Cristiane Rúbia Ferreira

Pertussis: the resurgence of a public health threat

Pertussis is an acute and very contagious pulmonary disease, clinically characterized by periods of coughing and paroxysms that may cause death. The disease afflicts mainly the pediatric population and is life threatening to children under the age of 1 year. Since the beginning of the second millennium, the number of cases of pertussis has increased, menacing public health, despite the availability of the pertussis vaccine. The resurgence of the disease among adults and older children creates a reservoir of infection that will afflict the unimmunized or incompletely immunized children. As newborns and infants show the highest mortality rate, immunization during pregnancy is a new strategy to reduce the burden of pertussis. The authors report the case of a newborn that presented respiratory distress accompanied by marked leukocytosis. Bronchiolitis was the initial diagnostic hypothesis, but the clinical picture became typical of pertussis when paroxysmal coughing ensued. Isolation of the Bordetella pertussis and antigenic demonstration by polymerase chain reaction were positive from respiratory secretion. Despite appropriate antibiotic therapy and intensive care management the child died and the autopsy showed characteristic diagnostic findings. The authors call attention to this diagnosis when facing respiratory failure among young children, mainly in the presence of marked leukocytosis. Thorough research on the immunization status of the patient’s social environment is of crucial importance.

Pulmonary cryptococcoma: a rare and challenging diagnosis in immunocompetent patients

Cryptococcal infection is commonly seen in immunocompromised patients, although immunocompetent patients may also be infected. The pathogen’s portal of entry is the respiratory tract; however, the central nervous system is predominantly involved. Pulmonary involvement varies from interstitial and alveolar infiltrations to large masses, which are frequently first interpreted as lung neoplasm. The diagnosis of pulmonary cryptococcosis, in these cases, is frequently challenging, which, in most cases, requires histopathological examination. The authors report the case of a young female patient who presented a 20-day history of chest pleuritic pain and fever at the onset of symptoms. HIV serology was negative and CD4 count was normal. The imaging work-up was characterized by a huge opacity in the left inferior pulmonary lobe with a wide pleural base. Computed tomography showed a heterogeneous mass involving the bronchial tree. Mediastinal involvement was poor, and there was a splenomegaly. The patient underwent an exploratory thoracotomy and inferior lobectomy. The histopathological examination revealed a cryptococcoma. As the serum antigenemia was positive, the patient was scheduled for long-term treatment with fluconazole. The authors call attention to including the cryptococcal infection in the differential diagnosis of lung mass, mainly when localized in the lung bases in immunocompetent patients.

Bacterial sinusitis and its frightening complications: subdural empyema and Lemierre syndrome.

The symptoms of a previously healthy 14-year-old female with an initial history of tooth pain and swelling of the left maxillary evolved to a progressive headache and altered neurological findings characterized by auditory hallucinations, sleep disturbances, and aggressiveness. She was brought to the emergency department after 21 days of the initial symptoms. An initial computed tomography (CT) scan showed frontal subdural empyema with bone erosion. The symptoms continued to evolve to brain herniation 24 hours after admission. A second CT scan showed a left internal jugular vein thrombosis. The outcome was unfavorable and the patient died on the second day after admission. The autopsy findings depicted rarefaction of the cranial bone at the left side of the frontal sinus, and overt meningitis. The severe infection was further complicated by thrombophlebitis of the left internal jugular vein up to the superior vena cava with septic embolization to the lungs, pneumonia, and sepsis. This case report highlights the degree of severity that a trivial infection can reach. The unusual presentation of the sinusitis may have wrongly guided the approach of this unfortunate case.

Subserous lymphangioma of the sigmoid colon: an uncommon cause of acute abdomen in pediatric patients.

