Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Some aspects of long-term results of treatment of acute hematogenous osteomyelitis

Objective. The aim of the study was to evaluate the treatment results of acute hematogenous osteomyelitis over 10 years and to determine the relationship between them and clinical course of the disease. Materials and methods. The patients with acute hematogenous osteomyelitis diagnosed 10 years ago (1995–1996) were enrolled in the study. The post mail or phone contacts with them or their parents were obtained, the questions were submitted, and the patients were invited for medical examination. The current complaints and limitations in physical activity were assessed. The amplitude of joint movements, tenderness during palpation, and deformations were evaluated during physical examination. Patients were divided into two groups: with and without complaints. The duration and severity of the disease, age, gender, presence of sepsis, arthritis, and subperiosteal abscess, the type of bone affected and bone lesion on x-ray, duration of fever, and maximal erythrocyte sedimentation rate during the acute period were compared between groups. Results. We made 50 phone calls and sent seven letters to the patients with acute hematogenous osteomyelitis who were treated in our department. In 14 (28%) cases, phone contact was obtained, and 7 of them were examined. Five answers (71%) to the letters were received. The overall response rate was 33%. Thirteen (68%) former patients had no any complaints; six (32%) had minor complaints, which were probably linked to acute hematogenous osteomyelitis. Fifteen (79%) former patients had no limitations in any physical activities, and four (21%) of them limit their physical activities. Seven patients were examined, but neither visible deformities and tenderness nor limitations in movement amplitude were found. Comparing the groups with complaints and without complaints by age, gender, presence of sepsis and arthritis, duration of the disease, and the presence of periosteal abscess, no statistically significant difference in any of variables was found. Conclusions. According to our data, 37% of the patients have complaints 10 years after acute hematogenous osteomyelitis, and these complaints are not associated with the clinical course of the disease

Kai kurie ūminio hematogeninio osteomielito vėlyvųjų gydymo rezultatų aspektai

Objective. The aim of the study was to evaluate the treatment results of acute hematogenous osteomyelitis over 10 years and to determine the relationship between them and clinical course of the disease. Materials and methods. The patients with acute hematogenous osteomyelitis diagnosed 10 years ago (1995–1996) were enrolled in the study. The post mail or phone contacts with them or their parents were obtained, the questions were submitted, and the patients were invited for medical examination. The current complaints and limitations in physical activity were assessed. The amplitude of joint movements, tenderness during palpation, and deformations were evaluated during physical examination. Patients were divided into two groups: with and without complaints. The duration and severity of the disease, age, gender, presence of sepsis, arthritis, and subperiosteal abscess, the type of bone affected and bone lesion on x-ray, duration of fever, and maximal erythrocyte sedimentation rate during the acute period were compared between groups. Results. We made 50 phone calls and sent seven letters to the patients with acute hematogenous osteomyelitis who were treated in our department. In 14 (28%) cases, phone contact was obtained, and 7 of them were examined. Five answers (71%) to the letters were received. The overall response rate was 33%. Thirteen (68%) former patients had no any complaints; six (32%) had minor complaints, which were probably linked to acute hematogenous osteomyelitis. Fifteen (79%) former patients had no limitations in any physical activities, and four (21%) of them limit their physical activities. Seven patients were examined, but neither visible deformities and tenderness nor limitations in movement amplitude were found [...]

Congenital hyperinsulinism

Hyperinsulinism is the most common cause of hypoglycemia in infants. In many cases conservative treatment is not effective and surgical intervention is required. Differentiation between diffuse and focal forms and localization of focal lesions are the most important issues in preoperative management. We present a case of persistent infancy hyperinsulinism. Clinical presentation, conservative treatment modalities, diagnostic possibilities of focal and diffuse forms, and surgical treatment, which led to total recovery, are discussed

Gonadotropin-Releasing Hormone Agonist Corrects Defective Mini-Puberty in Boys with Cryptorchidism: A Prospective Randomized Study

Introduction. This prospective study investigated the efficacy of a gonadotropin-releasing hormone agonist (LH-RHa) in restoring defective mini-puberty. Materials and Methods. Boys with isolated bilateral cryptorchidism and defective mini-puberty were randomly divided into two groups. The “surgery only” group underwent a second orchidopexy without hormonal treatment (control). The “LH-RHa” group received LH-RHa therapy followed by a second orchidopexy. The number of Ad spermatogonia and the total germ cell count per tubule (S/T) were analyzed. Results. Five boys were included in each arm. In the LH-RHa group, the median S/T increased from 0.11 to 0.42, p=0.04. In the surgery only group, the median S/T did not change. In the surgery only group, none of the testes had Ad spermatogonia. In contrast, in the LH-RHa group, all testes completed the transition from gonocytes to Ad spermatogonia (p=0.008). Conclusions. Treatment with LH-RHa was effective in rescuing defective mini-puberty in boys with bilateral cryptorchidism

Hamstring vs. All-Soft-Tissue Quadriceps Tendon Autograft for Anterior Cruciate Ligament Reconstruction in Adolescent Athletes: Early Follow-Up Results of a Prospective Study

