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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.129, No.5, May 2025
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Original Article
Title
Usefulness of "Pseudo-Koch Phenomenon" after BCG Vaccine for Early Diagnosing of Allergic Disease
Author
Hidemasa Sakai and Takeyasu Igarashi
Department of Pediatrics, Shizuoka City Shizuoka Hospital
Abstract
Background: "Pseudo-Koch phenomenon" means the early redness and swelling at the vaccination site after Bacille Calmette-Guerin (BCG) vaccination, which disappears in a few days and finally demonstrates redness and swelling about 3 or 4 weeks post-vaccination as a normal reaction, while latent tuberculosis is ruled out. However, the clinical significance of this phenomenon remains poorly understood.
Objectives: This study aimed to investigate allergic disease susceptibility of children with pseudo-Koch phenomenon.
Methods: The medical records of children who were diagnosed with pseudo-Koch phenomenon at our department between November 2018 and March 2023 were retrospectively referred, and data on the presence of various allergic disease diagnoses or their absence and other background factors were collected. These records were compared with those of other children who received BCG immunization during a similar period.
Results: The pseudo-Koch phenomenon group demonstrated significantly higher rates of being diagnosed with atopic dermatitis, food allergy, and bronchial asthma than the other groups. For bronchial asthma, the regression analysis adjusted for gender and family history of allergic diseases exhibited a considerable association with an odds ratio of 4.16. For atopic dermatitis and food allergy, the pseudo-Koch phenomenon group demonstrated a trend toward a higher risk of morbidity additively with a family history of allergic diseases.
Conclusion: Children with pseudo-Koch phenomenon should be treated considering the presence of potential allergic disease, which may result in early diagnosis and treatment of allergic disease.
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Case Report
Title
A Case of Non-traumatic Myositis Ossificans with Persistent Sensory Disturbance after Surgical Resection
Author
Tetsuro Kawakami1) Takahiro Matsushima1) Toshiki Nakamura1) Hiroshi Sakakibara1) Satoko Suzuki1) Natsuko Matsuoka2) and Hiroshi Hataya1)
1)Department of General Medical Practice, Tokyo Metropolitan Children's Medical Center
2)Department of Orthopedics, Tokyo Metropolitan Children's Medical Center
Abstract
[Background] Myositis ossificans is a rare benign tumor characterized by a painful mass and ectopic ossification. Some cases show neurovascular involvement and require differentiation from malignancies. However, there are no established diagnostic strategies for myositis ossificans in children. [Case] A 12-year-old boy presented with right lower abdominal pain and a mass. Two months prior, he had experienced right lower abdominal pain with hip motion. The pain worsened, restricting his daily activities for one month. One week before admission, he noticed a growing mass near the right superior anterior iliac spine and visited our hospital. On examination, right hip flexion contracture was noted, with increased pain during flexion and extension and a sensation disturbance localized to the right lateral femoral cutaneous nerve area. Laboratory tests showed slight elevations in C-reactive protein and neuron-specific enolase levels. Computed tomography revealed a 2.0 x 3.0 cm mass. Because of the mass enlargement with signs of nerve injury, we performed a biopsy and resection to differentiate it from a malignancy. As the resected specimen showed typical histopathological findings and USP6 gene rearrangement, the mass was diagnosed as myositis ossificans. Pain and motion restriction resolved within one week after surgery, but sensory disturbances persisted. [Discussion] Direct compression by local inflammation may have contributed to the sensory disturbance. [Conclusion] Although myositis ossificans is a benign tumor, it can cause irreversible neuropathy at an early stage. Evaluation of USP6 rearrangements is also an option for differentiating painful masses from malignancies.
