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181. Prenatal diagnosis of HNF1B-associated renal cysts: Need to differentiate intragenic variants from 17q 12 microdeletion syndrome? (PubMed)

Prenatal diagnosis of HNF1B-associated renal cysts: Need to differentiate intragenic variants from 17q 12 microdeletion syndrome? 17q12 microdeletions containing HNF1B and intragenic variants within this gene are associated with variable developmental, endocrine and renal anomalies, often already noted prenatally as hyperechogenic/cystic kidneys. Here, we describe pre- and postnatal phenotypes of seven individuals with HNF1B aberrations and compare their clinical and genetic data to previous

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2019 Prenatal diagnosis

182. <i>ALG9</i> Mutation Carriers Develop Kidney and Liver Cysts. (PubMed)

ALG9 Mutation Carriers Develop Kidney and Liver Cysts. Mutations in PKD1 or PKD2 cause typical autosomal dominant polycystic kidney disease (ADPKD), the most common monogenic kidney disease. Dominantly inherited polycystic kidney and liver diseases on the ADPKD spectrum are also caused by mutations in at least six other genes required for protein biogenesis in the endoplasmic reticulum, the loss of which results in defective production of the PKD1 gene product, the membrane protein (...) after age 50; seven (88%) had at least four kidney cysts, compared with none in matched controls without ALG9 mutations.ALG9 is a novel disease gene in the genetically heterogeneous ADPKD spectrum. This study supports the utility of phenotype characterization in genetically-defined cohorts to validate novel disease genes, and provide much-needed genotype-phenotype correlations.Copyright © 2019 by the American Society of Nephrology.

2019 Journal of the American Society of Nephrology

183. Correction to: A child with bilateral multiple renal cysts presenting with ascites and pleural effusion: answers. (PubMed)

Correction to: A child with bilateral multiple renal cysts presenting with ascites and pleural effusion: answers. The original version of this article unfortunately contained a mistake. The answer to question 3 was presented incorrectly. The correct answer is given below.

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2019 Pediatric Nephrology

184. Case Report: Laparoscopic Excision of a Primary Giant Splenic Hydatid Cyst: Literature Review. (PubMed)

Case Report: Laparoscopic Excision of a Primary Giant Splenic Hydatid Cyst: Literature Review. Cystic echinococcosis (CE), also known as hydatid cyst, is a zoonosis caused by the tapeworm Echinococcus granulosus. It is a common health problem in many countries. This condition predominantly affects the liver and the lungs, and the spleen to a less extent (splenic hydatid cyst, SHD). Indeed, it is estimated that SHD occurs in less than 2% of abdominal CE and 0.5-8% of CE cases. Here, we present (...) . In addition to this case, we review the previous literature on SHD treated by laparoscopy and reveal that laparoscopic approach is safe and effective for SHD. Particularly, we show that laparoscopic splenectomy is feasible for giant cysts (> 10 cm) at high risk of rupture or compressing other vital structures.

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2019 American Journal of Tropical Medicine & Hygiene

185. A longitudinal analysis of albendazole treatment effect on neurocysticercosis cyst evolution using multistate models. (PubMed)

A longitudinal analysis of albendazole treatment effect on neurocysticercosis cyst evolution using multistate models. In neurocysticercosis, the larval form of the pork tapeworm Taenia solium appears to evolve through three phases-active, degenerative and sometimes calcification-before disappearance. The antihelmintic drug, albendazole, has been shown to hasten the resolution of active cysts in neurocysticercosis. Little is known about the time cysts take to progress through each phase (...) , with or without treatment.We reconfigured brain imaging data from patient level to cyst level for 117 patients in a randomized clinical trial of albendazole in which images were taken at baseline, 1, 6, 12 and 24 mo. Applying a multistate model, we modelled the hazard of a cyst evolving to subsequent cyst phases before the next imaging (vs no change). We examined the impact of albendazole treatment overall and by patient and cyst characteristics on the hazard.Albendazole accelerated the evolution from

2019 Transactions of the Royal Society of Tropical Medicine & Hygiene Controlled trial quality: uncertain

186. Giant epidermoid cyst of the spleen in a pediatric patient: A case report. (PubMed)

Giant epidermoid cyst of the spleen in a pediatric patient: A case report. Splenic cysts (SCs) are rare findings in children, particularly the youngest. Here, we discuss a case that is useful for the differential diagnosis and treatment of SCs.A 9-year-old Albanian boy was admitted for severe abdominal pain localized mainly in the left hypochondrium for approximately 24 hours. His medical history was without significant clinical problems.Splenomegaly was diagnosed during the first clinical

