Cutaneous microvascular occlusion syndrome as the first manifestation of catastrophic lupus-associated antiphospholipid antibody syndrome: a case report.

BACKGROUND: Antiphospholipid syndrome (APS), defined by thrombotic events or obstetric complications in the presence of persistently high antiphospholipid antibodies, is characterized by a wide variety of clinical presentations and the effects of vascular occlusion can impact almost any organ system or tissue. Since adult-onset APS classification criteria are not well verified in pediatrics (where pregnancy-related problems are rare), estimating childhood prevalence is challenging. Stroke and pulmonary embolism are thromboembolic events occurring in children that can cause considerable long-term morbidity. Children with APS are more prone to recurrent thromboembolism than adults. Cutaneous symptoms are prominent and typically represent the first clue of APS. Although dermatologic findings are exceedingly heterogeneous, it is essential to consider which dermatological symptoms justify the investigation of antiphospholipid syndrome and the required further management. CASE PRESENTATION: We describe a seven-year-old Iranian boy with retiform purpura and acral cutaneous ischemic lesions as the first clinical presentation of antiphospholipid syndrome in the setting of systemic lupus erythematous. CONCLUSION: APS in pediatrics, is associated with a variety of neurologic, dermatologic, and hematologic symptoms. Therefore, it is essential for pediatricians to be aware of the rare appearance of Catastrophic APS as an initial indication of APS.

Bone mineral density in Iranian children with celiac disease.

Aim: The current study aims to evaluate bone mineral density (BMD) in patients with celiac disease who were referred to the celiac clinic of Shahid Rahimi Hospital in Khorramabad, Iran, in 2020. Background: Extraintestinal presentations of celiac disease are widespread and, if neglected, can be devastating. Osteoporosis, one of the extraintestinal manifestations of celiac disease, often remains undiagnosed until advanced stages and can impose a significant burden on patients with celiac and health systems. Nonetheless, the prevalence and characteristics of osteoporosis in celiac disease are unknown in Iran. Methods: This was a cross-sectional study at the celiac clinic of Shahid Rahimi Hospital in Khorramabad, Iran. Participants were 48 patients under 18 years diagnosed with Marsh II and Marsh III stages of celiac disease (who need to be on a gluten-free diet) at the pediatrics celiac clinic in 2020. All patients were recruited, completed a questionnaire, and had their blood biochemical parameters analyzed. Then their bone mineral density (BMD) was measured through dual-energy x-ray absorptiometry at the Asia Imaging Center in Khorramabad under the supervision of a radiologist and pediatric rheumatologist. Results: The mean age of the children was 9.96±3.17 years. The minimum and maximum ages of the participants were 4 and 17 years, respectively. Of all 48 children who were included (48), 34 (70.8%) were female, and 14 (29.2%) were male. In the femoral region bone densitometry, 35.4% were normal, 41.7% had lower limit normal, and 22.9% had low bone density. In the lumbar region, 39.6% were normal, 25% were Lower limit normal, and 35.4% had low bone density. No significant correlation was found between age, sex, place of residence, Marsh stage, gluten-free diet, and bone densitometry in both lumbar and femoral regions. Nonetheless, we detected a statistically significant relationship between bone density in the lumbar region and two HLA types, namely HLA DQ8 and HLA DQ2/8 (P=0.016). Conclusion: The results of the current study provided further evidence that all children with advanced celiac disease should be screened for metabolic bone diseases. Besides those in Marsh II and Marsh III, patients in Marsh I stage should also be investigated for low bone mineral density.

The role of nailfold capillaroscopy in pediatric patients with Kawasaki disease.

BACKGROUND: Nailfold capillaroscopy has been used as a non-invasive diagnostic method for microvasculature evaluation in various rheumatological disorders. The present study aimed to determine the utility of nailfold capillaroscopy in the diagnosis of Kawasaki Disease (KD). METHOD: In this case-control study nailfold capillaroscopy was performed in 31 patients with KD and 30 healthy controls. All nailfold images were evaluated for capillary distribution and capillary morphology such as enlargement, tortuosity, and dilatation of the capillaries. RESULT: Abnormal capillaroscopic diameter was identified in 21 patients from the KD group and 4 patients in the control group. The most common abnormality in capillary diameter was irregular dilatation in 11 (35.4%) KD patients and in 4 people (13.3%) in the control group. Distortions of the normal capillary architecture was commonly seen in the KD group (n=8). A positive correlation was observed between coronary involvement and abnormal capillaroscopic results (r=.65, P<.03). The sensitivity and specificity of capillaroscopy for the diagnosis of KD were 84.0% (95%CI: 63.9-95.5%) and 72.2% (95%CI: 54.8-85.8%), respectively. The PPV and NPV of capillaroscopy for KD were 67.7% (95%CI: 48.6-83.3) and 86.7% (95% CI: 69.3-96.2), respectively. CONCLUSION: Capillary alterations are more common in KD patients compared to control group. Thus, nailfold capillaroscopy can be useful in detecting these alterations. Capillaroscopy is a sensitive test for detecting capillary alternations in KD patients. It could be used as a feasible diagnostic modality for evaluating microvascular damage in KD.

