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1.
J Oral Implantol ; 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38822657

ABSTRACT

This report describes a step-by-step stagged digital protocol for the terminal dentition patient using a Provi-guide. This protocol will improve implant placement accuracy, shorten treatment time, and assure an implant-supported fixed complete denture immediately loaded after extraction of the remaining terminal dentition and complete edentulism. The novelty of the Provi-guide technique described is that it serves two objectives: 1. a non-limiting surgical guide, and 2. a fixed interim prosthesis. Implants placed in the described digital stagged protocol can serve both the interim and the final prosthesis.

2.
Int J Surg Case Rep ; 120: 109784, 2024 May 23.
Article in English | MEDLINE | ID: mdl-38823226

ABSTRACT

INTRODUCTION: Standard treatment of a juvenile angiofibroma (JNA) is surgical resection, usually with an endoscopic endonasal surgery and a preoperative embolization. However, standard intra-arterial embolization may fail to completely devascularize tumors. A novel technique of direct intranasal intratumoral onyx embolization has been described. The aim of this study is to demonstrate the safety and the usefulness of this embolization technique on a pediatric case of JNA and to compare our results to previously reported cases. PRESENTATION OF CASE: A twelve-year-old patient suffering from Von Willebrand disease presented with a voluminous JNA with intracranial extension. Internal carotid artery (ICA) branches partially vascularized the tumor. The patient had two previous incomplete surgical resections, which were preceded by a standard embolization, due to massive perioperative bleeding. DISCUSSION: A direct intratumoral embolization of onyx safely allowed complete tumoral devascularization. Tumoral resection was then completed by an endonasal endoscopic approach. Surgery time was decreased (4,5 h versus 5,5 and 6,5 h) and blood loss were minimized (300 ml versus 1 l and 1,3 l). No complication occurred. Twelve articles previously reported this embolization technique. We present the first reported case of onyx embolization being used for a pediatric patient with a coagulation disorder and a voluminous tumor. CONCLUSION: A direct intratumoral onyx embolization allowed complete resection of a massive JNA, for a patient with Von Willebrand disease. Our data suggest that this technique is safe and may be instrumental for a JNA's resection, even if little vascularization comes from ICA branches.

3.
Int J Surg Case Rep ; 120: 109751, 2024 May 10.
Article in English | MEDLINE | ID: mdl-38823229

ABSTRACT

INTRODUCTION AND CLINICAL RELEVANCE: Acute esophageal necrosis (AEN) is a condition characterized by the necrosis of the distal portion of the esophageal mucosa. Risk factors predisposing to this condition are associated to compromised vascular perfusion (e.g. diabetes mellitus, chronic kidney disease, advanced age, and hypertension, shock states). Complications of AEN can be severe including UGI stricture, perforation and overall increased mortality. The true incidence of AEN remains uncertain due to potential subclincal presentations and early resolution. CASE PRESENTATION: The case outlined involves a 66-years-old obese male with history of alcoholism and lymph-edema of the left leg who presented to the emergency department with hematemesis, haemodynamic instability and impaired consciousness. Shortly after initial assessment, the patient went into cardiac arrest with pulse-less electrical activity (PEA). Return of spontaneous circulation (ROSC) was achieved following instigation of ALS protocol, fluid resuscitation and the administration of a total of 5 mg of adrenaline. Following stabilization, a CT scan was performed which reported a moderately enlarged esophagus with a thickened wall, liquid hypodense material within the esophagus and stomach, and liver cirrhosis. The emergent esophagogastroduodenoscopy (EGDS) revealed extensive mucosal findings indicative of diffuse necrosis with initial scarring, which was later diagnosed as AEN. The patient unfortunately deceased in ICU after developing progression of the AEN, post-cardiac arrest syndrome and liver failure. CLINICAL DISCUSSION: The presented case highlights several crucial clinical issues and management problems related to AEN. To diagnose AEN, EGDS is still the gold-standard since it allows direct inspection of the esophageal mucosal layer. The management of AEN necessitates a multidisciplinary approach that includes aggressive resuscitation, treatment of underlying comorbidities, and supportive care (e.g. proton pump inhibitors). The mortality rate for AEN remains high despite improvements in diagnosis and treatment highlighting the need to recognize this condition early and intervene promptly in the patients affected. Moreover, long-term sequelae like stricture formation of the esophagus and impaired esophageal motility may contribute to morbidity requiring continuos monitoring. Therefore, to optimize outcomes while reducing complications among affected patients, prompt identification associated with appropriate medical measures are essential. More research needs to be done aiming to better understand the pathophysiology of AEN thereby identifying strategies for its prevention or cure. CONCLUSIONS: AEN is a rare syndrome characterized by upper gastrointestinal bleeding and hypoxic damage of the esophageal mucosa, often associated with ischemia, gastric outlet obstruction, and compromised protective barriers. Treatment involves aggressive resuscitation, proton pump inhibitors, and monitoring for infection or perforation. However, despite intensive efforts, the mortality rate for AEN remains high at 32 %.

