Kidney stones are becoming increasingly common, affecting up to 10% of adults. A small percentage are of monogenic origin, such as Dent\'s disease (DD). DD is a syndrome that causes low-molecular-weight proteinuria, hypercalciuria, nephrolithiasis, and nephrocalcinosis. It is X-linked, and most patients have mutations in the CLCN5 gene. We performed a review of the literature and evaluated the case series (n = 6) of a single center in Spain, reviewing the natural evolution of kidney stones, clinical implications, laboratory analyses, radiological development, and treatment. All patients had a genetically confirmed diagnosis, with the CLCN5 mutation being the most frequent (66%). All patients had proteinuria and albuminuria, while only two and three presented hypercalciuria and phosphate abnormalities, respectively. Only one patient did not develop lithiasis, with most (60%) requiring extracorporeal shock wave lithotripsy or surgery during follow-up. Most of the patients are under nephrological follow-up, and two have either received a renal transplant or are awaiting one. The management of these patients is similar to that with lithiasis of non-monogenic origin, with the difference that early genetic diagnosis can help avoid unnecessary treatments, genetic counseling can be provided, and some monogenic kidney stones may benefit from targeted treatments.

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The presence of calculi inside the uterine cavity is an uncommon finding, with few cases described in the literature. Here, we present a clinical case of a 30-year-old patient with uterine lithiasis. The patient presented with 3 months of evolution, characterized by abdominal pain in the hypogastrium, with a report of transvaginal ultrasound of hematometra with suggestive images of endometrial polyps. For this reason, she was scheduled for hysteroscopy, where three lesions of hard consistency were found, with the largest 1.5 cm. Currently, due to the few cases of uterine lithiasis described, there is no explanation of how the stones are formed in the uterine cavity. The age of the patient in this case report and the absence of predisposing factors for uterine calcifications reported in the literature is striking, which leads us to propose a hypothesis of the origin of uterine lithiasis, which is related to the history of cesarean section and the presence of hematometra, such hematic retention could have predisposed to the development of calculi in this patient. Uterine lithiasis is an extremely rare entity with few published cases; therefore, further studies are necessary.

BACKGROUND: Yucatan stands out as the state with the highest prevalence of urolithiasis in Mexico, placing significant demands on healthcare services, such as consultation and surgical intervention. Staghorn calculi are related to recurrent urinary tract infections, and their management is always surgical. The stone-free rate is a parameter used to measure the success of surgery, with residual stones considered those persisting four weeks after surgical management. There are understudied prognostic factors that can predict the success of achieving stone-free status, taking into account the number of stones, their location, and the anatomical variations of the patient\'s collecting system. The study aims to determine the prognostic factors for residual lithiasis in patients with staghorn calculi treated with percutaneous nephrolithotomy at the High Specialty Regional Hospital of the Yucatan Peninsula.
METHODS: A case-control study was performed including 188 patients, aged 18 years or older, and diagnosed with staghorn calculus from January 2022 to June 2023, grouping the patients according to their stone-free rate evidence on postoperative computed tomography. Data were collected from the records of the Urology Department at a high-specialty hospital in Yucatan. The groups were analyzed, aiming to establish an association between preoperative factors and postoperative outcomes measured in terms of stone-free rate.
RESULTS: A total of 188 patients with staghorn calculi were included, with a predominance in females (58.5%) and a mean age of 45.4 ± 11.9 years. The most common comorbidity was hypertension (29.8%), and 27.7% had a history of recurrent urinary tract infections. Regarding the Sampaio classification, B1 was the most prevalent in our population with 66 cases (35.1%), while Type A2 was the least common (13.8%). According to what was obtained through the multivariate logistic regression model, the calyceal anatomy Type A1 and A2 were associated with residual lithiasis (p= 0.016 OR: 2.994 CI: 1.223-7.331), and Grade IV was associated with a higher rate of residual lithiasis (p=0.005 CI: 1.586-13.100). A statistically significant association was found between stone burden and the presence of residual lithiasis (p=< 0.001).
CONCLUSIONS: Guy\'s Score Grade IV showed a higher incidence of residual lithiasis, seemingly associated with stone burden, leading to the conclusion that both factors were categorized as predictors for the development of post-surgical residual lithiasis. Regarding anatomical variations according to Sampaio, it was observed that types A1 and A2 showed a lower rate of stone-free status. Therefore, we also consider them as variables that may influence the achievement of success in endourological management. Personalized patient assessment allows for more accurate prognostic factors, enabling a more comprehensive surgical planning in the presence of staghorn calculi.