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2019 Medicine

187. Nonsurgical integrative Korean Medicine treatment of discal cyst: A case report and a retrospective chart review analysis. (PubMed)

Nonsurgical integrative Korean Medicine treatment of discal cyst: A case report and a retrospective chart review analysis. Discal cysts are rare lesions characterized by pain caused by neurogenic compression with similar symptoms as those of disc herniation. This study aims to report the spontaneous regression of discal cyst achieved through nonsurgical integrative Korean Medicine treatment and the clinical epidemiological features of discal cyst cases collected from 4 institutions.A 31-year (...) -old woman had low back pain and radiating pain equivalent to a numeral rating scale (NRS) of 8 and had limitations in daily work and activities.The patient was diagnosed as having discal cysts that compressed the left S1 based on findings of L-spine magnetic resonance imaging (MRI) performed at our hospital.The patient received nonsurgical Korean Medicine treatment and after 24 days of treatment in the hospital, she underwent 16 additional treatments as an outpatient.Spontaneous regression

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2019 Medicine

188. Esophageal duplication cyst presenting with stridor in a child with congenital pulmonary airway malformation: A case report and literature review. (PubMed)

Esophageal duplication cyst presenting with stridor in a child with congenital pulmonary airway malformation: A case report and literature review. Esophageal duplication cyst (EDC) is a rare developmental aberration originated from the embryonic foregut. It may remain asymptomatic but produce local mass effect on surrounding organs if rapid enlarges. EDC may sometimes accompany with other congenital malformations. Congenital pulmonary airway malformation (CPAM) is a congenital lung malformation (...) with an unknown chance of developing symptoms. Here we report a rare case of esophageal duplication cyst with type 2 congenital pulmonary airway malformation (CPAM).A 16-month old boy with a prenatal diagnosis of type 2 CPAM presented progressive stridor and respiratory distress and was admitted to our hospital under the diagnosis of pneumonia. The patient responded poorly to antibiotics. A chest Xray (CXR) showed consolidation over the left upper lobe with trachea deviated to right side. A chest computed

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2019 Medicine

189. The combination of Cytokines and albendazole therapy for prophylaxis and treatment of experimental ./hydatid cyst. (PubMed)

The combination of Cytokines and albendazole therapy for prophylaxis and treatment of experimental ./hydatid cyst. Albendazole is appropriate chemotherapy for treatment and prophylaxis of cystic echinococcosis (CE). The recent studies show Cytokine therapy could be useful for chronic and progressive diseases, therefore, the use of cytokine in prophylaxis and treatment of hydatidosis could be considerable. The aim of this study is to evaluate the efficacy of interleukin-12 + interferon-gamma (...) , in combination with albendazole for prophylaxis and treatment of CE in Balb/c mice. Albendazole and cytokines were administrated for prophylaxis and treatment in CE. The efficacy of this agents were determined by measuring of size, weight, number of cysts, histology and lymphocyte response. Lymphocyte stimulation index and production of interleukin-12 and interferon-gamma were measured by MTT assay and ELISA respectively. Combination of albendazole and cytokines was very effective for prophylaxis

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2019 Acta Tropica

190. PLCD1 and Pilar Cysts. (PubMed)

PLCD1 and Pilar Cysts. Trichilemmal or "pilar" cysts are commonly found on the scalp and are derived from the outer root sheath of the hair follicle. Multiple trichilemmal cysts present in an autosomal dominant pattern of inheritance, yet the genetic mechanism has remained elusive. In this issue, Hörer et al. (2019) highlight predisposing variants in PLCD1 in such families and propose a monoallelic mutational mechanism that drives cyst formation.Copyright © 2019 The Authors. Published

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2019 Journal of Investigative Dermatology

191. Follow-up of Incidentally Detected Pancreatic Cystic Neoplasms: Do Baseline MRI and CT Features Predict Cyst Growth? (PubMed)

