Sine Materia Research Articles

Long-Term Risks of Benign and Malign Complications after Endoscopic Sphincterotomy in the Management of Benign Biliopancreatic Pathology: A Cohort Study.

Endoscopic retrograde cholangiopancreatography (ERCP) is the preferred technique for treating pathologies of the bile duct. It has been suggested that this procedure, combined with sphincterotomy, may influence the subsequent development of long-term complications. The main objective of this study was to determine the long-term complications of biliopancreatic disease after ERCP and their potential association with the development of biliopancreatic neoplasms. This retrospective cohort study included 576 patients who underwent ERCP (referred to as index ERCP) with sphincterotomy for benign biliary disease, with a minimum follow-up period of more than 2 years. The incidence of long-term benign and neoplastic pathologies after ERCP was analyzed. The most common findings were recurrence of choledocholithiasis in 70 patients (12.1%), cholangitis "sine materia" in 27 patients (4.7%), and acute pancreatitis in 8 patients (1.4%). Eight patients (1.4%) developed hepatobiliopancreatic neoplasms, including 4 cases of pancreatic neoplasms (0.7%), 1 cholangiocarcinoma (0.2%), 1 ampulloma (0.2%), and 2 intrahepatic neoplasms (0.3%). Multivariate analysis revealed that factors such as age over 50 years, previous biliary surgery, diversion of the bile duct (BD) to the digestive tract, dilation of the BD, stent placement, biopsy, and cholecystectomy were factors associated with an increased risk of long-term benign complications. Endoscopic retrograde cholangiopancreatography with sphincterotomy is associated with an increased long-term risk of subsequent benign biliopancreatic disease. However, our data do not allow us to establish a direct relationship with the development of biliopancreatic neoplasms.

Commotio cordis: Another cardiac arrest “sine materia”. The 1707 early report and interpretation by G.M. Lancisi

Sudden death by commotio cordis is rare. It is the consequence of a blunt trauma of the chest overlying the heart.The mechanism is a cardiac arrest by ventricular fibrillation in the absence of grossly or microscopically apparent myocardial injury. It has been reproduced in animals. The first historical case was reported by Giovanni Maria Lancisi in his book "De Subitaneis Mortibus'' published in 1707. Sudden death occurred in a man receiving a powerful blow under the xiphoid cartilage. Lancisi advanced the hypothesis of acute heart failure by a diastolic stand still ("death in diastole'').

Systemic Inflammatory Response in Spontaneous Subarachnoid Hemorrhage from Aneurysmal Rupture versus Subarachnoid Hemorrhage of Unknown Origin.

It is well known that spontaneous non-aneurysmal subarachnoid hemorrhage (SAH), also known as sine materia SAH (smSAH), has usually a better course and prognosis than its aneurysmal counterpart (aSAH). This might depend on different inflammatory mechanisms initiated by bleeding events of different origins. The aim of the present study was to explore the systemic inflammatory response in spontaneous SAH, comparing aSAH and smSAH. We performed a prospective observational study over a consecutive series of patients with SAH. For these patients, we collected all clinical data and, furthermore, performed venous blood sampling over six time points to analyze blood cells. We further performed the analysis of lymphocytes and monocytes by means of flow cytometry to quantify common subtypes. Statistical analysis included a t-student test, Chi-square test, multivariate logistic regression, and ROC analysis. 48 patients were included: six (12.5%) with a diagnosis of spontaneous smSAH, and forty-two patients (87.5%) with aSAH. Significant differences on Day 0 were found for neutrophils and a systemic neuro-inflammatory index, namely, systemic inflammatory response index (SIRI). At the ROC analysis, neutrophil-to-lymphocyte ratio (NLR), lymphocyte-to-monocyte ratio (LMR), and SIRI exhibited satisfactory predictive power on day 0. At the multivariable logistic regression analysis, the combined index (NLR, LMR, SIRI at day 0) yielded an OR of 0.59 (95% CI 0.29-1.21]). LMR at day 0 yielded an OR of 1.25 ([95% CI 0.94-1.68]), NLR at day 0 exhibited an OR of 0.68 ([95% CI 0.42-1.09]), and SIRI at day 0 displayed an OR of 0.31 ([95% CI 0.06-1.49]). This preliminary study indicated a possible role of some inflammatory indices that point out the importance of innate and adaptive immunity in the etiopathogenetic mechanisms. Drugs modulating these responses could eventually counteract or, at least, reduce secondary damage associated with SAH.

