Multiple Small Cysts Research Articles

Mucinous cystic neoplasm of the liver: A rare case report

Mucinous cystic neoplasm of the liver is a rare cystic neoplasm accounting for approx. 5% of cystic liver in the liver parenchyma. We describe the histomorphology and immunophenotyping features of MCN-L in a 52-year-old woman who came to the surgical department with complaints of pain abdomen for 1.5 months. The ultrasonography showed a septated cyst located centrally in the liver. The CT abdomen showed a large hypodense multiloculated cystic lesion in the liver with thin enhancing septae and multiple small cysts involving the entire left lobe with extension into the right lobe and having a large infra-hepatic exophytic component. A provisional diagnosis of a Hydatid cyst was made. Drainage of the cyst with laparoscopic deroofing was done. Histopathology examination showed a cyst lined by cuboidal to columnar epithelium with mucinous cytoplasm and basally oriented nuclei. Subepithelium shows ovarian-like stroma. MCN-L has a lower frequency as compared to the counterpart of MCN in the pancreas, and further molecular studies are necessary to clarify the biology of MCN-L. The relevance of this rare entity's clinical and radiological suspicion, histological recognition and immunohistochemistry is emphasised.

MYCOLOGICAL ANALYSIS OF CYSTIC CONTENT IN PATIENTS WITH POLYMICROCYSTIC CHANGES OF THE MAXILLARY SINUS MUCOSA

The prevalence of mycotic infections of the paranasal sinuses is constantly increasing worldwide. Pathogens can enter the sinuses through the respiratory tract that leads to the colonization of microorganisms in both the lungs and paranasal sinuses as part of the normal microflora. The presence of filamentous fungi in the maxillary sinus may result from endodontic treatment of molars directly adjacent to the sinus, increasing the risk of fungal foci development. Fungal paranasal sinusitis manifests in invasive or non-invasive forms. While mycological culture remains the traditional gold standard for mycosis diagnosis, its sensitivity is reportedly low. Paranasal sinus cysts comprise 4-8% of general ENT pathologies and approximately 4-16% of chronic paranasal sinus diseases, with a predominant presence in the maxillary sinus. Factors that contribute to the development of sinusitis and create favorable conditions for fungal spore adhesion and growth include impaired ventilation and transport function of the sinus mucosa, inadequate use of broad-spectrum antibacterial drugs and steroid therapy, as well as household factors. Recently, there has been an increase in the number of cases of multiple small cysts in maxillary sinuses. Clinical manifestations range from asymptomatic incidental findings to varying degrees of discomfort and/or pain localized in the maxillary sinus area. The aim of this study is to identify the involvement of a fungal agent and to develop a set of therapeutic and prophylactic measures for polymicrocystic changes in the mucous membrane of the maxillary sinuses. The study enrolled 37 patients aged 18 to 65 years. Atypical cystic content was collected and cultured in Sabouraud's nutrient broth and Amies transport medium at 35-37°C for 5-7 days, with final measurements on day 21. Treatment involved a 14-day regimen of itraconazole (100 mg once daily) alongside topical miramistin (0.01% solution, 2 ml) sinus injections. The findings obtained demonstrate the following: positive fungal growth was observed in 26 cases (70.3%), predominantly Penicillium (48.6%) and Aspergillus (21.6%). Absence of growth was noted in 11 cases (29.7%). Follow-up examinations in 6 months and in a year revealed no crusts in the nasal cavity, moderate mucous discharge in the sinuses, and no polymicrocystic changes. Conclusion. The analysis has demonstrated the involvement of fungal flora in the development and recurrence of cystic changes in the maxillary sinus mucosa, advocating for mycological examination alongside bacteriological analysis. Itraconazole for systemic therapy and miramistin for topical therapy demonstrate efficacy in treatment and recurrence prevention.

