Protected: Therapeutic Plasma Exchange in a Patient with Chronic Hemodialysis and a New Diagnosis of Myasthenia Gravis

Abstract

Case Report. C.S.T. (♂, 71 years old) is a patient with multiple and severe comorbidities, undergoing thrice-weekly chronic hemodialysis since 2008 due to the progression of post-lithiasic uropathy. Over the past 2 months, the patient had been experiencing progressive ptosis of the eyelids, muscle weakness, and ultimately dysphagia and dysarthria that emerged in the last few days. Urgently admitted to the Neurology department, electromyography (EMG) was performed, leading to a diagnosis of predominant cranial myasthenia gravis (with borderline anti-acetylcholine receptor antibody serology). Prompt treatment with pyridostigmine and steroids was initiated.
Considering the high risk of acute myasthenic decompensation, therapeutic plasma exchange (TPE) with centrifugation technique was promptly undertaken after femoral CVC placement. TPE sessions were alternated with hemodialysis. The patient’s condition complicated after the third TPE session, with septic shock caused by Methicillin-Sensitive Staphylococcus Aureus (MSSA). The patient was transferred to the Intensive Care Unit (ICU). Due to hemodynamic instability, continuous veno-venous hemodiafiltration (CVVHDF) with citrate anticoagulation was administered for 72 hours.
After resolving the septic condition, intermittent treatment with Acetate-Free Biofiltration (AFB) technique was resumed. The patient completed the remaining three TPE sessions and, once the acute condition was resolved, was transferred back to Neurology. Here, the patient continued the treatment and underwent a rehabilitation program, showing significant motor and functional recovery until discharge.
Conclusions. The multidisciplinary interaction among Nephrologists, Neurologists, Anesthesiologists, and experts from the Immunohematology and Transfusion Medicine Service enabled the management and treatment of a rare condition (MG) in a high-risk chronic hemodialysis patient.

Keywords: Myasthenia Gravis, Plasmapheresis, Therapeutic Plasma Exchange, Hemodialysis, Continuous Renal Replacement Therapy

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Protected: New Mutation of CYP24A1 in a Case of Idiopathic Infantile Hypercalcemia Diagnosed in Adulthood

Abstract

Mutations in the 24-hydroxylase gene CYP24A1 have been recognized as causes of childhood idiopathic hypercalcemia (IIH), a rare disease (incidence <1:1,000,000 live births) [1] characterized by increased vitamin D sensitivity [2], with symptomatic severe hypercalcemia.
IIH was first described in Great Britain two years after the start of a program of vitamin D supplementation in milk for the prevention of rickets, manifesting in about 200 children with severe hypercalcemia, dehydration, growth failure, weight loss, muscle hypotonia, and nephrocalcinosis [3].
The association between the epidemic occurrence of IIH and vitamin D administration was quickly attributed to intrinsic hypersensitivity to vitamin D [4], and the pathogenic mechanism was recognized in the inactivation of Cytochrome P450 family 24 subfamily A member 1 (CYP24A1), which was identified as the molecular basis of the pathology [5].
The phenotypic spectrum of CYP24A1 mutation can be variable, manifesting predominantly with childhood onset and severe symptomatology (severe hypercalcemia, growth retardation, lethargy, muscle hypotonia, dehydration), but also with juvenile-adult onset forms with nephrolithiasis, nephrocalcinosis, and alterations in phosphocalcium homeostasis [6].
We describe the case of a patient in whom the diagnosis of IIH was made in adulthood, presenting with finding of nephrocalcinosis in childhood, and with subsequent onset of severe hypercalcemia with hypercalciuria, hypoparathyroidism, hypervitaminosis D, and recurrent renal lithiasis.
Genetic investigation revealed the presence in homozygosity of the c_428_430delAAG_p.Glu143del variant in the CYP24A1 gene with autosomal recessive transmission, a mutation not reported in the literature.