Intrahepatic cholestasis of pregnancy: Case series of a rare disease in an African setting

Intrahepatic cholestasis of pregnancy commonly presents in the second and third trimester with unexplained pruritus sine materia, abnormal liver function tests, and/or elevated serum total bile acid concentration. Its incidence ranges from less than 1% to 27.6%, with Araucana Indians from Chile being the highest affected. Intrahepatic cholestasis of pregnancy complicates pregnancy, hence influencing its outcomes. Our series involves three cases of pruritus in pregnancy ending with one elective cesarean delivery and two emergency cesarean deliveries without any significant complications to babies as well as immediate resolution of maternal symptoms. High index of suspicion for intrahepatic cholestasis of pregnancy is of paramount importance among clinicians even in primary care setup. Prompt treatment and intervention will prevent poor perinatal outcome.

English

The integrated approach is the new recommendation of the World Health Organization (WHO) for the control of neglected tropical diseases. The goal of this cross-sectional study was to implement this approach in 4 municipalities with a high prevalence of leprosy in Benin from September 2019 to August 2020. Mobile medical consultations were organized in these municipalities following the mobilization and sensitization of the populations. In the 4 municipalities, 6416 people were examined. The mean age was (27.3 ±19.8) years. Women represented 52.7% of people examined. Among the 6416 people examined, 1230 (19.2%) had skin conditions. The most common skin conditions were pityriasis versicolor (421 cases; 34.2%); eczema (203 cases; 16.5%); ringworm (159 cases; 12.9%); pruritus sine materia (81 cases; 6.6%); acne (62 cases; 5.0%); epidermophytia (37 cases; 3.0%); achromic nevus (35 cases; 2.8%); keloids (28 cases; 2.3%); chronic ulcers (27 cases; 2.2%) and depigmentation stigmata (24 cases; 2.0%). 8 new leprosy cases (0.7%) were detected. This study proves the efficiency of the integrated approach in the control of neglected tropical diseases (NTDs) with cutaneous manifestations in Benin. Key words: Neglected tropical disease, leprosy, skin disease, integration, disease control, Benin.

Good news for Johnson & Johnson in talc lawsuits, but Imerys backs out of the fray

Unexpected sudden cardiac death (SCD), sudden infant death syndrome (SIDS) and sudden intrauterine unexplained death (SIUD) are major unsolved, devastating forms of death that occur frequently. Obstructive sleep apnea (OSA) has been associated with increased cardiovascular and cerebrovascular morbidity and mortality, including sudden cardiac death (SCD). This editorial will review the pathology of SCD, including sudden infant death syndrome (SIDS) and sudden intrauterine unexplained death (SIUD); OSA with its cardiovascular consequences; the possible link between SCD and OSA, discussing the potential mechanisms underlying these two frequent, but yet overlooked pathologies. Finally, the possible preventive benefits of treating OSA and identifying patients at common risk for OSA and SCD and SIDS-SIUD to prevent unexpected deaths will be discussed. Post-mortem examination is of great importance in every case of SCD sine materia, with examination of the brainstem and cardiac conduction system on serial sections, when general autopsy fails, but it should be stressed that also the investigations of patients suffering from OSA should focus on the possibility of pathological findings in common with cases of SCD.

Allergic sensitization in patients with pruritus sine materia in the absence of a somatic cause

Aim: Pruritus sine materia in the absence of a somatic cause is generally attributed to psychogenic factors, but it is also well known that psychological stress may affect skin through its impacts on immune response. In this three-year retrospective study we aimed to reveal the allergic sensitization of patients with chronic pruritus without a somatic cause. Material and Methods: Three hundred and sixty five male and female patients with widespread chronic pruritus between 6 and 75 years old were retrospectively recruited. In addition to routine hematologic examination, skin prick test and serum total IgE measurement were performed on each patient.Results: In cutaneous examination, the patients had no visible manifestation except self excoriations in some, and all had normal values in hematologic laboratory examination. Altogether 10% of patients had positive results for both skin prick test and serum total IgE, and 42% of patients had positive results for either skin prick test and/or serum total IgE, which may be regarded as a presentation of immediate type hypersensitivity.Conclusion: Considering the quite high positive ratios obtained for both skin prick test and serum total IgE, it may be encouraging to perform these tests routinely on the patients with pruritus sine materia, both for revealing a probable allergic tendency and for better handling the treatment modalities.