Background: The quadriceps tendon (QT) autograft has recently drawn attention for anterior cruciate ligament reconstruction (ACLR). Finding the best autograft option for adolescents after an ACL injury is essential to ensure them a high-quality active lifestyle. There are no studies comparing the all-soft-tissue QT autograft with the hamstring tendon (HT) autograft in such population. Methods: In this study, 68 patients younger than 18 years of age were assigned to the HT (38 patients) or the QT (30 patients) ACLR group. The groups were similar, allowing their comparison. The instrumented knee laxity was evaluated with a Genourob arthrometer. In total, 48 patients (27 HT and 21 QT) were tested 3 months post-op, and 45 patients (26 HT and 19 QT) were tested 6 months post-op. Results: We found that 3 months post-op, the side-to-side anterior tibial translation measurements (GNRB1) were worse in the HT than in the QT group (1.4 (0.2–5.2; 1.715) vs. 0.6 (0.1–2.1; 0.905) mm, p = 0.02). There was no difference in side-to-side anterior tibial translation at 6 months post-op (GNRB2) between the HT and the QT group (1 (0.2–5.3; 1.519) vs. 1.1 (0.3–3.4; 1.279) mm, p = 0.927). At 3 months post-op, the side-to-side anterior tibial translation (GNRB1) was worse in males than in females. irrespective of the graft choice (1.45 (0.1–5.2; 1.696) vs. 0.4 (0.1–3.4; 0.89) mm, p = 0.016). The displacement curve slopes 6 months post-op were better for females (3 (0–13.1; 3.335) vs. 5.3 (0–26.1; 7.848), p = 0.014). At 3 months post-op, the GNRB measurements showed that the side-to-side anterior tibial translation (GNRB1) was better for females when the HT autograft had been used (0.45 (0.2–3.4; 0.942) vs. 2.4 (0.3–5.2; 2.333) mm, p = 0.003). In general, both autografts provided excellent objective outcomes at early follow-up. Conclusions: The all-soft-tissue QT autograft should be considered as a reliable alternative for ACLR in adolescents. This autograft has at least the same properties as the HT autograft in the early stages after ACL reconstruction in adolescent athletes and, in some respects, seems superior to the HT autograft. Research should continue to find the best possible graft choice for the most active and willing-to-return-to-sport population

Did the COVID-19 Pandemic Prolong the Time Till Diagnosis and Worsen Outcomes for Children with Acute Appendicitis?

Background and Objectives: Our aim was to see if the COVID-19 pandemic led to an increase of time until diagnosis, operation, and time spent in Emergency room (ER), and if it resulted in more cases of complicated appendicitis and complication rates in children. Materials and Methods: We conducted a retrospective analysis of patients admitted to the Pediatric Surgery Department with acute appendicitis during a 4-month period of the first COVID-19 pandemic and compared it to the previous year data—the same 4-month period in 2019. Results: During the pandemic, the time spent in the ER until arriving at the department increased significantly 2.85 vs. 0.98 h p p = 0.03. However, the time from the beginning of symptoms till ER, operation time and the length of stay at the hospital, as well as the overall time until operation did not differ and did not result in an increase of complicated appendicitis cases or postoperative complications. Conclusions: The COVID-19-implemented quarantine led to an increase of the time from the emergency room to the operating room by 4 h. This delay did not result in a higher rate of complicated appendicitis and complication rates, allowing for surgery to be postponed to daytime hours if needed

Does Multisystem Inflammatory Syndrome Only Mimic Acute Appendicitis in Children or Can It Coexist: When Should We Suspect MIS-C?

Background and Objectives: Acute abdominal pain in children has been noticed to be a primary reason to seek medical attention in multisystem inflammatory disorder (MIS-C), which can prevail separately or together with acute appendicitis. Our aim was to distinguish regular appendicitis cases from MIS-C and to suggest the best clinical and laboratory criteria for it. Materials and methods: Cases of patients, admitted to the Pediatric Surgery Department over a six-month period in 2021, were retrospectively analyzed. Confirmed MIS-C or acute appendicitis cases were selected. MIS-C cases were either separate/with no found inflammation in the appendix or together with acute appendicitis. Acute appendicitis cases were either regular cases or with a positive COVID-19 test. Four groups were formed and compared: A-acute appendicitis, B-MIS-C with acute appendicitis, C-MIS-C only and D-acute appendicitis with COVID-19. Results: A total of 76 cases were overall analyzed: A-36, B-6, C-29 and D-5. The most significant differences were found in duration of disease A—1.4 days, B—4.5 days, C—4 days, D—4 days (p p p p Conclusions: MIS-C could be suspected even when clinical data and performed tests suggest acute appendicitis especially when at least three out of four signs are present: CRP > 55.8 mg, symptoms last 3 days or longer, febrile fever is present, and any kind of other system involvement is noticed, especially with a known prior recent COVID-19 contact, infection or a positive COVID-19 antibody IgG test

Gene expression changes underlying idiopathic central hypogonadism in cryptorchidism with defective mini-puberty

The whole genome RNA profiling of testicular biopsies by DNA strand-specific RNA sequencing was examined to determine a potential causative role of isolated congenital cryptorchidism in azoospermia and/or infertility in the context of our previously published GeneChip data. Cryptorchid patients, aged 7 months to 5 years and otherwise healthy, were enrolled in this prospective study. During surgery, testicular tissue biopsies were obtained for histological examination and RNA sequencing. Fifteen patients were selected based on the histological results and were divided into 2 groups. Seven were classified as belonging to the high infertility risk (HIR) and 8 to the low infertility risk (LIR) group. Cryptorchid boys in the HIR group lacked transformation of gonocytes into Ad spermatogonia due to impaired mini-puberty. This group of patients will be infertile despite successful surgery. The new important finding was a decreased PROK2, CHD7, FGFR1, and SPRY4 gene expression in the HIR group. Furthermore, identification of multiple differences in gene expression between HIR and LIR groups underscores the importance of an intact hypothalamic-pituitary-gonadal axis for fertility development. Our RNA profiling data strongly support the theory that in the HIR group of cryptorchid boys insufficient PROK2/CHD7/FGFR1/SPRY4 gene expression induces deficient LH secretion, resulting in impaired mini-puberty and infertility. We therefore recommend hormonal treatment for this cohort of cryptorchid boys with defective mini-puberty following a seemingly successful orchidopexy