Cystic Disease Research Articles

Odontogenic keratocyst in the mandible of a 7‐year‐old child: A case report and literature review

AbstractOdontogenic keratocysts (OKCs) are one of the most frequently encountered cystic diseases of the jaw in clinical practice. Although OKC has been reported as a manifestation of nevoid basal cell carcinoma syndrome (Gorlin syndrome) in many pediatric patients, there have been few reports of OKC occurring in isolation. Herein, we report the case of a relatively large OKC in the mandibular body of a 7‐year‐old girl who was treated conservatively using cystectomy and open wound surgery, and the permanent teeth were unaffected. Regular check‐ups, including radiographic examinations, starting in childhood may contribute to the early detection of lesions in the maxillofacial region.

A Case Series on Hydatid Cyst Disease: Usual and Unusual Sites with Different Approaches and Management

Hydatid disease is a cyclo zoonotic parasitic infection caused by Echinococcus granulosus. This disease is commonly found in the liver and lungs but no organ of the body is immune. When found at unusual sites in the body they show atypical presentations and pose a diagnostic challenge. In such conditions, a high index of suspicion, radiological investigations as well as histopathological examination are necessary in establishing the diagnosis. We present a review of the occurrence of hydatid disease at common as well as unusual sites and our experience with clinical presentation and management of hydatid disease.

Prognostic Role of Prolactin-Induced Protein (PIP) in Breast Cancer.

Prolactin-inducible protein (PIP), also referred to as gross cystic disease fluid protein 15 (GCDFP-15), has been a trending topic in recent years due to its potential role as a specific marker in breast cancer. PIP binds to aquaporin-5 (AQP5), CD4, actin, fibrinogen, β-tubulin, serum albumin, hydroxyapatite, zinc α2-glycoprotein, and the Fc fragment of IgGs, and the expression of PIP has been demonstrated to be modulated by various cytokines, including IL4/13, IL1, and IL6. PIP gene expression has been extensively studied due to its captivating nature. It is influenced by various factors, with androgens, progesterone, glucocorticosteroids, prolactin, and growth hormone enhancing its expression while estrogens suppress it. The regulatory mechanisms involve important proteins such as STAT5A, STAT5B, Runx2, and androgen receptor, which collaborate to enhance PIP gene transcription and protein production. The expression level of PIP in breast cancer is dependent on the tumor stage and subtype. Higher expression is observed in early-stage tumors of the luminal A subtype, while lower expression is associated with luminal B, basal-like, and triple-negative subtypes, which have a poorer prognosis. PIP expression is also correlated with apocrine differentiation, hormone receptor positivity, and longer metastasis-free survival. PIP plays a role in supporting the immune system's antitumor response during the early stages of breast cancer development. However, as cancer progresses, the protective role of PIP may become less effective or diminished. In this work, we summarized the clinical significance of the PIP molecule in breast cancer and its potential role as a new candidate for cell-based therapies.

Sex/Gender-Based Disparities in Early Transplant Access by Attributed Cause of Kidney Disease–Evidence from a Multiregional Cohort in the Southeast United States

We examined sex/gender disparities across the continuum of transplant care by attributed cause of end-stage kidney disease (ESKD). All adults (18-79 years; N= 43,548) with new-onset ESKD in Georgia, North Carolina, or South Carolina between 2015 and 2019 were identified from the United States Renal Data System (USRDS). Individuals were linked to the Early Steps to Transplant Access Registry (E-STAR) to obtain data on referral and evaluation. Waitlisting data was ascertained from USRDS. Using a Cox-proportional hazards model, with follow-up through 2020, we assessed the association between sex/gender and referral within 12 months (among all incident dialysis patients), evaluation start within 6 months (among referred patients), and waitlisting (among all evaluated patients) by attributed cause of ESKD (type 1 diabetes mellitus, type 2 diabetes mellitus, hypertension, glomerulonephritis, cystic disease, and other). Overall, women (vs. men) with type 2 diabetes-attributed ESKD were 13% (crude hazard ratio [HR]: 0.87 [0.83-0.91]), 14% (crude HR: 0.86 [0.81-0.91]), and 14% (crude HR: 0.86 [0.78-0.94]) less likely to be referred, evaluated, and waitlisted, respectively. Women (vs. men) with hypertension-attributed ESKD were 14% (crude HR: 0.86 [0.82-0.90]) and 8% (crude HR: 0.92 [0.87-0.98]) less likely to be referred and evaluated, respectively, but similarly likely to be waitlisted once evaluated (crude HR: 1.06 [0.97-1.15]). For all other attributed causes of ESKD, there was no sex/gender disparity in referral, evaluation, or waitlisting rates. In the Southeast United States, sex/gender disparities in early access to kidney transplantation are specific to people with ESKD attributed to type 2 diabetes and hypertension.

