Introduction: Cajal like cells (CLCs) in the upper urinary tract have an ability to generate coordinated spontaneous action potentials and are hypothesized to help propel urine from renal pelvis into the ureter. The objective of this review was to describe the variations in the density and distribution of CLCs associated with ureteropelvic junction obstruction (UPJO).Materials and Methods: Studies comparing the density and distribution of CLCs in the human upper urinary tract in patients with UPJO and healthy controls were included in this systematic review. We searched online electronic databases; Ovid MEDLINE, Scopus, PubMed and Cochrane reviews for the studies published before October 31, 2020. A meta-analysis was conducted to compare the density of CLCs at the ureteropelvic junction (UPJ) in patients with UPJO and matched controls.Results: We included 20 and seven studies in the qualitative and quantitative synthesis, respectively. In majority (55%) CLCs were located between the muscle layers of the upper urinary tract. The CLC density in the UPJ gradually increased with aging in both healthy subjects and patients with UPJO. The pooled analysis revealed that the density of CLCs at the UPJ was significantly low in patients with UPJO compared to the controls (SMD = −3.00, 95% CI = −3.89 to −2.11, p < 0.01).Conclusions: The reduction in CLC density at the UPJ in patients with UPJO suggests a contribution from CLCs in the pathogenesis of UPJO. Since age positively correlates with CLC density, it is imperative to carefully match age when conducting case control studies comparing the CLC density and distribution.Protocol Registration Number: CRD42020219882.

Introduction Girls with classical congenital adrenal hyperplasia (CAH) are exposed to excess fetal adrenal androgens in-utero, and often born with masculinised genitalia. They are conventionally reared as females, but show more “boyish” gender-role behaviour (GRB) and gender-identity (GI) issues in childhood and adolescence. Male-rearing is also reported mainly due to delayed treatment and/or socio-cultural factors. We compared GRB/GI in girls with CAH with healthy age matched children, and explored for associations with socio-demographic and diagnosis/treatment related factors. Methods GRB and GI were assessed using the Gender Identity Questionnaire for children (GIQC) in 27 girls with classical CAH at a specialised clinic, and compared with 50 age-matched healthy controls, with exploratory-analysis based on socio-demographic and diagnosis/treatment-related factors. Results Girls with CAH had lower total GIQC scores compared to healthy children (3.29 vs. 4.04, p = < 0.001) with lower GRB score (3.39 vs. 4.23, p < 0.001), and tendency for lower GI score (3.19 vs. 3.5, p = 0.08). Exploratory analysis showed no differences based on diagnosis/treatment factors including age, degree of virilisation at diagnosis and surgical procedures. and only subtle changes based on ethnicity and maternal education. Discussion/conclusion Girls with CAH managed at a specialised centre showed more masculinised GRB and tendency for ambiguous GI, which did not vary upon diagnosis/treatment related factors, suggesting that prenatal androgen exposure was the likely contributor. Clinicians should be vigilant about the increased risk of gender-related problems in girls with CAH, irrespective of sociocultural background and despite early treatment.

Persistent umbilical drainage may be due to vestigial remnants of the omphalomesenteric duct. Rarely, it may be owing to the presence of ectopic pancreatic tissue within these remnants. An 18-month-old boy underwent surgical exploration for umbilical discharge. An umbilical nodule containing both ectopic gastric and pancreatic tissue was found. This is the first instance where both tissue types have been implicated as a cause.

Cronkhite-Canada syndrome is an extremely rare condition of gastrointestinal polyposis in which the main presenting features are diarrhoea and dysgeusia. The polyps in this condition are characteristically distributed throughout the entire gastrointestinal tract except the oesophagus, and these patients exhibit unique ectodermal abnormalities. Herein, we report a 50-year-old male who had recurrent episodes of severe haematochezia from the polyps in the colon. Further examination and investigations revealed a diagnosis of Cronkhite-Canada syndrome. Proctocolectomy was carried out for medically refractory haematochezia and the patient is asymptomatic at present.

IntroductionAcute abdomen and acute appendicitis are unusual clinical presentations that occur in dengue infection–caused illness. Lymphoid hyperplasia and mesenteric adenitis are possible explanations, although vasculitis in the pathology of dengue infection has not been reported. Authors of previous case reports have described mimicking of acute appendicitis discovered upon surgical treatment. Dengue virus has not been proven to cause acute appendicitis.Case presentationWe report a case of an 8-year-old Sinhalese boy who developed acute appendicitis during the acute phase of serologically confirmed dengue fever. Although abdominal pain, vomiting and right-sided tenderness were present at the time of admission, a diagnosis of acute appendicitis was considered only 18 hours later, when abdominal guarding and a well-defined mass in the right iliac fossa were detected clinically and ultrasonographically. Conservative management with intravenous antibiotics was successful.ConclusionIn areas where dengue is endemic, awareness of dengue viral infection as a non-surgical cause of acute abdomen, as well as its ability to mimic acute appendicitis, is important because unnecessary surgery-related morbidity can be decreased. However, delaying or missing the diagnosis of acute appendicitis can result in serious complications. This message is particularly relevant to clinicians, especially pediatricians and surgeons, who encounter large numbers of patients during dengue epidemics and run the risk of missing the diagnosis of acute appendicitis. Likewise, delaying or missing the diagnosis of dengue hemorrhagic fever can lead to dengue shock syndrome and even death. This case highlights the need for careful evaluation of each patient who presents with acute abdomen and dengue infection.