Hemojuvelin-mediated hepcidin induction requires both bone morphogenetic protein type I receptors ALK2 and ALK3.

ABSTRACT: Hemojuvelin (HJV) is a glycosylphosphatidylinositol-anchored protein of the repulsive guidance molecule family acting as a bone morphogenetic protein (BMP) coreceptor to induce the hepatic iron regulatory protein hepcidin. Hepcidin causes ubiquitination and degradation of the sole known iron exporter ferroportin, thereby limiting iron availability. The detailed signaling mechanism of HJV in vivo has yet to be investigated. In the current manuscript, we used an established model of adeno-associated virus (AAV)-mediated liver-specific overexpression of HJV in murine models of hepatocyte-specific deficiency of the BMP type I receptors Alk2 or Alk3. In control mice, HJV overexpression increased hepatic Hamp messenger RNA (mRNA) levels, soluble HJV (sHJV), splenic iron content (SIC), as well as phosphorylated small mothers against decapentaplegic protein (pSMAD1/5/8) levels. In contrast, in Alk2fl/fl;Alb-Cre and Alk3fl/fl;Alb-Cre mice, which present with moderate and severe iron overload, respectively, the administration of AAV-HJV induced HJV and sHJV. However, it did not rescue the iron overload phenotypes of those mice. Serum iron levels were induced in Alk2fl/fl;Alb-Cre mice after HJV overexpression. In phosphate-buffered saline-injected Alk3fl/fl;Alb-Cre mice, serum iron levels and the expression of duodenal ferroportin remained high, whereas Hamp mRNA levels were decreased to 1% to 5% of the levels detected in controls. This was reduced even further by AAV-HJV overexpression. SIC remained low in mice with hepatocyte-specific Alk2 or Alk3 deficiency, reflecting disturbed iron homeostasis with high serum iron levels and transferrin saturation and an inability to induce hepcidin by HJV overexpression. The data indicate that ALK2 and ALK3 are both required in vivo for the HJV-mediated induction of hepcidin.

Perioperative Tracking of Intravenous Iron in Patients Undergoing On-Pump Cardiac Surgery: A Prospective, Single-Center Pilot Trial.

BACKGROUND: Preoperative intravenous iron administration is a frequently used patient blood management procedure. If the timeframe of intravenous iron administration before surgery is short, (1) the concentration of the intravenous iron compound might still be high in patients' plasma when undergoing surgery and (2) this iron in patients' plasma is at risk to be lost due to blood loss. The aim of the current study was, therefore, to track the iron compound ferric carboxymaltose (FCM) before, during, and after cardiac surgery requiring cardiopulmonary bypass, with an emphasis on intraoperative iron losses in shed blood and potential recovery through autologous cell salvage. METHODS: Concentrations of FCM were analyzed in patients' blood using a hyphenation of liquid chromatography and inductively coupled plasma-mass spectrometry to distinguish between pharmaceutical compound FCM and serum iron. In this prospective, single-center pilot trial, 13 anemic and 10 control patients were included. Anemic patients with hemoglobin levels ≤12/13 g/dL in women and men were treated with 500 milligrams (mg) intravenous FCM 12 to 96 hours before elective on-pump cardiac surgery. Patients' blood samples were collected before surgery and at days 0, 1, 3, and 7 after surgery. One sample each was taken of the cardiopulmonary bypass, the autologous red blood cell concentrate generated by cell salvage, and the cell salvage disposal bag. RESULTS: Patients who had received FCM <48 hours before surgery had higher FCM serum levels (median [Q1-Q3], 52.9 [13.0-91.6]) compared to ≥48 hours (2.1 [0.7-5.1] µg/mL, P = .008). Of 500-mg FCM administered <48 hours, 327.37 (257.96-402.48) mg were incorporated compared to administration ≥48 hours with 493.60 (487.78-496.70) mg. After surgery, patients' plasma FCM concentration in the FCM <48 hours group was decreased (-27.1 [-30 to -5.9] µg/mL). Little FCM was found in the cell salvage disposal bag (<48 hours, 4.2 [3.0-25.8] µg/mL, equivalent to 29.0 [19.0-40.7] mg total; equivalent to 5.8% or 1/17th of the 500 mg FCM initially administered), almost none in the autologous red blood cell concentrate (<48 hours, 0.1 [0.0-0.43] µg/mL). CONCLUSIONS: The data generate the hypotheses that nearly all FCM is incorporated into iron stores with administration ≥48 hours before surgery. When FCM is given <48 hours of surgery, the majority is incorporated into iron stores by the time of surgery, although a small amount may be lost during surgical bleeding with limited recovery by cell salvage.

