users. METHODS: 322 people participated in our study, within this 51 occasional and 56 regular marijuana users and 215 non-marijuana users who formed the control group. The examined personality traits were measured with the Diffi - culties in Emotion Regulation Scale, the Coping Inventory for Stressful Situations, the Barratt Impulsiveness Scale, the Internal-External Locus of Control Scale and the Sensation Seeking Scale. RESULTS: Varying levels of marijuana use increased difficulty in emotion regulation as well as impulsivity. Addi tio nally, the use of emotion-oriented coping strategies were most common in occasional and regular marijuana use. Regular marijuana users were more likely to have external control than occasional users. Non-marijuana users were less sen - sation seekers that the two groups of marijuana users, however, we did not find significant difference between occasio nal and regular users.

Cronkhite­Canada syndrome / Cronkhite-Canada-szindróma.

Összefoglaló. A Cronkhite-Canada-szindróma egy extrém ritka, nem öröklodo, gyomor-bél rendszeri polyposissal, fehérjeveszto enteropathiával és ectodermalis elváltozásokkal járó megbetegedés. A világon eddig összesen körülbelül 500 esetet jegyeztek fel. Az etiológia pontosan nem tisztázott, hátterében elsosorban autoimmun folyamatot feltételeznek. A diagnózis a páciens kórtörténetén, a fizikális vizsgálaton, az endoszkópos képen és a szövettani leleten alapul. A jelen közleményben egy 71 éves férfi beteg esetét mutatjuk be. A klinikai kép és az elvégzett vizsgálatok alapján a tünetek hátterében Cronkhite-Canada-szindrómát igazoltunk, majd a szakirodalomban leggyakrabban alkalmazott kombinált protonpumpagátló, kortikoszteroid és meszalazin adását vezettük be, illetve táplálásterápiát alkalmaztunk. Tudomásunk szerint Cronkhite-Canada-szindrómás beteg esete Magyarországon elsoként kerül ismertetésre. Orv Hetil. 2021; 162(11): 432-438. Summary. Cronkhite-Canada syndrome is an extremely rare, noninherited disease, characterized by gastrointestinal polyposis, protein-losing enteropathy and ectodermal abnormalities. Approximately 500 cases have been reported worldwide. The aetiology is unknown, most probably autoimmune mechanisms may be involved. The diagnosis is based on patient history, physical examination, endoscopic findings and histology. Here we report the case of a 71-year-old male, diagnosed with Cronkhite-Canada syndrome. The treatment consisted of proton-pump inhibitor, corticosteroids, mesalazin and nutritional therapy. To the best of our knowledge, this is the first report of Cronkhite-Canada syndrome in Hungary. Orv Hetil. 2021; 162(11): 432-438.

Round Cell Sarcomas: Newcomers and Diagnostic Approaches.

Undifferentiated sarcomas of soft tissue and bone have been defined as tumors with no identifiable morphologic, immunohistochemical, or molecular features indicating tumor cell origin. In young patients, these tumors frequently have a round or spindle cell morphology. Recently described recurrent translocations within this category have led to the recognition of new molecular subtypes of round cell sarcomas, and several of them have a more aggressive clinical course and less chemosensitivity. Because these "newcomers" are diagnosed based on their molecular characteristics, molecular investigation is key in the diagnosis and optimal treatment of these challenging tumors.

Atypical malignancies after intestinal transplantation in children: A European single-centre experience.

BACKGROUND: Post-transplant malignancies, that is, lymphomas, are a recognized complication in intestinal transplant recipients but are mostly secondary to EBV infection. There is an increased risk for malignancies in unusual sites in intestinal transplant recipients as compared to other solid organ transplants and the general population. OBJECTIVE: To evaluate the incidence, course, and outcome of unusual malignancies in children after ITx. METHODS: Retrospective analysis of children who underwent ITx for primary digestive disorders at Birmingham Children's Hospital between January 1989 and December 2017. RESULTS: Ninety-eight intestinal transplants were performed in 90 children (49 males and 41 females) with an underlying primary digestive disorder. Median age was 2.7 years (0.6-16.2), and median weight was 14.5 kg (5.7-53.2) at the time of transplant. Within this cohort, we identified four cases of unusual malignancies at rare sites of presentation. One patient developed cerebral PTLD, two patients were diagnosed with SMT, located at the stomal orifice and in cervicothoracic paravertebral area, respectively, and the last patient developed a retroperitoneal angiosarcoma. Unfortunately, the overall patient outcome was poor in all but one child with SMT, who currently survives with cytotoxic T-cell therapy. CONCLUSION: Unusual malignancies can occur in approximately 5% of children following ITx. A high index of suspicion is required for a timely diagnosis and adequate treatment.

Plaque-like Myofibroblastic Tumor: 2 Cases of This Unusual Dermal Tumor Which Occurs in Infancy and Early Childhood.

Plaque-like myofibroblastic tumor (PLMT) is a rare dermal spindle cell tumor which occurs in infancy or childhood within the first 4 years of life. The tumor is often pruritic and mostly presents on the lower back. We describe 2 cases with characteristic clinical and histological features of this entity, thus adding to the 10 cases which have so far been reported. Histologically, the lesion resembles a dermatofibroma. However, diffuse and uniform immunohistochemical staining with smooth muscle actin favors a myofibroblastic lineage. PLMT should be considered in the differential diagnosis of a dermal spindle cell tumor in the pediatric age-group.

Congenital cutaneous lymphadenoma.

Cutaneous lymphadenoma is an uncommon benign neoplasm often considered to be an adamantinoid variant of trichoblastoma. Lesions present in both sexes, between 14 and 87 years of age, and are mainly located on the head and neck. Cases in children are rare and there is only 1 previous case of a congenital lymphadenoma. An 8-year-old Asian girl presented with a congenital lesion on her forehead comprising 4 pink papules, the largest 5 mm in diameter. Microscopy revealed a well-circumscribed tumor within the dermis and subcutis comprising well-demarcated epithelial lobules of basaloid and clear cells with subtle peripheral palisading, growing in a collagenous stroma but lacking retraction artefact. A relatively dense accompanying predominantly lymphocytic inflammatory cell infiltrate including both T-cells (CD3+) and B-cells (CD20+) permeated the nodules and spilled into the stroma. CD68+ histiocytes and CD1a+ Langerhans cells were moderately numerous. This is the second case of congenital lymphadenoma which-in spite of its rarity in childhood-widens the diagnostic possibilities of cutaneous lymphoepithelial tumors in children.