DISSEMINATED PATHERGIC GRANULOMATOSIS IN A 4-MONTH-OLD INFANT: A CASE REPORT

PEDIATRICS ◽  
1967 ◽  
Vol 40 (6) ◽  
pp. 975-979
Author(s):  
Joseph O. Collins ◽  
Harold A. Rosenberg ◽  
Philip Warren

A 4-month-old infant in whom sudden death terminated a clinical picture which included conjunctivitis but was dominated by high fever, leukocytosis, and clinical signs and x-ray evidence of pneumonitis is reported along with autopsy findings. The pathologic findings were dominated by a productive, exudative, and thrombotic occlusive vasculitis involving principally the coronary arteries and their branches with a lesser degree of vasculitis and granulomatous inflammation of lungs and spleen. The clinical picture may have given a clue to the underlying pathology. Fienberg's term, "pathergic granulomatosis," may be appropriately applied to cases of this type until more is known of the etiology.

2013 ◽  
Vol 3 (2) ◽  
pp. 170-172
Author(s):  
Emina Vukas ◽  
Aida Dizdarević ◽  
Senka Mesihović - Dinarević ◽  
Adisa Čengić

Common variable immunodeficiency (CVID) or acquired hypogammaglobulinemia is the type of primary immunodeficiency. Deregulation of the immune system, leading to hypogammaglobulinemia, defective activation and proliferation of T cells and dendritic cells, and malfunction of the cytokines are observed in CVID. The clinical picture of CVID varies, any organ or system can be affected, therefore the diagnosis is often difficult and delayed and sometimes is not always possible. This article describes a twelve years old boy with all the clinical signs of immunodeficiency, as confi rmed by laboratory. The main treatment consists of life-long immunoglobulin substitution in intravenous or subcutaneous form.


2015 ◽  
Vol 46 (S 01) ◽  
Author(s):  
J. Spiegler ◽  
Y. Hellenbroich ◽  
U. Ahting ◽  
P. Freisinger

2017 ◽  
Vol 2 (1) ◽  

Introduction: Congenital Syphilis (CS) occurs through the transplacental transmission of Treponema pallidum in inadequately treated or non-treated pregnant women, and is capable of severe consequences such as miscarriage, preterm birth, congenital disease and/or neonatal death. CS has been showing an increasing incidence worldwide, with an increase of 208% from 2009 to 2015 in Brazil. Case report: 2-month old infant receives care in emergency service due to edema of right lower limb with pain in mobilization. X-ray with osteolytic lesion in distal fibula. Infant was sent to the Pediatrics Oncology clinic. Perinatal data: 7 prenatal appointments, negative serology at 10 and 30 weeks of gestation. End of pregnancy tests were not examined and tests for mother’s hospital admission were not requested. Mother undergone elective cesarean section at 38 weeks without complications. During the pediatric oncologist appointment, patient showed erythematous-squamous lesions in neck and other scar-like lesions in upper body. A new X-ray of lower limbs showed lesions in right fibula with periosteal reaction associated with aggressive osteolytic lesion compromising distal diaphysis, with cortical bone rupture and signs of pathological fracture, suggestive of eosinophilic granuloma. She was hospitalized for a lesion biopsy. Laboratory tests: hematocrit: 23.1 / hemoglobin 7.7 / leukocytes 10,130 (without left deviation) / platelets 638,000 / Negative Cytomegalovirus IgG and IgM and Toxoplasmosis IgG and IgM / VDRL 1:128. Congenital syphilis diagnosis with skin lesions, bone alterations and anemia. Lumbar puncture: glucose 55 / total proteins 26 / VDRL non reagent / 13 leukocytes (8% neutrophils; 84% monomorphonuclear; 8% macrophages) and 160 erythrocytes / negative VDRL and culture. X-ray of other long bones, ophthalmological evaluation and abdominal ultrasound without alterations. Patient was hospitalized for 14 days for treatment with Ceftriaxone 100mg/kg/day, due to the lack of Crystalline Penicillin in the hospital. She is now under outpatient follow-up. Discussion: CS is responsible for high rates of morbidity and mortality. The ongoing increase of cases of this pathology reflects a severe health issue and indicates failures in policies for the prevention of sexually transmitted diseases, with inadequate follow-up of prenatal and maternity protocols.