Polycystic Ovarian Syndrome: An Overview with Special Consideration to Its Oral and Pediatric Clinical Manifestations

Polycystic ovary syndrome (PCOS) is a complex endocrine disorder affecting women of reproductive age. It is characterized by a combination of symptoms including irregular menstrual cycles, excessive androgen production, and the presence of multiple small cysts on the ovaries. This condition affects approximately 5-10% of women worldwide and is considered one of the most common endocrine disorders in women. The exact cause of PCOS is still unclear, but it is believed to involve a combination of genetic and environmental factors. Insulin resistance and hormonal imbalances play a significant role in the development of this syndrome. High levels of insulin can stimulate the ovaries to produce excess androgens, leading to hormonal dysregulation and subsequent complications such as hirsutism (excessive hair growth), acne, and male pattern baldness. PCOS is associated with a wide range of health problems, ranging from infertility and pregnancy complications to an increased risk of metabolic disorders such as type 2 diabetes and cardiovascular diseases. Furthermore, women with PCOS often experience psychological distress, as the visible symptoms, such as weight gain and fertility issues, can significantly impact their self-esteem and quality of life. Early diagnosis is crucial to prevent long-term complications and provide appropriate management. This review highlights an overview of PCOS, its clinical features, diagnosis, and treatment strategies involved.

Langerhans cell histiocytosis of the thyroid mimicking thyroiditis in a boy: a case report and literature review

BackgroundLangerhans cell histiocytosis affecting the thyroid commonly presents with nonspecific clinical and radiological manifestations. Thyroid Langerhans cell histiocytosis is typically characterized by non-enhancing hypodense lesions with an enlarged thyroid on computed tomography medical images. Thyroid involvement in LCH is uncommon and typically encountered in adults, as is salivary gland involvement. Therefore, we present a unique pediatric case featuring simultaneous salivary and thyroid involvement in LCH.Case presentationA 3-year-old boy with complaints of an anterior neck mass persisting for 1 to 2 months, accompanied by mild pain, dysphagia, and hoarseness. A physical examination revealed a 2.5 cm firm and tender mass in the left anterior neck. Laboratory examinations revealed normal thyroid function test levels. Ultrasonography revealed multiple heterogeneous hypoechoic nodules with unclear and irregular margins in both lobes of the thyroid. Contrast-enhanced neck computed tomography revealed an enlarged thyroid gland and bilateral submandibular glands with non-enhancing hypointense nodular lesions, and multiple confluent thin-walled small (< 1.5 cm) cysts scattered bilaterally in the lungs. Subsequently, a left thyroid excisional biopsy was performed, leading to a histopathological diagnosis of LCH. Immunohistochemical analysis of the specimen demonstrated diffuse positivity for S-100, CD1a, and Langerin and focal positivity for CD68. The patient received standard therapy with vinblastine and steroid, and showed disease regression during regular follow-up of neck ultrasonography.ConclusionsInvolvement of the thyroid and submandibular gland as initial diagnosis of Langerhans cell histiocytosis is extremely rare. It is important to investigate the involvement of affected systems. A comprehensive survey and biopsy are required to establish a definitive diagnosis.

Beyond BI-RADS: Nonmass Abnormalities on Breast Ultrasound.

Abnormalities on breast ultrasound (US) images which do not meet the criteria for masses are referred to as nonmass lesions. These features and outcomes have been investigated in several studies conducted by Asian researchers. However, the term "nonmass" is not included in the American College of Radiology (ACR) Breast Imaging Reporting and Data System (BI-RADS) 5th edition for US. According to the Japan Association of Breast and Thyroid Sonology guidelines, breast lesions are divided into mass and nonmass. US findings of nonmass abnormalities are classified into five subtypes: abnormalities of the ducts, hypoechoic areas in the mammary glands, architectural distortion, multiple small cysts, and echogenic foci without a hypoechoic area. These findings can be benign or malignant; however, focal or segmental distributions and presence of calcifications suggest malignancy. Intraductal, invasive ductal, and lobular carcinomas can present as nonmass abnormalities. For the nonmass concept to be included in the next BI-RADS and be widely accepted in clinical practice, standardized terminologies, an interpretation algorithm, and outcome-based evidence are required for both screening and diagnostic US.