Depression in patients with functional itch disorder

Objective: Patients with pruritus sine materia (PSM) are often misdiagnosed as idiopathic pruritus (pruritus of unknown origin) when the cause of pruritus is not found. Some of these patients may be diagnosed with functional itch disorder (FID) which is also known as psychogenic pruritus (pruritus of psychological origin). Since antidepressants can be used in the treatment of psychogenic pruritus, the differentiation of FID from idiopathic pruritus is important. The aim of this study was to investigate the prevalence of depression in patients with FID. Materials and Methods: A total of 117 patients with FID who were diagnosed as idiopathic pruritus or PSM in their previous assessments and 117 controls took part in the research. The psychiatric assessment for depression was conducted using the Beck Depression Inventory (BDI) and the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) major depression criteria. The study employed a descriptive and cross-sectional method. Results: Forty-four patients with FID (37.6%) had major depression (DSM-5) and 74 patients with FID (63.2%) had clinically significant depression (BDI). The frequency of depression among FID patients was significantly higher than controls. Conclusions: Our findings highlight the importance of diagnosing FID in patients with PSM. The presence of FID diagnostic criteria in a patient should prompt dermatologists for a psychiatry consultation.

Chronic pain in medullary sponge kidney: a rare and never described clinical presentation.

Medullary sponge kidney (MSK) is a cause of nephrocalcinosis, associated with hematuria, renal colic, pyelonephritis. There are rare and atypical MSK cases characterized by chronic severe pain (CP), whose features are unknown, in particular the relationship with the stone disease activity. This study analyzes a cohort of MSK-CP patients belonging to three North-America self-support Facebook groups. Patients had to self-administer an on-line questionnaire (on intensity, progression and MSK-associated conditions, stone-related disease, pain features, drug use), the Brief Pain Inventory, the Fatigue Severity Score, and Wisconsin Quality of Life (WQL) in stone formers questionnaires. Ninety-two patients with a diagnosis of MSK joined our survey. Stone rate was very high (3.1 stones per patient-year, < 15% of patients had ≤ 1 stone per year). Most patients had repeated hospitalizations for stones symptoms (p < 0.001) or pain (p < 0.005). 71% of participants referred a daily pain that interfered strongly with everyday life and quality of life (WQL mean value 29.4). 69% used pain medications daily (70% opioids). In most cases, pain was associated with stone passage, while 15% referred a sine materia pain. We showed how MSK-CP symptoms affect very negatively on the quality of life of these patients. They also have a definite risk of progressing to end-stage kidney disease. Generally, CP seems to be associated with an exceptionally high lithogenic activity, suggesting that a better and earlier metabolic treatment for stone prevention should be the first approach in these patients before mini-invasive treatments to prevent pain.

Particular cause of obstructive dyspnea in children

Dyspnea in infants and small children is a common symptom of addressability to a medical service. For a doctor, setting a diagnosis is challenging, because dyspnea can be a symptom in multiple conditions. The first step in establishing the diagnosis of dyspnea in children is explaining the mechanism (obstructive dyspnea, nonobstructive or “sine materia”). In the presence of an obstructive dyspnea, besides common causes (laryngitis, bronchiolitis, bronchial asthma, aspiration of foreign bodies), some mediastinal disorders should be evoked. We present the case of a one year and two month old male patient, presented for cough, expiratory dyspnea and wheezing, the presumed clinical diagnosis being acute bronchitis. Given that dyspnea was enhanced under treatment, a chest radiograph was performed, which revealed a homogeneous oval opacity occupying almost the entire right thorax. Thoracic CT revealed in the right lateromediastinal part a tumor with an apparently cystic form, with inner calcifications, with left displacement of the mediastinal structures. Thoracotomy with tumor excretion was performed, macroscopically seemed to be teratoma, microscopically confirmed. The child has undergone subsequent chemotherapy treatment with favorable evolution.