4.
J Cardiothorac Surg ; 19(1): 306, 2024 May 31.
Article in English | MEDLINE | ID: mdl-38822410

ABSTRACT

Peripheral venous catheter fracture with cardiovascular embolization is a rare but potentially serious complication. Herein, we report a case of peripheral venous catheter fracture with embolization in right ventricle in a preterm infant. The catheter fragment was successfully removed by surgical procedure via median sternotomy under cardiopulmonary bypass(CPB).We hope this case will increase awareness of this rare complication and improve cannulation safety.


Subject(s)
Catheterization, Peripheral , Device Removal , Infant, Premature , Humans , Infant, Newborn , Catheterization, Peripheral/methods , Device Removal/methods , Male , Equipment Failure , Heart Ventricles/surgery , Female
5.
Int J Surg Case Rep ; 120: 109836, 2024 May 31.
Article in English | MEDLINE | ID: mdl-38824740

ABSTRACT

INTRODUCTION: Midgut volvulus in adults based on congenital malrotation, which required emergency surgery, may occur under the stimulation of adverse factors and is rare and easy to be misdiagnosed. PRESENTATION OF CASE: A young male was taken to the emergency room of a local hospital after six hours abdominal pain. Computed tomography (CT) shows intestinal volvulus and exploratory laparotomy was performed. Postoperative CT revealed remission of small intestinal torsion and congenital malrotation of the midgut. The patient vomited frequently within 48 h after the surgery, and was transferred to our hospital for conservative treatment. After 4 days of conservative treatment, the vomiting symptoms were relieved at first, but worsened again after a liquid diet. CT showed complete duodenal obstruction and exploratory laparotomy was performed again. Congenital malrotation was found, which resulted in midgut volvulus and duodenal obstruction due to anomalous fixation of the mesentery. The bowel was placed in normal anatomical position, and the mesentery was sutured to the posterior abdominal wall. The patient was followed up for 24 months with no complaints. DISCUSSION: Due to the rare incidence and atypical pain clinical manifestations, it is difficult for the congenital malrotation in adults to be diagnosed. Midgut volvulus in adults with malrotation is even rarer and requires emergency operation, and may be misdiagnosed. CONCLUSION: Midgut volvulus with midgut malrotation is very rare in adults. Exploratory laparotomy must be careful to reduce misdiagnosis and recurrence of volvulus.

7.
Urol Case Rep ; 54: 102720, 2024 May.
Article in English | MEDLINE | ID: mdl-38827533

ABSTRACT

Chyluria, an abnormal lymphatic disorder, results in excessive abdominal lymph drainage into the urinary system, causing protein loss, nutritional deficiencies, and immune issues. Mainly linked to parasitic infections in developed countries, non-parasitic causes like trauma or tumors are rare. Typically appearing in adults with bilateral involvement, management options include conservative or surgical approaches. We present the case of a 13-year-old with congenital chyluria, treated with robot-assisted staged reno-lymphatic disconnection after failed interventional radiology. Bilateral scleroangiography followed, leading to persistently milky urine for a month. Finally, urine clarity improved, correlating with better urinalysis, emphasizing the need for a comprehensive, multi-disciplinary approach.