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Case Report
Title
A Case of a 3-Year-Old Boy Diagnosed as Protein S Deficiency Complicated by Cerebral Venous Thrombosis During the Treatment of Acute Upper Respiratory Tract Infection
Author
Ayaka Takeuchi Megumi Nukui Kohei Matsubara Risako Ishioka Naoki Yamada Takeshi Inoue Ichiro Kuki and Shin Okazaki
Department of Pediatric Neurology and Logopedics, Osaka City General Hospital
Abstract
Protein S deficiency (PSD) is an inherited thrombophilia characterized by low levels of PS antigen, free PS antigen, or activated protein C cofactor activity. Patients with PSD are at risk for venous thrombosis, which can be triggered by the interaction of several risk factors, such as immobility, infection, and dehydration.
We report a case of undiagnosed PSD complicated by cerebral venous sinus thrombosis (CVST) with certain risk factors, including mastoiditis, dehydration, and glucocorticoid use.
A 3-year-old healthy boy developed symptoms of a cold and presented to the emergency department of Osaka City General Hospital after experiencing status epilepticus. Computed tomography, magnetic resonance imaging, and magnetic resonance venography led to a diagnosis of CVST involving the superior sagittal sinus, right transverse sinus, and right internal jugular vein. Unfractionated heparin was administered intravenously. The patient was diagnosed with PSD based on blood test results showing low free PS levels and low PS activity. In addition, the patient was diagnosed with sinusitis, mastoid cellulitis, and dehydration and was treated with oral glucocorticoids for the cold. In this case, the interaction of several blood coagulation-accelerating factors, such as PSD, sinusitis, mastoid cellulitis, and oral glucocorticoids, led to CVST.
In conclusion, this case highlights the critical role of undiagnosed PSD and its interaction with other risk factors in the development of CVST. Given that PSD is a relatively common thrombophilia found in approximately 2% of Japanese individuals, it is important to consider thrombophilia, such as PSD, in everyday clinical practice.
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Case Report
Title
Immunosuppressive Therapy for Refractory Kawasaki Disease in a 22-Month-old Male Hepatitis B Virus Carrier
Author
Noriko Sato1) Yoji Uejima1) Satoshi Sato1) Masashi Yoshida2) and Eisuke Suganuma1)
1)Division of Infectious Diseases and Immunology, Saitama Children's Medical Center
2)Division of Gastroenterology and Hepatology, Saitama Children's Medical Center
Abstract
Attention should be paid to the risk of fulminant hepatitis due to hepatitis B virus (HBV) reactivation when administering immunosuppressive therapy to patients with a history of HBV infection. A 22-month-old boy, who was an HBV carrier due to mother-to-child transmission, was diagnosed with Kawasaki disease and treated with intravenous immunoglobulin (IVIG), prednisolone, and aspirin. After the gradual reduction of prednisolone, his symptoms flared up. He was transferred to our hospital after receiving the second administration of IVIG. To avoid HBV reactivation, the patient was given a third dose of IVIG, ulinastatin, and an increased dose of aspirin as additional treatment. However, the treatment was ineffective. Considering that he was a pre-seroconversion asymptomatic carrier, on the advice of a gastroenterology and hepatology physician and with the consent of his family, we administered cyclosporine without nucleic acid analogues. Subsequently, his symptoms and laboratory values improved, and he was discharged without fulminant hepatitis or coronary complications. When managing patients with a history of HBV infection, the antigen-antibody pattern should be used to assess the risk of immunosuppressive therapy and the indications for nucleic acid analogues. Many patients with a history of HBV infection and who are in the age group susceptible to Kawasaki disease are asymptomatic carriers of HBV. Immunosuppressive therapy could, therefore, be a candidate for additional therapy for IVIG-refractory Kawasaki disease for both asymptomatic carriers as well as non-carriers.