Keywords: CYP24A1, Hypercalcemia, Infantile Idiopatic Hypercalcemia, Vitamin D, Nephrocalcinosis

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Protected: Congenital Nephrotic Syndrome: Role of Podxl Gene

Abstract

In the last decades, our understanding of the genetic disorders of inherited podocytopathies has advanced immensely; this has been possible thanks to the development of next-generation sequencing technologies that offer the possibility to evaluate targeted genes at a lower cost than in the past. Identifying new genetic mutations has helped to recognize the key role of the podocyte in the health of the glomerular filter and to understand the mechanisms that regulate the cell biology and pathology of the podocyte. Here we describe a patient with congenital nephrotic syndrome due to a mutation in PODXL. This gene encodes podocalyxin, a podocyte-specific surface sialomucin known to maintain the characteristic architecture of the foot processes and the patency of the filtration slits.

Keywords: Proteinuria, Congenital nephrotic syndrome, Podocyte, PODXL, Podocalyxin

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Protected: Type I Hyperprolinemia – What about the Kidney?

Abstract

Hyperprolinemia is a rare genetic condition due to mutations in proline metabolic pathway. Type I Hyperprolinemia (HPI) typically causes neuropsychiatric disorders, and diagnosis is usually confirmed in pediatric population with suggestive neuropsychiatric involvement by elevated serum proline levels and elevated urinary proline, hydroxyproline, and glycine levels.
The possible coexistence of nephropathy in patients with HPI, often specified as malformative urinary disease, is often mentioned. However, reports of HPI diagnosis due to kidney impairment do not exist in scientific literature yet.
Here we present the case of a patient presenting with chronic kidney disease secondary to obstructive nephropathy who received a HPI diagnosis in adulthood.
Interestingly, the family study showed the same 22q11.21 deletion and elevated blood proline levels in the father, who had no clinical anomalies.
We therefore suggest, in light of the high frequency of mutations involving 22q11 and PRODH in the general population, to consider these rare alterations in patients with congenital urinary malformations, even in the presence of nuanced neurological symptoms and negative family history.

Keywords: hyperprolinemia, type I hyperprolinemia, genetic nephropathy, malformative urinary disease

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Contrast Induced Encephalopathy after carotid percutaneous transluminal angioplasty in a patient with end stage renal disease undergoing peritoneal Dialysis

Abstract

Introduction. Contrast Induced Encephalopathy (CIE) belongs to Major Adverse Renal and Cardiovascular Events (MARCE) after iodinated contrast medium (IOCM), especially for high-risk patients with several comorbidities such as hypertension, diabetes, heart failure, and Chronic Kidney Disease (CKD). We report a case of CIE in a Peritoneal Dialysis (PD)-patient.
Case report. A 78-year-old, affected by diabetes, hypertension, chronic heart failure, and End Stage Renal Disease (ESRD) treated with PD, underwent a carotid Percutaneous Angioplasty (PTA). Immediately after the exam, he developed mental confusion and aphasia. Encephalic CT scan and MRI excluded acute ischemia or hemorrhage but showed cerebral oedema.  Mannitol and steroids were administered and additional PD exchange was performed with depurative aim. Within 2 days the patient completely recovered.
Discussion. CIE mimics severe neurological diseases. It should be considered as a differential diagnosis if symptoms occur immediately after administration of IOCM, especially in high-risk patients and in case of intra-arterial injection. Clinical presentation includes transient cortical blindness, aphasia, focal neurological defects, and confusion. CIE is often a diagnosis of exclusion, and imaging plays a significant role. Symptoms generally resolve spontaneously within 24-48h, rarely in few days. Symptomatic therapy, including mannitol and steroids could be considered. In literature, CIE is reported only in a few patients affected by ESRD treated with chronic HD, and our is the first available case of a patient treated with chronic PD who developed this rare complication.

Keywords: Peritoneal Dialysis, Contrast-induced encephalopathy, contrast medium

Sorry, this entry is only available in Italian.

