Isolated Protein C Deficiency in a Newborn.

BACKGROUND: Congenital protein C deficiency is a rare hereditary thrombophilia, neonatal purpura fulminans is the most serious form of this deficit. The purpose of this observation is two-fold. The first is the need to make an early diagnosis in order to improve the prognosis. The second, is to discuss the need. In case of extensive purpura fulminans in the neonatal period, the search for a deficiency in anticoagulant factor, in particular the dosage of protein C, in the newborn and in both parents. METHODS: The diagnosis is biological and is based on the quantitative determination of functionally active protein C. We use the Berichrom® Protein C assay on an automated coagulation analyzer from Siemens Healthcare Diagnostics, which allows the chromogenic determination of Protein C activity. RESULTS: We report an observation of cutaneous necrosis in a newborn having developed a purpura fulminans extensive secondary to a total congenital protein C deficiency. In front of this clinical picture, thrombophilia assessment is requested, revealing an isolated deficit in protein C < 1%. CONCLUSIONS: In the case of extensive purpura fulminans in the neonatal period, the search for a deficiency in anticoagulant factor, in particular the dosage of protein C, is essential in the newborn and in both parents.

[Generalized bullous pemphigoid induced by radiotherapy]. / Pemphigoïde bulleuse généralisée induite par une radiothérapie.

BACKGROUND: There have been a very small number of reported cases of radiotherapy-induced autoimmune bullous disease. We describe a case of generalised autoimmune sub-epidermal bullous pemphigoid (BP) induced by radiotherapy in a female patient presenting squamous cell carcinoma of the vulva. CASE REPORT: In June 2008, a 48-year-old woman underwent vulvectomy with lymph node curettage for squamous cell carcinoma of the vulva. Following surgery, adjuvant radiotherapy was indicated. At the 16th session (dose of 32 Gy), erythema occurred on the irradiation field. At the 23rd session (46 Gy), the patient presented bullous lesions that became generalised after four days with involvement of the oral mucosa. Skin biopsy revealed sub-epidermal bullae and direct immunofluorescence showed continuous linear deposits of IgG and of C3 along the dermal-epidermal junction. Indirect immunofluorescence revealed the presence of antibodies directed against the basement membrane. A favourable outcome was achieved under systemic corticosteroids. No immunotransfer analysis of serum or any other immunological investigations were performed. DISCUSSION: The clinical and histological features of this autoimmune bullous disease were evocative of BP, despite the absence of any formal proof. Radio-induced BP is extremely rare, with only 28 cases being described to date in the literature. The trigger mechanism is poorly understood; radiotherapy appears to cause changes in the antigenic properties of proteins in the dermal-epidermal junction resulting in production of autoantibodies. The radio-induced nature of BP does not affect therapeutic response. The case we report is novel in terms of both generalisation of the eruption and mucosal involvement.

Outcomes of newborns to mothers with COVID-19.

INTRODUCTION: Coronavirus disease 2019 (COVID-19) has spread rapidly across the world. Given the sharply increased infection rate, the number of pregnant women and children with COVID-19 is correspondingly on the rise. SARS-CoV-2 infection is transmitted through droplets; though hypothesized, other transmission routes have not been confirmed. As of now, it remains unclear whether and how SARS-CoV-2 can possibly be transmitted from the mother to the fetus. METHOD: This study examines the medical records of 30 neonates born to women with COVID-19, the objective being to provide documented information on maternal-child transmission and infant outcomes. RESULTS: Out of the 30 newborns, 28 had negative PCR test results for SARS-CoV-2; among their mothers, fifteen had fever, nine had cough and twenty had delivered by cesarean section. The median birth term was 37wk2dy, and twenty of the neonates were male. Most of them were asymptomatic, except for the three who presented with shortness of breath. Two of them were intubated and both died, the first because of severe sepsis and the second due to severe hyaline membrane disease. As regards the two infected neonates, the first represents a probable case of congenital SARS-CoV-2 infection, which appears unlikely in the second case. The outcome for both of them was good, without any complications. CONCLUSION: Maternal-fetal transmission of the SARS- CoV-2 virus was not detected in the majority of the reported cases, although two of 30 neonates had positive qRT-PCR test results. Our study supports the hypothesis that though it seldom actually occurs, in utero SARS-CoV-2 vertical transmission is possible.

[Fixed food eruption caused by liquorice]. / Erythème pigmenté fixe induit par de la réglisse.

INTRODUCTION: Fixed drug eruption is a characteristic cutaneous adverse reaction that recurs at fixed localisations. It is due to medical drugs. However, food may sometimes be responsible for a similar eruption, known as "fixed food eruption". We report a case of fixed food eruption due to liquorice. CASE REPORT: A 22-year-old woman presented with liquorice-induced fixed drug reaction. Topical challenges remained negative both at previously affected sites and in unaffected skin. Therapeutic re-exposure to liquorice confirmed the diagnosis. DISCUSSION: To our knowledge, we report the first case of food drug reaction caused by liquorice.

[Leiomyosarcoma of the vagina: a rare case]. / Le léiomyosarcome du vagin: une observation rare.

