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Background: Since COVID-19 the number of girls referred to pediatric endocrinologist for suspected precocious puberty (PP) and early puberty (EP) has increased. The aim of the study was to evaluate the incidence, the anthropometric, biochemical and radiological characteristics of PP during the COVID-19 pandemic, compared to previous years. Method(s): We retrospectively evaluated medical records of 464 females (F) referred to academic pediatric endocrinology center for suspected PP/EP from January 2016 to June 2021 (Group1: January 2016-March 2020, n=295;Group2: March 2020-June 2021, n=169). At first visit (V1) 289F had Idiopathic CPP (Group1 n=175;Group2 n=114) of which n=89 met the Rapidly Progressive Idiopathic CPP criteria (RP-ICPP;Group1 n=45;Group2 n=44) and n=200 for Slowly Progressive Idiopathic CPP (SP-ICPP;Group1 n=130;Group2 n=70). Additional n=44 of the SP-ICPP met the RP-ICPP criteria (Group1 n=27;Group2 n=17) at a second assessment (V2). Girls were classified by age at diagnosis (<6;6-6.99;7-7.99;>8 years). The total number of RP-ICPP was 133 (n=27 no-caucasians). Result(s): We found a higher incidence of RP-ICPP in Group 2 compared to Group1 (36% vs 24%, p<0.05, respectively);comparing the annual RP-ICPP incidences, 2021 showed the highest (p<0.05). RP-ICPP in Group1 and Group2 differed in age at diagnosis (7.96+/-0.71 vs 7.61+/-0.94;p<0.05) and in the time interval between thelarche and diagnosis (0.93+/-0.75 vs 0.74+/-0.64 yrs, p=0.05). We found a progressive increase in the number of girls Group2 in each age group<8 years (<6 years n=4 vs n=2;6-6,99 years n=7 vs n=4;7-7,99 years n=34 vs n=20), and a significant inverted proportion in girls >8 years (n=41 Group1 vs n=21 Group2, p<0.05). There were no differences between Group1 and Group2 for any anthropometric, biochemical and radiological parameters analyzed, except for blood glucose and cholesterol HDL levels (both highest in Group2 (p<0.05)). These data were confirmed in the total group and only in caucasian girls. By regression analyses we found that age at diagnosis was anticipated in Group2, regardless of BMI SDS. Analyzing the subgroup of patients with V2, BMI SDS V2-V1 was not different (-0.12+/-0.5 in Group1 vs 0.11+/-0.44 in Group2, p=0.17). Conclusion(s): During the Covid-19 pandemic the number of girls with suspected PP/EP resulted 1.79 fold increased, with a 1.5 time higher incidence of RC-ICPP. BMI doesn't seem to play a role, but further studies are needed to understand the etiology and primarily the role of metabolic parameters.
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Introduction: The hypothalamus plays a crucial role in regulating vital functions and circadian rhythms. Both the tumor involving the hypothalamic area and its treatment can lead to hypothalamic dysfunction, resulting in disturbances in sleep-wake patterns, sleep fragmentation, and increased daytime sleepiness. We describe two patients with craniopharyngioma who came to our attention due to the occurrence of episodes characterized by psychomotor slowing and afinalistic limb movements, temporal and spatial disorientation, psychomotor agitation, and oneiric stupor like episodes diagnosed as severe sleep disturbances. Case reports: Patient 1 is a 19-year-old male diagnosed with surgically treated craniopharyngioma. Subsequently, episodes of psychomotor slowing, afinalistic movements of the upper limbs diagnosed as seizures in another neurological center appeared;antiepileptic treatment was started without improvement. At the first examination in our center, excessive daytime sleepiness (EDS), fragmented nighttime sleep, episodes characterized by bimanual automatic gestures occurring during drowsy state, hypnagogic hallucinations, and sudden loss of muscle tone while awake were recognized. Actigraphy demonstrated irregular bedtimes, frequent nocturnal activity, and inappropriate daytime rest episodes. The Epworth Sleepiness Scale (ESS) showed subjective EDS (ESS=19). At PSG, hypersomnolence, severe sleep-related breathing disorder (SRBD), and no interictal and ictal seizure abnormalities were found. A BiPAP NIV was started, and antiepileptic therapy was discontinued. In the following months, PSG revealed marked improvement in SRBD and 1 SOREMP, and the MSLT a mean SOL of 6 min and 10 sec and 3 SOREMPs. These data allowed the diagnosis of secondary narcolepsy, and treatment with pitolisant was initiated with clinical improvement and reduced daytime sleepiness (ESS=9). Patient 2 is a 12-year-old male, surgically treated for craniopharyngioma at the age of 4 years, who developed episodes of myoclonic jerks, temporal and spatial disorientation, and psychomotor agitation during the lockdown period for COVID-19 emergency. Surmising paroxysmal epileptic episodes, the patient was hospitalized. The anamnestic data collection revealed a sleep-wake rhythm dysregulation, fragmented nighttime sleep, EDS, oneiric stupor-like episodes during which the patient performed simple automatic gestures mimicking daily-life activity, and severe impairment of alertness. The Long-term video-EEG, including polygraphic measurements, showed destruction of the wake-NREM sleep-REM sleep boundaries, episodes of undetermined state of vigilance, and concurrence of elements typical of different sleep stages. Moreover, a severe SRBD (AHI 19/h) has been observed. The MRI showed a volumetric increase in the post-surgical interpeduncular fossa and right paramedian cysts. Therefore, a multifactorial therapeutic plan including sleep hygiene and slow-release melatonin was started with improvement in nighttime sleep, but EDS persisted. Surgical treatment of cyst fenestration improved sleep-wake rhythm and behavior;BiPAP NIV was initiated with very poor adherence. Discussion: We aim to focus on sleep disorders as a possible complication of tumors involving the hypothalamic region. Our cases highlight that the clinical manifestation of these dysfunctions can be challenging to diagnose and can lead to misdiagnosis and inappropriate treatment that can harm patients' health and the quality of life of patients and their families. Conclusion: These findings support the need to incorporate comprehensive sleep assessment in survivors from childhood brain tumors involving the suprasellar/hypothalamic region.