RISK OF HYPERTENSION WITH MULTICYSTIC KIDNEY DISEASE : A SYSTEMATIC REVIEW

Background: Multicystic dysplastic kidney (MCDK) is a congenital cystic kidney disease that can be incidentally seen during the antenatal ultrasound. The condition is most commonly asymptomatic. The clinical presentation is usually characterized by multiple small cysts or a single dominating cyst in the fetal kidney depending on the type of MCDK. Most cases undergo spontaneous involution, and complications like hypertension, infection, and malignancy are rare. Methods: By comparing itself to the standards set by the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) 2020, this study was able to show that it met all of the requirements. So, the experts were able to make sure that the study was as up-to-date as it was possible to be. For this search approach, publications that came out between 2013 and 2023 were taken into account. Several different online reference sources, like Pubmed and SagePub, were used to do this. It was decided not to take into account review pieces, works that had already been published, or works that were only half done. Result: In the PubMed database, the results of our search brought up 563 articles, whereas the results of our search on SagePub brought up 5 articles. The results of the search conducted for the last year of 2013 yielded a total 6 articles for PubMed and 3 articles for SagePub. In the end, we compiled a total of 5 papers, 2 of which came from PubMed and 1 of which came from SagePub. We included five research that met the criteria. Conclusion: Although the risk of hypertension in MCKD is low, the results of this study do not allow firm recommendations on the frequency and duration of blood pressure measurement follow up for these children. Large prospective cohort studies with a very long duration of follow up are needed.

SAT086 Early-onset HNF1B-MODY With Persistent Insulin Requirement In A 2-year-old Female

Abstract Disclosure: F. Ighalo: None. S.R. Kelly: None. M. Tosur: None. M.E. Craven: None. Introduction: Diabetes related to HNF1B pathogenic variants or deletions (maturity-onset diabetes of the young 5 [MODY 5]) typically presents in adolescence or early adulthood, with a median age of 20 years. Bustamante et al. previously described two patients with 17q12 deletion encompassing HNF1B presenting with diabetes and pre-diabetes before the age of 3, who were managed with dietary intervention alone without insulin requirement. This case describes a 2-year-old diagnosed with HNF1B-MODY after developing hyperglycemia in the setting of an acute illness, who continued to require insulin therapy following recovery. Clinical Case: A 2-year-old female with a history of recurrent viral infections and failure to thrive was admitted to the intensive care unit in hypovolemic shock due to hemolytic uremic syndrome due to Shiga toxin. On hospital day 7, while on supraphysiologic steroid doses, she developed hyperglycemia with glucoses above 600 mg/dL. She required an insulin drip to maintain blood glucoses between 100 and 200 mg/dL. Her four-month hospital stay was complicated by cardiogenic shock requiring extracorporeal mechanical oxygenation, renal failure requiring hemodialysis, respiratory failure resulting in a tracheostomy, and dysphagia resulting in a gastrostomy tube. During this time, she continued to require insulin despite being weaned off steroids and transitioning from total parenteral nutrition to gastrostomy tube feeds. Her hemoglobin A1c one month post-diagnosis was 4.9%, and continued to rise to 6.6% and 7.6% at 3 and 4 months post-diagnosis, respectively. Testing for islet autoantibodies was negative. A chromosomal microarray analysis revealed a micro-deletion in chromosome 17q12 that included the HNF1B gene. MRI showed a near total absent pancreas, and multiple small cysts scattered throughout both kidneys. She also had fecal elastase levels in the insufficient range (135 mcg/g, normal &amp;gt;200 mcg/g). Six weeks after the start of insulin drip, she was transitioned from an insulin drip to subcutaneous insulin injections, and eventually placed on an insulin pump prior to discharge. With continued clinical improvement, her insulin doses decreased, but she was not able to come off insulin completely. She had gradual decline in her insulin requirements from 1.0 units/kg/day down to 0.1 units/kg/day at time of discharge. Her current insulin requirement at 6 months post-diagnosis is 0.2 units/kg/day. Conclusion: This case represents the rare development of diabetes before adolescence in HNF1B-MODY, and one of the most severe presentations seen in a prepubertal child requiring persistent insulin therapy at 6 months post-diagnosis. Potential contribution of complicated hospital course on persistent insulin requirement in the setting of 17q12 micro-deletion cannot be excluded. Presentation: Saturday, June 17, 2023

Effect of Letrozole and Clomiphene Citrate versus Letrozole Alone for Ovulation Induction in Women with Polycystic Ovary Syndrome

