Marta Benedetta Maria Mazzoni

The cryptic interplay between systemic lupus erythematosus and infections

The underlying trigger for systemic lupus erythematosus (SLE) has remained elusive, and multiple interacting environmental and genetic factors likely contribute to the onset and perpetuation of the disease. Among environmental influences, infectious agents have been suggested to play a pivotal role in driving autoimmunity pathogenesis via structural or functional molecular mimicry, the expression of proteins that induce cross-reactive responses against self-antigens, and the aberrant activation or apoptosis of different immune system cells in the context of a peculiar genetic background. The increased viral load and changing subsets of lytic or latent viral proteins observed in selected populations with SLE have indicated that common viruses, such as Epstein-Barr virus, parvovirus B19, cytomegalovirus, retroviruses and transfusion-transmitted viruses, might be triggers for this disease. Alternatively, some infectious agents might exert a protective effect from autoimmunity. Existing achievements have not been fully investigated and clarified. Thus, the aim of this review is to analyze the medical literature within the last 15years regarding the role of infectious agents in the pathogenesis of SLE.

Early nutrition patterns and diseases of adulthood: A plausible link?

In the last decades several studies tested the hypothesis that at early development stages certain foods or nutrients, in specific amounts, fed during limited sensitive periods, may determine an endocrine metabolic asset leading to clinical alterations that take place decades later (early nutritional programming of long term health). Evidence is mounting for programming effects of infant feeding. Observational studies indicate that breast feeding, relative to formula feeding, reduces the risk for obesity at school age by about 20% even after adjustment for biological and sociodemographic confounders. Moreover, breastfeeding is constantly associated with increased neurodevelopmental scores up to early adulthood, while its outcome in terms of delayed decay of brain function is still unknown. Besides the environment surrounding breastfeeding, specific nutrients within human milk may play a direct role. With the introduction of solids the major changes in diet are represented by the sudden decrease of fat intake from 50 to 30% of total energy. A protein excess, commonly found throughout all European Countries, has been associated to a higher risk of adiposity in early childhood, as confirmed by first reports from a large European trial. The amount of fat does not seem to be associated with later adiposity, while its quality may affect blood lipoproteins, blood pressure and neurodevelopmental performance. Early intake of dietary fibers might also have beneficial effects. Epidemiologic data show that episodes of rapid growth (growth acceleration hypothesis), whichever the dietary habits, are associated with later unfavorable health conditions and should be prevented.

Renal vein obstruction and orthostatic proteinuria: a review

OBJECTIVES The cause of orthostatic proteinuria is not clear but may often relate to obstruction of the left renal vein in the fork between the aorta and the superior mesenteric artery (=renal nutcracker). However, reports dealing with proteinuria only marginally refer to this possible cause of orthostatic proteinuria. We analysed the corresponding literature. RESULTS Five reports addressed the frequency of renal nutcracker in 229 subjects with orthostatic proteinuria. Their age ranged between 5.2 and 17 years (female-to-male ratio: 0.96:1.00). Imaging studies demonstrated renal nutcracker in 156 (68%) subjects. Renal nutcracker was also demonstrated in 9 anecdotal reports for a total of 53 subjects with postural proteinuria. Very recently, 13 Italian subjects with orthostatic proteinuria associated with renal nutcracker were reassessed 6 years after the initial diagnosis: in nine subjects, both orthostatic proteinuria and renal nutcracker had disappeared; in three, both orthostatic proteinuria and renal nutcracker had persisted; and in one, orthostatic proteinuria had persisted unassociated with renal nutcracker. CONCLUSIONS These data provide substantial support for renal nutcracker as a common cause of orthostatic proteinuria.

Episodic idiopathic systemic capillary leak syndrome in a girl

Episodic idiopathic systemic capillary leak syndrome is a rare disorder that presents with attacks of circulatory shock due to plasma leakage into the extravascular space. Reported here is the case of a girl who had recurrent circulatory shock. The diagnosis of episodic idiopathic systemic capillary leak syndrome was made following the fourth episode. The course was favorable following acute i.v. cristalloids and methylprednisolone.

Postural proteinuria associated with left renal vein entrapment: a follow-up evaluation

Imaging studies show entrapment of the left renal vein in the fork between the aorta and proximal superior mesenteric artery in most cases of isolated postural proteinuria. Therefore, it has been postulated that partial obstruction to the flow in the left renal vein in the upright position is a cause of this form of proteinuria. In a girl with isolated postural proteinuria, kidney ultrasonic imaging and Doppler flow scanning showed left renal vein entrapment. Seven years later, a new evaluation showed resolution of both postural proteinuria and left renal vein entrapment. The longitudinal observation provides substantial additional support for entrapment of the left renal vein by the aorta and superior mesenteric artery as a cause of isolated postural proteinuria.

Arterial Hypertension and Posterior Reversible Cerebral Edema Syndrome Induced by Risperidone

Posterior reversible cerebral edema syndrome is a generally reversible neurologic condition that is diagnosed based on distinctive clinical and radiologic findings. The condition, which is mostly associated with severe arterial hypertension, has also been reported to be induced by several medications. We made the diagnosis of hypertension with posterior reversible cerebral edema syndrome in a lean 12-year-old girl treated with the second-generation antipsychotic risperidone. We applied the Naranjo Adverse Drug Reaction Probability Scale and the World Health Organization–Uppsala Monitoring Centre system for causality assessment to the present case. Both scales indicated that a relationship to risperidone was likely. Second-generation antipsychotic agents may occasionally induce an increase in blood pressure even in the absence of overweight. Given this possibility, we recommend routine monitoring of blood pressure during therapy with these agents.

