Sickle cell nephropathy with diffuse proliferative lupus nephritis: a case report

<p/> <p>Background</p> <p>Sickle cell nephropathy (SCN) is an important cause of mortality in patients with sickle cell disease. SCA with systemic lupus erythematosus (SLE) is known in children and less common in adults, however diffuse proliferative lupus nephritis (DPLN) with SCN has rarely been reported in adults. It requires early diagnosis and aggressive management.</p> <p>Case presentation</p> <p>We present here a 35 years old lady with sickle cell disease who presented with edema, dyspnoea on exertion, pyuria and had raised s. creatinine of 7 mg%. Her biopsy revealed SCN with DPLN. She is on maintenance hemodialysis after 2 months of diagnosis.</p> <p>Conclusion</p> <p>DPLN with SCN is a rare entity with poor prognosis, which may be overlooked and needs aggressive management.</p

Ahmedabad tolerance induction protocol and chronic renal allograft dysfunction: pathologic observations and clinical implications

<p>Abstract</p> <p>Background</p> <p>Chronic Renal Allograft Dysfunction (CRAD) is responsible for a large number of graft failures. We have abrogated acute T-cell rejections using Ahmedabad Tolerance Induction Protocol (ATIP) with hematopoietic stem cell transplantation (HSCT) under non-myeloablative conditioning pre-transplant. However B-cell mediated rejections and CRAD continue to haunt us. We carried out retrospective analysis of renal allograft biopsies performed in the last 4 years to evaluate the effect of ATIP on CRAD.</p> <p>Materials and methods</p> <p>Biopsies diagnosed as per modified Banff criteria belonged to 2 groups: ATIP under low dose immunosuppression of cyclosporine/Azathioprine/Mycofenolate mofetil+ Prednisolone, subjected to donor leucocyte transfusion, anti-T/B cell antibodies, low dose target specific irradiation, cyclophosphamide, cyclosporin followed by HSCT pre-transplant; controls who opted out of ATIP were transplanted under standard triple drug immunosuppression. Demographics of both groups were comparable.</p> <p>Results</p> <p>Incidence of chronic changes was higher in controls (17.5%) vs. 10.98% in ATIP over a mean follow up of 151.9 months in the former and 130.9 months in the latter. Proteinuria and hypertension were higher in controls (48.4%) vs. ATIP (32.7%) with chronic transplant glomerulopathy, focal global sclerosis in 67.7% in controls vs. 46.7% in ATIP, acute on chronic T/B cell rejection in 51.6% controls vs. 28.1% ATIP, with peritubular capillary C4d deposits in 19.4% controls vs. 1.9% ATIP biopsies. Acute on chronic calcineurin inhibitor toxicity was higher in ATIP (71.9%) vs. 48.4% in controls.</p> <p>Conclusion</p> <p>Chronic immune injury was less with ATIP vs controls as compared to a higher incidence of chronic calcineurin inhibitor toxicity in the former.</p

Co-infusion of autologous adipose tissue derived insulin-secreting mesenchymal stem cells and bone marrow derived hematopoietic stem cells: Viable therapy for type III.C. a diabetes mellitus

Transition from acute pancreatitis to insulin-dependent diabetes mellitus (IDDM) is a rare manifestation of primary hyperparathyroidism caused by parathyroid adenoma because of impaired glucose tolerance and suppresses insulin secretion. We report the case of a 26-year-old male with pancreatic diabetes caused by parathyroid adenoma induced chronic pancreatitis. He had serum C-peptide 0.12 ng/ml, glutamic acid decarboxylase antibody 5.0 IU/ml, and glycosylated hemoglobin (HbA1C) 8.9%, and required 72 IU/day of biphasic-isophane insulin injection for uncontrolled hyperglycemia. We treated him with his own adipose tissue derived insulin-secreting mesenchymal stem-cells (IS-ADMSC) along with his bone marrow derived hematopoietic stem cells (BM-HSC). Autologous IS-ADMSC + BM-HSC were infused into subcutaneous tissue, portal and thymic circulation without any conditioning. Over a follow-up of 27 months, the patient is maintaining fasting and postprandial blood sugar levels of 132 and 165 mg/dl, respectively, with HbA1C 6.8% and requiring 36 IU/day of biphasic-isophane insulin. Co-infusion of IS-ADMSC + BM-HSC offers a safe and viable therapy for type III.C.a Diabetes Mellitus

Successful renal transplantation after recovery from acute disseminated encephalomyelitis in a child with end-stage renal disease

Acute disseminated encephalomyelitis (ADEM), seen mostly in children, is an acute demyelinating disease, affecting mainly the white matter of brain and spinal cord. We report an unusual case of ADEM in an 11-year old boy with endstage renal disease, who underwent hemopoietic stem cell transplantation prior to renal transplantation. He needed admission to the intensive care unit and required mechanical ventilation. He responded to intravenous injection of steroids and upon recovery, underwent renal transplantation successfully

