Kienböck Disease: Recent Advances in the Basic Science, Assessment and Treatment

Hand Surgery ◽  
2015 ◽  
Vol 20 (03) ◽  
pp. 352-365 ◽  
Author(s):  
Gregory Ian Bain ◽  
Chong Jin Yeo ◽  
Levi Philip Morse
Keyword(s):  
Basic Science ◽  
Clinical Symptoms ◽  
Staging System ◽  
Grading System ◽  
Science Assessment ◽  
Recent Advances ◽  
Assessment And Treatment ◽  
Kienböck Disease ◽  
Wrist Function ◽  
High Index Of Suspicion

Kienböck disease is a disorder of impaired lunate vascularity which ultimately has the potential to lead to marked degeneration of the wrist and impaired wrist function. The aetiology of the avascular necrosis is uncertain, but theories relate to ulnar variance, variability in lunate vascularity and intraosseous pressures. Clinical symptoms can be subtle and variable, requiring a high index of suspicion for the diagnosis. The Lichtmann classification has historically been used to guide management. We present a review of Kienböck disease, with a focus on the recent advances in assessment and treatment. Based on our understanding thus far of the pathoanatomy of Kienböck’s disease, we are proposing a pathological staging system founded on the vascularity, osseous and chondral health of the lunate. We also propose an articular-based approach to treatment, with an arthroscopic grading system to guide management.

CASE REPORT: RARE FORM OF HIRSCHPRUNG’S DISEASE

2020 ◽  
Vol 30 (5) ◽  
pp. 82-84
Author(s):  
Ilja Skalskis
Keyword(s):  
Down Syndrome ◽  
Case Report ◽  
Clinical Symptoms ◽  
Hirschsprung Disease ◽  
Distal Colon ◽  
Total Colonic Aganglionosis ◽  
Sphincter Function ◽  
Anal Sphincter Function ◽  
History Of ◽  
High Index Of Suspicion

Hirschsprung disease (HD) is a developmental disorder characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. Incidence of total colonic aganglionosis (TCA) is 1 in 500 000 and it accounts for 5-10% of all cases of HD. HD should be suspected in patients with typical clinical symptoms and a high index of suspicion is appropriate for infants with a predisposing condition such as Down Syndrome (DS), or for those with a family history of HD. The treatment of choice for HD is surgical, such as Swenson, Soave, and Duhamel procedures. The goals are to resect the affected segment of the colon, bring the normal ganglionic bowel down close to the anus, and preserve internal anal sphincter function. We present a clinical case report of TCA in a child with Down syndrome (DS) and review of literature.

scholarly journals Clinical spectrum and radiographic features of the syndrome of the trephined

2015 ◽  
Vol 6 (03) ◽  
pp. 438-441 ◽  
Author(s):  
Cara L. Sedney ◽  
William Dillen ◽  
Terrence Julien
Keyword(s):  
Clinical Symptoms ◽  
Case Reports ◽  
Statistical Significance ◽  
Skin Flap ◽  
Surgical Care ◽  
Medical Providers ◽  
Demographic Information ◽  
Neurosurgical Procedure ◽  
High Index Of Suspicion ◽  
Syndrome Of The Trephined

ABSTRACT Object: Craniectomy is a common neurosurgical procedure. Syndrome of the trephined (ST) occurring after craniectomy results in neurologic symptoms that are reversible with cranioplasty. While well-documented, previous literature consisted of case reports, symptom spectrum and risk factors have not been well characterized. Materials and Methods: A retrospective review of 29 consecutive cases who underwent decompressive craniectomy within a 30-month period was performed. Patients were considered affected by ST if a previously stable neurological deficit improved within 3 weeks after cranioplasty. Prevalence of ST was measured and association with demographic information, clinical symptoms patterns, indication for and size of craniectomy, as well as radiological signs were tested. Results: Seven patients (24%) developed ST. Chronic rehabilitation arrest was more common than acute neurologic decline. Factors such as craniectomy size and patient age did not reach statistical significance in development of ST. Radiographic factors were predictive, with a sunken skin flap contour being most sensitive, while ventricular effacement was most specific.Conclusion: ST may have a higher incidence than previously thought, with a chronic rehabilitation arrest being a more common presentation than an acute decline. Medical providers involved in the post surgical care and rehabilitation of these patients should maintain a high index of suspicion for ST.

scholarly journals Intraductal Carcinoma of Prostate (IDC-P), Grade Group, and Molecular Pathology: Recent Advances and Practical Implication

