Alport syndrome misdiagnosed with IgA nephropathy from familial history: a case report and brief review.

BACKGROUND: Alport syndrome is a rare inherited disease resulting from a primary disorder of the glomerular basement membrane. This disease results from mutations in genes encoding alpha chains of type IV collagen. In the differential diagnosis of this disease, IgA nephropathy is the most common primary glomerular disease with gross or microscopic hematuria. CASE PRESENTATION: A 50-year-old woman was presented with microscopic hematuria and proteinuria of under one gram. Due to the diagnosis of IgA nephropathy in family members, she was treated and followed up for 4 years as a possible case of IgA nephropathy. Eye examination and audiometry were normal. She underwent renal biopsy with an exacerbation of proteinuria. There was no finding in favor of IgA nephropathy in the histological examination, but the findings of electron microscopy and family history favored Alport syndrome. CONCLUSIONS: This case demonstrates the importance of accurate history and electron microscopy in the complete histological evaluation and diagnosis of glomerular disease. Although in most cases the two can be differentiated based on clinical manifestations, laboratory findings, and histopathological examination, sometimes the association of these two diseases in the families involved or the lack of accurate history and complete histological examinations can complicate the diagnosis.

Choriocarcinoma transmitted with the transplant: Case study.

Choriocarcinoma is a rare kind of cancer, which may be either gestational or non-gestational. Choriocarcinoma is responsible for about a quarter of all documented neoplastic aneurysms. It is a descriptive case report of choriocarcinoma transmission from a donor, following kidney donation. A 45-year-old woman got a kidney from a 25-year-old woman who was taken to the hospital due to a non-traumatic cerebral hemorrhage. She delivered a healthy baby 48 days before her brain death. The transplant was successfully done. Five weeks' post-transplantation, the recipient had pain and erythema in the surgical area. Regarding the high level of beta-human chorionic gonadotropin in her blood, diagnostic tests were performed. Following the confirmation of the cancer, a five-phase chemotherapy plan with various pharmaceutical regimens was initiated. Liver function test values rose after the final round of chemotherapy, and the patient developed hepatic encephalopathy. Considering the thrombocytopenia, dialysis, or hemoperfusion, which are normally performed to reduce liver enzymes, were not initiated. Finally, she died due to the hepatic failure and disseminated intravascular coagulation. Although the nephrologists disagree on the optimal course of treatment, it seems that nephrectomy would be helpful in such instances. Physicians should be aware of the possibility of transplant-related choriocarcinoma in female donors of reproductive age who die because of intracerebral brain hemorrhage for unclear reasons. Every donor must undergo a thorough examination. It is critical to get documents, clarify history, and interview relatives.

Clinical, Functional, and Mental Health Outcomes in Kidney Transplant Recipients 3 Months After a Diagnosis of COVID-19.

BACKGROUND: Kidney transplant patients are at high risk for coronavirus disease 2019 (COVID-19)-related mortality. However, limited data are available on longer-term clinical, functional, and mental health outcomes in patients who survive COVID-19. METHODS: We analyzed data from adult kidney transplant patients in the European Renal Association COVID-19 Database who presented with COVID-19 between February 1, 2020, and January 31, 2021. RESULTS: We included 912 patients with a mean age of 56.7 (±13.7) y. 26.4% were not hospitalized, 57.5% were hospitalized without need for intensive care unit (ICU) admission, and 16.1% were hospitalized and admitted to the ICU. At 3 mo follow-up survival was 82.3% overall, and 98.8%, 84.2%, and 49.0%, respectively, in each group. At 3 mo follow-up biopsy-proven acute rejection, need for renal replacement therapy, and graft failure occurred in the overall group in 0.8%, 2.6%, and 1.8% respectively, and in 2.1%, 10.6%, and 10.6% of ICU-admitted patients, respectively. Of the surviving patients, 83.3% and 94.4% reached their pre-COVID-19 physician-reported functional and mental health status, respectively, within 3 mo. Of patients who had not yet reached their prior functional and mental health status, their treating physicians expected that 79.6% and 80.0%, respectively, still would do so within the coming year. ICU admission was independently associated with a low likelihood to reach prior functional and mental health status. CONCLUSIONS: In kidney transplant recipients alive at 3-mo follow-up, clinical, physician-reported functional, and mental health recovery was good for both nonhospitalized and hospitalized patients. Recovery was, however, less favorable for patients who had been admitted to the ICU.

