Posterior reversible leukoencephalopathy syndrome associated with acute postinfectious glomerulonephritis: systematic review.

BACKGROUND: Kidney diseases are a recognized cause of posterior reversible leukoencephalopathy syndrome, usually abbreviated as PRES. The purpose of this review was to systematically address the association between acute postinfectious glomerulonephritis and PRES. METHODS: We performed a systematic review of the literature on acute postinfectious glomerulonephritis associated with PRES. The principles recommended by the Economic and Social Research Council guidance on the conduct of narrative synthesis and on the Preferred Reporting Items for Systematic Reviews and Meta-analyses were used. Databases searched included Excerpta Medica, US National Library of Medicine, and Web of Science. RESULTS: For the final analysis, we evaluated 47 reports describing 52 cases (32 males and 20 females). Fifty patients were ≤ 18 years of age. Blood pressure was classified as follows: normal-elevated (n = 3), stage 1 hypertension (n = 3), stage 2 hypertension (n = 5), and severe hypertension (n = 41). Acute kidney injury was classified as stage 1 in 32, stage 2 in 16, and stage 3 in four cases. Neuroimaging studies disclosed a classic posterior PRES pattern in 28 cases, a diffuse PRES pattern in 23 cases, and a brainstem-cerebellum PRES pattern in the remaining case. Antihypertensive drugs were prescribed in all cases and antiepileptic drugs in cases presenting with seizures. A resolution of clinical findings and neuroimaging lesions was documented in all cases with information about follow-up. CONCLUSIONS: The main factor associated with PRES in acute postinfectious glomerulonephritis is severe hypertension. Prompt clinical suspicion, rapid evaluation, and management of hypertension are crucial. A higher resolution version of the Graphical abstract is available as Supplementary information.

Tracheal agenesis: optimization of computed tomography diagnosis by airway ventilation.

Tracheal agenesis is a rare and often lethal congenital defect that leads to airway emergency at birth. Computed tomography (CT) is the modality of choice to evaluate anomalous tracheal anatomy. The absence of spontaneous aeration of the tracheobronchial tree in children with tracheal agenesis makes CT interpretation difficult. We describe a procedure of airway management applied in two newborns with suspected tracheal agenesis. Correct airway management was performed immediately prior to CT examination by airway ventilation, with bag-valve mask alone in one case, and attached to an endotracheal tube placed into the esophagus in the other case. The images allowed for classification of tracheal agenesis. Computed tomography with appropriate airway ventilation is fundamental for the diagnosis of tracheal agenesis.

External Male Genitalia in Henoch-Schönlein Syndrome: A Systematic Review.

The external genitalia are notoriously implicated in every fifth male with Henoch−Schönlein syndrome. Nonetheless, the underlying conditions are poorly categorized. To characterize the involvement of the external male genitalia in this vasculitis, we performed a systematic review of the literature. For the final analysis, we selected 85 reports published between 1972 and 2022, which reported on 114 Henoch−Schönlein cases (≤ 18 years, N = 104) with a penile (N = 18), a scrotal (N = 77), or both a penile and a scrotal (N = 19) involvement. The genital involvement mostly appeared concurrently with or after the cutaneous features of Henoch−Schönlein syndrome, while it preceded the presentation of Henoch−Schönlein syndrome in 10 cases. Patients with penile involvement (N = 37) presented with swelling (N = 26), erythema (N = 23), and purpuric rash (N = 15). Most patients were otherwise asymptomatic except for transient micturition disorders (N = 2) or priapism (N = 2). Patients with scrotal involvement (N = 96) presented with pain (N = 85), swelling (N = 79), erythema (N = 42), or scrotal purpura (N = 22). The following scrotal structures were often involved: scrotal skin (N = 83), epididymis (N = 49), and testes (N = 39). An ischemic testicular damage was noted in nine patients (four with torsion and five without). The scrotal skin involvement was mostly bilateral, while that of the epididymis and testis were mostly (p < 0.0001) unilateral (with a significant predilection for the left side). In conclusion, this analysis allows for better categorization of the involvement of external male genitalia in Henoch−Schönlein vasculitis. Scrotal involvement can result from skin inflammation, epididymitis, orchitis, or testicular ischemia.