Lymphangioma is a rare, benign lesion derived from a malformation of the lymphatic system, which is more frequently found in the head, neck, and axilla. However, it may be present anywhere in the body, and the diagnosis involves adults as children with some distinct clinical features among them. In pediatric patients, abdominal cystic lymphangioma occurs mostly in the mesentery presenting abdominal pain, intestinal obstruction, or, more rarely, hemorrhage. The authors report the case of a child with a short-course history of fever, abdominal pain, and constipation. The physical examination disclosed the presence of an abdominal mass and signs of peritoneal irritation. Imaging was consistent with a cystic lesion compressing the sigmoid colon and laterally displacing the remaining loops. Exploratory laparotomy was undertaken, and a sigmoidectomy, followed by Hartman’s colostomy, was performed. Histological examination revealed the nature of the lesion as a cystic lymphangioma. The authors highlight the clinical features of this entity and call attention to this disease in the differential diagnosis of acute abdomen or abdominal pain, mainly in pediatric patients.

Lemierre's syndrome due to intratumoral abscess of the uvula.

Lemierre’s syndrome (LS), described in detail in 1936, used to be a life-threatening entity until the advent of antibiotics. Tonsillitis or pharyngitis are the main primary infections and oropharyngeal anaerobic flora is the predominant etiology. However, other primary site infections, as well as other microbiological agents have been reported since the first description. Inflammatory symptoms in the neck and marked findings on physical examination predominate the majority of cases. Nonetheless, the authors report the case of a 54-year-old man with a history of dysphagia followed by cough, purulent expectoration, and fever. The bad condition of his dentition was noteworthy. During the diagnostic work-up, an ulcerated lesion in the uvula and a middle lobe pneumonia were disclosed. Streptococcus viridans was isolated from blood culture. On the fifth day of hospitalization, the patient died after a copious episode of hemoptysis. The autopsy findings depicted an abscess within a squamous cell carcinoma of the uvula, pharyngitis with carotid sheath spreading accompanied by pylephlebitis and thrombosis of the internal jugular vein up to the innominate vein, surrounded by an abscess in the mediastinum. Alveolar hemorrhage and pneumonia were also present. We conclude that the ulcerated carcinoma of the uvula housed an abscess, facilitated by the poor oral hygiene, which triggered LS and the descending mediastinitis. Pulmonary involvement was due to the septic embolism from the internal jugular vein. We would like to highlight the uvula abscess as the primary site of infection in this case of LS with S. viridans as the causative agent.

Congenital generalized lymphangiectasia: a rare developmental disorder for non-immune fetal hydrops

Firstly described by Rudolf Virchow in the 19th century, congenital generalized lymphangiectasia is a rare entity characterized by dilation of lymphatic vessels, and was recently classified in primary or secondary lymphangiectasia. Generalized forms may be diagnosed during pre-natal follow-up with ultrasound examination, and, depending on its severity, the newborn outcome is very poor. The authors report the case of a female newborn with a previous diagnosis of fetal hydropsy who was born after a full-term gestation with respiratory failure due to bilateral voluminous pleural effusion and ascites. Physical examination also disclosed syndromic facies. Despite all efforts of the intensive supportive care, the patient died after 24 days of life. The autopsy findings were consistent with the diagnosis of congenital pulmonary lymphangiectasia. The authors call attention to this rare diagnosis in patients with cavitary effusion and respiratory insufficiency at birth.

The role of intratumoral lymphovascular density in distinguishing primary from secondary mucinous ovarian tumors

OBJECTIVE: Ovarian mucinous metastases commonly present as the first sign of the disease and are capable of simulating primary tumors. Our aim was to investigate the role of intratumoral lymphatic vascular density together with other surgical-pathological features in distinguishing primary from secondary mucinous ovarian tumors. METHODS: A total of 124 cases of mucinous tumors in the ovary (63 primary and 61 metastatic) were compared according to their clinicopathological features and immunohistochemical profiles. The intratumoral lymphatic vascular density was quantified by counting the number of vessels stained by the D2-40 antibody. RESULTS: Metastases occurred in older patients and were associated with a higher proportion of tumors smaller than 10.0 cm; bilaterality; extensive necrosis; extraovarian extension; increased expression of cytokeratin 20, CDX2, CA19.9 and MUC2; and decreased expression of cytokeratin 7, CA125 and MUC5AC. The lymphatic vascular density was increased among primary tumors. However, after multivariate analysis, the best predictors of a secondary tumor were a size of 10.0 cm or less, bilaterality and cytokeratin 7 negativity. Lack of MUC2 expression was an important factor excluding metastasis. CONCLUSIONS: The higher intratumoral lymphatic vascular density in primary tumors when compared with secondary lesions suggests differences in the microenvironment. However, considering the differential diagnosis, the best discriminator of a secondary tumor is the combination of tumor size, laterality and the pattern of expression of cytokeratin 7 and MUC2.