Central Nervous System Fungal Infections in Children With Leukemia and Undergoing Hematopoietic Stem Cell Transplantation: A Retrospective Multicenter Study.

BACKGROUND: Central nervous system fungal infections (CNSFI) are seen in patients with hematologic malignancies and have high morbidity and mortality. Because of their rarity, there is limited data on CNSFI in children with no established treatment protocols or guidelines. MATERIALS AND METHODS: In this multicenter retrospective study, 51 pediatric patients with leukemia, 6 of whom had undergone bone marrow transplantation, with proven or probable CNSFI were evaluated. Fungal infections were defined as proven or probable based on European Organisation for Research and Treatment of Cancer criteria. Proven CNSFI was diagnosed by appropriate central nervous system (CNS) imaging or tissue sample findings in combination with positive microbiological results of cerebrospinal fluid. A positive culture, microscopic evidence of hyphae, a positive result of the galactomannan assays are defined as positive microbiological evidence. Probable CNSFI was defined as appropriate CNS imaging findings together with proven or probable invasive fungal infections at another focus without CNS when there is no other explanatory condition. Data was collected by using the questionnaire form (Supplemental Digital Content 1, http://links.lww.com/JPHO/A541 ). RESULTS: Seventeen patients had proven, 34 patients had probable CNSFI. Headaches and seizures were the most common clinical findings. The median time between the onset of fever and diagnosis was 5 days. The most common fungal agent identified was Aspergillus . Sixteen patients received single-agent, 35 received combination antifungal therapy. Surgery was performed in 23 patients. Twenty-two patients (43%) died, 29 of the CNSFI episodes recovered with a 20% neurological sequelae. CONCLUSION: CNSFIs should be considered in the differential diagnosis in patients with leukemia and refractory/recurrent fever, headache, neurologicalocular symptoms, and a radiologic-serological evaluation should be performed immediately. Early diagnosis and prompt management, both medical and surgical, are essential for improving clinical outcomes.

Hepatitis-Associated Aplastic Anemia: Etiology, Clinical Characteristics and Outcome.

Hepatitis-associated aplastic anemia (HAA) is a form of acquired aplastic anemia (AA) in which bone marrow failure develops after an acute attack of hepatitis. Bone marrow failure leading to AA is generally severe in cases of HAA and fatal if left untreated. This retrospective multicenter study investigated clinical and laboratory characteristics, possible causes, treatment, and outcome of HAA in children. Twenty patients from 8 centers were included in the study. Aspartate aminotransferase and alanine aminotransferase were <3 to 5×upper limit of normal (ULN) in 2 patients, <5 to 10×ULN in 2 patients, and >10×ULN in 16 patients. Acute liver failure developed in 5 (29%) patients. Pancytopenia was simultaneously present in 6 of 20 (30%) patients. Eleven of the 20 patients (55%) were alive, in remission and transfusion free. Those who were alive either had undergone hematopoietic stem cell transplantation and/or immunosuppressive treatment, except 1 patient who had received no treatment. Patients with the diagnosis of acute hepatitis should be evaluated and followed up carefully for presence of cytopenia, so that definitive treatment of AA can be initiated in a timely and appropriate manner when needed.

Is Monitoring of Cytomegalovirus Disease Required in Nontransplant Pediatric Acute Lymphoblastic Leukemia?

INTRODUCTION: Cytomegalovirus (CMV) infections in developing countries are experienced at an early age. This study was performed to investigate the frequency of reactivation and risk factors of infection acquired at an early age of nontransplant acute lymphoblastic leukemia (ALL) patients receiving immunosuppressive therapy with weekly monitoring of CMV levels in Turkey. MATERIALS AND METHODS: This was a retrospective, single-center study of 172 pediatric patients (102 boys and 70 girls) with ALL. All patients were monitored routinely for CMV-DNA at the initial presentation of leukemia and twice a week during chemotherapy. The CMV immunoglobulin (Ig)M/IgG titers were measured at admission. RESULTS: CMV seropositivity at baseline was 90,11%. The overall prevalence of CMV infection (viremia) was 70.34%, 116 of whom were seropositive for CMV IgG and 5 of whom were negative for CMV at the time of ALL diagnosis. Reactivation was more common than de novo CMV infections (P=0.000). CMV seropositivity at the beginning of the leukemia diagnosis was found to be an independent predictor for developing CMV infection (P=0.001). A total of 60 CMV infection episodes were treated with antivirals. Four of these included organ involvement. The duration of CMV-DNA viremia episodes was longer in patients with CMV-DNA ≥1000 copies/mL (n=45) than in those with lower CMV-DNA levels (P=0.002). Infection was shown not to be associated with chemotherapy phase. CONCLUSION: This study suggests the importance of monitoring for CMV infections in developing countries because of frequent reactivations in seropositive ALL patients. It should be kept in mind that low CMV-DNA levels may also lead to organ involvement.