Introduzione

I mezzi di contrasto organo-iodati (Iodinated Contrast Medium, IOCM) sono largamente utilizzati per procedure diagnostiche e interventistiche. È risaputo che il loro uso si associa ad un aumentato rischio di eventi avversi maggiori cardiovascolari e renali (Major Adverse Renal and Cardiovascular Events, MARCE), soprattutto nelle persone ad alto rischio per la presenza di più comorbidità, come ipertensione arteriosa, diabete mellito, scompenso cardiaco e insufficienza renale cronica (Chronic Kidney Disease, CKD).
I MARCE comprendono sia eventi maggiori renali (Major Adverse Kidney Events, MAKE) – che includono il peggioramento della funzione renale, la necessità di avvio del trattamento dialitico – sia eventi cardiovascolari maggiori (MACE, Major Adverse Cardiac Event) tra cui l’infarto miocardico, l’ictus, lo scompenso cardiaco, fino al decesso [1].
Il rischio di eventi avversi si è dimostrato significativamente più basso con volumi minori di mezzo di contrasto, utilizzando mezzi di contrasto iso-osmolari, rispetto a mezzi di contrasto di tipo ipo- o iper-osmolare, e in caso di somministrazione endovenosa piuttosto che intra arteriosa: tale rischio è aumentato per i pazienti con funzione renale già compromessa e/o diabete [24].
Tra gli eventi avversi maggiori legati all’utilizzo di IOCM più rari, ma potenzialmente gravi, vi è l’encefalopatia indotta da mezzo di contrasto (Contrast-Induced Encefalopathy, CIE) [5, 6]. 

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Kidney Transplant in a Highly Sensitized Patient Treated with Imlifidase

Abstract

Through a clinical case, we will describe the difficulties associated with providing transplantation opportunities to highly immunized patients. We will therefore focus on new desensitization therapies and their pharmacological effects with the consequent improvement in clinical outcomes. The main desensitization strategies in use and the main future therapeutic prospects will also be discussed.

Keywords: Imlifidase, Kidney Transplant, Hyperimmune Patients

Sorry, this entry is only available in Italian.

Introduzione

Il numero di pazienti affetti da malattia renale allo stadio terminale è in forte aumento in tutto il mondo, sia nei paesi sviluppati che in quelli in via di sviluppo [1]. Tra questi molti risultano idonei al trapianto di rene, opzione che rispetto alla dialisi si associa a una qualità di vita e una sopravvivenza significativamente migliori. La presenza di un quadro di sensibilizzazione e il conseguente sviluppo di anticorpi rivolti contro antigeni leucocitari umani (HLA) di classe I e/o II rendono difficile trovare un aplotipo compatibile in caso di trapianto. Elevati livelli di anticorpi donatore specifici (HLA DSA) sono correlati con un maggiore rischio di rigetto iperacuto che può essere ulteriormente incrementato in seguito a numerosi eventi come: emotrasfusioni, pregressi aborti o gravidanze [24]. Per questo motivo, nonostante un marcato aumento del numero di allotrapianti da donatore vivente (DLA), molti potenziali riceventi con donatori idonei sono relegati nelle liste di attesa per la presenza di anticorpi anti antigene leucocitario umano preformati. Per rilevare rapidamente la presenza di anticorpi nel siero del ricevente rivolti contro linfociti isolati dal sangue del donatore, sono state sviluppate procedure di crossmatch (CM) che consentono un’efficace valutazione della sopravvivenza a breve termine degli alloinnesti renali.  La valutazione dello stato di pre-sensibilizzazione di un paziente in lista d’attesa per trapianto, avviene o tramite la tecnica di citotossicità complemento-mediata (CDC) o attraverso tecniche di fase solida (SPI) [5]. Indipendentemente dalla tecnica utilizzata, la percentuale di sensibilizzazione di un paziente nei confronti delle molecole HLA viene stimata attraverso il PRA (Panel Reactive Antibody), un test di fissazione del complemento attraverso cui viene testata la capacità del siero del ricevente di lisare un pannello di cellule T da un gruppo di potenziali donatori. 

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Autosomic Dominant Tubulo Interstitial Kidney Disease: Case Report of a New Variant of the UMOD Gene

Abstract

Autosomal dominant tubulointerstitial kidney disease (ADTKD) is a low-prevalence pathology mainly associated with pathogenic variants of the UMOD gene. It is characterized by the progressive deterioration of renal function, associated with hyperuricemia and accompanied by a family history of gout or hyperuricemia. Often, clinical variability and a lack of molecular testing results in diagnostic failure to determine the ADTKD-UMOD association.

