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Case Reports
. 2023;30(2):124-132.
doi: 10.15388/Amed.2023.30.2.4. Epub 2023 Jul 31.

Overt Hypothyroidism and Severe Growth Retardation in a Preschool Girl with Poorly Controlled Nephrotic Syndrome: Case Report and Literature Review

Joana Mikulevič  1 , Karolina Tumelienė  1 , Robertas Kemežys  2 , Augustina Jankauskienė  2
Affiliations
Case Reports

Overt Hypothyroidism and Severe Growth Retardation in a Preschool Girl with Poorly Controlled Nephrotic Syndrome: Case Report and Literature Review

Joana Mikulevič et al. Acta Med Litu. 2023.

Abstract
in English, Italian

Background: Nephrotic children may develop thyroid hormone dysfunction due to urinary excretion of thyroid hormones. In contrast to the subclinical hypothyroidism that affects around 30% of children with nephrotic syndrome (NS), the patient in this case had overt hypothyroidism and severe growth retardation.

Clinical case: A 5 years and 8 months old girl with steroid-dependent NS was referred from another center due to persistent edema and decreased diuresis, being treated with mycophenolate mofetil (MMF) 250 mg once a day and L-thyroxine 50 mcg daily since 4 months of NS onset because of hypothyroidism. Her albumin was 12.64 g/l, cholesterol 25.64 mmol/l and proteinuria 5 g/l. Severe growth retardation was observed: patient's height was 93.5 cm (-13 cm <3 percentile), weighted 17.2 kg (15-25 percentile). Her disease vintage was over 3 years. Girl's growth velocity has slowed down from 3.5 months. The patient received a high cumulative dose of prednisolone (approx. 7800 mg in 1 year and 8 months). Thyroid-stimulating hormone was higher (18.04 mU/L) with reduced FT4 11.43 pmol/l and IGF-1 < 15 µg/L. Kidney biopsy revealed minimal change disease, and genetic testing was negative. Intensive NS treatment with methylprednisolone pulse therapy, enlarged doses of MMF and albumin infusion were started and L-thyroxine dose was increased to 75 mcg. TPOAb was in normal range (12.65 IU/ml). After 3 weeks she was discharged with no edema and after stopping methylprednisolone treatment thyroid function normalized and L-thyroxin was discontinued. Two weeks later standard growth hormone stimulation test with clonidine showed partially insufficient growth hormone secretion. During NS remission with normalization of thyroid function (TSH 6.680 mU/l, FT4 13.85 pmol/l) and normalization of IGF-1 level (132 mcg/l) partial catch-up growth was observed (height velocity increased from 3.5 cm/year to 7.3 cm/year, based on 4-month calculation period).

Conclusions: Clinicians should be aware of a risk of developing hypothyroidism and consider thyroid function testing during the treatment of children with NS, as well as actively treat hypothyroidism and evaluate growth.

Apžvalga: Vaikams, sergantiems nefroziniu sindromu (NS), gali sutrikti skydliaukės funkcija dėl skydliaukės hormonᶙ netekimo su šlapimu. Skirtingai nuo subklinikinės hipotirozės, kuri nustatoma apie 30 % vaikᶙ, sergančiᶙ NS, šiuo atveju pacientei nustatyta kliniškai reikšminga hipotirozė ir ryškus augimo sulėtėjimas.

Klinikinis atvejis: 5 metᶙ ir 8 mėnesiᶙ pacientė, kuriai prieš trejus metus diagnozuotas nuo steroidᶙ priklausomas NS, buvo atsiᶙsta iš kito centro dėl išliekančiᶙ edemᶙ ir sumažėjusios diurezės. Pacientė buvo gydoma mikofenolato mofetiliu (MMF) 250 mg 1 k/d. ir L-tiroksinu 50 mcg 1 k/d., nes praėjus 4 mėnesiams nuo NS pradžios jai buvo diagnozuotas hipotiroidizmas. Atvykus albuminas buvo 12,64 g/l, cholesterolis 25,64 mmol/l ir proteinurija 5 g/l. Hospitalizacijos metu taip pat buvo ryškus augimo atsilikimas: pacientės ūgis buvo 93,5 cm (-13 cm < 3 procentilio), svoris 17,2 kg (15–25 procentilio). Mergaitės ūgio atsilikimas pastebėtas nuo 3,5 mėnesio amžiaus. Ji yra gavusi didelę suminę prednizolono dozę (apie 7800 mg per vienus metus ir 8 mėnesius). Hospitalizuojant skydliaukę stimuliuojantis hormonas (TTH/TSH) buvo padidėjęs (18,04 mU/L), o sumažėję FT4 (11,43 pmol/l) ir IGF-1 < 15 µg/L. Atlikus inkstᶙ biopsiją nustatyta minimaliᶙ pokyčiᶙ liga, o genetiniu ištyrimu pokyčiᶙ nerasta. Pacientė dėl NS pradėta intensyviai gydyti metilprednizolono pulsine terapija, padidinta MMF dozė, taip pat skirtos albumino infuzijos ir padidinta L-tiroksino dozė iki 75 mcg. Po 3 savaičiᶙ išnykus edemoms mergaitė išleista į namus, o remiantis dinamika nutrauktas gydymas metilprednizolonu, normalizavusis skydliaukės funkcijai nutrauktas ir L-tiroksino vartojimas. Praėjus 2 savaitėms atliktas standartinis augimo hormono stimuliacijos mėginys su klonidinu parodė iš dalies nepakankamą augimo hormono sekreciją. Pasiekus NS remisiją, kurios metu normalizavosi skydliaukės funkcija (TSH 6,680 mU/l, FT4 13,85 pmol/l) ir IGF-1 koncentracija (132 mcg/l), buvo matomas dalinis augimo greičio prisivijimas (augimo greitis padidėjo nuo 3,5 cm/m iki 7,3 cm/m, remiantis 4 mėnesiᶙ skaičiavimais).

Išvados: Gydytojams būtina žinoti hipotirozės išsivystymo riziką ir apsvarstyti skydliaukės funkcijos tyrimᶙ skyrimo vaikams, sergantiems NS, būtinumą bei aktyviai gydyti hipotirozę ir stebėti vaikᶙ augimo greitį.

Keywords: Hypothyroidism; children; growth retardation; nephrotic syndrome.

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Figures

Fig. 1
Fig. 1
Patient’s growth chart
Fig. 2
Fig. 2
Patient’s growth velocity dynamics
Fig. 3
Fig. 3
Suggested schematic explanation of thyroid dysfunction due to urinary loss of thyroid-binding proteins in NS
See this image and copyright information in PMC

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