8.
Front Neurol ; 15: 1363867, 2024.
Article in English | MEDLINE | ID: mdl-38827577

ABSTRACT

Background: While cerebral infarction in children is rare, its prognosis is poor, and this condition can seriously burden society and families. A correlation between patent foramen ovale (PFO) and ischemic stroke has not been found in pediatric patients. Case presentation: We report a 7-year-old boy who suffered from multiple cerebral infarctions. Subsequently, the patient was diagnosed with an abnormal shunt of PFO. He underwent PFO closure and was followed up for 1 year. The patient did not experience any further cerebral infarction. Conclusions: With this case report, we want to illustrate that although the incidence rate of ischemic cerebral infarction in adolescents is very low, we should not neglect the role of PFO. Therefore, after exclusion other causes of cerebral infarction, PFO should be considered in adolescent and adult stroke patients with adult closure criteria in the same way.

9.
Diagn Pathol ; 19(1): 72, 2024 Jun 03.
Article in English | MEDLINE | ID: mdl-38831436

ABSTRACT

BACKGROUND: Primary testicular lymphoma (PTL) is relatively rare. The contralateral testis is a common site of PTL relapse; therefore, once complete remission is achieved, radiation therapy (RT) is administered to the contralateral testis to prevent relapse. CASE PRESENTATION: A 76-year-old man was diagnosed with PTL and received RT as described above. However, despite achieving and maintaining complete remission, a mass diagnosed as diffuse large B-cell lymphoma by tissue biopsy developed in the glans penis 6.5 years after prophylactic RT. We investigated whether the glans penile lymphoma was PTL relapse or a new malignancy by genomic analysis using next-generation sequencing of DNA extracted from two histopathological specimens. CONCLUSIONS: We found the same variant allele fraction in four somatic genes (MYD88, IL7R, BLNK, and FLT3) at similar frequencies, indicating that the glans penile lymphoma had the same origin as the PTL. To the best of our knowledge, this is the first case report of PTL relapse in the glans penis.


Subject(s)
High-Throughput Nucleotide Sequencing , Lymphoma, Large B-Cell, Diffuse , Neoplasm Recurrence, Local , Penile Neoplasms , Testicular Neoplasms , Humans , Male , Aged , Testicular Neoplasms/pathology , Testicular Neoplasms/genetics , Testicular Neoplasms/radiotherapy , Lymphoma, Large B-Cell, Diffuse/genetics , Lymphoma, Large B-Cell, Diffuse/pathology , Lymphoma, Large B-Cell, Diffuse/radiotherapy , Penile Neoplasms/pathology , Penile Neoplasms/radiotherapy , Penile Neoplasms/genetics , Neoplasm Recurrence, Local/pathology , Neoplasm Recurrence, Local/genetics
10.
Clin Case Rep ; 12(6): e9037, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38827943

ABSTRACT

Key Clinical Message: The use of phototherapy is highly effective in treating various skin diseases. In this study, the aim is to present vesicular and blister lesions in patients treated with UVB for psoriasis. It is advisable to consider the possibility of BSLE in cases of vesiculobullous lesions following phototherapy, along with other potential diagnoses. Abstract: Bullous systemic lupus erythematosus (BSLE) is a rare form of cutaneous lupus erythematosus that presents as vesicles and blisters on various parts of the body. The pathological appearance of these lesions often shows subepidermal vesicles with deposits of IgG, IgM, IgA, and complement C3 in granular or linear forms under direct immunofluorescence (DIF) examination. Clinical studies demonstrate the effectiveness of phototherapy in treating various skin conditions. While several studies suggest a correlation between phototherapy and the development of vesiculobullous lesions, most of these reports are related to bullous pemphigoid, with limited research on the occurrence of BSLE following phototherapy. In this case report, vesicular and blistering lesions in a 70-year-old man undergoing UVB treatment for psoriasis are described. Pathological examination confirmed the diagnosis of bullous systemic lupus erythematosus, and the patient experienced significant improvement after treatment with dapsone tablets. A literature review was conducted on the development of vesiculobullous lesions after phototherapy, comparing different approaches presented in previous studies. Our conclusion highlights the importance of considering BSLE as a possible diagnosis in cases of vesiculobullous lesions post-phototherapy, alongside other potential conditions.

