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Cystic echinococcosis (CE), the second most significant foodborne parasitic disease worldwide, poses a significant global health burden. Understanding its clinical and laboratory features is crucial for effective management. This study aimed to investigate the epidemiological, laboratory, and clinical characteristics of pediatric CE in an Iranian referral hospital. A cross-sectional study reviewed hospital records of patients with CE admitted to Children's Medical Center, Tehran, Iran, from 2011 to 2020. Data on demographics, diagnostics, clinical presentation, laboratory findings, and treatment were collected and analyzed. A total of 114 patients, with a mean age of 7.33 ± 2.9 years, were diagnosed with CE. The male-to-female ratio was 1.78, and 73.7% were urban residents. Abdominal pain (69%) and coughing (65%) were the most common symptoms. In confirming the cyst involvement across anatomical sites, pathology emerged as the most reliable method, with effectiveness ranging from 95% to 100%. Abdominal ultrasonography and computed tomography scan were frequently utilized imaging modalities, displaying effectiveness percentages of 71-85%. Liver and lung involvement predominated (66%), with 39% of cases showing multiorgan involvement. Spleen involvement was less common (6%), and neurological involvement was rare (1-2%). The majority of patients (n = 63, 67.7%) displayed cysts larger than 50 mm. All patients received albendazole treatment, and 104 patients (91.2%) underwent surgical procedures, with three postsurgical deaths. In conclusion, hospital records over 9 years indicate an increasing prevalence of CE, emphasizing the need for heightened awareness and effective public health interventions to control this parasitic infection.

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Background: IgG4-related disease with multiorgan involvement predicts higher disease activity, thus, it is necessary to identify whether IgG4-related disease involves multiple organs at the early stage. To further clarify the clinical characteristics and risk factors for IgG4-related disease with multiorgan involvement, we conducted an observational study. Methods: We retrospectively analysed the clinical data of 160 patients who were primarily diagnosed with IgG4-related disease at the First Affiliated Hospital of Zhengzhou University from January 2015 to January 2021. According to the number of involved organs, patients were divided into two groups: multiorgan involvement and nonmultiorgan involvement. Patients were divided into a multiorgan group and a nonmultiorgan group according to multiple organ involvement. Results: There were 82 cases identified with multiorgan involvement and 78 cases diagnosed with no multiorgan involvement in this series. Most cases were elderly and male (p > 0.05). The most frequently affected organs in IgG4-RD were the lymph nodes (50.6 %), pancreas (38.7 %) and salivary glands (35.6 %). Multivariate analysis showed that eosinophilia, IgG4>2*ULN, lymph node involvement, salivary gland involvement and lung involvement were independent risk factors for multiorgan involvement (p < 0.05). Conclusions: The main issues in clinical practice are how to accurately diagnose the disease and screen the more vulnerable organs.

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Resumen Introducción: La encefalopatía hipóxico-isquémica (EHI) moderada-grave secundaria a asfixia perinatal puede afectar a cualquier órgano, empeorando el pronóstico. Objetivo: Evaluar la afectación renal y multiorgánica de estos pacientes. Material y método: Se incluyó a recién nacidos > 35 semanas con EHI moderada-grave tratados con hipotermia activa entre 2010 y 2020. Se evaluó la creatinina en tres periodos: 48-72 horas de vida, entre el 3.o y 7.o día de vida y del 7.o al 28.o día de vida. Resultados: Se incluyeron 135 pacientes: 112 con EHI moderada y 23 con EHI grave. Al comparar ambos grupos, se obtuvieron diferencias significativas a las 48-72 horas y entre 3.o-7.o día de vida. No hubo diferencias al comparar el método de hipotermia. Los pacientes con EHI grave presentaron mayor afectación hemodinámica, respiratoria y hepática. Conclusiones: Neonatos con EHI grave presentan aumento de los niveles de creatinina sérica y mayor afectación multiorgánica respecto a aquellos con EHI moderada.

Abstract Background: Hypoxic-ischemic encephalopathy (HIE) secondary to perinatal asphyxia can affect any organ, worsening the prognosis. Objective: To describe renal and multiorgan involvement in moderate-severe HIE. Material and method: Newborns > 35 weeks diagnosed with moderate-severe HIE who required active hypothermia between 2010-2020 were included. To assess renal involvement, serum creatinine was measured in three different periods: at 48-72 hours, between the 3rd and the 7th day, and from the 7th to the 28th day. Results: A total of 135 patients were included, 112 (83%) with moderate and 23 (17%) with severe HIE. Significant differences were obtained when comparing median creatinine levels at 48-72 hours and between 3-7 days in both groups. There were no differences in creatinine according to the hypothermia method. Patients with severe HIE presented greater hemodynamic, respiratory, and hepatic involvement. Conclusions: Neonates with severe HIE present increased serum creatinine levels and greater multi-organ involvement than those with moderate HIE.