Case presentation: We describe the case of a 14-year-old male who presented to the nephrology service with hyperuricemia, renal ultrasonographic changes, and progression to chronic kidney disease in 4 years. He had a family history of hyperuricemia. A probable genetic disease with an autosomal dominant inheritance pattern was considered, confirmed by the presence of a probably pathogenic variant of the UMOD gene, not previously reported in the literature.

Conclusion: The investigation of this case led to the identification of a new variant in the UMOD gene, broadening the spectrum of known variants for ADTKD-UMOD. In addition, in this case, a comprehensive anamnesis, that takes into account family history, was the key point to carry out genetic tests that confirmed the diagnosis suspicion. Directed Genetic tests are currently an essential diagnostic tool and should be performed as long as they are available and there is an indication to perform them.

Keywords: UMOD, Uromodulin, hyperuricemia, Uric acid, Familial Juvenile Hyperuricemic Nephropathy, case report

Introduction

Interstitial nephropathies (IN) compromise not only the interstitial tissue of the kidney, but also the renal parenchyma, affecting the glomerulus, tubules and blood vessels at the renal level [1, 2]. Autosomal dominant tubulointerstitial kidney disease (ADTKD) was recently introduced in the KDIGO (Kidney Disease: Improving Global Outcomes) guidelines [3], and includes genetic disorders with high penetrance (~100%); however, few cases have been reported in unaffected heterozygous individuals [4]. To date (April 2023), five genes associated with the disease have been described: UMOD (ADTKD-UMOD), REN (ADTKD-REN), MUC1 (ADTKD-MUC1), HNF1B (ADTKD-HNF1B) and SEC61A1, the last one still without clear clinical relevance and defined outcome [13, 5].

Regarding frequency, the main cause of ADTKD is secondary to pathogenic variants in the MUC-1 gene (Spain 42.5% and Ireland 64%), followed by the UMOD gene (35%), and in third place HNF1B gene variants (13.9%). So far, only 14 affected families have been reported in the literature with REN gene-related ADTKD [6, 7].

The clinical profile can be heterogeneous according to the variant and age group, even within the same families. Different levels of proteinuria, urinary sediment, and microscopic hematuria could be present [2]; despite this, the progressive decrease in renal function that leads to end-stage chronic kidney disease (ESKD) seems to be a characteristic sign [1, 7], which could be; depending on the genetic variant [3, 7, 8], also accompanied by bilateral renal hypoplasia [1]. 

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Collapsing Glomerulopathy Secondary to Anabolic Steroids for Bodybuilding: A Case Series

Abstract

The abuse of anabolic androgenic steroids (AAS) for competitive (and non-competitive) purposes for bodybuilding practice is increasingly common. The consequences of these substances on the various organs are only partially known. Cases of FSGS following the use of AAS have been reported in the literature, even with evolution to ESKD.
We describe three cases of bodybuilding athletes who presented alterations in renal function indices after taking AAS for a long time. Three renal biopsies were performed with histological diagnosis of FSGS collapsing variant. We examine the lesions observed on histological examination. Two athletes had rapid progression of renal disease requiring replacement therapy. The third one continues conservative treatment for chronic renal failure.
We discuss the risks related to the intake of doping substances and how bodybuilders are exposed to different causes of kidney damage: anabolic steroids, supplements, and a high-protein diet.

Keywords: anabolic-androgenic steroids, bodybuilding, FSGS, collapsing variant, chronic kidney disease

Sorry, this entry is only available in Italian.

Case series

Descriviamo di seguito tre casi clinici di pazienti bodybuilder a livello agonistico, che si sono attenuti per anni ad un regime dietetico iperproteico e hanno fatto uso di steroidi anabolizzanti e integratori durante la preparazione atletica. I pazienti in esame, tutti di etnia caucasica, presentavano recente riscontro di insufficienza renale e non assumevano farmaci. L’anamnesi familiare era negativa per patologie renali. In tutti e tre i casi la sierologia per l’autoimmunità risultava negativa e si escludevano infezioni (incluso infezione da HIV). 