The Male Sexual Pain: Issues and Treatments

Objective: There are several kinds of male genital pain which can cause mental suffering. Usually, they emerge during ordinary urological or andrological examinations. These distresses, sine materia, can be divided into different groups. Design and Method: The first group, which is the most frequent one, mainly affects the testicles; usually, this kind of distress last all life and it is often associated with surgery and/or frequent examinations to the genitals during childhood. The second group encompasses cases of real penodinia: the patients express their discomfort especially uncovering the glan and, for this reason, this action is never done. This distress is not always referred to as pain, but more often as bothersome. Sexual intercourses are permitted. Often the patients undergo a circumcision that usually has a complicated postoperative course. The males of the third group correspond to the women suffering from vaginismus. The patients do not even tolerate the approach of the hand to the penis and they make the same actions and the same movements as the vaginismic women. The patients are unable to have sex. This phenomenon is rare. In a perspective of multi-disciplinary and integrated methodology, the patients were treated with traditional surgery and/or with administration of drugs and/or with sexual therapy according with the integrated Kaplan method and/or with techniques for the rehabilitation of the pelvic floor. Results: Some clinical cases will be presented and discussed. Conclusions: The Authors describe new categories of male genital pain, never defined before, similar to Vulvodynia or Vaginism in women.

Bullous Pemphigoid as Pruritus in the Elderly

In the literature, patients with bullous pemphigoid have been reported to have itch without blisters. Clinical observations in these patients have varied from eczematous or urticarial to papular or nodular skin lesions. Here we investigated the spectrum of clinical variants. Fifteen patients with itch without blisters had immunopathologic findings of bullous pemphigoid. Mean age at diagnosis was 81.7 years. No blistering occurred during the mean 2.2 years of follow-up. Mean delay of diagnosis was 2.8 years. Clinical symptoms were heterogeneous: pruritus sine materia (no primary skin lesions), eczematous, urticarial, papular, and/or nodular skin lesions were seen. Treatment with potent topical corticosteroids or methotrexate sodium led to remission in 11 patients. Itch without skin lesions can be the only symptom of bullous pemphigoid. Therefore, it is important to include serologic and direct immunofluorescence in the diagnostic algorithm of itch. We propose the unifying term pruritic nonbullous pemphigoid for all patients with immunopathologic findings of bullous pemphigoid, itch, and no blisters.

Psychogenic Itch Responding to Topiramate

Itch (or pruritus) is defined as an unpleasant sensation inducing the desire of scratch. Chronic pruritus ( 6weeks duration) is a major and distressing symptom of many dermatological, systemic, neurological, or psychogenic diseases. A recent study assessed that patients with chronic pruritus carry a significant burden of disease with a substantial impact on quality of life that may be comparable to that of chronic pain. According to the neurophysiologic classification, pruritus can be classified into four major types: pruritoceptive, when itch fibers are activated by pruritogens that stimulate their sensory ending; neuropathic, due to primary lesion or dysfunction along the afferent fibers; neurogenic, generated by central mediators without neuronal damage; and psychogenic, when resulting from a pure central psychic processing in the absence of skin pathology or underlying medical disease. Hence, psychogenic pruritus (PP) is often a diagnosis of exclusion after other possible causes of pruritus have been ruled out. In particular, both PP and neuropathic itch present with no evidence of primary cutaneous lesions (pruritus sine materia), therefore clinical presentation and strategies of treatment can partially overlap. Psychiatric disturbances are common in patients who suffer from chronic itch, revealing a high psychiatric comorbidity in this population. PP can occur along with a known psychological abnormality or concurrently in patients suffering from other types of itch. While PP incidence in the general population is unknown, its incidence in patients seen in dermatology clinics is about 2%. Moreover, there is a female predominance with an average age of onset between 30 and 45 years of age. The typical clinical findings associated with psychogenic pruritus can vary considerably from superficial excoriations, erosions, or even ulcers to thickened hyperpigmented nodules and atrophic scars. Lesions are commonly found on body areas that are most accessible to the hand,

“Neurologist's contribution to the diagnosis of sine materia respiratory insufficiency: case report”

BackgroundRight-to-left shunt (RLS) may be the cause of marked hypoxemia, a respiratory insufficiency which is usually difficult to diagnose by respiratory physicians as it develops in the absence of an intrinsic lung disease.Case presentationWe report a case of RLS in a patient with a hepatopulmonary syndrome caused by chronic autoimmune cholangitis. RLS was suspected clinically by physical examination and by standard CT imaging and MIP reconstruction of the pulmonary vascular bed. Repeated previous transthoracic echocardiography (TTE) studies did not reveal shunts or any cardiac defect. The final diagnosis was made by means of a minimally invasive transcranial Doppler examination with the use of saline agitated with 0.5 ml of patient’s blood as contrast solution.ConclusionsTranscranial Colour-Coded Duplex Sonography (TCCS) with saline contrast medium injection is described to have a higher sensitivity than TTE and comparable to transesophageal echocardiography (TEE) in RLS diagnosis. The collaboration of neurologists in diagnosing respiratory insufficiency is very important as the examination is simple, well tolerated in comparison with the discomfort associated with transesophageal echocardiography, and minimally invasive in comparison with angiography, which is the last diagnostic procedure in this clinical scenario. In order to confirm RLS, TCCS with blood-saline contrast medium injection should be performed for the diagnosis of chronic hypoxemia for which causes are not detected with routine clinical examinations.