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Multilobar lung echinococcosis with multiorgan involvement is an extremely rare entity in pediatric populations. We would like to share an adolescent girl with very demonstrative postero-anterior chest X-ray and computed tomography images.

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Although the lungs are the primary organ involved, increasing evidence supports the neuroinvasive potential of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This study investigates the potential relationship between coronavirus disease (COVID-19)-related deterioration of brain structure and the degree of damage to lung function. Nine COVID-19 patients were recruited in critical condition from Jin Yin-tan Hospital (Wuhan, China) who had been discharged between 4 February and 27 February 2020. The demographic, clinical, treatment, and laboratory data were extracted from the electronic medical records. All patients underwent chest CT imaging, 129Xe gas lung MRI, and 1H brain MRI. Four of the patients were followed up for 8 months. After nearly 12 months of recovery, we found no significant difference in lung ventilation defect percentage (VDP) between the COVID-19 group and the healthy group (3.8 ± 2.1% versus 3.7 ± 2.2%) using 129Xe MRI, and several lung-function-related parameters-such as gas-blood exchange time (T)-showed improvement (42.2 ms versus 32.5 ms). Combined with 1H brain MRI, we found that the change in gray matter volume (GMV) was strongly related to the degree of pulmonary function recovery-the greater the increase in GMV, the higher degree of pulmonary function damage.

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Extranodal-multiorgan involvement is rarely presented in diffuse large B-cell non-Hodgkin lymphoma. 18Fluorine-fluorodeoxyglucose positron emission tomography/computed tomography findings of a 22-year-old female patient with supra/infra-diaphragmatic nodal and skeletal involvements and thyroid, pancreas, right breast, bilateral renal, and ovarian involvements were presented.

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BACKGROUND: Since the beginning of the SARS-CoV­2 pandemic, cases of the hyperinflammatory syndrome pediatric inflammatory multisystem syndrome (PIMS) have been accumulating. The clinical presentation is variable and it occurs 2-6 weeks after infection with SARS-CoV­2. As of today, immunoglobulins and/or steroids as well as ASS are used for medication. METHOD: In our clinic 11 patients presented with PIMS between 06/2020 and 06/2021, whose data were retrospectively collected and analyzed. RESULTS: Of the 11 patients 6 were male, the age distribution ranged from 4-18 years and 7 were overweight or obese. Almost all patients showed gastrointestinal and cardiovascular involvement, 4 had respiratory symptoms, 6 showed signs of nephritis. All showed blood count changes with anemia or leukocytosis and coagulopathy. CRP, ferritin, and soluble IL2 receptor were highly elevated in all patients. Only 2 patients had neither troponin­T nor NT-pro-BNP elevation and 7 patients had impaired left ventricular function. Positive SARS-CoV­2 serology was found in 10, and positive SARS-CoV­2 PCR via nasopharyngeal swabs in 2.All were initially treated with antibiotics, 3 patients required O2 supplementation, 6 required intensive care and 5 required vasoactive agents. All but one patient received immunoglobulins and ASS, 5 received prednisolone. Length of stay ranged from 4-51 days. CONCLUSION: PIMS is a severe acute hyperinflammatory disease, which was secured in 11 patients in our clinic. In some cases, there was a need for intensive care. Under anti-inflammatory therapy there was a good response without exception.

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Background & objectives: There are limited data from India on the post-COVID multisystem inflammatory syndrome in adults (MIS-A). The objective of the present study was to evaluate the clinical profile of patients with MIS-A admitted to a tertiary care centre in southern India. Methods: This single-centre retrospective study was conducted from November 2020 to July 2021, and included patients aged >18 yr admitted to the hospital as per the inclusion and exclusion criteria. Results: Nine patients (5 male, mean age 40±13 yr) met the criteria for MIS-A. Five patients had proven COVID-19 infection or contact history 36.8±11.8 days back. All patients were positive for SARS-CoV-2 IgG antibody, negative for COVID-19 PCR, and had negative blood, urine and sputum cultures. All patients had fever and gastrointestinal (GI) symptoms, and five patients had left ventricular dysfunction. All patients had neutrophilic leucocytosis at presentation and elevated biomarkers such as C-reactive protein serum procalcitonin, D-dimer and ferritin. The majority of the patients (7/9 i.e. 77.78%) were treated with intravenous hydrocortisone (50-100 mg q6h-q8h). Six patients recovered completely whereas three patients expired. Interpretation & conclusions: Fever and GI symptoms were the most common presentation of MIS-A. Elevated serum procalcitonin may not be useful in differentiating bacterial sepsis from MIS-A. Most patients responded to corticosteroids.

