Boletín médico del Hospital Infantil de México最新文献

Introduction: Acute lymphoblastic leukemia (ALL) is the most common oncological disease in the pediatric population; however, skin infiltration occurs only in 1-3% of the patients and almost always manifests after the diagnosis is made.

Clinical case: A male teenage patient who presented with facial edema and infiltration, associated with systemic symptoms such as asthenia and adynamia. On physical examination, the patient presented facial edema and indurated plaques, as well as cervical, inguinal, and axillary adenopathy. Complete blood count showed pancytopenia and a chest X-ray revealed a mediastinal mass. Due to a high suspicion of malignancy a bone marrow and skin biopsy was taken, both with pre-B ALL. Chemotherapy was started and the patient is now in maintenance phase.

Conclusions: Leukemia cutis manifestations are heterogenous, from a small papule to a big nodule. It is more common in patients with acute myeloid leukemia and it is rare in patients with pre-B ALL, specially in the pediatric population. The diagnosis should be done with a biopsy and the treatment is with systemic chemotherapy. The diagnosis should always be considered in patients with unexplained edematous or indurated lesions, especially in the context of systemic symptoms.

Background: Intramuscular vascular malformations (IVMs) are rare developmental congenital structural abnormalities. Their clinical diagnosis is difficult, and imaging studies are essential to determine the type and extent of vessels involved. Treatment can be challenging and must be managed by a multidisciplinary team.

Methods: A descriptive, observational, retrospective, longitudinal study of clinical records of patients diagnosed with IVMs who were evaluated at the vascular anomalies clinic from January 2011 to December 2021 was performed. Demographic, clinical, imaging, diagnosis, treatment, and response data were collected.

Results: Seven patients (five females and two males) with a mean age of 13.66 years (standard deviation 5.82 years) were included in the study. In all cases, the clinical diagnosis was venous and lymphatic malformation. The radiological findings were dilated and tortuous vascular structures or multilobulated lesions with septa inside, with or without vascular flow; these findings allowed diagnosis in all cases. Treatment modalities included sclerotherapy in five patients, surgical resection in two, medical treatment with sirolimus in three, and surveillance in one. Subsequent clinical evolution was favorable in all patients, with decreased pain in six (partial in four and total in two) and size reduction in one patient.

Conclusion: IVMs in our pediatric population most frequently affect the lower extremities. The main symptoms and signs were pain on exertion and volume increase. Treatment can be challenging given the extension and depth of the malformations, so a combination of therapeutic modalities may be necessary to obtain the best outcome.

Introduction: Linear IgA bullous dermatosis (LABD) is a rare autoimmune disease. Although dapsone is the initial treatment, other immunomodulators are used in resistant cases or when dapsone is unavailable.

Case report: A 12-year-old Mexican child, with no relevant medical history, developed in May 2023 a disseminated dermatosis affecting all body segments, including mucous membranes, characterized by erythematous patches and plaques evolving into the formation of serous and serosanguinous blisters and vesicles, distributed in a "string of pearls" pattern. LABD was suspected and confirmed by skin biopsy, which showed a subepidermal blister with neutrophilic infiltration and linear Immunoglobulin A deposits at the dermo-epidermal junction by direct immunofluorescence. Treatment with prednisone (2 mg/kg/day) and cyclosporine (5 mg/kg/day) resulted in improvement and lesion remission within 2 weeks. Both drugs needed to be discontinued for 3 months due to intermittent blistering. Cyclosporine was continued as maintenance therapy at a dose of 4 mg/kg/day for 8 months.

Conclusions: The report highlights the use of cyclosporine as an alternative immunomodulator for DAAL, an immunosuppressive agent used in autoimmune disorders. Few cases, including this one, have described complete remission and control of the dermatosis with cyclosporine, accompanied by prednisone at the start of treatment.

Introduction: The increase in the production of substances to enhance the quality of life, the biodiversity of the different ecosystems in Mexico, and the unique characteristics of pediatric patients, contribute to intoxications within this population.

Method: Analytical retrospective study of admissions to pediatric emergency care due to poisoning in < 16-year-old (2016 to 2020). Included variables were age, gender, type of toxic substance, exposure characteristics, and the season of the year. Frequencies, percentages, range, average and standard deviation were obteined. In the bivariate análisis, the Chi square test was used. A p-value < 0.05 was considered statistically significant.

Results: Over 5 years, there were 459 cases, with a prevalence of 3.16%. No gender predominance was observed. The most common was caused by venomous animals (28.5%), followed by medication ingestion (27.6%). Only 5% of cases were suicide attempts, the rest were accidental (95%). Children up to 5 years old were more commonly by medication ingestion, hydrocarbons, or household products (p = 0.03, p = 0.0001), while the causes in older children were contact with venomous animals and ingestion of stimulants drugs (alcohol, amphetamines, cannabis, cocaine) (p = 0.0001, p = 0.006). Intoxication with quaternary ammonium herbicides was lethal in all cases.

Conclusions: Children under the age of 5 are more susceptible to intoxication from common household products. Older kids tend to be more frequently a combination of stimulants drugs.

Background: The cochlear implant (CI) is effective for rehabilitating patients with severe to profound sensorineural hearing loss. However, its placement and use have been associated with various complications, such as those affecting the vestibular system. The objective of this study was to compare vestibular function using the video head impulse test (vHIT) in pediatric patients before and after CI placement.

Methods: A descriptive and retrospective study was conducted. The outcomes of 11 pediatric patients of both sexes with a history of profound hearing loss were evaluated. The results of vestibular-ocular reflex (VOR) gain, saccades, asymmetry, Pérez Rey (PR) index, and VOR/saccade ratio for both ears obtained by the vHIT test before and after CI placement were compared.