Generalized pruritus sine materia precipitated by exposure to hydroxyethyl starch

Generalized pruritus sine materia precipitated by exposure to hydroxyethyl starch

Sudden cardiac death with normal heart:

Several culprits may be identified at postmortem in sudden death (SD) victims, including coronary artery, myocardial, valve, conduction system, and congenital heart diseases. However, particularly in young people, the heart can be found grossly and histologically normal in a not-so-minor amount of cases (the so-called unexplained SD or "mors sine materia") and inherited ion channel diseases are implicated (long and short QT syndromes, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia). These channelopathies are due to defective genes encoding for proteins of sodium and potassium ion channels at the sarcolemma level or for receptors regulating intracellular calcium release at the sarcoplasmic reticulum level. Postmortem investigation may still represent the first opportunity to make the proper diagnosis also in the setting of a structurally normal heart and the employment of molecular biology techniques is of help to solve the puzzle of such "silent" autopsies. For these reasons, autopsy investigation of cardiac SD should always include sampling for genetic testing to search for the invisible inherited arrhythmogenic disorders, as recommended in the recent guidelines by the Association for European Cardiovascular Pathology.

Prevention of sudden cardiac death in the young and in athletes: dream or reality?

Cardiovascular diseases account for 40% of all deaths in the Western countries, and nearly two thirds of them occur suddenly. Young people (<35 years) are not spared from sudden death (SD) with a rate of 1/100,000 per year. Effort is a trigger with a threefold risk in athletes vs. nonathletes, and sports disqualification is by itself life-saving in people with underlying concealed cardiovascular diseases. Several culprits of cardiac SD may be identified at postmortem and atherosclerotic coronary artery disease is the leading cause (25% of SD cases in the young), mostly consisting of a single obstructive plaque with fibrocellular intimal proliferation. However, the spectrum of cardiovascular substrates is wide and include also congenital diseases of the coronary arteries (mainly anomalous origin), myocardium (arrhythmogenic and hypertrophic cardiomyopathies, myocarditis), valves (aortic stenosis and mitral valve prolapse), and conduction system (ventricular preexcitation, accelerated atrioventricular conduction and block). In up to 20% of cases, the heart is grossly and histologically normal at autopsy (unexplained SD or "mors sine materia"), and inherited ion channel diseases have been implicated (long and short QT syndromes, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia). Targets to treat and prevent SD in the young consist of the following: (a) avoid triggers like effort or emotion, (b) inhibit the onset of arrhythmias with drugs or ablation, (c) switch off arrhythmias with defibrillator, and (d) hinder the recurrence of the disease with genetic counseling and/or therapy. In vivo detection of cardiomyopathies is nowadays feasible by electrocardiogram and/or echocardiography, which resulted in a sharp decline of SD in the athletes in Italy, thanks to obligatory preparticipation screening for sport activity. Genetic screening could play a pivotal role in early detection of asymptomatic mutation carriers of cardiovascular diseases at risk of SD.

Validation of Diagnosis Criteria of Functional Itch Disorder or Psychogenic Pruritus

