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Introduction: Between 50-75% of children and adolescents with systemic lupus erythematosus (SLE) experience kidney involvement within the first year of diagnosis. The gold standard for diagnosing renal involvement in SLE is a renal biopsy. It is uncommon for SLE to cause isolated tubular involvement without any glomerular disease. Case Presentation: We report an adolescent girl with a known history of systemic lupus erythematosus who presented to the emergency department with progressively worsening weakness. The diagnosis revealed that she had distal renal tubular acidosis (RTA) without any glomerular disease. Her history of nephrocalcinosis and kidney stones on renal ultrasound is most consistent with distal renal tubular acidosis diagnosis. Conclusion: This case highlights the importance of considering renal tubular acidosis lupus patients who experience recurrent hypokalemic episodes. When a patient presents with a normal anion gap metabolic acidosis and hyperchloremia, without evidence of gastrointestinal HCO3 loss or absorption of exogenous acid, renal tubular acidosis (RTA) should be considered. [ABSTRACT FROM AUTHOR]

Erythema nodosum (EN) is a condition in which the skin suffers from oval-shaped and tender erythematous patches, mostly on the anterior region of the leg and sometimes other parts of the body, including the arms and affects subcutaneous adipose tissue. These patches are self-limiting and usually heal on their own within one to two weeks. This is a case report of a patient who is a 14-year-old boy complained of ovalshaped tender erythematous lesions on the legs of both legs and referred and was hospitalized 5 days ago. The patient continued to complain of pain in both thighs when standing and sitting. The patient developed a high fever upon admission. There was no history of the disease while we were taking his history. All laboratory tests were performed and rheumatic diseases and possible malignancies were evaluated and rejected. The patient was treated with the anti-naproxen, an inflammatory drug, and then the patient's pain and fever decreased. However, the patient suffered from multiple ulcerative lesions in the mouth on the final day of hospitalization. Infectious counseling was performed and he was discharged with a diagnosis of EN in the context of oral herpes. Two days after discharge, the patient suffered from hematochezia. Consequently, he was hospitalized and underwent colonoscopy, which showed multiple mucosal lesions in the esophagus and duodenum, and a sample was sent for pathology. The test result showed Crohn's disease, and the patient was treated immediately. [ABSTRACT FROM AUTHOR]

The B lymphocyte developmental blocks agammaglobulinemia, leading to peripheral B cell depletion and plasma immunoglobulin reduction. Agammaglobulinemia is a rare yet severe disease since it is presented with recurrent sinopulmonary and skin, central nervous system, bone, and joint infections. The onset of the disease is reported to be at the age of six months. Associations have been reported between arthritis and immunodeficiency disorders, such as agammaglobulinemia and common variable immunodeficiency (CVID). This study aimed to present the case of a 3.5-year-old female with a three-month history of the swelling of the left knee, mimicking oligoarticular juvenile idiopathic arthritis. After the initiation of immunosuppressive treatment, the patient developed large, tender, erythematous lesions on the inguinal region bilaterally, which developed to ecthyma gangrenosum due to Pseudomonas. The patient's mother also reported recurrent episodes of infections since the patient was a one-year-old infant. Subsequent to the immunological examinations and laboratory tests, the patient was diagnosed with autosomal recessive agammaglobulinemia due to low serum immunoglobulin concentration and the absence of peripheral B cells. Primary immunodeficiency conditions (particularly agammaglobulinemia) and CVID should be considered in children with arthritis and recurrent infections. Moreover, immunological analysis should be performed prior to treatment in these children. [ABSTRACT FROM AUTHOR]

Churg-Strauss syndrome (CSS) is a type of vasculitis of small-to-medium sized vessels. This syndrome is known by a history of bronchial asthma with systemic necrotizing vasculitis and peripheral blood hypereosinophilia. It is currently called eosinophilic granulomatosis with polyangiitis (EGPA). This disease affects both genders and all age groups, but it is very rare among children. CSS diagnosis is based on clinical findings such as asthma, eosinophilia, rhinosinusitis, and signs of vasculitis in major organs. In cases where steroids alone or in combination with other immunosuppressive agents are used as treatment, the outcome and long-term survival are usually satisfying. In comparison with other types of systemic vasculitis, the mortality rate of this syndrome is low. In this study, we present the case of a 7-yearold boy with poorly controlled bronchial asthma since three years of age. This case had developed purpuric skin lesions, sinusitis, arthritis, and weakness of the limbs with symptoms of mononeuritis multiplex at the age of seven. After being admitted to our hospital, a series of studies, including complete blood count-diff, chest X-ray, paranasal sinus radiography, brain magnetic resonance imaging, nerve conduction study, spirometry, and serological tests for autoantibodies, were performed and he was diagnosed with CSS. Thereafter, he received regular corticosteroid therapy in combination with methotrexate, and his symptoms were generally well-controlled with the beginning of the treatment. The clinical characteristics, diagnosis, and management of CSS in children are also reviewed in this study. [ABSTRACT FROM AUTHOR]

Thumb hypoplasia only in the intrinsic thenar muscles is a rare condition; this defect might be accompanied with cardiac diseases (Holt-Oram syndrome), ocular anomalies, and vascular anomalies of the hand and wrist (Okihiro syndrome). In addition, this condition may be detected in hypereosinophilic syndrome (HES), which gives rise to other hand anomalies, as well. Unilateral or bilateral absence of isolated thenar muscles is another rare abnormality. In this study, we presented a case of Cavanagh's syndrome in an eight-year-old boy with right-sided thenar hypoplasia who had difficulty in thumb opposition. For the treatment, the patient underwent abductor digiti minimi (ADM) opponensplasty. After three years of follow-up, he regained thumb opposition and was symptom-free. Care must be taken to avoid misdiagnosis of thenar atrophy/hypoplasia with carpal tunnel syndrome in case of children. [ABSTRACT FROM AUTHOR]