Obstructed hemivagina with an ipsilateral renal anomaly (OHVIRA) syndrome is a congenital malformation that presents as a uterine didelphys with an obstructed hemivagina and an associated ipsilateral renal aberration. The clinical symptoms usually manifest after menarche. Unlike the typical presentation in adolescence, this case report features a neonatal presentation of OHVIRA syndrome with an unusual renal association. A female twin delivered at 35 weeks of gestation was transferred to our institution after birth from an outside hospital due to respiratory distress and for evaluation of the left multicystic dysplastic kidney identified on prenatal ultrasound. Physical examination and lab results, including a complete blood count, and a basic metabolic panel, including blood urea and serum creatinine, were within the normal range for age. Abdominal and pelvic ultrasound showed multicystic dysplastic left pelvic kidney, congenital hepatic cyst measuring 6 mm, uterine didelphys with duplication of the vaginal canal, and obstructed left hemivagina corresponding to the OHVIRA syndrome. Further testing revealed a normal chromosomal microarray, small patent foramen ovale on the echocardiogram, no vertebral or rib anomalies on the spinal x-ray, normal hearing test, and mild optic cupping on the ophthalmological evaluation. The pediatric surgeon and urologist recommended an outpatient follow-up and elective surgery in the future. This is a unique case presenting in the neonatal period with an unusual association. Timely intervention can help prevent obstetric complications.
Osteomyelitis is a challenging diagnosis for a neonate. It might result from hematogenous spread or direct extension of a skin infection. The most common organism is Staphylococcus aureus. Preterm infants are at high risk for osteomyelitis because of their immature immune system, hypogammaglobulinemia, frequent blood draws, and invasive monitoring and procedures. We report a case of a male neonate born at 29 weeks by cesarean section, requiring intubation and transfer to the neonatal intensive care unit (NICU). At 34 weeks, he was noted to have an abscess in the lateral aspect of the left foot, which required incision, drainage, and antibiotic coverage of cefazolin as S. aureus was sensitive to penicillin. Four days later (34 + 4 weeks), a left inguinal abscess was noticed, which grew Enterococcus faecium on drainage, which is considered a contaminant initially, but one week later, another left-sided inguinal abscess was noticed growing E. faecium, and the patient was subsequently treated with linezolid.Immunoglobulin levels were found to be low for IgG and IgA. After two weeks of antibiotics, a repeat x-ray of the foot demonstrated changes likely due to osteomyelitis. The patient received seven weeks of antibiotics targeting methicillin-sensitive staphylococcus and three weeks of treatment with linezolid for an inguinal abscess. A repeat x-ray of the lower left extremity after the patient received one month of antibiotics in an outpatient setting showed no signs of acute osteomyelitis in the calcaneus. Immunoglobulin levels continued to be low during the outpatient immunology follow-up. During the third trimester of pregnancy, the transfer of maternal IgG across the placenta begins, leading to lower IgG levels in premature infants and predisposing them to severe infections. The usual site of osteomyelitis is the metaphyseal region of the long bones, but any bone can be affected. The depth of penetration of the puncture site during routine puncture of the heel can cause a local infection. Early x-rays can aid in diagnosis. Antimicrobial treatment is frequently administered intravenously for two to three weeks and then switched to oral medication.
Idiopathic pulmonary hemosiderosis (IPH) is a rare condition of unknown etiology characterized by hemoptysis, diffuse alveolar infiltrates and iron-deficiency anemia. Lane-Hamilton syndrome (LHS), a condition in which IPH is associated with celiac disease is an extremely rare condition. CASE PRESENTATION: 49-year-old male with no known past medical history presented with progressively worsening shortness of breath for 3 days. No associated fever, chest pain, cough or hemoptysis were noted. On presentation, temperature: 99F, RR: 34/min, O2 saturation of 85% on room air, HR: 130/min and BP: 100/60mmHg. Physical examination revealed diffuse crackles on chest auscultation. Blood work was significant for leukocytosis at 23.61, Hb of 9, MCV 76 and iron deficiency anemia. Liver function test showed mild transaminitis. Chest xray and CT chest showed diffuse ground glass opacities. He was started on broad spectrum antibiotics. Patient's respiratory status worsened requiring mechanical ventilation. Bronchoscopy and lavage showed diffuse alveolar hemorrhage, elevated white count and hemosiderin laden macrophages. Cultures and cytology were negative. A preliminary diagnosis of pulmonary hemosiderosis was made. Patient was started on pulse dose steroids. Rheumatologic workup was negative. On further review of chart, patient had frequent outpatient visits with bloating and diarrhea. Due to association of celiac disease and alveolar hemorrhage, further work up was sent. Anti-tissue glutaminase and Anti-Gliadin antibody were positive with gliadin IgA titers of 68.5 and IgG titers of 34.2. The probable diagnosis of LHS was made. Patient responded to steroids with clearing of infiltrates and decreasing oxygen requirement. DISCUSSION: LHS is an extremely rare combination of IPH and celiac disease. Both diseases are immunologically mediated and respond well to immunosuppression. Several hypotheses have been postulated to explain this association including deposition of circulating immune complexes from food allergens on the basement membrane of alveolar capillaries, the cross-reaction between the antireticulin antibodies and the alveolar basement membrane antigens. Lane-Hamilton syndrome was first seen in 1971. The association is mostly seen in children below 15 years but is very rare among adults. CONCLUSIONS: LHS should remain one of the differentials in patients presenting with diffuse alveolar hemorrhage. Concurrent screening for celiac disease should therefore be undertaken as disease can be successfully treated with a gluten-free diet once the diagnosis has been made.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.