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Background: Hemolytic uremic syndrome (HUS) is a complex disease with multi-organ involvement. Eculizumab therapy is recommended for treatment of complement mediated hemolytic uremic syndrome (cHUS). However, there are few studies evaluating eculizumab therapy among children with HUS. The primary objectives of the study were to describe and identify factors associated with eculizumab therapy in children with HUS. Design/Methods: This large, retrospective, multi-center, cohort study used the Pediatric Health Information System (PHIS) database to identify the index HUS-related hospitalization among patients ≤18 years of age from September 23, 2011 (Food and Drug Administration approval date of eculizumab) through December 31, 2018. Multivariate analysis was used to identify independent factors associated with eculizumab therapy during or after the index hospitalization. Results: Among 1,885 children included in the study, eculizumab therapy was noted in 167 children with a median age of 3.99 years (SD ± 4.7 years). Eculizumab therapy was administered early (within the first 7 days of hospitalization) among 65% of children who received the drug. Mortality during the index hospitalization among children with eculizumab therapy was 4.2 vs. 3.0% without eculizumab therapy (p = 0.309). Clinical factors independently associated with eculizumab therapy were encephalopathy [odds ratio (OR) = 3.09; p ≤ 0.001], seizure disorder (OR = 2.37; p = 0.006), and cardiac involvement (OR = 6.36, p < 0.001). Conclusion(s): Only 8.9% of children received eculizumab therapy. Children who presented with neurological and cardiac involvement with severe disease were more likely to receive eculizumab therapy, and children who received therapy received it early during their index hospitalization. Further prospective studies are suggested to confirm these findings.

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BACKGROUND: Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS) is the main cause of pediatric acute kidney injury (AKI) in Argentina. Endothelial injury is the trigger event in the microangiopathic process. The host inflammatory response to toxin and E. coli lipopolysaccharide (LPS) is involved in disease pathophysiology. METHODS: This retrospective study describes pediatric STEC-HUS patients with multiorgan involvement at the initial phase of disease. A retrospective study of critically ill HUS patients with evidence of E. coli infection was conducted through a period of 15 years. RESULTS: Forty-four patients 35.4 ± 4.1 months were admitted to the intensive care unit for 21 ± 2 days. Mechanical ventilation was required in 41 patients, early inotropic support in 37, and 28 developed septic shock. Forty-one patients required kidney replacement therapy for 12 ± 1 days. Forty-one patients showed neurological dysfunction. Dilated cardiomyopathy was demonstrated in 3 patients, left ventricular systolic dysfunction in 4, and hypertension in 17. Four patients had pulmonary hemorrhage, and acute respiratory distress syndrome in 2. Colectomy for transmural colonic necrosis was performed in 3 patients. Thirty-seven patients were treated with therapeutic plasma exchange, and 28 patients received methylprednisolone (10 mg/kg for 3 days). Of the surviving 32 patients, neurological sequelae were seen in 11 and chronic kidney failure in 5. CONCLUSIONS: Severe clinical outcome at onset suggests an amplified inflammatory response after exposure to Shiga toxin and/or E. coli LPS. STEC-HUS associated with severe neurological involvement, hemodynamic instability, and AKI requires intensive care and focused therapy.