Is the Hippo Pathway Effector Yes-Associated Protein a Potential Key Player of Dairy Cattle Cystic Ovarian Disease Pathogenesis?

Cystic ovarian disease (COD) in dairy cattle is characterized by preovulatory follicles that become cysts, fail to ovulate and persist in the ovary; consequently, interfering with normal ovarian cyclicity. The intraovarian key players that orchestrate the alterations occurring in the preovulatory follicle and that culminate with cyst formation and persistence, however, remain uncertain. Interestingly, the Hippo pathway effector yes-associated protein (YAP) has been described in humans and mice as a key player of anovulatory cystic disorders. To start elucidating if YAP deregulation in ovarian follicle cells can be also involved in the pathogenesis of COD, we have generated a series of novel results using spontaneously occurring cystic follicles in cattle. We found that mRNA and protein levels of YAP are significantly higher in granulosa (GCs) and theca cells (TCs) isolated from cystic follicles (follicular structures of at least 20 mm in diameter) in comparison to respective cell types isolated from non-cystic large follicles (≥12 mm). In addition, immunohistochemistry and Western blot analyses used to determine YAP phosphorylation pattern suggest that YAP transcriptional activity is augmented is cystic GCs. These results were confirmed by a significant increase in the mRNA levels encoding for the classic YAP-TEAD transcriptional target genes CTGF, BIRC5 and ANKRD1 in GCs from follicle cysts in comparison to non-cystic large follicles. Taken together, these results provide considerable insight of a completely novel signaling pathway that seems to play an important role in ovarian cystic disease pathogenesis in dairy cattle.

Probe-based confocal laser endomicroscopy in diagnosis of diffuse cystic lung disease in Sjögren's syndrome.

Sjögren's syndrome is systemic autoimmune disease characterized by lymphocytic infiltration of various organs with wide frequency of pulmonary involvement. Diffuse cystic lung disease in Sjögren's syndrome is a rare condition and requires differential diagnosis with other cystic pathologies such as lymphangioleyomiomatosis or Langerhans cell histiocytosis. Probe-based confocal laser endomicroscopy (pCLE) is a method of in vivo investigation of airways and lung tissue on microscopic level during bronchoscopy. We used this method in diffuse cystic lung disease caused by Sjögren's syndrome. The pCLE image showed a large number of fluorescent cells presumably lymphocytes in bronchioles, dilated alveolar spaces with fluid and thin alveolar walls. We think that the presence of the bronchiolar cells pattern can be used to differentiate between the pulmonary manifestations of Sjögren's disease and other cystic lung diseases.

Cystic fibrosis liver disease in the post-modulator era.

Highly effective modulators of cystic fibrosis transmembrane conductance regulator (CFTR) function have had dramatic impact on pulmonary and nutritional outcomes in persons with cystic fibrosis (pwCF). The impact on liver disease in pwCF was not the focus of the registration trials. The purpose of this review is to assess the current literature on the impact of HEMT on liver disease, progression, regression, and safety. Short-term studies of HEMT in pwCF have shown that there is no significant impact on the frequency of liver enzyme abnormalities. There is no evidence for significant improvement in liver enzymes over time on HEMT therapy. There is conflicting data on improvement in liver fibrosis determined by fibrosis indices (APRI and GPR) or elastography. One study showed improvement, and another showed worsening in younger (<20 years old) pwCF. There are reports of resolution or improvement in hepatic steatosis. There are rare reports of severe acute hepatitis and one report of hepatic decompensation leading to liver transplantation due to drug-induced liver disease. HEMT have not been shown to have a significant impact on improving liver disease or preventing fibrosis with short-term therapy. Longer studies are needed to assess the impact of HEMT on liver disease in pwCF.

Substrate-dependent metabolomic signatures of myeloperoxidase activity in airway epithelial cells: Implications for early cystic fibrosis lung disease.

