Challenging Treatment of Disseminated Aspergillosis in a Child With Nephrotic Syndrome.

INTRODUCTION: Central nervous system (CNS) aspergillosis is an opportunistic infection with an increasing incidence and a high mortality rate. It is seen in immunocompromised patients as well as in immunocompetent patients. Here, we present disseminated aspergillosis in a child with nephrotic syndrome treated with long-term and aggressive systemic antifungal treatment and intraventricular (IVent) liposomal amphotericin B (L-AmB) as well as surgical excision and drainage due to difficulty in management. CASE REPORT: A 10-year-old boy with nephrotic syndrome on steroid therapy was admitted with limping and weakness. The cranial magnetic resonance imaging showed multiple intraparenchymal scattered abscesses. The largest one was excised and drained. Abscess culture revealed Aspergillus fumigatus and histopathological examination revealed septate hyphae compatible with Aspergillosis. Intravenous (IV) voriconazole was started, and IV L-AmB was added. The size of lesions and perilesional edema continued to increase, and then IVent L-AmB was added. With IVent and systemic antifungal treatment, regression of the lesions was observed. He was followed up with oral voriconazole and weekly IVent L-AmB. After 2 and a half months, he was re-operated because of increased lesion size, number and perilesional edema, and IV voriconazole and other salvage antifungal therapies were started. Since the lesions had decreased and remained stable, IV voriconazole was switched to oral therapy, and he was followed up as an outpatient. Immunodeficiency diseases were excluded by immunological and genetic tests. CONCLUSION: Management of central nervous system aspergillosis can be challenging despite long-term and aggressive systemic and IVent antifungal treatment as well as surgical excision and drainage.

A 4-Year-Old Child with a Giant Cerebral Hydatid Cyst: A Case Report.

Echinococcosis is the most common cestode infection globally caused by the Echinococcus species. The most common organ involvement is the lungs and liver, but other organs can be rarely involved. Here, we present a case with a giant cerebral hydatid cyst. A 4-year-old boy presented with abnormal gait and walking at Marmara University School of Medicine Pendik Training and Research Hospital, Istanbul, Türkiye in September 2022. Cranial magnetic resonance imaging showed a cyst of 13 cm in diameter. The cyst was enucleated successfully with no rupture. Oral albendazole therapy was started. There was no eosinophilia, and the echinococcal indirect hemagglutination test was negative. Ultrasonography detected an anechoic cystic lesion in the liver. He was evaluated for deep-organ involvement; however, no cysts were detected in other organs. The histopathological examination was compatible with a hydatid cyst. Although intracranial hydatid disease in children is rare, it should be considered among the differential diagnoses in patients with neurological symptoms, especially in endemic regions.

A new method for quantification of frontal retrusion and complex skull shape in metopic craniosynostosis: a pilot study of a new outcome measure for endoscopic strip craniectomy.

OBJECTIVE: The objective of this study was to propose a new skull outline-based method to objectively quantify complex 3D skull shapes and frontal and supraorbital retrusion in metopic craniosynostosis using 3D photogrammetry. METHODS: A standard section from 3D photogrammetry, which represents the trigonocephalic shape, was used in this study. From the midpoint of the area of this section, half diagonals were calculated to the skull outline at 5° increments in the anterior half of the head. These half diagonals were used to create a sinusoidal curve, and the area under the sinusoidal curve (AUC) was used to represent the mathematical expression of the trigonocephalic head shape. The AUC from 0° to 180° (90° from the midline to each side) was calculated and is referred to as AUC0→180. The AUC from 60° to 120° (30° from the midline to each side) was also calculated and is referred to as AUC60→120. A total of 24 patients who underwent endoscopic strip craniectomy and 13 age- and sex-matched controls were included in the study. The AUC values obtained in patients at different time points and controls were analyzed. RESULTS: The mean preoperative AUC60→120 and AUC0→180 in the patients were significantly lower than those in control individuals. The increase in both AUC60→120 and AUC0→180 values is statistically significant at the discontinuation of helmet therapy and at final follow-up. Receiver operating characteristic curve analysis indicated that AUC60→120 is a more accurate classifier than AUC0→180. CONCLUSIONS: The proposed method objectively quantifies complex head shape and frontal retrusion in patients with metopic craniosynostosis and provides a quantitative measure for follow-up after surgical treatment. It avoids ionizing radiation exposure.