Background: Polycystic ovary syndrome (PCOS) is a common endocrinopathy characterized by oligo-ovulation or anovulation signs of androgen excess and multiple small ovarian cysts. It is thought to be one of the leading causes of female sub-fertility. It has been estimated that PCOS affects 5-10% of females in reproductive age. In this study, we used combination of letrozole and clomiphene vs letrozole alone in ovulation induction in infertile PCOS women. Objective: The objective of the study was to compare the effectiveness of the combination of letrozole and clomiphene citrate and letrozole alone in ovulation induction in infertile women with PCOS. Methods: Randomized controlled trial conducted in the Department of Reproductive Endocrinology and Infertility, Bangabandhu Sheikh Mujib Medical University, Dhaka, from July 2020 to June 2021. A total of 50 women 18-40 years of age with a diagnosis of infertility and PCOS as defined by the Rotterdam criteria and no other known cause of infertility were included in this study. Participants were randomly assigned into two groups by using a computer-generated random table to either 5 mg letrozole alone or the combination of 2.5 mg letrozole and 50 mg CC daily on cycle days 3-7 for two treatment cycle. Statistical analysis was performed by the SPSS program for Windows, version 22.0. Main outcome measured by number &amp; size of mature follicles, endometrial thickness, day 21 serum progesterone &amp; ovulation rate in both groups. The secondary outcome includes pregnancy rate and complication of both treatment arms. Results: Regarding demographic criteria, there was no significant difference between two groups. In the current study, clinical characteristics, laboratory parameters and cycle characteristics were also comparable to both groups and showed no significant difference. Dominant follicles were found 24(52.2%) in group A (CC+Letrozole) and 29(63.0%) in group B (Letrozole Alone) without any significant difference. Mono follicular development was found 18(75.0%) in group A and 24(82.8%) in group B which was not statistically significant. Endometrial thickness and serum progesterone level at 1st and 2nd cycle were not statistically significant between two groups (p&gt;0.05). Ovulation rate was higher in group B than group A (76.0% vs 72.0%) with absolute difference 4.0 and relative ratio 0.95. Pregnancy rate was also higher in group B than group A (24.0% vs 16.0%) with absolute difference 8.0 and relative ratio 0.67. Ovulation and pregnancy rate were not statistically significant between two groups. Regarding side effects, no significant difference was found between two groups. Conclusion: Although it was not statistically significant, ovulation and pregnancy rate was a little higher in alone letrozole group than combined group. The results of this preliminary study suggested that letrozole may have a better role as a first-line treatment for anovulatory patients with PCOS.

"Oncocytoid Renal Cell Carcinomas After Neuroblastoma" Represent TSC -mutated Eosinophilic Solid and Cystic Renal Cell Carcinomas : Association With Prior Childhood Malignancy and Multifocality With Therapeutic Implications.

The concept of oncocytoid renal cell carcinoma in patients who have survived neuroblastoma as a distinct biologic entity has been controversial since its original description in 1999. This is in part because similar oncocytoid renal cell carcinomas have been described in association with other pediatric cancers, and also because other renal cell carcinoma subtypes (such as MiT family translocation renal cell carcinoma) have been described in children who have survived neuroblastoma. We identified an index case of a child who survived medulloblastoma and developed multifocal bilateral oncocytoid renal cell carcinomas with morphology and immunophenotype compatible with eosinophilic solid and cystic renal cell carcinoma (ESC RCC) and demonstrated that both neoplasms harbored distinctive mutations in the TSC1/TSC2 genes. Remarkably, the child's remaining bilateral multifocal renal neoplasms completely responded to MTOR inhibitor therapy without need for further surgery. To confirm our hypothesis that oncocytoid renal cell carcinomas after childhood cancer represent ESC RCC, we obtained formalin-fixed paraffin-embedded tissue blocks from 2 previously published cases of oncocytoid renal cell carcinoma after neuroblastoma, confirmed that the morphology and immunophenotype was consistent with ESC RCC, and demonstrated that both cases harbored somatic TSC gene mutations. Both expressed markers previously associated with neoplasms harboring TSC gene mutations, glycoprotein nonmetastatic B, and cathepsin K. Of note, one of these patients had 2 ESC RCC which harbored distinctive TSC2 mutations, while the background kidney of the other patient had multiple small cysts lined by similar oncocytoid cells which showed loss of TSC2 protein. We then reviewed 3 of 4 cases from the original 1999 report of oncocytoid renal cell carcinomas after neuroblastoma, found that all 3 demonstrated morphology (including basophilic cytoplasmic stippling) that is characteristic of ESC RCC, showed that all 3 overexpressed glycoprotein nonmetastatic B, and showed that both cases with adequate material demonstrated loss of TSC2 protein and expressed cytokeratin 20 and cathepsin K by immunohistochemistry. In summary, "oncocytoid renal cell carcinomas after neuroblastoma" represent ESC RCC which are often multifocal in patients who have survived childhood cancer, likely representing an incompletely characterized tumor predisposition syndrome. MTOR-targeted therapy represents an effective therapeutic option for such patients to preserve functional nephrons.

