New Approaches to Diabetic Nephropathy from Bed to Bench.

Diabetic nephropathy (DN) is the main cause of end-stage kidney disease (ESKD). DN-related ESKD has the worst prognosis for survival compared with other causes. Due to the complex mechanisms of DN and the heterogeneous presentations, unmet needs exist for the renal outcome of diabetes mellitus. Clinical evidence for treating DN is rather solid. For example, the first Kidney Disease: Improving Global Outcomes (KDIGO) guideline was published in October 2020: KDIGO Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease. In December of 2020, the International Society of Nephrology published 60 (+1) breakthrough discoveries in nephrology. Among these breakthroughs, four important ones after 1980 were recognized, including glomerular hyperfiltration theory, renal protection by renin-angiotensin system inhibition, hypoxia-inducible factor, and sodium-glucose cotransporter 2 inhibitors. Here, we present a review on the pivotal and new mechanisms of DN from the implications of clinical studies and medications.

Recovery of Renal Function in a Kidney Transplant Patient After Receiving Hemodialysis for 4 Months.

Renal transplant is the preferred choice of treatment for end-stage renal diseases. To avoid rejection, increasingly potent immunosuppressants are administered to recipients of kidney transplants. Complications, when there is excess immunosuppression, include possible lethal infections, which reduce allograft survival and compromise patient survival. When there is insufficient immunosuppression, rejections could impair allograft outcomes. Moreover, recurrent primary diseases could also threaten allograft outcomes. Here, we report a case of a patient who underwent ABO-incompatible and living-related renal transplant in which the patient experienced 7 acute kidney injury episodes, including recurrent malignant hypertension, rejection, and infections. After the patient underwent 4 months of hemodialysis (with serum creatinine level of 17 mg/dL), which was later terminated, no adverse effects were found for 1 year (serum creatinine level of 3.7 mg/dL). Therefore, renal function recovery may be longer in patients with renovascular diseases with hypertension. For recipients undergoing hemodialysis with allograft failure, we suggest that the treatment should be not completely withdrawn of immunosuppressants so that acute kidney injuries are minimized. Even after prolonged hemodialysis (eg, for 4 months), recipients may still be able to recover renal function.

A patient with severe rhabdomyolysis and high levels of creatinine kinase had renal functions fully recovered after haemodialysis: a case report.

Rhabdomyolysis is diagnosed based on the levels of blood biomarkers such as creatine kinase (CK), but the use of CK levels to predict long-term renal function remains controversial. This current report presents a case with a very high CK level with the presentation of acute kidney injury (AKI) who regained full renal function. A 29-year-old man, in a manic mood and presenting with dyspnoea, was admitted to hospital following an episode of ketamine use along with a history of drug abuse. The laboratory analyses identified rhabdomyolysis (CK, 35 266 U/l) and AKI (serum creatinine, 3.96 mg/dl). Despite treatment with intravenous normal saline (4000 ml/day), his CK level reached at least 300 000 U/l. He underwent 13 sessions of haemodialysis and his renal function fully recovered. The final measurements were serum creatinine 1.0 mg/dl and CK 212 U/l. These findings support the view that the predictive power of CK level on AKI is limited, especially regarding long-term renal function. Close follow-up examinations of renal function after haemodialysis are mandatory for patients with rhabdomyolysis.

Case report: The first case of Achromobacter xylosoxidans-related tunnel infection in a patient receiving peritoneal dialysis.

RATIONALE: Achromobacter xylosoxidans infection is mostly reported in immunocompromised patients. Until now, it is still rarely reported in patients undergoing peritoneal dialysis. PATIENT CONCERNS: This is the 1st case of A xylosoxidans infection due to tunnel infection of a Tenckhoff catheter. DIAGNOSIS: The diagnosis was confirmed by the report of culture. INTERVENTIONS: Risk factors for this infection in peritoneal dialysis include uremia with an immunocompromised state, contamination due to inexperienced skills, and aqueous environment of the dialysate. OUTCOME: We believe that finding the source of A xylosoxidans contamination is the most important aspect of the overall treatment of the infection. LESSONS: Environmental investigation of suspected source contamination is warranted in those with A xylosoxidans infection. Once the diagnosis is made, removal of the Tenckhoff catheter should not be delayed.

