Patterns of kidney diseases diagnosed by kidney biopsy and the impact of the COVID-19 pandemic in Yogyakarta,Indonesia:A single-center study

BACKGROUND Glomerular diseases rank third among the causes of chronic kidney disease worldwide and in Indonesia,and its burden continues to increase,especially regarding the sociodemographic index.Kidney biopsy remains the gold standard for the diagnosis and classification of glomerular diseases.It is crucial for developing treatment plans,determining the degree of histologic changes,and identifying disease relapse.AIM To describe the patterns of biopsy-proven kidney diseases in adult patients.METHODS We retrospectively reviewed the demographic,histopathologic,clinical,and laboratory data of 75 adult patients with biopsy-proven kidney diseases at our institution recorded from 2017 to 2022.RESULTS Among the patients,43(57.3%)were females,and the mean age was 31.52 years±11.70 years.The most common histopathologies were lupus nephritis(LN)(33.3%),minimal change disease(MCD)(26.7%),and focal segmental glomerulosclerosis(10.7%).LN(41.7%)was frequently diagnosed in women and MCD(28.1%)in men.The most common cause of nephritic syndrome was LN(36.7%)and of nephrotic syndrome was MCD(40%).CONCLUSION Different kidney disease patterns were observed in different sexes,age categories,clinical syndromes,and biopsy dates relative to the coronavirus disease 2019 pandemic.

中国肾脏病界第一本国际英文杂志《Kidney Diseases》出版发行

由中国工程院院士,中华肾脏病学会前任主任委员,国际肾脏病学会常务理事刘志红教授担任主编,全球著名肾脏病专家参与的《Kidney Diseases》已于2015年5月由Karger出版社正式出版发行。近年来中国肾脏病学在临床研究和基础研究方面发展迅速,并开始在国际学术界产生影响和发挥作用。为了给中国肾脏病学者创造一个展示水平,加强与国际学术界交流对话的平台,我们有必要拥有一本国际化的学术期刊。《Kidney Diseases》正是在这一背景下应运而生。它的问世引起了国际学术界的广泛关注和好评,并已被PubMed收录。

Gut microbiota and diabetic kidney diseases: Pathogenesis and therapeutic perspectives

Diabetic kidney disease(DKD)is one of the major chronic complications of diabetes mellitus(DM),as well as a main cause of end-stage renal disease.Over the last few years,substantial research studies have revealed a contributory role of gut microbiota in the process of DM and DKD.Metabolites of gut microbiota like lipopolysaccharide,short-chain fatty acids,and trimethylamine N-oxide are key mediators of microbial–host crosstalk.However,the underlying mechanisms of how gut microbiota influences the onset and progression of DKD are relatively unknown.Besides,strategies to remodel the composition of gut microbiota or to reduce the metabolites of microbiota have been found recently,representing a new potential remedial target for DKD.In this minireview,we will address the possible contribution of the gut microbiota in the pathogenesis of DKD and its role as a therapeutic target.

Matrix metalloproteinases contribute to kidney fibrosis in chronic kidney diseases

Matrix metalloproteinases(MMPs) are members of the neutral proteinase family. They were previously thought to be anti-fibrotic because of their ability to degrade and remodel of extracellular matrix. However, recent studies have shown that MMPs are implicated in initiation and progression of kidney fibrosis through tubular cell epithelial–mesenchymal transition(EMT) as well as activation of resident fibroblasts, endothelial-mesenchymal transition(Endo MT) and pericyte-myofibroblast transdifferentiation. Interstitial macrophage infiltration has also been shown to correlate with the severity of kidney fibrosis in various chronic kidney diseases. MMPs secreted by macrophages, especially MMP-9, hasbeen shown by us to be profibrotic by induction of tubular cells EMT. EMT is mainly induced by transforming growth factor-β(TGF-β). However, MMP-9 was found by us and others to be up-regulated by TGF-β1 in kidney tubular epithelial cells and secreted by activated macrophages, resulting in EMT and ultimately kidney fibrosis. Therefore, MMP-9 may serve as a potential therapeutic target to prevent kidney fibrosis in chronic kidney disease. This review, by a particular focus on EMT, seeks to provide a comprehensive understanding of MMPs, especially MMP-9, in kidney fibrosis.

