Systemic lupus erythematosus nephritis and COVID-19 disease.

Of the more than 20 studies published on SLE patients with COVID-19, none of the studies focused on lupus nephritis. We report the outcomes of renal biopsy-proven systemic lupus erythematosus (SLE) nephritis patients after COVID-19 disease. Our institute has been declared as a state COVID-19 hospital in the last week of March 2020. From then till now, we have admitted and managed COVID-19 patients from several districts of Andhra Pradesh and neighbouring states. We collected the data of patients with SLE nephritis contemporaneously from admission to the outcomes on a computerised proforma. We had identified sixteen patients with SLE nephritis who were admitted with COVID-19 disease. Of them, fourteen were females and two were males. The mean age was 29.3 years. Out of sixteen patients, seven required a mechanical ventilator and dialysis and eventually succumbed. One more patient died due to disseminated tuberculosis. Our results suggested that with an approximately 50% mortality rate, the COVID-19 disease had a calamitous effect on SLE nephritis patients. Key Points • We identified the significant risk factors for mortality: younger age, higher serum creatinine at presentation, higher CT severity score and lower serum albumin. • After the analysis done for this article, we decided to reduce the medications for SLE nephritis to prednisolone 10 mg/day when COVID-19 disease is contracted.

A Rare Finding of Pancreatic Involvement in a Case of Burkitt's Lymphoma.

Burkitt's lymphoma (BL) is a highly aggressive type of B-cell non-Hodgkin lymphoma. BL commonly occurs in children of age 4-7 years and is uncommon in adults, with a worse prognosis. Patients often present with a rapidly growing mass typically involving the abdomen (liver and spleen) and head and neck (nodes, jaw, and facial bones). Pancreas involvement is very rare and very few case reports have been documented so far. Fluorine-18 positron emission tomography/computed tomography (F-18 PET/CT) is a whole-body survey commonly used for initial staging evaluation. Here, we present an interesting case of BL in an adult female of 43 years, who presented with swelling in the left submandibular region after tooth extraction with multiorgan involvement found on F-18 fluorodeoxyglucose PET/CT.

Pathology of Uddanam Endemic Nephropathy.

In the last decade, pockets of endemic nephropathy have been recognized worldwide, in regions of Central America, Sri Lanka, and India. In India, the nephropathy has been recognized in the Uddanam area of north Andhra Pradesh and has been termed the Uddanam endemic nephropathy (UEN). The disease is distinctive in that besides the geographic distribution, it affects rural populations engaged in farm labor and agriculture, often silent in the initial phase with most patients presenting with advanced renal failure. The renal biopsy findings in all geographic areas including UEN have been one of a chronic tubulointerstitial nephritis with varying degrees of tubular injury, interstitial inflammation, tubular atrophy, and interstitial fibrosis with nonspecific glomerular obsolescence and lack of immune deposits. More recently, the demonstration of dysmorphic lysosomes in renal biopsies has favored a toxic etiology. There are thus many gaps in the understanding of this serious disease prevalent among poorer populations.

Serum tumor markers in advanced stages of chronic kidney diseases.

Chronic kidney disease (CKD) is one of the most important noncommunicable diseases. Abnormal concentration of some tumor markers were found in a spectrum of nonmalignant diseases such as benign ovarian tumors, breast diseases, chronic hepatitis, cirrhosis, diseases of the bile duct, and in CKD. Hence, the present study was undertaken to evaluate carbohydrate antigen (CA) 15-3, carcinoembryonic antigen (CEA), CA 19-9, and human chorionic gonadotropin (HCG) concentrations in advanced stages of CKD (Stage 4 and 5) patients who are not on dialysis and with no known malignancy. Patients included 40 CKD patients and 40 healthy controls. CA 15-3, CEA, CA 19-9, and HCG in serum were estimated by enzyme-linked immunosorbent assay method. The differences in tumor marker levels between the controls and advanced stages of CKD (Stage 4 and 5) were assessed using one-way analysis of variance using the Statistical Package for the Social Sciences for Windows version 16.5. CKD patients had significantly elevated levels of CEA, HCG, CA 19-9, and CA 15-3 compared to the control group (P = 0.001). There was no difference in the tumor markers levels between CKD Stage 4 and 5. Elevation in serum tumor markers may be a possibility in patients with CKD even in the situations of the absence of a malignancy. This may be due to an alteration in their metabolism in CKD and reduction of glomerular filtration rate leading to impaired excretion. Hence, it may be prudent to exercise caution in the interpretation of serum tumor markers as a representative for underlined malignancy in patients of CKD.

Renal infarction due to lupus vasculopathy.

In the ISN/RPS 2003 classification of lupus nephritis (LN) renal vascular lesions are not mentioned. We present a patient with postpartum lupus vasculopathy. The renal biopsy in our patient showed concentric intimal thickening with narrowed lumen. No inflammatory changes were found. It also revealed immunoglobulin and complement deposition on the wall of the arteriole. These changes indicate lupus vasculopathy. The glomeruli revealed diffuse proliferative glomerulonephritis, with wire loops and cellular crescent in one glomerulus. The patient showed improvement with immunosuppression.

Mutations in exons 3 and 7 resulting in truncated expression of human ATP6V1B1 gene showing structural variations contributing to poor substrate binding-causative reason for distal renal tubular acidosis with sensorineural deafness.

Distal renal tubular acidosis (dRTA) is an autosomal recessive syndrome results defect in either proximal tubule bicarbonate reabsorption or in distal tubule H(+) secretion and is characterized by severe hyperchloraemic metabolic acidosis in childhood. dRTA is associated with functional variations in the ATP6V1B1 gene encoding ß1 subunit of H(+)-ATPase, key membrane transporters for net acid excretion of α-intercalated cells of medullary collecting ducts. In the present study, a 13-year-old male patient suffering with nephropathy and sensorineural deafness was reported in the Department of Nephrology. We predicted improper functioning of ATP6V1B1 gene could be the reason for diseased condition. Therefore, exons 3, 4, and 7 contributing active site of ATP6V1B1 gene was amplified and sequenced (Accession numbers: KF571726, KM222653). The obtained sequences were BLAST searched against the wild type ATP6V1B1 gene which showed novel mutations c.307 A > G, c.308 C > A, c.310 C > G, c.704 T > C, c.705 G > T, c.709 A > G, c.710 A > G, c.714 G > A, c.716 C > A, c.717delC, c.722 C > G, c.728insG, c.741insT, c.753G > C. These mutations resulted in the expression of truncated protein terminating at Lys 209. The mutated ATP6V1B1structure superimposed with wild type showed extensive variations with RMSD 1.336 Å and could not bind to substrate ADP leading to non-functional ATPase. These results conclusively explain these mutations in ATP6V1B1 gene resulted in structural changes causing accumulation of H(+) ions contributing to dRTA with sensorineural deafness.

Acute renal failure in a patient with mantle cell lymphoma.

Mantle cell lymphoma is a rare form of B-cell lymphoma. We present a 54-year-old gentleman with mantle cell lymphoma. It was diagnosed based on the demonstration of B-cell antigens CD20 and CD5. It was further confirmed by demonstration of overexpression of cyclin D1 on these atypical lymphocytes in the immunohistochemical staining. He also had acute renal failure and proteinuria. Renal biopsy revealed crescents and lymphomatous infiltration of tubulointerstitium. The presence of infiltrating cells with similar markers in both the lymph node and the kidney confirmed the infiltration of kidney with lymphomatous cells. Our present patient, after a thorough literature search, is found to be the second one with a glomerular lesion and tubulointerstitial infiltration by malignant lymphoma cells.