Follow-up of Incidentally Detected Pancreatic Cystic Neoplasms: Do Baseline MRI and CT Features Predict Cyst Growth? Background Incidental detection of pancreatic cystic neoplasm (PCN) has increased. Since a small percentage of PCNs possess malignant potential, management is challenging. The recently revised American College of Radiology (ACR) recommendations define PCN measurement and growth for different categories based on baseline cyst size. However, no data are available regarding PCN (...) recommendations. Cyst features, including location, septations, and mural nodules and multiplicity, were noted. Time to cyst progression (growth by ACR criteria) was examined by using baseline PCN size, among other factors. Results A total of 646 cysts in 390 patients were followed up for a median of 50 months (range, 12-186 months). A total of 184 (28.5%) cysts increased in size, 52 (8.1%) decreased in size, and 410 (63.4%) remained stable. For groups in which baseline PCN size was smaller than 5 mm, 5-14 mm

2019 Radiology

192. Simple Adnexal Cysts: SRU Consensus Conference Update on Follow-up and Reporting. (PubMed)

Simple Adnexal Cysts: SRU Consensus Conference Update on Follow-up and Reporting. This multidisciplinary consensus update aligns prior Society of Radiologists in Ultrasound (SRU) guidelines on simple adnexal cysts with recent large studies showing exceptionally low risk of cancer associated with simple adnexal cysts. Most small simple cysts do not require follow-up. For larger simple cysts or less well-characterized cysts, follow-up or second opinion US help to ensure that solid elements (...) are not missed and are also useful for assessing growth of benign tumors. In postmenopausal women, reporting of simple cysts greater than 1 cm should be done to document their presence in the medical record, but such findings are common and follow-up is recommended only for simple cysts greater than 3-5 cm, with the higher 5-cm threshold reserved for simple cysts with excellent imaging characterization and documentation. For simple cysts in premenopausal women, these thresholds are 3 cm for reporting

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2019 Radiology

193. Supratentorial arachnoid cysts and seizures/epilepsy: A population study in community dwellers aged ≥20 years. (PubMed)

Supratentorial arachnoid cysts and seizures/epilepsy: A population study in community dwellers aged ≥20 years. Literature on the association between arachnoid cysts and seizures/epilepsy is inconsistent, and most series have been flawed due to recruitment bias. In this study, we aimed to assess the prevalence of supratentorial arachnoid cysts (SACs) in Atahualpa residents aged ≥20 years, and to estimate whether these lesions were associated with seizures/epilepsy. A total of 1157 individuals

2019 Epilepsia

194. Exploring the complex associations over time among albendazole treatment, cyst evolution, and seizure outcomes in neurocysticercosis. (PubMed)

Exploring the complex associations over time among albendazole treatment, cyst evolution, and seizure outcomes in neurocysticercosis. To develop a causal model for the occurrence of neurocysticercosis (NC)-related seizures and test hypotheses generated from the model.We used data from a randomized controlled trial comparing albendazole with placebo among patients newly diagnosed with NC. Based on our causal model, we explored the associations among albendazole treatment, NC cyst evolution (...) increased over the first few months following treatment, then decreased over time. Generalized seizures exhibited a more complex association; those in the albendazole group had fewer seizures compared with those in the placebo group for the first few months after treatment, and then the association reversed and those in the placebo arm had fewer seizures. Adjusting for the number of NC cysts in each phase resulted in an attenuation of the strength of association between albendazole and seizure outcomes

2019 Epilepsia Controlled trial quality: predicted high

195. Facet joint cyst haematoma: a rare cause of cauda equina syndrome. (PubMed)

Facet joint cyst haematoma: a rare cause of cauda equina syndrome. Facet joint cysts are a feature of lumbar spondylosis and are an uncommon cause of radiculopathy. Facet joint cyst haematoma is a very rare entity and has previously been reported as a subacute cause of leg pain, back pain, sensory deficit and lower limb weakness. We present the unique case of facet joint cyst haematoma presenting as cauda equina syndrome. An 81 year old lady presented with a 7 day history of back pain and left

2019 British Journal of Neurosurgery

196. Spinal intradural arachnoid cyst associated with diastematomyelia in an adult: a case report and review of literature. (PubMed)