Current advances of stem cell-based therapy for kidney diseases

Kidney diseases are a prevalent health problem around the world.Multidrug therapy used in the current routine treatment for kidney diseases can only delay disease progression.None of these drugs or treatments can reverse the progression to an end-stage of the disease.Therefore,it is crucial to explore novel therapeutics to improve patients’quality of life and possibly cure,reverse,or alleviate the kidney disease.Stem cells have promising potentials as a form of regenerative medicine for kidney diseases due to their unlimited replication and their ability to differentiate into kidney cells in vitro.Mounting evidences from the administration of stem cells in an experimental kidney disease model suggested that stem cell-based therapy has therapeutic or renoprotective effects to attenuate kidney damage while improving the function and structure of both glomerular and tubular compartments.This review summarises the current stem cell-based therapeutic approaches to treat kidney diseases,including the various cell sources,animal models or in vitro studies.The challenges of progressing from proof-of-principle in the laboratory to widespread clinical application and the human clinical trial outcomes reported to date are also highlighted.The success of cell-based therapy could widen the scope of regenerative medicine in the future.

Evaluation of Medical Costs of Kidney Diseases and Risk Factors in Japan

Background: Kidney (renal) diseases and dialysis are among the most costly disorders and represent a worldwide burden. In this study, we evaluate the medical costs for individuals with kidney diseases and risk factors for the diseases in Japan. Data and Methods: The dataset used contained 113,979 medical checkups and 3,172,066 medical cost records obtained from 48,022 individuals in one health insurance society. The sample period was April 2013 to March 2016. We evaluated the distribution of all medical costs, and those of kidney diseases specifically. Then the power transformation Tobit model was used to remove the effects of other variables. Finally, a probit analysis was used to analyze the risk factors. Results: In 0.25% of all cases, individuals were diagnosed with kidney diseases. An individual with kidney disease cost 14.5 times more than those without kidney disease. If the diseases progressed into chronic kidney disease (CKD), the medical costs increased substantially. Even disregarding various characteristics of individuals, this conclusion did not vary. We found important risk factors included diabetes and blood pressure problems. In particular, an individual with both factors had a high probability of developing kidney disease. Conclusion: Kidney diseases are much costlier than other diseases. Screening high-risk individuals, educating patients, and ensuring that treatment begins at an early stage are critically important to controlling medical costs. Limitations: The dataset was observatory, and the sample period was only 3 years.

Multifaceted relationship between diabetes and kidney diseases:Beyond diabete

Diabetes mellitus is one of the most common causes of chronic kidney disease.Kidney involvement in patients with diabetes has a wide spectrum of clinical presentations ranging from asymptomatic to overt proteinuria and kidney failure.The development of kidney disease in diabetes is associated with structural changes in multiple kidney compartments,such as the vascular system and glomeruli.Glomerular alterations include thickening of the glomerular basement membrane,loss of podocytes,and segmental mesangiolysis,which may lead to microaneurysms and the development of pathognomonic Kimmelstiel-Wilson nodules.Beyond lesions directly related to diabetes,awareness of the possible coexistence of nondiabetic kidney disease in patients with diabetes is increasing.These nondiabetic lesions include focal segmental glomerulosclerosis,IgA nephropathy,and other primary or secondary renal disorders.Differential diagnosis of these conditions is crucial in guiding clinical management and therapeutic approaches.However,the relationship between diabetes and the kidney is bidirectional;thus,new-onset diabetes may also occur as a complication of the treatment in patients with renal diseases.Here,we review the complex and multifaceted correlation between diabetes and kidney diseases and discuss clinical presentation and course,differential diagnosis,and therapeutic opportunities offered by novel drugs.