The acute interstitial nephritis induced by azithromycin

Sir, Azithromycin, a well-tolerated macrolide antibiotic that can be dosed once daily, is commonly prescribed both in children and adults with respiratory tract infections. Interstitial nephritis induced by azithromycin has been previously described in two patients [1, 2]. The first case presented chronic renal failure after the second episode of acute interstitial nephritis (AIN) and the second case was still on haemodialysis 10 months after the onset of AIN.

Nutcracker phenomenon and idiopathic IgA nephropathy

Sir, Entrapment of the left renal vein between the aorta and the superior mesenteric artery is a documented cause of both postural proteinuria as well as intermittent gross haematuria associated with left flank pain. Idiopathic IgA nephropathy typically presents with recurrent episodes of gross haematuria, usually following an acute febrile illness. Rarely, the disease presents as microscopic haematuria and usually mild proteinuria. We present the first case of postural proteinuria caused by left renal vein entrapment and recurrent gross haematuria caused by an IgA nephropathy. In an 11-year-old boy with postural proteinuria, renal ultrasonic Doppler disclosed left renal vein entrapment: the anteroposterior diameter at the hilar portion divided by that at the aortomesenteric portion was 13.3 (reference ≤ 4.0) and the peak flow velocity at the aortomesenteric portion divided by that at the hilar portion 15.1 (reference ≤ 4.0). Two years later, three episodes of acute febrile diarrhoea were followed by gross haematuria that was not associated with flank pain. The kidney biopsy showed mild diffuse mesangial proliferation and matrix expansion. The diagnosis of IgA nephropathy was made on immunofluorescence microscopy, which demonstrated isolated prominent globular deposits of IgA, C3 and IgG in the mesangium. The link between left renal vein entrapment and postural proteinuria is well documented. Furthermore, both left renal vein entrapment and IgA nephropathy are recognized causes of recurrent gross haematuria. There are five reported patients affected with both left renal vein entrapment and idiopathic IgA nephropathy [1–4]. A 12-year-old German girl with microscopic haematuria was found to have left renal vein entrapment and mesangial deposits of IgA [1]. Similarly, left renal vein entrapment and mesangial deposits of IgA were reported in a 9-year-old Korean girl with isolated microscopic haematuria and recurrent gross haematuria [2] and in a 25-year old Taiwanese woman with recurrent gross haematuria and left flank pain [3]. Left renal vein entrapment and IgA nephropathy were also disclosed in a 20-year-old woman and in a 22-year-old Japanese man, who, in the context of a pharyngitis, developed gross haematuria and a tendency to microscopic haematuria with persisting proteinuria [4]. In addition, left renal vein entrapment was disclosed in two Korean children a 13-year-old Korean girl with Henoch–Schonlein syndrome and IgA nephropathy [5]. Finally, a 10-year-old Korean boy with a clinically characteristic and rapidly resolving form of Henoch–Schonlein syndrome followed by microscopic haematuria persisting for 26 months was found to have left renal vein entrapment; haematuria subsided in accordance with radiological improvement of renal vein entrapment [6]. Our patient is the first case with concurrent existence of postural proteinuria caused by left renal vein entrapment and recurrent gross haematuria caused by an idiopathic IgA nephropathy. Renal venous congestion may induce both proteinuria and haematuria, which have been implicated as a cause of renal damage. So, a causal relationship between left renal vein entrapment and IgA nephropathy cannot be ruled out. Further investigations are necessary to detect this correlation.

Predictive value of EEG for febrile seizure recurrence

OBJECTIVE To define the role of the EEG in predicting recurrence of febrile seizures (FS) in children after a first FS. METHODS Children with a first simple or complex FS who underwent EEG at our hospital were retrospectively enrolled. EEG recordings were classified in three groups: normal, abnormal (slow activity or epileptiform discharges), and pseudo-petit mal discharge (PPMD) pattern. Children were followed-up for at least three years. RESULTS A total of 126 patients met the entry criteria, and 113 of them completed the follow-up. Risk of FS recurrence decreased linearly with increasing age (-2% per month). The risk was higher among patients with PPMD pattern (absolute risk 86%, adjusted relative risk 2.00) and abnormal EEG (epileptiform discharges: absolute risk 71%, adjusted relative risk 2.00; slow activity: absolute risk 56%, adjusted relative risk 1.44), compared with those with normal EEG (absolute risk 41%). CONCLUSIONS PPMD and abnormal EEG should be considered as an independent risk factor for FS recurrence.

Neonatal focal seizures and hypomagnesemia: A case report

BACKGROUND In seizures caused by electrolytes disorders, a prompt diagnosis allows an appropriate treatment and reduces the risk of neurological complications. Hypomagnesemia is a recognized cause of generalized seizures, while it has been anecdotally reported in focal forms. CASE PRESENTATION We describe a newborn with recurrent focal seizures due to transient hypomagnesemia. CONCLUSION Physicians should consider the possible occurrence of such disorder also in cases of focal seizures.