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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.124, No.12, December 2020
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Original Article
Title
Contributing Factors for Maximal Sterile Barrier Precautions during Percutaneous Central Venous Catheter Insertion in Very-low-birth-weight Infants
Author
Yasushi Ohki1)2) Daisuke Kinoshita1)3) Nobuaki Matsunaga1)4) Satoshi Hada1)5) Haruyo Sakaki1)6) and Retsu Fujita1)7)
1)Neonatal Infection Control and Prevention Searching Group in Japan
2)Department of Pediatrics, Kiryu Kosei General Hospital
3)Department of Neonatology, Japanese Red Cross Kyoto Daiichi Hospital
4)Department of Pediatrics, Juntendo University Hospital
5)Department of Neonatology, Hiroshima Prefectural Hospital
6)Department of Nursing, Nishisaitama-chuo National Hospital
7)Innovation & Research Support Center, International University of Health & Welfare
Abstract
Objectives. To explore the contributing factors for maximal sterile barrier precautions (MSB) implementation during percutaneous central venous catheter (PICC) insertion in very-low-birth-weight infants.
Method. Data on 2,383 PICCs were prospectively collected. Contributing factors for MSB adherence were determined by comparing the MSB and non-MSB groups using multiple logistic regression analysis.
Result. Nine factors were significantly different between the 2 groups according to univariate analysis. Low Apgar score at 1 min, success during initial attempt, operator with less than 8 years postgraduate experience, usage of antiseptics other than 10% povidone iodine, adequate waiting time after skin preparation, and the use of a double-lumen catheter were the independent contributors to MSB implementation by multiple logistic regression analysis.
Conclusion. The contributing factors determined by this study may provide important information to improve the adherence to MSB.
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Original Article
Title
Effectiveness of Macrogol 4000 Plus Electrolytes in Improving the Quality of Life of Children with Chronic Constipation and Their Caregivers
Author
Tomomi Fujimura1)2) Ichiro Takeuchi1) Natsuki Ito1) Masaaki Usami1) Takuro Sato1) Hirotaka Shimizu1) Mitsuru Kubota3) Akira Ishiguro2) and Katsuhiro Arai1)
1)Division of Gastroenterology, National Center for Child Health and Development
2)Center for Postgraduate Education and Training, National Center for Child Health and Development
3)Department of General Pediatrics & Interdisciplinary Medicine, National Center for Child Health and Development
Abstract
【Background】
Chronic constipation (CC) affects the quality of life (QOL) of children and their caregivers. In Japan, from 2018, Macrogol 4000 plus electrolytes (PEG+E) was approved for use in patients with CC. This study aimed to evaluate the effectiveness of PEG+E in improving the QOL of children with CC and caregivers.
【Methods】
A self-administered survey was used to collect QOL data from children with CC and caregivers. An original 'Patient-QOL questionnaire' was designed to evaluate the QOL of constipated children. It was applied for children older than 6 years of age and all caregivers. The QOL of caregivers was measured using a 'Caregiver-QOL questionnaire'. Both items are non-validated, 3-point scaled, and measure the following aspects; emotions, daily life, social life, physical condition, and therapeutic effect. Data before and after the introduction of PEG+E were compared statistically.
【Results】
Data were collected from 26 patients (M: F=19: 7, 2-5 years old (7), 6-14 years old (19)) and caregivers. All items of the 'Patient-QOL' and the 'Caregiver-QOL' survey, apart from 'Therapeutic effect' from the Patient-QOL questionnaire, indicated significant improvement following the introduction of PEG+E.
【Conclusion】
PEG+E appeared to be a useful treatment in improving the QOL of children with CC and caregivers.
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Case Report
Title
A Case of Hepatoblastoma in a Child Treated for Chronic Heart Failure Due to Congenital Heart Disease
Author
Yuriko Matsuo1)2) Hideki Motomura1) Yoshinori Kuwahara1) Shinji Tanioka2) Nobuhiro Ito2) Yasutomo Funakoshi2) Hirofumi Fukunaga2) Tomoyuki Hasuwa2) Kaori Ishida3)4) and Hiroyuki Moriuchi2)
1)Department of Pediatrics, National Hospital Organization Nagasaki Medical Center
2)Department of Pediatrics, Nagasaki University Hospital
3)Department of Pathology, Nagasaki University Hospital
4)Department of Pathology and Laboratory Medicine, Kansai Medical University
Abstract
Early diagnosis of hepatoblastoma is often difficult because it lacks specific clinical features. Especially in the presence of an underlying disease that can cause heart failure, physicians often fail to consider hepatoblastoma as a possible cause of hepatomegaly and ascites. We herein report a case of a girl who developed hepatoblastoma during treatment of chronic heart failure due to congenital heart disease.