Myeloperoxidase (MPO) is released by neutrophils in inflamed tissues. MPO oxidizes chloride, bromide, and thiocyanate to produce hypochlorous acid (HOCl), hypobromous acid (HOBr), and hypothiocyanous acid (HOSCN), respectively. These oxidants are toxic to pathogens, but may also react with host cells to elicit biological activity and potential toxicity. In cystic fibrosis (CF) and related diseases, increased neutrophil inflammation leads to increased airway MPO and airway epithelial cell (AEC) exposure to its oxidants. In this study, we investigated how equal dose-rate exposures of MPO-derived oxidants differentially impact the metabolome of human AECs (BEAS-2B cells). We utilized enzymatic oxidant production with rate-limiting glucose oxidase (GOX) coupled to MPO, and chloride, bromide (Br-), or thiocyanate (SCN-) as substrates. AECs exposed to GOX/MPO/SCN- (favoring HOSCN) were viable after 24h, while exposure to GOX/MPO (favoring HOCl) or GOX/MPO/Br- (favoring HOBr) developed cytotoxicity after 6h. Cell glutathione and peroxiredoxin-3 oxidation were insufficient to explain these differences. However, untargeted metabolomics revealed GOX/MPO and GOX/MPO/Br- diverged significantly from GOX/MPO/SCN- for dozens of metabolites. We noted methionine sulfoxide and dehydromethionine were significantly increased in GOX/MPO- or GOX/MPO/Br--treated cells, and analyzed them as potential biomarkers of lung damage in bronchoalveolar lavage fluid from 5-year-olds with CF (n=27). Both metabolites were associated with increasing bronchiectasis, neutrophils, and MPO activity. This suggests MPO production of HOCl and/or HOBr may contribute to inflammatory lung damage in early CF. In summary, our in vitro model enabled unbiased identification of exposure-specific metabolite products which may serve as biomarkers of lung damage in vivo. Continued research with this exposure model may yield additional oxidant-specific biomarkers and reveal explicit mechanisms of oxidant byproduct formation and cellular redox signaling.

Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is an ultra-rare, slowly progressive neoplastic cystic disease, belonging to the group of PEComas. It can occur sporadically or associated to tuberous sclerosis complex disease and affects mainly women in child-birth age. Dyspnoea is the most frequent symptom referred to the time of diagnosis, however spontaneous pneumothorax may be a typical presentation associated to extrathoracic manifestations, such as renal angiomyolipomas. In the last decade, important advances in understanding molecular mechanisms underlying the LAM pathogenesis have been reached. It has allowed to obtain improvements in the research of novel biomarkers, treatment and a better management of the disease.

The Role of Genetic Testing in Pediatric Renal Diseases: Diagnostic, Prognostic, and Social Implications.

Pediatric renal diseases vary widely and are linked to high morbidity and mortality; hence, early diagnosis is vital. Presently, genetic testing is being incorporated into the standard of care for children and their families with kidney disease, primarily as a diagnostic tool. In the present review, we aim to collect all potential evidence from relevant studies that reported the role of genetic testing in pediatric renal disease diagnostic, prognostic, and social implications. We have conducted both electronic and manual searches within PubMed, the Cochrane Library, Web of Science, and Scopus to find relevant studies. Studies from the years 2013-2023 were included. Case reports with limited sample sizes and no descriptive statistics, along with review papers and meta-analyses, were excluded from this review. Quality assessment for all included studies was performed. The pooled diagnostic yields were calculated using the common effect and random effect models utilizing the R program (R Foundation for Statistical Computing, Vienna, Austria). The pooled result for the diagnostic yield as per the common effect model is a pooled proportion of 0.42 (42%) 95% confidence interval (CI): [0.39,0.44], while with the random effects model the pooled proportion is 0.43 (43%) 95% CI: [0.31,0.57]. The diagnostic yield for the included studies ranged from 78.10% to 16.8%. The spectrum of kidney diseases included nephrolithiasis/nephrocalcinosis, glomerular diseases, cystic kidney disease, ciliopathies, tubulopathies, chronic kidney disease, and congenital anomalies of the kidneys and urinary tracts (CAKUT), while hematuria and proteinuria were reported by two studies and autosomal recessive and autosomal dominant idiopathic kidney disease was reported by only one study. Genetic testing validates clinical diagnosis and aids in tailoring management strategies; hence, a more precise treatment plan is developed and unnecessary investigations are avoided, which is crucial in the case of children during routine nephrology clinic visits. Genetic counselling is of the utmost importance, so all ethical and social concerns related to genetic testing are addressed in addition to patient satisfaction.