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Treatment of a Severe Form of Euglycemic Ketoacidosis in a Patient Treated with SGLT-2 Inhibitors with the Aid of Somatostatin

Abstract

Currently, the use of SGLT2 inhibitors is becoming more widespread, both for their role in controlling diabetes, and for their pleiotropic effects on glomerular hyperfiltration and heart failure. Along with their positive effects, these drugs can lead to various complications, the most severe being euglycemic ketoacidosis. The clinical case we have reported precisely describes this potentially serious complication which occurred in a 47-year-old patient who had been on SGLT2 inhibitor therapy for 5 years. In the resolution of this case we used, in addition to standard therapy, the continuous infusion of somatostatin, resulting in a rapid resolution of ketoacidosis and an improvement in the clinical condition.

Keywords: SGLT-2, somatostatin, euglycemic ketoacidosis

Introduction

Euglycemic ketoacidosis was first described by Munro in 1973 [1]. It differs from classical DKA due to glycaemic levels below 200 mg/dl [2].

In their genesis, these forms may present an exogenous and/or endogenous insulin deficiency, associated with a reduction in caloric intake and fluids, inducing the increase of insulin counter-regulatory hormones (cortisol, glucagon, catecholamines, etc.). Glucagon promotes glycogenolysis but in the absence of glycogen deposits, it stimulates adipocytes to lipolysis, achieving an increase in free fatty acids that promote ketogenesis in the liver. SGLT2 inhibitors (SGLT2i) are also able to stimulate, in addition to the release of glucagon, the resorption of ketones in the renal tubules to further increase the concentration of ketone bodies [3]. For this reason, even in the presence of normoglycemia in subjects who use these drugs and who have symptoms compatible with a state of acidosis (lack of appetite, nausea, vomiting, tachypnoea, and mental confusion), it seems appropriate to look for the presence of ketones in the urine. In the 1980s, to counteract this effect, a therapy aimed at reducing hyperglycaemic levels through the administration of somatostatin [4] was hypothesized. In fact, the drug was indicated as an adjuvant to the treatment of diabetic ketoacidosis. The most marked benefits seemed to be realized in those forms of DKA where a kind of vicious circle amplified the effects of the glucagon [5]. Over the years, its use became less frequent, both for the improvement of insulin therapies and for the early diagnosis of diabetic disease.

 

Mechanism of euglycemic ketoacidosis with SGLT2i and Somatostatin action

Figure 1. Organs and pathological mechanisms involved in euglycemic ketoacidosis.
Figure 1. Organs and pathological mechanisms involved in euglycemic ketoacidosis.

SGLT2 inhibitors reduce blood sugar levels by increasing urinary glucose excretion, which in turn reduces insulin secretion from pancreatic beta cells. The decline of the circulating insulin levels results in a diminution of the anti-lipolytic activity of insulin and the consequent stimulation of the production of free fatty acids, which are converted into ketone bodies by β-oxidation in the liver (Figure 1) [3]. The decline of the circulating level of insulin promotes the production of ketone bodies. SGLT2 increase glucagon levels. It is unclear whether SGLT2 directly [6] affects glucagon secretion; the increase of glucagon is probably indirect, and it is mediated by the reduction of the secreted insulin while a direct stimulus on the alpha pancreatic cells has never been demonstrated [7].

 

Clinical Case

A 47-year-old woman arrived in the emergency room with strong back pain and polypnea and said she already had it for two weeks with blood pressure of 125/70 mmHg and H.R. 96 b/min. The patient informed that she suffers from type 2 diabetes mellitus since the age of 30. During the anamnesis, she reports to be on treatment with Metformin 1000 mg x 2 and 7 U.I. of Insulin Deglutec to which, in the last 5 years, had been added dapagliflozin 10 mg. A few days before, she did blood chemistry tests, which revealed a 9% glycated haemoglobin. This value led the patient to reduce the caloric intake and dosage of Deglutec insulin from 7 to 3 units. The patient had not had fever, vomiting or diarrhea in the previous days. The tests carried out in the emergency room showed a blood sugar of 150 mg/dl and a normal renal function (Table 3 – exams at time T0), but performing an ABGA, it was found pH 6.9, HCO3 1 mmol/l, K+ 3.4 mmol/l, and lactates 1 mmol/L so that 15 ampoules of 10 meq/10 ml bicarbonate were administered.