The role of nailfold capillaroscopy in pediatric patients with Kawasaki disease / El papel de la capilaroscopia periungueal pacientes pediátricos con la enfermedad de Kawasaki

Background: Nailfold capillaroscopy has been used as a non-invasive diagnostic method for microvasculature evaluation in various rheumatological disorders. The present study aimed to determine the utility of nailfold capillaroscopy in the diagnosis of Kawasaki Disease (KD). Method: In this case–control study nailfold capillaroscopy was performed in 31 patients with KD and 30 healthy controls. All nailfold images were evaluated for capillary distribution and capillary morphology such as enlargement, tortuosity, and dilatation of the capillaries. Result: Abnormal capillaroscopic diameter was identified in 21 patients from the KD group and 4 patients in the control group. The most common abnormality in capillary diameter was irregular dilatation in 11 (35.4%) KD patients and in 4 people (13.3%) in the control group. Distortions of the normal capillary architecture was commonly seen in the KD group (n=8). A positive correlation was observed between coronary involvement and abnormal capillaroscopic results (r=.65, P<.03). The sensitivity and specificity of capillaroscopy for the diagnosis of KD were 84.0% (95%CI: 63.9–95.5%) and 72.2% (95%CI: 54.8–85.8%), respectively. The PPV and NPV of capillaroscopy for KD were 67.7% (95%CI: 48.6–83.3) and 86.7% (95% CI: 69.3–96.2), respectively. Conclusion: Capillary alterations are more common in KD patients compared to control group. Thus, nailfold capillaroscopy can be useful in detecting these alterations. Capillaroscopy is a sensitive test for detecting capillary alternations in KD patients. It could be used as a feasible diagnostic modality for evaluating microvascular damage in KD.(AU)

Antecedentes: La capilaroscopia periungueal se ha utilizado como un método diagnóstico no-invasivo para evaluación de la microvasculatura en varios trastornos reumatológicos. El presente estudio pretendió determinar la utilidad de la capilaroscopia periungueal en el diagnóstico de la enfermedad de Kawasaki (KD). Método: En este estudio de casos y controles se realizó la capilaroscopia periungueal para 31 pacientes con KD y 30 controles sanos. Todas las imágenes de la ungueal se evaluaron para la distribución capilar y la morfología capilar, como la ampliación, la tortuosidad y la dilatación de los capilares. Resultado: Se identificó un diámetro capilaroscópico anormal en 21 pacientes del grupo KD y en 4 pacientes del grupo control. La anomalía más común en el diámetro de los capilares fue la dilatación irregular en 11 (35,4%) pacientes con KD y 4 personas (13,3%) en el grupo control. Las distorsiones de la arquitectura capilar normal se observaron comúnmente en el grupo KD (n=8). Se observó una correlación positiva entre la afectación coronaria y los resultados capilares anormales (r=0,65, p <0,03). La sensibilidad y la especificidad de una capilaroscopia para el diagnóstico de KD fueron del 84,0% (IC del 95%: 63,9-95,5%) y del 72,2% (IC del 95%: 54,8-85,8%), respectivamente. El VPP y el VAN de la capilaroscopia para KD fueron del 67,7% (IC del 95%: 48,6-83,3) y del 86,7% (IC del 95%: 69,3-96,2), respectivamente. Conclusión: Las alteraciones capilares son más frecuentes en los pacientes con KD en comparación con el grupo control. Así, la capilaroscopia periungueal puede ser útil en la detección de estas alteraciones. La capilaroscopia es una prueba sensible para detectar las alteraciones capilares en pacientes con KD. Podría utilizarse como modalidad diagnóstica factible para evaluación el daño microvascular en la KD.(AU)