Tacrolimus-induced HUS: an unusual cause of acute renal failure in nephrotic syndrome

Acute renal failure (ARF) is an uncommon complication in children with nephrotic syndrome. We report here the case of a 10-year-old male child with primary steroid-resistant nephrotic syndrome who was non-responsive to steroids and cyclophosphamide. A kidney biopsy revealed that he had focal segmental glomerulosclerosis. His treatment was initiated with tacrolimus (dose of 0.15 mg/kg/day) in two divided doses along with prednisolone 60 mg/m²/daily. After 1 month of treatment, he was diagnosed as having acute renal failure secondary to HUS. This was postulated to be due to the tacrolimus therapy, which was withdrawn. Two weeks after stopping the adminsitration of tacrolimus, his urine output improved, and the hemoglobin and serum creatinine normalized. Thus, tacrolimus-induced HUS is a rare cause of ARF in nephrotic syndrome. With the increasing use of tacrolimus in steroid-resistant nephrotic syndrome, the treating physicians need to be aware of this rare, but potentially life-threatening side effect.     

Complications of hidradenitis suppurativa after surgical management: A systematic review and meta-analysis

Hidradenitis suppurativa (HS) is a chronic inflammatory disease that affects the apocrine glands in the axillary, groin, and breast regions, with apparent physiological and psychosocial sequelae. As many studies have reported, surgical management is one of the treatment options. A comprehensive systematic review of surgical treatment for HS is provided in this study. Literature retrieval was conducted on MEDLINE, Embase, the Cochrane Central Register of Controlled Trials (CENTRAL), China National Knowledge Infrastructure (CNKI) and clinical registries from their inception to 1 May 2022. The principles of the PRISMA were adhered to in the process of literature search and the reference lists of the articles retrieved were also checked for further reference. Heterogeneity was assessed in meta-analysis by means of the Q-test and I² index. A total of 13 studies were finally included which involved 535 participants in total. The average estimated complication rate was 11.1% (95% confidence interval [CI], 6.4%–16.9%). The meta-analysis was conducted on pooled complication sample studies and pooled recurrence sample studies. The recurrence was 16.2% (95% CI, 9.1%–24.9%). Subgroup analysis revealed significant differences in HS complications among different closure types. Among different locations, the highest rate of 16.0% (95% CI, 6.8%–28.2%) was multiple locations. Besides, differences were also observed in diverse excision methods and the highest was deroofing 13.9% (95% CI, 2.2%–33.2%). Extensive resection, using skin flaps or skin grafts as a closure method, was associated with a higher incidence of complications. Providers should consider carefully the use of extensive resection or using skin flaps or skin grafts as a closure method so as to minimise the risk of adverse events.     

Minor degree of reversible renal insufficiency: a frequent complication of adult minimal-change nephrotic syndrome.

We report on a group of 27 patients with minimal-change nephrotic syndrome who presented complete recovery from the nephrotic syndrome and the other clinical signs during follow-up. At the start of the examination an elevated serum urea was found in 10 (37%) and of serum creatinine in 4 (15%) patients. Creatinine clearance was decreased in 10 (37%), systolic blood pressure was elevated in 8 (29%) and diastolic in 11 (41%). Interstitial oedema was found in 3, tubular parenchymal degeneration in 4, slight interstitial mononuclear infiltration in 6 and slight interstitial fibrosis in 1 patient. Complete recovery of renal function was seen in all patients.     

Reversible renal failure and nephrotic syndrome without interstitial nephritis from zomepirac

This report describes two patients with the clinical syndrome of reversible renal failure and nephrotic syndrome caused by the nonsteroidal anti-inflammatory agent, zomepirac sodium. What is unique about this report are the pathologic findings on renal biopsy which showed fusion of foot processes consistent with minimal change disease without evidence of an interstitial infiltrate. A cause-and-effect relationship of the disease to zomepirac administration is strongly suggested by the resolution of the renal dysfunction when the drug was stopped and by more than eighteen months of follow-up without evidence of any impairment in renal function.     

Kimura's disease associated with minimal change nephrotic syndrome: A case report

Kimura's disease is a rare disorder that involves regional lymph nodes and the major salivary glands, which become infiltrated by eosinophils and lymphocytes. Renal lesions associated with Kimura's disease are rare. We describe the case of a 60-year-old Japanese woman who first noted a nodular mass in a salivary gland. As the nodule grew, nephrotic syndrome and heart failure developed. A biopsy of the nodule revealed Kimura's disease, and surgical excision was performed. After the operation, the heart failure and nephrotic syndrome, which were diagnosed as minimal change disease on renal biopsy, improved rapidly without steroid therapy. Four months later, the nephrotic syndrome recurred without recurrence of Kimura's disease. The patient showed marked improvement during prednisolone therapy (40 mg/d) and was in complete remission 4 weeks after the initiation of steroid therapy. This case shows that surgical excision and prednisolone therapy are useful for nephrotic syndrome associated with Kimura's disease.     