Presuming it was caused by metformin toxicity, the case was brought to the notice of the nephrology department which, comparing the pharmacological history to the normality level of lactates, quickly made the diagnosis of euglycemic DKA, with high Anion Gap (33.5), secondary to therapy with SGLT2i. An immediate urine test was carried out showing an off-scale glycosuria much higher than 2000 mg/dl, with ketonuria higher than 80 mg/dl. The nephrologist, after the suspension of dapagliflozin, added moisturizing therapy with physiological in glucose 10% and insulin in continuous infusion [8]. After about 10 hours in Emergency with a total infusion of about 2.5 l of liquids, the ABG showed a slight increase in serum bicarbonates, pH slightly above 7, and K+ of 2.9 mmol/l with an extension of QT. Two ampuls of KCL and one of magnesium sulfate were added. Numerous ABGs were repeated, showing a situation that could be superimposed on the previous one, with the continuation of a situation of severe polypnea and back pain.

pH HCO3

mmol/l

Lactate

mmol/l

Na

mmol/l

K

mmol/l

CL

mmol/l

Glycemia

mg/dl

6.9 1 1 141 4.3 111 177
7.1 2.8 3.3 144 4.2 111 166
7.009 1.8 2.0 144 3.6 112 160
7.039 1.9 1.9 144 3.5 113 143
7.04 2 1 134 2.9 113 86
7.08 1.4 1 139 3.5 113 104
7.09 4.6 1 135 3.2 113 101
7.08 3.7 1 135 2.9 112 99
7.05 2.5 1 138 3.0 109 170
Table 1. Serial EAB performed during hospitalization in the Emergency Department.

After the administration of 40 ampoules of sodium bicarbonate and with abundant hydrating therapy, the hemodilution had reduced the urate to 2.1, the creatininemia to 0.47 and the Urea to 23 mg/dl (Table 3. Exams at time T1), but the pH (7.05) was still altered so that hydration alone seemed insufficient to resolve the complication. It was decided therefore to transfer the patient to the department of nephrology where the administration of Somatostatin with a dose of 500 mcg/h [9] was started.

After starting the administration of Somatostatin, we find the following values in the EAB:

pH HCO3

mmol/l

Lactate

mmol/l

Na

mmol/l

K

mmol/l

CL

mmol/l

Glycemia

mg/dl

7.17 4.7 0.5 133 3 110 139
7.18 8.2 0.5 135 3.2 111 166
7.21 7.7 0.5 135 3 112 170
7.24 13.4 0.5 135 2.8 112 173
7.32 18.1 0.5 134 3.3 110 201
7.34 24.1 0.5 138 3.6 110 162
Table 2. Serial EAB performed during hospitalization in Nephrology.

While suspending the infusion of bicarbonates due to the presence of hypokalaemia, after about 12 hours from the beginning of the administration of somatostatin, a pH of 7.24 and bicarbonate of 13.4 mmol/l with the disappearance of back pain and polypnea was observed. Continuing somatostatin for the other 24 hours, the pH was 7.32 and HCO3 18.1 mmol/l. In all nine days of hospitalization, the patient continued to have glycosuria higher than 2000 mg/dl and ketonuria higher than 80 mg/dl [10] and reported on the third day the biochemical values highlighted in Table 3 at time T2. The patient was discharged with a pH of 7.34 and HCO3 of 24 mmol/l.

On discharge he had the biochemical values shown in Table 3 at time T3.

Timing Creatinine 0.7 – 1.2 Azotemia v.n. 10 – 50

 

Hemoglobin 13 – 17 Ac. Uric v.n. 3.4 – 7
T0 0.74 mg/dl 23 mg/dl 14 g/dl 4 mg/dl
T1 0.59 mg/dl 18 mg/dl 11.4 g/dl 3.6 mg/dl
T2 0.47 mg/dl 16 mg/dl 11.1 g/dl 2.4 mg/dl
T3 0.33 mg/dl 24 mg/dl 11.1g/dl 1.8 mg/dl
Table 3. Biochemical values recorded in hospitalization timings.