Complement related kidney diseases:Recurrence after transplantation

The recurrence of renal disease after renal transplantation is becoming one of the main causes of graft loss afterkidney transplantation. This principally concerns some of the original diseases as the atypical hemolytic uremic syndrome(HUS), the membranoproliferative glomerulonephritis(MPGN), in particular the MPGN now called C3 glomerulopathy. Both this groups of renal diseases are characterized by congenital(genetic) or acquired(autoantibodies) modifications of the alternative pathway of complement. These abnormalities often remain after transplantation because they are constitutional and poorly influenced by the immunosuppression. This fact justifies the high recurrence rate of these diseases. Early diagnosis of recurrence is essential for an optimal therapeutically approach, whenever possible. Patients affected by end stage renal disease due to C3 glomerulopathies or to atypical HUS, may be transplanted with extreme caution. Living donor donation from relatives is not recommended because members of the same family may be affected by the same gene mutation. Different therapeutically approaches have been attempted either for recurrence prevention and treatment. The most promising approach is represented by complement inhibitors. Eculizumab, a monoclonal antibody against C5 convertase is the most promising drug, even if to date is not known how long the therapy should be continued and which are the best dosing. These facts face the high costs of the treatment. Eculizumab resistant patients have been described. They could benefit by a C3 convertase inhibitor, but this class of drugs is by now the object of randomized controlled trials.

Saudi Consensus on the Usage of Sodium-Glucose Cotransporter-2 Inhibitors on the Management of Chronic Kidney Diseases

According to recent epidemiological data, chronic kidney diseases (CKDs) affect approximately 10% of the global population. Like many countries, CKD is a significant public health issue in Saudi Arabia. The prevalence of CKD in Saudi Arabia is estimated to be around 4.5% of the adult population, with a higher prevalence in older age groups. Sodium-glucose cotransporter-2 inhibitors (SGLT2is) are a class of oral medications used to treat type 2 diabetes mellitus (T2DM). In addition to their glucose-lowering effects, SGLT2i have been shown to have beneficial effects on kidney function in patients with or without T2DM. Therefore, a Saudi task force gathered to develop an explicit, evidence-based consensus on SGLT2i use in CKD Saudi patients. A panel of 14 experts made up a task force. An initial concept proposal was obtained. The proposal was divided into several topics discussed on 24 May 2023. A literature review was carried out. The literature search was completed on 3rd June 2023. A drafted report was distributed to the entire panel. Approval of the recommendations required consensus, defined as a majority approval (i.e. above 75%). The recommendations were revised to accommodate any differences of opinion until a consensus was reached. Recommendations were finally formulated on 21st June 2023. Subsequently, the panel reviewed and discussed the supporting rationale of the revised recommendations. This article presents these practical recommendations.

Breast adenoid cystic carcinoma arising in microglandular adenosis:A case report and review of literature

BACKGROUND Breast adenoid cystic carcinoma(AdCC)is a rare invasive carcinoma composed of epithelial and myoepithelial cells.Microglandular adenosis(MGA)is a rare benign proliferative lesion consisting of small,uniform,and round glands formed by a single layer of epithelial cells and basement membrane without a myoepithelial cell layer.MGA may progress to atypical MGA and carcinoma arising in MGA.Among various invasive carcinomas from MGA,AdCC has been rarely reported.Here,we report a case of AdCC arising in MGA.CASE SUMMARY A 59-year-old woman was diagnosed with a newly developed density on a routine mammogram.The density was similar to or slightly lower than that of the breast parenchyma.Sonography showed an irregular mass with a slightly higher echo than that of fat.Magnetic resonance imaging showed an irregular mass with a similar T1 signal intensity and a slightly higher T2 signal intensity compared to muscles or the breast parenchyma.The lesion showed heterogeneous internal enhancement with an initially slow and delayed persistent enhancing pattern.Microscopically,the tumor was composed of invasive AdCC,in situ AdCC,and MGA.AdCC is composed of basaloid and ductal epithelial cells forming cribriform or solid sheets,or haphazardly scattered small cribriform or tubular glands.MGA showed small glands with a single epithelial lining and retained lumen.S-100 staining was strongly positive in MGA area.The patient underwent breast-conserving surgery with sentinel lymph node biopsy.CONCLUSION Breast AdCC arising in MGA showed unique imaging findings that was different from usual invasive cancer.