Bone Bruise and Anterior Cruciate Ligament Tears: Presence, Distribution Pattern, and Associated Lesions in the Pediatric Population.

BACKGROUND: Bone bruise characteristics after anterior cruciate ligament (ACL) injury have been correlated with the level of joint derangement in adults. However, the literature lacks information about younger patients, whose higher ligamentous laxity may lead to different lesion patterns. PURPOSE: To investigate the prevalence, size, location, and role of bone bruise associated with ACL rupture in the pediatric population. STUDY DESIGN: Cross-sectional study; Level of evidence, 3. METHODS: Knee magnetic resonance imaging scans (MRIs) of patients aged 8 to 16 years with ACL tears from 2010 to 2018 were selected from the institution database. Inclusion criteria were open or partially open physes, less than 90 days between trauma and MRI, and no history of injury or surgery. Presence, localization, and size of bone bruise were analyzed by 2 blinded researchers and scored with the Whole-Organ Magnetic Resonance Imaging Score (WORMS) bone bruise subscale. Ligamentous, cartilaginous, meniscal, and other lesions were documented. RESULTS: Of the 78 pediatric patients selected from the database, 54 (69%) had bone bruise. The mean area of bone bruise was larger in males than in females (femur, 3.8 ± 2.8 vs 2.2 ± 1.4 cm2, respectively, P = .006; tibia, 2.6 ± 1.6 vs 1.5 ± 0.8 cm2, respectively, P = .007). The subregions most affected by bone bruise were the lateral posterior tibia and the lateral central femur (in 83% and 80% of the knees affected, respectively). A low correlation was found between age and bone bruise area (biggest areas r = 0.30, P = .03, and sum of areas r = 0.27, P = .04), but no correlation was found between age and WORMS (femur, r = -0.03, P = .85; tibia, r = -0.04, P = .76). The injuries most associated with bone bruise were 23 meniscal lesions (43%), 10 lesions of other ligaments (19.0%), 2 cartilage lesions (3.7%), and 2 patellar fractures (3.7%). CONCLUSION: The prevalence of bone bruises in pediatric patients with ACL tears is high, although it seems slightly lower than the prevalence documented in adults but with similar localization. The area and the distribution pattern of bone bruises are similar among different ages. The pediatric patients had a lower presence of cartilage and meniscal lesions compared with that reported in adults, which suggests a different effect of this trauma on the knee of pediatric patients.

Erdheim-Chester disease: a systematic review.

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans-cell histiocytosis, associated in more than 50% of cases to BRAF(V600E) mutations in early multipotent myelomonocytic precursors or in tissue-resident histiocytes. It encompasses a spectrum of disorders ranging from asymptomatic bone lesions to multisystemic, life-threatening variants. We reviewed all published reports of histologically-confirmed ECD and explored clinical, radiological, prognostic and therapeutic characteristics in a population of 448 patients, including a unique patient from our Department. To find a clinically relevant signature defining differentiated prognostic profiles, the patients' disease features were compared in relation to their CNS involvement that occurred in 56% of the entire population. Diabetes insipidus, visual disturbances, pyramidal and extra-pyramidal syndromes were the most recurrent neurological signs, whereas concomitant pituitary involvement, retro-orbital masses and axial lesions in the presence of symmetric bilateral osteosclerosis of long bones depicted the typical ECD clinical picture. Patients with CNS infiltration showed a lower occurrence of heart involvement and a higher incidence of bone, skin, retro-peritoneal, lung, aortic and renal infiltration. No difference in the therapeutic algorithm was found after stratification for CNS involvement. A better understanding of the disease pathogenesis, including BRAF deregulation, in keeping with improved prognostic criteria, will provide novel suggestions for the management of ECD.