Managing PCOS with Samshodhan Therapy: A Case Report

Background: Polycystic Ovarian Syndrome is one of the most common metabolic and reproductive disorder among women of reproductive age. This condition is characterized by elevated androgen levels, anovulatory menstrual abnormalities and multiple small cysts in ovaries. This female endocrine disorder is prevalent in age group of 18-45 years with rate ranging from 2.2% to 26%. Sedentary life style, faulty dietary habits lead to obesity in young adults which results in metabolic and hormonal disturbances such as insulin resistance, hyperandrogenemia etc. In Ayurvedic literature, the features associated with PCOS closely resemble with Nasta Artava, Ksheen Artava and a group of Yoni Vyapada like Bandhya, Arajska, Lohitkshara and Artava Vaha Strotas Viddh Lakshana mentioned by Acharya Sushruta. PCOS also has close resemblance with Pushpghani Jatharini mentioned by Acharya Kashyapa. Symptoms of PCOS mimic with conditions like Sthoulya, Aratava Kshaya, Agni Mandya, Rrakta/Medo Dhatu Dushti, Granthi Roga. So, based upon Rog lakshana and dosha dushya involvement, Ayurveda explains Nidana parivarjanam and Shamshodhan as a prime line of treatment for PCOS. Aim &amp; Objectives: The main aim of this study is to establish the role of Samshodhan therapy in PCOS. PCOS can be considered as Tridoshaj Vyadhi with Vata &amp; Kapha predominance that is the reason Vamana for Kapha Shodhan and Virechana for Vata and Pitta Sanshuddhi were planned to achieve desired results. Results: In this case study, a 27 years female patient suffering from symptoms like Ksheen Artava, Granthibhoot artava, Sthoulya, was managed with Samshodhan therapy and got complete relief from symptoms. Conclusion: This case study corroborates the importance of Samshodhan Chikitsa in PCOS.

Cystic dystrophy in heterotopic pancreas.

Cystic dystrophy in heterotopic pancreas, or paraduodenal pancreatitis, is a rare and complicated presentation involving heterotopic pancreatic tissue in the duodenal wall. This condition is present in 5% of the general population but disease mainly affects middle-aged alcoholic-smoking men with chronic pancreatitis (CP). It may be purely duodenal or segmental (pancreatico-duodenopathy). Its pathophysiology arises from alcohol toxicity with obstruction of small ducts of heterotopic pancreatic tissue present in the duodenal wall and the pancreatic-duodenal sulcus, leading to repeated episodes of pancreatitis. The symptomatology includes episodes of acute pancreatitis, weight loss, and vomiting due to duodenal obstruction. Imaging shows thickening of the wall of the second portion of the duodenum with multiple small cysts. A stepwise therapeutic approach is preferred. Conservative medical treatment is favored in first intention (analgesics, continuous enteral feeding, somatostatin analogues), which allows complete symptomatic regression in 57% of cases associated with a 5% rate of complications (arterial thrombosis and diabetes). Endoscopic treatment may also be associated with conservative measures. Surgery achieves a complete regression of symptoms in 79% of cases but with a 20% rate of complications. Surgery is indicated in case of therapeutic failure or in case of doubt about a malignant tumor. Pancreaticoduodenectomy and duodenal resection with pancreatic preservation (PPDR) seem to be the most effective treatments. PPDR has also been proposed as a first-line treatment for purely duodenal location of paraduodenal pancreatitis, thereby preventing progression to an extended segmental form.

