Munchausen syndrome by proxy and pediatric nephrology

Munchausen syndrome by proxy and pediatric nephrology

G Model NEPHRO-972; No. of Pages 3 Ne´phrologie & The´rapeutique xxx (2017) xxx–xxx Available online at ScienceDirect www.sciencedirect.com Review...

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NEPHRO-972; No. of Pages 3 Ne´phrologie & The´rapeutique xxx (2017) xxx–xxx

Available online at

ScienceDirect www.sciencedirect.com

Reviews/Articles

Munchausen syndrome by proxy and pediatric nephrology Cristina Bertulli a,*, Pierre Cochat b a

Pediatric department of spedali civili, University of Brescia, Piazzale Spedali Civili 1, 25100 Brescia, Italy Service de ne´phrologie rhumatologie dermatologie pe´diatriques, centre de re´fe´rence des maladies re´nales rares, hoˆpital Femme–Me`re–Enfant, hospices civils de Lyon et universite´ Claude-Bernard Lyon 1, 3, quai des Ce´lestins, 69002 Lyon, France b

A R T I C L E I N F O

A B S T R A C T

Article history: Received 7 September 2016 Accepted 11 December 2016

Munchausen syndrome by proxy is a persistent fabrication of illness done by a person to another. Renal and urologic forms of this syndrome are not as uncommon as can be thought; a review of all the cases of Munchausen-by-proxy syndrome reveals that 25% of the children had renal or urologic issues. This syndrome can result in a serious diagnostic dilemma for the physicians; knowing this entity can allow early recognition of falsification and limit the physical and psychological damages caused in the victim. In this study, we reviewed the pediatric nephrology cases of Munchausen syndrome by proxy, grouping them through the principal signs of presentation.

C 2017 Socie ´ te´ francophone de ne´phrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved.

Keywords: Child Munchausen syndrome by proxy Nephrology Renal disease Urological disorder

1. Introduction Munchhausen syndrome by proxy (MSBP) is a behavioral pattern in which a caregiver creates, exaggerates or induces mental or physical illnesses in someone who is in his/her care, in order to gain the attention of medical providers and others [1]. Typical victims may be males or females, usually 4 years of age or less, and they averaged 21,8 months from onset of symptoms to diagnosis; mothers are perpetrators in 3 cases on 4 [2]. This syndrome was first described in 1977 by Meadow: he described two children treated for medical illness induced by their mother; one of them died [1]. Since that time, an increasing number of cases and different variety of this syndrome have been reported. Fabrications include completely false history, historical exaggeration of real illness, manufactured signs or symptoms or illness induction, tamper with specimen; it can relate to any organ system or disease process [2]. Children victims of this form of abuse undergo invasive diagnostic and surgical procedures because of the falsification, and they could develop iatrogenic complications as well as behavioral problems stemming from their abuse. Children may assume sick role themselves, collaborating with or inventing deceptions [3]: They can cooperate with their caregivers to fabricate illness manipulating their samples or simulating symptoms. In addition, cases of pediatric Munchausen * Corresponding author. E-mail address: [email protected] (C. Bertulli).

syndrome not by proxy are discussed, in which children create disease all alone without adult’s help. The diagnosis is frequently difficult to document and often delayed; early recognition or suspicion of this syndrome can reduce the number of unnecessary diagnostic procedures performed and may be lifesaving for the patient. In 1995, a database for all the cases of MSBP was started: 25% of the children included between 1974 and 2006 had renal or urologic concerns [4]. The most frequent nephrologic signs of presentation are urinary tract infections (UTIs), hematuria and urolithiasis, but there have been also reported cases of proteinuria, acute renal failure and electrolytic alterations [1,4–7]. The goal of this article was to review the pediatric nephrology cases described in the literature of MSBP, hoping that this could help physicians to early diagnosis and intervention. 2. Hematuria Factitious hematuria can be fabricated in different ways: urine samples can be contaminated by blood or other coloring substances. Laboratory findings demonstrate no other renal anomalies; usually random urinalyses obtained by medical staff members are negative for the presence of red blood cells [4]. The samples can be polluted by patient’s blood: Abrol et al. reported a case concerning a 10-year-old boy with factitious gross hematuria secondary to self-induced fingers sticks [8]. The blood added into urine can also come from urethral manipulations or traumatism of external genitalia: a 10-year-old female with relapsing gross hematuria underwent two cystoscopies which

http://dx.doi.org/10.1016/j.nephro.2016.12.006 C 2017 Socie ´ te´ francophone de ne´phrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved. 1769-7255/

Please cite this article in press as: Bertulli C, Cochat P. Munchausen syndrome by proxy and pediatric nephrology. Ne´phrol ther (2017), http://dx.doi.org/10.1016/j.nephro.2016.12.006

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revealed edema of the bladder neck. The hematuria was due to selfinduced urethral trauma [9]. In these cases, clinical examination can be normal or reveal signs of genitals or urinary tract traumatism. The blood in the urine samples can belong not to the patient but to the caregiver: Physicians can notice a discrepancy because all the urines samples collected in presence of caregiver or delivered by him/her to the laboratory contain blood cells while samples collected random by medical staff without caregiver’s assistance are normal. Blood cells source can be defined by typing the patient’s and caregiver’s blood group and checking which matches to the urines [1,10]. When patients and caregivers are of the same major blood group type, minor group blood typing can be also performed [11]. Hematuria can be secondary to drugs intoxication like anticoagulant or antiaggregant agents that can cause bleeding; unexplained menorrhagia and hematuria occurred in a 13-yearold female who was receiving Coumadin by her mother not prescribed by any doctor [12]. A lot of drugs and aliments can cause changes in urine color [13]. Urine samples can be contaminated by coloring substances and present an uncommon coloration: The observation of a colacolored dipstick positive for ‘‘hematuria’’ was made in an 11-yearold boy who added povidone iodine to the urine sample [14]. All positive dipstick with suspect for factitious hematuria should undergo microscopy to confirm the presence of red blood cells.

