In this study,
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mutations were linked to an autosomal recessive form of congenital immunodeficiency and early-onset bacterial and viral infections. Allogeneic hematopoietic stem-cell ...transplantation led to normalization of T-cell function and clinical improvement.
Combined immunodeficiencies comprise a heterogeneous group of inherited defects of the immune system that are characterized by quantitative or qualitative defects of T lymphocytes. These defects are associated with primary or secondary defects of B lymphocytes.
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In patients with combined immunodeficiencies, impairment of adaptive immunity causes increased susceptibility to early-onset, severe infections with a variety of viruses, bacteria, fungi, and parasites.
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,
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Autoimmune manifestations, allergies, and cancers can also occur.
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Identification of gene defects that cause combined immunodeficiencies has helped patients considerably, provided new and important insights into mechanisms governing T-cell development and function in humans,
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and led to an . . .
BACKGROUND:Colistin is active against most multidrug-resistant, aerobic Gram-negative bacteria. Because of the reported nephrotoxicity during the first years of use of colistin, there were concerns ...of its use in pediatrics where there was limited experience The aim of this study is to document the clinical characteristics and outcomes of use of colistin in pediatric patients at a pediatric intensive care unit in Turkey.
METHODS:We reviewed the medical and laboratory records of 29 critically ill children who were treated with colistin for 38 courses between January 2011 and December 2011 at the Department of Pediatric Intensive Care Unit in Ankara University Medical School, Turkey.
RESULTS:The median age was 17 months (range 3–217 months). Male-to-female ratio was 1:1.37. Ventilator-associated pneumonia (21 courses) was the leading diagnosis followed by catheter-related blood stream infection (6 courses), bacteremia (4 courses), ventriculoperitoneal shunt infection, peritonitis and pneumonia (1 course). The most commonly isolated microorganisms were Acinetobacter baumanni, Pseudomonas aeruginosa, Klebsiella pneumoniae, Serratia marcescens, Stenotrophomonas maltophilia, and Enterobacter cloacae. Two colistin formulations were used. Colimycin (Kocak Farma) was used in 21 colistin treatment episodes. The median dosage of colistin in this group was 5.0 mg/kg/d (2.3–5.6 mg/kg/d). Colomycin (Forest Laboratories) was used in 17 colistin treatment episodes. The median dosage of colistin in the second group was 75,000 International Unit/kg/d (50,000–80,000 International Unit/kg/d). Thirty colistin treatment episodes (79%) had a good or partial clinical response and 8 (21%) had a poor clinical response. Of the 8 colistin treatment episodes with poor clinical response, 3 were in the Colimycin group and 5 were in the Colomycin group. Ten patients died. There was no evidence of neurotoxicity in this study. Nephrotoxicity was observed in 1 patient but was not attributed to colistin because the patient had multiorgan failure at the same time.
CONCLUSIONS:This study in a small cohort of patients suggests that the use of colistin in severe nosocomial infections caused by multidrug-resistant Gram-negative bacteria is well-tolerated and efficacious.
Broad and deep perianal wounds are challenging in both adult and pediatric ICUs. These wounds, if contaminated with gastrointestinal flora, can cause invasive sepsis and death, and recovery can be ...prolonged. Controlling the source of infection without diverting stool from the perianal region is complicated. The option of protective colostomy is not well-known among pediatric critical care specialists, but it can help patients survive extremely complicated critical care management. These authors present three critically ill children who required temporary protective colostomy for perianal wounds because of various clinical conditions. Two patients were treated for meningococcemia, and the other had a total artificial heart implantation for dilated cardiomyopathy. There was extensive and profound tissue loss in the perianal region in the patients with meningococcemia, and the patient with cardiomyopathy had a large pressure injury. Timely, transient, protective colostomy was beneficial in these cases and facilitated the recovery of the perianal wounds. Temporary diverting colostomy should be considered as early as possible to prevent fecal transmission and accelerate perianal wound healing in children unresponsive to local debridement and critical care.
This study aimed to evaluate the cardiovascular manifestations and surveillance of multisystem inflammatory syndrome in children (MIS-C) and to determine the correlation of echocardiographic findings ...with cardiac magnetic resonance imaging findings.