Co-infusion of autologous adipose tissue derived neuronal differentiated mesenchymal stem cells and bone marrow derived hematopoietic stem cells, a viable therapy for post-traumatic brachial plexus injury: A case report

Stem cell therapy is emerging as a viable approach in regenerative medicine. A 31-year-old male with brachial plexus injury had complete sensory-motor loss since 16 years with right pseudo-meningocele at C5-D1 levels and extra-spinal extension up to C7-D1, with avulsion on magnetic resonance imaging and irreversible damage. We generated adipose tissue derived neuronal differentiated mesenchymal stem cells (N-AD-MSC) and bone marrow derived hematopoietic stem cells (HSC-BM). Neuronal stem cells expressed β-3 tubulin and glial fibrillary acid protein which was confirmed on immunofluorescence. On day 14, 2.8 ml stem cell inoculum was infused under local anesthesia in right brachial plexus sheath by brachial block technique under ultrasonography guidance with a 1.5-inch-long 23 gauge needle. Nucleated cell count was 2 × 10 4 /μl, CD34+ was 0.06%, and CD45-/90+ and CD45-/73+ were 41.63% and 20.36%, respectively. No untoward effects were noted. He has sustained recovery with re-innervation over a follow-up of 4 years documented on electromyography-nerve conduction velocity study

Successful renal transplantation after recovery from acute disseminated encephalomyelitis in a child with end-stage renal disease

Acute disseminated encephalomyelitis (ADEM), seen mostly in children, is an acute demyelinating disease, affecting mainly the white matter of brain and spinal cord. We report an unusual case of ADEM in an 11-year old boy with end-stage renal disease, who underwent hemopoietic stem cell transplantation prior to renal transplantation. He needed admission to the intensive care unit and required mechanical ventilation. He responded to intravenous injection of steroids and upon recovery, underwent renal transplantation successfully

Secondary renal amyloidosis in a patient of pulmonary tuberculosis and common variable immunodeficiency

Common variable immunodeficiency (CVID) usually manifests in the second or third decade of life with recurrent bacterial infections and hypoglobulinemia. Secondary renal amyloidosis with history of pulmonary tuberculosis is rare in CVID, although T cell dysfunction has been reported in few CVID patients. A 40-year-old male was admitted to our hospital with a 3-month history of recurrent respiratory infections and persistent pitting pedal edema. His past history revealed 3 to 5 episodes of recurrent respiratory tract infections and diarrhoea each year since last 20 years. He had been successfully treated for sputum positive pulmonary tuberculosis 8 years back. Laboratory studies disclosed high erythrocyte sedimentation rate (ESR), hypoalbuminemia and nephrotic range proteinuria. Serum immunoglobulin levels were low. CD4/CD8 ratio and CD3 level was normal. C3 and C4 complement levels were normal. Biopsy revealed amyloid A (AA) positive secondary renal amyloidosis. Glomeruli showed variable widening of mesangial regions with deposition of periodic schiff stain (PAS) pale positive of pink matrix showing apple green birefringence on Congo-red staining. Immunohistochemistry was AA stain positive. Immunofluorescence microscopy revealed no staining with anti-human IgG, IgM, IgA, C3, C1q, kappa and lambda light chains antisera. Patient was treated symptomatically for respiratory tract infection and was discharged with low dose angiotensin receptor blocker. An old treated tuberculosis and chronic inflammation due to recurrent respiratory tract infections were thought to be responsible for AA amyloidosis. Thus pulmonary tuberculosis should be considered in differential diagnosis of secondary causes of AA renal amyloidosis in patients of CVID especially in endemic settings

Role of 64 slice multidetector computed tomography and angiography to establish relationship between tumor size, aneurysm formation and spontaneous rupture of renal angiomyolipomas: Single center experience

Objective: To evaluate the role of computed tomography (CT) angiography using 64 slice multidetector CT scan to establish relationships among tumor size, aneurysm formation, and spontaneous rupture of renal angiomyolipomas (AML). Materials and Methods: Total 27 patients were diagnosed as having renal angiomyolipoma (AML) at institute of kidney disease and research center from June 2008 to June 2015. All patients with renal AML underwent contrast-enhanced CT (CECT) with CT angiography with 64 slice multidetector CT scan. Kidneys were divided into two groups; unruptured and ruptured AML based on CT findings. The single largest lesion in each kidney was evaluated for tumor size on CECT. CT angiography images were evaluated for size of an aneurysm if present; in each group. Tumor and aneurysm sizes were compared, and multiple regression analysis was performed to identify factors affecting the rupture. Results: Total 34 kidneys were found to be affected by AML. Out of which 6 AML were ruptured and remaining 28 were unruptured. If tumor size of 4 cm or larger is used as predictor of rupture; sensitivity 20%, specificity 89%, positive predictive value 83.3%, and negative predictive value 28.5%; and If aneurysm size >5 mm is used as predictor of rupture; sensitivity 75%, specificity 90%, positive predictive value 50%, and negative predictive value 96.4% was found. Conclusion: Tumor size, aneurysm size and tumor multiplicity cannot use as a predictor of spontaneous rupture of the tumor