History Of IgA Nephropathy Research Articles

Case report: Spinal anesthesia for cesarean section in a parturient with Potocki-Lupski syndrome

BackgroundPotocki -Lupski syndrome is an uncommon disorder caused by a micro-duplication in chromosome 17p11.2. Variable clinical manifestations bring troubles to the general and neuraxial anesthesia, including mental retardation, facial dysmorphisms, structural cardiovascular anomalies, scoliosis, and malignant hyperthermia. Until now, the anesthesia management for cesarean section in these patients has not been reported yet.Case presentationHere we present a 23-year-old Chinese parturient with Potocki -Lupski syndrome who underwent elective cesarean section under spinal anesthesia. She was transferred to our hospital in her 40th week of gestation. She had a history of IgA nephropathy for more than three years and was diagnosed with Potocki -Lupski syndrome (17p12p11.2 segment 3.1 Mb repeat) in the 29th week of pregnancy. Amniocentesis showed the fetus had no abnormal autosomes. Preoperative multidisciplinary consultation suggested that she should terminate the pregnancy as soon as possible. She was ASA II. Her BMI was 26.43 kg/m2. Her airway evaluation was normal. Her spine could bend well and her spinal interspace could be touched clearly. We did the single spinal anesthesia at L2-3 interspace and gave 0.5% bupivacaine 1.7 ml. The absolute anesthesia level reached T8. The Apgar score for the newborn infant was 10 for 1st minute, 5th minute, and 10th minute. The vital signs were steady without using any vasoactive drugs. The patient had a good prognosis, and was subsequently discharged from hospital.ConclusionTo date, the case may be the first reported spinal anesthesia for the parturient with Potocki -Lupski syndrome. Although its manifestations are variable, the spinal anesthesia is feasible under careful and comprehensive preoperative evaluation.

Spontaneous Rapid Improvement of Small Intestinal Edema

Question: A 69-year-old woman was admitted to our hospital because of sudden abdominal pain and vomiting. She had a history of IgA nephropathy, hypertension, lipid metabolism abnormality, and cholelithiasis. She had been taking perindopril erbumine and pitavastatin calcium for hypertension. On presentation, she had a mild tenderness in the lower abdomen but no sign of peritoneal irritation or fever.

POS-161 A CASE OF EGPA THAT DEVELOPED DURING REMISSION OF IGA NEPHROPATHY

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis that affects small and medium sized vessels. EGPA is also characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. EGPA is a rare disease, and its prevalence is about 11-13 cases per million. On the other hand, IgA nephropathy is the most common disease found to cause primary glomerulonephritis all over the world. There has been no case report of EGPA who had a history of IgA nephropathy.

A CHALLENGING CASE OF DIFFUSE ALVEOLAR HEMORRHAGE

SESSION TITLE: Fellows Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is commonly seen in the intensive care unit and has a variety of causes including vasculitis. Most commonly, a vasculitis is linked to either antineutrophil cytoplasmic antibodies (ANCA) or an anti-glomerular basement membrane antibody. In rare cases, it manifests in a pauci-immune state. CASE PRESENTATION: 52-year-old male with a past medical history of biopsy confirmed IgA nephropathy, who presented with fatigue and hemoptysis. The patient was noted to be from Cambodia and recently visited his home country. During his hospitalization, patient was noted be hypoxic requiring oxygen supplementation. A CT chest was obtained revealing a left upper lung mass concerning for infection verses malignancy (Figure 1). CT guided biopsy by IR was performed, however, while waiting for the studies to return, patient decompensated and required ICU level of care secondary to massive hemoptysis. He was intubated and bronchoscopy revealed serial aliquots consistent with DAH (Figure 2). Transthoracic echocardiogram revealed severe mitral stenosis secondary to calcifications. Cardiology consultation recommended mitral valve replacement however, patient was in critical condition at this time. The patient’s CT guided left upper lobe biopsy returned positive for alveolar hemorrhage. There was no bacterial or viral infection noted. Additionally, vasculitis workup including ANA, ANCA, MPO, PR3, hepatitis panel, and HIV were all negative. Thoracic surgery was consulted and VATS of the left upper and right upper and lower lobe biopsy was performed. During his hospital course, the patient developed progressive AKI requiring hemodialysis. Open lung biopsies returned positive for focal active capillaritis. There was no specific staining noted for IgA, IgM, or IgG. The patient was started on pulse dose steroids and received seven days of plasma exchange therapy. His clinical status improved and he was discharged from the ICU. DISCUSSION: DAH can be caused from a variety of diseases. Interestingly, our patient had a history of IgA nephropathy and severe mitral stenosis, both of which can cause DAH, however, at the end of his workup and hospital course his diagnosis was pauci-immune vasculitis. This is a rare systemic disease that can cause pulmonary renal syndrome. Pulmonary renal syndrome is most commonly associated with ANCA associated vasculitis, however, in 10% this may be absent. Diagnosis is confirmed by open lung biopsy and immunosuppression is the mainstay of treatment. Patients with a negative ANCA were shown to have a worse renal outcome compared with those that had a positive ANCA, but similar survival rates. CONCLUSIONS: A careful evaluation of DAH must be performed even when the etiology is obvious. The gold standard for a definitive diagnosis is through open lung biopsy and should be performed if the patient can tolerate it. Reference #1: Saladi L, Shaikh D, Saad M, et al. Pulmonary renal syndrome: A case report of diffuse alveolar hemorrhage in association with ANCA negative pauci-immune glomerulonephritis. Medicine (Baltimore). 2018;97(23):e10954. doi:10.1097/MD.0000000000010954 DISCLOSURES: No relevant relationships by Alireza Nathani, source=Web Response No relevant relationships by Sean OConnor, source=Web Response No relevant relationships by Heath White, source=Web Response