The girl was diagnosed at birth with Ebstein's anomaly, ventricular septal defect (VSD), and noncompaction of the left ventricular myocardium. At 4 months of age, she underwent VSD closure and tricuspid valve plasty for relief of pulmonary hypertension. At 1 year of age, she underwent the Glenn procedure in response to right heart failure and a decrease in left ventricular function. At 2 years of age, she presented with progressive hepatomegaly, ascites, new-onset vomiting, and fever. Her clinical history, along with the findings of abdominal plain CT and ultrasonography, suggested hepatic abscess; however, antibiotic therapy was ineffective. On the 17th day after admission, rapid progression of anemia and abdominal distension were observed. Her alpha-fetoprotein (AFP) level was 546,952 ng/mL, and abdominal enhanced CT and MRI showed a large heterogeneously enhancing mass in the right lobe of the liver, which was compatible with hepatoblastoma. She failed to respond to intensive combined therapy including transcatheter hepatic arterial embolization, drainage, and chemotherapy, and died on the 44th day after admission. Autopsy provided histological confirmation of embryonic hepatoblastoma.
Oxygen therapy, furosemide treatment, and frequent radiography may be etiologically involved in hepatoblastoma in extremely low-birth-weight-infants. Children with congenital heart disease, who are frequently exposed to the aforementioned factors, may have a higher risk of hepatoblastoma. Malignant hepatic tumors must be considered in the differential diagnosis of hepatomegaly with ascites in patients with congenital heart disease who are refractory to heart failure treatment. Differential diagnosis of hepatomegaly should involve a series of examinations, including imaging studies and AFP measurement.
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Case Report
Title
Refractory Kawasaki Disease Complicated with Acute Generalized Exanthematous Pustulosis
Author
Kana Yamada1) Yuichi Nomura1) Atsunori Baba2) Naohiro Ikeda1) Tomohiro Kubota1) Daisuke Hazeki1) Yuichi Shinkoda1) Ryo Kusubae1) and Koji Sameshima1)
1)Department of Pediatrics, Kagoshima City Hospital
2)Department of Dermatology, Kagoshima City Hospital
Abstract
A 1-year-old boy was given a diagnosis of Kawasaki disease (KD) and initiated treatment with aspirin (ASA), high dose of immunoglobulin (IVIG), and prednisolone (PSL). Just after the disappearance of fever and eruption, there was a recurrence of fever and eruption, with a change in form. An additional IVIG was not effective, therefore he was transferred to our hospital. At referral, he showed severe swollen and scabbing lips and multiform eruptions on the whole body, with some pustular eruptions. After continuation of treatment with PSL, administration of infliximab (IFX), and termination of ASA, his fever subsided. Eruptions disappeared gradually, and putsulosis formations were observed in some eruptions. Biopsy of the eruption at the time of transfer revealed subcorneal blisters filled with neutrophils, consistent with acute generalized exanthematous pustulosis (AGEP).
Discussions: AGEP is one of the severe adverse cutaneous drug reactions. Usefulness of IFX is known in patients with refractory KD, but it has also been reported in patients with refractory AGEP despite treatment with PSL. In the present case of refractory KD and refractory AGEP, continuation of PSL, administration of IFX, and termination of ASA were effective despite treatment with PSL.
Conclusions: Treatment with IFX is one effective option for patients with recurrent KD complicated with refractory AGEP.
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Case Report
Title
Childhood Acute Nephritic Syndrome with Histopathological Features of IgA-dominant Infection-related Glomerulonephritis: A Case Report
Author
Yusuke Takase1)2) Yusuke Nakayama1) Tatsuki Urakawa1) Syuji Akashi1) Hiromu Yamada1) Ayako Sakamoto1) Toshihiko Shirakawa2) Yumiko Nakashima2) Hiroyuki Moriuchi2) and Kazuhisa Nakashima1)
1)Department of Pediatrics, Nagasaki Harbor Medical Center
2)Department of Pediatrics, Nagasaki University Hospital
Abstract
IgA-dominant infection-related glomerulonephritis (IRGN) is a recently established clinical entity of glomerulonephritis that affects adults with underlying diseases and is associated with staphylococcal infection. We report a case of a 10-year-old boy with biopsy-proven IgA-dominant IRGN who was initially suspected of having poststreptococcal acute glomerulonephritis (PSAGN) but lacked serological evidence of streptococcal involvement. He presented with fever, throat pain, and cough. After 2 weeks, palpebral edema appeared. Acute nephritic syndrome was diagnosed at admission and treated with water-salt restriction and a diuretic, resulting in quick edema resolution. The antistreptolysin O (ASO) test did not support the initial diagnosis of PSAGN, which was based on the disease course and decreased serum C3. Histopathological findings, such as endocapillary proliferation, presence of IgA, C3, and IgG deposits in the glomerular capillary wall, and hump-shaped subepithelial electron-dense deposits were compatible with a diagnosis of IgA-dominant IRGN. Under post-biopsy treatment with an angiotensin-converting enzyme inhibitor, his serum creatinine level decreased and proteinuria disappeared.
The prevalence and prognosis of IgA-dominant IRGN in children remain unknown. This disease will likely be overlooked in children with non-PSAGN acute nephritic syndrome unless renal biopsy is performed. Further studies are necessary to clarify our findings.