11.
Clin Case Rep ; 12(6): e8969, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38827939

ABSTRACT

Key Clinical Message: The case highlights the importance of decisive action in addressing large gallstones causing gastric outlet obstruction. The chosen single-stage surgical approach reflects the need to manage both obstruction and the gallstone simultaneously. Abstract: Bouveret's syndrome is a rare cause of gastric outlet obstruction secondary to gallstones entering the enteric system through an acquired cholecystoduodenal fistula. Here, we present the case of an 85-year-old female who presented to our emergency department with gastric outlet obstruction secondary to a large gallstone in the third part of the duodenum. Abdominal X-ray did not demonstrate air-fluid levels but revealed a dilated gastric shadow, suggesting gastric outlet obstruction. EGD showed a dilated stomach and a hard, golf ball-sized gallstone in the duodenum. CT scan showed a distended stomach with a large gallstone obstructing the DJ junction and air in the biliary tree. Findings were suggestive of perforation of the gallbladder with stone impaction in the duodenojejunal (DJ) junction. The patient was managed surgically with a one-stage procedure comprising enterotomy, fistula closure, and cholecystectomy. Although Bouveret's syndrome is rare, it is important for practicing surgeons to have a high index of suspicion for this condition due to the high mortality associated with it.

12.
Clin Case Rep ; 12(6): e9021, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38827948

ABSTRACT

Key Clinical Message: Podiatrists and orthopedists should be vigilant for chronically evolving, hyperalgic soft lumps in the foot with vascular radiological features, prompting early detection of glomus tumor, timely mass removal, providing pain relief and improving patient's quality of life. Abstract: Glomus tumors refers to a rare group of benign perivascular neoplasms that originate from a neuromyoarterial structure called a glomus body. These tumors are characterized by their painful nature and predominant distribution in the extremities mainly the fingers, the hands and the feet. Nonetheless, the diagnosis is usually made after several years of symptoms experience as the lesions are mostly small, not palpable, and have variable presentations. Radiological workup especially with magnetic resonance imaging is very useful for diagnosing the tumoral process, however, confirmation can only be obtained by histological analysis. The treatment is purely surgical, and it is successful in most cases. Herein, we describe a case of glomus tumor of the foot dorsal side among a middle age male patient.

13.
Eur Heart J Case Rep ; 8(6): ytae245, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38828207

ABSTRACT

Background: Coronary artery bypass graft (CABG) surgery represents a major cardiovascular operation and may be associated with post-operative ST-elevation myocardial infarction (STEMI) due to graft failure. This is challenging to diagnose and treat as the implanted grafts may be prone to complications when treated percutaneously with drug-eluting stents. Case summary: A man in his 60 s underwent CABG and developed new persistent ST elevations of 2 mm in anterior leads with no significant chest pain, although, administered with intravenous opiates post-operatively. Transthoracic echocardiography was non-diagnostic. Invasive angiography performed emergently showed a thrombotic occlusion of the mid-left anterior descending artery at the site of the anastomosis with the left internal mammary artery (LIMA) graft. Intervention via the graft was considered high risk of complications, therefore, native coronary arteries were used to approach the occlusion, which was successfully cleared with a combination balloon angioplasty with a semi-compliant and then a drug-eluting balloon. The LIMA started working again with the resolution of ST elevation and no immediate complications. Discussion: Early post-operative ST elevations in continuous leads should not be ignored as they often may be the only feature of new-onset STEMI. Drug-eluting balloons represent a feasible and possibly safer option than drug-eluting stents to treat these conditions.

14.
Pan Afr Med J ; 47: 119, 2024.
Article in English | MEDLINE | ID: mdl-38828422

ABSTRACT

Superior ophthalmic vein thrombosis (SOVT) is a rare orbital pathology. It can cause serious complications if it isn´t diagnosed appropriately. It can be secondary to many etiologies, septic or aseptic ones. Diabetic ketoacidosis (DKA) may disturb the vascular endothelium and promote a prothrombotic state. The presence of which is related to a significantly increased risk of morbidity and mortality. We report the case of a 45-year-old woman who presented a SOVT revealing DKA. Orbit magnetic resonance imaging (MRI) showed thrombosis of the right superior ophthalmic vein. A treatment based on thrombolytic treatment, associated with antibiotic coverage and a glycemic balance was initiated. This case highlights the importance of considering both infection and diabetes as an important part of the diagnosis and management of SOVT.