Mirizzi syndrome in an anomalous cystic duct:A case report

Mirizzi syndrome is a rare complication of gallstone disease,and results in partial obstruction of the common bile duct or a cholecystobiliary fistula. Moreover,congenital anatomical variants of the cystic duct are common,occurring in 18%-23% of cases,but Mirizzi syndrome underlying an anomalous cystic duct is an important clinical consideration. Here,we present an unusual case of typeⅠMirizzi syndrome with an uncommon anomalous cystic duct,namely,a low lateral insertion of the cystic duct with a common sheath of cystic duct and common bile duct.

Pulmonary cystic disease associated with integumentary and renal manifestations

A 69-year-old man with multiple skin lesions on his face, neck and upper torso, which first appeared in the 3rd decade of his life, was admitted to our hospital. He had cystic changes in his lungs noted on chest computed to- mography （CT） scanning, as well as a left kidney mass. This patient exhibited a rare complex of renal, cutaneous and pulmonary manifestations, eponymously named Birt-Hogg-Dube syndrome, with characteristic skin features （fibrofolliculomas, trichodiscomas and acrochordons）. This syndrome is due to an autosomal dominant germ-line mutation of thefolliculin （FLCN） gene located at chromosome 17p11.2. Diagnosis and differentiation from other disease complexes including the skin, kidneys and lungs are important in prognostication and management of po- tentially life-threatening complications such as renal cell carcinoma and pneumothoraces.

Large-duct pattern invasive adenocarcinoma of the pancreas–a variant mimicking pancreatic cystic neoplasms: A minireview

Pancreatic cancer currently has no subtypes that inform clinical decisions;hence,there exists an opportunity to rearrange the morphological and molecular taxonomy that guides a better understanding of tumor characteristics.Nonetheless,accumulating studies to date have revealed the large-duct type variant,a unique subtype of pancreatic ductal adenocarcinoma(PDA)with cystic features.This subtype often radiographically mimics intraductal papillary mucinous neoplasms(IPMNs)and involves multiple small cysts occasionally associated with solid masses.The“bunch-of-grapes”sign,an imaging characteristic of IPMNs,is absent in large-duct PDA.Large-duct PDA defines the mucin profile,and genetic alterations are useful in distinguishing large-duct PDA from IPMNs.Histologically,neoplastic ducts measure over 0.5 mm,forming large ductal elements.Similar to classic PDAs,this subtype is frequently accompanied by perineural invasion and abundant desmoplastic reactions,and KRAS mutations in codon 12 are nearly ubiquitous.Despite such morphological similarities with IPMNs,the prognosis of large-duct PDA is equivalent to that of classic PDA.Differential diagnosis is therefore essential.

Evaluation of hepatic cystic lesions

Hepatic cysts are increasingly found as a mere coincidence on abdominal imaging techniques, such as ultrasonography (USG), computed tomography (CT) and magnetic resonance imaging (MRI). These cysts often present a diagnostic challenge. Therefore, we performed a review of the recent literature and developed an evidence-based diagnostic algorithm to guide clinicians in characterising these lesions. Simple cysts are the most common cystic liver disease, and diagnosis is based on typical USG characteristics. Serodiagnostic tests and microbubble contrast-enhanced ultrasound (CEUS) are invaluable in differentiating complicated cysts, echinococcosis and cystadenoma/cystadenocarcinoma when USG, CT and MRI show ambiguous findings. Therefore, serodiagnostic tests and CEUS reduce the need for invasive procedures. Polycystic liver disease (PLD) is arbitrarily defined as the presence of > 20 liver cysts and can present as two distinct genetic disorders: autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (PCLD). Although genetic testing for ADPKD and PCLD is possible, it is rarely performed because it does not affect the therapeutic management of PLD. USG screening of the liver and both kidneys combined with extensive family history taking are the cornerstone of diagnostic decision making in PLD. In conclusion, an amalgamation of these recent advances results in a diagnostic algorithm that facilitates evidence-based clinical decision making.