Surgical treatment of hidradenitis suppurativa: case series and review of the literature

The chronic and relapsing nature of hidradenitis suppurativa leads to physical and psychological damage. The absence of a proven cure further worsens the scenario. Patient 1 was a 28-year-old woman with a 10-year history of abscesses and non healing sinuses with foul-smelling discharge from her axilla, submammary and groin areas. This led to an episode of self-harm due to severe depression. After failed medical therapy, she was referred for surgery with wide excision of the skin and healing by secondary intention. Her wounds were managed by our specialist wound clinic with the use of topical and systemic antibiotics and thus remained free from symptoms. Patient 2 was a 32-year-old woman with a similar history for 15 years. Patient 3 was a 41-year-old man with a 20-year history of discharging sinuses and abscesses. All the patients had endured a long period of medical treatment and subsequently required surgery for a long-term relief of symptoms. This has undoubtedly led to psychological symptoms and a decrease in quality of life.     

Reconstruction for axillary hidradenitis suppurativa using one‐stage local tissue rearrangement: A retrospective analysis of 53 cases

Wide local excision offers a potential cure for severe axillary hidradenitis suppurativa. However, the gold standard for reconstruction has yet to be defined. Here, we describe our rotation advancement flap technique, which allows for one‐stage closure of large axillary defects, with minimal functional morbidity to the axilla. We performed a retrospective review of all patients who underwent one‐stage surgical management for axillary hidradenitis suppurativa at a single‐centre tertiary care hospital from 2009 to 2018. We identified 34 patients, with a total of 53 operative sites. The majority were female (85%) with a mean age of 31 years and body mass index 35 kg/m². The median defect size was 84 cm² and the majority were treated using the rotation advancement flap technique (86%). A quarter of operative sites experienced minor complications with only one requiring re‐operation. At a median follow‐up of 32 months, two (4%) sites showed decreased range of motion. However, all patients had achieved remission without any further recurrence of disease. We describe a one‐stage rotation advancement flap technique for management of moderate to severe hidradenitis suppurativa that achieves a high local cure rate, minimal functional morbidity, and acceptable wound complication rates.     

Synchronous carcinomas of stomach and bladder together with AA amyloidosis

Although the association and causality between chronic inflammatory states and systemic AA amyloidosis have been well established, the evidence linking solid malignancies to reactive AA amyloidosis is scarce. Here, a case of diagnosed AA amyloidosis associated with synchronous carcinomas of stomach and bladder complicated with nephrotic syndrome and renal failure is reported.     

Acute renal failure in children with idiopathic nephrotic syndrome

Acute renal failure (ARF) is an uncommon but alarming complication of idiopathic nephrotic syndrome. The renal failure could be secondary to causes evident from the history and evaluation, such as severe intravascular volume depletion, acute tubular necrosis, allergic interstitial nephritis, bilateral renal vein thrombosis, acute pyelonephritis, or rapid progression of the original glomerular disease. It may be termed idiopathic if the underlying cause is undetermined. We present three children with idiopathic nephrotic syndrome who were admitted with acute renal failure. One case was due to drug-induced allergic interstitial nephritis. The other two were idiopathic in nature. Improvement in renal function occurred in the three patients over a variable period of 10 days to 4 weeks. After careful exclusion of well-known causes of acute renal failure, idiopathic acute renal failure (IARF) should be considered as a diagnostic possibility in these patients. The exact pathophysiology of IARF is not understood. Possible proposed explanations include interstitial edema, tubular obstruction, altered glomerular permeability, and unrecognized hypovolemia.     

Infliximab and nephrotic syndrome.

Infliximab is a chimeric tumor necrosis factor-alpha (TNF-alpha) monoclonal antibody, which has been used extensively in patients with rheumatoid arthritis and inflammatory bowel disease. It also appears to be effective in other conditions such as psoriasis and ankylosing spondylitis. The major side effect of infliximab is infection. Renal complications are uncommon and not well recognized. This report describes a probable case of infliximab-induced membranous nephropathy.     

Autoimmune thyroiditis and vitiligo in a child with minimal change nephrotic syndrome

We report a 12-year-old child with multiple autoimmune components consisting of minimal change nephrotic syndrome (MCNS), Hashimoto’s thyroiditis and vitiligo. This boy presented with MCNS, complicated by acute renal failure, and subsequently developed Hashimoto’s thyroiditis and vitiligo. Conventional prednisone treatment did not induce remission, but multiple high dose infusions of methylprednisolone led to remission. The occurrence of nephrotic syndrome, Hashimoto’s thyroiditis and vitiligo suggests that similar immunological mechanisms may be involved in the pathogenesis of these diseases. Received: 9 February 2001 / Revised: 10 July 2001 / Accepted: 12 July 2001     

Renal involvement in systemic amyloidosis: an Italian collaborative study on survival and renal outcome.

