SuO037LONG-TERM OUTCOME IN NEPHROGENIC DIABETES INCIPIDUS
Bibliographic record
Abstract
INTRODUCTION AND AIMS: Inherited Nephrogenic diabetes insipidus (NDI) is a rare disorder characterized by impaired urinary concentrating ability with consequent polyuria and risk of dehydration. There is paucity of clinical data on long-term outcome to help inform prognosis and management. METHODS: We performed a single center retrospective medical record review of patients with diagnosis of NDI patients followed between 1985 and 2017. We collected available data on growth, weight, school performance, complications and comorbidities. RESULTS: We identified 36 patients with available data and a clinical diagnosis of NDI, which was genetically confirmed in 33. Patients presented at median age of 0.6 years (range 0.01-9) and median length of follow-up was 9.5 years (0.8-16.9). The chief symptoms at presentation were failure to thrive, vomiting /feeding concerns, polyuria /polydipsia, febrile illness and hypernatraemic dehydration. Four were investigated before symptoms based on family history. Median weight standard deviation scores (SDS) improved from-2.1 at presentation to 0.2 at last follow-up. In contrast height SDS remained essentially unchanged at -1.1 at presentation and -0.9 at last follow-up. Most patients were treated with NSAID and thiazides, yet weaned off during school age without apparent change in urine output. No apparent long-term toxicity was noted. Median estimated glomerular filtration rate (eGFR) at last follow up was 81 (56-129) ml/min/1.73m2. Urological complications were noted in fifteen patients. Constipation was recorded in eleven and learning difficulty in five patients. Median age of resolution of nocturnal enuresis was 11.3 (range: 4-16, N=19) years. Estimated median daily fluid intake at median age of 13y (N=28) was 3800 ml /m2 (1600-9200). CONCLUSIONS: The overall prognosis in inherited NDI is favorable with regular treatment. Thiazide and NSIAD treatment appears to lose efficacy with age. As expected, most complications were related to polyuria. Our data inform the prognosis and management of patients with NDI.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".