2019 ◽  
pp. 1-10
Author(s):  
Ashwyna Sunassee ◽  
Ghadah Al Sannaa ◽  
Jae Y. Ro
Keyword(s):  
Prostate Cancer ◽  
Molecular Pathology ◽  
Invasive Carcinoma ◽  
World Health ◽  
Intraductal Carcinoma ◽  
Grading System ◽  
Grade Group ◽  
Group System ◽  
Gleason Grading ◽  
Recent Advances

The Gleason grading system for prostatic carcinoma is widely used internationally and is based on microscopic architectural patterns of tumors. Over the years, there have been modifications to the original grading system established by Donald F Gleason in 1966 and refined in 1974 which have subsequently been established by the World Health Organization in its WHO Classification of Tumors of the Urinary System and Male Genital Organs book, published in 2016. There have been certain practical issues associated with the changes, of note, the addition of intraductal carcinoma of prostate (IDC-P), which unlike its breast counterpart rarely occurs in isolation without association with invasive carcinoma and tends to be associated with high-grade invasive carcinoma. In addition, the Grade group system has been introduced which categorizes tumors into prognostically relevant groups based on the histological grade scores. The grade group system brings to light the importance of making accurate scoring and subsequent grouping of the tumors as it affects the clinical treatment, prognostic implication and stage assignment. Molecular pathology of the prostate is not widely utilized in clinical practice, but is emerging. The most common genomic aberration in prostate cancer includes gene fusion, amplification, deletion, and mutation. In addition, up and down regulation of gene expression in critical cellular pathways is also at play. A series of long noncoding RNA expression changes have been also unveiled from transcriptome sequencing data. They play a regulatory role in prostate cancer and are promising diagnostic and potentially prognostic markers as well as molecular treatment strategy. In this review, we summarize recent advances in molecular pathology of prostate cancer and their emerging clinical utility with currently available molecular tests. In this review article, we discuss the followings: 1) Gleason grading system with its modification, 2) Grade group, 3) Intraductal carcinoma, and 4) molecular pathology. Additionally, we present that molecular studies continue to emerge, and there is significant opportunity for targeted therapeutic options that remains to be explored in depth.

scholarly journals Characteristics and treatment response of chronic lymphocytic leukemia patients in Dharmais National Cancer Centre Hospital, Indonesia.

2019 ◽  
Vol 5 (suppl) ◽  
pp. 121-121
Author(s):  
Noorwati Sutandyo ◽  
Nia Novianti Siregar ◽  
Hilman Tadjoedin ◽  
Syafrizal Syafei ◽  
Julyanti Agustina
Keyword(s):  
High Risk ◽  
Chronic Lymphocytic Leukemia ◽  
Treatment Response ◽  
Group Treatment ◽  
Clinical Symptoms ◽  
Staging System ◽  
Lymphocytic Leukemia ◽  
Cancer Centre ◽  
Centre Hospital ◽  
Worse Prognosis

121 Background: Chronic lymphocytic leukemia (CLL) was a common hematological malignancy in America and Europe, but rarely found in Asia. Differences in clinical characteristics and treatment response were suspected. This study aimed to find out characteristics and treatment response of patient with CLL in Dharmais National Cancer Centre Hospital Indonesia. Methods: This was a retrospective cohort study of CLL patients in Dharmais Hospital during 2013-2015. Data were retrieved from medical record included patients’ demography, blood examination, bone marrow puncture, and immunophenotyping. Prognosis factors such as Rai and Binet staging system were studied. Results: A total of 36 CLL patients were retrieved. Most of patients (58.3%) were male. Median age was 58.5 years old. As much as 25.9% of patients were asymptomatic. There were 17 patients (47.2%) with negative CD5, and most of them (63.2%) had isolated splenomegaly (p = 0.019). Twenty patients (55.6%) were high-risk based on Rai staging group, but there were only 14 patients (38.9%) were high-risk based on Binet staging group. Positive CD13 was found in 30.6% patients and classified as high risk Rai (p < 0.001) and Binet (p = 0.043) staging group. Treatment response was available in 12 cases, consisted of 8 (66.7%) partial responses, 3 (25.0%) progressive diseases, and 1 (8.3%) stable disease. Conclusions: Chronic lymphocytic leukemia is a rare disease in Indonesia. Patients tend to be younger and have worse prognosis. Many cases are categorized as high-risk and present without specific clinical symptoms. Early detection is challenging in the future for better management of CLL cases.

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