A rare neurodegenerative disorder with a novel mutation in ROGDI and Rett- like phenotype: Kohlschütter- Tönz syndrome

@#Kohlschütter-Tönz syndrome (KTZS) is a rare neurodegenerative disorder that presents with seizures, developmental delay, psychomotor regression, hypoplastic dental enamel morphology characteristic for amelogenesis imperfecta, and dysmorphologies. Genetic analysis has identified loss of function mutations within the coding region of the ROGDI and SLC13A5 genes in KTZS. In this report, we documented the clinical, radiological, electroencephalographic, and genetic results of a 3.5-year-old Turkish girl, born to nonconsanguineous parents, who was the first patient diagnosed with KTZS in Turkey. The patient presented with Rett syndrome-like phenotype, neurodevelopmental delay, refractory seizures, and amelogenesis imperfecta. After obtaining informed consent, chromosomal DNAwas extracted from the peripheral blood of our patient and her parents. To investigate the moleculardiagnosis of the patient, the clinical exome sequencing was performed. The Sanger sequencing analysiswas performed for all of the family members for the validation and segregation of this mutation. PubMed/Medline, Web of Science, and Google Scholar were also searched to find all of the publisheddata on KTZS. The literature comprises 18 published studies about KTZS. The genetic analysis of ourpatient revealed a novel homozygous c.201-1G>T mutation in the ROGDI gene. The same mutationwas also found to be heterozygous in her mother and father. The mutation caused alternative splicingof the ROGDI translation and resulted in a disruption of the ROGDI protein.

Silencing Breast Cancer Genes Using Morpholino Embedded DNA-Tile-AuNPs Nanostructures.

There is an ongoing effort to increase the efficiency of gene delivery for the regulation of diseases-related genes. In this report, we demonstrate the efficiency of a DNA-based nanostructure to deliver morpholino antisense oligonucleotides for the upregulated genes in breast cancer as an alternative to the currently used delivery systems. A DNA-tile structure is constructed by embedding antisense oligonucleotides targeting the HER2 and ERα genes. Then, the sticky ends of the DNA-tile nanostructures are hybridized to gold nanoparticles (AuNPs) coated with the complementary oligonucleotides to enhance their cellular uptake. It is found that the morpholino antisense oligonucleotide embedded DNA-tile-AuNPs structure is 30% more effective than the liposome-based system to deliver morpholinos and induce gene silencing in breast cancer cells. The results of the study suggest that the prepared novel nanostructure is an effective and biocompatible carrier that can be used in in vitro gene silencing studies and can be further pursued in in vivo studies.

Síndrome de Stevens-Johnson asociado al tratamiento con metotrexato de la leucemia linfoblástica aguda: a propósito de un caso / Stevens-Johnson Syndrome associated with methotrexate treatment for acute lymphoblastic leukemia: a case report

La necrólisis epidérmica tóxica y el síndrome de StevensJohnson son enfermedades mucocutáneas raras que están asociadas a una evolución prolongada y a un desenlace potencialmente mortal. Principalmente están inducidas por fármacos y las tasas de mortalidad son muy elevadas. Aunque la piel es la más comprometida, también pueden estar afectados múltiples aparatos o sistemas como el cardiovascular, pulmonar, gastrointestinal y urinario. En este artículo, describimos el caso de un paciente con síndrome de Stevens-Johnson asociado al tratamiento con metotrexato, quien desarrolló insuficiencia cardíaca aguda y hemorragia gastrointestinal además de las manifestaciones en la piel. El paciente recibió un tratamiento satisfactorio con metilprednisolona e inmunoglobulina por vía intravenosa y continuó la quimioterapia con metotrexato.

