Pediatric rheumatology is still considered a mysterious branch of pediatric medicine, especially among developing countries. Long-term consequences usually follow delayed diagnosis, referral, and management of rheumatic disorders. We aim to describe the clinical spectrum and the frequency of pediatric rheumatic diseases (PRDs) in AL-Mukalla hospital in Hadhramout province/Yemen. A case record retrospective study was conducted among all patients who attended the pediatric rheumatology clinic in Al-Mukalla hospital (from January 2010 to December 2016) with a musculoskeletal complaint or systemic symptoms suggestive of rheumatic disease. Data collected included: gender, address, age at the onset of symptoms, initial manifestations, the presence of complications, referral diagnosis, final diagnosis and the lag period before attending the rheumatology clinic. PRDs were present in 86% (37/43) of cases, 83.8% (31/37) were from Hadhramout province. Male to female ratio was 0.9:1 (Male: 18; Female: 19) and the commonest age group affected was (>9-12) years. The mean age at first presentation was 8.859 ± 4.11 years (four months-14 years). One year (0.13-98.4 months) was the median time before referral to the rheumatologist. About 70.3% of cases were referred from other specialists or peripheral hospitals. Joint swelling (54.1%) was the most common presentation and juvenile idiopathic arthritis (24.3%) was the commonest diagnosis. The discrepancy between the referral and the final diagnosis was noticed in 21(48.8%) cases. Only eight cases (18.6%) matched the final diagnosis. Two patients died with a mortality rate of 5.4%. Knowledge of the spectrum and incidence of PRDs will increase the awareness of specialists and general practitioners for early referral and diagnosis to avoid long-term sequels.
Background
Nutcracker syndrome is an easily missed cause of hematuria in children. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. Presence of variable non-specific symptoms and non-significant physical findings results in a delayed diagnosis.
Case presentation
We present a ten -year -old girl with four episodes of painless gross hematuria and recurrent microscopic hematuria since the age of two years. Doppler ultrasound showed left renal vein compression while 3 D computerized tomography angiography confirmed the diagnosis of an anterior nutcracker. The patient was conservatively treated with nutritional support (pediasure complete formula and high calorie food), iron supplements and followed up, monitored for anemia, hypertension and renal insufficiency.
Conclusion
Nutcracker syndrome is a rare cause of recurrent gross hematuria in children. A high index of suspicion and proper imaging is needed to reach a proper diagnosis and avoid the psychological and financial stress on the family.
Hematopoietic stem cell transplantation (HSCT) is the definitive therapy for a variety of primary immunodeficiency syndromes (PIDs). However, no more than 30% of the patients will have a human leukocyte antigen (HLA)-identical sibling. We retrospectively analyzed our results of ten patients with PID; severe combined immunodeficiency (SCID) (n = 7), hyper IgM (HIgM) (n = 1) and combined immunodeficiency (CID) (n = 2), who lacked a fully matched donor and underwent mismatched related HSCT during the period from 2008 to 2010. The median age at the time of transplantation ranged between 3 and 84 months (median 6.5 months). Peripheral blood stem cells (PBSC) were used in all HSCTs. The mean value of the peripheral CD34+ cells infused was 9.19 × 10 (6) /kg recipient weight. Patients received different conditioning protocols. All patients received anti graft versus host disease (GVHD) prophylaxis and all were engrafted. Mixed chimerism (5-55%) was noticed. GVHD was observed in 50% of the patients. Post-transplant follow-up ranged from 3 weeks to 36 months (median 15 months). Five patients are still alive while one patient developed engraftment syndrome followed by graft slippage for which a second transplant with CD34+ stem cells 5.8 × 10 (6) /kg recipient's weight was infused. The others died from sepsis and transplant-related complications. Immune reconstitution was noticed in four patients. In conclusion, HLA-haploidentical stem cell transplantation may be feasible, with appropriate GVHD prophylaxis, for patients with PID who lack a fully matched donor.
Background: Dengue is a serious public health threat with a variable clinical course ranging from self-limited illness to fatal complications. Atypical manifestations and uncommon clinical pattern of presentations have been observed during the recent past.
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