BACKGROUND: It is unclear whether laparoscopic hepatectomy (LH) for hepatolithiasis confers better clinical benefit and lower hospital costs than open hepatectomy (OH). This study aim to evaluate the clinical and economic value of LH versus OH.
METHODS: Patients undergoing OH or LH for primary hepatolithiasis at Yijishan Hospital of Wannan Medical College between 2015 and 2022 were divided into OH group and LH group. Propensity score matching (PSM) was used to balance the baseline data. Deviation-based cost modelling and weighted average median cost (WAMC) were used to assess and compare the economic value.
RESULTS: A total of 853 patients were identified. After exclusions, 403 patients with primary hepatolithiasis underwent anatomical hepatectomy (OH n=143; LH n=260). PSM resulted in 2 groups of 100 patients each. Although LH required a longer median operation duration compared with OH (285.0 versus 240.0 min, respectively, P<0.001), LH patients had fewer wound infections, fewer pre-discharge overall complications (26 versus 43%, respectively, P=0.009), and shorter median postoperative hospital stays (8.0 versus 12.0 days, respectively, P<0.001). No differences were found in blood loss, major complications, stone clearance, and mortality between the two matched groups. However, the median overall hospital cost of LH was significantly higher than that of OH (CNY¥52,196.1 versus 45,349.5, respectively, P=0.007). Although LH patients had shorter median postoperative hospital stays and fewer complications than OH patients, the WAMC was still higher for the LH group than for the OH group with an increase of CNY¥9,755.2 per patient undergoing LH.
CONCLUSIONS: The overall clinical benefit of LH for hepatolithiasis is comparable or even superior to that of OH, but with an economic disadvantage. There is a need to effectively reduce the hospital costs of LH and the gap between costs and diagnosis-related group reimbursement to promote its adoption.

The term hepatolithiasis describes the presence of biliary stones within the intrahepatic bile ducts, above the hilar confluence of the hepatic ducts. The disease is more prevalent in Asia, mainly owing to socioeconomic and dietary factors, as well as the prevalence of biliary parasites. In the last century, owing to migration, its global incidence has increased. The main pathophysiological mechanisms involve cholangitis, bile infection and biliary strictures, creating a self-sustaining cycle that perpetuates the disease, frequently characterised by recurrent episodes of bacterial infection referred to as syndrome of \"recurrent pyogenic cholangitis\". Furthermore, long-standing hepatolithiasis is a known risk factor for development of intrahepatic cholangiocarcinoma. Various classifications have aimed at providing useful insight of clinically relevant aspects and guidance for treatment. The management of symptomatic patients and those with complications can be complex, and relies upon a multidisciplinary team of hepatologists, endoscopists, interventional radiologists and hepatobiliary surgeons, with the main goal being to offer relief from the clinical presentations and prevent the development of more serious complications. This comprehensive review provides insight on various aspects of hepatolithiasis, with a focus on epidemiology, new evidence on pathophysiology, most important clinical aspects, different classification systems and contemporary management.