Toxic epidermal necrolysis and Stevens-Johnson syndrome are rare mucocutaneous diseases which are associated with a prolonged course and potentially lethal outcome. They are mostly drug induced and mortality rates are very high. Although mostly skin is involved, multiple organ systems such as cardiovascular, pulmonary, gastrointestinal, and urinary systems may be affected. Here, we report a case of StevensJohnson Syndrome associated with methotrexate treatment who developed acute cardiac failure and gastrointestinal hemorrhage beside skin findings. He had been treated with intravenous immunglobulin and methylprednisolone succesfully and continued chemotherapy with methotrexate treatment again.

Stevens-Johnson Syndrome associated with methotrexate treatment for acute lymphoblastic leukemia: a case report. / Síndrome de Stevens-Johnson asociado al tratamiento con metotrexato de la leucemia linfoblástica aguda: a propósito de un caso.

Toxic epidermal necrolysis and Stevens-Johnson syndrome are rare mucocutaneous diseases which are associated with a prolonged course and potentially lethal outcome. They are mostly drug induced and mortality rates are very high. Although mostly skin is involved, multiple organ systems such as cardiovascular, pulmonary, gastrointestinal, and urinary systems may be affected. Here, we report a case of Stevens- Johnson Syndrome associated with methotrexate treatment who developed acute cardiac failure and gastrointestinal hemorrhage beside skin findings. He had been treated with intravenous immunglobulin and methylprednisolone succesfully and continued chemotherapy with methotrexate treatment again.

La necrólisis epidérmica tóxica y el síndrome de Stevens- Johnson son enfermedades mucocutáneas raras que están asociadas a una evolución prolongada y a un desenlace potencialmente mortal. Principalmente están inducidas por fármacos y las tasas de mortalidad son muy elevadas. Aunque la piel es la más comprometida, también pueden estar afectados múltiples aparatos o sistemas como el cardiovascular, pulmonar, gastrointestinal y urinario. En este artículo, describimos el caso de un paciente con síndrome de Stevens-Johnson asociado al tratamiento con metotrexato, quien desarrolló insuficiencia cardíaca aguda y hemorragia gastrointestinal además de las manifestaciones en la piel. El paciente recibió un tratamiento satisfactorio con metilprednisolona e inmunoglobulina por vía intravenosa y continuó la quimioterapia con metotrexato.

Examination of foramen tympanicum: an anatomical study using cone-beam computed tomography.

BACKGROUND: The foramen tympanicum (FT) is an osseous dehiscence of the temporal bone and usually closes by the age of 5 years. The foramen is located posteromedial to the temporomandibular joint and anteroinferior of the external auditory canal. The aim of this study is to define the prevalence, location and size of the foramen. MATERIALS AND METHODS: We retrospectively examined 200 cone-beam computed tomography (CBCT) images (400 ears). We used a CBCT dental imaging system (GALILEOS, Sirona Dental Systems, Bensheim, Germany) working at 15-30 mA and 98 kV. We noted size and location (unilateral and bilateral) of the present FT. RESULTS: Foramen tympanicum was determined in 11.5% of 200 patients (they had FT at least on one side of the head). This was bilateral in 5 (2.5%) patients. Prevalence of the FT was significintly higher in females (8%) than in males (3.5%). FT was found more frequently on the left side (7.5%) than on the right side (4%). Mean axial diameter was 1.13 mm (range 0.23-4.43 mm), and mean sagittal diameter was 1.44 mm (range 0.22-3.99 mm). CONCLUSIONS: Considering that FT was present in 11.75% of patients, radiologists and clinicians should be aware of the possible presence of this defect. It is known that this developmental dehiscence may cause herniation of temporomandibular joint, formation of salivary otorrhea, and spread of tumour or infection to the infratemporal fossa from external auditory canal. This study showed that CBCT may be preferred for imaging these conditions. (Folia Morphol 2018; 77, 2: 335-339).

‘Ponytail’ artefact on Waters radiography

@#Various factors may lead to artefacts in many radiological examinations that are commonly used in daily clinical practice.1,2 There are some simple but important rules to obtain high quality X-ray images and proper diagnosis with the imaging guidance. Artefacts may result in difficulty in interpretation of images, unnecessary workup and even improper diagnosis.3 Hair artefact that were seen in young girls and female patients, has been rarely reported to be a problem.4 It has been reported on conventional radiographs of the skull, neck and even chest radiographs. Hair artefacts may be caused by structural features or style of hair bounding. In most cases these radiopacities were caused by braids, curls and, obviously synthetic hair braid extensions. Ponytail style hair artefact on radiography has been reported very rarely. We report here ponytail hair style artefact seen on paranasal sinus X-ray images of a young girl. The parents has consented to this report

[Value of Doppler ultrasound for the assessment of carotid artery invasion by tumours of the upper aerodigestive tract]. / Intérêt de l'échographie Doppler dans l'évaluation de l'envahissement de l'artère carotidienne dans les tumeurs des voies aérodigestives supérieures.