857 Congenital Pulmonary Airway Malformation: A Case Report of Concealed Malignancy

Abstract Introduction Congenital pulmonary airway malformation (CPAM) is a developmental disorder in which hamartomatous lesions arise in the lower respiratory tract. Large population data suggests the incidence to be between 0.87 and 1.92/10,000 live births. Adults most often present with persistent cough or recurrent LRTIs. Their rarity can lead to misdiagnosis as a cystic lesion. Surgery is the intervention of choice for symptomatic patients. The role of expectant management in asymptomatic patients remains controversial. In adults, resection may be favoured as these lesions are prone to infection and malignancy develops in approximately 1%. Method We present the case of a 44-year-old woman, with shortness of breath and pleuritic chest pain. CT revealed a 9.7 x 5.4cm lucent lesion in the left lower lobe, initially identified as a large bulla or parenchymal cyst. Decision to resect was made post discussion with the patient, who was keen to proceed to surgery, due to ongoing symptoms. Results A left lower lobectomy was performed via VATS. She was discharged on post-operative day 6, made an excellent recovery on 6-week follow-up and has entered surveillance. Histopathology revealed that the cyst was partially lined with respiratory epithelium and palisading histiocytes; favouring a CPAM type 1 diagnosis. Microscopically, there were foci of mucinous lepidic adenocarcinoma. Conclusions It is important to consider CPAM in the differential for cystic lung diseases. Surgical resection should be considered for CPAM regardless of symptoms, due to the uncertainty in diagnosis from imaging alone and the potential for malignant change.

198 Clinical and Radiological Features of Cardiac Hydatid Cysts: A Case Series

Abstract Aim Hydatid cyst disease is a pathology caused by tapeworm Echinococcus. The cardiac involvement is rare, so the aim of this study is to highlight its clinical polymorphism and different radiological features. Method This is a retrospective study of 7 patients diagnosed with cardiac hydatid cysts who were admitted to our department in a 5-year duration. Results We see that most affected individuals were women (4 out of 7), the revealing symptoms were mostly chest pain and dyspnea, only one case presented AV block with faintness. Concerning the localizations: 2 patients had pericardial cysts, 2 had intramyocardial cysts, 2 others had both pericardial and intramyocardial cysts and the last one presented an interventricular septum cyst. Six patients were diagnosed through transthoracic echocardiography (TTE) and one patient with CT of the chest. In all cases, TTE described a cystic mass except one case that presented multivesicular aspect. The diameter of the cysts ranged between 20 and 88 mm. Two patients had associated liver hydatid cyst and one had pulmonary hydatid cyst. All these patients underwent open-heart surgery for conservative treatment under cardiopulmonary bypass. Only one patient presented recurrence which was treated with albendazole with good results. No patient benefited from pre-operative medication with anthelmintic. Conclusions Cardiac hydatic cysts are rare and usually discovered after dyspnea or chest pain. TTE is the most efficient method of diagnosis. Due to life-threatening complications that these cysts can cause, surgery is the main modality of treatment.

Pathogenic Relationships in Cystic Fibrosis and Renal Diseases: CFTR, SLC26A9 and Anoctamins.

The Cl--transporting proteins CFTR, SLC26A9, and anoctamin (ANO1; ANO6) appear to have more in common than initially suspected, as they all participate in the pathogenic process and clinical outcomes of airway and renal diseases. In the present review, we will therefore concentrate on recent findings concerning electrolyte transport in the airways and kidneys, and the role of CFTR, SLC26A9, and the anoctamins ANO1 and ANO6. Special emphasis will be placed on cystic fibrosis and asthma, as well as renal alkalosis and polycystic kidney disease. In essence, we will summarize recent evidence indicating that CFTR is the only relevant secretory Cl- channel in airways under basal (nonstimulated) conditions and after stimulation by secretagogues. Information is provided on the expressions of ANO1 and ANO6, which are important for the correct expression and function of CFTR. In addition, there is evidence that the Cl- transporter SLC26A9 expressed in the airways may have a reabsorptive rather than a Cl--secretory function. In the renal collecting ducts, bicarbonate secretion occurs through a synergistic action of CFTR and the Cl-/HCO3- transporter SLC26A4 (pendrin), which is probably supported by ANO1. Finally, in autosomal dominant polycystic kidney disease (ADPKD), the secretory function of CFTR in renal cyst formation may have been overestimated, whereas ANO1 and ANO6 have now been shown to be crucial in ADPKD and therefore represent new pharmacological targets for the treatment of polycystic kidney disease.