Human cystic echinococcosis:epidemiologic,zoonotic,clinical,diagnostic and therapeutic aspects

This review represents an updated scenario on the transmission cycle,epidemiology,clinical features and pathogenicity,diagnosis and treatment,and prevention and control measures of a cestode parasite Echincoccus granulosus(E.granulosus) infection causing cystic echinococcosis (CE) in humans.Human CE is a serious life-threatening neglected zoonotic disease that occurs in both developing and developed countries,and is recognized as a major public health problem. The life cycle of E.granulosus involves a definitive host(dogs and other canids) for the adult E.granulosus that resides in the intestine,and an intermediate host(sheep and other herbivores) for the tissue-invading metacestode(larval) stage.Humans are only incidentally infected;since the completion of the life cycle of E.granulosus depends on carnivores feeding on herbivores bearing hydatid cysts with viable protoscoleces,humans represent usually the dead end for the parasite.On ingestion of E.granulosus eggs,hydatid cysts are formed mostly in liver and lungs, and occasionally in other organs of human body,which are considered as uncommon sites of localization of hydatid cysts.The diagnosis of extrahepatic echinococcal disease is more accurate today because of the availability of new imaging techniques,and the current treatments include surgery and percutaneous drainage,and chemotherapy(albendazole and mebendazole).But.the wild animals that involve in sylvatic cycle may overlap and interact with the domestic sheepdog cycle,and thus complicating the control efforts.The updated facts and phenomena regarding human and animal CE presented herein are due to the web search of SCI and non-SCI journals.

Epidemiology and clinical features of cystic hydatidosis in Western Sicily:A ten-year review

AIM:To assess retrospectively the epidemiological and clinical aspects of cystic echinococcosis(CE)and to evaluate follow-up and response to treatment in patients affected by CE.METHODS:From January 2000 to December 2010,all patients affected by CE at the Infectious Diseases Units of the University of Catania and of Basilotta Hospital in Nicosia-Enna,were enrolled as participants in the study.Epidemiological,clinical and laboratory data were collected for each patient.Diagnosis of CE was performed using clinical imaging and laboratory parameters.Response to treatment was categorized as follows:"cure"as the disappearance or complete calcification of cyst/s;"improvement"as a reduction in the diameter and/or number of existing cysts;and"impairment"as an increase in the diameter and/or number of existing cyst/s and the onset of relapses(i.e.,the onset of new cyst/s and an increase in the diameter of previously existing cyst/s and/or complications.Immunoglobulin E(IgE)titers and eosinophil percentages were evaluated at diagnosis,at six months after the initiation of treatment and again in the case of relapse.Hyper-eosinophilia was defined as an eosinophil percentage of≥6%.RESULTS:Thirty-two patients were diagnosed with CE in our Unit during the research period,with a malefemale ratio of 2:1.At the time of diagnosis,40%of patients presented a single CE cyst.Sixty percent showed multi-organ involvement.The liver-lung localization ratio was 2:1.Patients below the age of 50 at diagnosis were more likely to have multiple cysts(73.7%vs 35.5%,P<0.05).Regarding treatment,30 patients were treated medically and 16 surgically.Fourteen patients were treated both medically and surgically.Relapses were seen to be less frequent in patients treated with albendazole before and after surgery.Complete cure or an improvement was achieved in 23 patients.Impairment was observed in one patient.Two patients showed no improvement.Relapses were more frequent in those patients treated before 2005.At diagnosis,71%of patients were positive for specific CE IgE,and 56.3%showed an eosinophil percentage of≥6%.Patients who were diagnosed with hyper-eosinophilia developed complications more frequently than the other patients,but did not suffer relapses.CONCLUSION:On the basis of our results,we propose cystic echinococcosis screening for family members of patients,appropriate pre-and post-surgery treatment and the assessment of anti-echinococcus IgE titer or eosinophil percentage as a therapy response marker in settings with limited resources.