Calcium oxalate crystal related kidney injury in a patient receiving Roux-en Y hepaticojejunostomy due to gall bladder cancer.

BACKGROUND: Calcium oxalate nephropathy is rare in current practice. It was a common complication during jejunoileal bypass, but much less seen in modern gastric bypass surgery for morbid obesity. The major cause of it is enteric hyperoxaluria. CASE PRESENTATION: We report on a patient here with acute kidney disease due to calcium oxalate nephropathy, rather than the conditions mentioned above. The male patient received a Roux-en Y hepaticojejunostomy and common bile duct drainage. In addition to enteric hyperoxaluria, chronic kidney disease related metabolic acidosis, chronic diarrhea related volume depletion, a high oxalate and low potassium diet, long term ascorbic acid intake and long term exposure to antibiotics, all predisposed him to having oxalate nephropathy. CONCLUSION: This is the first case with such conditions and we recommend that similarly diagnosed patients avoid all these predisposing factors, in order to avoid this rare disease and its undesired outcome.

Minimal change disease in a patient with myasthenia gravis: A case report.

BACKGROUND: Myasthenia gravis superimposed with proteinuria is a very rare disorder with only 39 cases reported so far. Of these cases, the most commonly associated disorder is minimal change disease. Myasthenia gravis and minimal change disease are both related to the dysfunction of T lymphocytes and hence the 2 disorders may be connected. METHODS: Here we report the first case on a patient diagnosed with myasthenia gravis concurrently with the minimal change disease, and it was presented in the absence of thymoma or thymic hyperplasia. RESULTS: Treatment for myasthenia gravis also lowered proteinuria of minimal change disease. He ever experienced good control for myasthenia gravis and minimal change disease. However, pneumonia related septic shock occurred to him and finally he was dead. Minimal change disease is generally considered to occur subsequent to the onset of myasthenia gravis with causal association. After extensive literature review, we noted only 47.8% minimal change disease had occurred after the onset of myasthenia gravis. CONCLUSION: Minimal change disease mostly occurs in children and if diagnosed in adults, clinicians should search for a potential cause such as myasthenia gravis and other associated thymic disorders.

Gallbladder bleeding-related severe gastrointestinal bleeding and shock in a case with end-stage renal disease: A case report.

Gallbladder (GB) bleeding is very rare and it is caused by cystic artery aneurysm and rupture, or GB wall rupture. For GB rupture, the typical findings are positive Murphy's sign and jaundice. GB bleeding mostly presented as hemobilia. This is the first case presented with severe GI bleeding because of GB rupture-related GB bleeding. After comparing computed tomography, one gallstone spillage was noticed. In addition to gallstones, uremic coagulopathy also worsens the bleeding condition. This is also the first case that patients with GB spillage-related rupture and bleeding were successfully treated by nonsurgical management. Clinicians should bear in mind the rare causes of GI bleeding. Embolization of the bleeding artery should be attempted as soon as possible.

Paraquat Poisoning in Patients With HIV Infection: A Case Report and Literature Review.

Paraquat poisoning is very severe. Most victims, including those who have ingested a small amount, will die from Paraquat poisoning. The cause of death in the majority of such cases is lung fibrosis. Paraquat poisoning in patients with positive human immunodeficiency virus (HIV) infection status has seldom been reported. Herein, we report a case of an HIV patient with Paraquat poisoning who had an excellent outcome even without standard treatment. Currently, only 3 such cases have been reported in the literature and in each case there was a good outcome, which was not expected according to predictive models. A possible mechanism may involve the relative lack of functional macrophages in HIV patients, which would tend to result in much less severe lung injury. None of the available predictive models of Paraquat poisoning appear to be appropriate for HIV patients.Paraquat poisoning in HIV patients may have better survival due to less lung injury.

Current Safety of Renal Allograft Biopsy With Indication in Adult Recipients: An Observational Study.