The diagnosis of invasion of the carotid axis preoperatively is extremely helpful and determines the therapeutic approach as well as the prognosis for patients with tumours of the upper aerodigestive tract. A review of literature to refine the radiological choice between the different existing techniques is provided. It appears that echography gives excellent results.

Le diagnostic d'un envahissement de l'axe carotidien en période préopératoire est très utile et détermine l'attitude thérapeutique de même que le pronostic des patients présentant une tumeur des voies aérodigestives supérieures. A l'aide de cas cliniques, les différentes techniques d'imagerie ont été évaluées et une revue de littérature a été réalisée pour affiner le choix de la méthode radiologique. Il apparaît que l'échographie est particulièrement performante.

An unusual foreign body in the maxillary sinus: Dental impression material.

Foreign bodies in paranasal sinuses are very rare and most of them are encountered in the maxillary sinus. These foreign bodies may be organic or inorganic and can enter the maxillary sinus through an oro-antral fistula. The oro-antral fistula is formed by a break in the bony segment of the maxillary sinus floor and usually arises subsequent to maxillary premolar and molar extractions. A 63-year-old female patient evaluated for a nonhealing, left, toothless palate lesion and chronic headache occurring over 4 years. Radiography and computed tomography revealed bone discontinuity in the left floor of the maxillary sinus and calcifications within the antrum. A blue foreign body, later identified as dental impression material, was removed by intranasal endoscopy. A careful oral examination is recommended prior to prosthetic restorations. In addition, paranasal sinus foreign bodies should be surgically removed to prevent secondary soft tissue reactions.

Recalcitrant hypocalcemia after total thyroidectomy and bariatric surgery.

PROBLEM: Patients with Roux en Y gastric bypass (RYGBP) who undergo total thyroidectomy may be at increased risk for recalcitrant symptomatic hypocalcemia. METHODS: All patients who underwent total thyroidectomy with a history of preceding RYGBP from 2007 to 2012 were identified retrospectively. Cases were matched 2:1 for age, gender, and BMI to a control group undergoing total thyroidectomy without previous RYGBP during the same study period. RESULTS: Age and body mass index were equivalent between cases (n = 14) and controls (n = 23). A comparison between groups demonstrated that cases had a significantly higher incidence of symptomatic hypocalcemia resulting in paresthesia and tetany (38% vs. 0%; P <0 .01), received intravenous calcium more often (18% vs. 0%; P < 0.01), and had longer hospital stays (2.2 vs. 1.2 days, P = 0.02) than controls. CONCLUSIONS: Physicians and surgeons need to be aware of this complication and take measures to identify and prevent it, and patients should be informed. In this patient population, calcium levels should be closely monitored and early calcium and vitamin D spplementation should be initiated preemptively.

Prognostic value of FDG PET/CT in head and neck squamous cell carcinomas.

INTRODUCTION: The purpose of this study was to evaluate the use of (18)F-fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG PET/CT) to identify the presence of cervical lymph nodes metastases and extracapsular spread with histologic correlations in head and neck squamous cell carcinoma. METHODS: The medical records of 54 patients who underwent (18)F-FDG PET/CT for head and neck squamous cell carcinoma before surgery were reviewed. Receiver operating characteristic (ROC) analysis was performed to differentiate patients with cervical lymph node metastasis from those without lymph node metastasis. The same statistical analysis was done to differentiate cervical lymph nodes with extracapsular spread from those without extracapsular spread. RESULTS: Metastatic disease was diagnosed histologically in 49% (26 of 54) of the patients. Extracapsular spread was present in ten of the 54 patients (19%). When ROC curve analysis and maximum standardized uptake (SUVmax) values were used to detect cervical lymph node metastasis, the area under the ROC curve was 0.96 and the optimal cutoff value for SUVmax was 4.05 based on ROC curve analysis. The sensitivity and specificity of SUVmax for the detection of cervical lymph node metastasis using this cutoff point were 92% and 88%, respectively. When ROC curve analysis and SUVmax values were used in order to detect extracapsular spread, the area under the ROC curve was 0.86, and the optimal cutoff value for SUVmax was 4.15 based on ROC curve analysis. Using this cutoff value, the sensitivity and specificity of SUVmax for the detection of extracapsular spread were 83% and 88%, respectively. CONCLUSION: In our study, a median (18)F-FDG PET/CT SUVmax cutoff value of 4.15 was found to be related with cervical lymph node metastasis and extracapsular spread in patients with head and neck cancer.

