Adult Hirschsprung's disease presenting as chronic constipation: a case report.

BACKGROUND: Hirschsprung's disease is a congenital disorder identified by the absence of ganglion cells at the Meissner's plexus of the submucosa and Auerbach's plexus of the muscularis. This disease can be found in approximately 1 in 5000 live births. It is a congenital disorder that is rarely diagnosed in adults, where 95% of cases are diagnosed in infants aged under 1 year old. Here we present a rare case of adult Hirschsprung's disease to enrich the body of knowledge  in diagnosing adult patients with chronic refractory constipation symptoms. CASE REPORT: An 18-year-old Indonesian woman came to the general surgery department of Unggul Karsa Medika Teaching Hospital with a defecating problem (constipation) since childhood. There was no history of her passage of meconium. A contrast enema study showed that the sigmoid colon was dilated and the rectum was narrowed, with rectosigmoid index < 1. With these findings, it was suspected that the patient may have ultra-short segment Hirschsprung's disease. The patient was then referred to the digestive surgery department of referral hospital for surgical treatment. CONCLUSION: In adult patients presenting with history of constipation since childhood, it is necessary to consider the possibility of Hirschsprung's disease that was not diagnosed in early childhood. Hirschsprung's disease in adults is usually a short or ultra-short aganglionic segment because it shows relatively mild symptoms. Surgical removal of the aganglionic segment of the gut is the definitive treatment for Hirschsprung's disease.

Right abdominal pregnancy with hemorrhagic shock after previous left tubal pregnancy: A case report.

Abdominal pregnancy is the rarest ectopic pregnancies, with an incidence of 1 per 10,000 live births, and life-threatening because the symptoms are not specific and diagnosis is made after abdominal pain, amenorrhea and vaginal bleeding occur. We present a rare case of abdominal pregnancy in a 31-year-old Indonesian woman with severe abdominal pain within 24 hours before hospital admission, accompanied by nausea, vomiting, dizziness and weakness. She felt the pain increasing since the last 2 weeks and limiting her movement. She has a history of a left tubal pregnancy 5 years ago. Ultrasonography examination revealed an ectopic pregnancy, and she was rushed to the operation room for emergency exploratory laparotomy. An abdominal pregnancy was found, located in the right adnexa with excessive fluid in cavum Douglass and a foetus in around 11-12 weeks of gestation accompanied by free fluid in the subdiaphragmatic, subhepatic and pelvic cavity. The surgery was a successful, four units of whole blood were transfused, and the patient was safely discharged from the hospital. The current concept on management of abdominal pregnancy supports immediate surgical intervention with pregnancy termination, as found in this case, because the patient's condition is hemodynamically unstable indicating hemorrhagic shock correlated with massive hemoperitoneum. A prompt diagnosis and good teamwork in treatment plays an important role for such a life-threatening condition to avoid maternal morbidity and mortality in a case of abdominal pregnancy.

Prognostic Significance of Fms-Like Tyrosine Kinase 3 Internal Tandem Duplication Mutation in Non-Transplant Adult Patients with Acute Myeloblastic Leukemia: A Systematic Review and Meta-Analysis.

BACKGROUND: Fms-like tyrosine kinase-3, internal tandem duplication (FLT3-ITD) mutation, is a known predictor for worse outcome in patients with acute myeloblastic leukemia (AML). However, the prognostic significance of FLT3-ITD mutation in adult, non-transplant patients is still unclear therefore we conducted a systematic review and meta-analysis to explain this issue. The main outcome was overall survival (OS), while additional outcomes included event-free survival (EFS). METHODS: Seven Databases (ScienceDirect, Scopus, PubMed, Cochrane, SpringerLink, ProQuest, and EBSCOhost) were searched up to August 2020.  Studies investigating the prognostic value of AML in adults with FLT3-ITD mutational status were selected. Studies which patients had received transplantation, diagnosed with acute promyelocytic leukemia (APL) or secondary AML were excluded. The selected studies were divided into subgroups based on their cytogenetic profile. Summary hazard ratios (HR) and 95% confidence intervals (CI) were calculated using fixed-effects models. Heterogeneity tests were conducted and presented in I2 value. Forest plot was presented to facilitate understanding of the results. Publication bias was analyzed by Funnel Plot test. RESULTS: A total of ten studies describing research conducted from 1999 to 2020, met the inclusion criteria for this study. Nine studies reported OS and four studies reported EFS in HR. The highest HR for OS is 6.33 (95% CI, 2.61-15.33; p < 0.001), for EFS is 3.58 (95% CI, 1.59 - 8.05); p = 0.002)., while the lowest for OS is 1.33 (95% CI, 0.88-2.01; P = 0.174) and for EFS is 1.29 (95% CI, 0.75-2.23; p = 0.34). Nine studies were included in meta-analysis with HR for OS 1.91 (95% CI, 1.59-2.30, p < 0.00001), whereas 4 studies were included in meta-analysis for EFS with HR 1.64 (95% CI, 1.25-2.14; p = 0.0003). CONCLUSION: FLT3-ITD mutation is associated with worse prognosis in adult, non-transplant patients with AML, both for OS and EFS.