 

Discussion

The euglycemic ketoacidosis achieved in this case report requires significant consideration, both regarding the selection of patients for whom SGLT2i treatment is appropriate, and the need to implement a therapeutic protocol for this complication that, in rare cases, can be fatal. The administration of Somatostatin with a dose of at least 500 mcg/h [9] in combination with the use of glucose, insulin, hydration, and KCL seems to be an excellent cure for treating this complication [3]. The use of bicarbonates, however, does not improve the clinical picture but promotes the appearance of hypokalaemia; therefore, it is recommended only in cases of pH lower than 7. The prolonged presence of ketonuria higher than 80 mg/dl highlights a prolonged effect of glucagon with the consequential advantage of the use of somatostatin in these forms of euglycemic DKA. The use of this drug has speeded up, in the reported case, the healing timing [11], reducing the ketogenic effects.

A temporary increase in AST/ALT from 24/27 to a maximum of 102/53 was observed during the hospitalization and it could be linked to the hepatic uptake of fatty acids produced by lipolysis. Moreover, anti-Gad antibody dosage and Peptide C with positive C-peptide of 0.02 have been practiced, and this supports the diagnosis of LADA type diabetes (Latent Autoimmune Diabetes in Adults). When prescribing SGLT2i, if there is a doubt between type 1 or type 2 diabetes, a screening with Peptide C should be carried out, combining the dosage of autoantibodies to rule out type 1 diabetes. The anamnesis of the contributory causes also shows how in this type of patient the excessive reduction of carbohydrates and insulin therapy [1] is one of the triggers of this complication even after years from the start of drug administration [12]. All patients with type 2 DM should be educated regarding sufficient hydration and adequate carbohydrate intake while using SGLT2i [3]. Clinicians should avoid using SGLT2i in patients who are unable to tolerate oral food intake or in those who have an excessive drop in body weight or follow a very low carbohydrate diet.

 

Bibliography

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Case Report: MPO-ANCA Associated Vasculitis After Pfizer-BioNTech COVID-19 mRNA Vaccination

Abstract

We report a case of MPO-anti-neutrophil cytoplasmic antibody ANCA-associated vasculitis, with pulmonary-renal syndrome, after the mRNA booster third dose vaccine Pfizer BioNTech against COVID-19 in 71-year-old Caucasian man with no specific past medical history. A kidney biopsy diagnosed ANCA-associated pauci-immune crescentic glomerulonephritis. Renal function and constitutional symptoms have been partially improved with treatment with dialysis, intravenous rituximab and steroid pulse therapy. No disease following either infection or vaccination with fourth dose against COVID-19.

Keywords: ANCA vasculitis (AAV), booster dose, cytokine storm, mRNA vaccine, SARS-CoV-2

Sorry, this entry is only available in Italian.

Introduzione

L’Efficacia e la sicurezza dei vaccini contro il COVID-19 sono ampiamente dimostrati da autorevoli studi scientifici. Tuttavia, l’incremento delle vaccinazioni è associato a possibili effetti collaterali a lungo termine [1]. Le dosi di richiamo con vaccino Pfizer-BioNTech COVID-19 mRNA che codificano la glicoproteina SARS-CoV-2S, risultano essere efficaci a contrastare lo sviluppo di nuove varianti da SARS-CoV-2 [2]. Nonostante la sicurezza di questi vaccini, sono riportati eventi avversi comuni sia lievi che moderati che riguardano principalmente il sito d’iniezione, manifestazioni febbrili, sindromi parainfluenzali, mialgie, cefalea [3, 4]. Tuttavia, in letteratura emergono segnalazioni di casi clinici che evidenziano una possibile correlazione tra vaccini a COVID-19 mRNA e insorgenza di malattia autoimmuni in soggetti predisposti [5, 6]. La stimolazione sistemica del sistema immunitario, da parte dei nuovi vaccini mRNA, potrebbe scatenare la tempesta di citochine causando infiammazione dei vasi di piccolo e medio calibro fino alla necrosi e danneggiando anche organi e tessuti [7]. Queste rare forme di vasculiti secondarie a vaccinazione mRNA sono tipicamente associate alla predominante presenza di anticorpi anti-citoplasma dei neutrofili (ANCA) contro le proteine mieloperossidasi (MPO) [8]. 

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