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Case Report
Title
ROHHAD Syndrome Following Rapid Obesity and Acute Respiratory Failure in an Infant with Anti-ZSCAN1 Autoantibodies
Author
Taro Akama1) Yuichi Suzuki1) Ichiri Sakuma1) Mika Yamada1) Yuichiro Asano1) Maki Nodera1) Akari Utsunomiya2)3) Hirohito Shima4) and Mitsuaki Hosoya1)
1)Department of Pediatrics, Fukushima Medical University School of Medicine
2)Department of Medical Pediatrics/Genetics, Graduate School of Biomedical and Health Sciences, Hiroshima University
3)Department of Pediatrics, Hiroshima City North Medical Center Asa Citizens Hospital
4)Division of Pediatric Pathology, Graduate School of Medicine, Tohoku University University Hospital
Abstract
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation and autonomic dysregulation (ROHHAD) syndrome is a disease characterized by sudden weight gain, central hypoventilation, hypothalamic dysfunction and autonomic dysregulation. Due to the risk of sudden death from central hypoventilation, it is crucial to diagnose this disease as early as possible, and to provide appropriate respiratory management. We report a case of ROHHAD syndrome that was diagnosed in infancy and was surgically treated via tracheostomy.
The patient was a 2-year-old girl who had had autonomic dysregulation since the age of 12 months, rapid weight gain since the age of 24 months, and respiratory failure at the age of 34 months, which revealed central hypoventilation. ROHHAD syndrome was suspected, based on the patient's symptoms and the absence of a PHOX2B mutation. She experienced hypoxemia and hypercapnia even when awake, and was refractory to respiratory management via noninvasive positive pressure ventilation. As a result, tracheotomy was performed.
Although this disease can be fatal, early diagnosis is challenging due to the absence of a symptom profile in the early stages of the disease. In the present case, central hypoventilation led to suspicion of this disease, but a definitive diagnosis was not reached, and the patient underwent respiratory management as her symptoms progressed. The patient was positive for anti-ZSCAN1 antibodies at the onset of respiratory failure, which was confirmed after tracheostomy. This suggests that anti-ZSCAN1 antibodies may serve as an important marker for early diagnosis and may guide aggressive therapeutic intervention in ROHHAD syndrome.
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Case Report
Title
Invasive Group A Streptococcal Infection Following Influenza in a School-Aged Boy with Generalized Erythema
Author
Hiroki Ando Katsuhiko Kitazawa Akihito Honda Masayoshi Senda Hironobu Kobayashi Mariko Arakawa Takashi Kiyama Kazuma Komori Ryo Hamasaki and Tokio Hoshika
Department of Pediatrics, Asahi General Hospital
Abstract
We report a case of a 10-year-old boy who presented with prolonged fever and erythema following influenza, initially suspected as drug eruption but subsequently diagnosed as invasive group A streptococcal infection (iGAS). The patient was initially diagnosed with influenza and was prescribed medications, accordingly, including antivirals and acetaminophen, on the second day of fever. However, he was later hospitalized because of a prolonged fever (9 days) with generalized erythema. Although blood testing on admission revealed severe inflammation, no clinical findings indicated pharyngitis, and a rapid throat GAS antigen test was negative. Following dermatologic consultation and skin biopsy, prednisolone was initiated for suspected drug eruption, achieving prompt resolution of fever, erythema, and desquamation the following day. However, 3 days later, he presented again with a high fever (41.2°C), somnolence, cerebrospinal fluid pleocytosis, and elevated liver enzymes. Following a blood culture, intravenous antibiotics were initiated, which promptly made him afebrile, facilitating regained consciousness. Culture results for a cerebrospinal fluid sample obtained after initiation of antibiotic treatment were negative, but blood cultures revealed GAS, confirming the diagnosis of iGAS, and 14-day antimicrobial treatment was completed. Although the dermatopathologic findings could not exclude the possibility of a drug eruption, they were not inconsistent with a diagnosis of scarlet fever. The prolonged fever and erythema may have been manifestations of scarlet fever. Increasing evidence indicates that influenza triggers the onset of iGAS infection. Notably, the clinical course of pediatric influenza with upper respiratory tract GAS colonization or infection can differ from that of typical influenza and has occasionally been reported to be associated with the development of iGAS.
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