Role of Pathyapathya Kalpana in Polycystic Ovary Syndrome

Background : Polycystic Ovary Syndrome (PCOS) is one of the most prevalent endocrine disorders among females. It includes oligo/amenorrhoea, hirsutism, obesity and enlarged ovaries with multiple small cysts and thickened tunica. It is caused mainly due to Apathya Aahara (unwholesome diet) and Apathya Vihara (unwholesome lifestyle). Ayurveda states that even the best medicines will not be useful if Pathya Aahara (wholesome diet) is not consumed. Objective: The objective of the present study was to explore the role of Pathya Aahara in the management of PCOS. Methodology: It was a literary study. The available literature has been reviewed from ayurvedic texts, modern medicine, and research journals regarding Pathya Aahara with special reference to PCOS. The references were critically analysed to reach to conclusion. Results: Acharya Charaka mentioned Pathya as that which does not adversely affect the Patha (channels) related to body and mind; while Apathya is opposite of that. PCOS involves aggravation of Kapha and Vata Dosha along with Pitta Dosha. Being one of the Santarpanottha Vyadhi, Rasavaha, Medovaha and Artavavaha Srotas are involved in it. Laghu, Ruksha, Ushana Gunatmaka, Katu – Tikta - Kashaya Rasatmaka Aahara according to dietetic principles mentioned in Ayurveda should be advised along with Shodhana and Shamana Chikitsa in PCOS. Conclusion: Use of Pathya Aahara according to Ayurvedic principles plays significant role in PCOS management.

Prevalence of polycystic ovarian disease among undergraduate medical students

Background: Poly cystic ovarian syndrome is a common endocrinopathy identified by oligo-ovulation or ano-ovulation, sign of androgen excess and multiple small ovarian cysts. The present study was conducted to find out the prevalence of poly cystic ovarian disease among undergraduate female medical students of RMCH &amp; RC, Hapur. Materials &amp; Methods: 185 undergraduate female medical students of RMCH &amp; RC, Hapur were selected and all students were provided with a questionnaire comprising of average menstrual cycle during menstruating years, tendency to grow dark, coarse hair on body, obese or overweight between age 16-40 years, sleep cycle and duration of sleep, late night person, dietary habit, family history of irregular cycle, a milky discharge from nipples (not including during pregnancy or childbirth) between the age of 16-40 years was recorded. Results: Out of 185 students, PCOS was seen in 6 (3.24%). 159 had no tendency to grow dark, coarse hair, 6 had on belly, 6 on breast, 9 on upper lip, 1 on back and 4 on chest between breast. 38 were overweight and 147 were normal, 55 were obese or overweight between age 16-40 years and 130 were not.

Monoallelic pathogenic ALG5 variants cause atypical polycystic kidney disease and interstitial fibrosis

Disorders of the autosomal dominant polycystic kidney disease (ADPKD) spectrum are characterized by the development of kidney cysts and progressive kidney function decline. PKD1 and PKD2, encoding polycystin (PC)1 and 2, are the two major genes associated with ADPKD; other genes include IFT140, GANAB, DNAJB11, and ALG9. Genetic testing remains inconclusive in ∼7% of the families. We performed whole-exome sequencing in a large multiplex genetically unresolved (GUR) family affected by ADPKD-like symptoms and identified a monoallelic frameshift variant (c.703_704delCA) in ALG5. ALG5 encodes an endoplasmic-reticulum-resident enzyme required for addition of glucose molecules to the assembling N-glycan precursors. To identify additional families, we screened a cohort of 1,213 families with ADPKD-like and/or autosomal-dominant tubulointerstitial kidney diseases (ADTKD), GUR (n= 137) or naive to genetic testing (n= 1,076), by targeted massively parallel sequencing, and we accessed Genomics England 100,000 Genomes Project data. Four additional families with pathogenic variants in ALG5 were identified. Clinical presentation was consistent in the 23 affected members, with non-enlarged cystic kidneys and few or no liver cysts; 8 subjects reached end-stage kidney disease from 62 to 91 years of age. We demonstrate that ALG5 haploinsufficiency is sufficient to alter the synthesis of the N-glycan chain in renal epithelial cells. We also show that ALG5 is required for PC1 maturation and membrane and ciliary localization and that heterozygous loss of ALG5 affects PC1 maturation. Overall, our results indicate that monoallelic variants of ALG5 lead to a disorder of the ADPKD-spectrum characterized by multiple small kidney cysts, progressive interstitial fibrosis, and kidney function decline.

A Term Neonate with Multiorgan Dysfunction, Severe Metabolic Acidosis, and Hyperkalemia.