Forty-four children diagnosed as MIS-C with cardiac involvement were enrolled in this observational descriptive study. The diagnosis of MIS-C was made according to the criteria of Centers for Disease Control and Prevention. Clinical findings, laboratory parameters, and electrocardiographic and echocardiographic findings at the time of diagnosis and during follow-up were evaluated. Cardiac magnetic resonance was performed on 28 (64%) cases. The 1-year follow-up imaging was performed in all cases with abnormal initial cardiac magnetic resonance findings.
Forty-four patients (56.8% male) with a mean age of 8.5 ± 4.8 years were enrolled in this study. There was a significant positive correlation between high-sensitivity cardiac troponin T (mean: 162 ± 444.4 pg/ml) and N-terminal pro b-type natriuretic peptide (mean: 10,054 ± 11,604 pg/ml) (p < 0.01). Number of cases with an electrocardiographic and echocardiographic abnormality was 34 (77%) and 31 (70%), respectively. Twelve cases (45%) had left ventricular systolic dysfunction and 14 (32%) cases had pericardial effusion on admission. Three cases (11%) had cardiac magnetic resonance findings that may be attributed to the presence of myocardial inflammation, and pericardial effusion was present in seven (25%) cases. Follow-up cardiac magnetic resonances of all cases were normal. Cardiac abnormalities were completely resolved in all except two cases.
Myocardial involvement can be seen during acute disease, but MIS-C generally does not lead to prominent damage during a year of surveillance. Cardiac magnetic resonance is a valuable tool to evaluate the degree of myocardial involvement in cases with MIS-C.
Whooping cough-like respiratory tract infections (WCLRTI) caused by factors other than the Bordetella pertussis are available. Clinical picture is difficult to differentiate between the B. pertussis ...and viral respiratory infections.
Eighty-five patients with the diagnosis of WCLRTI were divided into 3 groups. Group 1 involved patients with pertussis shown by nasopharyngeal aspirate culture (NAC) and/or PCR. Group 2 consisted of patients who B. pertussis was not detected by NAC however, clinicians still evaluated them as potential patients of pertussis. Group 3 involved patients with the diagnosis of WCLRTI and those with VRTI detected by antigen detection/PCR.
Patients with pertussis had longer duration of the symptoms prior to admission. Paroxysmal cough, whooping, vomiting after coughing, cyanosis, apnea, seizures and abdominal hernias were more common in patients with pertussis. Fever, wheezing, tachypnea, retraction, fine crackles and rhonchi were more common in Group 3. Chest radiographs of patients in Group 3 revealed more bronchopneumonic infiltration, increased aeration, and atelectasis. CRP (C-reactive protein) and ESR (erythrocyte sedimentation rate) were significantly higher in Group 3. Of the patients 43.6% had no pertussis vaccination due to being < 2 months in age and 29.4% had 1 dose.
Pertussis should be thought in differential diagnosis of children with complaints of episodes of paroxysmal cough, cough accompanied by gasping, vomiting after coughing; with leukocytosis, lymphocytosis and a normal chest X-ray. The majority of children with pertussis infection are those who have not had the opportunity for vaccination.
The aim of this study was to determine the clinical characteristics of children demonstrating neurological complications with pandemic influenza (H1N1). We reviewed the medical and laboratory records ...of all children who were hospitalized with neurological symptoms and who had proven influenza virus infection by reverse transcriptase–polymerase chain reaction on nasal and throat swabs. Eight children aged between 10 months and 7 years had neurological complications due to pandemic influenza (H1N1) and five of them were female. Four of them were previously healthy; there was chronic renal failure (CRF) in one and neurologic disease in three patients. Seven of them had seizure and altered consciousness. Seven of them were followed in pediatric intensive care units. We performed lumbar puncture in four patients and their cerebrospinal fluid examinations showed pleocytosis in one and no cell in three specimens. Neuroimaging was performed in four patients and three of them had abnormalities. We diagnosed aseptic meningitis in one, acute disseminated encephalomyelitis (ADEM) in one, acute necrotizing encephalopathy (ANE) in one, meningoencephalitis in one, and status epilepticus in four patients. All patients were treated with oseltamivir and antiepileptic drugs. One patient with CRF died; four previously healthy patients recovered fully, and three patients who had neurologic disorder returned to their previous neurological status. In conclusion, during pandemic influenza (H1N1) infection, neurological complications may be seen in addition to the respiratory infection. The type of neurological involvement may be variable such as triggering seizure, aseptic meningitis, encephalitis, ADEM, and ANE. Neurological complications frequently recover fully especially in previously healthy children, but sometimes a severe clinical course occurs.
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