3. Proteinuria Factitious proteinuria, sometimes in nephrotic range, is never associated with hypoprotidemia, edema or renal failure. The source of factitious proteinuria is exogenous, in most of cases it has an animal origin [15,16]. Proteinuria can be fabricated using egg proteins: Tojo et al. described the case of a 16-year-old girl with proteinuria. Electrophoresis of the urinary protein showed two abnormal fractions at the alpha and beta globulins and immunoelectrophoresis revealed that these abnormal proteins were not derived from human serum proteins, but were egg proteins that were injected into the bladder by the girl [17]. In some cases, the exogenous source of proteins can be human: Ulinski et al. reported the case of a 5-year-old boy with massive proteinuria who underwent two kidney biopsies which revealed no abnormalities; electrophoresis of urine proteins demonstrated the unusual finding of an albumin fraction of 99.4%. The mother, who was a nurse, finally confessed to adding human albumin to the urine samples [18]. Whatever is the source of proteins, animal or human, the urinary protein electrophoresis can be helpful by revealing almost pure albumin without transferrin fraction, and lack of IgG fragments and alpha 2 fraction proteins, suggesting factitious proteinuria.

4. Urolithiasis Stones or sand may be added to voided urines or inside the urethra to simulate renal stones: Microscopy examination of the urine can identify lack of cellular sediment [4] or sand and gravel contamination [19]. Physical examination can be normal or reveal a dilated external urethral meatus [20]; the clinical findings of renal colic and macrohematuria are typically absent [21]. Mineral metabolic pattern on blood, spot urine and 24 hours urine samples reveal no abnormalities; endoscopic examination

can be negative for the presence of stones and urinary tract dilatation or show a ‘‘stone’’ in the distal urethra. Senocak et al. described the case of a 2-year-old boy with an history of repeated passage of urinary stones; retrograde urethrocystography showed a dilated urethra with a stone at the end. After being removed, the stones were analyzed and reported as a silicious pebble. It was the child’s step mother who inserted pebbles in his urethra [20]. Stone analysis using infra-red spectrometry is important for differential diagnosis, by revealing an abnormal composition [22]: Herring reported that, in a series of 10,000 analyzed urinary calculi, 85 were artificial in nature, the commonest being pebbles [23]. Absolute de la Gastine reported on a 8-year-old boy with a positive anamnesis for urinary stones; although he declared to passed several urinary stones, the physicians became suspicious because of the absence of renal colic, hematuria and urinary tract dilatation. The diagnosis of Munchausen syndrome was further confirmed by chemical analysis, which reported that samples were in fact ordinary pebbles, and by the child’s confession [21]. Other components have been used, such as wall plaster or mixture of insects (Cochat, unpublished).

5. Urinary tract infection Factitious UTIs can be recurrent and recalcitrant. The infection can be either due to exogenous contamination of urine, or induced by urethral manipulation: The urine culture can reveal the presence of unusual bacteria (e.g., salivary organisms), polymicrobial infection, bacteria resistant to concurrent antibiotic treatment [24]. Infection can persist when appropriate antibiotics are not administrated. Feldman et al. described two sisters with recurrent UTIs, one of them underwent Mitrofanoff stoma as medical treatment because of recurrent UTIs and urine retention [4].

6. Conclusions Renal and urologic issues in Munchausen syndrome by proxy are only limited by the inventiveness and motivation of the caregiver and/or the child. Since Meadow first described this entity, not only new cases but also different diagnostic problems have become evident [25,26]. Rosenberg proposed clinical diagnostic criteria for identifying MSBP: physicians should consider MSBP as a possible diagnosis if there is evidence of recurrent illnesses without a defined cause, if discrepancies occur between biochemical findings and clinical conditions; also the presence of a caregiver who does not object or repent painful interventions in the child may alarm, especially if he/she has previous medical experience [27]. It is important to consider and exclude MSBP before performing unnecessary and potentially harmful investigations and treatments: victims of Munchausen syndrome by proxy could report physical or psychological damages and could even die as the results of induced illness or for medical procedures and therapies [3,28]. Evaluation of these cases should be multi-disciplinary to assure that unusual illnesses are not missed. While it may be difficult to understand the motivation of the abusing caretaker, reviews of proven cases demonstrate a clear need for the perpetrator to use the child as a way of getting the attention of physicians and other professionals [25]. Once diagnosed, the protection of the child must be imperative.

Please cite this article in press as: Bertulli C, Cochat P. Munchausen syndrome by proxy and pediatric nephrology. Ne´phrol ther (2017), http://dx.doi.org/10.1016/j.nephro.2016.12.006

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NEPHRO-972; No. of Pages 3 C. Bertulli, P. Cochat / Ne´phrologie & The´rapeutique xxx (2017) xxx–xxx

Disclosure of interest The authors declare that they have no competing interest.

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Please cite this article in press as: Bertulli C, Cochat P. Munchausen syndrome by proxy and pediatric nephrology. Ne´phrol ther (2017), http://dx.doi.org/10.1016/j.nephro.2016.12.006