A Term Neonate with Multiorgan Dysfunction, Severe Metabolic Acidosis, and Hyperkalemia.

Laparoscopic management of hydatid cyst of liver

Background: Hydatic disease is caused by a parasite named Ecchinococcus granulosus. This disease is endemic in Pakistan. The disease mostly affects liver forming cysts. The treatment is mainly surgical which can be performed laparoscopically. This study was thus designed to assess the outcomes of the laparoscopic treatment of hydatid cyst of liver in terms of recovery of patient, complications and recurrence.Subjects and methods: This case series was conducted from March 2015 to December 2020 in Surgical Department of Services Institute of Medical Sciences Lahore. Patients who were planned for laparoscopic surgery for hepatic hydatid cysts (CE2, CE3) were included in the study. Patients who have past history of hepatobiliary surgery, with multiple small cysts or those who has refused for laparoscopic surgery were excluded from study. Demographic data, presenting symptoms, clinicopatholigical findings, duration of surgery, hospital stay and complications were recorded on pre-designed Performa. Results: Total of 42 patients were managed laparoscopically for hydatid liver cyst. The mean age of the patients was 39.42 + 12.04 years. There were 20 (47.62%) male and 22 (52.38%) female patients. Thirty patients (71.43%) were from rural areas. Twenty patients (47.62%) had history of close contacts with dogs or domestic animals. Patients mostly presented with symptoms of pain in right hypochondrium and abdominal mass. Majority of the patients had single hydatid cyst in the liver. Right lobe of the liver was more frequently involved than left lobe. Both lobes were involved in 2 (4.8%) patients. The mean duration of surgery was 76 minutes. No patient developed intra or postoperative anaphylactic shock. Biliary leakage was seen in 4 (9.5%), port site infection in 2 (4.8%) patients and conversion to laparotomy was done in 2 patients (4.8%). The mean hospital stay of patients was 3.41+ 2.82 days. There was no mortality of the patients or recurrence of cyst on 12 week follow up.Conclusion: Laparoscopic surgery for hepatic hydatid cyst is safe and effectual treatment in carefully selected patients. It is a simple technique with lesser postoperative morbidity and potentially decreased risk of complications especially recurrence of disease.

Twin Reversed Arterial Perfusion Sequence.

Twin Reversed Arterial Perfusion Sequence.

Síndrome de Caroli em um cão

ABSTRACT: Caroli syndrome is characterized by a combination of intrahepatic biliary ductal ectasia and congenital ductal fibrosis due to the failure of involution of ductal plates and large intrahepatic ducts. This study aims to report Caroli syndrome in a dog, with emphasis on clinical characteristics, complementary examinations, and diagnostic approach. A 1-year-old mixed-breed intact male dog was presented with a 7-day history of acute vomiting and anorexia. Clinical evaluation revealed severe jaundice, abdominal pain, dehydration, lethargy, and abdominal distension. Ultrasonography revealed hepatic cysts filled with fluid. Exploratory laparotomy was performed to clarify the imaging findings; however, the dog died shortly after surgery. Postmortem examination revealed that the liver was reduced in size and had multiple interconnected and distended saccular dilations. The bile ducts were markedly dilated and replaced a large part of the liver parenchyma. Multiple small cysts were also observed in the kidneys. Microscopically, the hepatic lesions were characterized by extensive bridging fibrosis associated with proliferating and ectatic bile ducts contiguous with the biliary tree. Marked fibrosis and small cysts were observed in the kidneys. Therefore, a final diagnosis of Caroli syndrome was made. While uncommon, this syndrome should be considered as a differential diagnosis for young dogs with biliary cysts and hepatic fibrosis.