Vaginal leiomyosarcoma is unfrequent. We report on a case in a 50-year-old multipara patient who had presented a posterior vaginal swelling since 6 months. The tumor was discovered at the occasion of pains and non hemorragic discharge. The histological pattern of the tumor was well-differentiated spindle cell sarcoma with pleiomorphic areas. The immunohistochemistry confirmed the smooth myogenic differentiation. The treatment consisted of posterior pelvic exenteration extended to the vagina. The patient is alive and free of disease at 20 months of fellow-up.

[Coronary aneurysm in Behcet's disease. Report of a case]. / Anévrisme coronaire au cours de la maladie de Behçet. A propos d'un cas.

Cardiac involvement is rare in Behçet's disease. The authors report the case of a young patient with Behçet's disease and multiple cardiac complications. The remarkable feature of this case was the presence of a coronary artery (first diagonal) aneurysm associated with multiple venous thromboses. Anticoagulant therapy was changed for a platelet aggregant. Surgery was not indicated because of the fragility of the vessels.

Cerebellar liponeurocytoma (lipomatous medulloblastoma).

Liponeurocytoma (lipomatous medulloblastoma) is an uncommon clinicopathologic entity. We report a case of cerebellar liponeurocytoma in an adult and attempt to better characterize this uncommon lesion. A 43-year-old woman presented with symptoms and signs of increased intracranial pressure and cerebellar dysfunction. CT and MRI showed a heterogeneous well-circumscribed mass in the left cerebellar hemisphere with hydrocephalus. Complete surgical excision of the tumor was accomplished through a suboccipital craniotomy. Pathological examination with immunohistochemical study revealed a medulloblastoma with neuronal, astrocytic and lipomatous differentiation. Postoperative radiation therapy was given. After surgery, the patient was followed up for a 16 month-period; symptoms improved dramatically and no evidence of tumor recurrence was found. Careful analysis of the histopathological and immunohistochemical studies correctly identifies liponeurocytoma (lipomatous medulloblastoma). To our knowledge, only 16 cases have been reported. Although this lesion appears to have a better prognosis than the conventional form of adult medulloblastoma, complete surgical removal with postoperative radiation therapy is recommended.

[Caffey disease (infantile cortical hyperostosis)]. / La maladie de Caffey.

CASE REPORT: A case of infantile cortical hyperostosis is reported in a 4-month old infant. The disease was revealed by changes in behavior and swelling of bones. X-rays of bones led to the correct diagnosis. The spontaneous outcome was favorable. CONCLUSION: The clinical, biological and radiological aspects of this disease are described in order to recognize it early.

[Nosocomial infections in neonatal and pediatric intensive care. The appeal of ciprofloxacin]. / Les infections nosocomiales en réanimation néonatale et pédiatrique. Intérêt de la ciprofloxacine.

UNLABELLED: Nosocomial infections, caused by multiresistant bacteria, are very common in neonatal intensive care units (NIU) and they engage the vital prognosis. MATERIAL AND METHODS: From January 1994 to December 1995, 29 children suffered from nosocomial infections due to multiresistant bacteria. RESULTS: Bacteria were isolated in blood cultures and/or in cerebrospinal fluid and included Klebsiella (14 cases), Enterobacter (eight cases), Pseudomonas (three cases), Acinetobacter (one case), Stenotrophomonas maltophilia (one case) and Flavobacterium odorantum (one case). After preliminary antibiotic therapy, ciprofloxacin was introduced and associated with another antibiotic for 10 days. Outcome was favorable in 25 cases with sterilization of blood culture. Four deaths were due to acute respiratory failure. One case of skin rash and five cases of transient thrombocytopenia were observed during the six days of ciprofloxacin therapy. No articular complication and no dental abnormalities were observed during the 14-38-month follow-up. Ciprofloxacin appears to be a good therapeutic choice for the treatment of severe nosocomial infections in NICU. Side effects are rare, mild, and transient. However, the prevention of nosocomial infection remains essential.

[Orbital location of Kaposi's sarcoma]. / A propos d'une localisation orbitaire du sarcome de Kaposi.

Kaposi's sarcoma is a proliferative disease, probably induced by human herpes virus type 8 (HHV8). Its expression is cutaneous and visceral, with four clinical forms. An orbital location of Kaposi's sarcoma remains exceptional. In order to evaluate the epidemiological, clinical, and therapeutic objectives of this tumor location, we report a case of a 58-year-old patient hospitalized in the ophthalmology department for a surgically treated right orbital tumor.

[Angiosarcoma of the right atrium. Presentation of a surgically treated case and comparison with data of the literature]. / Angiosarcome de l'oreillette droite. Présentation d'un cas opéré et comparaison avec les données de la littérature.