The relationship between glycemic control and osteocalcin, type 1 collagen C-terminal telopeptide, bone-specific alkaline phosphatase and the effects of anti-diabetic regimens on circulating markers of bone turnover in newly diagnosed diabetic patients: Bone health in diabetics.

AIM: Bone mineral disorders are being increasingly seen among diabetic populations as the frequency of diabetes mellitus (DM) is rising at an alarming rate. Our aim is to examine the relationship between glycemic control and bone turnover markers like osteocalcin (OC), C-terminal carboxy telopeptide (CTX), and bone-specific alkaline phosphatase (ALP) in patients with type 2 diabetes, and the effects of anti-diabetic regimens on these markers. MATERIALS AND METHODS: A total of 80 newly diagnosed type 2 DM patients were enrolled into the study and divided into two groups according to glucose regulation (group 1 HbA1c < 7 and group 2 HbA1c ≥ 7). They were also classified into three groups according to antidiabetic regimen. Physical examination findings, demographic characteristics, and anti-diabetic regimens of the patients were recorded. Hemogram and biochemical parameters were studied after 12 hours of fasting. Serum levels OC and CTX were examined by ELISA method. Bone-specific ALP was examined by Chemiluminesence immuneassay (CLIA) method. Bone densitometry was performed on the 2016 model Stratos DR device of DMS brand, and T scores of the patients were recorded. All parameters were repeated at the 6th month of the study. RESULTS: Serum vitamin D and OC levels of group 1 were higher, while ALP was higher in group 2. However, we failed to determine a significant difference in CTX levels between the groups. OC levels were enhanced only in patients receiving metformin plus vildagliptin therapy. The CTX levels increased in all groups, whereas they decreased in the metformin plus DPP-4 group. CONCLUSION: Better glucose regulation is associated with better bone formation, and among three groups metformin plus vildagliptin therapy has a favorable effect on both bone formation and resorption.

Clinical and imaging features of children with autoimmune encephalitis and MOG antibodies.

OBJECTIVE: To describe the presentations, radiologic features, and outcomes of children with autoimmune encephalitis associated with myelin oligodendrocyte glycoprotein antibodies (MOG abs). METHODS: Identification of children fulfilling the diagnostic criteria for possible autoimmune encephalitis (AE) and testing positive for serum MOG abs. Chart review and comprehensive analysis of serum MOG abs using live cell assays and rat brain immunohistochemistry. RESULTS: Ten children (4 girls, 6 boys) with AE and serum MOG abs were identified. The median age at onset was 8.0 years (range: 4-16 years). Children presented with a combination of encephalopathy (10/10), headache (7/10), focal neurologic signs (7/10), or seizures (6/10). CSF pleocytosis was common (9/10, median 80 white cell count/µL, range: 21-256). Imaging showed cortical and deep gray matter involvement in all in addition to juxtacortical signal alterations in 6/10 children. No involvement of other white matter structures or contrast enhancement was noted. MOG abs were detected in all children (median titer 1:640; range: 1:320-1:10,540). Nine children had a favorable outcome at discharge (modified Rankin scale of < 2). Five of 10 children had up to 3 additional demyelinating relapses associated with persisting MOG abs. One child had NMDA receptor (NMDAR) abs at initial presentation. A second child had a third demyelinating episode with MOG abs with overlapping NMDAR encephalitis. DISCUSSION: AE associated with serum MOG abs represents a distinct form of autoantibody-mediated encephalitis in children. We therefore recommend including MOG abs testing in the workup of children with suspected AE.

Analyses of Copy Number Variations in Myxopapillary Ependymomas of Cauda Equina.

AIM: To identify the copy number variations that are specific to myxopapillary ependymomas (MPEs) of the cauda equina. MATERIAL AND METHODS: The patient cohort included five patients who underwent resection of histologically confirmed MPEs. Tumor samples collected during surgery and stored in liquid nitrogen as well as corresponding blood samples collected were analyzed. Genomic DNA from the venous blood and tumor samples was obtained using standard techniques and hybridized to a Cytoscan 750K Array in accordance with the manufacturer’s introductions. RESULTS: As a novel finding, amplification on chromosome 14q32.33 was detected in all tumor and blood samples, except one tumor sample. All tumor tissues also showed amplification on chromosomes 5, 7, 9, and 16. CONCLUSION: Although further studies with larger cohorts are required to identify genes involved in MPE tumorigenesis and to validate our results, these findings provide a basis for advanced molecular biological and genetic studies of MPEs.