Spinal intradural arachnoid cyst associated with diastematomyelia in an adult: a case report and review of literature. We report a case of a spinal intradural arachnoid cyst in a thirty year old female with a previously undiagnosed and untreated diastematomyelia. The cyst was located exactly between the two hemi-cords of the split cord malformation. The patient was treated successfully by laminoplasty and total removal of the cyst. Additionally, sectioning of the filium terminale was done (...) in the same sitting by a separate incision. This case posed a diagnostic dilemma. Though the clinical findings favoured an extramedullary lesion, the possibility of cystic degeneration in the spinal cord due to the tethered cord syndrome could not be completely negated. Spinal intradural arachnoid cysts have been reported in association with various neural tube defects, mainly in the paediatric age group. However, such a lesion associated with diastematomyelia in an adult, to the best of our knowledge

2019 British Journal of Neurosurgery

197. Spinal intradural arachnoid cyst as a complication of insertion of an interspinous device. (PubMed)

Spinal intradural arachnoid cyst as a complication of insertion of an interspinous device. Spinal intradural arachnoid cysts (SACs) account for approximately 10% of total arachnoid cysts, and iatrogenic attribution is rare. A 50-year-old male presented with low back pain and severe radiating pain in his right leg along the S1 dermatome. He had an interspinous device (ISD) inserted 10 years earlier. Plain radiographs showed breakage and angular movement of the ISD at L4/5. Magnetic resonance (...) imaging (MRI) of the lumbosacral spine disclosed multiple intradural cystic tumors in the entire lumbar region. He underwent removal of the ISD, laminectomy, and resection of the intradural cyst. The pathologic finding of the cystic tumor was compatible with arachnoid cyst. Antibodies to parasites, including cysticercosis, were negative in both the serum and cerebrospinal fluid (CSF). The ventral surface of the implant was clearly broken.

2019 British Journal of Neurosurgery

198. Endoscopic Removal of a Cervical Esophageal Duplication Cyst. (PubMed)

Endoscopic Removal of a Cervical Esophageal Duplication Cyst. A 6-month-old female presented for 2 months of noisy breathing. Flexible laryngoscopy showed limited bilateral vocal fold abduction. Computed tomography revealed a non-enhancing 3.6 × 2.3 × 3.5 cystic prevertebral mass spanning C2-T. Using an endoscopic approach, the overlying mucosa was incised, and the cyst was freed and fully excised from the surrounding mucosa with blunt microlaryngeal instruments without complication. Three (...) months postoperatively she had no respiratory issues and was eating well. Flexible laryngoscopy revealed bilateral vocal fold mobility. We propose that endoscopic removal of a cervical esophageal duplication cyst in selected cases is an alternative to open excision. Laryngoscope, 2019.© 2019 The American Laryngological, Rhinological and Otological Society, Inc.

2019 Laryngoscope

199. A 10-year study of the etiopathogenesis of cysts with a study of seromucinous glands in vocal folds. (PubMed)

A 10-year study of the etiopathogenesis of cysts with a study of seromucinous glands in vocal folds. An increasing number of vocal fold cysts excised, as compared to polyps, over the last decade led us to review these cases. We found a statistically significant increase in cysts excised as compared to polyps, over the latter 5-year period (2013-2017). This prompted us to analyze possible factors responsible for this increase. We also performed a histological study of the normative distribution (...) pattern of seromucinous glands in the apparently normal vocal folds.A retrospective review of all cysts and polyps excised over a 10-year period was performed. Patient demographics, air-pollution levels, videostroboscopic findings and histologic analysis of pathology were reviewed. Findings were compared between the initial and latter 5-year period of all cysts excised. The second part of the study entailed a histological study of the presence and distribution pattern of seromucinous glands in 40

2019 Laryngoscope

200. An Unusual Cause of Pulmonary Arterial Hypertension: Hydatid Cyst. (PubMed)

An Unusual Cause of Pulmonary Arterial Hypertension: Hydatid Cyst. Hydatid cyst is an important health problem throughout the world and it is caused by the larval form of Echinococcus granulosus. Although it is most commonly located in the liver and lungs, hydatid cyst can also affect other organs because the oncospheres spread through the bloodstream. The disease may have many different clinical presentations. Although it is a benign disease, sometimes it can cause serious morbidity and even (...) mortality. Here, the case of a 33-year-old male patient who underwent surgery for a right ventricle and bilateral lung hydatid cyst 9 years ago and had pulmonary hypertension is presented.Copyright © 2019. Published by Elsevier Inc.

2019 Annals of Thoracic Surgery

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