Cystic adventitial disease van de arteria poplitea: casus en literatuuroverzicht

Cystic adventitial disease of the arteria poplitea: case report and literature review Cystic adventitial disease (CAD) is a relatively rare condition (responsible for 1 in 1,200 cases of calf claudication) with the formation of a mucinous cyst in the tunica adventitia. The arterial, rather than the venous, vascular structures are affected in a relatively young, predominantly male population. Several locations have been described, most of them in the popliteal artery (80-85%). Etiologically, several hypotheses have been put forward, without clear consensus, although the synovial and developmental theories are currently considered the most plausible. The patient’s history generally involves complaints of claudication without the presence of important cardiovascular risk factors. Typically, a spasmodic feeling in the calf with varying degrees of exertion is described, with spontaneous recovery after some period of rest. Of paramount importance is the consideration of CAD in a story of intermittent claudication. A Doppler ultrasound, as well as a CT scan and/or an MRI with associated angiography can all be used for the diagnosis. Surgery with complete excision and interposition of a venous graft is the treatment of choice. The cyst can also be drained by means of a percutaneous ultrasound-assisted needle aspiration, but this is associated with a high need of revision. Percutaneous endovascular treatment with a balloon angioplasty (and possibly stenting) does not show favorable results. Recurrence of CAD after surgical resection is minimal. Recently, several cases have been described with spontaneous resolution of claudication after conservative management without surgical resection need and/or aspiration.

Certain heterozygous variants in the kinase domain of the serine/threonine kinase NEK8 can cause an autosomal dominant form of polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) resulting from pathogenic variants in PKD1 and PKD2 is the most common form of PKD, but other genetic causes tied to primary cilia function have been identified. Biallelic pathogenic variants in the serine/threonine kinase NEK8 cause a syndromic ciliopathy with extra-kidney manifestations. Here we identify NEK8 as a disease gene for ADPKD in 12 families. Clinical evaluation was combined with functional studies using fibroblasts and tubuloids from affected individuals. Nek8 knockout mouse kidney epithelial (IMCD3) cells transfected with wild type or variant NEK8 were further used to study ciliogenesis, ciliary trafficking, kinase function, and DNA damage responses. Twenty-one affected monoallelic individuals uniformly exhibited cystic kidney disease (mostly neonatal) without consistent extra-kidney manifestations. Recurrent de novo mutations of the NEK8 missense variant p.Arg45Trp, including mosaicism, were seen in ten families. Missense variants elsewhere within the kinase domain (p.Ile150Met and p.Lys157Gln) were also identified. Functional studies demonstrated normal localization of the NEK8 protein to the proximal cilium and no consistent cilia formation defects in patient-derived cells. NEK8-wild type protein and all variant forms of the protein expressed in Nek8 knockout IMCD3 cells were localized to cilia and supported ciliogenesis. However, Nek8 knockout IMCD3 cells expressing NEK8-p.Arg45Trp and NEK8-p.Lys157Gln showed significantly decreased polycystin-2 but normal ANKS6 localization in cilia. Moreover, p.Arg45Trp NEK8 exhibited reduced kinase activity invitro. In patient derived tubuloids and IMCD3 cells expressing NEK8-p.Arg45Trp, DNA damage signaling was increased compared to healthy passage-matched controls. Thus, we propose a dominant-negative effect for specific heterozygous missense variants in the NEK8 kinase domain as a new cause of PKD.

Novel α-1,3-Glucosyltransferase Variants and Their Broad Clinical Polycystic Liver Disease Spectrum.