Renal biopsy remains the golden standard diagnosis of renal function deterioration. The safety in native kidney biopsy is well defined. However, it is a different story in allograft kidney biopsy. We conduct this retrospective study to clarify the safety of allograft kidney biopsy with indication.All variables were grouped by the year of biopsy and they were compared by Mann-Whitney U test (for continuous variables) or Chi-square test (for categorical variables). We collected possible factors associated with complications, including age, gender, body weight, renal function, cause of uremia, status of coagulation, hepatitis, size of needle, and immunosuppressants.We recruited all renal transplant recipients undergoing allograft biopsy between January of 2009 and December of 2014. This is the largest database for allograft kidney biopsy with indication. Of all the 269 biopsies, there was no difference in occurrence among the total 14 complications (5.2%) over these 6 years. There were only 3 cases of hematomas (1.11%), 6 gross hematuria (2.23%), 1 hydronephrosis (0.37%), and 2 hemoglobin decline (0.74%). The outcome of this cohort is the best compared to all other studies, and it is even better than the allograft protocol kidney biopsy. Among all possible factors, patients with pathological report containing "medullary tissue only" were susceptible to complications (P < 0.001, 1.8 of relative risk).In modern era, this study demonstrates the safety of allograft kidney biopsy with indication. Identifying the renal capsule before biopsy to avoid puncture into medulla is the most important element to prevent complications.

Long-Term Follow-Up of a Homozygous Familial Hypercholesterolemic Patient Receiving Regular Double Filtration Plasmapheresis - Case Report and Literature Review.

Homozygous familial hypercholesterolemia (HoFH) is a very rare condition (1 case per 1 million people) with a dismal outcome due to inevitable coronary artery disease that occurs when left untreated. Lipoprotein apheresis (LA), previously known as low-density lipoprotein (LDL) apheresis, is very effective in reducing LDL-cholesterol (LDL-C) if HoFH is refractory to aggressive drug therapy and diet control. In this study, we report a case with HoFH, who presented with xanthomata over the 4 limbs when she was 3 years old. When she was 11 years old, she began treatment with semi-selective LA with double filtration plasmapheresis (DFPP) once per week because HoFH was refractory to high-dose statin and diet control. LDL-C was reduced from 8.2 ± 0.9 to 2.69 ± 0.75 mmol/l (reduction rate = 67.3 ± 6.1%). The xanthomata over the 4 limbs were nearly completely resolved after 2 years of DFPP. Two years later, after the initiation of DFPP, we performed coronary angiography and echocardiography for regular checkup in the absence of chest pain, and the result was negative. To date (11 years after initiation of DFPP), she has not complained of any chest pain, shown intolerance to exercise, or exhibited ST-T change on electrocardiography. At the age of 20, multidetector computed tomography showed no significant stenosis over the coronary arteries. At the most recent follow-up visit, she was found to have good heart function and no xanthomata. LA is effective in the treatment of HoFH when drug therapy and diet control fail. With this treatment, pre-existing xanthomata can regress and coronary artery disease can be prevented.

Case Report: A Rare Cause of Complicated Urinary Tract Infection in a Woman with Herlyn-Werner-Wunderlich Syndrome.

INTRODUCTION: Urinary tract infection is a common disease in the general population. However, in patients with frequent urinary tract infection, it is important to determine any treatable cause to avoid recurrence. CASE PRESENTATION: Herlyn-Werner-Wunderlich syndrome or OHVIRA syndrome is a very rare congenital anomaly with uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The earliest presentation of this syndrome is hematocolpos that develops during menstruation and results in dysmenorrhea and a pelvic mass shortly after menarche. Herein, we report a patient with Herlyn-Werner-Wunderlich syndrome manifested with unusual symptoms, delayed onset and without surgery. The unique point of this patient is the partial obstruction of cervico-vaginal junction. CONCLUSIONS: Early diagnosis and timely treatment of OHVIRA syndrome can prevent long-term complications, such as recurrent urinary tract infection and infertility. A high index of suspicion is required, even though OHVIRA syndrome is extremely rare and may have an atypical presentation.