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AIM: To review multiorgan involvement and management in children with Down syndrome (DS). METHODS: A literature review of articles from 1980 to 2019 using the MEDLINE interface of PubMed was performed using the following search terms- [Down syndrome] or [Trisomy 21] AND [Cardiology] or [Respiratory] or [neurodevelopment] or [epilepsy] or [musculoskeletal] or [immune system] or [haematological] or [endocrine] or [gastrointestinal] or [ophthalmological] or [Ear Nose Throat] or [dermatology] or [renal]. RESULTS: Congenital heart disease particularly septal defects occur in over 60% of infants with DS and 5%-34% of infants develop persistent pulmonary hypertension of the newborn irrespective of a diagnosis of congenital heart disease. Early recognition and management of aspiration, obstructive sleep apnoea and recurrent lower respiratory tract infections (LRTI) could reduce risk of developing pulmonary hypertension in later childhood. Children with DS have an increased risk of autistic spectrum disorder, attention deficit disorder and epilepsy particularly infantile spasms, which are associated with poor neurodevelopmental outcomes. Congenital anomalies of the gastrointestinal and renal system as well as autoimmune diseases, coeliac disease, arthropathy, thyroid dysfunction fold diabetes mellitus and dermatological conditions are more common. Hearing and visual anomalies are also well recognised association with DS (Table 1). CONCLUSION: Children with DS are at an increased risk of multiorgan comorbidities. Organ-specific health surveillance may provide holistic care for the children and families with DS throughout childhood.

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Amyloid light chain (AL) amyloidosis is a protein conformational disease. AL amyloidosis results from aggregation of misfolded proteins that are deposited in tissues as amyloid fibrils. Diagnosis of AL amyloidosis can be challenging due to its low incidence and clinical complexity. Therapy requires a risk-adapted approach involving dose reductions and schedule modifications of chemotherapy regimens along with close monitoring of hematologic and organ responses. We herein describe a patient whose condition was diagnosed as systemic AL amyloidosis and presented with splenic rupture as the initial symptom. Congo red staining of the kidney biopsy was positive. The normal structure of the liver and spleen had been replaced by amyloid deposition. The chemotherapy strategy involved a combination of bortezomib, cyclophosphamide, thalidomide, and dexamethasone.

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OBJECTIVE: To assess the effectiveness of apheresis therapy (AT) in treating the clinical manifestations of patients with complicated cryoglobulinemic vasculitis (CV). METHODS: A retrospective cohort study of 159 CV patients attending 22 Italian Centers who underwent at least one AT session between 2005 and 2015. The response to AT was evaluated on the basis of a defined grading system. RESULTS: Peripheral neuropathy was the most frequent clinical condition leading to AT. Therapeutic plasma exchange was used in 70.4% of cases. The outcome of AT was rated very good in 19 cases, good in 64, partial/transient in 40, and absent/not assessable in 36. Life-threatening CV-related emergencies and renal impairment independently correlated with failure to respond to AT. The independent variables associated with an increased risk of death were age at the time of the first AT session, multi-organ life-threatening CV, the presence of renal impairment and failure to respond to AT. The time-dependent probability of surviving until CV-related death in the second year was 84%, with an AHR in patients with absent/not assessable response to AT of 11.25. CONCLUSION: In this study AT is confirmed to be a safe procedure in patients with CV. Early AT should be considered in patients with severe CV, especially in cases with impending renal involvement, in order to prevent irreversible kidney damage. Although its efficacy in patients with multi-organ failure is limited, AT is the only treatment that can rapidly remove circulating cryoglobulins, and should be considered an emergency treatment.

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AIM: Identifying early clinical and biological factors associated with severe forms of postdiarrheal hemolytic uremic syndrome (D+HUS) that may help practitioners determine appropriate treatment. METHODS: This retrospective study was conducted in 49 children with D+HUS between 2001 and 2011. Severe forms were defined as occurrence of one of the following conditions: death, major neurological involvement, cardiovascular involvement, and/or the presence of sequelae (neurological, cardiovascular, pancreatic, or renal). RESULTS: During the acute phase, 35 children exhibited at least one type of extrarenal involvement including 13 severe forms with a median delayed occurrence after admission of 4.5 days (range: 1-8) for comatose children and 5 days (range: 2-6) for cardiovascular involvement; 32 children required dialysis and three died. In multivariate analysis, (i) major neurological involvement (n=13), (ii) dialysis (n=32), and (iii) sequelae (n=12) were associated with (i) fever during the prodromal phase requiring dialysis at admission, (ii) C-reactive protein level (CRP) >22mg/L at admission, and (iii) major neurological involvement and a white blood cell count (WBC)>20×103/mm3 during the acute stage, respectively. CONCLUSIONS: D+HUS is a multiorgan disease with a delayed occurrence of life-threatening extrarenal organ involvement. Severe forms appear to be associated with early biological and clinical inflammatory parameters.