New insights into the pathogenesis of intestina dysfunction:secretory diarrhea and cystic fibrosis

INTRODUCTIONA major function of the intestinal epithelium is to controlthe amount of fluid entering into and being absorbed fromthe lumen.In healthy conditions,net fluid movementfollows an absorptive vector,although significant secretionalso takes place to subserve digestive function.Thus。

Rare cystic liver lesions: A diagnostic and managing challenge

Cystic formations within the liver are a frequent finding among populations.Besides the common cystic lesions,like simple liver cysts,rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis.Thorough knowledge of each entity’s nature and course are key elements to successful treatment.Detailed search in PubMed,Cochrane Database,and international published literature regarding rare cystic liver lesions was carried out.In our research are included not only primary rare lesions like cystadenoma,hydatid cyst,and polycystic liver disease,but also secondary ones like metastasis from gastrointestinal stromal tumors lesions.Up-to date knowledge regarding diagnosis and management of rare cystic liver lesions is provided.A diagnostic and therapeutic algorithm is also proposed.The need for a multidisciplinary approach by a team including radiologists and surgeons familiar with liver cystic entities,diagnostic tools,and treatment modalities is stressed.Patients with cystic liver lesions must be carefully evaluated by a multidisciplinary team,in order to receive the most appropriate treatment,since many cystic liver lesions have a malignant potential and evolution.

Instrumental detection of cystic duct stones during laparoscopic cholecystectomy

Residual cystic duct stones (CDSs) after cholecystectomy have been recognized as a cause of post cholecystectomy pain. This study was undertaken to determine the incidence of CDSs during laparoscopic cholecystectomy(LC). A cohort of 330 consecutive patients (80 males and 250females) undergoing LC between November 2006 and May2010 was studied. Their age ranged between 16 and 88 years(median 50, IQR: 36.62). The data were prospectively collected of preoperative liver function tests, imaging, the presence of intraoperative CDSs, and common bile duct stones at on-table cholangiogram. CDSs were detected intraoperatively in 64 of the 330 patients (19%). Ultrasound failed to detect CDSs in any of these cases. Deranged liver function tests were noted in 73% of the patients with CDSs and in 57% without CDSs Common bile duct stones were detected in 9% (29) of the 330patients. CDSs occur commonly at routine cholecystectomy, and preoperative investigations are not helpful in their diagnosis As CDSs may lead to postoperative morbidity, they should be actively sought out during surgery if present.

Risk factors for hepatic steatosis in adults with cystic fibrosis: Similarities to non-alcoholic fatty liver disease

AIM To investigate the clinical, biochemical and imaging characteristics of adult cystic fibrosis(CF) patients with hepatic steatosis as compared to normal CF controls.METHODS We performed a retrospective review of adult CF patients in an academic outpatient setting during 2016. Baseline characteristics, genetic mutation analysis as well as laboratory values were collected. Abdominal imaging(ultrasound, computed tomography, magnetic resonance) was used to determine presence of hepatic steatosis. We compare patients with hepatic steatosis to normal controls.RESULTS Data was collected on 114 patients meeting inclusion criteria. Seventeen patients(14.9%) were found to have hepatic steatosis on imaging. Being overweight(BMI > 25)(P = 0.019) and having a higher pp FEV1(75 vs 53, P = 0.037) were significantly associated with hepatic steatosis. Patients with hepatic steatosis had a significantly higher median alanine aminotransferase level(27 vs 19, P = 0.048). None of the hepatic steatosis patients had frank CF liver disease, cirrhosis or portal hypertension. We found no significant association with pancreatic insufficiency or CF related diabetes.CONCLUSION Hepatic steatosis appears to be a clinically and phenotypically distinct entity from CF liver disease. The lack of association with malnourishment and the significant association with higher BMI and higher pp FEV1 demonstrate similarities with non-alcoholic fatty liver disease. Long term prospective studies are needed to ascertain whether CF hepatic steatosis progresses to fibrosis and cirrhosis.