Protein-truncating variants in α-1,3-glucosyltransferase (ALG8) are a risk factor for a mild cystic kidney disease phenotype. The association between these variants and liver cysts is limited. We aim to identify pathogenic ALG8 variants in our cohort of autosomal dominant polycystic liver disease (ADPLD) individuals. In order to fine-map the phenotypical spectrum of pathogenic ALG8 variant carriers, we performed targeted ALG8 screening in 478 ADPLD singletons, and exome sequencing in 48 singletons and 4 patients from two large ADPLD families. Eight novel and one previously reported pathogenic variant in ALG8 were discovered in sixteen patients. The ALG8 clinical phenotype ranges from mild to severe polycystic liver disease, and from innumerable small to multiple large hepatic cysts. The presence of <5 renal cysts that do not affect renal function is common in this population. Three-dimensional homology modeling demonstrated that six variants cause a truncated ALG8 protein with abnormal functioning, and one variant is predicted to destabilize ALG8. For the seventh variant, immunostaining of the liver tissue showed a complete loss of ALG8 in the cystic cells. ALG8-associated ADPLD has a broad clinical spectrum, including the possibility of developing a small number of renal cysts. This broadens the ADPLD genotype-phenotype spectrum and narrows the gap between liver-specific ADPLD and kidney-specific ADPKD.

Pkhd1cyli/cyli mice have altered renal Pkhd1 mRNA processing and hormonally sensitive liver disease.

Autosomal-recessive polycystic kidney disease (ARPKD; MIM #263200) is a severe, hereditary, hepato-renal fibrocystic disorder that causes early childhood morbidity and mortality. Mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene, which encodes the protein fibrocystin/polyductin complex (FPC), cause all typical forms of ARPKD. Several mouse lines carrying diverse, genetically engineered disruptions in the orthologous Pkhd1 gene have been generated, but none expresses the classic ARPKD renal phenotype. In the current study, we characterized a spontaneous mouse Pkhd1 mutation that is transmitted as a recessive trait and causescysticliver (cyli), similar to the hepato-biliary disease in ARPKD, but which is exacerbated by age, sex, and parity. We mapped the mutation to Chromosome 1 and determined that an insertion/deletion mutation causes a frameshift within Pkhd1 exon 48, which is predicted to result in a premature termination codon (UGA). Pkhd1cyli/cyli (cyli) mice exhibit a severe liver pathology but lack renal disease. Further analysis revealed that several alternatively spliced Pkhd1 mRNA, all containing exon 48, were expressed in cyli kidneys, but in lower abundance than in wild-type kidneys, suggesting that these transcripts escaped from nonsense-mediated decay (NMD). We identified an AAAAAT motif in exon 48 upstream of the cyli mutation which could enable ribosomal frameshifting, thus potentially allowing production of sufficient amounts of FPC for renoprotection. This mechanism, expressed in a species-specific fashion, may help explain the disparities in the renal phenotype observed between Pkhd1 mutant mice and patients with PKHD1-related disease. KEY MESSAGES: The Pkhd1cyli/cylimouse expresses cystic liver disease, but no kidney phenotype. Pkhd1 mRNA expression is decreased in cyli liver and kidneys compared to wild-type. Ribosomal frameshifting may be responsible for Pkhd1 mRNA escape from NMD. Pkhd1 mRNA escape from NMD could contribute to the absent kidney phenotype.

Comparison of the activity of boiling water extract of purple cabbage leaves and albendazole drug against protoscolices of Echinococcus granulosus In vitro study.

Hydatid cystic disease is a financial burden in Iraq because it reduces the productivity of sheep, goats, cows, and camels by leaving their afflicted organs unsuitable for human consumption, causing weight loss and poor health. This study aimed to evaluate the efficacy and applicability of Brassica oleracea boiling water extract as an in-vitro anti-protoscolices therapy model to albendazole. Fresh leaves of purple cabbages (Brassica oleracea var. capitata) were extracted comprehensively by maceration in boiling water, and the required 100, 200, and 300 mg/ml concentrations were tested. Hydatid (human or animal) samples were collected from the hospital and external laboratories in Babylon province and stored in Kreb ringers culture media and cyst fluid (4:1) until use. For comparison, several concentrations of albendazole were generated. In three replications, the control group received one milliliter of distilled water and all of the extract and drug concentrations stated above. The viability rates of the protoscolices were determined for (0, 24, 48, 72, 96, 120, 144, 168, 192, 216) hours. According to the findings, the proportion of viable protoscolices was 84 % at zero time and 0 % on the ninth day after the experiment began. The boiling water extract for purple cabbages with a concentration of 300 mg/ml was shown to be the most successful in removing protoscolices viability at a percentage of 0.67% after 96 hours, compared to the albendazole medicine at a percentage of 0% after 120 hours. The findings of a boiling water extract from Brassica oleracea var. capitata revealed that protoscolices' viability had been effective. It can also be used as an alternative to chemotherapy in treating cyst hydatid infection. Keywords: Purple cabbages, Protoscolices, Echinococcosis, Albendazole, plant extract