Subject(s)
Magnetic Resonance Imaging , Venous Thrombosis , Humans , Female , Middle Aged , Venous Thrombosis/diagnosis , Venous Thrombosis/drug therapy , Diabetic Ketoacidosis/complications , Diabetic Ketoacidosis/diagnosis , Anti-Bacterial Agents/administration & dosage , Thrombolytic Therapy/methods , Orbit/blood supply , Orbit/diagnostic imaging
15.
Int J Surg Case Rep ; 120: 109847, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38830334

ABSTRACT

INTRODUCTION AND IMPORTANCE: Ovarian fibromas are benign tumours arising from the connective tissue of the ovarian cortex, classified into three pathological subtypes: fibroma, thecoma, and fibrothecoma. Their diagnosis is complicated by their solid nature and potential association with ascites and pleural effusion, resembling Meigs syndrome. Elevated serum CA125 levels can further complicate differentiation from malignant ovarian epithelial tumours. CASE PRESENTATION: A 37-year-old female from a rural area presented with a distended abdomen and weight loss lasting 2 months. Clinical examinations revealed a solid pelvic mass and diagnostic tests showed significantly elevated CA125 levels. Imaging suggested a large ovarian mass and surgical intervention confirmed a fibrothecoma of the left ovary. The postoperative course was uneventful, with subsequent resolution of ascites and pleurisy. CLINICAL DISCUSSION: The diagnosis of ovarian fibromas/fibrothecomas poses challenges due to their asymptomatic nature, solid appearance, and occasional association with the Meigs syndrome. Elevated CA125 levels can mislead the diagnosis of epithelial ovarian carcinoma. The case underscores the importance of considering ovarian fibromas/fibrothecomas in the differential diagnosis of ovarian tumours with elevated CA125 levels, especially in women of reproductive age. The benign nature of these tumours necessitates a conservative surgical approach, emphasizing the importance of intraoperative frozen section analysis. CONCLUSION: Ovarian fibrothecomas associated with elevated serum CA125 levels are rare. Their presentation can mimic malignant ovarian neoplasms, leading to potential diagnostic confusion. Surgical removal remains the treatment of choice, with a favorable prognosis post-surgery.

16.
Int J Surg Case Rep ; 120: 109848, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38830333

ABSTRACT

INTRODUCTION: Avulsion fractures of the calcaneal tuberosity with significant displacement can cause soft tissue complications in the heel. However, a treatment strategy for calcaneal tuberosity fractures with poor heel-skin condition is yet to be established. Here, we report a case involving avulsion fracture of the calcaneal tuberosity presenting with superficial skin necrosis that was treated with a plate lacking the anterior component and screws inserted percutaneously. PRESENTATION OF CASE: A 74-year-old woman presented with progressive right heel pain following an injury to her heel sustained approximately six weeks previously. She had experienced difficulty walking due to heel pain and superficial necrosis was observed on the posterior surface of the heel. Radiography and computed tomography revealed an avulsion fracture of the calcaneal tuberosity with superior displacement. Open reduction was performed using a lateral L-shaped incision. After inserting two cancellous screws percutaneously into the calcaneal tuberosity, we fixed a plate lacking the anterior component to the lateral surface of the calcaneus. The superficial necrosis healed gradually post-operatively. Bone union was confirmed using radiography six months post-operatively. DISCUSSION: We developed a novel surgical procedure to treat avulsion fractures of the calcaneal tuberosity with poor skin condition. The combination of a plate lacking the anterior component and the percutaneous insertion of canulated cancellous screws can reduce the risk of post-operative soft tissue complications while maintaining fixation of the fractured fragment. CONCLUSION: Our findings provide a novel surgical method for the treatment of avulsion fractures of the calcaneal tuberosity with soft tissue complications.

18.
Ital J Pediatr ; 50(1): 111, 2024 Jun 03.
Article in English | MEDLINE | ID: mdl-38831374

ABSTRACT

BACKGROUND: Lysinuric protein intolerance (LPI) is a multi-organ metabolic disorder characterized by the imbalance in absorption and excretion of cationic amino acids like lysine, ornithine and arginine. Infants with LPI typically present with recurrent vomiting, poor growth, interstitial lung disease or renal impairment. The early onset of pulmonary alveolar proteinosis (PAP) has been reported to be associated with a severe form of LPI. Treatment of PAP most commonly consists of whole-lung lavage (WLL) and in autoimmune PAP, granulocyte-macrophage colony stimulating factor (GM-CSF) administration. Nevertheless, GM-CSF therapy in LPI-associated PAP has not been scientifically justified. CASE PRESENTATION: We describe the case of an 8-month-old infant presenting with respiratory failure due to LPI associated with PAP, who was twice treated with WLL; firstly, while on veno-venous ECMO assistance and then by the use of a selective bronchial blocker. After the two treatments with WLL, she was weaned from daytime respiratory support while on initially subcutaneous, then on inhaled GM-CSF therapy. CONCLUSIONS: This case supports the notion that GM-CSF therapy might be of benefit in patients with LPI-associated PAP. Further studies are needed to clarify the exact mechanism of GM-CSF in patients with LPI-associated PAP.