A Case Study on Ayurvedic Management in PCOS

Female reproductive system consists of hypothalamo-pituitary-ovarian axis and intact uterine-adnexa which maintains a complex mechanism. In human body all the systems are interrelated to function properly; any imbalance in one system may cause multisystem pathogenesis. Polycystic ovarian syndrome (PCOS) is a common endocrinopathy, which is multifactorial and polygenic condition, manifested as oligoovulation or anovulation, signs of hyperandrogenism and multiple small ovarian cysts. Signs and symptoms vary within individual’s overtime. This adversely affect the reproductive system by menstrual disorders, infertility, obesity, depression, sleep apnea, insulin resistance and in due course may result in diabetes mellitus, endometrial cancer, cardiovascular disease etc. According to Ayurveda PCOS is a disorder which involves the three Doshas, Dhathus like Rasa, Raktha and Medas. The Srothas involved in this condition are Rasa, Rakta and Arthava vaha which eventually manifests features such as Anarthava (amenorrhea), Vandhyathwa, Pushpagni, Abeeja rtuchakra (anovular bleeding). Here is a case report of 19 year old girl who presented with irregular menstruation, rapid weight gain and hair loss. On USG she was detected to have bilateral PCO pattern. Based on the clinical features, treatment principles adopted were Aamapachana, Vata anulomana, Kaphapittahara and Arthava janana. After 2 months of internal medications, symptoms reduced markedly and menstruation was normal. Adherence to Ayurvedic principles is found to be helpful in PCOS for a healthy and fruitful life.

S2679 An Unusual Suspect for Cholestatic Liver Injury

Introduction: Vanishing Bile Duct Syndrome (VBDS) and idiopathic cholestasis (IC) have been associated with lymphoma in rare cases. In VBDS, the defining feature on liver biopsy is ductopenia, while in IC there is canalicular cholestasis and mixed inflammatory changes. Existing literature reports a few cases of paraneoplastic cholestasis with pathological features of either VBDS or IC. We describe an unique case of severe cholestasis as the presenting sign of Hodgkin’s lymphoma in the absence of bile duct loss or inflammatory changes. Case Description/Methods: 60-year-old man with no past medical history presented to the ED with increasing fatigue and jaundice that started five months earlier. He underwent cholecystectomy and intraoperative liver biopsy four months earlier which showed bland lobular cholestasis and mild lobular injury with no fibrosis, inflammatory changes, abnormal lymphocytic infiltration, or evidence of bile duct loss. An MRI/MRCP 6 weeks before presentation showed multiple small hepatic cysts and no biliary obstruction or ductal dilatation. Ursodiol 14 mg/kg/d was started 2 weeks later but he developed hypotension and presented to the ED. In the ED, vital signs were normal. Physical exam showed jaundice and hepatomegaly. Labs revealed ALP 1002 U/L, AST 81 U/L, ALT 90 U/L, total bilirubin 10.8 mg/dL, and direct bilirubin 8.1 mg/dL. Further work up showed negative AMA, ANA, ASMA and vasculitis serologies, normal ceruloplasmin, and negative viral studies including hepatitis A, B, C, HSV, CMV, HIV and COVID-19. EBV PCR was 69,697 copies/mL with positive IgG suggestive of reactivation of previous infection. Lyme and Ehrlichia serologies were negative. Repeat MRI/MRCP showed normal bile ducts but extensive retroperitoneal lymphadenopathy. Lymph node core needle biopsy revealed classic Hodgkin’s lymphoma. He was started on chemotherapy and liver enzymes improved. Discussion: Hodgkin’s lymphoma can present with cholestatic liver injury as shown in our patient who was on no medications and had no other identifiable causes of cholestasis. In the existing literature, VBDS and IC have been described as the pathological features for these patients where either ductopenia or canalicular cholestasis and mixed inflammatory changes are seen. Our case is unique because our patient had neither of these features. Bland cholestasis associated with lymphoma should be kept in the differential in someone with otherwise unexplained cholestatic liver injury.Figure 1.: Panel A: High power view of liver biopsy (200x, H&E). Yellow-green bile can be seen in dilated canaliculi. There is no significant lobular inflammation, steatosis, or other alterations. Panel B: High power view of liver biopsy (400x, H&E). Pale yellow-green watery bile can be seen in dilated canaliculi (yellow arrows). There is no significant lobular inflammation, steatosis, or other alterations. Panel C: High power view of lymph node (200x, H&E). The lymph node shows loss of normal architecture with replacement by fibrosis and an atypical lymphohistiocytic infiltrate. Scattered large cells with irregular nuclear contours and hyperchromasia are seen suggestive of Hodgkin cells. Panel D: Immunoperoxidase studies show the Hodgkin Reed-Sternberg (HRS) cells are positive for CD15 and CD30. PAX-5 is weakly expressed in the large cells in comparison to the background small B cells. CD20 stains background small B cells but is negative in the HRS cells. The immunophenotype is consistent with classic Hodgkin lymphoma.