Hematopoietic stem cell donor with IgA nephropathy: Challenges and management algorithm

Donor safety is of prime importance in allogeneic hematopoietic cell transplantation. The Worldwide Network for Blood and Marrow Transplantation (WBMT) standing committee on donor issues has issued a consensus statement regarding suitability criteria for related adult donors. This committee recommends that donors with a history of immune-mediated glomerulonephritis and abnormal urine tests should preferably undergo bone marrow harvest, to avoid the theoretical risk of granulocyte colony-stimulating factor (G-CSF) induced immune flare-up. We discuss here a unique situation where a related donor with a history of IgA nephropathy (IgAN) insisted on a peripheral blood stem cell harvest. We propose a management plan for this situation, which posed challenges about donor suitability.

Spontaneous pulmonary hematoma with diffuse pulmonary ossification requiring emergency treatment: a case report

BackgroundPulmonary hematoma is most often caused by thoracic trauma. However, rare cases of pulmonary hematoma without any obvious cause are sometimes reported, when the condition is referred to as spontaneous or idiopathic pulmonary hematoma. Herein, we report a very rare case—to the best of our knowledge, this is the first case of pulmonary hematoma associated with diffuse pulmonary ossification requiring emergency treatment reported in the English literature.Case presentationOur patient was a 44-year-old man with a history of IgA nephropathy and had no history of trauma or anticoagulant use. He presented with the chief complaint of bloody sputum and was referred to our hospital for detailed examination. Chest computed tomography showed a right pleural effusion and a large round mass in the right lower lobe. We performed chest tube drainage of the right thorax and confirmed bloody pleural effusion. Although bronchial artery embolization was performed, the patient’s anemia worsened, and we performed right lower lobectomy. Histopathological examination of the resected specimen showed a hematoma with diffuse pulmonary ossification, although the relationship between the two was unclear. There was no evidence of malignancy or angiitis. Therefore, we made the diagnosis of spontaneous pulmonary hematoma in this case. The postoperative course was uneventful. The patient is currently under observation as an outpatient, and a recent chest X-ray showed no evidence of recurrence.ConclusionWe report a case of spontaneous pulmonary hematoma with diffuse pulmonary ossification. Although the relationship between the two remains unclear, considering the possibility of appearance of new lesions/recurrence, we believe that careful follow-up is necessary for this patient.

