Primary Immunodeficiency Disorders in Infancy

1999 ◽  
Vol 18 (1) ◽  
pp. 13-22 ◽  
Author(s):  
Barbara Pappas
Keyword(s):  
Primary Immunodeficiency ◽  
Defense Mechanisms ◽  
Clinical Presentation ◽  
Treatment Strategies ◽  
Basic Knowledge ◽  
First Year ◽  
Primary Immunodeficiency Disorders ◽  
Specific Disorders ◽  
First Year Of Life ◽  
Prompt Diagnosis

Certain primary immunodeficiency disorders may become evident during the first year of life. These disorders are characterized by recurrent infections. Prompt diagnosis is necessary, for infants may suffer significant morbidity and mortality. A basic knowledge of host defense mechanisms, including the natural and humoral systems, is essential to the understanding of immunodeficiency disorders.This article discusses the clinical presentation, evaluation, and treatment strategies available to infants with primary immunodeficiency disorders. Five specific disorders are discussed in detail, and specific treatments are reviewed.

Esophageal Interventions in Infants Born with Esophageal Atresia: A Comprehensive Analysis of a National Database

2021 ◽  
Author(s):  
Tatjana T. König ◽  
Emilio Gianicolo ◽  
Luisa Frankenbach ◽  
Eva Wittenmeier ◽  
Oliver J. Muensterer
Keyword(s):  
Esophageal Atresia ◽  
Primary Anastomosis ◽  
Treatment Strategies ◽  
Primary Treatment ◽  
National Database ◽  
First Year ◽  
Rare Malformation ◽  
Low Incidence ◽  
First Year Of Life ◽  
And Training

Abstract Introduction Esophageal atresia (EA) is a rare malformation that often requires a series of procedures, including surgical primary anastomosis, staged repair, and endoscopic procedures. Actual numbers and trends in interventions and variety in treatment strategies remain unclear. Materials and Methods Data from the German federal bureau of statistics containing all EA-related inpatient procedures encoded from 2005 until 2018 were analyzed for children during the first year of life. The sum of esophageal anastomoses and replacements was used to calculate an estimate of incidence of EA. Results Over 14 years, 12,627,888 inpatient cases were recorded in infants in Germany. The mean incidence of EA was 1 per 4,217 live births. On average, 163.3 (95% confidence interval [CI]: 150.8–176.1) esophageal anastomoses, 11.2 (95% CI: 8.7–13.7) esophageal lengthening procedures, and 6.7 (95% CI: 5.42–8.00) esophageal replacements were recorded annually. Overall, 187.8 (95% CI: 147.1–200.4) endoluminal treatments (ballon dilatation, bougienage, stent placement, or injection) were performed per 100 anastomoses. Over the years, bougienage was increasingly replaced by ballon dilatation as primary treatment. Boys had a significantly higher number of esophageal procedures than girls, but the incidence of endoscopic treatments in relation to anastomoses was the same for both genders. Conclusion The low incidence of EA in relation to a relatively large number of units treating those patients in Germany may pose challenges for maintaining competency and training of all specialists involved. The number of esophagoscopic treatments for esophageal stricture per anastomosis is lower than previously estimated.

scholarly journals Suprapatellar bursitis in a newborn

2021 ◽  
Vol 10 (2) ◽  
pp. 199-202
Author(s):  
IIya O. Erenkov ◽  
Ivan S. Denisov ◽  
Svetlana A. Zarubina
Keyword(s):  
Knee Joint ◽  
Blood Test ◽  
White Cell Count ◽  
Treatment Strategies ◽  
Rare Condition ◽  
Well Being ◽  
Patient Treatment ◽  
First Year ◽  
First Year Of Life

Introduction. One of the most frequent pathologies in childhood is bursitis of the knee joint. Such conditions may occur in infectious pathologies, traumatic disorders, overuse syndromes, and other various pathologies. Diagnostic and treatment strategies depend on the cause of bursitis development. Particular attention should be paid to bursitis cases in newborns since the frequency of such pathologies is casuistic and clinical guidelines for treating patients in this age group are absent. Materials and methods. A 26-day-old newborn arrived for consultation with complaints of knee joint pain and movement restrictions for four days. From five to 21 days of life, she has had in-patient treatment at another clinic because of pneumonia and a urinary tract infection. Intravenous and intramuscular injections of antibacterial drugs were prescribed for two weeks. The condition of the newborn was satisfactory upon consultation in our clinic. Her physical examination showed swelling and muscle tightness on the distal part of the thigh. The range of motion of the shinbone was restricted. Her blood test showed that her white cell count was unchanged. According to an X-ray examination of the knee, no pathology was detected. According to the US-examination, exudative suprapatellar bursitis was detected. The nonsteroidal anti-inflammatory drug, Nimesulid, was prescribed for seven days. Results. Daily remote monitoring of the patient's well-being during treatment was performed. Movement restrictions reduced the swelling on day 3 of treatment. A follow-up blood test on day 4 of treatment showed no pathology. According to the follow-up US-examination after six days of drug therapy, no signs of bursitis were detected. Conclusion. Inflammation of the suprapatellar bursa is a rare condition in newborns. In isolated cases of bursitis in children during the first year of life, a bacterial presence was noted, with the predominance of staphylococcal and streptococcal floras. However, in our opinion, there is a possibility of developing aseptic forms of bursitis in infants. In these cases, it may be prudent to refrain from prescribing courses of antibiotic therapy.