ORL ro ◽  
2016 ◽  
Vol 1 (1) ◽  
pp. 48-50
Author(s):  
Adina A. Zamfir-Chiru-Anton ◽  
D.C. Gheorghe

The authors present the case of a 4-year-old child admitted to the ENT Department with possible pulmonary foreign body aspiration. A detailed history revealed a clinical picture that seemed to depict an absence episode (with partial loss of conscience and cianosis) occured when eating, less the symptomes of a respiratory foreign body. Diagnosis needed full respiratory endoscopy and neurologic evaluation for correct assesment and effective therapy approach.


Author(s):  
Valentin N. Druzhinin ◽  
Vadim G. Suvorov ◽  
Nikolay V. Druzhinin ◽  
Aleksandr N. Cherniyi ◽  
Sergey N. Troynyakov

Currently, the problem of reducing the risk of developing fat liver hepatosis from exposure to household and industrial toxicants among the working-age population continues to be an important medical and social problem, since not timely diagnosis of the disease can lead to its progressive course with the development of inflammatory changes, necrosis and liver fibrosis up to cirrhosis and hepatocellular cancer. In this regard, the search for methods and techniques that optimize the diagnosis of fat hepatosis is relevant. Modern methods of radiation diagnostics of liver density characteristics can significantly reduce subjectivity in the assessment of changes due to the use of quantitative indicators. The aim of study - improving the quality of x-ray diagnostics of fat liver disease based on a precision assessment of the density of the liver parenchyma using computed tomography. A comparative retrospective analysis of the results of a comprehensive clinical and radiological examination of 115 men of working age in the range of 40-55 years was performed. The main group (48 people) - employees of machine-building plants: shapers, stumpers, fitters-assemblers who had industrial contact with such factors as local vibration, dust, noise, muscle strain, burdened with a long alcoholic history and the presence of signs of metabolic syndrome: hyperlipidemia, impaired tolerance to carbohydrates, diabetes, abdominal obesity. The comparison group included representatives of auxiliary professions without clinical signs of pathology (47 people), comparable in age and experience with the main group. X-ray examinations were performed using computer tomographs: "HI Spead CT/e Dual" by GE Medical Systems and "Aqulion 64" by Toshiba. To measure the liver density in Hounsfield units (HU), the ROI (zone of interest) tool was used, which allows determining the desired value over areas of different dimensions. Measurements were performed on computer screens in 4 zones of interest at 4 levels of scanning of the liver lobes (apex, level of the caval gate, level of the left lobe, level of the portal gate) with the calculation of the average values of the density index (IDH) and density gradients (IDG) relative to the aorta, spleen and kidney. Analysis of the results of a posteriori CT densitometry of various parts of the liver within the framework of the developed algorithm, including the use of absolute and relative (gradient) x-ray density indicators of hepatic, vascular (aorta),splenic and renal structures, allowed us to expand our understanding of the quantitative density characteristics both in normal and in patients with signs of diffuse fat hepatosis (FH). It was found that the liver parenchyma density indicators can be a kind of (conditional), sometimes the only indicators of the degree of severity of changes that objectively manifest positive or negative dynamics of pathophysiological processes and, in particular, at the initial stages of the development of the studied pathology. Density differences in the right and left liver parenchyma in the control group (conditional norm) in terms of absolute density and its gradient, regardless of the level of scanning, were insignificant (statistically unreliable). In patients with clinical signs of fatty liver infiltration at the stage of steatosis, in the absence of x-ray morphologically detectable structural changes, a decrease in IDH and the dynamics of its increase (recovery) at various stages of observation were revealed. Even with comparatively equal IDH of the evaluated departments, the IDG of different people differed, manifesting the individuality of metabolic processes occurring in the body, in particular in the liver, is a kind of indicator of their direction and severity. The significance of density indicators as predictors of the subsequent stages of the pathology under consideration was particularly evident in the analysis of the results of primary diagnostics and its development in the dynamics of observations. The application of the developed methodological approach allowed us to expand our understanding of the possibilities of KT-liver densitometry in patients with metabolic syndrome (hyperlipidemia, impaired carbohydrate tolerance, diabetes mellitus, abdominal obesity) in the diagnosis of fatty liver disease (FLD) at various stages of examination, including in the early subclinical phases of pathology development. The results obtained indicate the predominant role of ethyl alcohol as a hepatotoxicant in the development of FLD in the estimated cohort of the working-age population. The use of an original algorithm for evaluating tissue density makes it possible to significantly ensure the objectivity of the interpretation of research results.