Subject(s)
Amino Acid Metabolism, Inborn Errors , Bronchoalveolar Lavage , Granulocyte-Macrophage Colony-Stimulating Factor , Pulmonary Alveolar Proteinosis , Humans , Pulmonary Alveolar Proteinosis/therapy , Granulocyte-Macrophage Colony-Stimulating Factor/therapeutic use , Infant , Female , Amino Acid Metabolism, Inborn Errors/therapy , Amino Acid Metabolism, Inborn Errors/complications
19.
BMC Oral Health ; 24(1): 646, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38824565

ABSTRACT

BACKGROUND: Immature teeth with necrotic pulps present multiple challenges to clinicians. In such cases, regenerative endodontic procedures (REPs) may be a favorable strategy. Cells, biomaterial scaffolds, and signaling molecules are three key elements of REPs. Autologous human dental pulp cells (hDPCs) play an important role in pulp regeneration. In addition, autologous platelet concentrates (APCs) have recently been demonstrated as effective biomaterial scaffolds in regenerative dentistry, whereas the latest generation of APCs-concentrated growth factor (CGF), especially liquid phase CGF (LPCGF)-has rarely been reported in REPs. CASE PRESENTATION: A 31-year-old woman presented to our clinic with the chief complaint of occlusion discomfort in the left mandibular posterior region for the past 5 years. Tooth #35 showed no pulp vitality and had a periodontal lesion, and radiographic examination revealed that the tooth exhibited extensive periapical radiolucency with an immature apex and thin dentin walls. REP was implemented via transplantation of autologous hDPCs with the aid of LPCGF. The periodontal lesion was managed with simultaneous periodontal surgery. After the treatment, the tooth was free of any clinical symptoms and showed positive results in thermal and electric pulp tests at 6- and 12-month follow-ups. At 12-month follow-up, radiographic evidence and three-dimensional models, which were reconstructed using Mimics software based on cone-beam computed tomography, synergistically confirmed bone augmentation and continued root development, indicating complete disappearance of the periapical radiolucency, slight lengthening of the root, evident thickening of the canal walls, and closure of the apex. CONCLUSION: hDPCs combined with LPCGF represents an innovative and effective strategy for cell-based regenerative endodontics.


Subject(s)
Dental Pulp , Regenerative Endodontics , Humans , Female , Adult , Dental Pulp/cytology , Regenerative Endodontics/methods , Dental Pulp Necrosis/therapy , Cell Transplantation/methods , Transplantation, Autologous
20.
Kidney Med ; 6(6): 100824, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38826567

ABSTRACT

Primary hyperoxaluria (PH) is a rare genetic disorder characterized by excessive oxalate production because of specific gene defects. PH1 is the most prevalent type, causing recurrent kidney stone disease and often leading to chronic kidney disease and kidney failure. Our previous study suggested that pregnancy did not adversely affect kidney function in female patients with PH. In this study, we identified 4 PH1 cases with urinary oxalate (UOx) measurements during pregnancy from the Rare Kidney Stone Consortium and Oxalosis and Hyperoxaluria Foundation PH registry to investigate UOx levels during pregnancy in patients with PH1. The PH Registry is approved by the Institutional Review Board of Mayo Clinic (Rochester, MN). All 4 showed a decrease in UOx during pregnancy when compared with before pregnancy and after delivery. These findings contrast with those of the general population, in which the UOx tends to increase during pregnancy because of a simultaneous physiological increase in the glomerular filtration rate. Elucidating the mechanism underlying reduced UOx during pregnancy in PH1 could suggest novel PH therapies. These findings could also affect the clinical management and have implications regarding the safety of withholding novel PH1-directed molecular therapies that currently have uncertain safety profiles during pregnancy. We highlight the need for additional data on urinary changes in patients with PH and other populations while pregnant to clarify changes in UOx throughout pregnancy.

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