scholarly journals A clinical perspective on food-relatA Clinical Perspective on Food-Related Diseases in the First Year of Lifeed diseases in the first year of life

2020 ◽  
Vol 8 (10) ◽  
Author(s):  
Russell Hopp
Keyword(s):  
Gastrointestinal Disease ◽  
First Year ◽  
Food Protein ◽  
Clinical Perspective ◽  
Related Disease ◽  
The Past ◽  
Ige Mediated ◽  
Allergic Proctocolitis ◽  
First Year Of Life ◽  
Prompt Diagnosis

Food-related disease processes can have a wide presentation picture. We present here a review of their clinical patterns in infants to allow a prompt diagnosis and appropriate management. Included in the discussion in infants from birth to 12 months includes IgE-mediated food allergy, food protein–induced allergic proctocolitis , food protein enterocolitis syndrome, atopic dermatitis, eosinophilic esophagitis, and eosinophilic gastrointestinal disease. The bibliography is updated to the past two years unless the latest guideline pre-dated 2018, or the reference was of a classic nature.

scholarly journals A Case Report on Melanotic Neuroectodermal Tumor of Infancy and Review of the Literature

2020 ◽  
pp. 1-6
Author(s):  
Gailann Jugmohansingh ◽  
Gailann Jugmohansingh ◽  
Medford S ◽  
Sharma V ◽  
Ramsingh C ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Benign Neoplasm ◽  
Male Infant ◽  
Neuroectodermal Tumor ◽  
First Year ◽  
Current Case ◽  
Clinical Recurrence ◽  
Neural Crest Origin ◽  
First Year Of Life

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign neoplasm of neural crest origin that most commonly occurs in the first year of life. The craniofacial area is affected 90% of the time with the maxilla being the affected site in the majority of cases. It has a recurrence rate of up to 27% and is associated with malignant transformation in up to 6.5% of patients. The current case describes the clinical presentation and management of a 3-month old male infant with MNTI of the maxilla. Following initial presentation, the tumor grew rapidly to compromise the aerodigestive tract. Surgical resection was performed and the adjacent bone was burred. There has been no clinical recurrence over a 4-year period of follow up.

The Development of Swallowing Respiratory Coordination

2009 ◽  
Vol 18 (1) ◽  
pp. 19-24
Author(s):  
Maggie-Lee Huckabee
Keyword(s):  
Cortical Activation ◽  
First Year ◽  
Healthy Children ◽  
Nasal Airflow ◽  
Single Session ◽  
Primary Finding ◽  
Patterns Of Behavior ◽  
Cortical Modulation ◽  
First Year Of Life ◽  
Swallowing Apnea

Abstract Research exists that evaluates the mechanics of swallowing respiratory coordination in healthy children and adults as well and individuals with swallowing impairment. The research program summarized in this article represents a systematic examination of swallowing respiratory coordination across the lifespan as a means of behaviorally investigating mechanisms of cortical modulation. Using time-locked recordings of submental surface electromyography, nasal airflow, and thyroid acoustics, three conditions of swallowing were evaluated in 20 adults in a single session and 10 infants in 10 sessions across the first year of life. The three swallowing conditions were selected to represent a continuum of volitional through nonvolitional swallowing control on the basis of a decreasing level of cortical activation. Our primary finding is that, across the lifespan, brainstem control strongly dictates the duration of swallowing apnea and is heavily involved in organizing the integration of swallowing and respiration, even in very early infancy. However, there is evidence that cortical modulation increases across the first 12 months of life to approximate more adult-like patterns of behavior. This modulation influences primarily conditions of volitional swallowing; sleep and naïve swallows appear to not be easily adapted by cortical regulation. Thus, it is attention, not arousal that engages cortical mechanisms.

Gastric electrical activity in normal neonates during the first year of life. Effect of lactation with breast milk and formula

2001 ◽  
Vol 120 (5) ◽  
pp. A209-A209
Author(s):  
G RIEZZO ◽  
R CASTELLANA ◽  
T DEBELLIS ◽  
F LAFORGIA ◽  
F INDRIO ◽  
...  
Keyword(s):  
Breast Milk ◽  
Electrical Activity ◽  
First Year ◽  
Gastric Electrical Activity ◽  
First Year Of Life ◽  
Normal Neonates
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