BACKGROUND: Few data are available from large population-based studies on survival and renal outcome of patients with renal involvement and different types of systemic amyloidosis. METHODS: Two hundred and ninety of over 373 patients affected from systemic amyloidosis with renal involvement diagnosed in Italy between January 1995 and December 2000 were followed from diagnosis to death or until the last available clinical control. Eighty-three patients were excluded from analysis either because the amyloid type remained undetermined or they were lost at follow-up. Clinical and laboratory information was collected according to the different types of amyloidosis using a specific form which included renal function with 24 h proteinuria at diagnosis and at the end of follow-up, the type and the date of onset of dialysis and the kind of treatment they underwent. RESULTS: The median time of follow-up was 24 months in primary (AL) amyloidosis (range: 1-88 months), 16 months in AL with associated multiple myeloma (MM + AL: range 1-76 months), 30 months in reactive (AA) amyloidosis (range: 1-99 months) and 52 months in patients with familial forms (AF: range 14-82 months). Patients with AL showed a significantly shorter survival than AA. Despite no significant differences of renal outcome or survival on dialysis being observed between the two groups, a lower renal survival with a higher number of patients who progressed to end-stage renal disease (ESRD) was observed in patients with AA. Overall survival was markedly improved in patients with AL who underwent a specific therapy (conventional chemotherapy or autologous stem cell transplantation (ASCT)) even in the absence of a positive kidney response. Multivariate analysis showed cardiac involvement and specific therapy to significantly influence survival in AL whereas age, serum creatinine (sCr) and heart involvement significantly affected survival in AA. In both groups, sCr and heart involvement were the most relevant predictors for renal outcome, together with urinary protein excretion, in patients with AA. CONCLUSIONS: Our results show a worse survival in AL due to the higher prevalence of heart involvement in this group and emphasize that a specific therapy significantly prolongs survival and slows the progression of renal disease in patients with AL. We suggest that a late nephrological referral is likely the cause of the higher sCr found at presentation in patients with AA and probably accounts for the lower renal survival observed in the short term in these patients. At the time being, renal transplantation and ASCT are still rare therapeutic options for renal patients affected from systemic amyloidosis.     

Congenital microcephaly and infantile nephrotic syndrome — a case report

A 22-month-old girl with nephrotic syndrome and microcephaly is described. The had dismorphic facies and psychomotor retardation. Her parents were first-degree relatives and one of her siblings had died with nephrotic syndrome and renal failure in infancy. An autosomal recessive inheritance is suggested. The diagnosis of this rare combination is discussed and the relevent literature is reviewed.     

An unusual cause of nephrotic syndrome and hypertension in a young woman.

Introduction The most common causes of adult nephrotic syndrome in our countryare focal glomerulosclerosis, membranous nephropathy and minimal-changedisease [1]. There are, however, less common causes to be considered[2–8]. We describe a 15-year-old Spanish woman with nephrotic syndromesecondary to pre-eclamptic nephropathy in association with atotal hydatidiform mole, in whom pregnancy was not initiallyconsidered. Case A 15-year-old Spanish woman was admitted to our hospital becauseof a 4-week history of asthenia, anorexia, and progressive oedemaof her face, hands, and lower extremities. Two weeks beforeadmission she had developed acute back pain without urinaryor digestive symptoms. After an unsuccessful trial of non-steroidalanti-inflammatory drugs, she was brought to our hospital. Atadmission she had vaginal bleeding, which was interpreted asnormal menstruation, starting a few     

Axillary hidradenitis suppurativa: wide excision and flap coverage is best

Summary The extent of axillary excision and method of closure in 17 patients (29 axillae) with symptomatic chronic axillary hidradenitis suppurativa was analyzed. Limited excision of only the diseased skin led to recurrence in 2 of 3 patients, and excision of the hair-bearing skin alone, in 2 of 5 patients. There was no recurrence in 11 patients after wide excision. Some patients had more than one procedure. Three of four patients who had skin grafts used for closure, developed axillary contractures. Primary closure was achieved successfully in 7 patients (14 axillae). Flap coverage was used to close 12 axillae in 9 patients (3 patients had both axillae treated). These flaps included Limberg 4; random fasciocutaneous 3; parascapular fasciocutaneous: 5. Our experience suggests that wound closure after wide excision in the axilla is best achieved by means of a flap. While smaller defects can be closed with random fasciocutaneous flaps, wide axillary excision often leads to a large defect. The fasciocutaneous parascapular flap should be considered where a large defect exists.     

Neuroblastoma presenting with the nephrotic syndrome.

A case of neuroblastoma in a 4-yr-old boy presenting with the nephrotic syndrome is reported. Nether thrombosis nor stenosis of the renal veins and the inferior vena cava was present. Electron microscopy revealed lumps of subepithelial deposits as well as thickening and tortuosity of the glomerular basement membrane compatible with membranous nephropathy. It is postulated that deposition of neuroblastoma-associated immune complexes on the glomerular basement membrane-was responsible for the development of the nephrotic syndrome in this patient. Five pediatric cases of nephrotic syndrome associated with extrarenal neoplasia were collected from the world literature and are briefly discussed.