Sclerosing Encapsulating Peritonitis in A Peritoneal Dialysis Patient

Clinical Image A 56-year-old male presented with abdominal pain, vomiting and peritoneal irritation signs on physical examination. He had history of IgA nephropathy in peritoneal dialysis since fourteen years ago with multiple episodes of bacterial peritonitis responding to intraperitoneal antibiotics. Void residual urine output. His peritoneal permeability was high requiring automated peritoneal dialysis with elevated glucose load for appropriate volume overload management. He refused hemodialysis repeatedly. In the last year the patient reported anorexia, weight loss and several minor episodes of abdominal pain accompanied by constipation or diarrhea that were blamed on a known diverticular disease. In admission the peritoneal effluent was clear. A CT of abdomen showed diffuse thickening of the peritoneum with linear calcifications and bands of fibrosis that were arranged between the loops of small intestine and forming several compartments which were confined. The laparotomy showed a peritoneum of “carapacelike” appearance (Figure 1), brownish, with a thick fibrous cover that included all intestinal loops (Figures 2 and 3) suggestive of abdominal cocoon due to a sclerosing encapsulating peritonitis. Treatment with adhesiolysis, antibiotics, parenteral nutrition, tamoxifen and hemodialysis was unsuccessful; patient died ten days later because of septic shock.

Bilateral serous retinal detachments associated with IgA nephropathy

Case reportA 41-year-old woman with a bilateral loss of visual acuity and a history of IgA nephropathy. The ophthalmic examination revealed bilateral neurosensory detachments that resolved completely after 4 months of peritoneal dialysis. DiscussionBilateral serous retinal detachments are a rare manifestation of IgA nephropathy, in which the etiology is probably multifactorial and their resolution depends on the underlying disease.

Desprendimientos de retina serosos bilaterales asociados a glomerulonefritis primaria por depósito de Inmunoglobulina A

Case reportA 41-year-old woman with a bilateral loss of visual acuity and a history of IgA nephropathy. The ophthalmic examination revealed bilateral neurosensory detachments that resolved completely after four months of peritoneal dialysis. DiscussionBilateral serous retinal detachments are a rare manifestation of IgA nephropathy, in which the etiology is probably multifactorial and their resolution depends on the underlying disease.

Streptococcus pneumoniae: An Unusual Pathogen Seen In High Vaginal Swab

Streptococcus pneumoniae are Gram positive lanceolate diplococci. In some individuals they are present as normal commensals of the nasopharynx. They cause pneumonia, otitis media, sinusitis, bronchitis and bacterial meningitis. Infection may be exogenous or endogenous in origin. S.pneumoniae however are not a part of the normal vaginal flora. We present a case of a 19 year old primigravida of 25 weeks amenorrhea, who presented with a history of cough since 3 days, abdominal pain since a day and spotting per vagina. She had a previous history of IgA nephropathy, and underwent dialysis and was on oral steroids. S. pneumoniae was isolated from the high vaginal swab culture. As it may predispose to neonatal sepsis, she was started on IV Cefazolin. Due to severe pregnancy induced hypertension and premature rupture of membrane an elective caesarean section was done and a preterm low birth weight live female child was delivered. As the baby had an acute respiratory distress syndrome, feed intolerance, and a raised C- Reactive protein, she was put on a ventilator and given antibiotics. This signifies that S. pneumoniae can be a resident vaginal flora especially in patients with predisposing factors. Patients with high risk of S.pneumoniae infection should be screened and vaccinated to reduce morbidity and mortality outcome for both mother and child.

Obesity-Related Nephropathy Associated with a History of IgA Nephropathy

This report describes the case of a 57-year-old man who underwent a repeated renal biopsy 25 years after the first biopsy in which the diagnosis of IgA nephropathy was made. Although the patient exhibited gradually increasing proteinuria and a slowly progressive renal impairment, the histological findings of the repeat biopsy revealed no evidence of either glomerular inflammatory changes or IgA deposition. Instead, a marked decrease in the glomerular density and hypertrophy of the remnant glomeruli were noted. Almost a complete disappearance of urinary protein excretion by a calorie-restricted diet indicated that the patient's obesity and its related factors may have contributed to the present nephropathic development.

Vogt-Koyanagi-Harada syndrome in two patients with immunoglobulin A nephropathy.

We describe herein 2 patients who developed Vogt-Koyanagi-Harada syndrome in the course of renal biopsy-proven immunoglobulin A (IgA) nephropathy. A 61-year-old man with an 11-year history of IgA nephropathy and a 16-year history of thyroiditis, and a 56-year-old man with a 5-year history of IgA nephropathy developed Vogt-Koyanagi-Harada syndrome. At the time of the eye disease presentation, IgA nephropathy was stable without corticosteroids in both patients. Vogt-Koyanagi-Harada syndrome was successfully treated with intravenous administration of prednisolone tapered from 200 mg daily. Vogt-Koyanagi-Harada syndrome is associated with IgA nephropathy, suggesting a similar autoimmune mechanism for both diseases.