Hybrid Percutaneous Brachiofemoral Shunt and Open Abdominal Aortic Aneurysm Repair in a Kidney Transplant Recipient.

Abdominal aortic aneurysm (AAA) repair in kidney transplant recipients may cause ischemia in the transplanted kidney. As a result, various techniques have been described for protection of the renal allograft during AAA repair including temporary shunt, extracorporeal bypass, cold renal perfusion, endovascular aortic aneurysm repair (EVAR), and operation without renal allograft protection. We successfully treated a 56-year-old man, a case of kidney transplantation with AAA, using a temporary hybrid percutaneous brachiofemoral shunt using vascular prosthesis with a long 7-French (Fr) catheter sheath introducer (CSI) in the aortic arch via the right brachial artery and 8 Fr CSI in the right femoral artery that were connected together with a 7 Fr guiding catheter, before aortic cross-clamping and repair of AAA using a Dacron tube graft. The patient recovered well from the surgery without any complication and was discharged on the 6th postoperative day. To our knowledge, this is the first report of using a temporary hybrid percutaneous brachiofemoral shunt for renal allograft protection in AAA repair surgery in a patient with kidney transplantation, and we think that this temporary shunt is an easy, safe, and rapid method for renal allograft protection from ischemia.

Adaptation of a quality of life questionnaire for Iranian patients with esophageal cancer.

INTRODUCTION: Evaluation of quality of life is very important in cancer patients. Esophagus-specific quality of life questionnaire (QLQ-OES18) is a disease-specific questionnaire for assessing quality of life in esophageal cancer (EC). So we aimed to translate and evaluate the reliability and validity of the QLQ-OES18 when applied to Iranian patients. MATERIALS AND METHODS: This study was designed as cross-sectional study on 62 newly confirmed EC in two referral hospital in Tehran, Iran. Reliability of the subscales was evaluated by intraclass correlation coefficients. Pearson's correlations of an item with its own scale and other scales were calculated to assess convergent and discriminant validity. Clinical validity was also evaluated by known-group comparisons. RESULTS: Cronbach's alpha was higher than 0.7 in most subscales. All subscales met the standards of convergent and discriminant validity. Also QLQ-OES18 had discriminatory power for differentiation between patient's groups with different clinical status. CONCLUSION: Our results provide evidences that Persian version of QLQ-OES18 is a valid and reliable questionnaire when applied to a sample of Iranian patients with EC and is recommended for use in clinical research.

Methemoglobinemia in aluminum phosphide poisoning.

INTRODUCTION: Acute aluminum phosphide (AlP) poisoning is one of the most common causes of acute pesticide poisoning in Iran. Hydrogen phosphide or phosphine gas is produced following reaction of AlP with water even at ambient humidity. Methemoglobinemia is a rare finding following phosphine poisoning. In this paper, two cases of fatal AlP poisoning complicated by methemoglobinemia are reported. CASE REPORT: Two patients presented following suicidal ingestion of AlP tablets. In the Emergency Department (ED), they received gastric lavage with sodium bicarbonate and potassium permanganate. Both of them received supportive care. In each case, hematuria and hemolysis were significant events. The patients also showed a decrease in O(2) saturation in spite of high FIO(2). Methemoglobin levels of 40% and 30% were detected by co-oximetry. Neither patient responded to treatment (ascorbic acid in one case, methylene blue in the other). Both patients died due to systemic effects of phosphine poisoning. DISCUSSION AND CONCLUSION: Hemolysis and methemoglobinemia may complicate the course of phosphine poisoning that seems resistant to methylene blue and ascorbic acid. Therefore, other treatments including hyperbaric oxygen therapy and exchange blood transfusion should be considered.