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Farah Hendara Ningrum
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Journal : Medica Hospitalia

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Seorang Anak Perempuan 1 Bulan Dengan Apert Syndrom (Acrocephalosyndactyly Syndrome Type 1) Nareswari, Tara; Ningrum, Farah Hendara
Medica Hospitalia : Journal of Clinical Medicine Vol. 9 No. 1 (2022): Med Hosp
Publisher : RSUP Dr. Kariadi

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (1050.023 KB) | DOI: 10.36408/mhjcm.v9i1.707

Abstract

Latar Belakang Sindrom apert merupakan salah satu jenis acrocephalosyndactyly yang paling dikenal dan disebabkan oleh mutasi gen fibroblast growth factor receptors2 (FGFR2) Acrocephalosyndactyly didefinisikan sebagai sindrom kongenital ditandai dengan penutupan yang terlalu dini dari sutura sutura tengkorak (craniosynostosis) Hal ini menghasilkan kepala dan wajah yang berbentuk tidak normal, serta fusi jari tangan dan kaki (sindactyly) 1. Tujuan studi kasus ini adalah untuk mengetahui penegakhan diagnosis sindrom apert Laporan kasus Pasien datang ke poli anak RS Kariadi Semarang tanggal 15 Oktober 2018 yang merupakan rujukan dari RS Keluarga Sehat Pati, dengan assesment suspek sindrom tertentu DD/ Craniosinostosis syndrom, Baller gerold syndrom. Orangtua pasien mengeluhkan bahwa saat bernafas anak berbunyi grok grok. Kesan dari pemeriksaan radiologi skeletal survey adalah Craniosynostosis yang membentuk gambaran Harlequin Eye, Syndactyly manus kiri, Polyndactyly pedis kiri, yang mana dari keseluruhan tanda klinis tersebut mendukung gambaran Apert Syndrome. Sedangkan kesan dari pemeriksaan MSCT kepala tanpa kontras adalah Sutura coronaria kanan kiri tampak sudah menutup, microcephali, brachicephalic, tak tampak gambaran hidrocephalus, tak tampak kalsifikasi patologis yang mencurigakan suatu infeksi kongenital, tak tampak tanda tanda peningkatan tekanan intracranial Pembahasan Apert syndrome (acrocephalosyndactyly) adalah kelainan perkembangan yang jarang terjadi, ditandai oleh craniosynostosis, hipoplasia midface, syndactili simetris tangan dan kaki. Karakteristik prodromal untuk penampilan wajah-cranio yang khas adalah craniosynostosis awal jahitan koronal, dasar kranial dan agenesis dari jahitan sagital. Apert Syndrome adalah sindrom craniosynostosis yang paling dikenal luas Apert syndrome merupakan acrocephalosyndactyly tipe I adalah malformasi kongenital yang langka dengan dikarakteristikan oleh kraniosinostosis, hipoplasia mid-face, syndactyly pada tangan dan kaki. Pada pasien ini tampak synostosis sutura coronaria kanan kiri yang membentuk gambaran harlequin eye. Syndactyly tangan dan kaki, dengan syndactyly pada tangan yaitu tipe 2 atau biasa disebut mitten hand yaitu tampak fusi jari ke 2- 4 tanpa keikutsertaan ibu jari. Dengan penampilan klinis pada mid face pasien menunjukan wajah dismorfik, flat facies, mata proptosis, hidung low nasal bridge, dan mulut palatum letak tinggi Kesimpulan Apert Syndrom merupakan salah satu tipe Acrocephalosyndactyly yaitu kelainan kongenital akibat mutasi heterozigot pada gen FGFR2 dengan sifat dominant autosomal, yang ditandai dengan craniosinostosis, hipoplasia midface, dan syndactyly pada tangan dan kaki. Pemeriksaan radiologi memiliki peranan penting dalam mendiagnosa kelainan ini
Correlation between Preoperative Osteitis Degree with Postoperative Endoscopic Score in Chronic Rhinosinusitis Apriansyah, Apriansyah; Dewi, Anna Mailasari Kusuma; Ningrum, Farah Hendara
Medica Hospitalia : Journal of Clinical Medicine Vol. 10 No. 1 (2023): Med Hosp
Publisher : RSUP Dr. Kariadi