Sir The demonstration of the role of the brain in the pathogenesis of pruritus confirms that a psychological component could be present in every case of pruritus (1) and that a specific psychogenic pruritus is possible (2). Unfortuna tely, this diagnosis is too often mislabelled as idiopathic pruritus. In order to try to avoid these misdiagnoses, following consensus meetings the French Psycho Dermatology Group (FPDG) has proposed a definition and 10 diagnostic criteria (3). The FPDG proposed to prefer the words “functional itch disorder” (FID) rather than “psychogenic pruritus” and to define FID as “an itch di sorder, where itch is at the centre of the symptomatology, and where psychological factors play an evident role in the triggering intensity, aggravation or persistence of the pruritus”. The 10 diagnostic criteria can be divided into 3 compulsory criteria and 7 optional ones (Table I). For a diagnosis of functional itch disorder, all of the 3 compulsory criteria and at least 3/7 of the optional ones must be met. METHODS With the aim of validating these criteria, the FPDG asked 3 non-French investigators, who are well-known to treat lot of patients with pruritus and who did not participate in FPDG consensus meetings, to use FPDG criteria in cases with a previous diagnosis of psychogenic pruritus. This diagnosis was made by the investigators using their usual diagnostic approach. Questionnaires were completed by investigators, who were selected in Germany, Poland and Sweden. RESULTS AND DISCUSSION Thirty-one patients were included in the study: 23 women and 12 men. The mean age was 64.2 years, range 19–85 years. Seventeen patients were from the Swedish centre, 10 from the German centre and 8 from the Polish centre. All patients presented with chronic pruritus (more than 6 weeks). This pruritus was sine materia, i.e. not associated with primary skin lesion, in all cases and was generalized or localized. There was no identified cause in all cases. A chronological relationship of the occurrence of pruritus with one or several life events that could have psychological repercussions was found in 16 cases (46%), variations in intensity associated with stress in 16 cases (46%), nocturnal variations in 24 cases (69%), predominance during rest or inaction in 20 cases (57%). A psychological disorder was associated in 26 cases (74%). Pruritus was improved by psychotropic drugs in 21 cases (60%) and by psychotherapy in 5 cases (14%). In all cases, 3 compulsory criteria were present. Twenty-seven patients out of 35 presented with 3 optional criteria or more out of 7 (3 criteria: 7; 4 criteria: 13; 5 criteria: 4; 6 criteria: 3). Compulsory criteria appeared well-defined because they were present in all patients. In 8/35 patients (23%), only 1 or 2 optional criteria were found. We propose to classify the diagnosis of FID as doubtful in these cases. In 77%, FPDG criteria allow to confirm this diagno sis. Among optional criteria, nocturnal variations and predominance during rest or inaction appear as very good clinical criteria because they were reported by 69% and 57%, respectively, of patients. An associated psychological disorder was noted by investigators in 73%. Variations according to stress and a chronological relationship of the occurrence of pruritus with one or several life events that could have psychological repercussions were present in only 46%: it could be more difficult spontaneously to spot such a chronology for patients. The effects of treatments do not appear to be discriminant criteria, but they were not used in most cases (especially psychotherapies). They are useful to confirm diagnosis later and to convert a doubtful diag nosis of FID into a definite one. Hence, FPDG criteria and their repartition between compulsory and optional criteria seem to be adequate and discriminant and their use pertinent and helpful. Further studies are needed to validate these criteria

Functional Itch Disorder or Psychogenic Pruritus: Suggested Diagnosis Criteria From the French Psychodermatology Group

Functional itch disorder or psychogenic pruritus is a poorly defined diagnosis. This paper sets out the proposed diagnostic criteria of the French Psychodermatology Group (FPDG). There are three compulsory criteria: localized or generalized pruritus sine materia, chronic pruritus (>6 weeks) and the absence of a somatic cause. Three additional criteria from the following seven items should also be present: a chronological relationship of pruritus with one or several life events that could have psychological repercussions; variations in intensity associated with stress; nocturnal variations; predominance during rest or inaction; associated psychological disorders; pruritus that could be improved by psychotropic drugs; and pruritus that could be improved by psychotherapies.

La coqueluche maligne du petit nourrisson

Pertussis remains in France the first cause of bacterial, infectious death in infant aged 10 days to 2 months. It is especially in this age group that malignant pertussis occurs. Case report. – A 40-day-old infant was admitted in the intensive care unit with symptoms of bronchiolitis along with a 200 bpm permanent tachycardia. He presented a marked leukocytosis with lymphocytosis. On the second day, convulsions and coma occurred, followed rapidly by respiratory failure, with a subsequent deterioration due to the development of severe pulmonary hypertension. Circulatory failure caused the infant’s death on the beginning of the 5th day. Pertussis was confirmed by PCR on nasopharyngeal swab. Intra-familial contamination was most likely. Comments. – Malignant pertussis is characterized by the very young age of patients, permanent tachycardia sine materia, dyspnea with early respiratory failure, frequent neurological symptoms, severe hyperleukocytosis and hyperlymphocytosis, and deep hyponatremia with oliguria and edema. Mortality remains superior to 75% despite the various treatments and life support measures that have been attempted. Adult pertussis, which represents one third of the cases of prolonged cough in this age group, is the main source of contamination of non-immunized young infants. This mode of transmission stresses the importance of the generalization of pertussis vaccine booster in early adolescence, which is recommended in France since 1998. Its extension towards the adult age is under study.