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Case Report
Title
Toxic Shock Syndrome Due to Community-acquired Methicillin-resistant Staphylococcus aureus in a Boy with Minor Burns
Author
Shigeki Ishii1) Masaya Suzuhigashi1) Ryohei Yokoyama1) Kazunari Takamura1) Noriko Hidaka1) Yoshiko Yamamura1) Tomoko Ohhira1) Hidemi Shimonodan1) Hiroshi Moritake2) Naoko Imuta3) Jyunichiro Nishi3) and Keigo Nakatani1)
1)Miyazaki Prefectural Miyazaki Hospital
2)Division of Pediatrics, Developmental and Urological-Reproductive Medicine Faculty of Medicine, University of Miyazaki
3)Department of Microbiology, Kagoshima University, Graduate School of Medical and Dental Sciences
Abstract
A 4-year-old boy developed toxic shock syndrome (TSS) due to community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) infection after a minor burn.
He sustained a burn at home from accidental spilling of hot water, 6 days prior to presentation and received an antibiotic-containing ointment without hydrocolloid dressing from his family physician. On admission, he developed fever, compensated shock, generalized erythema, and pyuria; therefore, we immediately administered rapid fluid infusion, intravenous antibiotics, and immunoglobulin for suspected severe bacterial infection or TSS. Culture of a skin specimen from his head yielded enterotoxin-producing S. aureus, which was identified as CA-MRSA based on genetic analysis that revealed the SCCmec type IV strain. He was discharged 15 days later without any complications with a diagnosis of probable TSS secondary to CA-MRSA based on the results of the aforementioned testing and the fact that he fulfilled the clinical criteria.
Recently, the prevalence of CA-MRSA infection to all MRSA infections is increasing in Japan. Most cases of pediatric TSS are associated with skin disorders; therefore, pediatricians should consider TSS in the differential diagnosis, particularly in children with burns.
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Case Report
Title
Avoidance of Drug Resistance by Gastrostomy in a Very-Low-Birth-Weight Infant Born with HIV Infection
Author
Eitaro Kume1)2) Chisato Miyakoshi1)2) Masami Sugawara2) Satoru Tsuruta1)2) and Masaru Yamakawa1)2)
1)Department of Pediatrics, Kobe City Medical Center General Hospital
2)Department of Neonatology, Kobe City Medical Center General Hospital
Abstract
Although the number of newborns with HIV infection has decreased, few cases are still reported every year. Moreover, HIV infections in premature infants are rare, thus clinical evidence concerning HIV drugs is insufficient among newborns.
A very-low-birth-weight preterm infant was born from a mother with untreated AIDS. Blood tests revealed extremely high levels of HIV-RNA, leading to the diagnosis of HIV infection. Initial treatment was effective at first, showing a significant decrease in HIV-RNA levels, but we had difficulty achieving completely undetectable levels. The patient had started to refuse to take the medication, resulting in poor drug adherence and leading to antiretroviral drug resistance. In order to achieve infallible delivery of HIV medication, we chose to establish a gastrostoma in the patient. Following gastrostomy, HIV-RNA levels fell below the detection limit and the patient has been free of complications since.
We used the guidelines established by DHHS as reference to determine the dosage, administration and regimen of HIV drugs, also collaborating with other departments and facilities, managing treatment without any severe adverse events. Additionally, referring to previous reports, we discussed the benefits and issues of gastrostomy in patients with poor oral HIV medication adherence.
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Case Report
Title
Diagnosis of Blau Syndrome Based on Cytological Examination of Skin Biopsy and Tendon Sheath Fluid
Author
Yasuhiro Kondo Yasushi Kanda and Mutsuo Ishii
Department of Pediatrics, Japanese Red Cross Nagoya Daini Hospital
Abstract
A 4-year-old boy presented to the Dermatology Department with a chief complaint of sporadic subcutaneous nodules on his abdomen and thighs, which first appeared around the age of 3. The skin rash improved and worsened alternately, regardless of oral antibiotic administration. Skin biopsy suggested epithelioid granuloma, and the patient was referred to the Pediatrics Department with a suspected diagnosis of systemic disease. Cyst-like masses with no associated subjective symptoms were also observed on the dorsal regions of both feet and hands. However, we did not consider the subcutaneous nodules and cyst-like masses to be manifestations of the same illness. Sonography of the non-purulent cyst-like masses on the dorsal areas of the hand indicated fluid accumulation within the dorsal tendon sheaths of the extensor carpi radialis muscle. Cytological puncture indicated granulocyte clusters, suggestive of a systemic inflammatory disease. Blau syndrome was suspected, and further investigation showed p.Met513Thr mutation of the NOD2 gene. Cytological examination of the synovial cystomas led to a diagnosis based on their link to the subcutaneous nodules. Diagnosis of Blau syndrome is often missed owing to exclusive focus on skin rashes. Given that early diagnosis can improve patient quality of life, we should recognize that cyst-like masses in the dorsal regions of both feet and hands are characteristic of Blau syndrome.
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