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.36408/mhjcm.v10i1.784

Abstract

BACKGROUND: In chronic rhinosinusitis patients, there is a subgroup of patients with persistent symptoms after treatment, who often shows bone thickening and remodeling in their paranasal sinuses and described as osteitis. Osteitis can be evaluated by several methods, one of which is the Global Osteitis Score (GOS) AIM: To analyse the correlation between preoperative osteitis degree with the postoperative endoscopic scores in chronic rhinosinusitis METHOD: It was an observational study with cross-sectional design. We use GOS from CT scan before surgery and Lund Mackay’s endoscopic score 4 weeks after Functional Endoscopy Sinus Surgery (FESS). The analysis performed with Spearman correlation test. RESULT:  There were 30 patients as subjects. There statistical analysis test between preoperative osteitis degree with post operative endoscopic score showed p = 0.296 with r = 0.197. The analysis result between risk factors for wound healing with the postoperative endoscopic score were allergic rhinitis (p = 1.000), polyps (p = 0.624), and smoking (p = 0.309) CONCLUSION: There is no significant correlation between preoperative osteitis degree with the postoperative endoscopic score, but there is a tendency for a positive correlation between preoperative osteitis degree with the postoperative endoscopic score. There was no significant correlation between allergic rhinitis, polyps, and smoking habits with postoperative endoscopic score
Significant Relationship Between Brixia Score And The Degree Of Acute Respiratory Distress Syndrome In Covid 19 Patients Ibrahim, Irni Dwi Aprianty; Satoto, Bambang; Handoyono, Thomas; Santoso, Antonius Gunawan; Sukmaningtyas, Hermina; NINGRUM, FARAH HENDARA
Medica Hospitalia : Journal of Clinical Medicine Vol. 11 No. 2 (2024): Med Hosp
Publisher : RSUP Dr. Kariadi

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.36408/mhjcm.v11i2.993

Abstract

BACKGROUND: Chest X-ray has an important role in detecting early features of COVID-19. To improve risk stratification, a scoring system in chest x-ray called Brixia Score was developed. The Brixia score is designed to measure the severity of lung abnormalities in COVID-19, with an 18-point severity scale. Deaths in COVID-19 occur mainly due to Acute Respiratory Distress Syndrome (ARDS). ARDS is classified into mild, moderate, and severe degrees. If the degree can be predicted earlier, patients can receive earlier therapy and death rate can be reduced. AIMS:  to analyze relationship between Brixia Score and degree of ARDS in COVID-19 patients. METHOD: the research used an observational analytic method with a cross-sectional approach to 95 subjects who are positive for COVID-19 and diagnosed with ARDS, in January to December 2021. Brixia Score data was collected based on chest X-ray expertise, ARDS degree was based on medical records and blood gas analysis. Analysis of relationship between Brixia score and degree of ARDS was carried out using the Kruskal-Wallis test. RESULT:  There was a significant difference in Brixia score based on degree of ARDS (p value <0.05). The highest Brixia score was obtained in severe ARDS, while the low Brixia score was obtained in mild ARDS. This proves that there is a relationship between Brixia score and degree of ARDS. CONCLUSION: Brixia score has a significant relationship to the degree of ARDS in COVID-19 patients.
Correlation Between the Severity Of Chronic Rhinosinusitis and The Degree of Osteitis Based On Computerized Tomography Evaluation Israchmadi, Ardiga; Ningrum, Farah Hendara; Baskoro, Nurdopo; Dewi, Anna Mailasari Kusuma
Medica Hospitalia : Journal of Clinical Medicine Vol. 11 No. 2 (2024): Med Hosp
Publisher : RSUP Dr. Kariadi

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.36408/mhjcm.v11i2.1070

Abstract

BACKGROUND: The incidence of chronic rhinosinusitis (CRS) is increasing every year, characterized by inflammation of the nasal and paranasal sinuses mucoperiosteum for more than 12 weeks. The inflammatory process of CRS sometimes spreads to the surrounding bone tissue resulting in osteitis. Computerized tomography scan (CT scan) can assess the degree of mucosal inflammation using the Lund-Mackay score (LMS) while the degree of bone thickening and remodelling are assessed with Global osteitis score (GOS) and Kennedy osteitis score (KOS). AIMS: To evaluate the correlation between CRS severity assessment using LMS and osteitis severity assessment using GOS and KOS METHODS: A retrospective analysis using a cross-sectional design was conducted that included 63 CT scans of the paranasal sinus of CRS patients. The spearman rank test was used to analyze data. RESULTS: Assessment using LMS showed 44% patients were classified as severe, while 29% and 27% patients were classified as moderate and mild respectively. Global osteitis score showed 2% patients were categorized as severe, while 22% and 46% patients were categorized as moderate and mild respectively, and 30% patients were not significant. Based on KOS assessment, it was found that 3% patients were classified as severe, while 38% and 59% patients were classified as moderate mild respectively. There was a significant correlation between CRS severity using LMS and GOS (p 0.000) with rho= 0.951. There was a significant correlation between CRS severity using LMS and KOS (p 0.000) with rho value= 0.452. CONCLUSION: This study shows a significant correlation between CRS severity assessment using LMS and bone thickening and remodelling assessment using GOS and KOS. In comparison with KOS, GOS has stronger relationship with LMS.
Co-Authors . Apriansyah Bambang Satoto, Bambang Ch. Nawangsih Ch. Nawangsih Dewi, Anna Mailasari Kusuma Erwin Kresnoadi Handoyono, Thomas Hermina Sukmaningtyas Ibrahim, Irni Dwi Aprianty Iqhbal, Rahmadonal Muhammad Israchmadi, Ardiga Nareswari, Tara Novia Khoerunnisa Nurdopo Baskoro Prihharsanti, CH Nawangsih Rona Ayu Hanifah Santoso, Antonius Gunawan Satrio Adi Wicaksono Syela Nirmada Herdiyanti Tanti Ajoe Kesoema