Chemoradiation for advanced oral and (pharyngo) laryngeal carcinoma: single institution outcome analysis.

AIM: Chemoradiation ( CRT) is a valuable treatment option for(pharyngo)laryngeal squamous cell cancer and a palliative cure in advanced oral cancers. However, toxicity is scarcely reported. Therefore, efficacy, acute and toxic effects of chemoradiation for advanced head and neck squamous cell carcinomas were evaluated, using retrospective study. METHODS: Previously untreated patients with stage III-IV head and neck squamous cell carcinomas were included. Radiotherapy consisted of 70 Gy/7 weeks/35 fractions. All patients received concurrent Cisplatin 40 mg/m2 weekly. RESULTS: The most common acute toxic effects were dysphagia and mucositis. Dysphagia and xerostomia remained problematic during follow-up. Loco regional disease control was respectively 91% and 98% after 6 months. The median overall survival (calculating starting form the end of the treatment) was 33 months (range 0-111months). The 5-year disease specific survival was 41%. CONCLUSION: The results indicated that concurrent chemoradiotherapy for advanced head and neck squamous cell carcinoma is associated with high locoregional control and disease-specific survival. However, significant acute and long-term toxic effects occur, and organ preservation appears not necessarily equivalent to preservation of function in pharyngolaryngeal cancers.

The investigation of relationship between joint findings and serum angiogenic and inflammatory factor levels in severe hemophilia A patients.

Despite the use of primary prophylactic Factor VIII replacement in severe hemophilia A patients, bleeding into joints cannot be prevented completely and early diagnosis and treatment of the joint bleedings are important for prevention of permanent joint damage. Recent studies have shown that neoangiogenesis plays important role in development of synovitis after recurrent joint bleedings. This study aimed to investigate the relationship between joint findings and levels of serum angiogenic and inflammatory factors in severe hemophilia A patients.The patient groups consisted of 10 severe hemophilia A patients with acute joint bleeding and 25 severe hemophilia A patients without acute joint bleeding. They were all inhibitor negative. The control group consisted of 22 healthy male children. Complete blood cell count analysis, C-reactive protein (CRP), serum ferritin, lactic acid, and ELISA-based detection of vascular endothelial growth factor (VEGF), intercellular adhesion molecule-1, thrombomodulin, macrophage migration inhibitory factor (MIF), and endostatin were performed from peripheral blood of patient and the control groups. CRP and MIF levels were detected significantly higher in hemophilia patients with acute joint bleeding than patients without acute joint bleeding. There was a positive correlation between serum thrombomodulin, VEGF, and MIF levels. In this study, we demonstrated that serum CRP and MIF levels increases in acute bleeding period regardless of the presence of previous joint damage in children with severe hemophilia. CRP elevation may be a useful and rapid marker for acute bleeding in these patients.

Hemophagocytic lymphohistiocytosis associated with H1N1 virus infection and visceral leishmaniasis in a 4.5-month-old infant / Linfohistiocitose hemofagocítica associada pela infecção do vírus H1N1 e leishmaniose visceral em um bebê de 4,5 meses de vida

We present a case of a 4.5-month-old boy from Turkey with hemophagocytic lymphohistiocytosis (HLH) associated with H1N1 virus and Leishmania spp. coinfection. Because visceral leishmaniasis can mimic hematologic disorders like HLH, it is important to rule out this clinical condition before starting immunosuppressive therapy. In our case, treatment with liposomal amphotericin B resulted in a dramatic resolution of clinical and laboratory abnormalities.

É relatado um caso de um menino de 4,5 meses de idade, da Turquia, com linfohistiocitose hemofagocítica (HLH) associado à coinfecção com o vírus H1N1 e leishmaniose visceral. Como a leishmaniose visceral pode imitar doenças hematológicas como HLH, é importante afastar essa condição clínica antes de iniciar a terapia imunossupressora. No caso relatado, o tratamento com anfotericina B lipossomal resultou em uma resolução dramática das anomalias clínicas e laboratoriais.