A 91-year-old man had a history of cholecystectomy and choledochostomy for cholecystolithiasis and choledocholithiasis. Eleven years earlier, intrahepatic stones were found in the posterior bile duct, and he did not wish to undergo treatment. Over time, worsening of the intrahepatic stones and dilation of the intrahepatic bile duct were observed. At 91 years old, enhanced abdominal CT revealed wall thickening of the hilar bile duct, and MRCP showed stenosis of the hilar bile duct. Endoscopic retrograde cholangiography showed no contrast in the right intrahepatic bile duct and marked dilation of the left intrahepatic bile duct. Brush cytology confirmed adenocarcinoma, leading to a diagnosis of hilar cholangiocarcinoma. He underwent open right and caudal lobectomy with biliary reconstruction. Histopathological examination revealed a hilar cholangiocarcinoma, T3N1M0, Stage Ⅲc, mainly located at the confluence of the right and left hepatic ducts. This case suggests a potential association between hepatolithiasis and hilar cholangiocarcinoma, emphasizing the importance of regular imaging examinations for timely surgical resection. Early intervention, including liver resection, is recommended for the management of hepatolithiasis.

BACKGROUND: This study investigated the clinical application of the indocyanine green (ICG) fluorescence navigation technique in bile duct identification during laparoscopic common bile duct exploration (LCBDE) for complex hepatolithiasis.
METHODS: Eighty patients with complex hepatolithiasis were admitted to our department between January 2022 and June 2023 and randomly divided into control and observation groups. The control group underwent conventional LCBDE, while the observation group underwent LCBDE guided by ICG fluorescence.
RESULTS: Intraoperatively, the observation group had shorter operation and search times for the common bile duct (CBD), as well as reduced intraoperative blood loss and fewer complications, such as conversion to laparotomy and various injuries (gastroduodenal, colon, pancreatic, and vascular) than the control group, with statistical significance (P < 0.05). Postoperatively, the observation group had lower rates of postoperative bile leakage, abdominal infection, postoperative hemorrhage, and residual stone than the control group. Additionally, the observation group demonstrated significantly shorter times for resuming flatus, removal of the abdominal drainage tube, and hospitalization than the control group, with statistical significance (P < 0.05).
CONCLUSIONS: ICG fluorescence navigation technology effectively visualizes the bile duct, improves its identification rate, shortens the operation time, prevents biliary tract injury, and reduces the occurrence of complications.

BACKGROUND: This study aimed to reveal the early and late postoperative complications and outcomes after surgery for congenital biliary dilatation (CBD) by reviewing cases over the past 40 years.
METHODS: We retrospectively evaluated 59 patients with CBD who underwent radical surgery for complications and outcomes, based on medical records. Early complications were defined as those requiring treatment within 5 years of the initial operation. Late complications were defined as those treated more than 5 years later.
RESULTS: The median age at the first surgery was 37 months. Regarding biliary reconstruction, 54 of the 59 patients (91.5%) underwent hepaticojejunostomy. Although three patients underwent cholecystoduodenostomy and one patient underwent hepaticoduodenostomy, all were converted to hepaticojejunostomy after a median of 12.5 years. One patient developed synchronous biliary carcinoma and underwent pancreaticoduodenectomy. Early complications occurred in seven patients with 10 events (surgical site infection, n = 3 bile leakage, n = 3; ileus, n = 3; bile duct obstruction, n = 1 and intussusception, n = 1). Late complications occurred in nine patients with 12 events (ileus, n = 3; anastomotic stricture, n = 3; hepatolithiasis, n = 3; asynchronous biliary carcinoma, n = 2; pancreatolithiasis, n = 1). Two of the three patients with hepatolithiasis underwent hepatectomy refractory to the endoscopic approach. Two patients developed asynchronous biliary carcinoma at 34 and 13 years after last operation; both ultimately died of the carcinoma. Only 35 patients (61.4%) underwent a follow-up examination. A total of 11 female patients (45.8%) eventually married, and all successfully gave birth.
CONCLUSIONS: Although the long-term prognosis is excellent with complete cyst excision and hepaticojejunostomy, we emphasize the importance of long-term follow-up.