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Neurometabolic and neurodegenerative diseases in children (NDDC) differ from those in adults in that most of the former are autosomal-recessively inherited - few have X-linked inheritance - while the latter are often sporadic or autosomal-dominantly inherited. NDDC may be catabolic and/or anabolic conditions, some of which combine maldevelopmental and degenerative features, for instance, peroxisomal biogenesis disorders or congenital disorders of glycosylation. NDDC are often multiorgan disorders, such as lysosomal, peroxisomal, and polyglucosan disorders. This multiorgan involvement may be marked by extracerebral formation of disease-specific neuropathologic findings, especially in lysosomal diseases allowing diagnostic biopsies in easily accessible tissues, e.g., blood lymphocytes, skin, skeletal muscle, and rectum to be investigated by electron microscopy. NDDC comprise nonvacuolar and vacuolar lysosomal, peroxisomal, polyglucosan, amino and organic acid, white-matter disorders, and congenital disorders of glycosylation.

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Resumen: Introducción: La sarcoidosis es una enfermedad sistémica de etiología desconocida que raramente se presenta en la infancia. Generalmente afecta los pulmones; sin embargo, puede involucrar diversos órganos. Ocasionalmente afecta el estado general, y origina fiebre, hepatomegalia y esplenomegalia. Caso clínico: Se presenta el caso de un adolescente de doce años de edad con sarcoidosis infantil de inicio tardío, cuyo diagnóstico fue confirmado con un estudio histopatológico de ganglio linfático. El paciente cursó con afección general, hipercalcemia, eritema nodoso, alteraciones pulmonares graves, adenopatías, hepatomegalia y masa testicular. Recibió tratamiento con esteroides, con excelente respuesta clínica. Conclusiones: Se resalta la importancia de considerar el diagnóstico de sarcoidosis en los pacientes con hepatomegalia, adenopatías, daño pulmonar difuso, eritema nodoso, masa testicular e hipercalcemia, así como la necesidad del abordaje multidisciplinario para valorar el compromiso orgánico múltiple y el inicio oportuno de la terapia con esteroides, con el fin de evitar la progresión de la enfermedad.

Abstract: Background: Sarcoidosis is a systemic disease of unknown etiology that rarely occurs in children. It usually affects the lungs, however, it may involve various organs. It occasionally affects the general condition, and causes fever, hepatomegaly and splenomegaly. Case report: We report the case of a twelve-year-old adolescent with late-onset childhood sarcoidosis which diagnosis was confirmed by lymph node histopathological study. The patient presented general condition, hypercalcemia, erythema nodosum, severe lung disorders, lymphadenopathy, hepatomegaly and testicular mass. He received treatment with steroids, with excellent clinical response. Conclusions: We highlight the importance of considering the diagnosis of sarcoidosis in patients with hepatomegaly, lymphadenopathy, diffuse lung damage, erythema nodosum, testicular mass and hypercalcemia, as well as the need for a multidisciplinary approach to assess multiple organ involvement and the early beginning of steroid treatment in order to prevent the progression of the disease.

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BACKGROUND: Sarcoidosis is a systemic disease of unknown etiology that rarely occurs in children. It usually affects the lungs, however, it may involve various organs. It occasionally affects the general condition, and causes fever, hepatomegaly and splenomegaly. CASE REPORT: We report the case of a twelve-year-old adolescent with late-onset childhood sarcoidosis which diagnosis was confirmed by lymph node histopathological study. The patient presented general condition, hypercalcemia, erythema nodosum, severe lung disorders, lymphadenopathy, hepatomegaly and testicular mass. He received treatment with steroids, with excellent clinical response. CONCLUSIONS: We highlight the importance of considering the diagnosis of sarcoidosis in patients with hepatomegaly, lymphadenopathy, diffuse lung damage, erythema nodosum, testicular mass and hypercalcemia, as well as the need for a multidisciplinary approach to assess multiple organ involvement and the early beginning of steroid treatment in order to prevent the progression of the disease.

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Hydatid disease is commonly encountered in specific geographic areas of the world. Hydatidosis affects multiple organs and has diverse radiologic presentations. Sonography remains an important modality for diagnosing this condition, as it optimally detects cystic structures, floating membranes, and debris. Sonography forms the crux of radiologic diagnosis of hydatid disease. It not only helps diagnose the disease but also aids in guiding therapeutic interventions. The main objective of this article is to describe the imaging features of hydatid disease in its various stages. This article gives an overview of the spectrum of sonographic manifestations of hydatid disease in various locations, along with common differential diagnoses. A brief description of therapeutic management is also presented.

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