Effect of Alhagi maurorum Hydroalcoholic Extract on Preventing Secondary Hydatid Cysts in BALB/c Mice

Background: Surgery is the main treatment for hydatid cyst disease, during which the spillage of live protoscoleces is a major cause of hydatidosis recurrence. Scolicidal agents are used to prevent this problem. However, they may have some unpleasant side effects. Objectives: This study assessed the scolicidal effects of Alhagi maurorum hydroalcoholic extract on developing secondary cysts in BALB/c mice infected with Echinococcus granulosus parasites. Methods: BALB/c mice were inoculated intraperitoneally with viable protoscoleces. One month after the infection, the mice were randomly divided into 4 groups (7 per group). Group 1 (control group) was orally administered PBS daily. Group 2 was orally administered albendazole daily (150 mg/kg). Group 3 was orally administered Alhagi maurorum hydroalcoholic extract daily (500 mg/kg). Group 4 was orally administered Alhagi maurorum hydroalcoholic extract daily (250 mg/kg). After 6 weeks of treatment, the weight (g), size (mm), number, and fertility of hydatid cysts were measured after the euthanasia and necropsy of all mice. Results: The weight (g), size (mm), and the number of cysts was significantly lower in the groups receiving 500 mg/kg Alhagi maurorum hydroalcoholic extract, 250 mg/kg Alhagi maurorum hydroalcoholic extract, and 150 mg/kg albendazole than in the control group (P &lt; 0.05). The fertility percentage of hydatid cysts did not show a significant difference between the four groups (P &gt; 0.05). Moreover, there were no statistically significant differences in the parasitological parameters between the three groups: Alhagi maurorum hydroalcoholic extract 500 mg/kg, Alhagi maurorum hydroalcoholic extract 250 mg/kg, and albendazole 150 mg/kg (P &gt; 0.05). Conclusions: The findings demonstrated the effect of Alhagi maurorum hydroalcoholic extract against protoscoleces in BALB/c mice. However, cellular and molecular research should be carried out to gain a deeper understanding of the various effects of this extract on hydatid cysts.

Fetal renal cystic disease and post-natal follow up-a single center experience.

Prenatal sonographic evidence of large, echogenic, or cystic kidneys may indicate any one of a diverse set of disorders including renal ciliopathies, congenital anomalies of the kidney and urinary tract (CAKUT), or multisystem syndromic disorders. Systematic transition planning for these infants from in utero detection to post-natal nephrology management remains to be established. We sought to evaluate the presentation and transition planning for infants identified in utero with bilateral renal cystic disease. Our retrospective observational study identified 72 pregnancies with bilateral renal cystic disease in a single center from 2012 to 2022; 13 of which had a confirmed renal ciliopathy disorder. Clinical and imaging data, genetic test results, and documentation of postnatal follow-up were collected and compared. In our suspected renal ciliopathy cohort (n = 17), autosomal recessive polycystic disease (ARPKD) was the most common diagnosis (n = 4), followed by Bardet-Biedl syndrome (BBS, n = 3), autosomal dominant polycystic disease (ADPKD, n = 2), HNF1B-related disease (n = 2), and Meckel-Gruber syndrome (MKS, n = 2). Four cases were not genetically resolved. Anhydramnios was observed primarily in fetuses with ARPKD (n = 3). Polydactyly (n = 3) was detected only in patients with BBS and MKS, cardiac defects (n = 6) were identified in fetuses with ARPKD (n = 3), MKS (n = 2), and BBS (n = 1), and abnormalities of the CNS (n = 5) were observed in patients with ARPKD (n = 1), MKS (n = 2), and BBS (n = 3). In general, documentation of transition planning was incomplete, with post-natal nephrology management plans established primarily for infants with renal ciliopathies (n = 11/13; 85%). Prenatal sonographic detection of echogenic kidneys should raise suspicion for a broad range of disorders, including renal ciliopathies and CAKUT. Multicenter collaboration will be required to standardize the implementation of transition guidelines for comprehensive nephrology management of infants identified in utero with enlarged, echogenic kidneys.