Results: Of the 11 patients evaluated, the VOR gain showed that 81.8% had normal function, 18.2% had hypofunction, and no patients had hyperfunction before implantation. No statistically significant differences were found when compared with post-implant off and post-implant on conditions (p > 0.05). The extracted variables, asymmetry, PR index, and the VOR/saccades ratio also showed no statistically significant differences between the pre- and post-implant conditions, whether off or on.

Conclusions: The vestibular function of pediatric patients did not show significant changes before and after CI placement. The vHIT test is a valuable tool for assessing vestibular function and could be considered a criterion for surgical and rehabilitation decisions in patients undergoing CI placement.

Background: Pediatric cancer is a complex disease that requires interdisciplinary interventions. This study aims to describe the quality of life and exhaustion levels in children diagnosed with cancer, using validated instruments that reflect the peculiarities of this disease.

Method: An observational analytical study was conducted on children and adolescents aged 2 to 18 years with cancer. The PedsQL Cancer Module and FACIT-F instruments were used to assess quality of life and fatigue, respectively, with statistical analysis performed to identify correlations and develop an explanatory model.

Results: Notable physical and psychological symptoms such as nausea, fatigue, hyporexia, irritability, and sadness were identified. The PedsQL and FACIT-F indicated a diminished quality of life. There was high concordance between the perceptions of children and their parents, except in the anxiety related to procedures, where children reported higher levels. These symptoms reflect the impact of cancer treatment on children's well-being. The concordance in evaluations suggests that parents have a good understanding of these experiences, highlighting the relevance of psychosocial interventions to improve quality of life through an adequate support network.

Conclusions: It is crucial that pediatric cancer treatment addresses not only medical aspects but also the comprehensive support for the emotional and psychosocial well-being of the patients and their families.

Introduction: Septic shock is a potentially life-threatening condition. The aim of this study was to identify clinical and epidemiological factors associated with mortality in pediatric patients admitted to a pediatric intensive care unit (PICU) with septic shock.

Materials and methods: A retrospective comparative case series study was conducted with children aged 1 month to 14 years with septic shock from 2018 to 2020 in a PICU in Lima, Peru. Patients were divided into deceased and survivor groups based on their condition at discharge from the PICU. The influence of each variable on mortality was assessed using a logistic regression model.

Results: A total of 174 patients were included in the study, with 51 (29.3%) fatalities. Deceased patients, compared to survivors, were older, had a higher incidence of oncological disease (31.4% vs. 14.6%; p = 0.011), more frequently presented with hemoglobin ≤ 9 g/dL (44% vs. 28%; p = 0.043), lactate > 2 mmol/L (70% vs. 44%; p = 0.002), platelets ≤ 150 (×10³)/μL (77% vs. 42%; p < 0.001), and pH ≤ 7.1 (31% vs. 6%; p < 0.001). In the logistic regression model, factors related to mortality were having a pH ≤ 7.1 (odds ratio [OR] = 8.95; 95% confidence interval [CI]: 2.52-31.75) and platelets ≤ 150 (×10³)/μL (OR = 3.89; 95% CI: 1.40-10.84).

Conclusions: Factors associated with mortality in pediatric patients with septic shock were a pH ≤ 7.1 and platelets ≤ 150 (×10³)/μL in the assessments conducted upon admission to the PICU.

Background: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. The polyarticular course (polyarthritis) represents 63-66% of patients with JIA. The aim was to determine the direct medical costs (DMC) of JIA of the polyarthritis type in pediatric patients of a tertiary hospital in Mexico.

Methods: An analysis of the disease costs was developed from the perspective of the Instituto de Seguridad Social del Estado de México y Municipios Maternal and Child Hospital (HMI). The time horizon was 12 years. All patients diagnosed with JIA with polyarticular course treated by the pediatric rheumatology service of the HMI from January to September 2022 and with an active clinical record were included. Different costing techniques were used. The cost components were consultations, medications, hospitalization, and office and laboratory studies. The costs are reported in USD 2021.

Results: Twenty-six records of patients with polyarticular arthritis from the HMI were analyzed, with a mean of 4,555.2 USD (standard deviation [SD] = 1,456.7) and a median of 3,828 USD (SD = 1,492) in the first 10 years of treatment. The components of DMC were medications (82.7%), office and laboratory studies (8.4%), hospitalization (8.0%), and consultations (1.8%). Biological disease-modifying drugs (bDMARDs) accounted for 95.3% of the drug component cost.

Conclusion: The cost of bDMARDs represented the most critical cost of polyarticular JIA, reflected in the 2^nd year of treatment. Including generic bDMARDs and reviewing purchase prices by health institutions in Mexico is necessary.

Background: We present the case of a patient who underwent hematopoietic progenitor cell transplantation from a fully compatible unrelated donor and subsequently developed Grade IV skin graft-versus-host disease (GVHD) resembling toxic epidermal necrolysis (TEN).

Clinical case: An 11-year-old female, post-transplantation of hematopoietic progenitor cells from a 100% compatible unrelated donor, developed rash-like skin lesions on the trunk and extremities on day +35. A skin biopsy revealed dermal atrophy, vacuolization of the basal layer, and confluent apoptotic keratinocytes with mononuclear inflammatory cells in the dermoepidermis, confirming the diagnosis of TENlike acute cutaneous GVHD.

Conclusion: The patient experienced an 80% remission of symptoms following dynamic management of immunosuppressants.