Tay-Sachs Disease

Rarely in this history of pediatrics has a single group of workers so rapidly and effectively brought into focus the special problems of an uncommon disease as have the staff of the Jewish Chronic Disease Hospital in Brooklyn with their studies on Tay-Sachs disease. For a decade they have maintained a steady program of intense interest in this disease led by Drs. Stanley Aronson, Bruno Volk, and Abraham Saifer. A valuable series of personal contributions to the knowledge of this syndrome have resulted, but even more they have stimulated a widespread concern for these pediatric patients in the medical world generally.

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SOME PEDIATRIC PATIENTS OF MORE THAN PASSING INTEREST—WHO WERE THEY?

PEDIATRICS ◽

10.1542/peds.51.5.821 ◽

1973 ◽

Vol 51 (5) ◽

pp. 821-821

Author(s):

T. E. C.

Keyword(s):

Pediatric Patients ◽

History Of ◽

History Of Pediatrics

Each of the eight children below played an important role in the history of pediatrics. Who were they?

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Family History of Diabetes Is Associated with Increased Risk of Recurrent DKA in Pediatric Patients in Rhode Island

Diabetes ◽

10.2337/db18-1378-p ◽

2018 ◽

Vol 67 (Supplement 1) ◽

pp. 1378-P

Author(s):

JANAKI D. VAKHARIA ◽

SUNGEETA AGRAWAL ◽

JANINE BACIC ◽

LISA S. TOPOR

Keyword(s):

Family History ◽

Rhode Island ◽

Pediatric Patients ◽

Family History Of Diabetes ◽

Increased Risk ◽

History Of

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Right frontotemporal craniotomy for ECA-to-MCA direct and indirect bypass and occipital artery indirect bypass to the posterior circulation: case report

Journal of Neurosurgery Pediatrics ◽

10.3171/2020.7.peds20181 ◽

2020 ◽

pp. 1-5

Author(s):

Mitchell W. Couldwell ◽

Samuel Cheshier ◽

Philipp Taussky ◽

Vance Mortimer ◽

William T. Couldwell

Keyword(s):

Case Report ◽

Pediatric Patients ◽

Posterior Circulation ◽

Occipital Artery ◽

Transient Ischemic Attacks ◽

Collateral Vessel ◽

Suboccipital Craniotomy ◽

Uncommon Disease ◽

Indirect Revascularization ◽

The Brain

Moyamoya is an uncommon disease that presents with stenoocclusion of the major vasculature at the base of the brain and associated collateral vessel formation. Many pediatric patients with moyamoya present with transient ischemic attacks or complete occlusions. The authors report the case of a 9-year-old girl who presented with posterior fossa hemorrhage and was treated with an emergency suboccipital craniotomy for evacuation. After emergency surgery, an angiogram was performed, and the patient was diagnosed with moyamoya disease. Six months later, the patient was treated for moyamoya using direct and indirect revascularization; after surgery there was excellent vascularization in both regions of the bypass and no further progression of moyamoya changes. This case illustrates a rare example of intracerebral hemorrhage associated with moyamoya changes in the posterior vascularization in a pediatric patient and subsequent use of direct and indirect revascularization to reduce the risk of future hemorrhage and moyamoya progression.

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Developmental history of pediatrics in Turkestan

International Journal of Pharmaceutical Research ◽

10.31838/ijpr/2020.12.04.097 ◽

2020 ◽

Vol 12 (04) ◽

Keyword(s):

Developmental History ◽

History Of ◽

History Of Pediatrics

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Evaluation of Hypersensitivity Reactions in Pediatric Patients Using Biological Drugs

International Archives of Allergy and Immunology ◽

10.1159/000515500 ◽

2021 ◽

pp. 1-8

Author(s):

Ozge Yilmaz Topal ◽

Volkan Kose ◽

Banu Acar ◽

Umut Selda Bayrakci ◽

Derya Ozyoruk ◽

...

Keyword(s):

Pediatric Patients ◽

Additional Treatment ◽

Hypersensitivity Reactions ◽

Biological Drugs ◽

Rheumatologic Diseases ◽

Culprit Drug ◽

Dose Infusion ◽

History Of ◽

Oncologic Diseases ◽

Biologic Drug

Introduction: Biological drugs are currently used for the treatment of chronic inflammatory, autoimmune, and neoplastic diseases. With their expanding indication spectrum and increasing use, hypersensitivity reactions to these drugs are also becoming more frequent. The present study aimed to report the incidence and the features of such reactions in pediatric patients using biologicals for the treatment of various diseases. Methods: The medical records of pediatric patients treated with biological agents between October 1, 2011 and August 31, 2019 were reviewed and adverse reactions were evaluated retrospectively. Results: During the study period, 211 patients (116 boys, 55%) used 21 different biological drugs for the treatment of various diseases. Their median age at the time of the first treatment was 139.9 (IQR: 92.2–187.8) months. Hematologic-oncologic diseases were the most common indication for biological therapy (97/211; 46.0%), followed by rheumatologic diseases (82/211; 38.9%). Of the 211 patients, 14 (6.64%) experienced reactions to biological drugs. The most common culprit agent was rituximab (57.1%). Most of the patients (85.7%) had a history of reactions either during the infusion or within 1 h after taking the drug. Five patients underwent desensitization to the culprit drug, while 7 other patients continued treatment with a reduced dose/infusion rate or premedication. Also 1 patient continued to take the drug without any additional treatment. Conclusion: It was reported that 6.64% of the patients who received biologic drug therapy for various reasons in our hospital had hypersensitivity. The most common culprit agent was rituximab, and most of the reactions were immediate reactions.