Schistosomiasis: a case of severe infection with fatal outcome intensive care settings: case report and evidence-based literature review

Schistosomiasis is one of the most common parasitic diseases, still considered of public health significance. Acute schistosomiasis is of difficult diagnosis and therefore has been overlooked, misdiagnosed, underestimated and underreported in endemic areas. The delay between the exposure to contaminated water and the initial symptoms may explain this challenging diagnosis. Acute schistosomiasis is frequently reported in non-immune individuals while reinfection cases occurring in endemic areas is scarcely documented. The later usually shows a benign course but fatal cases do exist. The authors report a case of a young female patient, in the late puerperium, with a three-month history of weight loss, intermittent fever, cough, thoracic and abdominal pain and increased abdominal girth. Physical examination showed a tachycardia, tachypnea and hypotension. Laboratory tests showed a mild anemia, eosinophilia, and a slightly elevation of liver enzymes. Thorax and abdominal multidetector computed tomography evidenced a diffuse and bilateral pulmonary micronodules and peritoneal and intestinal wall thickening. The patient progressed rapidly to hepatic insufficiency, and death after respiratory insufficiency. An autopsy was performed and the findings were compatible with acute Schistosomiasis in a patient previously exposed to Schistosoma mansoni.

Streptococcus agalactiae septicemia in a patient with diabetes and hepatic cirrhosis

Streptococcus agalactiae is a well-known pathogen during pregnancy and in neonates. Among non-pregnant adults, invasive infection, although rare, is showing increasing frequency, especially in chronically ill, immunosuppressed, or older patients. Although rare, the clinical features of meningeal infection caused by S. agalactiae are similar to other bacterial meningitis. The authors report the case of a middle-aged man previously diagnosed with hypertension, diabetes mellitus, and alcoholic liver cirrhosis, who was admitted at the emergency department with a Glasgow Coma Scale of 11/12, generalized spasticity, bilateral Babinski sign, and hypertension. The clinical outcome was bad, with refractory shock and death within 24 hours of hospitalization. The bacteriological work-up isolated S. agalactiae in the cerebral spinal fluid (CSF), blood, and urine. An autopsy revealed meningoencephalitis, acute myocardial infarction, and pyelonephritis due to septic emboli. The authors point out the atypical CSF findings, the rapid fatal outcome, and the importance of including this pathogen among the etiologic possibilities of invasive infections in this group of patients.

Hemophagocytic syndrome: a dilemma chasing the intensivists

Hemophagocytic lymphohistiocytosis or hemophagocytic syndrome is represented by an uncontrolled inflammatory response characterized by marked histiocyte activation and a cytokine storm. The entity may present a primary or genetic type, and the secondary type is usually triggered by infectious diseases of any kind, autoimmune disease, or neoplasia. This entity, although well described and with definite diagnostic criteria, still remains misdiagnosed because of the overlap presentation with other inflammatory processes. The authors present the case of a 13-year-old girl who was submitted to an appendicectomy complicated with a pericolic abscess, which required a second operation in order to be drained surgically. During the postoperative period of this second surgical procedure, the patient remained febrile, developing cytopenias, and multiple organ failure. Unfortunately, she died despite the efforts of the intensive care staff. The autopsy findings were characteristic of hemophagocytic syndrome. The authors report the case to call attention to this diagnosis whenever unexpected outcomes of infections are experienced.