The authors report the case of a 28-year-old woman referred to their department by a respiratory medicine department with an inferior mediastinal tumour arising from the right atrium, presenting in the form of dysponea, dry cough and chest pain associated with a general syndrome composed of fever, weight loss and physical asthenia. Physical examination revealed a superior vena cava syndrome, the electrocardiogram showed diffuse repolarization disorders and the chest x-ray showed an opacity of the anterior and inferior mediastinum. The diagnosis of tumour of the right atrium was based on echocardiography and thoracic CT scan. Subtotal surgical resection under cardiopulmonary bypass allowed examination of the histological type of the tumour. After routine chemotherapy, despite negative secondary staging and a favourable immediate course, the patient died 11 months after the operation in a context of local recurrence and hepatic and bone metastases.

[Undifferentiated sarcoma of the prostate: report of a case]. / Sarcome indifférencié de la prostate: à propos d'une observation.

The authors report one case of indifferentiated sarcoma of the prostate revealed by phlebitis of the right inferior limb and lung's metastases in a man 41 years old. The tumor progress rapidly and infiltrate the bladder and the posterior urethra. The patient died five months later. They review the literature and study the clinical histological, therapeutic and evolutive aspects of this unusual tumor.

[Spontaneous perirenal hematomas. Report of 3 cases]. / Les hématomes spontanés de la loge rénale. A propos de trois observations.

Spontaneous retroperitoneal hemorrhage is an uncommon affection, the diagnosis was recognized by sonography and CT scan, but the etiology remains unknown and exploration for diagnosis may become necessary. In the absence of an apparent etiology, patients with spontaneous renal bleeding should undergo radical nephrectomy, because of the extremely high incidence of small undetectable occult tumors. Three further cases were reported by the authors, who made a review of the literature.

[Multiocular renal cysts in adults. Two case reports]. / Les kystes multiloculaires du rein chez l'adulte. A propos de deux observations.

The authors report two cases of renal multilocular cyst and review the cases reported in the literature, revealing the original features of this benign tumour which has an equal incidence in children and adults with a controversial acquired or malformative aetiopathogenesis, precise histological features, but a difficult preoperative and intraoperative diagnosis despite modern imaging techniques. In the great majority of cases, treatment consists in nephrectomy.

[Aglomerular segmental hypoplasia of the kidney: report of a clinical case]. / L'hypoplasie segmentaire aglomérulaire du rein: à propos d'un cas clinique.

Aglomerular segmental hypoplasia of the kidney is a disease that is uncommon in the adult; the average age at appearance of symptoms is between ten and 15 years. A clear female predominance has been noted, and this disease is often misdiagnosed as chronic pyelonephritis. However, the radiological and pathological characteristics are different to the latter. The etiology of aglomerular segmental hypoplasia has not yet been determined. It is important to acquire further knowledge regarding this disease, as in certain cases this information could help to avoid unnecessary nephrectomy.

[Pyelocolic fistula: a case study]. / Les fistules pyélocoliques: à propos d'une observation.

In this study, the case has been reported of a left renocolic fistula that was detected in a patient with hepatitis C and cirrhotic complications who was hospitalized for urogenital tuberculosis. It was decided to perform left nephrectomy and digestive suture, but surgery was delayed due to hemostatic abnormalities and massive cytolysis, and the patient died before surgical treatment, three days after the discovery of the fistula. The findings in the literature have been reviewed, and the common occurrence of this type of fistula has been underlined. They are the most frequently encountered type with of entero-urinary fistula, and account for 60% of documented cases. Their etiological and clinical characteristics of have been described in detail. In general, treatment consists of performing a nephrectomy and digestive suture.

[Testicular metastasis of prostatic adenocarcinoma: report of 2 cases]. / Métastases testiculaires d'un adénocarcinome de la prostate: à propos de deux cas.

Prostatic carcinoma metastasizing to the testis is an extremely rare occurrence. In this study, two new cases have been reported of unilateral testicular metastases from an adenocarcinoma of the prostate. Unlike primary tumors of the testis, this type of tumor develops later in life (between 50 and 60 years of age), and is in most instances unilateral. It is generally discovered accidentally during orchiectomy or autopsy. It is not always easy to establish a morphological diagnosis, as the macroscopic appearance of the tumor may be normal. However, microscopic investigation reveals the presence of neoplasms of the testis.

[Anorectal tuberculosis disclosed by hemorrhoidal thrombosis]. / Tuberculose ano-rectale révelée par une thrombose hemorroïdaire.

The authors report on a case of anorectal tuberculosis. Revealed by an hemorroïdal thrombosis with febrile symptoma and purulent discharge, its macroscopic aspect was the one of anal mucous membrane ulceration, irregular with elevated border. The diagnosis was confirmed by anatomopathologic exam. The course was favourable under specific treatment. Etiologic investigation did not show any other localisation. Then, present aspects of anorectal tuberculosis are discussed, as well as possible relationships with hemorroïdal disease and its complications, particularly the fissural ones.

[Chronic alveolar syndrome]. / Syndrome de condensation alvéolaire chronique.

We report a case of primary pulmonary Hodgkin's disease in a 20 year-old woman. The chest x-ray showed a chronic alveolar syndrome. The diagnosis was established from a pulmonary biopsy. The radiological features and the options for diagnosis of primary pulmonary Hodgkin's disease are discussed.