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1339. Antibiotic Prescribing Varies among Patients with a Documented Penicillin or Cephalosporin Adverse Drug Reaction

Open Forum Infectious Diseases ◽

10.1093/ofid/ofaa439.1521 ◽

2020 ◽

Vol 7 (Supplement_1) ◽

pp. S681-S681

Author(s):

Brian R Lee ◽

Jason Newland ◽

Jennifer Goldman

Keyword(s):

Pediatric Patients ◽

Antibiotic Prescribing ◽

Hospitalized Children ◽

Beta Lactam ◽

Research Support ◽

Point Prevalence Study ◽

History Of ◽

Gi Infections ◽

To Receive ◽

Indication For Treatment

Abstract Background Studies have shown that over half of hospitalized children receive an antibiotic during their encounter, of which between 30-50% is considered inappropriate. Antibiotic prescribing is further complicated as approximately 10% of children are labeled beta-lactam allergic, resulting in the use of either broad-spectrum or suboptimal therapy. The purpose of this study was to compare antibiotic prescribing between patients with a documented ADR vs. those without using a nationwide sample of hospitalized children. Methods We performed a point prevalence study among 32 hospitals between July 2016-December 2017 where data were collected via chart review on pediatric patient and antimicrobial characteristics, including the indication for all antimicrobials. In additional, ADR history data were collected on which antimicrobial(s) were documented (e.g., penicillin, cephalosporins). Patients were mutually assigned into either: 1) no documented ADR; 2) penicillin ADR-only; 3) cephalosporin ADR-only; and 4) ADR for both penicillin and cephalosporin. The distribution of antibiotics were compared between the ADR groups, stratified by the indication for treatment. Results A total of 12,250 pediatric patients (17,929 antibiotic orders) who were actively receiving antibiotics were identified. A history of penicillin and cephalosporin ADR was documented in 5.5% and 2.8% of these patients, respectively. When compared to patients with no documented ADR, penicillin ADR patients were more likely to receive a fluoroquinolone for a SSTI infection (odds ratio [OR]: 5.6), surgical prophylaxis (OR: 18.8) or for surgical treatment (OR: 5.2) (see Figure). Conversely, penicillin ADR patients were less likely to receive first-line agents, such as narrow-spectrum penicillin for bacterial LRTI (OR: 0.08) and piperacillin/tazobactam for GI infections (OR: 0.22). Cephalosporin ADR patients exhibited similar patterns with increased use of carbapenems and fluoroquinolones when compared to patients with no ADR. Figure 1: Odds of Receiving Select Antimicrobials Among PCN ADR Patients When Compared to Non-ADR patients, by Indication Conclusion A large, nationwide sample of pediatric patients who were actively prescribed antibiotics helped identify several diagnoses where comprehensive guidelines for appropriate ADR prescribing and increased ADR de-labeling initiatives are needed to ensure optimal treatment. Disclosures Brian R. Lee, MPH, PhD, Merck (Grant/Research Support) Jason Newland, MD, MEd, FPIDS, Merck (Grant/Research Support)Pfizer (Other Financial or Material Support, Industry funded clinical trial)

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Sigmoid Volvulus in 16-year-old Boy with an Associated Anomalous Congenital Band

The American Surgeon ◽

10.1177/000313481307901114 ◽

2013 ◽

Vol 79 (11) ◽

pp. 1140-1141 ◽

Cited By ~ 4

Author(s):

Ann A. Albert ◽

Tracy L. Nolan ◽

Bryan C. Weidner

Keyword(s):

Abdominal Pain ◽

African American Male ◽

Pediatric Patients ◽

Primary Anastomosis ◽

Exploratory Laparotomy ◽

Sigmoid Volvulus ◽

Sudden Onset ◽

Contrast Enema ◽

Pediatric Age Group ◽

History Of

Sigmoid volvulus, a condition generally seen in debilitated elderly patients, is extremely rare in the pediatric age group. Frequent predisposing conditions that accompany pediatric sigmoid volvulus include intestinal malrotation, omphalomesenteric abnormalities, Hirschsprung's disease, imperforate anus and chronic constipation. A 16-year-old previously healthy African American male presented with a 12 hour history of sudden onset abdominal pain and intractable vomiting. CTwas consistent with sigmoid volvulus. A contrast enema did not reduce the volvulus, but it was colonoscopically reduced. Patient condition initially improved after colonoscopy, but he again became distended with abdominal pain, so he was taken to the operating room. On exploratory laparotomy, a band was discovered where the mesenteries of the sigmoid and small bowel adhered and created a narrow fixation point around which the sigmoid twisted. A sigmoidectomy with primary anastomosis was performed. The diagnosis of sigmoid volvulus may be more difficult in children, with barium enema being the most consistently helpful. Seventy percent of cases do not involve an associated congenital problem, suggesting that some pediatric patients may have congenital redundancy of the sigmoid colon and elongation of its mesentery. The congenital band found in our patient was another potential anatomic factor that led to sigmoid volvulus. Pediatric surgeons, accustomed to unusual problems in children, may thus encounter a condition generally found in the debilitated elderly patient.

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