Author(s):  
Oscar Westin ◽  
Abbas Ali Qayyum

Background: Recurrent episodes of isolated pericardial effusion due to tuberculosis, leading to reduced Left Ventricle Ejection Fraction (LVEF), are uncommon. Methods: This is a case report of a previously healthy 32-years old male with tuberculous induced pericardial effusion as isolated manifestation. The only known exposure of tuberculosis was a brother with whom the patient did not have physical contact during the last year. The pericardial effusion repeatedly appeared after being drained a total of three times. Due to recurrent episodes of pericardial effusion, severe thickening of the pericardium, pericardial adherences and increasing affection on the heart, pericardiectomy was ultimately performed. Results: Biochemical examination, chest X-ray, computed tomography of thorax and abdomen and cytology report did not reveal any signs of malignancy, connective tissue disease or other infections including extra-pulmonary/pulmonary tuberculosis. However, the pericardial biopsy was Polymerase Chain Reaction positive (PCR) for tuberculosis DNA and showed granulomatous inflammation with necrosis. After 6 months anti-tuberculous therapy, biochemical parameters, LVEF and the clinical condition of the patient were normalized. Conclusion: Tuberculosis can be difficult to diagnose when it only manifests as pericardial effusion especially if the time for exposure is long before the appearance of symptoms and admission.


1970 ◽  
Vol 3 (1) ◽  
pp. 27-34
Author(s):  
Lucas Tavares Dos Santos ◽  
Tânia Massini Evangelista

Introdução: A hérnia diafragmática congênita é a falha do fechamento embrionário do músculo diafragmático, resultando em um defeito de continuidade. Esta patologia pode ocorrer pela passagem de estruturas do abdome através de um defeito no diafragma, ou haver herniação parcial do estômago através do hiato esofágico, paralisia frênica com deslocamento do conteúdo abdominal para cima, mas sem herniação, e, eventração do diafragma. Casuística: Foi relatado um caso de hérnia diafragmática congênita, hérnia de Bochdalek, em um recém – nascido do sexo feminino, que nos ultra-sonografias da gestante apresentavam sem alterações. O diagnóstico da patologia foi feito apenas após a realização de raios-X de tórax e abdome para confirmar a posição do cateterismo umbilical venoso. Discussão/Conclusão: A apresentação clínica da hérnia de diafragmática congênita inclui desconforto respiratório moderado a grave com repercussão sistêmica. O diagnóstico, em cerca de 80% dos casos, é feito por ultrassom pré-natal. O tratamento proposto foi intubação endotraqueal com ventilação mecânica e programação para correção cirúrgica da hérnia. Após correção cirúrgica da patologia, a paciente permaneceu na unidade de terapia intensiva neonatal por 21 dias para acompanhamento de pós – operatório e intercorrências na evolução. Palavras-chave: hérnia diafragmática congênita, recém-nascido, hérnia de BochdalekABSTRACTIntroduction: Congenital diaphragmatic hernia is the failure of embryonic closure of the diaphragm, resulting in a lack of continuity. This condition can occur by passing structures of the abdomen through a defect in the diaphragm, or be part herniation of the stomach through the esophageal hiatus, phrenic paralysis with displacement of abdominal contents up but no herniation, and eventration of the diaphragm. Case Report: We report a case congenital diaphragmatic hernia, such as Bochdalek hernia, in a new - born female that in ultrasounds of pregnant women showed without change. The diagnosis of the disease was made only after conducting X-ray of the chest and abdomen to confirm the position of umbilical venous catheterization. Discussion/Conclusion: Clinical presentation of congenital diaphragmatic hernia includes moderate to severe respiratory distress with systemic repercussions. The diagnosis in about 80% of the cases is done by ultrasound prenatally. The proposed treatment was endotracheal intubation with mechanical ventilation and programming for surgical correction of the hernia. After surgical pathology, the patient remained in neonatal intensive care unit for 21 days to monitor post - operative complications and evolution.  Keywords: congenital diaphragmatic hernia, newborn, Bochdalek hernia 


Sign in / Sign up

Export Citation Format

Share Document